Wegener's granulomatosis and acute upper limb digital ischemia

Abstract

Wegener's granulomatosis is a rare disease which occasionally causes digital gangrene. We describe a patient who developed gangrene in an upper limb despite being on adequate treatment with intravenous cyclophosphamide and corticosteroids. Further management included administration of prostacyclin analogue (Iloprost) resulting in stabilization of disease.

Utilisation of plasma exchange in the treatment of digital infarcts in Wegener’s granulomatosis

“…Review of other case reports revealed that some digital infarctions responded to cyclophosphamide and steroid therapy 1,4–9 . A report by Avcin et al.…”

“…There have only been under a dozen case reports of digital ischemia in WG 1,4–11 . Review of these case reports suggests poor outcome with digital infarcts, often resulting in amputation of the affected area 4 .…”

“…Onset of digital ischemia appears to be variable, ranging from the acute presentation to months after initial symptoms. This manifestation can occur despite immunosuppressive therapy 1 …”

“…In another report by Kejriwal et al. 1 a Maori woman with WG was treated with i.v. cyclophosphamide and prednisolone but later developed digital infarcts and gangrenous fingers, and was then treated with iloprost (prostaglandin analogue) infusion and further i.v.…”

“…Wegener’s granulomatosis (WG) affects 3 per 100 000 people with an average age of 55 years at presentation 1 . WG is classified into limited and classic, with limited WG predominantly involving the upper respiratory tract and has a better prognosis.…”