Abstract:
Background: The 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 presentation: An 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.
Conclusion: This 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.
