Clinical characteristics and favorable long-term outcomes for patients with idiopathic inflammatory myopathies: a retrospective single center study in China

Abstract

BackgroundLittle is known about the clinical features and true survival risk factors in Chinese Han population. We conducted the current study to investigate the clinical features, long-term outcome and true potential indicators associated with mortality of idiopathic inflammatory myopathies (IIM) in China.MethodsWe restrospectvely investigated 188 patients diagnosed with IIM at our hospital from January 1986 to April 2009. The primary outcome was determined with mortality. The secondary outcomes for survival patients were organ damage and disease activity, health status, and disability, which were assessed with Myositis Damage Index, Myositis Disease Activity Assessment Visual Analogue Scales, Health Assessment Questionnaire Disability Index, and the Modified Rankin Scale, respectively. Potential prognostic factors for mortality were analyzed with the multivariate Cox regression model.ResultsMean age at disease onset was 43.8 ± 15.8 years and male to female ratio was 1:2.1 in this cohort. The 1-, 5-, 10-, 15- and 20-year survival rates were 93.6%, 88.7%, 81%, 73.6% and 65.6%. The independent predicators for mortality were age at disease onset [hazard ratio (HR):1.05, 95% CI 1.02 - 1.08], presence of cancer (HR:3.68, 95%CI 1.39 - 9.74), and elevated IgA level at diagnosis (HR:2.80, 95% CI 1.16-6.74). At the end of the follow-up, 29 patients manifested drug withdrawal within an average 4.1 years (range 0.5-15.2 year), most patients (85.9%) had no disease activity and 130 patients (83.4%) had no disability.ConclusionsThe long-term outcomes of IIM patients in our cohort have improved dramatically. Those patients most likely to survive had a high chance of reaching stable disease status, and obtained long-term or possibly permanent remission to a large extent.

Inflammatory Muscle Disease, 15-Year Experience at Tertiary Centers

“…Our analysis showed a PM predominance of (32.14%), consistent with the American and European reports [21][22][23][24], and in contrast to a Chinese study [19], which showed a DM predominance.…”

“…This is in accordance with Arabian Studis in Riyadh and Jordan where the mean age of onset was 37.3 ± 16.3, and the female:male ratio was 2.7:1 and 34.3 ± 9.2 years, and female:male ratio was 1.7:1 [14,18] and differing from data reported by Chinese, [19] and Tunisian [20] studies showing higher ratio and mean age.…”

“…The most common clinical presentation was muscular weakness with predominance of proximal myopathy in (93.9%) followed by arthralgia/arthritis (82.1%), both with a higher prevalence than other studies [14,19]. Several nonmuscular manifestations have been wildly described, and dysphagia is the most common among all [6,7,23,25,26], it is noted that we have lower prevalence of dysphagia (35.70%) than other studies [14,27] which could be contributed to early diagnosis and intervention.…”

Abnormally increased low-density granulocytes in peripheral blood mononuclear cells are associated with interstitial lung disease in dermatomyositis

“…In our previous study, data showed that the Myositis Disease Activity Assessment Visual Analogue Scale (MYOACT) recommended by the international myositis outcome assessment collaboration study (IMACS) can be used to reliably evaluate disease activity in Chinese patients with PM/DM [14,15]. Therefore, we used the MYOACT to assess disease activity [15].…”

Early Illness Features Associated With Mortality in the Juvenile Idiopathic Inflammatory Myopathies

“…Of the mortality features from our univariable analyses, those also described in adult IIM studies included older age at illness onset (4–6, 10–13, 29), delay in diagnosis or treatment (4, 5, 10), ILD (6, 12–14), anti-synthetase autoantibodies (8, 9, 30) and dysphagia (6). Our study in JIIM did not identify other important mortality risk factors that have been seen in adult IIM, such as malignancy (4, 5, 10–13), cardiac involvement and ischemic cardiovascular disease (6, 8, 11), skin ulcers (10, 13) and male gender (6, 11, 29). The presence of anti-SRP autoantibodies has been found to be a risk factor for death by some investigators (30), but not by others (31); anti-SRP autoantibodies did not appear to increase the risk of death in JIIM in our study.…”