Idiopathic pulmonary fibrosis differential diagnosis: Difference between revisions

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==Overview==
==Overview==
==Differentiating Idiopathic Pulmoary Fibrosis from other Disease==
==Differentiating Idiopathic Pulmoary Fibrosis from other Disease==
The key issue facing clinicians is whether the presenting history, symptoms/signs, radiology, and pulmonary function testing are collectively in keeping with the diagnosis of IPF (which carries the relatively poor prognosis described above) or whether the findings are due to another process.  It has long been recognized that patients with interstitial lung disease related to [[asbestos]] exposure, [[drug]]s (particularly [[chemotherapeutic]] agents), a [[connective tissue]] disease, or other diseases may have features that are difficult to distinguish from IPF.  Important differential diagnostic considerations include asbestosis; interstitial lung disease related to [[scleroderma]], [[mixed connective tissue disease]], or [[rheumatoid arthritis]]; advanced sarcoidosis, [[hypersensitivity pneumonitis]], or [[Langerhans cell histiocytosis]]; chronic [[pulmonary aspiration]]; radiation-induced fibrosis; as well as previous therapy with [[cyclophosphamide]], [[nitrofurantoin]], [[methotrexate]], and other drugs.
==Differentiating Interstitial Lung Disease from other Diseases==
'''''To review the complete differential diagnosis of dyspnea, click here.'''''


When diagnostic uncertainty remains, a surgical lung biopsy may be required to establish the diagnosis. Generally, lung biopsy is only undertaken when it is deemed that its risks are outweighed by the potential benefits of identifying a disease process that may be amenable to a treatment that the patient would likely be able to tolerate.
'''''To review the complete differential diagnosis of hemoptysis, click here.'''''
 
'''''To review the complete differential diagnosis of restrictive lung disease, click here.'''''
 
<small>
 
'''Abbreviations''': '''ABG''': Arterial blood gas, '''BAL''': Bronchoalveolar lavage, '''ESR''': Erythrocyte sedimentation rate, '''CRP''': C–reactive protein, '''FVC''': Forced vital capacity, '''RV''': Residual volume, '''FEV1''': Forced expiratory volume during the 1st second, '''DLCO''': Diffusing capacity of the lungs for carbon monoxide, '''O2''': Oxygen, '''TLC''': Total lung capacity, '''PaO2''': Arterial partial pressure of oxygen, '''FiO2''': Fraction of inspired oxygen, '''LDH''': Lactate dehydrogenase, '''CEA''': Carcinoembryonic antigen, '''Anti-GBM antibody''': Anti-glomerular basement membrane antibody, '''A−a gradient''': Alveolar-arterial gradient, '''PAS''': Periodic acid-Schiff stain, '''LAM''': Lymphangiomyomatosis, '''IgE''': Immunoglobulin E, '''ANCA''': Anti-neutrophil cytoplasmic antibody, '''RBC''': Red blood cell, '''ACE''': Angiotensin-converting enzyme
 
<small><small>
 
{| class="wikitable"
! colspan="2" rowspan="3" style="background:#4479BA; color: #FFFFFF;" align="center" |Disease
! colspan="15" style="background:#4479BA; color: #FFFFFF;" align="center" |Clinical manifestation
! colspan="6" style="background:#4479BA; color: #FFFFFF;" align="center" |Investigations
|-
! colspan="7" style="background:#4479BA; color: #FFFFFF;" align="center" |History
! colspan="4" style="background:#4479BA; color: #FFFFFF;" align="center" |Symptoms
! colspan="4" style="background:#4479BA; color: #FFFFFF;" align="center" |Physical examination
! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Lab findings
! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Imaging
! colspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Pulmonary function test
! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Bronchoscopy and BAL
! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Gold standard
|-
! style="background:#4479BA; color: #FFFFFF;" align="center" |Duration
! style="background:#4479BA; color: #FFFFFF;" align="center" |Age
! style="background:#4479BA; color: #FFFFFF;" align="center" |Gender
! style="background:#4479BA; color: #FFFFFF;" align="center" |Family history
! style="background:#4479BA; color: #FFFFFF;" align="center" |Smoking history
! style="background:#4479BA; color: #FFFFFF;" align="center" |Environmental exposure
! style="background:#4479BA; color: #FFFFFF;" align="center" |HIV
! style="background:#4479BA; color: #FFFFFF;" align="center" |Dyspnea
! style="background:#4479BA; color: #FFFFFF;" align="center" |Cough
! style="background:#4479BA; color: #FFFFFF;" align="center" |Wheezing
! style="background:#4479BA; color: #FFFFFF;" align="center" |Chest pain
! style="background:#4479BA; color: #FFFFFF;" align="center" |Tachypnea  
! style="background:#4479BA; color: #FFFFFF;" align="center" |Auscultation
! style="background:#4479BA; color: #FFFFFF;" align="center" |Cyanosis
! style="background:#4479BA; color: #FFFFFF;" align="center" |Clubbing
! style="background:#4479BA; color: #FFFFFF;" align="center" |Spirometry
! style="background:#4479BA; color: #FFFFFF;" align="center" |ABG
|-
! colspan="2" style="background:#DCDCDC;" align="center" |Idiopathic pulmonary fibrosis<ref name="PolettiRavaglia2013">{{cite journal|last1=Poletti|first1=Venerino|last2=Ravaglia|first2=Claudia|last3=Buccioli|first3=Matteo|last4=Tantalocco|first4=Paola|last5=Piciucchi|first5=Sara|last6=Dubini|first6=Alessandra|last7=Carloni|first7=Angelo|last8=Chilosi|first8=Marco|last9=Tomassetti|first9=Sara|title=Idiopathic Pulmonary Fibrosis: Diagnosis and Prognostic Evaluation|journal=Respiration|volume=86|issue=1|year=2013|pages=5–12|issn=1423-0356|doi=10.1159/000353580}}</ref>
| align="center" |Chronic
| align="center" |60−70 years old
| align="center" |Men
| align="center" | +
| align="center" | +
| align="center" |±
| align="center" | −
| align="center" | +
| align="center" |Dry
| align="center" | +
| align="center" | +
| align="center" | +
|
* Inspiratory high−pitched [[rhonchi]]
* Bibasilar inspiratory [[Rales|crackles]]
| align="center" | −
| align="center" | +
|
* [[Anti-nuclear antibody|Antinuclear antibody]] +
* [[Rheumatoid factor]] +
* Elevated [[Erythrocyte sedimentation rate|ESR]]
* Elevated [[C-reactive protein|CRP]]
* [[Polycythemia]]
|
* Bibasilar, peripheral reticular abnormalities
* Focal honeycomb cyst formation
* Traction [[bronchiectasis]]
|
* ↓ [[Vital capacity|FVC]]
 
* ↑ [[Residual volume|RV]]
* Normal [[FEV1/FVC ratio|FEV1/FVC]]
* ↓ [[DLCO]]
|
* ↓ [[Oxygen|O2]]
|
* Not required
* ↑ [[Neutrophil|Neutrophils]] 
* ↑ [[Eosinophil granulocyte|Eosinophils]] 
|
* Diagnosis of exclusion 
* Lung [[biopsy]]
|-
! colspan="2" style="background:#DCDCDC;" align="center" s |Idiopathic nonspecific interstitial pneumonia<ref name="TravisHunninghake2008">{{cite journal|last1=Travis|first1=William D.|last2=Hunninghake|first2=Gary|last3=King|first3=Talmadge E.|last4=Lynch|first4=David A.|last5=Colby|first5=Thomas V.|last6=Galvin|first6=Jeffrey R.|last7=Brown|first7=Kevin K.|last8=Chung|first8=Man Pyo|last9=Cordier|first9=Jean-François|last10=du Bois|first10=Roland M.|last11=Flaherty|first11=Kevin R.|last12=Franks|first12=Teri J.|last13=Hansell|first13=David M.|last14=Hartman|first14=Thomas E.|last15=Kazerooni|first15=Ella A.|last16=Kim|first16=Dong Soon|last17=Kitaichi|first17=Masanori|last18=Koyama|first18=Takashi|last19=Martinez|first19=Fernando J.|last20=Nagai|first20=Sonoko|last21=Midthun|first21=David E.|last22=Müller|first22=Nestor L.|last23=Nicholson|first23=Andrew G.|last24=Raghu|first24=Ganesh|last25=Selman|first25=Moisés|last26=Wells|first26=Athol|title=Idiopathic Nonspecific Interstitial Pneumonia|journal=American Journal of Respiratory and Critical Care Medicine|volume=177|issue=12|year=2008|pages=1338–1347|issn=1073-449X|doi=10.1164/rccm.200611-1685OC}}</ref>
| align="center" |Acute/Chronic
| align="center" |50−60 years old
| align="center" |Female
| align="center" | +
| align="center" |−
| align="center" | −
| align="center" | +
| align="center" | +
| align="center" | +
| align="center" | +
| align="center" |−
| align="center" | +
|
* Bibasilar [[Rales|crackles]]
| align="center" | −
| align="center" |±
|
* Normal
|
* Bilateral ground−glass opacities 
* Fine reticular infiltrates
* Traction [[bronchiectasis]]
* [[Consolidation (medicine)|Consolidation]]
|
* Normal to ↑ [[FEV1/FVC ratio|FEV1/FVC]]
* ↓ [[Vital capacity|FVC]] 
* ↓ [[Total lung capacity|TLC]]
|
* ↓ [[Oxygen|O2]]
|
* Nonspecific
|
* Lung [[biopsy]] and multidisciplinary approach
|-
! colspan="2" style="background:#DCDCDC;" align="center" |Cryptogenic organising pneumonia<ref name="MehrianDoroudinia2017">{{cite journal|last1=Mehrian|first1=P.|last2=Doroudinia|first2=A.|last3=Rashti|first3=A.|last4=Aloosh|first4=O.|last5=Dorudinia|first5=A.|title=High-resolution computed tomography findings in chronic eosinophilic vs. cryptogenic organising pneumonia|journal=The International Journal of Tuberculosis and Lung Disease|volume=21|issue=11|year=2017|pages=1181–1186|issn=1027-3719|doi=10.5588/ijtld.16.0723}}</ref>
| align="center" |Acute/subacute
| align="center" |50−60 years old
| align="center" |Both
| align="center" |−
| align="center" |±
| align="center" |−
| align="center" |−
| align="center" | +
| align="center" |Dry
| align="center" | −
| align="center" |−
| align="center" |−
|
* Inspiratory [[Rales|crackles]]
| align="center" | −
| align="center" |−
|
* [[Leukocytosis]]
 
