Refractory secondary thrombotic microangiopathy with kidney injury associated with systemic lupus erythematosus in a ped
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CASE REPORT
Refractory secondary thrombotic microangiopathy with kidney injury associated with systemic lupus erythematosus in a pediatric patient Tomoya Kaneda1 · Eriko Tanaka1,2 · Yuko Akutsu1 · Toru Kanamori1 · Mariko Mouri3 · Tomohiro Morio1 · Masaaki Mori3 Received: 5 January 2020 / Accepted: 2 April 2020 © Japanese Society of Nephrology 2020
Abstract Thrombotic microangiopathy (TMA) is generally diagnosed through clinical features characterized as microangiopathic hemolytic anemia, thrombocytopenia, and multiple organ injury, as well as by pathological findings such as vascular damage and endothelial cell injury. Rheumatic and autoimmune diseases could be accompanied by secondary TMA; in fact, systemic lupus erythematosus (SLE) is a common disease associated with secondary TMA, and SLE complicated with TMA has been reported to have a poor prognosis. Although TMA occurs rarely in pediatric SLE patients, it often leads to severe clinical conditions. Here, we report a rare case of severe juvenile-onset SLE complicated with TMA and kidney injury. The 5-yearold patient showed renal dysfunction, thrombocytopenia, hemolytic anemia, nephrotic syndrome, hypocomplementemia, and elevation of anti-dsDNA IgG levels. Kidney biopsy revealed mesangial proliferation and endocapillary proliferation, as well as plumped endothelial cells, with full-house pattern deposits in immunofluorescence study. Combination treatment of methylprednisolone pulse therapy followed by oral prednisolone, mycophenolate mofetil, and plasma exchange was effective, whereas eculizumab did not show therapeutic effects. The patient further showed recurrent deterioration, and we initiated intravenous cyclophosphamide in addition to combination treatment and eventually succeeded in controlling the disease. Genome analysis by whole-exome sequencing revealed no particular gene mutation related to either complement disorders or type-1 interferon. Further elucidations concerning the pathogenic mechanisms causing juvenile-onset SLE are needed to establish an efficient treatment strategy for TMA with SLE. Keywords Thrombotic microangiopathy · Systemic lupus erythematosus · Kidney injury · Pediatric rheumatology · Pediatric nephrology
Introduction Thrombotic microangiopathy (TMA) is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and multiple organ injury [1, 2]. In particular, primary TMA occurs in case of complement disorders—such as atypical hemolytic uremic syndrome, as well as in ADAMTS13 * Eriko Tanaka tanaka‑[email protected]‑u.ac.jp 1
Department of Pediatrics and Developmental Biology, Tokyo Medical and Dental University, Tokyo, Japan
2
Department of Pediatrics, Kyorin University School of Medicine, 6‑20‑2 Shinkawa, Mitaka, Tokyo 181‑8611, Japan
3
Department of Lifetime Clinical Immunology, Tokyo Medical and Dental University, Tokyo, Japan
deficiency (also known as thrombotic thrombocytopenic purpura)—whereas secondary TMA is triggered by various factors, such as drugs, bone marrow transplantations, and autoimmune disea
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