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Scleroderma Overlapping With Sjögren's Syndrome: A Case Report of a Renal Emergency
Suela Mumajesi1, Saimir Seferi1, Nereida Spahia1
1Nephrology, University Hospital Center "Mother Tereza", Tirana, ALB.
None:
Scleroderma renal crisis (SRC) is the most common hallmark of renal involvement in systemic sclerosis (SSc), characterized by acute kidney injury and malignant hypertension. The pathophysiology of SRC involves endothelial dysfunction, activation of the renin-angiotensin-aldosterone system (RAAS), and thrombotic microangiopathy (TMA), leading to renal ischemia and hypertension. When accompanied by overlapping Sjögren's syndrome (SS), the clinical presentation and therapy become considerably more complex. We discuss the diagnosis, management, and outcomes of a patient presenting with SRC and overlapping SS. It highlights the importance of early detection and rapid treatment of SRC, particularly in the presence of overlap syndromes. Early intervention with angiotensin-converting enzyme (ACE) inhibitors and careful monitoring of renal function are crucial for optimal outcomes. The occurrence of overlapping SS complicates the clinical presentation and therapy, emphasizing the importance of an individualized approach to treatment. Healthcare personnel must be especially mindful of this since early detection of renal crisis has a major impact on survival rates and long-term prognosis.
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