Abstract: PUB272
Recurrence of Scleroderma Renal Crisis After Kidney Transplantation
Session Information
Category: Trainee Case Report
- 1902 Transplantation: Clinical
Authors
- Portocarrero Caceres, Juan Pablo, Northwestern University Feinberg School of Medicine, Chicago, Illinois, United States
- Ghossein, Cybele, Northwestern University Feinberg School of Medicine, Chicago, Illinois, United States
- Kanwar, Yashpal S., Northwestern University Feinberg School of Medicine, Chicago, Illinois, United States
Introduction
Diffuse cutaneous Systemic Sclerosis (dcSSc) is a disease of generalized inflammation, vascular damage, and organ fibrosis. Scleroderma Renal Crisis (SRC), one of the most devastating complications of dcSSc occurs in 5-20% of patients and leads to end stage renal disease (ESRD) 20-50% of the time. SRC is characterized by malignant hypertension and acute kidney injury (AKI). Thrombotic microangiopathic anemia (TMA) and heart failure (HF) can also be seen. SRC patients with ESRD can undergo kidney transplantation (KT) with excellent graft survival. Recurrence of SRC in KT is very rare presumably because the renal vasculature is from a donor kidney without dSSc. We report a case of SRC in a patient post KT
Case Description
A 52-year-old male-to-female transgender patient with a history of ESRD due to SRC, who had a living unrelated KT 2 months prior to admission is admitted hypertension and pulmonary edema. She was found to have HF with reduced ejection fraction (37%) a new pericardial effusion and AKI. Kidney biopsy showed arterioles with thickened walls, bland arteriolitis with semi occlusive changes. She was diuresed and discharged home. She returned 2 weeks later with microangiopathic hemolytic anemia, thrombocytopenia, and worsening kidney function. Repeat kidney biopsy was consistent with TMA. Given her clinical presentation and her biopsy findings, a presumptive diagnosis of SRC was made. The patient was also found to have an antibody-mediated graft rejection. She received captopril, multiple sessions of PLEX, eculizumab, and belatacept, without response. She was initiated on dialysis where she remains today.
Discussion
SRC post KT is unusual but should be considered in the differential of AKI in the right clinical setting. Active dcSSc disease, use of steroids and calcineurin inhibitors may increase the risk of post transplant SRC