* Elevated [[Erythrocyte sedimentation rate|ESR]]
* Elevated [[C-reactive protein|CRP]]
|
* [[Alveolus|Alveolar]] filling and air bronchograms
* Bilateral ground−glass opacities 
* Bilateral [[Consolidation (medicine)|consolidation]]
|
* ↓ [[Vital capacity|FVC]]
 
* Normal [[FEV1/FVC ratio|FEV1/FVC]]
* ↓ [[DLCO]]
|
* ↓ [[Oxygen|O2]]
|
* ↑ [[Lymphocyte|Lymphocytes]]
 
* ↑ [[Neutrophil|Neutrophils]] 
* ↑ [[Eosinophil granulocyte|Eosinophils]] 
|
* Lung [[biopsy]]
|-
! colspan="2" rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Disease
! colspan="7" style="background:#4479BA; color: #FFFFFF;" align="center" |History
! colspan="4" style="background:#4479BA; color: #FFFFFF;" align="center" |Symptoms
! colspan="4" style="background:#4479BA; color: #FFFFFF;" align="center" |Physical examination
! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Lab findings
! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Imaging
! colspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Pulmonary function test
! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Bronchoscopy and BAL
! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Gold standard
|-
! style="background:#4479BA; color: #FFFFFF;" align="center" |Duration
! style="background:#4479BA; color: #FFFFFF;" align="center" |Age
! style="background:#4479BA; color: #FFFFFF;" align="center" |Gender
! style="background:#4479BA; color: #FFFFFF;" align="center" |Family history
! style="background:#4479BA; color: #FFFFFF;" align="center" |Smoking history
! style="background:#4479BA; color: #FFFFFF;" align="center" |Environmental exposure
! style="background:#4479BA; color: #FFFFFF;" align="center" |HIV
! style="background:#4479BA; color: #FFFFFF;" align="center" |Dyspnea
! style="background:#4479BA; color: #FFFFFF;" align="center" |Cough
! style="background:#4479BA; color: #FFFFFF;" align="center" |Wheezing
! style="background:#4479BA; color: #FFFFFF;" align="center" |Chest pain
! style="background:#4479BA; color: #FFFFFF;" align="center" |Tachypnea  
! style="background:#4479BA; color: #FFFFFF;" align="center" |Auscultation
! style="background:#4479BA; color: #FFFFFF;" align="center" |Cyanosis
! style="background:#4479BA; color: #FFFFFF;" align="center" |Clubbing
! style="background:#4479BA; color: #FFFFFF;" align="center" |Spirometry
! style="background:#4479BA; color: #FFFFFF;" align="center" |ABG
|-
! colspan="2" style="background:#DCDCDC;" align="center" |Acute interstitial pneumonia (Hamman−Rich syndrome)<ref name="ParambilMukhopadhyay2012">{{cite journal|last1=Parambil|first1=Joseph|last2=Mukhopadhyay|first2=Sanjay|title=Acute Interstitial Pneumonia (AIP): Relationship to Hamman-Rich Syndrome, Diffuse Alveolar Damage (DAD), and Acute Respiratory Distress Syndrome (ARDS)|journal=Seminars in Respiratory and Critical Care Medicine|volume=33|issue=05|year=2012|pages=476–485|issn=1069-3424|doi=10.1055/s-0032-1325158}}</ref>
| align="center" |Acute
| align="center" |50−60 years old
| align="center" |Both
| align="center" | −
| align="center" |−
| align="center" |−
| align="center" |−
| align="center" | +
| align="center" | +
| align="center" |−
| align="center" |−
| align="center" | +
|
* Diffuse [[Rales|crackles]]
| align="center" | −
| align="center" | −
|
* [[Leukocytosis]] 
|
* Bilateral and symmetric, diffuse ground glass
* [[Alveolus|Alveolar]] consolidation opacities
* Traction [[bronchiectasis]]
* Honeycomb [[fibrosis]] 
|
* N/A
|
* ↓ [[Oxygen|O2]]
* [[Pulmonary gas pressures|PaO2]]/[[FiO2]] <200 mmHg
|
* Nonspecific
* ↑ [[Neutrophil|Neutrophils]] 
* ↑ Atypical [[Epithelium|epithelial cells]]
|
* Lung [[biopsy]]
|-
! colspan="2" style="background:#DCDCDC;" align="center" |Lymphocytic interstitial pneumonia<ref name="PanchabhaiFarver2016">{{cite journal|last1=Panchabhai|first1=Tanmay S.|last2=Farver|first2=Carol|last3=Highland|first3=Kristin B.|title=Lymphocytic Interstitial Pneumonia|journal=Clinics in Chest Medicine|volume=37|issue=3|year=2016|pages=463–474|issn=02725231|doi=10.1016/j.ccm.2016.04.009}}</ref>
| align="center" |Subacute
| align="center" |30−40 years old
| align="center" |Female
| align="center" |−
| align="center" |−
| align="center" |−
| align="center" |±
| align="center" | +
| align="center" | +
| align="center" | +
| align="center" | +
| align="center" | −
|
* Diffuse [[Rales|crackles]]
| align="center" | −
| align="center" | +
|
* [[Gammopathy]]
|
* Diffuse ground glass attenuation with [[fibrosis]]
* Centrilobular and subpleural [[Nodule (medicine)|nodules]]
* Lung [[Cyst|cysts]]
|
* ↓ [[Vital capacity|FVC]]
* ↓ [[Total lung capacity|TLC]]
* ↓ [[DLCO]]
|
* ↓ [[Oxygen|O2]]
|
* Nonspecific
* ↑ Total [[Bronchoalveolar lavage|BAL]] cell count
* [[Bronchoalveolar lavage|BAL]] [[lymphocytosis]]
|
* Lung [[biopsy]]
|-
! colspan="2" style="background:#DCDCDC;" align="center" |Respiratory bronchiolitis−interstitial lung disease<ref name="SieminskaKuziemski2014">{{cite journal|last1=Sieminska|first1=Alicja|last2=Kuziemski|first2=Krzysztof|title=Respiratory bronchiolitis-interstitial lung disease|journal=Orphanet Journal of Rare Diseases|volume=9|issue=1|year=2014|issn=1750-1172|doi=10.1186/s13023-014-0106-8}}</ref>
| align="center" |Subacute
| align="center" |30−40 years old
| align="center" |Both
| align="center" |−
| align="center" | +
| align="center" |−
| align="center" | −
| align="center" | +
| align="center" |Dry
| align="center" | +
| align="center" | −
| align="center" | −
|
* Inspiratory high−pitched [[rhonchi]]
* Fine, bibasilar end−inspiratory [[Rales|crackles]]
| align="center" |−
| align="center" |−
|
* Nonspecific 
|
* Diffuse or patchy ground glass opacities in a mosaic pattern 
* Fine [[Nodule (medicine)|nodules]] 
* Air trapping
|
* ↓ [[Vital capacity|FVC]]
* ↓ [[Total lung capacity|TLC]]
* ↓ [[DLCO]]
|
* ↓ [[Oxygen|O2]]
|
* ↑ [[Macrophage|Macrophages]]
|
* Clinical evaluation and investigations
|-
! colspan="2" style="background:#DCDCDC;" align="center" |Desquamative interstitial pneumonia<ref name="RyuMyers2005">{{cite journal|last1=Ryu|first1=Jay H.|last2=Myers|first2=Jeffrey L.|last3=Capizzi|first3=Stephen A.|last4=Douglas|first4=William W.|last5=Vassallo|first5=Robert|last6=Decker|first6=Paul A.|title=Desquamative Interstitial Pneumonia and Respiratory Bronchiolitis-Associated Interstitial Lung Disease|journal=Chest|volume=127|issue=1|year=2005|pages=178–184|issn=00123692|doi=10.1378/chest.127.1.178}}</ref><ref name="CraigWells2004">{{cite journal|last1=Craig|first1=P J|last2=Wells|first2=A U|last3=Doffman|first3=S|last4=Rassl|first4=D|last5=Colby|first5=T V|last6=Hansell|first6=D M|last7=du Bois|first7=R M|last8=Nicholson|first8=A G|title=Desquamative interstitial pneumonia, respiratory bronchiolitis and their relationship to smoking|journal=Histopathology|volume=45|issue=3|year=2004|pages=275–282|issn=0309-0167|doi=10.1111/j.1365-2559.2004.01921.x}}</ref>
| align="center" |Chronic
| align="center" |40−50 years old
| align="center" |Both
| align="center" |−
| align="center" | +
| align="center" |−
| align="center" |−
| align="center" | +
| align="center" |Dry
| align="center" | +
| align="center" |−
| align="center" |−
|
* Fine, bibasilar end−inspiratory [[Rales|crackles]]
| align="center" |−
| align="center" |−
|
* Nonspecific 
|
* Ground glass opacities without the peripheral reticular and reticulonodular opacities
|
* ↓ [[DLCO]]
|
* ↓ [[Oxygen|O2]]
|
* ↑ [[Eosinophil granulocyte|Eosinophils]] 
|
* Lung [[biopsy]]
|-
