Cystic fibrosis differential diagnosis: Difference between revisions

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__NOTOC__
__NOTOC__
{{Cystic fibrosis}}
[[Image:Home_logo1.png|right|250px|link=https://www.wikidoc.org/index.php/Cystic_fibrosis]]
{{CMG}}; {{AE}} {{SHH}}
{{CMG}}; {{AE}} {{SHH}}, {{KZ}}, {{Anmol}}
 


==Overview==
==Overview==
Cystic fibrosis has to be differentiated from other conditions with similar presentation of cough and wheeze like [[asthma]], [[bronchiolitis]], [[Chronic obstructive pulmonary disease|COPD]], [[bacterial pneumonia]], [[emphysema]], [[Primary ciliary dyskinesia|primary ciliary dyskinesia]] ([[Kartagener's Syndrome|Kartagener syndrome]]) and [[Alpha 1-antitrypsin deficiency]].
Cystic fibrosis has to be differentiated from other conditions with similar presentation of [[cough]] and [[wheeze]] like [[Acute viral nasopharyngitis (common cold)|common cold]], [[asthma]], [[bronchiolitis]], [[emphysema]], [[Primary ciliary dyskinesia|primary ciliary dyskinesia]] ([[Kartagener's Syndrome|Kartagener syndrome]]), [[bronchitis]], [[bronchiectasis]], [[Pulmonary aspiration|foreign body aspiration]], [[pneumoconiosis]], [[interstitial lung disease]], cardiogenic [[pulmonary edema]], [[Gastroesophageal reflux disease|GERD]] and [[sarcoidosis]].  
 
==Differentiating cystic fibrosis from other Diseases==
 
[[Differential diagnosis]] of [[cough]] with [[Wheezing|wheezes]] is :
{| class="wikitable"
! rowspan="2" |Diseases
! colspan="2" |Symptoms
!
! colspan="3" |Signs
! colspan="2" |Diagosis
|-
!Fever
!Cough
!Chest pain
!Wheezes
!Crackles
!Tachypnea
!Lab tests
!Imaging
|-
|[[Asthma]]
|<nowiki>-</nowiki>
|Dry/Productive
| -
|<nowiki>+</nowiki>
| -
| +
|
* Lab tests to exclude other [[Disease|diseases]].
* Serum examination shows elevated level of [[Eosinophil|eosinophils]] due to [[allergy]]. 
|
* [[CT scan]] shows:
** Dilated [[bronchi]].
** Bronchial wall thickening.
** Air trapping.
|-
|[[Bronchiolitis]]
| +/-
|Dry
|<nowiki>-</nowiki>
|<nowiki>+</nowiki>
| +
| +/-
|
* [[ELISA]] and [[immunoassays]] may be done in case of [[RSV]] [[infection]].
* [[Pulmonary function test]] to exclude other [[lung diseases]].<ref name="pmid18339530">{{cite journal| author=Ghanei M, Tazelaar HD, Chilosi M, Harandi AA, Peyman M, Akbari HM et al.| title=An international collaborative pathologic study of surgical lung biopsies from mustard gas-exposed patients. | journal=Respir Med | year= 2008 | volume= 102 | issue= 6 | pages= 825-30 | pmid=18339530 | doi=10.1016/j.rmed.2008.01.016 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18339530  }} </ref>
|
* [[CT scan]] shows:
** Intense [[Bronchiolar epithelium|bronchiolar]] mural [[inflammation]]. 
** [[bronchial]] wall thickening.
** Centrilobular [[nodules]] with tree-in-bud pattern. 
|-
|[[COPD]]
|<nowiki>+</nowiki>
|Productive
|<nowiki>-</nowiki>
| +
| +
| +
|
* [[Spirometry]]: [[FEV1/FVC ratio|FEV1/FVC]] < 70%.
* Arterial blood gases: [[hypoxemia]] and [[hypercapnia]].
* [[Sputum culture]]. 
|
* EKG may show:
** [[P pulmonale]].
** [[right ventricular hypertrophy]].
** Narrow QRS.<ref name="pmid23653989">{{cite journal| author=Lazović B, Svenda MZ, Mazić S, Stajić Z, Delić M| title=Analysis of electrocardiogram in chronic obstructive pulmonary disease patients. | journal=Med Pregl | year= 2013 | volume= 66 | issue= 3-4 | pages= 126-9 | pmid=23653989 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23653989  }} </ref>
* CT scan is more sensitive in diagnosing COPD than X ray. 
|-
|[[Bacterial pneumonia]]
|<nowiki>+</nowiki>
|[[Productive cough|Productive]]
| +
| +
| +
| +/-
|
* Diagnosis depends on presentation and physical examination.
* Laboratory tests:
** [[arterial blood gases]] may show [[hypoxia]] and [[acidosis]].
** [[Sputum culture]].
|
* X ray is performed to detect:
** [[pleural effusion]].
** Inflitrates within the [[lungs]].
* CT scan shows:
** [[Consolidation (medicine)|Consolidation]].
** Ground glass appearance.
|-
|[[Cystic Fibrosis]]
| +/-
|[[Productive cough|Productive]]
| +/-
| -
| -
| +
|[[Cystic fibrosis]] transmembrane conductance regulator (CFTR) dysfunction evidenced by :
* Elevated [[Sweat chloride test|sweat chloride]] ≥60 mmol/L (on two occasions).


