dc1c865100deb526beeb8a581f984632 306975.pdf 8bb86caf0f6130b8796b15ff13a2cfa4e00d6696 306975.pdf 8e801cc1fa23a4498d05ead238b532ea8dadd6d9b785e6eab173309ca94c2c67 306975.pdf Title: Non-Classical Complications of Adult-Onset Still’s Disease:A Multicenter Spanish Study Subject: Objective: 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. Methods: Multicenter cross-sectional study of AODS cases included in the Spanish registry on Still’s disease. Results: 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, p = 0.044) and the systemic score system (SSC) index (OR 1.86, 95% CI 1.29–2.69, p = 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, p = 0.041). Conclusions: 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. Keywords: adult-onset still disease; clinical manifestations; prognostic Author: 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-Manubens, Judith Sanchez-Manubens and Jordi Anton Creator: LaTeX with hyperref Producer: pdfTeX-1.40.25 CreationDate: Wed Jan 8 10:05:17 2025 CET ModDate: Wed Jan 8 10:09:09 2025 CET Custom Metadata: no Metadata Stream: no Tagged: no UserProperties: no Suspects: no Form: none JavaScript: no Pages: 12 Encrypted: no Page size: 595.276 x 841.89 pts (A4) Page rot: 0 File size: 272643 bytes Optimized: no PDF version: 1.7 name type encoding emb sub uni object ID ------------------------------------ ----------------- ---------------- --- --- --- --------- DGBDNV+VnURWPalladioL-Bold Type 1 Custom yes yes yes 10 0 HPOJQD+URWPalladioL-Roma Type 1 Custom yes yes yes 16 0 TRSFKR+URWPalladioL-Bold Type 1 Custom yes yes yes 22 0 SINCUW+URWPalladioL-Ital Type 1 Custom yes yes yes 27 0 IYDCBB+VnURWPalladioL Type 1 Custom yes yes yes 32 0 UUHHNQ+CMSY10 Type 1 Builtin yes yes yes 61 0 QLBIEB+URWPalladioL-BoldItal Type 1 Custom yes yes yes 66 0 LWLIVD+EURM10 Type 1 Builtin yes yes yes 89 0 Jhove (Rel. 1.28.0, 2023-05-18) Date: 2025-01-31 02:45:55 CET RepresentationInformation: 306975.pdf ReportingModule: PDF-hul, Rel. 1.12.4 (2023-03-16) LastModified: 2025-01-30 19:27:06 CET Size: 272643 Format: PDF Version: 1.7 Status: Well-Formed and valid SignatureMatches: PDF-hul MIMEtype: application/pdf PDFMetadata: Objects: 254 FreeObjects: 1 IncrementalUpdates: 0 DocumentCatalog: PageLayout: SinglePage PageMode: UseNone Outlines: Item: Title: Introduction Destination: section.1 Item: Title: Methods Destination: section.2 Item: Title: Results Destination: section.3 Children: Item: Title: Non-Classical Clinical Manifestations Destination: subsection.3.1 Item: Title: Predictors of Development of Non-Classical Clinical Manifestations Destination: subsection.3.2 Item: Title: Discussion Destination: section.4 Item: Title: References Destination: section.5 Info: Title: Non-Classical Complications of Adult-Onset Still’s Disease:A Multicenter Spanish Study Author: 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-Manubens, Judith Sanchez-Manubens and Jordi Anton Subject: Objective: 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. Methods: Multicenter cross-sectional study of AODS cases included in the Spanish registry on Still’s disease. Results: 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, p = 0.044) and the systemic score system (SSC) index (OR 1.86, 95% CI 1.29 2.69, p = 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, p = 0.041). Conclusions: 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. 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