Cureus
The Battle Against Systemic Sclerosis: A Case Report of a Patient With Scleroderma Renal Crisis
By Noor Elkurwi, Zeineb Garmeh, Qabas Elkurwi and 2 others.
Abstract
Systemic scleroderma (SSc) is an uncommon autoimmune disorder that causes excessive fibrosis of the skin and internal organs, especially the lungs, kidneys, and heart.Scleroderma renal crisis (SRC) is a rare and potentially fatal complication of SSc.In recent years, there has been an increasing interest in SSc treatment, but so far, the targeted management approach has been lacking.We report a 44-year-old male patient with overlap syndrome (systemic scleroderma, Sjogren's syndrome, and myositis), who underwent treatment with therapeutic plasma exchange (TPE) and received hemodialysis, angiotensin-converting enzyme inhibitors, glucocorticosteroids, and cyclophosphamide.The patient presented prominent cutaneous scleroderma with sclerodactyly and Raynaud's phenomenon.Despite the initial stabilization of the patient's condition on the multimodal pharmacotherapy combined with TPE, he later died from multi-organ failure.Although TPE performed during the early course of the disease has been reported as a potential adjunctive therapy, its efficacy and long-term benefits remain uncertain.Given the complexity of our patient's presentation, including scleroderma overlap syndrome and advanced, irreversible organ damage, it is difficult to determine whether TPE provided a meaningful clinical benefit.




