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Case Reports
. 2025 Jun 12;17(6):e85861.
doi: 10.7759/cureus.85861. eCollection 2025 Jun.

Scleroderma Overlapping With Sjögren's Syndrome: A Case Report of a Renal Emergency

Suela Mumajesi  1 , Saimir Seferi  1 , Nereida Spahia  1 , Elena Gjecka  1 , Floreta Kurti  2
Affiliations
Case Reports

Scleroderma Overlapping With Sjögren's Syndrome: A Case Report of a Renal Emergency

Suela Mumajesi et al. Cureus. .

Abstract

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.

Keywords: chronic kidney disease; elevated bun. hemodialysis; localized scleroderma; scleroderma renal crises; severe hypertension.

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Conflict of interest statement

Human subjects: Consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work.

Figures

Figure 1
Figure 1. “Salt-and-pepper” skin discoloration
Figure 2
Figure 2. Chest CT scan showing pleural and pericardial effusion
CT: computed tomography
Figure 3
Figure 3. Spirometry flow-volume showing mixed ventilatory dysfunction
Figure 4
Figure 4. Cardiac ultrasound findings
a: Normal ejection fraction. b: Left ventricular hypertrophy. c: Mitral valve regurgitation. d. Continuous wave Doppler assessment of tricuspid regurgitation
Figure 5
Figure 5. Renal ultrasound findings
Left kidney (a) and right kidney (b) showing normal parenchyma. Right renal artery (c) and left renal artery (d) showing elevated peak systolic velocity, but normal renal blood flow
Figure 6
Figure 6. SCr, urine output, and Hgb progression during the first 10 days of hospitalization
Hgb: hemoglobin; SCr: serum creatinine
See this image and copyright information in PMC

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