! colspan="2" rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Disease
! colspan="7" style="background:#4479BA; color: #FFFFFF;" align="center" |History
! colspan="4" style="background:#4479BA; color: #FFFFFF;" align="center" |Symptoms
! colspan="4" style="background:#4479BA; color: #FFFFFF;" align="center" |Physical examination
! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Lab findings
! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Imaging
! colspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Pulmonary function test
! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Bronchoscopy and BAL
! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Gold standard
|-
! style="background:#4479BA; color: #FFFFFF;" align="center" |Duration
! style="background:#4479BA; color: #FFFFFF;" align="center" |Age
! style="background:#4479BA; color: #FFFFFF;" align="center" |Gender
! style="background:#4479BA; color: #FFFFFF;" align="center" |Family history
! style="background:#4479BA; color: #FFFFFF;" align="center" |Smoking history
! style="background:#4479BA; color: #FFFFFF;" align="center" |Environmental exposure
! style="background:#4479BA; color: #FFFFFF;" align="center" |HIV
! style="background:#4479BA; color: #FFFFFF;" align="center" |Dyspnea
! style="background:#4479BA; color: #FFFFFF;" align="center" |Cough
! style="background:#4479BA; color: #FFFFFF;" align="center" |Wheezing
! style="background:#4479BA; color: #FFFFFF;" align="center" |Chest pain
! style="background:#4479BA; color: #FFFFFF;" align="center" |Tachypnea  
! style="background:#4479BA; color: #FFFFFF;" align="center" |Auscultation
! style="background:#4479BA; color: #FFFFFF;" align="center" |Cyanosis
! style="background:#4479BA; color: #FFFFFF;" align="center" |Clubbing
! style="background:#4479BA; color: #FFFFFF;" align="center" |Spirometry
! style="background:#4479BA; color: #FFFFFF;" align="center" |ABG
|-
! colspan="2" style="background:#DCDCDC;" align="center" |Pulmonary Langerhans cell granulomatosis<ref name="BlakleyDutcher2018">{{cite journal|last1=Blakley|first1=Matthew P.|last2=Dutcher|first2=Janice P.|last3=Wiernik|first3=Peter H.|title=Pulmonary Langerhans cell histiocytosis, acute myeloid leukemia, and myelofibrosis in a large family and review of the literature|journal=Leukemia Research|volume=67|year=2018|pages=39–44|issn=01452126|doi=10.1016/j.leukres.2018.01.011}}</ref>
| align="center" |Chronic
| align="center" |20−40 years old
| align="center" |Both
| align="center" | +
| align="center" | +
| align="center" |−
| align="center" |−
| align="center" |±
| align="center" |Dry
| align="center" | +
| align="center" | +
| align="center" |−
|
* Unremarkable
|−
|−
|
* Nonspecific 
|
* Mid to upper lung zone [[Cyst|cysts]] and [[Nodule (medicine)|nodules]]
* Reticular and [[Nodule (medicine)|nodular]] opacities
* Recurrent spontaneous [[pneumothorax]]
|
* ↓ [[FEV1/FVC ratio|FEV1/FVC]]
|
* Normal
|
* >5 percent [[Langerhans cell|langerhans cells]] (CD−1a positive)
|
* Lung [[biopsy]]
|-
! colspan="2" style="background:#DCDCDC;" align="center" |Pulmonary alveolar proteinosis<ref name="pmid29493933">{{cite journal |vauthors=Carrington JM, Hershberger DM |title= |journal= |volume= |issue= |pages= |date= |pmid=29493933 |doi= |url=}}</ref><ref name="KianiParsa2018">{{cite journal|last1=Kiani|first1=Arda|last2=Parsa|first2=Tahereh|last3=Adimi Naghan|first3=Parisa|last4=Dutau|first4=Hervé|last5=Razavi|first5=Fatemeh|last6=Farzanegan|first6=Behrooz|last7=Pourabdollah Tootkaboni|first7=Mahsa|last8=Abedini|first8=Atefeh|title=An eleven-year retrospective cross-sectional study on pulmonary alveolar proteinosis|journal=Advances in Respiratory Medicine|volume=86|issue=1|year=2018|pages=7–12|issn=2543-6031|doi=10.5603/ARM.2018.0003}}</ref>
| align="center" |Acute/chronic
| align="center" |40−50 years old
| align="center" |Male
| align="center" | +
| align="center" | +
| align="center" | +
| align="center" |−
| align="center" | +
| align="center" | +
| align="center" | +
| align="center" |−
| align="center" |−
|
*  Inspiratory [[Rales|crackles]]
| align="center" | +
| align="center" | +
|
* ↑ [[Lactate dehydrogenase|LDH]]
* ↑ [[CEA]]
* ↑ [[Surfactant]] protein A, B, and D
* [[Anti-glomerular basement membrane antibody|Anti-GBM antibody]] +
* [[Polycythemia]]
* [[Hypergammaglobulinemia]]
|
* Bbilateral perihilar and basilar [[Alveolus|alveolar]] opacities without air−bronchograms
* "Bat wing" distribution
* Intralobular thickening
* Diffuse ground−glass opacities
|
* ↑ [[FEV1/FVC ratio|FEV1/FVC]]
* ↑ [[A-a gradient|A−a gradient]]
* ↓ [[DLCO]]
|
* ↓ [[Pulmonary gas pressures|PaO2]]
* ↓ [[Oxygen|O2]]
* [[Respiratory alkalosis]]
|
* Large foamy [[Macrophage|macrophages]] with amorphous [[Periodic acid-Schiff stain|PAS]]−positive material
* ↑  [[Lymphocyte|Lymphocytes]]
|
* [[Bronchoscopy]] and [[Bronchoalveolar lavage|BAL]]
|-
! colspan="2" style="background:#DCDCDC;" align="center" |Pulmonary lymphangioleiomyomatosis<ref name="XuLo2014">{{cite journal|last1=Xu|first1=Kai-Feng|last2=Lo|first2=Bee Hong|title=Lymphangioleiomyomatosis: differential diagnosis and optimal management|journal=Therapeutics and Clinical Risk Management|year=2014|pages=691|issn=1178-203X|doi=10.2147/TCRM.S50784}}</ref>
| align="center" |Acute/chronic
| align="center" |30−40 years old
| align="center" |Female
| align="center" | +
| align="center" | +
| align="center" |−
| align="center" |−
| align="center" | +
| align="center" |Bloody
| align="center" | +
| align="center" | +
| align="center" |−
|
* Decreased [[breath sounds]]
| align="center" |−
| align="center" | +
|
* ↑ [[Vascular endothelial growth factor|Vascular endothelial growth factor−D]] (VEGF−D)
|
* [[Pneumothorax]]
* [[Chylothorax]]
* Thin−walled round [[Cyst|cystic]] lesions
|
* ↓ [[FEV1/FVC ratio|FEV1/FVC]]
|
* ↓ [[Pulmonary gas pressures|PaO2]]
* ↓ [[Oxygen|O2]]
|
* [[LAM]] cells +
|
* Lung [[biopsy]]
|-
! colspan="2" style="background:#DCDCDC;" align="center" |Eosinophilic pneumonia<ref name="BernheimMcLoud2017">{{cite journal|last1=Bernheim|first1=Adam|last2=McLoud|first2=Theresa|title=A Review of Clinical and Imaging Findings in Eosinophilic Lung Diseases|journal=American Journal of Roentgenology|volume=208|issue=5|year=2017|pages=1002–1010|issn=0361-803X|doi=10.2214/AJR.16.17315}}</ref>
| align="center" |Acute/chronic
| align="center" |20−40 years old
| align="center" |Male
| align="center" |−
| align="center" |−
| align="center" |−
| align="center" |−
| align="center" | +
| align="center" |Dry
| align="center" | +
| align="center" | +
| align="center" | +
|
* Decreased [[breath sounds]]
| align="center" |−
| align="center" |−
|
* Neutrophilic [[leukocytosis]]
* ↑ [[Eosinophil granulocyte|Eosinophils]]
* Elevated [[Erythrocyte sedimentation rate|ESR]]
* Elevated [[C-reactive protein|CRP]]
* Elevated [[Immunoglobulin E|IgE]] level
|
* Bilateral diffuse mixed ground glass and reticular opacities
* Small bilateral [[Pleural effusion|pleural effusions]]
* Centrilobular [[Nodule (medicine)|nodules]] and air−space [[Consolidation (medicine)|consolidation]]
|
* ↑ [[FEV1/FVC ratio|FEV1/FVC]]
* ↓ [[DLCO]]
|
* ↓ [[Pulmonary gas pressures|PaO2]]
* ↓ [[Oxygen|O2]]
|
* [[Eosinophilia]] 
|
* Clinical evaluation and investigations
|-
