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.
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. Oral lesions can be seen with any of the clinical presentations of GPA, and consist of ulcerations and/or "strawberry" gingivitis.
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.
This summary will focus on oral manifestations of the disease.
M31.30 – Wegener's granulomatosis without renal involvement
M31.31 – Wegener's granulomatosis with renal involvement
195353004 – Wegener's granulomatosis
- Drug-induced gingival hyperplasia may have a micropapillary surface, but no petechiae are seen. 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 and requires FTA-ABS to rule out this infection.
- Deep fungal and mycobacterial infections may have pulmonary symptoms that mimic GPA. Biopsy should demonstrate the causative organisms.
- Cocaine mucosal ulcer (oral, nasal)
- Gingival fibromatosis syndromes
- Inflammatory gingival hyperplasia usually has a smooth surface with no petechiae.
Last Updated: 07/26/2017