Collagen vascular diseases
(Chest 2013;143:814)
- Rheumatologic disease
Scleroderma: ILD in ~50%; PHT seen in ~10% of Pts with limited disease
PM-DM: ILD & skin/muscle findings; MCTD: PHT & fibrosis; Sjogren’s: ILD & sicca sx
SLE & RA: pleuritis and pleural effusions more often than ILD; SLE can cause DAH
- Vasculitis (can p/w DAH)
Granulomatosis w/ polyangiitis (GPA): ⊕ c-ANCA w/ necrotizing granulomas
Eosinophilic GPA (EGPA): ⊕ c- or p-ANCA w/ eosinophilia & necrotizing granulomas
Microscopic polyangiitis: ⊕ p-ANCA w/o granulomas
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Goodpasture’s syndrome = DAH + RPGN; typically in smokers; ⊕ anti-GBM in 90%
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Lymphangioleiomyomatosis (LAM): cystic, ↑ in ♀, Rx w/ sirolimus (NEJM 2011;364:1595)