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A Rare Case of Renal Crisis in Systemic Sclerosis–Systemic Lupus Eritematosus Overlap Syndrome: A Case Report and Literature Review

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Abstract

We present a patient with systemic sclerosis–systemic lupus eritematosus (SSc-SLE) overlap syndrome who was initially diagnosed as SLE. The patient underwent kidney biopsy and was diagnosed as SSc renal crisis as a result of the biopsy. With this case, we aimed to raise awareness about renal crisis in limited scleroderma cases. We present a rare case of renal crisis in a 49-year-old male patient with limited SSc-SLE overlap syndrome. The patient, who applied to the rheumatology clinic with complaints of bruising on the fingers and toes, weight loss, and swelling in the legs, was diagnosed with SLE in April 2022. Renal function tests were normal at that time. About 2 months after this diagnosis, he applied to the emergency department of our hospital with complaints of shortness of breath and chest pain. He was admitted to our clinic because his blood pressure was 160/100 mmHg and his creatinine (cre) value was 3.8 mg/dl. We detected SSc renal crisis as a result of kidney biopsy performed in our patient with marked sclerodactyly. The patient was diagnosed with a rare coexistence of limited SSc and SLE. Renal crisis in limited SSc is a rare condition. Our patient is still dialysis dependent. In SSc-SLE overlapping patients presenting with renal failure, the picture may be associated with SSc kidney crisis, lupus nephritis, or a coexistence of kidney crisis and lupus nephritis. The differential diagnosis of renal involvement of SSc-SLE overlap syndrome is a challenging clinical management. Renal crisis is rare, especially in limited SSc. However, renal crisis must be kept in mind in the differential diagnosis who has resistant hypertension and SSc skin findings even though other signs of SSc are subtle.

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Abbreviations

SSc :

Systemic Sclerosis

SLE :

Systemic Lupus Eritematosus

cre :

Creatinine

TMA :

Thrombotic Microangiopathy

ACEİ :

Angiotensin-Converting Enzyme Blockers

CTDs :

Connective Tissue Diseases

Hb :

Hemoglobin

Plt :

Platelet

Wbc :

White Blood Cell

ANA :

Antinuclear Antibody

IFA :

İNdirect Fluorescent Antibody Method

anti-Ds DNA :

Anti-Double-Stranded DNA

anti-SM :

Anti-Smith Antibody

anti-scl-70 :

Anti-Topoisomerase I

anti-RNP :

Anti-Ribonucleoprotein

anti-RNAP-III :

Anti-RNA-Polymerase III

C3 :

Complement 3

C4 :

Complement 4

BUN :

Blood Urea Nitrogen

GFR :

Glomerular Filtration Rate

LDH :

Lactate Dehydrogenesis

MAHA :

Microangiopathic Hemolytic Anemia

CT :

Computed Tomography

ECHO :

Echocardiography

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Dr. Zengin Acemoğlu participated in article preparation. All authors materially participated in the research. All authors participated in data collection. All authors have approved the final article.

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Correspondence to Şerife Şeyda Zengin Acemoğlu.

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Acemoğlu, Ş.Ş.Z., Türk, İ., Suleymanlı, G. et al. A Rare Case of Renal Crisis in Systemic Sclerosis–Systemic Lupus Eritematosus Overlap Syndrome: A Case Report and Literature Review. SN Compr. Clin. Med. 6, 26 (2024). https://doi.org/10.1007/s42399-024-01654-w

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