Microscopic Polyangiitis with Spinal Cord Involvement: A Case Report and Review of the Literature
Martha L Decker1, Derek J Emery1, Penelope S Smyth1
1Division of Rheumatology, University of Alberta, Edmonton, Alberta, Canada.
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.
Insights
Microscopic polyangiitis (MPA) and other ANCA-associated vasculitis (AAV) can rarely affect the central nervous system, including the spine. While serious, some cases respond to immunosuppressive therapy, though outcomes vary.
Area of Science:
- Neurology
- Rheumatology
- Immunology
Background:
- Microscopic polyangiitis (MPA) is a rare ANCA-associated vasculitis (AAV) causing small blood vessel inflammation.
- While renal and pulmonary involvement is common, central nervous system (CNS) and spinal cord disease are infrequent complications.
Observation:
- This study reviewed a case of MPA with spinal intradural and intracerebral hemorrhage.
- A literature search identified 20 cases of AAV with spinal cord involvement, including Granulomatosis with Polyangiitis (GPA), Eosinophilic Granulomatosis with Polyangiitis, and MPA.
Findings:
- CNS involvement in AAV, though associated with poor prognosis, showed remission in 14 of 18 reported cases.
- Outcomes varied, with some patients achieving remission and others succumbing to the disease, as seen in the MPA case presented.
Implications:
- ANCA-associated vasculitis can manifest with CNS and spinal cord disease, even without systemic symptoms.
- Immunosuppressive treatments, including steroids and cyclophosphamide, show potential efficacy in managing CNS AAV, highlighting the need for early diagnosis and intervention.
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