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

**Authors:** Suela Mumajesi, Saimir Seferi, Nereida Spahia, Elena Gjecka, Floreta Kurti

PMC · DOI: 10.7759/cureus.85861 · Cureus · 2025-06-12

## TL;DR

This case report discusses a rare and complex medical situation where a patient with scleroderma and overlapping Sjögren's syndrome experiences a severe kidney emergency.

## Contribution

The paper presents a case highlighting the challenges of diagnosing and managing scleroderma renal crisis in the context of overlapping Sjögren's syndrome.

## Key findings

- Scleroderma renal crisis combined with Sjögren's syndrome presents complex clinical challenges.
- Early detection and treatment with ACE inhibitors are crucial for better outcomes.
- Overlap syndromes require individualized treatment approaches for optimal care.

## 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.

## Linked entities

- **Diseases:** scleroderma (MONDO:0005100), systemic sclerosis (MONDO:0005100), malignant hypertension (MONDO:0006846), thrombotic microangiopathy (MONDO:0019737)

## Full-text entities

- **Genes:** REN (renin) [NCBI Gene 5972] {aka ADTKD4, HNFJ2, RTD}
- **Diseases:** TMA (MESH:D057049), acute kidney injury (MESH:D058186), renal involvement (MESH:C565423), malignant hypertension (MESH:D006974), hypertension (MESH:D006973), SS (MESH:D012859), SRC (MESH:D012595), renal ischemia (MESH:D007511), renal crisis (MESH:D006030)
- **Chemicals:** aldosterone (MESH:D000450)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

6 figures with captions in the complete paper: https://tomesphere.com/paper/PMC12255470/full.md

## References

17 references — full list in the complete paper: https://tomesphere.com/paper/PMC12255470/full.md

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Source: https://tomesphere.com/paper/PMC12255470