Granulomatosis with polyangiitis
The disease typically presents with upper airway disease and nonspecific complaints such as malaise, arthralgias, fever, and weight loss. Respiratory tract symptoms include sinusitis, otitis media, rhinorrhea, cough, sputum production, chest pain, dyspnea, and hemoptysis.
The disease presents variably on a continuum from limited involvement (which may remain limited for variable periods of time) to a more generalized form with involvement of the upper and lower respiratory tract, kidney, skin, and other organs. Untreated, the generalized form is fatal in less than 2 years; however, long-term remissions are now possible in more than 90% of patients with the advent of cytotoxic therapy.
Complications of GPA include pericarditis, coronary arteritis, myocardial infarction, pancarditis, granulomatous valvulitis, mononeuritis multiplex, and polyneuritis. Laryngotracheal stenosis with stridor can be a complication of GPA and initially may be misdiagnosed as asthma.
M31.30 – Wegener's granulomatosis without renal involvement
M31.31 – Wegener's granulomatosis with renal involvement
195353004 – Wegener's granulomatosis
- Cutaneous small vessel vasculitis
- Systemic lupus erythematosus
- Polyarteritis nodosa
- Cocaine levamisole toxicity can often present with positive antineutrophil cytoplasmic autoantibodies (ANCA) studies and may mimic vasculitides.
- Deep fungal and mycobacterial infections (eg, mucormycosis, tuberculosis) – May have pulmonary symptoms that mimic GPA. Biopsy should demonstrate the causative organisms.
- Pyoderma gangrenosum
Last Updated: 06/18/2019