* Presence of two disease-causing [[mutations]] in CFTR, one from each [[Allele|parental allele]].
* Abnormal [[Potential difference|nasal potential difference]].
|[[X-ray]] :
Hyperinflation presents as:
* Flattening of the [[diaphragm]].
* Anterior bowing of the infant [[sternum]].
* Increased retrosternal air space.
* Generalized [[pulmonary]] overinflation.
* Multiple nodular densities represent [[Mucus|mucus plugging]] and may present in finger-in-glove shape or as a combination of V- or Y-shaped branching and bandlike shadows.
Abdominal findings include dilated multiple loops of the [[small bowel]] are seen in [[Meconium ileus|neonatal meconium ileus]].
|-
|[[Emphysema]]
| +/-
|[[Productive cough|Productive]]
| -
| +
| +/-
| +
|
* [[Arterial blood gases|Arterial blood gas analysis]]: mild-to-moderate [[hypoxemia]] without [[hypercapnia]] that progresses to worsening [[hypoxemia]]  and [[hypercapnia]] develops.
* Chronic [[hypoxemia]] may lead to [[polycythemia]].
* [[Sputum]] is mucoid and the predominant cells are [[macrophages]].
|[[Chest X-rays|Chest X-ray]] reveals signs of [[emphysema]] include:
* Flattening of [[diaphragm]].
* Increased retrosternal air space (see on lateral chest films).
* A long narrow [[heart]] shadow.
* Tapering vascular shadows.
* Hyperlucency of the [[lungs]].
|-
|[[Primary ciliary dyskinesia|Primary Ciliary Dyskinesia]] ([[Kartagener's Syndrome|Kartagener Syndrome]])
| +/-
|[[Productive cough|Productive]]
| -
| +
| +
| +
|
* Low or absent amount of nasal [[nitric oxide]] (nNO).
* [[Mucociliary clearance]] may be useful for [[screening]].
* Confirmation with tests of ciliary function.
|[[Chest X-rays|Chest X-ray]] reveals :
* [[Bronchial]] wall thickening.
* [[Bronchiectasis]] and hyperinflation.
* Cystic [[bronchiectasis]] with air-fluid levels may be visible.
* Usually involves the lower and middle lobes.
|-
|[[Alpha 1-antitrypsin deficiency]]
| +/-
|[[Productive cough|Productive]]
| -
| +
| +
| +
|
* Reduced concentration of serum [[Alpha1 antitrypsin|alpha1-antitrypsin levels]] is diagnostic of AATD.
* Moderate-to-severe airflow obstruction with an [[FEV1]].
* Reduced [[vital capacity]].
* Increased [[lung volumes]] secondary to air trapping ([[residual volume]] >120% of predicted value) are usually present.
|[[Chest X-rays|Chest X-ray]] Alpha1-antitrypsin deficiency (AATD) [[emphysema]] presents as:
* a hyperlucent appearance because healthy tissue has been destroyed.
* Affected regions also are described as oligemic because they lack the normal rich pattern of branching blood vessels.
* An unusual characteristic in alpha1-antitrypsin deficiency is found in about 60% of PiZZ patients is a striking basilar distribution.
* In contrast, [[cigarette smoking]] is associated with more severe apical disease.
|}
==Cough==
==Cough==
Cystic fibrosis must be differentiated from other diseases presenting with cough and wheeze.
Cystic fibrosis must be differentiated from other diseases presenting with [[cough]] and [[wheeze]] include:


{| class="wikitable"
{| class="wikitable"
Line 315: Line 144:
* Pollution
* Pollution
|-
|-
| style="background:#DCDCDC;" align="center" + |'''Non-asthmatic eosinophilic bronchitis'''<ref name="pmid16428700">{{cite journal |vauthors=Brightling CE |title=Chronic cough due to nonasthmatic eosinophilic bronchitis: ACCP evidence-based clinical practice guidelines |journal=Chest |volume=129 |issue=1 Suppl |pages=116S–121S |year=2006 |pmid=16428700 |doi=10.1378/chest.129.1_suppl.116S |url=}}</ref><ref name="pmid29317659">{{cite journal| author=Cho J, Choi SM, Lee J, Park YS, Lee SM, Yoo CG et al.| title=Clinical Outcome of Eosinophilic Airway Inflammation in Chronic Airway Diseases Including Nonasthmatic Eosinophilic Bronchitis. | journal=Sci Rep | year= 2018 | volume= 8 | issue= 1 | pages= 146 | pmid=29317659 | doi=10.1038/s41598-017-18265-2 | pmc=5760521 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29317659  }}</ref>
| style="background:#DCDCDC;" align="center" + |'''[[Primary ciliary dyskinesia|Primary Ciliary Dyskinesia]]'''
'''([[Primary ciliary dyskinesia|Kartagener Syndrome]])'''
| style="background:#F5F5F5;" align="center" + |Chronic
| style="background:#F5F5F5;" align="center" + |Chronic
| style="background:#F5F5F5;" + |
| style="background:#F5F5F5;" + |
* More than 8 weeks
* Years
| style="background:#F5F5F5;" align="center" + |✔ [[Eosinophilic]] [[sputum]]
| style="background:#F5F5F5;" align="center" + |✔  
| style="background:#F5F5F5;" align="center" + |
| style="background:#F5F5F5;" align="center" + |✔
| style="background:#F5F5F5;" align="center" + |<nowiki>-</nowiki>
| style="background:#F5F5F5;" align="center" + |✔
| style="background:#F5F5F5;" align="center" + | -
| style="background:#F5F5F5;" align="center" + | -
| style="background:#F5F5F5;" align="center" + |✔
| style="background:#F5F5F5;" align="center" + |✔  
| style="background:#F5F5F5;" + |
| style="background:#F5F5F5;" + |
* [[Wheeze|Wheezing]]
* [[Wheeze|Wheezing]]
* [[Shortness of breath]]
* [[Rales|Crackles]]
| style="background:#F5F5F5;" + |
| style="background:#F5F5F5;" + |
* High levels of [[Immunoglobulin E|IgE]]
* Low or absent amount of nasal [[nitric oxide]] (nNO).
* Airway [[eosinophilia]] in [[sputum]] induction or bronchial wash fluid from [[bronchoscopy]] ([[bronchoalveolar lavage]])
* [[Mucociliary clearance]] may be useful for [[screening]]  
* Confirmation with tests of ciliary function.
| style="background:#F5F5F5;" + |
| style="background:#F5F5F5;" + |
* Normal [[chest X-Ray]]
* [[Chest X-rays|Chest X-ray]] reveals :
 