! colspan="2" style="background:#DCDCDC;" align="center" |Hypersensitivity pneumonitis<ref name="MillerAllen2018">{{cite journal|last1=Miller|first1=Ross|last2=Allen|first2=Timothy Craig|last3=Barrios|first3=Roberto J.|last4=Beasley|first4=Mary Beth|last5=Burke|first5=Louise|last6=Cagle|first6=Philip T.|last7=Capelozzi|first7=Vera Luiza|last8=Ge|first8=Yimin|last9=Hariri|first9=Lida P.|last10=Kerr|first10=Keith M.|last11=Khoor|first11=Andras|last12=Larsen|first12=Brandon T.|last13=Mark|first13=Eugene J.|last14=Matsubara|first14=Osamu|last15=Mehrad|first15=Mitra|last16=Mino-Kenudson|first16=Mari|last17=Raparia|first17=Kirtee|last18=Roden|first18=Anja Christiane|last19=Russell|first19=Prudence|last20=Schneider|first20=Frank|last21=Sholl|first21=Lynette M.|last22=Smith|first22=Maxwell Lawrence|title=Hypersensitivity Pneumonitis A Perspective From Members of the Pulmonary Pathology Society|journal=Archives of Pathology & Laboratory Medicine|volume=142|issue=1|year=2018|pages=120–126|issn=0003-9985|doi=10.5858/arpa.2017-0138-SA}}</ref>
| align="center" |Acute/subacute/chronic
| align="center" |40−60 years old
| align="center" |Both
| align="center" |−
| align="center" |±
| align="center" | +
| align="center" | −
| align="center" | +
| align="center" |Dry/productive
| align="center" | +
| align="center" | +
| align="center" | +
|
* Diffuse fine bibasilar [[crackles]]
| align="center" | −
| align="center" | +
|
* Neutrophilic [[leukocytosis]]
|
* Centrilobular ground−glass or nodular opacities of mid−to−upper zone 
* Air−trapping
|
* ↓ [[FEV1]]
* ↓ [[Vital capacity|FVC]]
|
* ↓ [[Pulmonary gas pressures|PaO2]]
* ↓ [[Oxygen|O2]]
|
* [[Lymphocytosis]]
|
* Lung [[biopsy]]
|-
! colspan="2" rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Disease
! colspan="7" style="background:#4479BA; color: #FFFFFF;" align="center" |History
! colspan="4" style="background:#4479BA; color: #FFFFFF;" align="center" |Symptoms
! colspan="4" style="background:#4479BA; color: #FFFFFF;" align="center" |Physical examination
! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Lab findings
! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Imaging
! colspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Pulmonary function test
! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Bronchoscopy and BAL
! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Gold standard
|-
! style="background:#4479BA; color: #FFFFFF;" align="center" |Duration
! style="background:#4479BA; color: #FFFFFF;" align="center" |Age
! style="background:#4479BA; color: #FFFFFF;" align="center" |Gender
! style="background:#4479BA; color: #FFFFFF;" align="center" |Family history
! style="background:#4479BA; color: #FFFFFF;" align="center" |Smoking history
! style="background:#4479BA; color: #FFFFFF;" align="center" |Environmental exposure
! style="background:#4479BA; color: #FFFFFF;" align="center" |HIV
! style="background:#4479BA; color: #FFFFFF;" align="center" |Dyspnea
! style="background:#4479BA; color: #FFFFFF;" align="center" |Cough
! style="background:#4479BA; color: #FFFFFF;" align="center" |Wheezing
! style="background:#4479BA; color: #FFFFFF;" align="center" |Chest pain
! style="background:#4479BA; color: #FFFFFF;" align="center" |Tachypnea  
! style="background:#4479BA; color: #FFFFFF;" align="center" |Auscultation
! style="background:#4479BA; color: #FFFFFF;" align="center" |Cyanosis
! style="background:#4479BA; color: #FFFFFF;" align="center" |Clubbing
! style="background:#4479BA; color: #FFFFFF;" align="center" |Spirometry
! style="background:#4479BA; color: #FFFFFF;" align="center" |ABG
|-
! colspan="2" style="background:#DCDCDC;" align="center" |Occupational lung disease<ref name="SirajuddinKanne2009">{{cite journal|last1=Sirajuddin|first1=Arlene|last2=Kanne|first2=Jeffrey P.|title=Occupational Lung Disease|journal=Journal of Thoracic Imaging|volume=24|issue=4|year=2009|pages=310–320|issn=0883-5993|doi=10.1097/RTI.0b013e3181c1a9b3}}</ref>
| align="center" |Chronic
| align="center" |Elderly
| align="center" |Male
| align="center" | +
| align="center" | +
| align="center" | +
| align="center" |−
| align="center" |±
| align="center" | +
| align="center" | +
| align="center" | +
| align="center" | +
|
* Fine [[Rales|crackles]]
| align="center" |Peripheral/central
| align="center" | +
|
* [[Anemia]]
* [[Neutrophilia]]
* Elevated [[Erythrocyte sedimentation rate|ESR]]
* Elevated [[C-reactive protein|CRP]]
* Elevated [[Antibody|immunoglobulin]]
|
* Pleural thickening and [[Plaque|plaques]]
* [[Calcification]]
* Nodular or reticular opacities
* Lobar consolidation
* [[Atelectasis]]
* Parenchymal bands
* Enlarged hilar or mediastinal [[Lymph node|lymph nodes]]
* Granulomata
|
* ↑ [[FEV1/FVC ratio|FEV1/FVC]]
|
* ↓ [[Oxygen|O2]]
* ↑ CO2
* [[Respiratory acidosis]]
|
* Mineral dust +
|
* History of environmental exposure and imaging
* Lung [[biopsy]] not required
|-
! colspan="2" style="background:#DCDCDC;" align="center" |Radiation−induced lung injury<ref name="pmid25854336">{{cite journal |vauthors=Giridhar P, Mallick S, Rath GK, Julka PK |title=Radiation induced lung injury: prediction, assessment and management |journal=Asian Pac. J. Cancer Prev. |volume=16 |issue=7 |pages=2613–7 |date=2015 |pmid=25854336 |doi= |url=}}</ref>
| align="center" |Subacute/chronic
| align="center" |Any age
| align="center" |Both
| align="center" |−
| align="center" |−
| align="center" | +
| align="center" |−
| align="center" | +
| align="center" |Dry
| align="center" | +
| align="center" | +
| align="center" | +
|
* [[Rales|Crackles]]
* [[Pleural friction rub|Pleural rub]]
* Dullness to [[percussion]]
| align="center" | +
| align="center" |−
|
* Nonspecific
|
* Perivascular haziness to patchy [[Alveolus|alveolar]] filling densities
* Straight line effect
* [[Pleural effusion]]
|
* ↓ [[Total lung capacity|TLC]]
* ↓ [[Vital capacity|FVC]]
* ↓ [[Spirometry|FEV1]]
* ↓ [[DLCO]]
|
* ↓ [[Pulmonary gas pressures|PaO2]]
* ↓ [[Oxygen|O2]]
|
* [[Lymphocytosis]]
|
* History of irradiation and clinical presentation
|-
! rowspan="3" style="background:#DCDCDC;" align="center" |Pulmonary hemorrhage syndromes
! style="background:#DCDCDC;" align="center" |Goodpasture syndrome<ref name="pmid29083697">{{cite journal |vauthors=DeVrieze BW, Hurley JA |title= |journal= |volume= |issue= |pages= |date= |pmid=29083697 |doi= |url=}}</ref>
| align="center" |Chronic
| align="center" |All ages
| align="center" |Male
| align="center" | +
| align="center" |±
| align="center" | −
| align="center" |−
| align="center" |±
| align="center" |Bloody
| align="center" |±
| align="center" |−
| align="center" |−
|
* Bilateral coarse [[crepitations]]
| align="center" |−
| align="center" |−
|
* [[Anti-glomerular basement membrane antibody|Anti-GBM antibody]] +
* [[Anti-neutrophil cytoplasmic antibody|ANCA]] +
* [[Anemia]]
* [[Red blood cell|RBC]] in the urine
|
* Pulmonary infiltrates
|
* ↑ [[DLCO]]
|
* Normal
|
* NA
|
* Kidney [[biopsy]]
|-
! style="background:#DCDCDC;" align="center" |Idiopathic pulmonary hemosiderosis<ref name="KhorashadiWu2015">{{cite journal|last1=Khorashadi|first1=L.|last2=Wu|first2=C.C.|last3=Betancourt|first3=S.L.|last4=Carter|first4=B.W.|title=Idiopathic pulmonary haemosiderosis: spectrum of thoracic imaging findings in the adult patient|journal=Clinical Radiology|volume=70|issue=5|year=2015|pages=459–465|issn=00099260|doi=10.1016/j.crad.2014.11.007}}</ref>
| align="center" |Acute/subacute/chronic
| align="center" |Children − 10 years old
| align="center" |Both
| align="center" | +
| align="center" |±
| align="center" | −
| align="center" |−
| align="center" | +
| align="center" |Bloody
| align="center" | +
| align="center" | +
