Clinical features of major causes of immune or idiopathic alveolar hemorrhage

Disorder	Extrapulmonary features	Serology	Essential diagnostic criteria
Anti-GBM disease	GN >90%	+Anti-GBM antibodies	+Serum anti-GBM antibodies
	Extrarenal disease rare		Linear IgG in glomeruli
SLE	GN >90%	+ANA	Clinical features, +ANA, immune complexes in glomeruli by IF, EM
	Extrarenal disease common (arthritis, serositis, purpura, photosensitivity)
GPA	GN >90%	+ANCA	Necrotizing granulomatous vasculitis in upper airway or lung biopsy or Pauci-immune necrotizing GN with extensive upper airway disease or cavitary lung nodules
	Extrarenal disease common
	Distinctive features include upper airway disease, pulmonary nodules
	Nonspecific features may include arthritis, purpura, mononeuropathy
Nonspecific systemic necrotizing vasculitis (microscopic polyarteritis, polyangiitis, overlap syndrome)	GN >90%	±ANCA (usually + if include p-ANCA)	Necrotizing, crescentic GN (or pathologic evidence of vasculitis elsewhere) and exclude GPA to extent possible
	Extrarenal features may include arthritis, purpura, mononeuropathy
Idiopathic pulmonary hemosiderosis	None	± IgA tissue transglutaminase antibodies ± IgA endomysial antibodies	Exclusion of all other causes

Anti-GBM: glomerular basement membrane antibodies; GN: glomerulonephritis; SLE: systemic lupus erythematosus; ANA: antinuclear antibody; IF: immunofluorescence; EM: electron microscopy; GPA: granulomatosis with polyangiitis; ANCA: antineutrophil cytoplasmic antibody; p-ANCA: perinuclear ANCA; IgA: immunoglobulin A.

Redrawn from: Leatherman JL. In: Immunologically Mediated Pulmonary Diseases, Lynch JP, DeRemee RA (Eds), JB Lippincott, Philadelphia, 1991, p.490.

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خرید پکیج

Clinical features of major causes of immune or idiopathic alveolar hemorrhage