| 제목 |
Microscopic Polyangiitis with unusual clinical manifestations: A Case Report |
| 소속 |
Seoul National University College of Medicine, Seoul National University Hospital, Seoul, Republic of Korea, Department of Rehabilitation Medicine1, Seoul National University College of Medicine, SMG-SNU Boramae Medical Center, Seoul, Republic of Korea, Department of Rehabilitation Medicine2 |
| 저자 |
Yae Lim Lee1*, Jung Woo Kim1, Sang Yoon Lee2† |
Introduction
Microscopic polyangiitis (MPA) is an idiopathic autoimmune disease characterized by systemic small-vessel vasculitis. This case report describes a patient with MPA presented with mononeuritis multiplex and rectal perforation, and rarely accompanied antiphospholipid syndrome (APS) presented with pulmonary embolism.
Case
A 61-year-old woman presented with general myalgia and high fever. She was treated with antibiotics under the diagnosis of proctitis, but her symptoms did not improve. After one month, she reported right foot drop with hypesthesia, left 2nd and 3rd finger tingling sensation, and urinary and fecal incontinence of an acute onset. Laboratory study results showed mild leukocytosis and elevated CRP, but there was neither eosinophilia nor any abnormalities in the level of creatine kinase (CK) or antibodies. There was no definite spinal cord signal abnormality.
Nerve conduction study revealed sensorimotor polyneuropathy in the left arm and bilateral legs (Table 1 and 2). Mononeuritis multiplex of inflammatory cause, in particular vasculitic neuropathy, was suspected, and sural nerve biopsy confirmed the diagnosis (Figure 1). She was treated with methylprednisolone pulse therapy and subsequent cyclophosphamide pulse therapies. After first cyclophosphamide pulse therapy, rectal perforation occurred, and emergency Hartmann operation was performed. Four months later, follow-up electrodiagnostic study was performed (Table 1 and 2).
Meanwhile, acute pulmonary thromboembolism was simultaneously found. Since the possibility of massive venous embolism is low in the case of MPA, additional blood test was performed to rule out accompanied APS. Anti-cardiolipin antibody was positive, and lupus anticoagulant was weak positive. Re-examination is required after 12 weeks to confirm the diagnosis, but it has not been performed yet.
Conclusion
This is a rare case of accompanied MPA and APS presented with mononeuritis multiplex, proctitis, rectal perforation, and pulmonary embolism. Since MPA can present with a variety of symptoms, clinicians should always consider the possibility of diagnosis. |
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Table1. Results of sensory and motor nerve conduction study
Table 2. Results of needle electromyography, H reflex, and F wave
Figure 1. Sural nerve biopsy
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