Systemic Sclerosis Sine Scleroderma in Mexican Patients. Case Reports.

Area of Science:

• Rheumatology
• Internal Medicine
• Immunology

Background:

• Systemic sclerosis sine scleroderma (ssSSc) presents with visceral involvement and anticentromere antibodies (ACA) but lacks skin thickening.
• Raynaud's phenomenon (RP) is a common initial symptom.

Purpose of the Study:

• To investigate the clinical characteristics and prevalence of ssSSc.
• To highlight key diagnostic indicators for ssSSc.

Main Methods:

• Retrospective study of 10 patients diagnosed with ssSSc.
• Analysis of clinical signs including RP, esophageal, cardiac, and pulmonary manifestations, and ACA positivity.

Main Results:

• ssSSc was found in 2% of patients.
• Prevalence of clinical signs: RP (90%), esophageal manifestations (80%), pulmonary arterial hypertension (40%), interstitial lung disease (40%), cardiac signs (30%), and ACA (80%).

Conclusions:

• Anticentromere antibodies (ACA) are a key serological marker for ssSSc.
• In patients with RP, prompt testing for ACA is crucial for diagnosing ssSSc, especially when esophageal dysmotility, interstitial lung disease, or pulmonary arterial hypertension are present.