Restrictive Lung Diseases

Posted by e-Medical PPT
Chronic Restrictive Lung Diseases
Group of different diseases
Similar clinical, pulmonary function test and pathological findings
Represent 15% of non-infectious diseases of lungs
End-stage: diffuse interstitial pulmonary fibrosis (Honeycomb lung)

Primary lung disease
Acute: Acute Respiratory Distress Syndrome ARDS
Occupational: Asbestosis, silicosis, coal worker pneumoconiosis
Interstitial lung disease (interstitial pneumonia), Idiopathic pulmonary fibrosis
Immune diseases: Sarcoidosis, SLE, RA, Wegener
Physical injury: : Radiation
Drugs: Chemotherapy, methotrexate

Idiopathic Pulmonary Fibrosis
Cryptogenic fibrosing alveolitis
Unknown etiology
Diffuse interstitial fibrosis
Sever hypoxemia, and cyanosis
Male>Female, 60y
Diagnosis of exclusion

Alveolar wall injury
Alveolitis: inflammation, edema
Neutrophil, macrophages, lymphocytes
Interstitial Fibrosis
? Immune disorder: association with immune diseases: RA, SLE, Sjogren….
Macrophages: IL-8, FGF, TGF-beta, PDGF

Multisystem disease
Unknown etiology
Non-caseating granuloma in many tissues and organs
Diagnosis of exclusion

Presenting Picture:
Bilateral hilar lymphadenopathy
Lung involvement, lung nodules
Other organs: skin, eye, any tissue
Adult, younger than <40y
Common in US blacks
Higher in non-smokers..

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