Maria Giovanna Danieli1, Simona Gambini2, Lucia Pettinari2, Francesco Logullo3, Giovanni Veronesi4, Armando Gabrielli2. 1. Clinica Medica, Dipartimento di Scienze Cliniche e Molecolari, Università Politecnica delle Marche, Ancona, Italy. Electronic address: m.g.danieli@univpm.it. 2. Clinica Medica, Dipartimento di Scienze Cliniche e Molecolari, Università Politecnica delle Marche, Ancona, Italy. 3. Clinica Neurologica, Università Politecnica delle Marche, Ancona, Italy. 4. Centro ricerche EPIMED - Epidemiologia e Medicina Preventiva, Università degli studi dell'Insubria, Varese, Italy.
Abstract
OBJECTIVE: To assess the long-term outcome in polymyositis (PM) and dermatomyositis (DM), with a particular emphasis on mortality and influence of treatment. METHODS: Diagnosis was based according to the Bohan and Peter's criteria. Patients have been followed up by a standardised protocol. Deaths were registered and causes of death were ascertained. Survival probability at 5 and 10years was estimated according to the Kaplan-Meier method, in the overall series and by a diagnostic group and an initial treatment. Mortality hazard ratios (95% CI) for major clinical and demographic features were estimated through univariate and multivariate Cox proportional hazard models. RESULTS: 91 patients (43 PM and 48 DM) were available for the study. Baseline characteristics were not different from those previously reported. Twenty-two patients (24%) died after a median follow-up of 8.7years. As for idiopathic myositis, the survival probabilities at 5 and 10years from the diagnosis were 96.2% and 88.8% for PM respectively; and 93.9% for DM, whereas a higher mortality was documented for cancer-associated myositis and overlap myositis. Male sex [HR=2.4, 95% CI 1.0 to 5.6], heart involvement (HR=1.8), interstitial lung disease (HR=2.3) and arthritis (HR=1.8) increased the risk of mortality, these risk excesses were confirmed in the multivariate analysis. Independent of these features, a higher mortality was documented for patients treated with glucocorticoids (HR=2.3) or immunosuppressants (HR=2.1) when compared to patients treated with immunoglobulins. CONCLUSION: Our study, with longitudinal and statistical analyses, suggests that survival has considerably increased in patients with PM/DM. Prognostic factors for mortality are male sex, and heart and lung involvement. Immunoglobulin treatment, intravenously or subcutaneously, is associated with a better survival.
OBJECTIVE: To assess the long-term outcome in polymyositis (PM) and dermatomyositis (DM), with a particular emphasis on mortality and influence of treatment. METHODS: Diagnosis was based according to the Bohan and Peter's criteria. Patients have been followed up by a standardised protocol. Deaths were registered and causes of death were ascertained. Survival probability at 5 and 10years was estimated according to the Kaplan-Meier method, in the overall series and by a diagnostic group and an initial treatment. Mortality hazard ratios (95% CI) for major clinical and demographic features were estimated through univariate and multivariate Cox proportional hazard models. RESULTS: 91 patients (43 PM and 48 DM) were available for the study. Baseline characteristics were not different from those previously reported. Twenty-two patients (24%) died after a median follow-up of 8.7years. As for idiopathic myositis, the survival probabilities at 5 and 10years from the diagnosis were 96.2% and 88.8% for PM respectively; and 93.9% for DM, whereas a higher mortality was documented for cancer-associated myositis and overlap myositis. Male sex [HR=2.4, 95% CI 1.0 to 5.6], heart involvement (HR=1.8), interstitial lung disease (HR=2.3) and arthritis (HR=1.8) increased the risk of mortality, these risk excesses were confirmed in the multivariate analysis. Independent of these features, a higher mortality was documented for patients treated with glucocorticoids (HR=2.3) or immunosuppressants (HR=2.1) when compared to patients treated with immunoglobulins. CONCLUSION: Our study, with longitudinal and statistical analyses, suggests that survival has considerably increased in patients with PM/DM. Prognostic factors for mortality are male sex, and heart and lung involvement. Immunoglobulin treatment, intravenously or subcutaneously, is associated with a better survival.
Authors: Laura Nuño-Nuño; Beatriz Esther Joven; Patricia E Carreira; Valentina Maldonado-Romero; Carmen Larena-Grijalba; Irene Llorente Cubas; Eva Gloria Tomero; María Carmen Barbadillo-Mateos; Paloma García De la Peña Lefebvre; Lucía Ruiz-Gutiérrez; Juan Carlos López-Robledillo; Henry Moruno-Cruz; Ana Pérez; Tatiana Cobo-Ibáñez; Raquel Almodóvar González; Leticia Lojo; María Jesús García De Yébenes; Francisco Javier López-Longo Journal: Rheumatol Int Date: 2017-09-02 Impact factor: 2.631
Authors: Jacqueline Kerr; Isabella Quinti; Martha Eibl; Helen Chapel; Peter J Späth; W A Carrock Sewell; Abdulgabar Salama; Ivo N van Schaik; Taco W Kuijpers; Hans-Hartmut Peter Journal: Front Immunol Date: 2014-12-12 Impact factor: 7.561