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Scleroderma, myositis and related syndromes - clinical aspects and treatment
FRI0463 Survival and Mortality Analysis in a Large Cohort of Spanish Patients with Anti-JO1 Antisynthetase Syndrome from the Geas-IIM Group
  1. E. Trallero-Araguás1,
  2. J.M. Grau-JUnyent2,
  3. M. Monteagudo3,
  4. F. Garcia-Hernandez4,
  5. G. Fraile-Rodriguez5,
  6. I. Les-Bujan6,
  7. L. Saez-Comet7,
  8. M. Rodriguez-Carballeira8,
  9. R. Rios-Fernandez9,
  10. L. Caminal-Montero10,
  11. X. Solanich-Moreno11,
  12. A. Selva-O'Callaghan1
  13. on behalf of Idiopathic Inflammatory Myositis (IIM) Study Group and Autoimmune Diseases Study Group (GEAS) of the Spanish Society of Internal Medicine (SEMI)
  1. 1Vall d'Hebron Hospital
  2. 2Hospital Clinic, Barcelona
  3. 3Hospital Parc Tauli, Sabadell
  4. 4Hospital Virgen del Rocio, Sevilla
  5. 5Hospital Ramon y Cajal, Madrid
  6. 6Hospital de Cruces, Bilbao
  7. 7Hospital Miguel Servet, Zaragoza
  8. 8Hospital Mutua de Terrasa, Terrasa
  9. 9Hospital San Cecilio, Granada
  10. 10Hospital Central de Asturias, Oviedo
  11. 11Hospital Bellvitge, Barcelona, Spain

Abstract

Background Antisynthetase syndrome (ASS) can be a life-threatening condition. Patients with ASS in the context of idiopathic inflammatory myositis (IIM) have been described to have a worse prognosis compared to patients either with dermatomyositis (DM) or polymyositis (PM) alone.

Objectives To evaluate survival and mortality in a large cohort of Spanish patients with anti-Jo1 ASS and compare it with mortality rates of Spanish general population.

Methods From March 2011 to June 2014 we conducted a national multicentre study, including retrospective and prospective data, among members of the GEAS-IIM Group of the SEMI. Clinicians from 18 Spanish centers participated in the development of a registry of patients with Anti-Jo1 ASS. For data collection a specific electronic database including broad information about demographic and clinical data was developed. Continuous variables were compared by the t-student or non-parametric Mann-Whitney tests when appropriated, while X2 test were used to compare categorical variables. The Kaplan-Meier method and long-rank tests were used to compare survival between groups. The standardized mortality ratio (SMR) was calculated using the Spanish mortality registry. Results were expressed with its 95% interval confiance (IC).

Results A total of 148 anti-Jo1 ASS patients, 90 women (60.8%), were included in the study. Mean (SD) age at diagnosis was 50.8 (16) years. Range follow-up was 1.3 to 412 months [mean (SD) 95 (76.4) months], with only 12 patients with a follow-up fewer than 12 months. Fifteen percent of patients had ILD as the unique main ASS clinical manifestation, other 15% had myositis and 69.3% had both ILD and myositis. Thirty seven patients (25%) died after a median follow-up of 70.3 months (range 4.9 – 308 months). The SMR (95%IC) was 4.77 (3.31-6.67). Main causes of death included respiratory failure (6.8%), cancer (6.1%), infection (4.1%), myositis (2.7%) and acute cardiovascular events (1.3%). A directly ASS-related death were considered in 18 patients (46.6%). Estimated survival rate (95% IC) was 87.5% (81.6% - 93.4%) and 75.4% (66.4% - 84.4%) at 5 and 10 years respectively. Median survival rate (95% IC) was 20.3 years (13.8-26.8 years). No difference in survival was found between patients with myositis (DM or PM) and those with ILD without associated myopathy. The presence of cancer (p=0.02) and an advanced age at ASS diagnosis (p<0.0001) were the unique clinical features significantly related to poorer survival rates.

Conclusions Mortality in ASS patients is four-fold and a half higher than in the general population. Mortality can be directly attributed to ASS in near a half of the cases. Respiratory failure, cancer and infection are the main causes of death. Estimated survival rate at 10 years is around 75%.

References

  1. Marie I. Morbidity and mortality in adult polymyositis and dermatomyositis. Curr Rheumatol Rep.2012;14:275-285.

Disclosure of Interest None declared

https://doi.org/10.1136/annrheumdis-2015-eular.6064

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