Response to ‘Orbital Wegener's granulomatosis as the initial presentation in a 9-year-old child’

We were interested to read the article by Dey et al. describing the ocular presentation of Wegener's granulomatosis in a child. ¹ The authors considered corticosteroids to be the first line of treatment in this condition. This varies from the usual induction regimen, which is a combination of cyclophosphamide and corticosteroid.

Wegener's granulomatosis, often referred to as granulomatosis with polyangiitis, is a fatal disease, with mortality if untreated of 90% at two years. In 1973, Fauci and Wolff showed that a regimen that combined cyclophosphamide and prednisolone induced disease remission. ² This combination was superior to corticosteroid treatment alone. Subsequent long-term experience has demonstrated that the cyclophosphamide and prednisolone regimen induces remission in 85–90% of patients within two to six months. ³

More recently the introduction of the anti B-cell monoclonal antibody, rituximab, has provided an alternative induction therapy to cyclophosphamide or for use in cyclophosphamide-resistant disease. ⁴ It is currently recommended as an initial induction regimen only in those patients who have a contraindication to cyclophosphamide.

While single therapy with either corticosteroids or co-trimoxazole have had their advocates, current advice is that in treatment of limited disease combination therapy with corticosteroid and either methotrexate or cyclophosphamide should be used. Co-trimoxazole can be used as part of maintenance therapy to prevent Pneumocystis infection. ⁵

Once remission is achieved patients are then switched to maintenance immunosuppression such as methotrexate or azathioprine. Thus corticosteroids alone appear inferior for both induction and maintenance therapy.