Article information
Abstract
Systemic sclerosis (SSc) is a severe autoimmune disease characterized by fibrosis, inflammation, and vasculopathy, with high morbidity and mortality. Cutaneous involvement is an important marker of disease severity, while interstitial lung disease and pulmonary arterial hypertension represent the leading causes of death.
This manuscript presents the first national pharmacologic treatment guideline, developed using the GRADE methodology and structured around a flexible, stepwise therapeutic algorithm. Unlike international guidelines, this proposal prioritizes clinical applicability and context-specific decision-making, given the heterogeneity of resources and access to treatments within our healthcare system.
The guideline was developed by a multidisciplinary panel including rheumatologists, dermatologists, pulmonologists, cardiopulmonologists, and methodologists. Authors formulated ten PICO questions, and a systematic search was conducted in PubMed, Cochrane Library, EMBASE, and LILACS (2006–February 2024), including key earlier literature. Of 594 records identified, 114 studies were ultimately included after evaluation using the GRADE methodology. Steroids, immunosuppressants, immunomodulators, hematopoietic stem cell transplantation, and vasodilators were assessed. A total of 26 recommendations were issued and subjected to Delphi consensus, with agreement defined as ≥80% acceptance.
Keywords:
Systemic sclerosis
Clinical guidelines
Cutaneous involvement
Interstitial lung disease
Arterial pulmonary hypertension
Resumen
La esclerosis sistémica (ES) es una enfermedad autoinmune grave caracterizada por fibrosis, inflamación y vasculopatía, con alta morbimortalidad. La afección cutánea constituye un marcador de gravedad, mientras que la enfermedad pulmonar intersticial y la hipertensión arterial pulmonar representan las principales causas de muerte.
Este manuscrito presenta la primera guía nacional de tratamiento farmacológico, desarrollada mediante la metodología GRADE y estructurada en un algoritmo terapéutico flexible y escalonado. A diferencia de guías internacionales, esta propuesta prioriza la aplicabilidad clínica y la toma de decisiones contextualizada, considerando la heterogeneidad de recursos y de acceso a tratamientos en nuestro sistema de salud.
La guía fue elaborada por un grupo multidisciplinario de reumatólogos, dermatólogos, neumólogos, cardioneumólogos y metodólogos. Se formularon 10 preguntas PICO y se realizó una búsqueda sistemática en PubMed, Cochrane Library, EMBASE y LILACS (2006–febrero 2024), incluyendo la literatura crítica previa. De 594 registros identificados, 114 estudios fueron finalmente incluidos tras su evaluación con la metodología GRADE. Se evaluaron esteroides, inmunosupresores, inmunomoduladores, trasplante de células progenitoras hematopoyéticas y vasodilatadores. Se emitieron 26 recomendaciones, que fueron sometidas a un consenso Delphi, considerándose acuerdo cuando la aceptación fue ≥80%.
Palabras clave:
Esclerosis sistémica
Guía clínica
Afección cutánea
Enfermedad pulmonar intersticial
Hipertensión arterial pulmonar
Article
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