* [[Bronchial]] wall thickening.
* [[Bronchiectasis]] and hyperinflation.
* [[Cyst|Cystic]] [[bronchiectasis]] with air-fluid levels may be visible.
* Usually involves the lower and middle lobes.
| style="background:#F5F5F5;" + |
| style="background:#F5F5F5;" + |
* [[FEV1/FVC ratio|FEV1/FVC]] >70%
* Mild to moderate obstructive ventilatory defect
* No response of short acting [[bronchodilator]]
* Not specific
* May be normal
| style="background:#F5F5F5;" + |
| style="background:#F5F5F5;" + |
* [[Bronchial]] [[biopsy]] [[eosinophilia]]
* [[Transmission electron microscopy|Transmission electron microscopy (TEM)]] for assessment of ciliary [[ultrastructure]]
| style="background:#F5F5F5;" + |
| style="background:#F5F5F5;" + |
* Exposure to an occupational cause
* Recurrent [[Respiratory tract infection|respiratory infections]]
* Poor sense of [[smell]]
|-
|-
| style="background:#DCDCDC;" align="center" + |[[Bronchiectasis|'''Bronchiectasis''']]<ref name="pmid166509702">{{cite journal |vauthors=King PT, Holdsworth SR, Freezer NJ, Villanueva E, Holmes PW |title=Characterisation of the onset and presenting clinical features of adult bronchiectasis |journal=Respir Med |volume=100 |issue=12 |pages=2183–9 |year=2006 |pmid=16650970 |doi=10.1016/j.rmed.2006.03.012 |url=}}</ref>
| style="background:#DCDCDC;" align="center" + |[[Bronchiectasis|'''Bronchiectasis''']]<ref name="pmid166509702">{{cite journal |vauthors=King PT, Holdsworth SR, Freezer NJ, Villanueva E, Holmes PW |title=Characterisation of the onset and presenting clinical features of adult bronchiectasis |journal=Respir Med |volume=100 |issue=12 |pages=2183–9 |year=2006 |pmid=16650970 |doi=10.1016/j.rmed.2006.03.012 |url=}}</ref>
Line 397: Line 235:
* Exposure of tobacco and air pollution
* Exposure of tobacco and air pollution
|-
|-
| style="background:#DCDCDC;" align="center" + |'''Foreing body [[Aspiration of foreign body|aspiration]]'''<ref name="pmid29221325">{{cite journal| author=Hewlett JC, Rickman OB, Lentz RJ, Prakash UB, Maldonado F| title=Foreign body aspiration in adult airways: therapeutic approach. | journal=J Thorac Dis | year= 2017 | volume= 9 | issue= 9 | pages= 3398-3409 | pmid=29221325 | doi=10.21037/jtd.2017.06.137 | pmc=5708401 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29221325  }}</ref><ref name="pmid11444115">{{cite journal |vauthors=Rafanan AL, Mehta AC |title=Adult airway foreign body removal. What's new? |journal=Clin. Chest Med. |volume=22 |issue=2 |pages=319–30 |year=2001 |pmid=11444115 |doi= |url=}}</ref><ref name="pmid26568942">{{cite journal| author=Haddadi S, Marzban S, Nemati S, Ranjbar Kiakelayeh S, Parvizi A, Heidarzadeh A| title=Tracheobronchial Foreign-Bodies in Children; A 7 Year Retrospective Study. | journal=Iran J Otorhinolaryngol | year= 2015 | volume= 27 | issue= 82 | pages= 377-85 | pmid=26568942 | doi= | pmc=4639691 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26568942  }}</ref>
| style="background:#DCDCDC;" align="center" + |'''Foreign body [[Aspiration of foreign body|aspiration]]'''<ref name="pmid29221325">{{cite journal| author=Hewlett JC, Rickman OB, Lentz RJ, Prakash UB, Maldonado F| title=Foreign body aspiration in adult airways: therapeutic approach. | journal=J Thorac Dis | year= 2017 | volume= 9 | issue= 9 | pages= 3398-3409 | pmid=29221325 | doi=10.21037/jtd.2017.06.137 | pmc=5708401 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29221325  }}</ref><ref name="pmid11444115">{{cite journal |vauthors=Rafanan AL, Mehta AC |title=Adult airway foreign body removal. What's new? |journal=Clin. Chest Med. |volume=22 |issue=2 |pages=319–30 |year=2001 |pmid=11444115 |doi= |url=}}</ref><ref name="pmid26568942">{{cite journal| author=Haddadi S, Marzban S, Nemati S, Ranjbar Kiakelayeh S, Parvizi A, Heidarzadeh A| title=Tracheobronchial Foreign-Bodies in Children; A 7 Year Retrospective Study. | journal=Iran J Otorhinolaryngol | year= 2015 | volume= 27 | issue= 82 | pages= 377-85 | pmid=26568942 | doi= | pmc=4639691 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26568942  }}</ref>
| style="background:#F5F5F5;" align="center" + |Acute
| style="background:#F5F5F5;" align="center" + |Acute
| style="background:#F5F5F5;" align="center" + |
| style="background:#F5F5F5;" align="center" + |
Line 454: Line 292:
|-
|-
| rowspan="3" style="background:#DCDCDC;" align="center" + |[[Parenchyma|'''Parenchyma''']]
| rowspan="3" style="background:#DCDCDC;" align="center" + |[[Parenchyma|'''Parenchyma''']]
| style="background:#DCDCDC;" align="center" + |[[Cystic fibrosis|'''Cystic fibrosis''']]  <ref name="pmid18639722">{{cite journal |vauthors=Farrell PM, Rosenstein BJ, White TB, Accurso FJ, Castellani C, Cutting GR, Durie PR, Legrys VA, Massie J, Parad RB, Rock MJ, Campbell PW |title=Guidelines for diagnosis of cystic fibrosis in newborns through older adults: Cystic Fibrosis Foundation consensus report |journal=J. Pediatr. |volume=153 |issue=2 |pages=S4–S14 |year=2008 |pmid=18639722 |pmc=2810958 |doi=10.1016/j.jpeds.2008.05.005 |url=}}</ref><ref name="pmid1285737">{{cite journal |vauthors=Kerem E, Reisman J, Corey M, Canny GJ, Levison H |title=Prediction of mortality in patients with cystic fibrosis |journal=N. Engl. J. Med. |volume=326 |issue=18 |pages=1187–91 |year=1992 |pmid=1285737 |doi=10.1056/NEJM199204303261804 |url=}}</ref>
