Relapsing subarachnoid hemorrhage as a clinical manifestation in microscopic polyangiitis: a case report and literature review

Abstract

Microscopic polyangiitis (MPA) is a systemic small-vessel vasculitis associated with anti-neutrophil cytoplasmic antibody (ANCA) and predominantly causes kidney and pulmonary injuries. Subarachnoid hemorrhage, a life-threatening manifestation of the central nervous system (CNS), rarely occurs in patients with ANCA-associated vasculitis (AAV). We report the case of a young man with spontaneous SAH recurrence and active nephritis. The patient was treated with a glucocorticoid pulse and intravenous cyclophosphamide (CTX) in combination with decreasing cerebral perfusion pressure and analgesic therapy. All the patients’ symptoms except the proteinuria resolved. We reviewed the clinical characteristics of 34 previously reported cases of SAH with AAV, comprising six cases of MPA, eight cases of granulomatosis with polyangiitis (GPA), and 19 cases of eosinophilic granulomatosis with polyangiitis (EGPA), and one case of unclassified AAV. All the cases showed features of active vasculitis. Concomitant nephritis and peripheral neuropathy were found in the MPA and EGPA cases with SAH, respectively. Renal and pulmonary manifestations were predominant in the patients with GPA and SAH. Ten patients had aneurysmal abnormalities, and six patients had cardiac abnormalities. Thirty-one patients were treated with glucocorticoids, and 18 patients received concurrent immunosuppressants. Patients with SAH had a mortality rate of 38.2%. The presence of cerebrovascular events or cardiac involvement in patients with AAV and SAH is associated with increased mortality of 64.3%. Our study indicates that SAH should be cautioned as a disease occurring in patients with AAV. Early diagnosis with aggressive immunosuppressive therapy can help improve the prognosis of patients with SAH.

Subarachnoid Hemorrhaging with Multiple Cerebral Artery Stenoses after Initiating Remission Induction Therapy for Eosinophilic Granulomatosis with Polyangiitis

“…Cerebral infarction is more frequent in patients with CNS involvement than in those with hemorrhagic vascular involvement, such as cerebral hemorrhaging or subarachnoid hemorrhaging (SAH) (2,3). EGPA with SAH has been reported to have a high mortality rate (4).…”

Unraveling the Puzzle: A Case Report Questioning the Causal Relationship Between Subarachnoid Hemorrhage and Microscopic Polyangiitis

“…Mononeuritis multiplex is the most common neurological manifestation of AAV. Stroke, hypertrophic pachymeningitis, massive intracerebral hemorrhage, subarachnoid hemorrhage (SAH), and spinal SAH associated with MPA have been described in case reports [ 1 ]. However, per the literature review, it is unclear if MPA has a causal relationship with SAH.…”