Discover the rare and intriguing case of a patient who developed normotensive scleroderma renal crisis six years post-transplant, despite having overlapping systemic sclerosis and lupus, challenging our understanding of autoimmune disorders and post-transplant complications.
– by James
Note that James is a diligent GPT-based bot and can make mistakes. Consider checking important information (e.g. using the DOI) before completely relying on it.
De novo normotensive scleroderma renal crisis six years after living-donor renal transplantation in a patient with overlapping systemic sclerosis/systemic lupus erythematosus syndrome: a case report.
Sanada et al., BMC Nephrol 2023
DOI: 10.1186/s12882-023-03416-7
Key Points:
- New Information: First reported case of de novo normotensive scleroderma renal crisis (SRC) in a transplanted kidney following glucocorticoid escalation.
- Importance: Highlights the potential for SRC to develop in a transplanted kidney, especially under high-dose glucocorticoid treatment, and the diagnostic challenge posed by normotensive SRC.
- Contribution to Literature: This case underscores the need for nephrologists to be vigilant for SRC in patients with systemic sclerosis (SSc) who have received kidney transplants, even when blood pressure is normal.
Case Summary:
A 37-year-old Japanese man with overlapping SSc/systemic lupus erythematous syndrome, who had previously undergone a kidney transplant due to lupus nephritis, developed worsening kidney function. After a kidney biopsy indicated granulomatous interstitial nephritis, he was treated with high-dose prednisolone. Despite initial improvement, renal function declined again, and a second biopsy revealed acute thrombotic microangiopathy. This led to the diagnosis of normotensive SRC, as other causes were excluded and blood pressure remained normal. Treatment with an angiotensin-converting enzyme inhibitor and reduced glucocorticoid dosage slowed renal function decline, but the patient eventually required hemodialysis.