| align="center" |−
|
* [[Rales|Crackles]]
| align="center" | −
| align="center" |−
|
* [[Iron deficiency anemia]]
* ↑ Plasma [[bilirubin]]
* ↑ Urinary excretion of [[urobilinogen]]
* ↑ [[Reticulocytes]]
* [[Fecal occult blood]] +
|
* Mid to lower zone [[Alveolus|alveolar]] opacities
* Multiple honeycomb [[Cyst|cysts]]
|
* ↓ [[Lung volumes|TLC]]
* ↓ [[Vital capacity|FVC]]
* ↓ [[Spirometry|FEV1]]
* ↑ [[FEV1/FVC ratio|FEV1/FVC]]
* ↑ [[DLCO]]
|
* ↓ [[Oxygen|O2]]
* ↓ CO2
|
* ↑  [[Hemosiderin]]−laden [[Macrophage|macrophages]]
|
* Clinical evaluation and investigations
|-
! style="background:#DCDCDC;" align="center" |Isolated pulmonary capillaritis<ref name="ThompsonKlecka2016">{{cite journal|last1=Thompson|first1=Gwen|last2=Klecka|first2=Mary|last3=Roden|first3=Anja C.|last4=Specks|first4=Ulrich|last5=Cartin-Ceba|first5=Rodrigo|title=Biopsy-proven pulmonary capillaritis: A retrospective study of aetiologies including an in-depth look at isolated pulmonary capillaritis|journal=Respirology|volume=21|issue=4|year=2016|pages=734–738|issn=13237799|doi=10.1111/resp.12738}}</ref>
| align="center" |Chronic
| align="center" |40−60 years old
| align="center" |Both
| align="center" | +
| align="center" |−
| align="center" |±
| align="center" | −
| align="center" | +
| align="center" |Bloody
| align="center" | +
| align="center" | +
| align="center" | +
|
* Decreased [[breath sounds]]
| align="center" |−
| align="center" |−
|
* [[Anemia]]
* [[Leukocytosis]]
|
* Diffuse [[Alveolus|alveolar]] haemorrhage
|
* ↓ [[FEV1/FVC ratio|FEV1/FVC]]
|
* ↓ [[Oxygen|O2]]
|
* Diffuse [[Alveolus|alveolar]] haemorrhage
|
* Diagnosis of exclusion
|-
! colspan="2" rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Disease
! colspan="7" style="background:#4479BA; color: #FFFFFF;" align="center" |History
! colspan="4" style="background:#4479BA; color: #FFFFFF;" align="center" |Symptoms
! colspan="4" style="background:#4479BA; color: #FFFFFF;" align="center" |Physical examination
! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Lab findings
! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Imaging
! colspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Pulmonary function test
! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Bronchoscopy and BAL
! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Gold standard
|-
! style="background:#4479BA; color: #FFFFFF;" align="center" |Duration
! style="background:#4479BA; color: #FFFFFF;" align="center" |Age
! style="background:#4479BA; color: #FFFFFF;" align="center" |Gender
! style="background:#4479BA; color: #FFFFFF;" align="center" |Family history
! style="background:#4479BA; color: #FFFFFF;" align="center" |Smoking history
! style="background:#4479BA; color: #FFFFFF;" align="center" |Environmental exposure
! style="background:#4479BA; color: #FFFFFF;" align="center" |HIV
! style="background:#4479BA; color: #FFFFFF;" align="center" |Dyspnea
! style="background:#4479BA; color: #FFFFFF;" align="center" |Cough
! style="background:#4479BA; color: #FFFFFF;" align="center" |Wheezing
! style="background:#4479BA; color: #FFFFFF;" align="center" |Chest pain
! style="background:#4479BA; color: #FFFFFF;" align="center" |Tachypnea  
! style="background:#4479BA; color: #FFFFFF;" align="center" |Auscultation
! style="background:#4479BA; color: #FFFFFF;" align="center" |Cyanosis
! style="background:#4479BA; color: #FFFFFF;" align="center" |Clubbing
! style="background:#4479BA; color: #FFFFFF;" align="center" |Spirometry
! style="background:#4479BA; color: #FFFFFF;" align="center" |ABG
|-
! colspan="2" style="background:#DCDCDC;" align="center" |Sarcoidosis<ref name="LiTao2018">{{cite journal|last1=Li|first1=Cheng-Wei|last2=Tao|first2=Ru-Jia|last3=Zou|first3=Dan-Feng|last4=Li|first4=Man-Hui|last5=Xu|first5=Xin|last6=Cao|first6=Wei-Jun|title=Pulmonary sarcoidosis with and without extrapulmonary involvement: a cross-sectional and observational study in China|journal=BMJ Open|volume=8|issue=2|year=2018|pages=e018865|issn=2044-6055|doi=10.1136/bmjopen-2017-018865}}</ref>
| align="center" |Acute/subacute/chronic
| align="center" |20−40 years old
| align="center" |Female
| align="center" | +
| align="center" |±
| align="center" | −
| align="center" | −
| align="center" |±
| align="center" | +
| align="center" | +
| align="center" |±
| align="center" | −
|
* [[Rales|Crackles]]
* [[Wheeze|Wheezing]]
* Decreased [[breath sounds]]
| align="center" | +
| align="center" | −
|
* [[Hypercalciuria]] 
* [[Hypercalcemia]]
* High [[Angiotensin-converting enzyme|ACE]]
* [[Hypergammaglobulinemia]]
|
* [[Hilar lymphadenopathy|Hilar adenopathy]]
* Reticular opacities
* [[Pneumothorax]]
* [[Pleural cavity|Pleural]] thickening
* [[Chylothorax]]
* [[Pulmonary hypertension]]
|
* ↓ [[Lung volumes|TLC]]
* ↓ [[Vital capacity|FVC]]
* ↓ [[Spirometry|FEV1]]
* ↑ [[FEV1/FVC ratio|FEV1/FVC]]
* ↓ [[DLCO]]
|
* ↓ [[Oxygen|O2]]
* ↓ CO2
* [[Respiratory acidosis]]
|
* [[Lymphocytosis]] 
* Elevated [[adenosine deaminase]]
* [[D-dimer]] +
|
* Clinical evaluation and investigations
|-
! rowspan="2" style="background:#DCDCDC;" align="center" |Granulomatous vasculitides
! style="background:#DCDCDC;" align="center" |Granulomatosis with polyangiitis (Wegener)<ref name="pmid26684637">{{cite journal |vauthors=Greco A, Marinelli C, Fusconi M, Macri GF, Gallo A, De Virgilio A, Zambetti G, de Vincentiis M |title=Clinic manifestations in granulomatosis with polyangiitis |journal=Int J Immunopathol Pharmacol |volume=29 |issue=2 |pages=151–9 |date=June 2016 |pmid=26684637 |pmc=5806708 |doi=10.1177/0394632015617063 |url=}}</ref>
| align="center" |Chronic
| align="center" |Elderly
| align="center" |Both
| align="center" | +
| align="center" | −
| align="center" | −
| align="center" | −
| align="center" | +
| align="center" | +
| align="center" | +
| align="center" |±
| align="center" | −
|
* [[Rales|Crackles]]
| align="center" | −
| align="center" | −
|
* [[Anti-neutrophil cytoplasmic antibody|ANCA]] +
* [[Anemia]]
* [[Leukocytosis]]
* [[Thrombocytosis]]
* Elevated [[Erythrocyte sedimentation rate|ESR]]
* Elevated [[C-reactive protein|CRP]]
* Elevated [[creatinine]]
* Urine [[protein]] +
* [[Hematuria]] 
|
* Cavitate [[Nodule (medicine)|nodules]]
* Ground−glass opacity
* [[Consolidation (medicine)|Consolidation]]
* [[Pleural effusion]]
* [[Hilar lymphadenopathy|Hilar adenopathy]]
|
* ↓ [[FEV1/FVC ratio|FEV1/FVC]]
|
* ↓ [[Oxygen|O2]]
|
* Alveolar hemorrhage
|
* Lung [[biopsy]]
|-
! style="background:#DCDCDC;" align="center" |Eosinophilic granulomatosis with polyangiitis (Churg Strauss)<ref name="pmid25500434">{{cite journal |vauthors=Greco A, Rizzo MI, De Virgilio A, Gallo A, Fusconi M, Ruoppolo G, Altissimi G, De Vincentiis M |title=Churg-Strauss syndrome |journal=Autoimmun Rev |volume=14 |issue=4 |pages=341–8 |date=April 2015 |pmid=25500434 |doi=10.1016/j.autrev.2014.12.004 |url=}}</ref>
| align="center" |Chronic
| align="center" |40−50 years old
| align="center" |Both
| align="center" | +
| align="center" | −
| align="center" | −
| align="center" | −
| align="center" | −
| align="center" | +
| align="center" | +
| align="center" | −
| align="center" | −
|
* Scattered [[Wheeze|wheezing]]
| align="center" | −
| align="center" | −
|
* [[Eosinophilia]]
* Elevated [[Immunoglobulin E|IgE]] titers
* [[Anti-neutrophil cytoplasmic antibody|ANCA]] +
|
* Areas of parenchymal opacification
* Mixed interstitial patchy [[Alveolus|alveolar]] opacities
|
* ↓ [[Lung volumes|TLC]]
 
