Clinical Characteristics and Course of Patients with Juvenile Dermatomyositis – Case Series

Rajarathinam, Indhuja; Raghuram, Jyothi; Rao, Anand Prahalad

doi:10.4103/0028-3886.338646

Neurology India

2022

DOI: 10.4103/0028-3886.338646

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Clinical Characteristics and Course of Patients with Juvenile Dermatomyositis – Case Series

Indhuja Rajarathinam

Jyothi Raghuram

Anand Prahalad Rao³

Abstract: Background: Juvenile dermatomyositis (JDM) is one of the commonest forms of inflammatory myositis in childhood. Objective: The objective study was to study the clinical characteristics and course of JDM patients. Material and Methods: Retrospective analysis of the charts of 25 JDM patients admitted to two hospitals in Bangalore from March 2011 to July 2017. Results: … Show more

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Cited by 3 publications

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Achieving Medication‐Free Remission in Patients With Juvenile Dermatomyositis

Maccora,

Brunner,

Cassedy

et al. 2024

ACR Open Rheumatology

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ObjectivePrognostic factors associated with medication discontinuation in children with juvenile dermatomyositis (JDM) remain largely elusive. We aim to identify the predictors of medication‐free remission (MFR) in children with JDM.MethodsIn this retrospective study, patients diagnosed with JDM according to Peter & Bohan criteria and followed for ≥18 months at a tertiary care center from 2006 through 2022 were included. Data extracted included demographics, physical examination, laboratory results, and medications. MFR was defined as inactive JDM after discontinuation of all systemic immunosuppressives for ≥6 months, in line with international consensus guidelines for trials of therapies in idiopathic inflammatory myopathies. A two‐sided P < 0.05 was considered statistically significant.ResultsOf 55 patients with JDM (63.6% female, age median [interquartile range (IQR)] 6 [3.5–12] years), 29 (52.7%) achieved MFR after a median (IQR) of 33 (22.5–55.2) months. MFR was more common in those who were younger at JDM diagnosis (median 5 vs 8 years, P = 0.008), had early resolution of disease activity (median 11 vs 18 months, P < 0.001), and presented with Gottron papules (χ2 = 5.25; P = 0.022) and elevated lactate dehydrogenase (χ2 = 4.82, P = 0.028). Diagnosis of JDM before 5 years old (odds ratio 4.5, 95% confidence interval [CI] 1.2–16.7) was the only predictor of MFR in our multivariate model (area under the curve 0.65, 95% CI 0.53–0.76).ConclusionHalf of our patients with JDM achieved MFR. Age at JDM diagnosis may be an important predictor of achieving MFR.

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Achieving Medication‐Free Remission in Patients With Juvenile Dermatomyositis

Maccora,

Brunner,

Cassedy

et al. 2024

ACR Open Rheumatology

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

Shukla,

Gaur

2023

Int J of Rheum Dis

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IntroductionThere have been major changes in the classification and treatment of patients with idiopathic inflammatory myositis (IIM) in the last 2 decades. A major challenge is to identify the parameters that can affect the outcome and prognosis of these patients. Here, we have longitudinally followed a well‐characterized cohort of IIM patients in a rheumatology center and reported the outcome using the validated tools.MethodPatients with a clinical diagnosis of IIM and a follow‐up duration of greater than 2 years were prospectively included in the study. The duration of the study was 6 years: July 2016–July 2022. Clinical details and follow‐up were recorded using pro‐formas and outcomes were noted using validated tools. Ethics approval and written informed consent were taken.ResultsForty patients had a clinical diagnosis of IIM. Mean follow‐up duration was 43.8 (15) months. Out of 40 patients, 32 (80%) achieved remission (8 patients each were off corticosteroid and off treatment for >6 months), 5 (12%) expired and 3 (8%) had active disease. Disease course was non‐relapsing in 22/35 (73%) patients. Mean manual muscle testing‐8 score (n = 29) and myositis disease activity assessment tool score (n = 35) at the final visit were 75.6 (6.8) and 0.048 (0.07) respectively. Thirteen patients had damage (37%). Patients with disease duration >1 year at the time of presentation were more likely to develop chronic‐continuous disease course (P = .023, odds ratio [OR] = 7.6), more frequently required second‐line or third‐line immunosuppression (P = .001, OR = 24) with higher myositis damage index score (p = .0002, OR = 47).ConclusionsIIM patients had good outcomes with the majority achieving remission and near‐complete muscle recovery. However, the patients presenting late to the rheumatologists were more likely to have smoldering disease, more immunosuppressive medicines, and greater accumulated damage.

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Clinicoepidemiological Spectrum of Connective Tissue Diseases in the Pediatric Population in a Tertiary Care Center in North India: A Cross-sectional Observational Study

Senapati,

Mendiratta,

Maheshwari

et al. 2024

Indian Journal of Paediatric Dermatology

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Background: Connective tissue diseases (CTDs) are chronic in nature and carry a high risk of morbidity, disability, and mortality if not recognized and treated promptly. Data on clinical spectrum of CTDs in the pediatric age group are sparse from developing countries such as India. Objective: To study the clinicoepidemiological spectrum of CTDs in the pediatric age group. Methodology: A hospital-based, cross-sectional, observational study was undertaken over a period of 16 months from January 2021 to June 2022 in the dermatology outpatient department and pediatric rheumatology clinic in a tertiary care hospital in North India. Clinical and laboratory findings were recorded. Results: A total of 100 children diagnosed with a CTD were included in the study. The mean age was 11.11 ± 4.20 years. Out of 100 children, 55% were female and 45% were male with a male-to-female ratio of 1:1.2. Our study included 63% juvenile idiopathic arthritis, 17% systemic lupus erythematosus, 7% juvenile dermatomyositis, 6% Kawasaki disease, 3% systemic sclerosis, and 2% Henoch–Schonlein purpura and mixed CTD patients each. Mucocutaneous features commonly seen were rash (40%), oral ulcers (22%), and photosensitivity (18%). Commonly seen systemic features were joint pain (77%), followed by fever (63%). Conclusion: Mucocutaneous manifestations are frequently seen in CTDs and constitute a frequent and an early manifestation of CTD. Early recognition of CTD in the pediatric age group will help in timely initiation of treatment to prevent disabilities and mortality.

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Clinical Characteristics and Course of Patients with Juvenile Dermatomyositis – Case Series

Cited by 3 publications

References 10 publications

Achieving Medication‐Free Remission in Patients With Juvenile Dermatomyositis

Achieving Medication‐Free Remission in Patients With Juvenile Dermatomyositis

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

Clinicoepidemiological Spectrum of Connective Tissue Diseases in the Pediatric Population in a Tertiary Care Center in North India: A Cross-sectional Observational Study

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