Mathiesen, Pernille Raasthøj2; Buchvald, Frederik Fouirnaies3; Nielsen, Kim G4; Herlin, Troels7; Friis, Tina5; Nielsen, Susan6
1 Department of Clinical Medicine - Department of Paediatrics, Department of Clinical Medicine, Health, Aarhus University2 Gynækologi, Obstetrik og Pædiatri3 Institut for Klinisk Medicin4 The Department of Ophthalmology, Faculty of Health Sciences, Aarhus University, Aarhus University5 Statens Serum Institut6 Department of Integrated Pest Management, Faculty of Agricultural Sciences, Aarhus University, Aarhus University7 Department of Clinical Medicine - Department of Paediatrics, Department of Clinical Medicine, Health, Aarhus University
Objectives. Pulmonary disease is a rare complication in JDM, described in only a few studies. This long-term follow-up study aimed to (i) describe pulmonary involvement in a national cohort of JDM patients estimated by conventional spirometry, (ii) compare pulmonary impairment with overall JDM outcome, and (iii) identify possible associations between pulmonary impairment and myositis-specific autoantibodies (MSAs).Methods. Fifty-one JDM patients performed conventional spirometry in a cross-sectional follow-up study. The scores of the Myositis Damage Index (MDI), Myositis Damage by visual analogue scale (MYODAM-VAS) and physician's global damage assessment were used to estimate JDM outcome. ANAs, MSAs and myositis-associated autoantibodies were analysed in all patients.Results. Forty-two patients (82%) (mean follow-up time 14.3 years) had normal lung function. Four patients (8%) were diagnosed with JDM-related restrictive interstitial lung disease. No patients reported pulmonary symptoms. Patients with restrictive pulmonary function had increased long-term damage estimated by MDI (P = 0.008), MYODAM-VAS (P = 0.04), global assessment (P = 0.03) and number of organ systems involved (P = 0.009). We found significant correlation between the restrictive pulmonary function test and damage by the MDI (r = 0.43, P = 0.003), MYODAM-VAS (r = 0.44, P = 0.002), and global damage assessment (r = 0.43, P = 0.003). No association was found between the restrictive pulmonary function test and autoantibodies.Conclusion. In a long-term follow-up study of JDM patients, the majority of patients demonstrated normal lung function. However, restrictive pulmonary impairment was identified in 8% of patients, indicating a need for repetitive pulmonary follow-up in JDM patients. Restrictive pulmonary involvement was associated with increased long-term JDM damage.
Rheumatology (online), 2014, Vol 53, Issue 4, p. 644-649