Tatiana Cobo-Ibáñez1, Francisco-Javier López-Longo2, Beatriz Joven3, Patricia E Carreira3, Santiago Muñoz-Fernández4, Valentina Maldonado-Romero5, Carmen Larena-Grijalba5, Irene Llorente Cubas6, Eva Tomero Muriel6, Carmen Barbadillo Mateos7, Paloma García de la Peña Lefebvre8, Alejandro Gomez-Gomez8, Laura Barrio Nogal9, Ana Pérez9, Raquel Almodovar10, Leticia Lojo11, Lucía Ruiz-Gutiérrez12, Juan Carlos López-Robledillo12, María Jesús García de Yébenes13, Laura Nuño-Nuño14. 1. Rheumatology Department, Hospital Universitario Infanta Sofía, Universidad Europea, Paseo de Europa 34, 28702, Madrid, San Sebastián de los Reyes, Spain. mtcoboiba@yahoo.es. 2. Rheumatology Department, Hospital General Universitario Gregorio Marañón, Dr. Esquerdo 46, 28007, Madrid, Spain. 3. Rheumatology Department, Hospital Universitario 12 de Octubre, Avda. Córdoba s/n, 28041, Madrid, Spain. 4. Rheumatology Department, Hospital Universitario Infanta Sofía, Universidad Europea, Paseo de Europa 34, 28702, Madrid, San Sebastián de los Reyes, Spain. 5. Rheumatology Department, Hospital Universitario Ramón y Cajal, Ctra. de Colmenar Viejo Km. 9,100, 28034, Madrid, Spain. 6. Rheumatology Department, Hospital Universitario de la Princesa, Diego de León 62, 28006, Madrid, Spain. 7. Rheumatology Department, Hospital Universitario Puerta de Hierro Majadahonda, Manuel de Falla 1, 28220, Madrid, Majadahonda, Spain. 8. Rheumatology Department, Hospital Universitario HM Sanchinarro, Oña 10, 28050, Madrid, Spain. 9. Rheumatology Department, Hospital Universitario Príncipe de Asturias, Alcalá-Meco s/n, 28805, Madrid, Alcalá de Henares, Spain. 10. Rheumatology Department, Hospital Universitario Fundación Alcorcon, Budapest s/n, 28922, Madrid, Spain. 11. Rheumatology Department, Hospital Universitario Infanta Leonor, Gran Vía del Este 80, 28031, Madrid, Spain. 12. Rheumatology Department, Hospital Infantil Universitario Niño Jesús, Avenida Menéndez Pelayo 65, 28009, Madrid, Spain. 13. Instituto de Salud Musculoesquelética (InMusc), Conde de la Cimera 6, Oficina C, 28040, Madrid, Spain. 14. Rheumatology Department, Hospital Universitario La Paz, Paseo de la Castellana 261, 28046, Madrid, Spain.
Abstract
OBJECTIVE: To study prognostic factors in different types of idiopathic inflammatory myopathies (IIM) associated with interstitial lung disease (ILD). PATIENTS AND METHODS: Multicenter retrospective study of a Spanish cohort of patients diagnosed with IIM. Patients were classified into four categories: polymyositis (PM), dermatomyositis (DM), antisynthetase syndrome (ASS), and overlap myositis (OM). Sociodemographic data, clinical characteristics, antibodies, and treatments were collected. Cox regression models were calculated to identify factors associated with mortality, the necessity for long-term oxygen therapy (LTOT), and deterioration in respiratory function tests (RFT). RESULTS: The number of patients included was 478, of whom 112 (23.4%) suffered from ILD: 17% PM, 16% DM, 45% ASS, and 22% OM. Factors associated with mortality in the multivariate analysis were clinically meaningful progression of ILD after 3 months (CMP 3m) (hazard ratio (HR) 9.48, p = 0.005), severe infections (HR 6.41, p = 0.016), heliotrope erythema (HR 31.1, p = 0.002), delay in diagnosis (HR 1.29; p = 0.011), and Raynaud's phenomenon (HR 11.9, p = 0.007). However, being female (HR 0.19, p = 0.044) and positivity solely for ANAs (HR 0.08, p = 0.008) presented a protective effect. CMP 3m (HR 22.7, p = 0.027) was associated with the need for LTOT, while basal aldolase (HR 0.90; p = 0.049) had a protective effect. Likewise, joint manifestations (HR 0.04, p = 0.034) were shown to reduce risk of deterioration in RFT. CONCLUSIONS: CMP 3m, severe infections, delay in diagnosis, heliotrope erythema, and Raynaud's phenomenon were identified as factors of poor prognosis in different IIM associated with ILD.
OBJECTIVE: To study prognostic factors in different types of idiopathic inflammatory myopathies (IIM) associated with interstitial lung disease (ILD). PATIENTS AND METHODS: Multicenter retrospective study of a Spanish cohort of patients diagnosed with IIM. Patients were classified into four categories: polymyositis (PM), dermatomyositis (DM), antisynthetase syndrome (ASS), and overlap myositis (OM). Sociodemographic data, clinical characteristics, antibodies, and treatments were collected. Cox regression models were calculated to identify factors associated with mortality, the necessity for long-term oxygen therapy (LTOT), and deterioration in respiratory function tests (RFT). RESULTS: The number of patients included was 478, of whom 112 (23.4%) suffered from ILD: 17% PM, 16% DM, 45% ASS, and 22% OM. Factors associated with mortality in the multivariate analysis were clinically meaningful progression of ILD after 3 months (CMP 3m) (hazard ratio (HR) 9.48, p = 0.005), severe infections (HR 6.41, p = 0.016), heliotrope erythema (HR 31.1, p = 0.002), delay in diagnosis (HR 1.29; p = 0.011), and Raynaud's phenomenon (HR 11.9, p = 0.007). However, being female (HR 0.19, p = 0.044) and positivity solely for ANAs (HR 0.08, p = 0.008) presented a protective effect. CMP 3m (HR 22.7, p = 0.027) was associated with the need for LTOT, while basal aldolase (HR 0.90; p = 0.049) had a protective effect. Likewise, joint manifestations (HR 0.04, p = 0.034) were shown to reduce risk of deterioration in RFT. CONCLUSIONS: CMP 3m, severe infections, delay in diagnosis, heliotrope erythema, and Raynaud's phenomenon were identified as factors of poor prognosis in different IIM associated with ILD.