Interstitial lung disease: Difference between revisions

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==Classification==
==Classification==
<small>
{{Family tree/start}}
{{Family tree| | | | | | | | | | | | | | | A01 | | | | | | | | | | | | A01=Interstitial lung disease}}
{{Family tree| | | | | | | | | | |,|-|-|-|-|^|-|-|-|-|-|-|-|-|.| |}}
{{Family tree| | | | | | | | | | B01 | | | | | | | | | | | | B02 | | | | | |B01=Lung Response:<br>Granulomatous|B02=Lung Response:<br>Alveolitis,<br>Interstitial Inflammation,<br>and Fibrosis }}
{{Family tree| | | | | | |,|-|-|-|^|-|-|-|.| | | | | | | | | |!| | | | | | | | | | |}}
{{Family tree| | | | | | C01 | | | | | | C02 | | | | | | | | |!| | | | | | | | | | | |C01=Known|C02=Idiopathic (Unknown)}}
{{Family tree| | |,|-|-|-|(| | | |,|-|-|-|+|-|-|-|v|-|-|-|.| |!| | | | | | | | | | |}}
{{Family tree| | D01 | | D02 | | D03 | | D04 | | D05 | | D06 |!| | | | | | | | | | |D02=Inorganic dusts|D01=Hypersensitivity pneumonitis (organic dusts)|D03=Sarcoidosis||D04=Lymphomatoid granulomatosis|D05=Granulomatous vasculitides|D06=Bronchocentric granulomatosis}}
{{Family tree| | | | |,|-|^|-|.| | | | | | | |,|-|^|-|.| | | |!| | | | | | | | | | |}}
{{Family tree| | | | E01 | | E02 | | | | | | D07 | | D08 | | |!| | | | | | | | | | |E01=Beryllium|E02=Silica|D07=Eosinophilic granulomatosis with polyangiitis (Churg Strauss syndrome)|D08=Granulomatosis with polyangiitis (Wegener)}}
{{Family tree| | | | | | | | | | | | | | | | | | | | | | | | |!| | | | | | | | | | | | | | | | | | |}}
{{Family tree| | | | | | | | | | | | | | |,|-|-|-|-|-|-|-|-|-|(| | | | | | | | | | | | |}}
{{Family tree| | | | | | | | | | | | | | F01 | | | | | | | | F02 | | | | | | | | | | | | | | F01=Known cause|F02=Idiopathic (Unknown)}}
{{Family tree| |,|-|-|-|v|-|-|-|v|-|-|-|v|-|-|-|-|-|-|-|.| | |!| | | | | | | | | | | | | | | | | | | }}
{{Family tree| G01 | | G06 | | G02 | | G03 | | G04 | | G05 | |!| | | | | | | | | | | | | | | | | | | G01=Drug-induced pulmonary toxicity|G06=Occupational and environmental exposure|G02=Radiation-induced lung injury|G03=Aspiration pneumonia|G05=Residual of acute respiratory distress syndrome|G04=Smoking-related}}
{{Family tree| | | | | |!| | | | | | | | | | | |!| | | | | | |!| | | | | | | | | | | | | | | | | | | }}
{{Family tree| |,|-|-|-|+|-|-|-|.| | | |,|-|-|-|+|-|-|-|.| | |!| | | | | | | | | | | | | | | | | | | }}
{{Family tree| I01 | | I02 | | I03 | | G07 | | G08 | | G09 | |!| | | | | | | | | | | | | | | | | | |I01=Inhaled inorganic dust|I02=Inhaled organic dusts|I03=Inhaled agents other than inorganic or organic dusts|G07=Desquamative interstitial pneumonia|G08=Respiratory bronchiolitis–associated interstitial lung disease|G09=Pulmonary Langerhans cell granulomatosis|}}
{{Family tree| | | | | | | | | | | | | | | | | | | | | | | | |!| | | | | | | | | | | | | | | | | | | }}
{{Family tree| | | | | | | | | | | | | | | | | | | | | | | | |!| | | | | | | | | | | | | | | | | | | }}
{{Family tree| | | | | | | | | | | | | | | | | | | | | | | | |!| | | | | | | | | | | | | | | | | | | }}
{{Family tree| |,|-|-|-|v|-|-|-|v|-|-|-|v|-|-|-|v|-|-|-|v|-|-|^|v|-|-|-|v|-|-|-|v|-|-|-|v|-|-|-|.|}}
{{Family tree| H01 | | H07 | | H02 | | H08 | | H10 | | H09 | | H11 | | H06 | | H03 | | H04 | | H05 |H07=Idiopathic interstitial pneumonias|H01=Pulmonary alveolar proteinosis|H02=Lymphocytic infiltrative disorders<br>(lymphocytic interstitial pneumonitis<br>associated with connective tissue disease)|H08=Connective tissue<br>diseases|H03=Eosinophilic<br>pneumonias|H09=Inherited diseases|H04=Lymphangioleiomyomatosis|H10=Gastrointestinal or<br>liver diseases|H05=Amyloidosis|H11=Graft-versus-host disease|H06=Pulmonary hemorrhage syndromes}}
{{Family tree| | | | | |!| | | | | | | |!| | | |!| | | |!| | | |!| | | |!| | | | | | | | | | }}
{{Family tree| |,|-|-|-|+|-|-|-|.| | | |!| | | |!| | | |!| | | |!| | | |!| | | | | | | | | | }}
{{Family tree| I04 | | I05 | | I06 | | |!| | | |!| | | |!| | | |!| | | |!| | | | | | | | | | |I04=Major idiopathic interstitial pneumonias|I05=Rare idiopathic interstitial pneumonias|I06=Unclassifiable idiopathic interstitial pneumonias}}
{{Family tree| |!| | | |!| | | | | | | |!| | | |!| | | |!| | | |!| | | |!| | | | | | | | | |}}
{{Family tree| |!| | | |!| | | | | | | |!| | | |!| | | |!| | | |!| | | |!| | | | | | | | | | }}
{{Family tree|boxstyle=text-align: left; | J01 | | J02 | | | | | | J07 | | J09 | | J08 | | J10 | | J11 | | | | | | | | |J01=• Idiopathic pulmonary fibrosis<br>• Idiopathic nonspecific interstitial pneumonia<br>• Respiratory bronchiolitis-interstitial lung disease<br>• Desquamative interstitial pneumonia<br>• Cryptogenic organising pneumonia<br>• Acute interstitial pneumonia<br>|J02=• Idiopathic lymphoid interstitial pneumonia<br>• Idiopathic pleuroparenchymal fibroelastosis<br>|J07=• Systemic lupus erythematosus<br>• Rheumatoid arthritis<br>• Ankylosing spondylitis<br>• Systemic sclerosis<br>• Sjögren syndrome<br>• Polymyositis<br>• Dermatomyositis<br>|J08=• Tuberous sclerosis<br>• Neurofibromatosis<br>• Niemann-Pick disease<br>• Gaucher disease<br>• Hermansky-Pudlak syndrome<br>|J09=• Crohn disease<br>• Primary biliary cirrhosis<br>• Chronic active hepatitis<br>• Ulcerative colitis<br>|J10=• Bone marrow transplantation<br>• Solid organ transplantation<br>|J11=• Goodpasture syndrome<br>• Idiopathic pulmonary hemosiderosis<br>• Isolated pulmonary capillaritis
|}}
{{Family tree| | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | }}
{{Family tree| | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | }}
{{Family tree| | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | }}
{{Family tree| | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | | }}
{{Family tree/end}}
</small>


