Differential diagnosis of Granulomatosis with polyangiitis
The below table lists the distinguishing features of various syndromes that may mimic Granulomatosis with polyangiitis.
Distinguishing Features of Syndromes that May Mimic GPA
| Syndrome | Example | Distinguishing Features |
|---|---|---|
| Primary vasculitis syndromes | EGPA | Atopic history |
| Marked eosinophilia | ||
| Microscopic polyangiitis | Destructive upper airway disease unusual | |
| Cavitary pulmonary nodules unusual | ||
| Absence of granuloma | ||
| Angiocentric immunoproliferative lesions | Lymphomatoid granulomatosis | Glomerulonephritis unusual |
| Pulmonary renal syndromes | Goodpasture disease | Anti-basement membrane antibodies |
| Immunofluorescence: linear deposition | ||
| Immune complex disease (e.g., SLE) | ANA, anti-dsDNA and Sm antibodies | |
| Immunofluorescence: granular deposition | ||
| Granulomatous infections | Mycobacterium | Proper stains and cultures |
| Fungi | ||
| Actinomycosis | ||
| Syphilis | ||
| Intranasal drug abuse | Cocaine | Antineutrophil elastase antibodies |
| Predominantly nasal septal pathology (CIMDL) | ||
| Pseudovasculitis syndromes | Atrial myxoma | Echocardiography |
| Subacute bacterial endocarditis | Blood cultures | |
| Cholesterol emboli syndrome | Echocardiography (transesophageal) | |
| Angiography | ||
| Skin biopsy | ||
| Neoplastic | Lethal midline granuloma | Nose/palate destruction |
| NK T cell lymphoma |
CIMDL, Cocaine-induced midline destructive lesion, which is usually associated with p-ANCA directed against human neutrophil elastase.
