Internal Medicine

0521779407-10 CUNY1086/Karliner 0 521 77940 7 June 7, 2007 18:40

Interstitial Lung Diseases 843

Pathology usual interstitial pneumonia (UIP)

Patchy lung involvement (subpleural, basilar)

Familial form (autosomal dominant)

➣Nonspecific interstitial pneumonia (NSIP): uniform cellular alve-

olar wall infiltration

➣Acute interstitial pneumonia (Hamman-Rich syndrome: rapid

onset and death in months; uniform fibrosis

➣Respiratory bronchiolitis and ILD:

Desquamative interstitial pneumonitis (DIP)

Cigarette abuse

Intraalveolar macrophages

➣Unclassified (primary) disease:

Diagnosis: clinical or imaging may avoid biopsy:

Sarcoid

Eosinophilic granuloma

Lymphangioleiomyomatosis (LAM)

Tuberous sclerosis

Amyloid

Eosinophilic pneumonia

Alveolar proteinosis

➣Autoimmune lung fibrosis:

IBD

Primary biliary cirrhosis

Thyroiditis

➣Other:

Bronchiolitis obliterans organizing pneumonia (BOOP)

Lymphocytic interstitial pneumonia (LIP) (AIDS, connective

tissue disease, thyroiditis)

HIV: nonspecific interstitial pneumonia

Pulmonary hemorrhage syndromes; hemoptysis may be

absent

management

What to Do First

■Establish diagnosis

■Clinical, radiographic &/or biopsy

General Measures

■Eliminate relevant exposures:

➣Cigarettes in DIP, eosinophilic granuloma

➣Antigen in hypersensitivity pneumonitis