* ↑ [[Residual volume|RV]]
|
* Normal
|
* [[Eosinophilia]] 
|
* Lung [[biopsy]]
|-
! colspan="2" style="background:#DCDCDC;" align="center" |Bronchocentric granulomatosis<ref name="Myers1989">{{cite journal|last1=Myers|first1=Jeffrey L.|title=Bronchocentric Granulomatosis|journal=Chest|volume=96|issue=1|year=1989|pages=3–4|issn=00123692|doi=10.1378/chest.96.1.3}}</ref>
| align="center" |Chronic
| align="center" |30−70 years old
| align="center" |Both
| align="center" | −
| align="center" |−
| align="center" |−
| align="center" |−
| align="center" |±
| align="center" |±
| align="center" | +
| align="center" |±
| align="center" | −
|
* Scattered [[Wheeze|wheezing]]
| align="center" | −
| align="center" |−
|
* [[Eosinophilia]]
* Elevated [[Immunoglobulin E|IgE]] titers
* Elevated circulating IgE antibodies to [[Aspergillus]] species
|
* Upper zone single or multiple pulmonary [[Nodule (medicine)|nodules]] 
* [[Consolidation (medicine)|Consolidation]]
* [[Atelectasis]]
|
* ↓ [[FEV1/FVC ratio|FEV1/FVC]]
|
* Normal
|
* [[Eosinophilia]] 
|
* Lung [[biopsy]]
|-
! colspan="2" style="background:#DCDCDC;" align="center" |Pulmonary lymphomatoid granulomatosis<ref name="AnkitaShashi2016">{{cite journal|last1=Ankita|first1=Grover|last2=Shashi|first2=Dhawan|title=Pulmonary Lymphomatoid Granulomatosis- a Case Report with Review of Literature|journal=Indian Journal of Surgical Oncology|volume=7|issue=4|year=2016|pages=484–487|issn=0975-7651|doi=10.1007/s13193-016-0525-1}}</ref>
| align="center" |Chronic
| align="center" |30−50 years old
| align="center" |Male
| align="center" | −
| align="center" |−
| align="center" |−
| align="center" |−
| align="center" | +
| align="center" | +
| align="center" | +
| align="center" | +
| align="center" |−
|
* Normal
| align="center" |−
| align="center" |−
|
* [[Epstein Barr virus|Epstein-Barr virus]] (EBV) serology +
|
* Mid to lower zone multiple poorly defined [[Nodule (medicine)|nodules]]
* Diffuse reticular abnormalities
|
* Normal
|
* ↓ [[Oxygen|O2]]
* Chronic [[respiratory alkalosis]]
|
* Normal
|
* Lung [[biopsy]]
|-
! colspan="2" style="background:#DCDCDC;" align="center" |Amyloidosis<ref name="KhoorColby2017">{{cite journal|last1=Khoor|first1=Andras|last2=Colby|first2=Thomas V.|title=Amyloidosis of the Lung|journal=Archives of Pathology & Laboratory Medicine|volume=141|issue=2|year=2017|pages=247–254|issn=0003-9985|doi=10.5858/arpa.2016-0102-RA}}</ref><ref name="MilaniBasset2017">{{cite journal|last1=Milani|first1=Paolo|last2=Basset|first2=Marco|last3=Russo|first3=Francesca|last4=Foli|first4=Andrea|last5=Palladini|first5=Giovanni|last6=Merlini|first6=Giampaolo|title=The lung in amyloidosis|journal=European Respiratory Review|volume=26|issue=145|year=2017|pages=170046|issn=0905-9180|doi=10.1183/16000617.0046-2017}}</ref>
| align="center" |Subacute/chronic
| align="center" |50−70 years old
| align="center" |Male
| align="center" | +
| align="center" | −
| align="center" | −
| align="center" | −
| align="center" | −
| align="center" | Bloody
| align="center" | +
| align="center" | −
| align="center" | −
|
* [[Rales|Crackles]]
| align="center" | −
| align="center" | −
|
* Congophilia with apple−green birefringence under polarized light
|
* Tracheobronchial infiltration
* Persistent [[Pleural effusion|pleural effusions]]
* Parenchymal [[Nodule (medicine)|nodules]]
|
* ↓ [[FEV1/FVC ratio|FEV1/FVC]]
|
* ↓ [[Oxygen|O2]]
|
* Normal
|
* Lung [[biopsy]]
|-
! colspan="2" rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Disease
! colspan="7" style="background:#4479BA; color: #FFFFFF;" align="center" |History
! colspan="4" style="background:#4479BA; color: #FFFFFF;" align="center" |Symptoms
! colspan="4" style="background:#4479BA; color: #FFFFFF;" align="center" |Physical examination
! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Lab findings
! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Imaging
! colspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Pulmonary function test
! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Bronchoscopy and BAL
! rowspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" |Gold standard
|-
! style="background:#4479BA; color: #FFFFFF;" align="center" |Duration
! style="background:#4479BA; color: #FFFFFF;" align="center" |Age
! style="background:#4479BA; color: #FFFFFF;" align="center" |Gender
! style="background:#4479BA; color: #FFFFFF;" align="center" |Family history
! style="background:#4479BA; color: #FFFFFF;" align="center" |Smoking history
! style="background:#4479BA; color: #FFFFFF;" align="center" |Environmental exposure
! style="background:#4479BA; color: #FFFFFF;" align="center" |HIV
! style="background:#4479BA; color: #FFFFFF;" align="center" |Dyspnea
! style="background:#4479BA; color: #FFFFFF;" align="center" |Cough
! style="background:#4479BA; color: #FFFFFF;" align="center" |Wheezing
! style="background:#4479BA; color: #FFFFFF;" align="center" |Chest pain
! style="background:#4479BA; color: #FFFFFF;" align="center" |Tachypnea  
! style="background:#4479BA; color: #FFFFFF;" align="center" |Auscultation
! style="background:#4479BA; color: #FFFFFF;" align="center" |Cyanosis
! style="background:#4479BA; color: #FFFFFF;" align="center" |Clubbing
! style="background:#4479BA; color: #FFFFFF;" align="center" |Spirometry
! style="background:#4479BA; color: #FFFFFF;" align="center" |ABG
|}
</small></small>


