Jesús Loarce-Martos, José Luis Tandaipan, Marta Domínguez-Álvaro, Irene Carrión-Barberà, Laura Nuño-Nuño, Julia Martínez-Barrio, Vega Jovaní, Fredeswinda Romero-Bueno, Esther Ruiz-Lucea, Eva Tomero, Ernesto Trallero-Araguás, Javier Narváez, Jordi Camins-Fàbregas, Alberto Ruiz-Román, Isabel de la Cámara-Fernández, Anne Riveros-Frutos, V Miguel Flores-Rodríguez, Francisca Sivera, Carolina Merino-Argumanez, Antonio Juan-Mas, Irene Altabás-González, María Martín-López, Joaquín María Belzunegui-Otano, Carmen Carrasco-Cubero, Mercedes Freire-González, Iñigo Rúa-Figueroa, Nuria Lozano-Rivas, Julio David Suarez-Cuba, Ana Isabel Turrión-Nieves, Rafaela Ortega-Castro, Patricia Alcocer, Chamaida Plasencia-Rodríguez, Francisco Javier Prado-Galbarro, Tatiana Cobo-Ibáñez
ASyS-DM-skin patients displayed a distinct clinical phenotype, characterized by DM-type cutaneous involvement while retaining ASyS features, particularly interstitial lung disease, Raynaud's phenomenon, and mechanic's hands.
OBJECTIVES: To characterize the clinical profile of patients with anti-synthetase syndrome (ASyS) presenting with dermatomyositis (DM)-type skin lesions (ASyS-DM-skin) and compare them with patients with ASyS without DM-type skin lesions (ASyS-nonDM-skin) and patients with DM.
METHODS: We performed a cross-sectional study of baseline data from the Spanish registry of patients with IIM (Myo-Spain). Patients with ASyS (classified as ASyS-DM-skin or ASyS-nonDM-skin) and DM were included. We compared characteristics between groups using univariable analysis. Two multivariable logistic regression models were evaluated: (1) factors associated with DM-type skin lesions among ASyS patients; and (2) factors associated with an ASyS diagnosis among patients with DM-type skin lesions.
RESULTS: We included 194 patients with ASyS (61 [31.4%] ASyS-DM-skin) and 116 with DM. In the multivariable model restricted to patients with ASyS, DM-type skin lesions were positively associated with periungual capillary alterations (OR 2.49, 95% CI 1.10-5.65), and negatively associated with age at diagnosis (OR 0.97, 95% CI 0.94-0.99) and arthralgia (OR 0.39, 95% CI 0.15-1.00). Among patients with DM-type skin lesions, ASyS diagnosis was positively associated with Raynaud's phenomenon (OR 6.84, 95% CI 1.04-44.81), mechanic's hands (OR 12.60, 95% CI 1.55-102.7), and interstitial lung disease (OR 27.83, 95% CI 4.55-170.5), and negatively associated with heliotrope rash or Gottron sign/papules (OR 0.10, 95% CI 0.01-0.73) and muscle weakness at diagnosis (OR 0.11, 95% CI 0.01-0.83). Malignancy was less frequent in ASyS-DM-skin than in DM.
CONCLUSION: ASyS-DM-skin patients displayed a distinct clinical phenotype, characterized by DM-type cutaneous involvement while retaining ASyS features, particularly interstitial lung disease, Raynaud's phenomenon, and mechanic's hands.