Granulomatosis with polyangiitis
See also in: External and Internal Eye,Oral Mucosal LesionAlerts and Notices
Synopsis
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. Ocular involvement occurs in 30%-50% of patients with GPA and can occur without systemic manifestations. Oral lesions can be seen with any of the clinical presentations of GPA and consist of ulcerations and/or "strawberry" gingivitis.
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, with the advent of cytotoxic therapy, long-term remissions are possible in more than 90% of patients.
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.
Codes
ICD10CM:M31.30 – Wegener's granulomatosis without renal involvement
M31.31 – Wegener's granulomatosis with renal involvement
SNOMEDCT:
195353004 – Wegener's granulomatosis
Look For
Subscription Required
Diagnostic Pearls
Subscription Required
Differential Diagnosis & Pitfalls
- Microscopic polyangiitis – Absence of granulomatous inflammation and sparing of upper respiratory tract. Overlap of clinical phenotypes is common.
- Eosinophilic granulomatosis with polyangiitis – Associated with peripheral eosinophilia and a history of asthma.
- Cutaneous small vessel vasculitis
- Sarcoidosis
- Cryoglobulinemia
- Cryofibrinogenemia
- Systemic lupus erythematosus (SLE)
- Polyarteritis nodosa
- Coccidioidomycosis
- Cocaine levamisole toxicity can often present with positive antineutrophil cytoplasmic autoantibodies (ANCA) studies and may mimic vasculitides.
- Drug-induced vasculitis with positive ANCA studies (propylthiouracil, hydralazine, anti-tumor necrosis factor alpha, sulfasalazine, D-penicillamine, minocycline)
- Deep fungal and mycobacterial infections (eg, mucormycosis, tuberculosis) – May have pulmonary symptoms that mimic GPA. Biopsy should demonstrate the causative organisms.
- Pyoderma gangrenosum
- Calciphylaxis
- Oxalosis
- Ecthyma gangrenosum
- Meningococcemia
- Strongyloidiasis
- Erythema nodosum leprosum
- Immunoglobulin A vasculitis
- Leukemic vasculitis
- Embolism (septic, fat, air, cholesterol, marantic)
- Hypercoagulable state
- Disseminated intravascular coagulation
- Purpura fulminans
- Warfarin necrosis
- Deficiency of adenosine deaminase type 2
- Other collagen vascular diseases or vasculitis (eg, rheumatoid arthritis, dermatomyositis)
- Giant cell arteritis
Best Tests
Subscription Required
Management Pearls
Subscription Required
Therapy
Subscription Required
Drug Reaction Data
Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.Subscription Required
References
Subscription Required
Last Reviewed:05/17/2020
Last Updated:09/27/2022
Last Updated:09/27/2022
Granulomatosis with polyangiitis
See also in: External and Internal Eye,Oral Mucosal Lesion