Disorder Unknown Cause Commonly Affects Young

Granulomatous DPLD • Sarcoidosis – – – –

Multi-system disorder Unknown cause Commonly affects young and middle-aged adults Most common presentation: ILD, thoracic LN enlargement – Other organ involvment: skin, eyes, nose/sinuses, liver, heart, CNS – Histology: non-caseating epithelioid granulomas (w/out evidence of infxn or foreign body rxn) – Immunological features: ↓ cutaneous DTH and ↑ Th1 response at sites of disease ATS, AJRCCM 1999; 160:736-755

Sarcoidosis

Sarcoidosis

Sarcoidosis

Flow-Volume Loop

Pressure-Volume Curve

ILD: Diagnosis • History is critical • HRCT pattern • Bronchoscopic transbronchial Bx — too small for Dx of most diseases, except: – granulomatous processes (e.g., sarcoidosis, HSP) – neoplasm – infection

• Surgical lung Bx – via thoracotomy (OLBx) or video-assisted thoracoscopic surgery (VATS) – gold standard for Dx of most ILD

ILD: Treatment • Removal from offending agent, if identified • Corticosteroids – IPF: infrequent response (≤ 25%) – Sarcoidosis: frequent response

• Cytotoxic agents – azathioprine – cyclophosphamide

• Immunmomodulating/anti-fibrotic agents – interferon-γ1b, etanercept, bosentan, imatinab

ILD: Treatment • Oxygen • Influenza and pneumococcal vaccines • Pulmonary rehabilitation • Lung transplantation