title: Microscopic Polyangiitis with Spinal Cord Involvement: A Case Report and Review of the Literature.
abstract: BACKGROUND
  Microscopic polyangiitis (MPA) is an ANCA-associated vasculitis (AAV; ANCA denotes antineutrophil cytoplasmic antibody) that causes necrotizing inflammation of small blood vessels. Renal and pulmonary manifestations are common whereas central nervous system (CNS) involvement, and in particular spinal disease, is rare.
  
  
  METHODS
  We reviewed a case of MPA presenting with spinal intradural hemorrhage and intracerebral hemorrhage. We also summarized all reported cases of AAV with spinal cord involvement in the literature (database search included MEDLINE, Embase, Scopus, and Proquest with no date or language restriction).
  
  
  RESULTS
  We reviewed 20 cases of AAV with spinal cord involvement (12 granulomatosis with polyangiitis [GPA], 4 eosinophilic granulomatosis with polyangiitis, 2 MPA, and 2 cases diagnosed as AAV only) and reported demographic information, clinical features, methods of diagnosis, treatment, and patient outcome. Although CNS involvement has been associated with a poor prognosis, 14 of 18 cases that reported outcome data achieved remission during follow-up. Death occurred in 3 patients diagnosed with GPA and in 1 patient with MPA. Our patient with MPA deteriorated rapidly despite use of prednisone and died.
  
  
  CONCLUSIONS
  AAV can present with brain and spinal cord involvement, even in the absence of systemic disease. CNS disease may be responsive to immunosuppressive therapy (e.g., steroids and cyclophosphamide) in several of the cases reviewed.
authors.affiliation: Division of Rheumatology, University of Alberta, Edmonton, Alberta, Canada.
authors.forename: Martha L
authors.initials: ML
authors.lastname: Decker
authors.affiliation: Division of Rheumatology, University of Alberta, Edmonton, Alberta, Canada.
authors.forename: Derek J
authors.initials: DJ
authors.lastname: Emery
authors.affiliation: Division of Rheumatology, University of Alberta, Edmonton, Alberta, Canada.
authors.forename: Penelope S
authors.initials: PS
authors.lastname: Smyth
authors.affiliation: Division of Rheumatology, University of Alberta, Edmonton, Alberta, Canada.
authors.forename: Jian-Qiang
authors.initials: JQ
authors.lastname: Lu
authors.affiliation: Division of Rheumatology, University of Alberta, Edmonton, Alberta, Canada.
authors.forename: Atilano
authors.initials: A
authors.lastname: Lacson
authors.affiliation: Division of Rheumatology, University of Alberta, Edmonton, Alberta, Canada. Electronic address: eyacyshyn@ualberta.ca.
authors.forename: Elaine
authors.initials: E
authors.lastname: Yacyshyn
mesh_terms: D000328:Adult; D000368:Aged; D001706:Biopsy; D002543:Cerebral Hemorrhage / Q000209:etiology; D018450:Disease Progression; D017809:Fatal Outcome; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007796:Laminectomy; D008279:Magnetic Resonance Imaging; D008297:Male; D055953:Microscopic Polyangiitis / Q000150:complications / Q000000981:diagnostic imaging / Q000628:therapy; D008875:Middle Aged; D011241:Prednisone / Q000627:therapeutic use; D012307:Risk Factors; D013118:Spinal Cord Diseases / Q000150:complications / Q000000981:diagnostic imaging / Q000628:therapy; D013345:Subarachnoid Hemorrhage / Q000209:etiology; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D011241:Prednisone
keywords: Microscopic polyangiitis; antineutrophil cytoplasmic antibody-associated vasculitis; central nervous system; myelopathy; spinal cord