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

Abstract

Digital ischemia is a rare manifestation of Wegener's granulomatosis (WG) with previous management approaches based on the hypotheses of thromboembolic phenomenon and vasospasm. WG is an autoimmune, antineutrophil cytoplasmic antibody-predominant small-to-medium-vessel necrotising vasculitis which mainly affects the pulmonary-renal system. We report on a patient with WG presenting with digital infarction. We successfully treated this patient who had digital infarcts refractory to cyclophosphamide, steroids and vasodilator agents, with plasma exchange.

A Rare Case of Digital Ischemia and Gangrene in ANCA-Associated Vasculitis with Review of the Literature

“…Only two cases (12%), including ours, have described simultaneous involvement of both the upper and lower extremities [20]. The pathophysiology of the digital ischemia and gangrene is thought to be from destruction of medium sized vessels from active vasculitis, which has been demonstrated in case reports where histological examination of affected tissue showed arteritis [9, 15]. However, there is also evidence that in situ thrombosis, as a result of active vasculitis, can lead to ischemia and gangrene, as seen in angiographic examination of affected patients, as well as on histological examination of affected tissue showing arterial thrombi [12, 13].…”

“…Only 3 patients demonstrated granulomas on biopsy. Confounding risk factors were inconsistently reported but include a past medical history of HTN in three cases [9, 16, 19], DM in three cases [13, 15, 16], tobacco use in two cases [13, 18], and dyslipidemia in one case [13]. The clinical phenotype usually involves multiple disparate digits.…”

“…The diagnosis is usually made based on clinical grounds, but angiographic examination can support vasculitic or thrombotic lesions [7–20]. Pathology can demonstrate either vascular arteritis or thrombi formation [9, 12, 13, 15]. There is no consensus on treatment regarding GPA with digital vasculitis with ischemia and gangrene.…”

Sensory-Motor Polyneuropathy and Digital Ischemia: A Rare Presentation of Granulomatosis with Polyangiitis

“…The most commonly observed histopathological pattern of cutaneous GPA was leukocytoclastic vasculitis [ 16 ]. The immediate cause of digital ischemia and gangrene is most probably the destruction of medium-sized vessels due to active vasculitis as suggested by histopathological examination of affected tissues [ 17 , 18 ]. However, it can be due to in situ thrombosis as a result of active vasculitis as seen in angiographic examination of affected patients as well as on histological examination [ 19 , 20 ].…”