Interstitial lung disease

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Interstitial Lung Disease


Patient Information



Fibrosis lung response
Occupational lung disease
Drug-induced lung injury
Radiation-induced lung injury
Smoking related interstitial lung disease
Idiopathic interstitial pneumonia
Pulmonary alveolar proteinosis
Lymphocytic infiltrative disorders
Pulmonary lymphangioleiomyomatosis
Pulmonary hemorrhage syndromes
Interstitial lung disease associated with systemic diseases
Granulomatous lung response


Differentiating Interstitial Lung Disease from other Diseases

Laboratory Finidngs

For the WikiPatient page for this topic, click here

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sadaf Sharfaei M.D.[2], Anmol Pitliya, M.B.B.S. M.D.[3], Saarah T. Alkhairy, M.D.

Synonyms and keywords: Diffuse parenchymal lung disease; DPLD; ILD


Interstitial lung disease is a group of disorders involving pulmonary parenchyma. The exact pathogenesis of these disorders is not completely understood. There are multiple initiating factors that may lead to pulmonary injury. However, immunopathogenic responses of lung tissue are quite similar. The major histopathologic patterns in response to lung injury include inflammation, fibrosis and granulomatous response. Interstitial lung disease may be classified into several subtypes based on the lung response to tissue injury and the cause of injury. The underlying cause of interstitial lung disease may include factors such as toxic environmental or occupational exposure, cigarette smoking, and radiation. Interstitial lung disease may also be idiopathic.


Interstitial lung disease may be classified on the basis of lung response to tissue injury. The lung response to tissue injury may be characterized as:[1]

Interstitial lung disease
Lung Response:
Lung Response:
Interstitial Inflammation,
and Fibrosis
Idiopathic (Unknown)
Hypersensitivity pneumonitis (organic dusts)
Inorganic dusts
Lymphomatoid granulomatosis
Granulomatous vasculitides
Bronchocentric granulomatosis
Eosinophilic granulomatosis with polyangiitis (Churg Strauss syndrome)
Granulomatosis with polyangiitis (Wegener's)
Known cause
Idiopathic (Unknown)
Drug-induced pulmonary toxicity
Occupational and environmental exposure
Radiation-induced lung injury
Aspiration pneumonia
Residual of acute respiratory distress syndrome
Inhaled inorganic dust
Inhaled organic dusts
Inhaled agents other than inorganic or organic dusts
Desquamative interstitial pneumonia
Respiratory bronchiolitis–associated interstitial lung disease
Pulmonary Langerhans cell granulomatosis
Pulmonary alveolar proteinosis
Idiopathic interstitial pneumonias
Lymphocytic infiltrative disorders
(lymphocytic interstitial pneumonitis
associated with connective tissue disease)
Connective tissue
Gastrointestinal or
liver diseases
Inherited diseases
Graft-versus-host disease
Pulmonary hemorrhage syndromes
Major idiopathic interstitial pneumonias
Rare idiopathic interstitial pneumonias
Unclassifiable idiopathic interstitial pneumonias
• Idiopathic pulmonary fibrosis
• Idiopathic nonspecific interstitial pneumonia
• Respiratory bronchiolitis-interstitial lung disease
• Desquamative interstitial pneumonia
Cryptogenic organising pneumonia
• Acute interstitial pneumonia
• Idiopathic lymphoid interstitial pneumonia
• Idiopathic pleuroparenchymal fibroelastosis
Systemic lupus erythematosus
Rheumatoid arthritis
Ankylosing spondylitis
Systemic sclerosis
Sjögren syndrome
Crohn disease
Primary biliary cirrhosis
• Chronic active hepatitis
Ulcerative colitis
Tuberous sclerosis
Niemann-Pick disease
Gaucher disease
• Hermansky-Pudlak syndrome
Bone marrow transplantation
• Solid organ transplantation
Goodpasture syndrome
• Idiopathic pulmonary hemosiderosis
• Isolated pulmonary capillaritis


  • Interstitial lung disease is a group of disorders affecting pulmonary parenchyma.
  • The exact pathogenesis of these disorders is not completely understood.
  • There are multiple initiating factors that may cause pulmonary injury. However, immunopathogenic responses of lung tissue are quite similar.
  • There are two major histopathologic patterns in response to lung injury which include:

Algorithm showing pathophysiology of Interstitial Lung Disease[2]