| style="background:#F5F5F5;" align="center" + |Chronic
| style="background:#F5F5F5;" align="center" + |
* Variable
| style="background:#F5F5F5;" align="center" + |✔
| style="background:#F5F5F5;" align="center" + | -
| style="background:#F5F5F5;" align="center" + |✔
| style="background:#F5F5F5;" align="center" + |
| style="background:#F5F5F5;" align="center" + |✔
| style="background:#F5F5F5;" + |
* Barrel-shaped chest
* [[Wheezing]]
* [[Tachypnea]]
| style="background:#F5F5F5;" + |
* [[Respiratory tract]] [[Culture media|culture]] for [[Cystic fibrosis|CF]]
* [[Bronchoalveolar lavage]] for cytology
* ≥ 60 mmol/L [[Sweat chloride test]]
* [[CFTR (gene)|CFTR]] [[mutation]] in molecular testing
| style="background:#F5F5F5;" + |
* Hyperinflation, [[atelectasis]], and infiltrates on [[Chest X-ray|chest X-Ray]]
* Severe patients present bronchietasis, "tram tracks" [[Peribronchial cuffing|peribronchial cuffin]]<nowiki/>g in [[Chest X-ray|chest X-Ray]]
* The extension of [[bronchietasis]] can be defined by [[Computed tomography|CT]]
| style="background:#F5F5F5;" + |
* [[Residual volume|RV]]/[[Total lung capacity|TLC]] ratio increased
* [[FEV1/FVC ratio]] <70%
* Low levels of [[FEV1]]
* High levels of [[Total lung capacity|TLC]]
* [[Residual volume|RV]] increased
| style="background:#F5F5F5;" + |
* [[Sweat chloride test]]
| style="background:#F5F5F5;" + |
* Evidence of [[Cystic fibrosis transmembrane conductance regulator|CFTR]] dysfunction
|-
| style="background:#DCDCDC;" align="center" + |[[Pneumoconiosis|'''Pneumoconioses''']]<ref name="pmid27980247">{{cite journal |vauthors=Jp NA, Imanaka M, Suganuma N |title=Japanese workplace health management in pneumoconiosis prevention |journal=J Occup Health |volume=59 |issue=2 |pages=91–103 |year=2017 |pmid=27980247 |pmc=5478517 |doi=10.1539/joh.16-0031-RA |url=}}</ref><ref name="pmid12668748">{{cite journal |vauthors=Weiland DA, Lynch DA, Jensen SP, Newell JD, Miller DE, Crausman RS, Kuhn C, Kern DG |title=Thin-section CT findings in flock worker's lung, a work-related interstitial lung disease |journal=Radiology |volume=227 |issue=1 |pages=222–31 |year=2003 |pmid=12668748 |doi=10.1148/radiol.2271011063 |url=}}</ref>
| style="background:#DCDCDC;" align="center" + |[[Pneumoconiosis|'''Pneumoconioses''']]<ref name="pmid27980247">{{cite journal |vauthors=Jp NA, Imanaka M, Suganuma N |title=Japanese workplace health management in pneumoconiosis prevention |journal=J Occup Health |volume=59 |issue=2 |pages=91–103 |year=2017 |pmid=27980247 |pmc=5478517 |doi=10.1539/joh.16-0031-RA |url=}}</ref><ref name="pmid12668748">{{cite journal |vauthors=Weiland DA, Lynch DA, Jensen SP, Newell JD, Miller DE, Crausman RS, Kuhn C, Kern DG |title=Thin-section CT findings in flock worker's lung, a work-related interstitial lung disease |journal=Radiology |volume=227 |issue=1 |pages=222–31 |year=2003 |pmid=12668748 |doi=10.1148/radiol.2271011063 |url=}}</ref>
| style="background:#F5F5F5;" align="center" + |Acute, Chronic
| style="background:#F5F5F5;" align="center" + |Acute, Chronic
Line 514: Line 385:
| style="background:#F5F5F5;" + |
| style="background:#F5F5F5;" + |
* Clubbing is common in [[asbestosis]] and [[idiopathic pulmonary fibrosis]]
* Clubbing is common in [[asbestosis]] and [[idiopathic pulmonary fibrosis]]
|-
| style="background:#DCDCDC;" align="center" + |[[Cystic fibrosis|'''Cystic fibrosis''']]  ([[Cystic fibrosis|CF]])<ref name="pmid18639722">{{cite journal |vauthors=Farrell PM, Rosenstein BJ, White TB, Accurso FJ, Castellani C, Cutting GR, Durie PR, Legrys VA, Massie J, Parad RB, Rock MJ, Campbell PW |title=Guidelines for diagnosis of cystic fibrosis in newborns through older adults: Cystic Fibrosis Foundation consensus report |journal=J. Pediatr. |volume=153 |issue=2 |pages=S4–S14 |year=2008 |pmid=18639722 |pmc=2810958 |doi=10.1016/j.jpeds.2008.05.005 |url=}}</ref><ref name="pmid1285737">{{cite journal |vauthors=Kerem E, Reisman J, Corey M, Canny GJ, Levison H |title=Prediction of mortality in patients with cystic fibrosis |journal=N. Engl. J. Med. |volume=326 |issue=18 |pages=1187–91 |year=1992 |pmid=1285737 |doi=10.1056/NEJM199204303261804 |url=}}</ref>
| style="background:#F5F5F5;" align="center" + |Chronic
| style="background:#F5F5F5;" align="center" + |
* Variable
| style="background:#F5F5F5;" align="center" + |✔
| style="background:#F5F5F5;" align="center" + | -
| style="background:#F5F5F5;" align="center" + |✔
| style="background:#F5F5F5;" align="center" + |
| style="background:#F5F5F5;" align="center" + |✔
| style="background:#F5F5F5;" + |
* Barrel-shaped chest
* [[Wheezing]]
* [[Tachypnea]]
| style="background:#F5F5F5;" + |
* [[Respiratory tract]] [[Culture media|culture]] for [[Cystic fibrosis|CF]]
* [[Bronchoalveolar lavage]] for cytology
