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Journal Article Epublish 2 citations

PubMed: 39797367 DOI: 10.3390/jcm14010285

Non-Classical Complications of Adult-Onset Still's Disease: A Multicenter Spanish Study.

J Clin Med 14(1)

Authors: Javier Narváez, Judith Palacios-Olid, María Jesús García de Yebenes, Susana Holgado, Alejandro Olivé, Ivette Casafont-Solé, Santos Castañeda, Cristina Valero-Martínez, María Martín-López, Patricia E Carreira, Maribel Mora-Limiñana, Laura Nuño-Nuño, Angel Robles-Marhuenda, Pilar Bernabeu, José Campos, Jenaro Graña, Vera Ortiz-Santamaria, Marisol Camacho-Lovillo, Carmen Vargas, Judith Sanchez-Manubens, Jordi Anton

Affiliations

  • Department of Rheumatology, Hospital Universitario de Bellvitge, Bellvitge Biomedical Research Institute (IDIBELL), 08907 Barcelona, Spain.
  • Instituto de Salud Musculoesquelética, 28045 Madrid, Spain.
  • Hospital Universitari Germans Trias i Pujol, 08916 Badalona, Spain.
  • Hospital Universitario de La Princesa, IIS-Princesa, Cátedra UAM-Roche, Universidad Autónoma de Madrid, 28006 Madrid, Spain.
  • Hospital Universitario 12 de Octubre, 28041 Madrid, Spain.
  • Hospital Universitario La Paz, 28046 Madrid, Spain.
  • Hospital General Universitario de Alicante, 03010 Alicante, Spain.
  • Hospital Universitario Puerta de Hierro, 28222 Madrid, Spain.
  • Complejo Hospitalario Universitario de A Coruña, 15006 A Coruña, Spain.
  • Hospital General de Granollers, 08402 Barcelona, Spain.
  • Hospital Universitario Virgen del Rocío, 41013 Sevilla, Spain.
  • Hospital Universitario Virgen Macarena, 41009 Sevilla, Spain.
  • Hospital Universitari Parc Taulí, 08208 Sabadell, Spain.
  • Department of Pediatric Rheumatology, Institut de Recerca Sant Joan de Déu, Hospital Sant Joan de Déu, Universitat de Barcelona, 08950 Barcelona, Spain.

Abstract

To investigate the prevalence and clinical spectrum of atypical or non-classical complications in adult-onset Still's disease (AOSD) beyond macrophage activation syndrome (MAS) and to identify factors linked to their occurrence. Multicenter cross-sectional study of AODS cases included in the Spanish registry on Still's disease. This study included 107 patients (67% women), of whom 64 (59.8%) developed non-classical complications. These include macrophage activation syndrome in 9.5%, atypical skin manifestations in 38.8%, cardiac involvement in 22.7% (comprising pericarditis, myocarditis, pulmonary arterial hypertension, and noninfectious endocarditis), pleuritis in 28.9%, transient pulmonary infiltrates in 4%, significant headache in 14.1%, lower abdominal pain with evidence of peritonitis in 8.4%, and secondary amyloidosis in 0.9%. In the multivariate logistic regression analysis, lymphadenopathy (OR 2.85, 95% CI 1.03-7.91, = 0.044) and the systemic score system (SSC) index (OR 1.86, 95% CI 1.29-2.69, = 0.001) were independently associated with the development of non-classical clinical manifestations. In contrast, typical exanthema was associated with a reduced risk of these complications (OR 0.32, 95% CI 0.11-0.95, = 0.041). In addition to the typical clinical manifestations and MAS, a significant proportion of patients with AOSD develop uncommon complications, some of which can be potentially life-threatening. These should be considered in the evaluation and follow-up of patients. Early recognition and prompt management are crucial to significantly reduce morbidity and mortality.

MeSH

Macrophage Activation, Macrophages, Still'S Disease, Adult-Onset, Macrophage Activation Syndrome

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