The 2002 American Thoracic Society/European Respiratory Society Consensus Guidelines on the Idiopathic Interstitial Pneumonias have formalized criteria for situations in which it is possible to establish the diagnosis of IPF without a lung biopsy.
==References==
==References==
{{reflist|2}}
{{reflist|2}}

Revision as of 00:12, 15 March 2018

Idiopathic pulmonary fibrosis Microchapters

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Differentiating Idiopathic Pulmoary Fibrosis from other Disease

Differentiating Interstitial Lung Disease from other Diseases

To review the complete differential diagnosis of dyspnea, click here.

To review the complete differential diagnosis of hemoptysis, click here.

To review the complete differential diagnosis of restrictive lung disease, click here.

Abbreviations: ABG: Arterial blood gas, BAL: Bronchoalveolar lavage, ESR: Erythrocyte sedimentation rate, CRP: C–reactive protein, FVC: Forced vital capacity, RV: Residual volume, FEV1: Forced expiratory volume during the 1st second, DLCO: Diffusing capacity of the lungs for carbon monoxide, O2: Oxygen, TLC: Total lung capacity, PaO2: Arterial partial pressure of oxygen, FiO2: Fraction of inspired oxygen, LDH: Lactate dehydrogenase, CEA: Carcinoembryonic antigen, Anti-GBM antibody: Anti-glomerular basement membrane antibody, A−a gradient: Alveolar-arterial gradient, PAS: Periodic acid-Schiff stain, LAM: Lymphangiomyomatosis, IgE: Immunoglobulin E, ANCA: Anti-neutrophil cytoplasmic antibody, RBC: Red blood cell, ACE: Angiotensin-converting enzyme

Disease Clinical manifestation Investigations
History Symptoms Physical examination Lab findings Imaging Pulmonary function test Bronchoscopy and BAL Gold standard
Duration Age Gender Family history Smoking history Environmental exposure HIV Dyspnea Cough Wheezing Chest pain Tachypnea   Auscultation Cyanosis Clubbing Spirometry ABG
Idiopathic pulmonary fibrosis[1] Chronic 60−70 years old Men + + ± + Dry + + + +
  • Bibasilar, peripheral reticular abnormalities
  • Focal honeycomb cyst formation
  • Traction bronchiectasis
  • Diagnosis of exclusion 
  • Lung biopsy
Idiopathic nonspecific interstitial pneumonia[2] Acute/Chronic 50−60 years old Female + + + + + + ±
  • Normal
  • Nonspecific
  • Lung biopsy and multidisciplinary approach
Cryptogenic organising pneumonia[3] Acute/subacute 50−60 years old Both ± + Dry
Disease History Symptoms Physical examination Lab findings Imaging Pulmonary function test Bronchoscopy and BAL Gold standard
Duration Age Gender Family history Smoking history Environmental exposure HIV Dyspnea Cough Wheezing Chest pain Tachypnea   Auscultation Cyanosis Clubbing Spirometry ABG
Acute interstitial pneumonia (Hamman−Rich syndrome)[4] Acute 50−60 years old Both + + +
  • N/A
Lymphocytic interstitial pneumonia[5] Subacute 30−40 years old Female ± + + + + +
Respiratory bronchiolitis−interstitial lung disease[6] Subacute 30−40 years old Both + + Dry +
  • Inspiratory high−pitched rhonchi
  • Fine, bibasilar end−inspiratory crackles
  • Nonspecific 
  • Diffuse or patchy ground glass opacities in a mosaic pattern 
  • Fine nodules 
  • Air trapping
  • Clinical evaluation and investigations
Desquamative interstitial pneumonia[7][8] Chronic 40−50 years old Both + + Dry +
  • Fine, bibasilar end−inspiratory crackles
  • Nonspecific 
  • Ground glass opacities without the peripheral reticular and reticulonodular opacities
Disease History Symptoms Physical examination Lab findings Imaging Pulmonary function test Bronchoscopy and BAL Gold standard
Duration Age Gender Family history Smoking history Environmental exposure HIV Dyspnea Cough Wheezing Chest pain Tachypnea   Auscultation Cyanosis Clubbing Spirometry ABG
Pulmonary Langerhans cell granulomatosis[9] Chronic 20−40 years old Both + + ± Dry + +
  • Unremarkable
  • Nonspecific 
  • Normal
Pulmonary alveolar proteinosis[10][11] Acute/chronic 40−50 years old Male + + + + + + + +
  • Bbilateral perihilar and basilar alveolar opacities without air−bronchograms
  • "Bat wing" distribution
  • Intralobular thickening
  • Diffuse ground−glass opacities
Pulmonary lymphangioleiomyomatosis[12] Acute/chronic 30−40 years old Female + + + Bloody + + +
Eosinophilic pneumonia[13] Acute/chronic 20−40 years old Male + Dry + + +
  • Clinical evaluation and investigations
Hypersensitivity pneumonitis[14] Acute/subacute/chronic 40−60 years old Both ± + + Dry/productive + + + +
  • Centrilobular ground−glass or nodular opacities of mid−to−upper zone 
  • Air−trapping
Disease History Symptoms Physical examination Lab findings Imaging Pulmonary function test Bronchoscopy and BAL Gold standard
Duration Age Gender Family history Smoking history Environmental exposure HIV Dyspnea Cough Wheezing Chest pain Tachypnea   Auscultation Cyanosis Clubbing Spirometry ABG
Occupational lung disease[15] Chronic Elderly Male + + + ± + + + + Peripheral/central +
  • Mineral dust +
  • History of environmental exposure and imaging
  • Lung biopsy not required
Radiation−induced lung injury[16] Subacute/chronic Any age Both + + Dry + + + +
  • Nonspecific
  • History of irradiation and clinical presentation
Pulmonary hemorrhage syndromes Goodpasture syndrome[17] Chronic All ages Male + ± ± Bloody ±
  • Pulmonary infiltrates
  • Normal
  • NA
Idiopathic pulmonary hemosiderosis[18] Acute/subacute/chronic Children − 10 years old Both + ± + Bloody + +
  • O2
  • ↓ CO2
  • Clinical evaluation and investigations
Isolated pulmonary capillaritis[19] Chronic 40−60 years old Both + ± + Bloody + + +
  • Diagnosis of exclusion
Disease History Symptoms Physical examination Lab findings Imaging Pulmonary function test Bronchoscopy and BAL Gold standard
Duration Age Gender Family history Smoking history Environmental exposure HIV Dyspnea Cough Wheezing Chest pain Tachypnea   Auscultation Cyanosis Clubbing Spirometry ABG
Sarcoidosis[20] Acute/subacute/chronic 20−40 years old Female + ± ± + + ± +
  • Clinical evaluation and investigations
Granulomatous vasculitides Granulomatosis with polyangiitis (Wegener)[21] Chronic Elderly Both + + + + ±
  • Alveolar hemorrhage
Eosinophilic granulomatosis with polyangiitis (Churg Strauss)[22] Chronic 40−50 years old Both + + +
  • Areas of parenchymal opacification
  • Mixed interstitial patchy alveolar opacities
  • Normal
Bronchocentric granulomatosis[23] Chronic 30−70 years old Both ± ± + ±
  • Normal
Pulmonary lymphomatoid granulomatosis[24] Chronic 30−50 years old Male + + + +
  • Normal
  • Mid to lower zone multiple poorly defined nodules
  • Diffuse reticular abnormalities
  • Normal
  • Normal
Amyloidosis[25][26] Subacute/chronic 50−70 years old Male + Bloody +
  • Congophilia with apple−green birefringence under polarized light
  • Normal
Disease History Symptoms Physical examination Lab findings Imaging Pulmonary function test Bronchoscopy and BAL Gold standard
Duration Age Gender Family history Smoking history Environmental exposure HIV Dyspnea Cough Wheezing Chest pain Tachypnea   Auscultation Cyanosis Clubbing Spirometry ABG