* ≥ 60 mmol/L [[Sweat chloride test]]
* [[CFTR (gene)|CFTR]] [[mutation]] in molecular testing
| style="background:#F5F5F5;" + |
* Hyperinflation, [[atelectasis]], and infiltrates on [[Chest X-ray|chest X-Ray]]
* Severe patients present bronchietasis, "tram tracks" [[Peribronchial cuffing|peribronchial cuffin]]<nowiki/>g in [[Chest X-ray|chest X-Ray]]
* The extension of [[bronchietasis]] can be defined by [[Computed tomography|CT]]
| style="background:#F5F5F5;" + |
* [[Residual volume|RV]]/[[Total lung capacity|TLC]] ratio increased
* [[FEV1/FVC ratio]] <70%
* Low levels of [[FEV1]]
* High levels of [[Total lung capacity|TLC]]
* [[Residual volume|RV]] increased
| style="background:#F5F5F5;" + |
* [[Sweat chloride test]]
| style="background:#F5F5F5;" + |
* Evidence of [[Cystic fibrosis transmembrane conductance regulator|CFTR]] dysfunction
|-
|-
| colspan="2" style="background:#DCDCDC;" align="center" + |[[Heart|'''Cardiac''']]
| colspan="2" style="background:#DCDCDC;" align="center" + |[[Heart|'''Cardiac''']]
Line 608: Line 446:
| style="background:#F5F5F5;" align="center" + | --
| style="background:#F5F5F5;" align="center" + | --
|-
|-
| colspan="2" rowspan="4" style="background:#DCDCDC;" align="center" + |[[Autoimmune disease|'''Autoinmune''']]
| colspan="2" style="background:#DCDCDC;" align="center" + |[[Autoimmune disease|'''Autoinmune''']]
| style="background:#DCDCDC;" align="center" + |[[Granulomatosis with polyangiitis|'''Wegener's disease''']] ([[Granulomatosis with polyangiitis|'''GPA''']]) <ref name="pmid1739240">{{cite journal |vauthors=Hoffman GS, Kerr GS, Leavitt RY, Hallahan CW, Lebovics RS, Travis WD, Rottem M, Fauci AS |title=Wegener granulomatosis: an analysis of 158 patients |journal=Ann. Intern. Med. |volume=116 |issue=6 |pages=488–98 |year=1992 |pmid=1739240 |doi= |url=}}</ref><ref name="pmid21374588">{{cite journal |vauthors=Falk RJ, Gross WL, Guillevin L, Hoffman GS, Jayne DR, Jennette JC, Kallenberg CG, Luqmani R, Mahr AD, Matteson EL, Merkel PA, Specks U, Watts RA |title=Granulomatosis with polyangiitis (Wegener's): an alternative name for Wegener's granulomatosis |journal=Arthritis Rheum. |volume=63 |issue=4 |pages=863–4 |year=2011 |pmid=21374588 |doi=10.1002/art.30286 |url=}}</ref>
| style="background:#F5F5F5;" align="center" + |Chronic
| style="background:#F5F5F5;" + |
* Months
| style="background:#F5F5F5;" align="center" + |✔
| style="background:#F5F5F5;" align="center" + |✔
| style="background:#F5F5F5;" align="center" + |✔
| style="background:#F5F5F5;" align="center" + |✔
| style="background:#F5F5F5;" align="center" + |✔
| style="background:#F5F5F5;" + |
* [[Hoarseness]]
* [[Stridor]]
* [[Wheeze|Wheezing]]
| style="background:#F5F5F5;" + |
* [[Anti-neutrophil cytoplasmic antibody|ANCA]], [[P-ANCA]], [[C-ANCA]]
* [[Blood urea nitrogen|BUN]]
* [[Creatinine]]
* [[Complete blood count]]
* [[Urinalysis]]
* Lung [[biopsy]]
| style="background:#F5F5F5;" + |
* [[Nodules]], [[Lung|pulmonary]] infiltrates, reticular margins, pleural opacities and [[Cavity|cavities]] in [[Chest X-ray|chest X-Ray]]
* [[Nodule (medicine)|Nodules]], [[cavities]] and stellate-shaped peripherial [[pulmonary]] in chest [[Computed tomography|CT]]
* [[Bronchoscopy]]
| style="background:#F5F5F5;" + |
* Low levels of [[DLCO]]
* Reduce [[lung volumes]]
| style="background:#F5F5F5;" + |
* Tissue [[biopsy]]
| style="background:#F5F5F5;" + |
* Nasal crusting, sinus pain, chronic [[rhinosinusitis]], nasal obstruction and discharge in [[Upper respiratory tract|upper airway]]
* [[Saddle nose|Saddle nose deformity]]
* [[Purpura]] in lower extremities
|-
| style="background:#DCDCDC;" align="center" + |[[Sarcoidosis|'''Sarcoidosis''']]<ref name="pmid27378039">{{cite journal |vauthors=Carmona EM, Kalra S, Ryu JH |title=Pulmonary Sarcoidosis: Diagnosis and Treatment |journal=Mayo Clin. Proc. |volume=91 |issue=7 |pages=946–54 |year=2016 |pmid=27378039 |doi=10.1016/j.mayocp.2016.03.004 |url=}}</ref><ref name="pmid12803116">{{cite journal |vauthors=Yanardağ H, Pamuk GE, Karayel T, Demirci S |title=Bone marrow involvement in sarcoidosis: an analysis of 50 bone marrow samples |journal=Haematologia (Budap) |volume=32 |issue=4 |pages=419–25 |year=2002 |pmid=12803116 |doi= |url=}}</ref>
| style="background:#DCDCDC;" align="center" + |[[Sarcoidosis|'''Sarcoidosis''']]<ref name="pmid27378039">{{cite journal |vauthors=Carmona EM, Kalra S, Ryu JH |title=Pulmonary Sarcoidosis: Diagnosis and Treatment |journal=Mayo Clin. Proc. |volume=91 |issue=7 |pages=946–54 |year=2016 |pmid=27378039 |doi=10.1016/j.mayocp.2016.03.004 |url=}}</ref><ref name="pmid12803116">{{cite journal |vauthors=Yanardağ H, Pamuk GE, Karayel T, Demirci S |title=Bone marrow involvement in sarcoidosis: an analysis of 50 bone marrow samples |journal=Haematologia (Budap) |volume=32 |issue=4 |pages=419–25 |year=2002 |pmid=12803116 |doi= |url=}}</ref>
| style="background:#F5F5F5;" align="center" + |Chronic
| style="background:#F5F5F5;" align="center" + |Chronic
Line 678: Line 482:
* Young adults
* Young adults
* [[Skin]], [[joint]] and [[eye]] lesions