==Pathophysiology==
==Pathophysiology==

Revision as of 21:43, 7 March 2018

Interstitial Lung Disease

Home

Patient Information

Overview

Classification

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

Pathophysiology

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: Saarah T. Alkhairy, M.D.

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

Overview

Classification

 
 
 
 
 
 
 
 
 
 
 
 
 
 
Interstitial lung disease
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Lung Response:
Granulomatous
 
 
 
 
 
 
 
 
 
 
 
Lung Response:
Alveolitis,
Interstitial Inflammation,
and Fibrosis
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Known
 
 
 
 
 
Idiopathic (Unknown)
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Hypersensitivity pneumonitis (organic dusts)
 
Inorganic dusts
 
Sarcoidosis
 
Lymphomatoid granulomatosis
 
Granulomatous vasculitides
 
Bronchocentric granulomatosis
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Beryllium
 
Silica
 
 
 
 
 
Eosinophilic granulomatosis with polyangiitis (Churg Strauss syndrome)
 
Granulomatosis with polyangiitis (Wegener)
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Known cause
 
 
 
 
 
 
 
Idiopathic (Unknown)
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Drug-induced pulmonary toxicity
 
Occupational and environmental exposure
 
Radiation-induced lung injury
 
Aspiration pneumonia
 
Smoking-related
 
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
diseases
 
Gastrointestinal or
liver diseases
 
Inherited diseases
 
Graft-versus-host disease
 
Pulmonary hemorrhage syndromes
 
Eosinophilic
pneumonias
 
Lymphangioleiomyomatosis
 
Amyloidosis
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
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
• Polymyositis
• Dermatomyositis
 
• Crohn disease
• Primary biliary cirrhosis
• Chronic active hepatitis
• Ulcerative colitis
 
• Tuberous sclerosis
• Neurofibromatosis
• Niemann-Pick disease
• Gaucher disease
• Hermansky-Pudlak syndrome
 
• Bone marrow transplantation
• Solid organ transplantation
 
• Goodpasture syndrome
• Idiopathic pulmonary hemosiderosis
• Isolated pulmonary capillaritis
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 

Pathophysiology

References

Template:Respiratory pathology


de:Interstitielle Lungenerkrankung fi:Keuhkoparenkyymisairaudet


Template:WikiDoc Sources