References

  1. Poletti, Venerino; Ravaglia, Claudia; Buccioli, Matteo; Tantalocco, Paola; Piciucchi, Sara; Dubini, Alessandra; Carloni, Angelo; Chilosi, Marco; Tomassetti, Sara (2013). "Idiopathic Pulmonary Fibrosis: Diagnosis and Prognostic Evaluation". Respiration. 86 (1): 5–12. doi:10.1159/000353580. ISSN 1423-0356.
  2. Travis, William D.; Hunninghake, Gary; King, Talmadge E.; Lynch, David A.; Colby, Thomas V.; Galvin, Jeffrey R.; Brown, Kevin K.; Chung, Man Pyo; Cordier, Jean-François; du Bois, Roland M.; Flaherty, Kevin R.; Franks, Teri J.; Hansell, David M.; Hartman, Thomas E.; Kazerooni, Ella A.; Kim, Dong Soon; Kitaichi, Masanori; Koyama, Takashi; Martinez, Fernando J.; Nagai, Sonoko; Midthun, David E.; Müller, Nestor L.; Nicholson, Andrew G.; Raghu, Ganesh; Selman, Moisés; Wells, Athol (2008). "Idiopathic Nonspecific Interstitial Pneumonia". American Journal of Respiratory and Critical Care Medicine. 177 (12): 1338–1347. doi:10.1164/rccm.200611-1685OC. ISSN 1073-449X.
  3. Mehrian, P.; Doroudinia, A.; Rashti, A.; Aloosh, O.; Dorudinia, A. (2017). "High-resolution computed tomography findings in chronic eosinophilic vs. cryptogenic organising pneumonia". The International Journal of Tuberculosis and Lung Disease. 21 (11): 1181–1186. doi:10.5588/ijtld.16.0723. ISSN 1027-3719.
  4. Parambil, Joseph; Mukhopadhyay, Sanjay (2012). "Acute Interstitial Pneumonia (AIP): Relationship to Hamman-Rich Syndrome, Diffuse Alveolar Damage (DAD), and Acute Respiratory Distress Syndrome (ARDS)". Seminars in Respiratory and Critical Care Medicine. 33 (05): 476–485. doi:10.1055/s-0032-1325158. ISSN 1069-3424.
  5. Panchabhai, Tanmay S.; Farver, Carol; Highland, Kristin B. (2016). "Lymphocytic Interstitial Pneumonia". Clinics in Chest Medicine. 37 (3): 463–474. doi:10.1016/j.ccm.2016.04.009. ISSN 0272-5231.
  6. Sieminska, Alicja; Kuziemski, Krzysztof (2014). "Respiratory bronchiolitis-interstitial lung disease". Orphanet Journal of Rare Diseases. 9 (1). doi:10.1186/s13023-014-0106-8. ISSN 1750-1172.
  7. Ryu, Jay H.; Myers, Jeffrey L.; Capizzi, Stephen A.; Douglas, William W.; Vassallo, Robert; Decker, Paul A. (2005). "Desquamative Interstitial Pneumonia and Respiratory Bronchiolitis-Associated Interstitial Lung Disease". Chest. 127 (1): 178–184. doi:10.1378/chest.127.1.178. ISSN 0012-3692.
  8. Craig, P J; Wells, A U; Doffman, S; Rassl, D; Colby, T V; Hansell, D M; du Bois, R M; Nicholson, A G (2004). "Desquamative interstitial pneumonia, respiratory bronchiolitis and their relationship to smoking". Histopathology. 45 (3): 275–282. doi:10.1111/j.1365-2559.2004.01921.x. ISSN 0309-0167.
  9. Blakley, Matthew P.; Dutcher, Janice P.; Wiernik, Peter H. (2018). "Pulmonary Langerhans cell histiocytosis, acute myeloid leukemia, and myelofibrosis in a large family and review of the literature". Leukemia Research. 67: 39–44. doi:10.1016/j.leukres.2018.01.011. ISSN 0145-2126.
  10. Carrington JM, Hershberger DM. PMID 29493933. Missing or empty |title= (help)
  11. Kiani, Arda; Parsa, Tahereh; Adimi Naghan, Parisa; Dutau, Hervé; Razavi, Fatemeh; Farzanegan, Behrooz; Pourabdollah Tootkaboni, Mahsa; Abedini, Atefeh (2018). "An eleven-year retrospective cross-sectional study on pulmonary alveolar proteinosis". Advances in Respiratory Medicine. 86 (1): 7–12. doi:10.5603/ARM.2018.0003. ISSN 2543-6031.
  12. Xu, Kai-Feng; Lo, Bee Hong (2014). "Lymphangioleiomyomatosis: differential diagnosis and optimal management". Therapeutics and Clinical Risk Management: 691. doi:10.2147/TCRM.S50784. ISSN 1178-203X.
  13. Bernheim, Adam; McLoud, Theresa (2017). "A Review of Clinical and Imaging Findings in Eosinophilic Lung Diseases". American Journal of Roentgenology. 208 (5): 1002–1010. doi:10.2214/AJR.16.17315. ISSN 0361-803X.
  14. Miller, Ross; Allen, Timothy Craig; Barrios, Roberto J.; Beasley, Mary Beth; Burke, Louise; Cagle, Philip T.; Capelozzi, Vera Luiza; Ge, Yimin; Hariri, Lida P.; Kerr, Keith M.; Khoor, Andras; Larsen, Brandon T.; Mark, Eugene J.; Matsubara, Osamu; Mehrad, Mitra; Mino-Kenudson, Mari; Raparia, Kirtee; Roden, Anja Christiane; Russell, Prudence; Schneider, Frank; Sholl, Lynette M.; Smith, Maxwell Lawrence (2018). "Hypersensitivity Pneumonitis A Perspective From Members of the Pulmonary Pathology Society". Archives of Pathology & Laboratory Medicine. 142 (1): 120–126. doi:10.5858/arpa.2017-0138-SA. ISSN 0003-9985.
  15. Sirajuddin, Arlene; Kanne, Jeffrey P. (2009). "Occupational Lung Disease". Journal of Thoracic Imaging. 24 (4): 310–320. doi:10.1097/RTI.0b013e3181c1a9b3. ISSN 0883-5993.
  16. Giridhar P, Mallick S, Rath GK, Julka PK (2015). "Radiation induced lung injury: prediction, assessment and management". Asian Pac. J. Cancer Prev. 16 (7): 2613–7. PMID 25854336.
  17. DeVrieze BW, Hurley JA. PMID 29083697. Missing or empty |title= (help)
  18. Khorashadi, L.; Wu, C.C.; Betancourt, S.L.; Carter, B.W. (2015). "Idiopathic pulmonary haemosiderosis: spectrum of thoracic imaging findings in the adult patient". Clinical Radiology. 70 (5): 459–465. doi:10.1016/j.crad.2014.11.007. ISSN 0009-9260.
  19. Thompson, Gwen; Klecka, Mary; Roden, Anja C.; Specks, Ulrich; Cartin-Ceba, Rodrigo (2016). "Biopsy-proven pulmonary capillaritis: A retrospective study of aetiologies including an in-depth look at isolated pulmonary capillaritis". Respirology. 21 (4): 734–738. doi:10.1111/resp.12738. ISSN 1323-7799.
  20. Li, Cheng-Wei; Tao, Ru-Jia; Zou, Dan-Feng; Li, Man-Hui; Xu, Xin; Cao, Wei-Jun (2018). "Pulmonary sarcoidosis with and without extrapulmonary involvement: a cross-sectional and observational study in China". BMJ Open. 8 (2): e018865. doi:10.1136/bmjopen-2017-018865. ISSN 2044-6055.
  21. Greco A, Marinelli C, Fusconi M, Macri GF, Gallo A, De Virgilio A, Zambetti G, de Vincentiis M (June 2016). "Clinic manifestations in granulomatosis with polyangiitis". Int J Immunopathol Pharmacol. 29 (2): 151–9. doi:10.1177/0394632015617063. PMC 5806708. PMID 26684637.
  22. Greco A, Rizzo MI, De Virgilio A, Gallo A, Fusconi M, Ruoppolo G, Altissimi G, De Vincentiis M (April 2015). "Churg-Strauss syndrome". Autoimmun Rev. 14 (4): 341–8. doi:10.1016/j.autrev.2014.12.004. PMID 25500434.
  23. Myers, Jeffrey L. (1989). "Bronchocentric Granulomatosis". Chest. 96 (1): 3–4. doi:10.1378/chest.96.1.3. ISSN 0012-3692.
  24. Ankita, Grover; Shashi, Dhawan (2016). "Pulmonary Lymphomatoid Granulomatosis- a Case Report with Review of Literature". Indian Journal of Surgical Oncology. 7 (4): 484–487. doi:10.1007/s13193-016-0525-1. ISSN 0975-7651.
  25. Khoor, Andras; Colby, Thomas V. (2017). "Amyloidosis of the Lung". Archives of Pathology & Laboratory Medicine. 141 (2): 247–254. doi:10.5858/arpa.2016-0102-RA. ISSN 0003-9985.
  26. Milani, Paolo; Basset, Marco; Russo, Francesca; Foli, Andrea; Palladini, Giovanni; Merlini, Giampaolo (2017). "The lung in amyloidosis". European Respiratory Review. 26 (145): 170046. doi:10.1183/16000617.0046-2017. ISSN 0905-9180.

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