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

• Published in: Journal of clinical medicine 2025-01, Vol.14 (1), p.285
• Main Authors: Narváez, Javier, Palacios-Olid, Judith, García de Yebenes, María Jesús, Holgado, Susana, Olivé, Alejandro, Casafont-Solé, Ivette, Castañeda, Santos, Valero-Martínez, Cristina, Martín-López, María, Carreira, Patricia E, Mora-Limiñana, Maribel, Nuño-Nuño, Laura, Robles-Marhuenda, Angel, Bernabeu, Pilar, Campos, José, Graña, Jenaro, Ortiz-Santamaria, Vera, Camacho-Lovillo, Marisol, Vargas, Carmen, Sanchez-Manubens, Judith, Anton, Jordi
• Format: Article
• Language: English
• Subjects: Arthritis; Data collection; Hematology; Liver; Peritonitis; Regression analysis; Ultrasonic imaging; Variables
• ISSN: 2077-0383; 2077-0383
• DOI: 10.3390/jcm14010285

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.