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.

Competing interests

None declared