GRANULOMATOSIS WITH POLYANGIITIS

By Gary M. White, MD

Granulomatosis with polyangiitis aka Wegener's Granulomatosis


Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis is a small-vessel vasculitis of adults that is usually antineutrophil cytoplasmic antibody (ANCA)-positive.

Clinical

Cutaneous lesions include palpable purpura, hemorrhagic or necrotic papulonodules, pustules, erosions, bulla, and ulcers. The extremities are most commonly affected. A distinctive gingivitis (granular with petechia) occurs. The surface may resemble a strawberry and has been termed strawberry gingivitis. Oral ulcers are characteristic and are persistent in contrast to aphthous ulcers. Cutaneous ulcers sometimes resemble pyoderma gangrenosum and may exhibit pathergy. They usually lack the raised, tender, undermined border however. Subglottic stenosis and nasal deformity are much more common in childhood cases.

Diagnosis

The criteria for diagnosis includes nasal or oral inflammation (e.g., nasal discharge, oral ulcers), an abnormal CXR, an abnormal urine sediment, granulomatous inflammation on biopsy, and a positive c-ANCA.

C-ANCA

The antineutrophil cytoplasmic antibody is characteristic and is positive in approximately 85-100% of patients with active disease. Positivity rates may decrease to only 50% after successful treatment.

Treatment

Referral to a rheumatologist is in order. Rituxan is FDA-approved for this condition.

In the past, the following have been used: Prednisone and cyclophosphamide have been usually used in combination. NSAIDs may also be helpful. Trimethoprim/sulfamethoxazole is also helpful to maintain remission [NEJM 1996;335;16]. Weekly, low-dose methotrexate (0.3-25 mg/wk) combined with prednisone (1 mg/kg/day) induced remission in about 70% of patients with non-life-threatening WG.

Additional Pictures

Granulomatosis with polyangiitis aka Wegener's Granulomatosis

References

Strawberry gingivitis. Rheumatology Image Databank

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