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Distinct clinical trajectories of gastrointestinal progression among patients with systemic sclerosis.
Perin, J; Hughes, M; Mecoli, C A; Paik, J J; Gelber, A C; Wigley, F M; Hummers, L K; Shah, A A; Zeger, S L; McMahan, Z H.
Afiliación
  • Perin J; Department of International Health, Johns Hopkins Bloomberg School of Public Health, Baltimore, MD, USA.
  • Hughes M; Department of Rheumatology, Northern Care Alliance NHS Foundation Trust, Salford Care Organisation, Salford, UK.
  • Mecoli CA; Division of Musculoskeletal and Dermatological Sciences, The University of Manchester, Manchester, UK.
  • Paik JJ; Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
  • Gelber AC; Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
  • Wigley FM; Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
  • Hummers LK; Department of Epidemiology, Johns Hopkins Bloomberg School of Public Health, Baltimore, MD, USA.
  • Shah AA; Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
  • Zeger SL; Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
  • McMahan ZH; Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
Rheumatology (Oxford) ; 2024 Aug 30.
Article en En | MEDLINE | ID: mdl-39213328
ABSTRACT

OBJECTIVES:

Systemic sclerosis (SSc) is heterogeneous in its clinical presentation. Common manifestations cluster together, defining unique subgroups. This investigation aims to characterize gastrointestinal (GI) phenotypes and determine whether they can be distinguished by temporal progression.

METHODS:

We examine a well-established SSc patient cohort with a modified Medsger GI severity score measured over time to determine heterogeneity in disease progression. Growth mixture models estimate each patient's phenotype and disease severity trajectory over time. We compare the characteristics of estimated phenotypes using non-parametric statistics and linear and logistic regression to compare patient characteristics between phenotypes while adjusting for disease duration.

RESULTS:

We examined 2696 SSc patients with at least two Medsger GI scores, identifying four unique phenotypes. The most common phenotype (n = 2325) ("Stable") had an average score of 1 that was consistent over time. Two phenotypes were progressive ["Early Progressive" (n = 142) and "Late Progressive" (n = 115)] with an initial average score of 1. The Early Progressive group increased initially and stabilized, and the Late Progressive group worsened slowly over time. A fourth phenotype ["Early Severe GI"; (n = 114)] had an initial average Medsger GI score just below 3 with high mortality and improving GI severity over time.

CONCLUSIONS:

Clinically distinct GI phenotypes exist among patients with SSc. These phenotypes are not only distinguished by GI and extra-intestinal SSc clinical complications, but they are also temporally distinct. Distinct autoantibody profiles are associated strongly with more severe GI disease.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Idioma: En Revista: Rheumatology (Oxford) Asunto de la revista: REUMATOLOGIA Año: 2024 Tipo del documento: Article País de afiliación: Estados Unidos Pais de publicación: Reino Unido
Texto completo
Añadir a Mi BVS
Imprimir
XML
PubMed Links
Buscar en Google

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Idioma: En Revista: Rheumatology (Oxford) Asunto de la revista: REUMATOLOGIA Año: 2024 Tipo del documento: Article País de afiliación: Estados Unidos Pais de publicación: Reino Unido