* [[Skin]], [[joint]] and [[eye]] lesions
|-
| style="background:#DCDCDC;" align="center" + |'''Microscopic polyangitis ([[Microscopic polyangiitis|MPA]])'''<ref name="JennetteFalk1997">{{cite journal|last1=Jennette|first1=J. Charles|last2=Falk|first2=Ronald J.|title=Small-Vessel Vasculitis|journal=New England Journal of Medicine|volume=337|issue=21|year=1997|pages=1512–1523|issn=0028-4793|doi=10.1056/NEJM199711203372106}}</ref>
| style="background:#F5F5F5;" align="center" + |Chronic
| style="background:#F5F5F5;" + |
* Variable
| style="background:#F5F5F5;" align="center" + |✔
| style="background:#F5F5F5;" align="center" + |✔
| style="background:#F5F5F5;" align="center" + |✔
| style="background:#F5F5F5;" align="center" + |✔
| style="background:#F5F5F5;" align="center" + |✔
| style="background:#F5F5F5;" + |
* [[Hoarseness]]
* [[Stridor]]
* [[Wheeze|Wheezing]]
| style="background:#F5F5F5;" + |
* [[ANCA]] positive
* [[Blood urea nitrogen|BUN]]
* [[Creatinine]]
* [[Complete blood count]]
* [[Urinalysis]]
| style="background:#F5F5F5;" + |
* [[Cavitation]], [[Nodule (medicine)|nodules]], and alveolar opacities in [[chest X-ray]]
* Head and chest [[Computed tomography|CT]]
* [[Electromyography]]/[[nerve conduction study]]
| style="background:#F5F5F5;" + |
* Reduced [[lung volumes]]
| style="background:#F5F5F5;" + |
* Tissue [[biopsy]]
| style="background:#F5F5F5;" + |
* [[Nerve]] damage
* [[Rhinosinusitis]]
* [[Purpura]] involving lower extremities
|-
| style="background:#DCDCDC;" align="center" + |[[Eosinophilic granulomatosis with polyangiitis|'''Churg-Strauss''']]<ref name="pmid23330816">{{cite journal |vauthors=Vaglio A, Buzio C, Zwerina J |title=Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): state of the art |journal=Allergy |volume=68 |issue=3 |pages=261–73 |year=2013 |pmid=23330816 |doi=10.1111/all.12088 |url=}}</ref><ref name="pmid6366453">{{cite journal |vauthors=Lanham JG, Elkon KB, Pusey CD, Hughes GR |title=Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome |journal=Medicine (Baltimore) |volume=63 |issue=2 |pages=65–81 |year=1984 |pmid=6366453 |doi= |url=}}</ref>
| style="background:#F5F5F5;" align="center" + |Chronic
| style="background:#F5F5F5;" + |
* Variable
| style="background:#F5F5F5;" align="center" + |✔
| style="background:#F5F5F5;" align="center" + |✔
| style="background:#F5F5F5;" align="center" + |✔
| style="background:#F5F5F5;" align="center" + |✔
| style="background:#F5F5F5;" align="center" + |✔
| style="background:#F5F5F5;" + |
* [[Wheeze|Wheezing]]
* [[Rales]]
* [[Rhonchi]]
* Expiratory sounds(related to [[asthma]])
| style="background:#F5F5F5;" + |
* Peripherial [[eosinophilia]]
* In active phase [[C-reactive protein|CRP]] and [[Red blood cell|erytrocyte]] [[sedimentation]] rate high
* High levels of [[Immunoglobulin E|IgE]]
* [[Anti-neutrophil cytoplasmic antibody|ANCA]] positive
| style="background:#F5F5F5;" + |
* Infiltrates in [[Chest X-ray|chest X-Ray]]
* Ground glass opacities, tree-in-bud sign and small nodules  in chest [[Computed tomography|CT]]
| style="background:#F5F5F5;" + |
* [[Lung volumes]] decreased
* [[Vital capacity|FVC]] reduced
* [[FEV1/FVC ratio]] <70%
| style="background:#F5F5F5;" + |
* Tissue [[biopsy]]
| style="background:#F5F5F5;" + |
* [[Asthma]]
* [[Eosinophilia]]
* [[Rhinosinusitis]]
|-
| colspan="2" style="background:#DCDCDC;" align="center" + |[[Medication|'''Medication''']]
| style="background:#DCDCDC;" align="center" + |[[ACE inhibitor|'''ACE inhibitors''']]<ref name="pmid1616218">{{cite journal |vauthors=Israili ZH, Hall WD |title=Cough and angioneurotic edema associated with angiotensin-converting enzyme inhibitor therapy. A review of the literature and pathophysiology |journal=Ann. Intern. Med. |volume=117 |issue=3 |pages=234–42 |year=1992 |pmid=1616218 |doi= |url=}}</ref><ref name="pmid7619667">{{cite journal| author=Wood R| title=Bronchospasm and cough as adverse reactions to the ACE inhibitors captopril, enalapril and lisinopril. A controlled retrospective cohort study. | journal=Br J Clin Pharmacol | year= 1995 | volume= 39 | issue= 3 | pages= 265-70 | pmid=7619667 | doi= | pmc=1365002 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7619667  }}</ref>
| style="background:#F5F5F5;" align="center" + |Acute (depend on the medication)
| style="background:#F5F5F5;" + |
* From 2 weeks to 6 months
| style="background:#F5F5F5;" align="center" + |<nowiki>-</nowiki>
| style="background:#F5F5F5;" align="center" + |<nowiki>-</nowiki>
| style="background:#F5F5F5;" align="center" + |<nowiki>-</nowiki>
| style="background:#F5F5F5;" align="center" + |<nowiki>-</nowiki>
| style="background:#F5F5F5;" align="center" + |✔
| style="background:#F5F5F5;" + |
* [[Wheeze|Wheezing]]
| style="background:#F5F5F5;" + |
* Not  required
| style="background:#F5F5F5;" + |
* No required
| style="background:#F5F5F5;" + |
* Normal function
| style="background:#F5F5F5;" + |
* Clinical diagnosis
| style="background:#F5F5F5;" a+ |
* Resolves in four to five days of stopping the medication
*[[Angioedema]]
|}
|}


