Failure Is a Learning Experience: A Case of Lupus Vasculopathy without Clinical Response to Rituximab

Introduction: Lupus vasculopathy (LV) is a rare entity and the pathogenesis is poorly understood. Renal involvement in systemic lupus erythematosus (SLE) is typically an immune complex glomerulonephritis. LV however is a non-inflammatory form of vascular injury. The prognosis is poor and currently there is no standard recommended treatment available. We hereby describe a case of LV that progressed to ESKD despite treatment with high dose corticosteroids, plasmapheresis and rituximab. Case Description: A 29 year old female with a history of SLE, hypertension and multiple ischemic strokes presented with a 3-week history of malaise and weight loss. The onset of chest pain and shortness of breath prompted medical evaluation with concern for lupus myopericarditis. On presentation serum creatinine was 1.2 mg/dL (baseline 0.7) and UPCR was 4.8 (previously 0.1-0.15). These findings together with hypocomplementemia and elevated dsDNA raised suspicion for lupus nephritis. A kidney biopsy performed revealed findings of lupus nephritis class II. Notably, half of the glomeruli sampled had ischemic changes, likely a result of prominent luminal narrowing of small arteries and arterioles due to immune complexes admixed with hyalin and fibrin. The diagnosis was consistent with LV. The non-ischemic glomeruli on this biopsy were preserved without features of glomerulonephritis. Serological testing for scleroderma and antiphospholipid syndrome were negative. 5 sessions of plasmapheresis were performed followed by rituximab infusions in conjunction with high dose steroids. Despite this, renal function progressively worsened over two months requiring initiation of dialysis without recovery. Discussion: There is a paucity of data on LV and the existing literature is limited to isolated case reports. Most of these cases have an associated severe lupus nephritis, and use of cyclophosphamide was often reported. Immunosuppressive medications however are “glomerulocentric” and are stratified based largely on the ISN/RPS lupus nephritis classification. There is no standardized treatment of LV alone, and future studies are needed to determine optimal treatment for LV specifically. This report uncovers another rare case of lupus vasculopathy, and highlights the need for a clinically effective treatment.