Tissue injury in lungs
Parenchymal injury
Vascular injury
Mast cells in lungs in response to tissue injury
LPA6, LPA2, and LPA4 receptors[3]
Decreased sFRP-1 (secreted frizzled-related protein 1) in fibroblasts[4]
Secretes tryptase
Transforming growth factor-β (TGF-β)[5]
Insulin-like growth factor (IGF) signalling[4]
Reduced expression of angiogenic factors,
vascular endothelial growth factor (VEGF)[6]
Elevation of angiostatic factors,
pigment epithelium-derived factor[7]
Wnt/β-catenin signalling pathway[8][9]
PAR-2/protein kinase (PK)C-α/Raf-1/p44/42 signaling pathway[10]
Upregulation of Egr-1 (early growth response protein 1)[11]
IGF-binding protein 5 (IGFBP-5)[12]
IGF-binding protein 3 (IGFBP-3)
Loss of endothelial barrier function
Dysregulation of repair in lung tissue and activation of fibroblasts[13]
Regulates transforming growth factor-β (TGF-β)
Induction of syndecan-2 (SDC2)[14]
Activation,proliferation, and migration of fibroblast to the site of injury
Altered PTEN (phosphatase and tensin homologue)/Akt axis
Acquire contractile stress fibres
Inactivates Fox (forkhead box) O3a[15]
Protomyofibroblast, composed of cytoplasmic actins
Pleural mesothelial cells (PMCs)[16][17]
Downregulation of caveolin-1 (cav-1) and Fas expression[18]
De novo expression of α-smooth muscle actin (α-SMA)
TGF-β1-dependent mesothelial–mesenchymal transition
Fibroblast resistant to apoptosis[19]
Different ranges of contractions mediated by RhoA/Rho-associated kinase
Changes in intracellular calcium concentrations
Recruitement of fibrocytes in lungs
Lock step mechanism of cyclic and contractile events[21]
T-helper cell type 2 on site of injury[22][23]
Upregulation of C-X-C chemokine receptor type 4 (CXCR4)
on fibrocytes and its ligand
CXCL12 (stromal cell-derived factor 1)[24]
Excess extracellular matrix production
Exerting traction force
Migration of fibrocytes to the site of injury[25]
Tissue remodelling[26]
Alternate pathway activation of macrophages[27]
Lung Fibrosis

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[28] 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[29] Acute/Chronic 50−60 years old Female + + + + + + ±
  • Normal
  • Nonspecific
  • Lung biopsy and multidisciplinary approach
Cryptogenic organizing pneumonia[30] 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)[31] Acute 50−60 years old Both + + +
  • N/A
Lymphocytic interstitial pneumonia[32] Subacute 30−40 years old Female ± + + + + +
Respiratory bronchiolitis−interstitial lung disease[33] 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[34][35] 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[36] Chronic 20−40 years old Both + + ± Dry + +
  • Unremarkable
  • Nonspecific 
  • Normal
Pulmonary alveolar proteinosis[37][38] 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[39] Acute/chronic 30−40 years old Female + + + Bloody + + +
Eosinophilic pneumonia[40] Acute/chronic 20−40 years old Male + Dry + + +
  • Clinical evaluation and investigations
Hypersensitivity pneumonitis[41] 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[42] Chronic Elderly Male + + + ± + + + + Peripheral/central +
  • Mineral dust +
  • History of environmental exposure and imaging
  • Lung biopsy not required
Radiation−induced lung injury[43] Subacute/chronic Any age Both + + Dry + + + +
  • Nonspecific
  • History of irradiation and clinical presentation
Pulmonary hemorrhage syndromes Goodpasture syndrome[44] Chronic All ages Male + ± ± Bloody ±
  • Pulmonary infiltrates
  • Normal
  • NA
Idiopathic pulmonary hemosiderosis[45] Acute/subacute/chronic Children − 10 years old Both + ± + Bloody + +
  • O2
  • ↓ CO2
  • Clinical evaluation and investigations
Isolated pulmonary capillaritis[46] 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[47] Acute/subacute/chronic 20−40 years old Female + ± ± + + ± +
  • Clinical evaluation and investigations
Granulomatous vasculitides Granulomatosis with polyangiitis (Wegener)[48] Chronic Elderly Both + + + + ±
  • Alveolar hemorrhage
Eosinophilic granulomatosis with polyangiitis (Churg Strauss)[49] Chronic 40−50 years old Both + + +
  • Areas of parenchymal opacification
  • Mixed interstitial patchy alveolar opacities
  • Normal
Bronchocentric granulomatosis[50] Chronic 30−70 years old Both ± ± + ±
  • Normal
Pulmonary lymphomatoid granulomatosis[51] Chronic 30−50 years old Male + + + +
  • Normal
  • Mid to lower zone multiple poorly defined nodules
  • Diffuse reticular abnormalities
  • Normal
  • Normal
Amyloidosis[52][53] 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

Laboratory Finidngs

There are multiple laboratory tests that may be helpful to ascertain or rule out the diagnosis of interstitial lung disease.