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[[Category:Medicine]]
[[Category:Medicine]]
[[Category:Pulmonary]]
[[Category:Up-To-Date]]
[[Category:Gastroenterology]]
[[Category:Pediatrics]]
[[Category:Pulmonology]]

Latest revision as of 19:22, 19 February 2019

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shaghayegh Habibi, M.D.[2], Karina Zavaleta, MD [3], Anmol Pitliya, M.B.B.S. M.D.[4]


Overview

Cystic fibrosis has to be differentiated from other conditions with similar presentation of cough and wheeze like common cold, asthma, bronchiolitis, emphysema, primary ciliary dyskinesia (Kartagener syndrome), bronchitis, bronchiectasis, foreign body aspiration, pneumoconiosis, interstitial lung disease, cardiogenic pulmonary edema, GERD and sarcoidosis.

Cough

Cystic fibrosis must be differentiated from other diseases presenting with cough and wheeze include:

Organ system Diseases Clinical manifestations Diagnosis Other features
Symptoms Physical exam
Onset Duration Productive cough Hemoptysis Weight lost Fever Dyspnea Ascultation Lab findings Imaging PFT Gold standard
Respiratory Upper airway diseases Common Cold[1] Acute
  • 3-10 days
 ✔ - - ✔ -
  • Bacterial culture is not indicated
  • Normal function
  • Clinical diagnosis
Lower airway Asthma[2] Chronic
  • Years
 ✔ Clear mucoid or yellow sputum - - - ✔
  • Family history
  • Seasonal variation
Acute Bronchitis[3] Acute
  • From 5 days to 1 or 3 weeks
 ✔ - - - ✔
  • FEV1 < 80%
  • Clinical diagnosis
Chronic Bronchitis[4][5] Chronic
  • Most of the days for three months in the las two years.
 ✔ Clear sputum - - ✔ ✔
Primary Ciliary Dyskinesia

(Kartagener Syndrome)

Chronic
  • Years
 ✔ ✔ ✔ - ✔
  • Mild to moderate obstructive ventilatory defect
  • Not specific
  • May be normal
Bronchiectasis[6] Chronic
  • Months to years
 ✔ Mucopurulent sputum ✔ - ✔
  • CT of chest
Emphysema [7] Chronic
  • Months to years
 ✔ Mucoid or purulent sputum - - ✔ ✔
  • Exposure of tobacco and air pollution
Foreign body aspiration[8][9][10] Acute
  • Variable
 ✔ ✔ - ✔ ✔
  • No specific
  • Not specific
  • In children <1 year and adults >75 years
  • Organic materials in children
  • Inorganic materials in adults
Bronchiolitis[11][12] Acute
  • 8-15 days
 ✔ - ✔ ✔
  • Clinical diagnosis
Parenchyma Cystic fibrosis [13][14] Chronic
  • Variable
 ✔ - ✔ ✔
  • Evidence of CFTR dysfunction
Pneumoconioses[15][16] Acute, Chronic
  • Years
 - - ✔ ✔ ✔
Interstitial lung disease[17][18] Chronic
  • Variable
 - ✔ ✔ - ✔
  • Lung biopsy when lab, imaging, and PFT has indeterminate result
Cardiac Cardiogenic pulmonary edema[19][20] Acute
  • Days to weeks
 ✔ Pink frothy, liquid - ✔ - ✔
  • Not specific
  • Clinical diagnosis
  • Tests are supportive
Gastrointestinal Gastroesophageal reflux[21][22] Chronic
  • Variable
 ✔ - ✔ - ✔
  • Not specific
  • Normal function
  • PH testing
 --
Autoinmune Sarcoidosis[23][24] Chronic
  • Years
 - - ✔ ✔ ✔

