Wegener Granulomatosis With Massive Intracerebral Hemorrhage

Abstract

Wegener granulomatosis (WG) is a multisystem necrotizing vasculitis that primarily involves the upper and lower respiratory tract and kidneys but can affect almost any organ, including the central nervous system (CNS). We present a patient with WG whose disease was complicated by a massive intracerebral hemorrhage (ICH) despite standard treatment with prednisone and cyclophosphomide. Although CNS involvement is not uncommon in WG, ICH is a rare complication of WG, and although the majority of patients with WG complicated by a cerebrovascular accident have a fatal outcome, our patient survived this event. The disease subsequently progressed with recurrent pulmonary involvement and renal failure. Our patient either did not tolerate or failed to respond to several immunosuppressive agents, including cyclophosphamide, methotrexate, and mycophenolate mofetil, but achieved remission after treatment with rituximab.

Massive intracerebral hemorrhage associated with Wegener granulomatosis

“…Mucosal hemorrhage in the lungs and gastrointestinal tract is frequent complication of small vessel vasculitides including WG, but much less common are intracerebral and intraventricular hemorrhage. ICH may occur as an initial manifestation or latter in the disease course despite being on standard immunosuppressive therapy as our patient [2,6]. This case also demonstrates that the development of CNS vasculitis may correlate with disease activity in other organs such as diffuse pulmonary hemorrhage.…”

“…Neurologic involvement occurs in 22-54% of patients with WG. It usually manifests as mononeuritis multiplex and polyneuritis; however, CNS involvement (7-11%) is uncommon in WG and ICH as a complication has been rarely reported [1][2][3][4][5].…”

Intracerebral haemorrhage, a possible presentation in Churg-Strauss syndrome: Case report and review of the literature

“…Cerebral haemorrhage, in particular, is anecdotally reported [18,19], whereas ischemic cerebrovascular events secondary to vasculitis, seizures, diffuse encephalopathy, pituitary gland involvement, chronic hypertrophic pachymeningitis and cranial neuropathies are the most common manifestations [20,21].…”

Immunotherapies for Neurological Manifestations in the Context of Systemic Autoimmunity

“…Noninferiority of rituximab over cyclophosphamide was proven in the RAVE trial, a multicenter, randomized, double-blind, noninferiority study (grade 1A) [134], and it has also shown efficacy in a few patients with neurological involvement [135,136].…”