Granulomatosis with polyangiitis - Oral Mucosal Lesion
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.
M31.30 – Wegener's granulomatosis without renal involvement
M31.31 – Wegener's granulomatosis with renal involvement
195353004 – Wegener's granulomatosis
Differential Diagnosis & Pitfalls
- Drug-induced gingival hyperplasia may have a micropapillary surface, but no petechiae are seen. The patient also has a history of taking phenytoin, cyclosporin, or one of the calcium channel-blocking agents.
- Gingival peripheral ossifying fibromas
- Leukemic oral infiltrate is not typically micropapular, and patients often will have systemic signs and symptoms (eg, fatigue, shortness of breath, pallor).
- Lymphoma is usually localized and would not typically have pulmonary or kidney involvement.
- The gumma of tertiary syphilis may appear similar to GPA.
- Deep fungal and mycobacterial infections may have pulmonary symptoms that mimic GPA. Biopsy should demonstrate the causative organisms.
- Cocaine mucosal ulcer (oral, nasal)
- Giant cell arteritis
- Gingival fibromatosis syndromes
- Inflammatory gingival hyperplasia usually has a smooth surface with no petechiae.
Drug Reaction Data