Condition Disease Investigation
All patients with suspicious interstitial lung disease Complete blood count and differential
Liver function tests Alanine aminotransferase (ALT, SGPT)
Aspartate aminotransferase (AST, SGOT)
Alkaline phosphatase
Renal function tests Urinalysis
Blood urea nitrogen (BUN)
Creatinine (Cr)
Suspicious of systemic rheumatic disease RA Serology Anti−cyclic citrullinated peptide (Anti-CCP)
SLE Serology Anti−double stranded DNA antibodies (Anti-dsDNA antibody)
Amyopathic dermatomyositis Serology Anti-melanoma differentiation-associated gene 5 (MDA-5)
Nonspecific Serology Antinuclear antibody (ANA)
Serology Rheumatoid factor (RF)
Serology Anti-neutrophil cytoplasmic antibody (ANCA)
Enzyme Creatine kinase (CK), aldolase
Mechanic hands Myositis Myositis−associated antibodies Anti-tRNA synthetases Jo-1
Anti-tRNA synthetases PL-7
Anti-tRNA synthetases PL-12
Sicca features or positive anti−extractable nuclear antigen (ENA) Sjögren’s syndrome Serology Anti-RO (SS−A)
Serology Anti-La (SS−B)
Mixed connective tissue disease  Serology Anti-ribonucleoprotein (RNP)
IgG4-related disease Serology Serum IgG4
Severe GERD or sclerodactyly Limited systemic scleroderma Serology Anti-centromere
Systemic scleroderma Serology Anti-topoisomerase I (anti-Scl-70)
Dyspnea Heart failure Enzyme Brain natriuretic peptide (BNP)
Pulmonary hypertension N-terminal proBNP (NT-proBNP)
Anemia and/or hemoptysis Coagulopathies Coagulation studies
Goodpasture syndrome Serology Anti−glomerular basement membrane (GBM) antibodies
Antiphospholipid syndrome Serology Antiphospholipid antibodies
Idiopathic pulmonary hemosiderosis Serology Serum IgA endomysial or tissue transglutaminase antibodies 
Mediastinal lymphadenopathy Multiple myeloma Serum protein electrophoresis
Beryllium exposure Berylliosis Peripheral blood beryllium lymphocyte proliferation test
Risk factors for HIV HIV ELISA
Western blot test

Occupational Lung Disease

For more information about occupational lung disease, click here.

Drug-induced lung injury

More than 600 medications have pulmonary toxicity and may cause lung injury.[55]

Drug−induced lung injury
Antimicrobial Agents Anti−Inflammatory Agents Biological Agents Cardiovascular Agents Immunomodulator agents Antineoplastic agents Miscellaneous

Radiation-induced Lung Injury

Radiation has been considered as one of the causes of lung injury. About 5 to 15% of patients receiving radiation therapy may present with pulmonary symptoms.[71]

Smoking related Interstitial Lung Disease

Cigarette smoking may cause various adverse effects on pulmonary tissue.[74][75][76]

Idiopathic Interstitial Pneumonia

The idiopathic interstitial pneumonias (IIP) are a broad range of interstitial lung diseases of unknown etiology.[77][78][79][80][81][82]

For more information about Idiopathic interstitial pneumonia, click here.

For more information about idiopathic pulmonary fibrosis, click here.

For more information about Cryptogenic organizing pneumonia, click here.

Pulmonary Alveolar Proteinosis

Pulmonary alveolar proteinosis (PAP) is a rare lung disease that is characterized by the intra-alveolar accumulation of surfactant phospholipid and apoproteins.[83][84]

For more information about pulmonary alveolar proteinosis, click here.

Lymphocytic Infiltrative Disorders

Lymphocytic infiltrative disorders might cause interstitial lung disease in mostly HIV positive children.[85][86]

  • The etiology of lymphocytic infiltrative disorders is unknown. However, there is an evidence of infectious cause such as EBV in HIV positive patients.
  • The two main manifestations of lymphocytic infiltrative disorders include:

For more information about lymphocytic interstitial pneumonitis, click here.

Pulmonary Lymphangioleiomyomatosis

Lymphangiomyocytosis (LAM) is defined as a multifocal neoplasm with differentiation of the perivascular epithelioid cell that involves multiple organs.[87]

For more information about pulmonary lymphangioleiomyomatosis, click here.

Pulmonary Hemorrhage Syndromes

Pulmonary hemorrhage syndromes might cause interstitial lung disease.

  • Several pulmonary hemorrhage syndromes that affect the lung parenchyma and eventually lead to pulmonary fibrosis. Some of these are as follows:

Interstitial Lung Disease Associated with Systemic Diseases

Interstitial lung disease associated with connective tissue diseases

Interstitial lung disease associated with inherited diseases

Interstitial lung disease associated with gastrointestinal or liver diseases

Interstitial lung disease associated with graft−versus−host disease

Granulomatous Lung Response

For more information about hypersensitivity pneumonitis, click here.


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