Clinical, electrophysiologic, and histopathologic profile, and outcome in idiopathic inflammatory myositis: An analysis of 68 cases

Abstract

Objectives:To study the electroclinical and histopathologic profile of idiopathic inflammatory myositis (IIM) with reference to prognosis and survival rate.Materials and Methods:Diagnosis of IIM was based on the Bohan and Peter criteria. Patients who improved and those whose condition worsened or who expired due to IIM per se at last follow-up were classified to have favorable and poor outcomes, respectively. Fisher’s exact test was used for univariate analysis of prognostic factors.Results:The study cohort consisted of consecutive 68 patients with IIM. The mean age at diagnosis was 36.5 years and females constituted 71%. Of these patients, 62% had definite IIM, 49% had polymyositis, 20% had dermatomyositis, and 29% had overlap syndrome. The mean follow-up period was 5.4 years. Prednisolone alone was used in 55 (80%), and azathioprine (1–3 mg/kg/day) alone in 12 (17.6%) as the initial treatment. Relapse of IIM with drug withdrawal was seen in 15 patients (22%); 70% had favorable outcome and 16% had expired. The treatment delay of ≤6 months (P = 0.001), absence of cardiac or lung involvement (P < 0.001), and positive biopsy (P = 0.033) were predictive of a favorable prognosis in the univariate analysis. In multivariate analysis, only the duration of illness of ≤6 months (P = 0.008) and the absence of cardiac or lung involvement (P = 0.001) predicted the favorable outcome at last follow-up. Cumulative survival rate was 95% at 1 year, 86% at the 5th year, and 80% at the 10th year.Conclusions:Approximately, two-thirds of the patients showed good electroclinical and histopathologic correlations and an equal number improved with treatment. The treatment delay (≥6 months), presence of cardiac or pulmonary involvements, and negative muscle biopsy are bad prognostic factors.

Favourable Functional Outcomes In Idiopathic Inflammatory Myositis- A Single Centre Experience Over 15 Years

“…31 Another Indian Cohort by Ramesha et al have described a medication free remission in 39/68 (57%) of their cohort, however their cohort size is much smaller with lesser duration of follow-up. 35 Treatment free remission in our cohort was 13.8%; it was associated with longer duration of followup, absence of baseline dysphagia and Ro52 positivity. Treatment free remission is a subset which needs a dedicated study in future prospective cohorts.…”

Observational longitudinal study to assess the outcome of patients with idiopathic inflammatory myositis using validated tools

“…4 From India, Ramesh et al reported 70% of their patients were in remission which they have termed as a favorable outcome, while 57% were off medication termed as an excellent outcome. 11 We could not find any baseline factors predicting inactive disease or good response. Few studies had additionally reported the predictors of the patient's good response to the treatment, such as duration of illness less than 6 months, absence of cardiac involvement, 4,11 age less than 60 years, and absence of dysphagia.…”

“…4,5,7,10 Indian data focusing on cohort-based long-term outcomes such as remission status, muscle strength regaining, and damage accumulation are limited. 11,12 The validated tools were not used in either of the studies for measuring the outcome of this patient group. In contrast, significant data are available on the outcome of the juvenile form of dermatomyositis.…”

Clinical, Serological, and Genetic Characteristics of a Hungarian Myositis-Scleroderma Overlap Cohort

“…To date there are not many studies reported in the literature of scleroderma-myositis overlap. Most of them are limited to a small number of overlap cases [ 4 , 25 – 28 ]. The comparison of our data with the results of other workgroups is challenging, since despite the definitions of overlap syndromes being obvious, sometimes it is not clear, whether myositis is represented as muscle involvement of SSc or as a different disease.…”