References

  1. ↑ Eccles R (2005). "Understanding the symptoms of the common cold and influenza". Lancet Infect Dis. 5 (11): 718–25. doi:10.1016/S1473-3099(05)70270-X. PMID 16253889.
  2. ↑ Ukena D, Fishman L, Niebling WB (2008). "Bronchial asthma: diagnosis and long-term treatment in adults". Dtsch Arztebl Int. 105 (21): 385–94. doi:10.3238/arztebl.2008.0385. PMC 2696883. PMID 19626179.
  3. ↑ Wenzel RP, Fowler AA (2006). "Clinical practice. Acute bronchitis". N. Engl. J. Med. 355 (20): 2125–30. doi:10.1056/NEJMcp061493. PMID 17108344.
  4. ↑ Brusasco V, Martinez F (2014). "Chronic obstructive pulmonary disease". Compr Physiol. 4 (1): 1–31. doi:10.1002/cphy.c110037. PMID 24692133.
  5. ↑ Qaseem A, Snow V, Shekelle P, Sherif K, Wilt TJ, Weinberger S, Owens DK (2007). "Diagnosis and management of stable chronic obstructive pulmonary disease: a clinical practice guideline from the American College of Physicians". Ann. Intern. Med. 147 (9): 633–8. PMID 17975186.
  6. ↑ King PT, Holdsworth SR, Freezer NJ, Villanueva E, Holmes PW (2006). "Characterisation of the onset and presenting clinical features of adult bronchiectasis". Respir Med. 100 (12): 2183–9. doi:10.1016/j.rmed.2006.03.012. PMID 16650970.
  7. ↑ Rossi A, Butorac-Petanjek B, Chilosi M, Cosío BG, Flezar M, Koulouris N; et al. (2017). "Chronic obstructive pulmonary disease with mild airflow limitation: current knowledge and proposal for future research - a consensus document from six scientific societies". Int J Chron Obstruct Pulmon Dis. 12: 2593–2610. doi:10.2147/COPD.S132236. PMC 5587130. PMID 28919728.
  8. ↑ Hewlett JC, Rickman OB, Lentz RJ, Prakash UB, Maldonado F (2017). "Foreign body aspiration in adult airways: therapeutic approach". J Thorac Dis. 9 (9): 3398–3409. doi:10.21037/jtd.2017.06.137. PMC 5708401. PMID 29221325.
  9. ↑ Rafanan AL, Mehta AC (2001). "Adult airway foreign body removal. What's new?". Clin. Chest Med. 22 (2): 319–30. PMID 11444115.
  10. ↑ Haddadi S, Marzban S, Nemati S, Ranjbar Kiakelayeh S, Parvizi A, Heidarzadeh A (2015). "Tracheobronchial Foreign-Bodies in Children; A 7 Year Retrospective Study". Iran J Otorhinolaryngol. 27 (82): 377–85. PMC 4639691. PMID 26568942.
  11. ↑ Bordley WC, Viswanathan M, King VJ, Sutton SF, Jackman AM, Sterling L, Lohr KN (2004). "Diagnosis and testing in bronchiolitis: a systematic review". Arch Pediatr Adolesc Med. 158 (2): 119–26. doi:10.1001/archpedi.158.2.119. PMID 14757603.
  12. ↑ "www.nice.org.uk".
  13. ↑ Farrell PM, Rosenstein BJ, White TB, Accurso FJ, Castellani C, Cutting GR, Durie PR, Legrys VA, Massie J, Parad RB, Rock MJ, Campbell PW (2008). "Guidelines for diagnosis of cystic fibrosis in newborns through older adults: Cystic Fibrosis Foundation consensus report". J. Pediatr. 153 (2): S4–S14. doi:10.1016/j.jpeds.2008.05.005. PMC 2810958. PMID 18639722.
  14. ↑ Kerem E, Reisman J, Corey M, Canny GJ, Levison H (1992). "Prediction of mortality in patients with cystic fibrosis". N. Engl. J. Med. 326 (18): 1187–91. doi:10.1056/NEJM199204303261804. PMID 1285737.
  15. ↑ Jp NA, Imanaka M, Suganuma N (2017). "Japanese workplace health management in pneumoconiosis prevention". J Occup Health. 59 (2): 91–103. doi:10.1539/joh.16-0031-RA. PMC 5478517. PMID 27980247.
  16. ↑ Weiland DA, Lynch DA, Jensen SP, Newell JD, Miller DE, Crausman RS, Kuhn C, Kern DG (2003). "Thin-section CT findings in flock worker's lung, a work-related interstitial lung disease". Radiology. 227 (1): 222–31. doi:10.1148/radiol.2271011063. PMID 12668748.
  17. ↑ Lama VN, Martinez FJ (2004). "Resting and exercise physiology in interstitial lung diseases". Clin. Chest Med. 25 (3): 435–53, v. doi:10.1016/j.ccm.2004.05.005. PMID 15331185.
  18. ↑ Chetta A, Marangio E, Olivieri D (2004). "Pulmonary function testing in interstitial lung diseases". Respiration. 71 (3): 209–13. doi:10.1159/000077416. PMID 15133338.
  19. ↑ Gheorghiade M, Zannad F, Sopko G, Klein L, Piña IL, Konstam MA, Massie BM, Roland E, Targum S, Collins SP, Filippatos G, Tavazzi L (2005). "Acute heart failure syndromes: current state and framework for future research". Circulation. 112 (25): 3958–68. doi:10.1161/CIRCULATIONAHA.105.590091. PMID 16365214.
  20. ↑ Yancy CW, Jessup M, Bozkurt B, Butler J, Casey DE, Drazner MH, Fonarow GC, Geraci SA, Horwich T, Januzzi JL, Johnson MR, Kasper EK, Levy WC, Masoudi FA, McBride PE, McMurray JJ, Mitchell JE, Peterson PN, Riegel B, Sam F, Stevenson LW, Tang WH, Tsai EJ, Wilkoff BL (2013). "2013 ACCF/AHA guideline for the management of heart failure: a report of the American College of Cardiology Foundation/American Heart Association Task Force on practice guidelines". Circulation. 128 (16): e240–327. doi:10.1161/CIR.0b013e31829e8776. PMID 23741058.
  21. ↑ Kahrilas PJ, Hughes N, Howden CW (2011). "Response of unexplained chest pain to proton pump inhibitor treatment in patients with and without objective evidence of gastro-oesophageal reflux disease". Gut. 60 (11): 1473–8. doi:10.1136/gut.2011.241307. PMID 21508423.
  22. ↑ Badillo R, Francis D (2014). "Diagnosis and treatment of gastroesophageal reflux disease". World J Gastrointest Pharmacol Ther. 5 (3): 105–12. doi:10.4292/wjgpt.v5.i3.105. PMC 4133436. PMID 25133039.
  23. ↑ Carmona EM, Kalra S, Ryu JH (2016). "Pulmonary Sarcoidosis: Diagnosis and Treatment". Mayo Clin. Proc. 91 (7): 946–54. doi:10.1016/j.mayocp.2016.03.004. PMID 27378039.
  24. ↑ Yanardağ H, Pamuk GE, Karayel T, Demirci S (2002). "Bone marrow involvement in sarcoidosis: an analysis of 50 bone marrow samples". Haematologia (Budap). 32 (4): 419–25. PMID 12803116.

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