Systemic lupus erythematosus and antineutrophil cytoplasmic antibodies-associated vasculitis overlap in an elderly woman: A case-based literature review

Abstract

BackgroundThe overlap of systemic lupus erythematosus (SLE) and anti-neutrophil cytoplasmic antibody (ANCA) associated-vasculitis (AAV) is a rare entity. Aim of the work. To report a rare case of overlap SLE and AAV complicated by small bowel perforations and nephritis. Case presentationAn 81-years-old Chinese woman presented with a two-weeks history of progressive bilateral lower limb weakness and dysuria. An incidental uterine mass was found, and a total hysterectomy was performed with extensive small bowel adhesion and multiple enteric perforations discovered intra-operatively. SLE was diagnosed based on the presence of cutaneous vasculitis, positive antinuclear antibody, anti-double stranded deoxyribonucleic acid, consumed complements, thrombocytopenia, nephritis, and pleural effusion. Positive perinuclear-ANCA and histological findings of the resected small bowel led to evidence of co-existing AAV. Hence, these findings have led to a diagnosis of overlap SLE and microscopic polyangiitis (MPA). The patient received daily hydroxychloroquine (200 mg), azathioprine (50 mg) followed by intravenous (IV) hydrocortisone (200 mg/8 h) and cyclophosphamide (750 mg/m2). The patient’s condition deteriorated with respiratory failure and hypotension and was eventually intubated and ventilated. IV immunoglobulin (4 mg/kg/day) was given for 3 days with resolution of the vasculitic lesions. The renal function rapidly declined with hemodynamic and clinical deterioration and the patient died. ConclusionThis case demonstrates the diagnostic conundrum and complexity in the management of a late presentation of an overlap syndrome with rare life-threatening complications. To our knowledge, this is the first case diagnosed and managed in Malaysia and the oldest patient diagnosed with overlap SLE/AAV in the literature.