Ocular manifestations of Wegener's granulomatosis. Analysis of nine cases.

PURPOSE: To report a series of nine patients of Wegener's granulomatosis (WG) with diverse ocular and systemic manifestations.

METHODS

Retrospective analysis of nine consecutive patients seen between 1987 and 2002.

RESULTS

The mean age at the time of diagnosis was 43.89 years (range: 33-56 years). Redness, pain and photophobia (8 patients) were the common presenting complaints. Sinusitis (6 patients) and arthralgia (6 patients) were the commonly associated systemic complaints. Necrotising scleritis with peripheral keratopathy (6 patients) was the most common ocular sign. Serum antibodies against the cytoplasmic component of neutrophils and monocytes (cANCA) were positive in 7 of 8 patients. Biopsy diagnosis was done in one patient for whom cANCA was not done. Cyclophosphamide and corticosteroids alleviated the symptoms in 6 patients. Ocular and systemic condition remained stable in 7 patients. One patient expired due to the severity of the disease and another patient was lost to follow-up.

CONCLUSIONS

Scleritis with peripheral corneal involvement was the most commonly observed ocular manifestation of WG in our series. cANCA was a useful adjunct in the diagnosis of WG. When clinical and serologic findings were inconclusive, biopsy remained indispensable. A combination of cyclophosphamide and corticosteroids is essential and critical not only for the ocular condition but also for the survival of the patient.