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Abstract: SA-PO858

Normotensive Scleroderma Renal Crisis 8 Years After Living Donor Renal Transplantation: A Case Report

Session Information

Category: Transplantation

  • 2002 Transplantation: Clinical

Authors

  • Sanada, Hajime, Kanazawa University Hospital Department of Rheumatology, Kanazawa, Ishikawa, Japan
  • Hara, Satoshi, Kanazawa University Hospital Department of Rheumatology, Kanazawa, Ishikawa, Japan
  • Tsuge, Shunsuke, Kanazawa University Hospital Department of Rheumatology, Kanazawa, Ishikawa, Japan
  • Nishioka, Ryo, Kanazawa University Hospital Department of Rheumatology, Kanazawa, Ishikawa, Japan
  • Ito, Kiyoaki, Kanazawa University Hospital Department of Rheumatology, Kanazawa, Ishikawa, Japan
  • Mizushima, Ichiro, Kanazawa University Hospital Department of Rheumatology, Kanazawa, Ishikawa, Japan
  • Kawano, Mitsuhiro, Kanazawa University Hospital Department of Rheumatology, Kanazawa, Ishikawa, Japan
Introduction

Scleroderma renal crisis rarely occurs in transplanted kidneys, and it remains unknown whether prednisolone for transplantation could be the risk factor.

Case Description

A 36-year-old man was diagnosed with systemic sclerosis 19 years ago based on skin involvement and positive anti-topoisomerase I antibody. He often had to be hospitalized due to intractable digital ulcers and interstitial lung disease. He developed class 5 lupus nephritis and systemic lupus erythematosus 11 years ago. It was refractory and hemodialysis was started 9 years ago, and he underwent ABO-compatible living donor renal transplantation with his father as the donor 8 years ago. His renal function remained at serum creatinine (sCr) 1.0 mg/dL, but he had suffered from cytomegalovirus (CMV) infections such as esophagitis and enteritis after transplantation. Thus, we switched mycophenolate mofetil to everolimus 2 months ago. One month before, renal function had gradually declined to sCr 1.65 mg/dL, and a renal biopsy was performed. Pathological findings indicated granulomatous interstitial nephritis (GIN), which was diagnosed as idiopathic without any obvious secondary cause. The dose of prednisolone (PSL) was increased from 5 to 30 mg/day after steroid pulse therapy, but his renal function continued to deteriorate to sCr 2.9 mg/dL, and urine protein increased to 4.5 g/day. The second kidney biopsy next month showed the GIN improvement, and microarterial thrombus and glomerular endothelial cell damage were observed, leading to a diagnosis of acute thrombotic microangiopathy (TMA). The patient was considered to have normotensive scleroderma renal crisis (SRC), and an angiotensin-converting enzyme inhibitor was initiated. His renal function stopped falling at the sCr 3 mg/dL

Discussion

SRC rarely develops in transplanted kidneys, and it has been suggested that PSL administration for transplantation may not be a risk factor for the development of SRC. However, we could not identify other causes of TMA except for PSL dose increase which may cause SRC in our case. In conclusion, SRC could emerge in the transplanted kidney and might be induced by PSL dose increase. PSL dose for patients with scleroderma should be taken carefully even after kidney transplantation.