ESCLEROSIS SISTÉMICA: SERIE DE CASOS CLÍNICOS Y REVISIÓN INTEGRATIVA DE LA EVIDENCIA FISIOPATOLÓGICA ACTUAL

Description

Abstract (English)

Systemic sclerosis (SSc) is a heterogeneous and potentially life-threatening connective tissue disease, characterized by vasculopathy, autoimmunity, and fibrosis. This study aimed to describe a series of four clinical cases and to conduct an integrative review of current pathophysiological and therapeutic evidence. We present a case series with clinical–serological phenotyping and a narrative literature review (1988–2024) using indexed databases, prioritizing guidelines and high-quality studies. Case 1: diffuse SSc with severe cutaneous involvement and digital vasculopathy. Case 2: diffuse SSc with interstitial lung disease (ILD). Case 3: diffuse SSc complicated by scleroderma renal crisis (SRC). Case 4: limited SSc with esophageal gastrointestinal involvement. In all patients, a clear association was observed between autoantibodies (anti-Scl-70, anti-centromere, anti-RNA polymerase III) and clinical phenotype. Fatal outcomes were linked to advanced ILD, sepsis from chronic digital ulcers, refractory SRC, and gastrointestinal bleeding secondary to Barrett’s esophagus/adenocarcinoma. A common factor was treatment discontinuation due to geographic and social barriers in rural settings. Early diagnosis, serological stratification, and standardized monitoring (PFT/DLCO, HRCT, echocardiography) are essential to improve survival. Organ-specific management (MMF, nintedanib, pulmonary hypertension therapies, ACE inhibitors for SRC, PPIs/prokinetics) should be integrated with strategies aimed at reducing inequities in access to care. Future research must focus on predictive biomarkers and personalized approaches that incorporate social determinants of health.