Disease characteristics and clinical outcomes of adults and children with anti-MDA-5 antibody-associated myositis: a prospective observational bicentric study

Dunga, Saikumar; Kavadichanda, Chengappa; Gupta, Latika; Naveen, R; Agarwal, Vikas; Negi, Vir Singh

doi:10.1007/s00296-021-04897-1

Cited by 13 publications

(14 citation statements)

References 51 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…In our cohort, cutaneous ulcers were the most common skin manifestation and were seen in 61% consistent with the prevalence of 40–80% reported in literature. 6 , 11 , 14 , 15 , 16 These ulcers were seen on typical locations over knuckles and elbows as described in various MDA5 DM cohorts. However, the typically reported classical DM rashes such as Gottron’s papules, heliotrope rash, Gottron’s sign were seen in only 50% of our cohort compared to 68% reported by Dunga et al 6 We also report the presence of digital pits, digital gangrene, and vasculitic rashes.…”

Section: Discussionmentioning

confidence: 62%

“… 6 , 11 , 14 , 15 , 16 These ulcers were seen on typical locations over knuckles and elbows as described in various MDA5 DM cohorts. However, the typically reported classical DM rashes such as Gottron’s papules, heliotrope rash, Gottron’s sign were seen in only 50% of our cohort compared to 68% reported by Dunga et al 6 We also report the presence of digital pits, digital gangrene, and vasculitic rashes. These do not find a mention in previous reports from India.…”

Section: Discussionmentioning

confidence: 62%

“… 3 However in our cohort of anti-MDA5 DM we report a prevalence of 44% for myositis, very early after the first symptom of the disease which is almost similar to the reported prevalence of 56% from another Indian cohort of MDA5 DM. 6 …”

Section: Discussionmentioning

confidence: 99%

“… 4 The information on MDA5 DM from India is scarce. 5 , 6 This study informs regarding the clinical profile and outcomes in this disease from a tertiary care University teaching hospital in India.…”

Section: Introductionmentioning

confidence: 92%

See 3 more Smart Citations

Fever, Cutaneous Ulcers, and Arthritis Define the MDA5 Phenotype in Indian Patients with Idiopathic Inflammatory Myositis

Devarasetti¹,

Irfan²,

Desai³

et al. 2022

MJR

View full text Add to dashboard Cite

Background: In idiopathic inflammatory myositis (IIM), anti-MDA5 (melanoma differentiation-associated gene 5) antibody-associated DM (MDA5 DM) is a distinct subset characterised by presentation with cutaneous involvement, association with ILD and delayed onset of muscle involvement which is believed to be not severe. Objectives: To study the clinical profile and treatment outcomes of MDA5 DM patients. Methods: Records of patients fulfilling the ACR/EULAR classification criteria for IIM and testing positive for MDA5 antibody, were retrieved from the ongoingMyoIN registry database of our centre. Clinical, laboratory and treatment data were analysed. Follow-up details were noted. Parameters were compared between survivors and non-survivors using student’s t-test or Mann-Whitney U tests for continuous variables and Chi square test and Fisher’s exact for categorical variables. Results: Eighteen patients (5 juvenile) were identified. Median age was 30(16–41) years and disease duration at time of diagnosis was 5.5 months with median follow up duration of 18 months (12–31). The prevalence of constitutional symptoms, cutaneous involvement, arthritis, and myositis were 94%, 100%, 78%, and 44% respectively. Ulcers were the most common cutaneous finding. Myositis when present was severe. Interstitial lung disease (ILD) was present in 6 patients which was rapidly progressive (RPILD) in 3. Anti-Ro-52 antibody (n=5) was the most common myositis associated antibody (MAA). There was no difference in the clinical profile of children and adults. Seven patients succumbed. RPILD was a predictor of mortality (p=0.04). Cutaneous relapses were common among survivors but responded well to further therapy. Conclusion: The MDA5 DM phenotype in this Indian cohort is characterised by fever, cutaneous ulcers and arthritis. RPILD was not uncommon and predicted mortality.

show abstract

Section: Discussionmentioning

confidence: 62%

Section: Discussionmentioning

confidence: 62%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 92%

See 2 more Smart Citations

Fever, Cutaneous Ulcers, and Arthritis Define the MDA5 Phenotype in Indian Patients with Idiopathic Inflammatory Myositis

Devarasetti¹,

Irfan²,

Desai³

et al. 2022

MJR

View full text Add to dashboard Cite

show abstract

“…Among the different subtypes of DM, the prevalence of anti-MDA5 DM ranges from 7-60%, with a higher prevalence in Asians (11-60%) than in the Caucasian (7-16%) population [ 4 ]. The outcomes also depend on ethnicity, with poorer outcomes in Japanese but excellent outcomes in the Caucasian population [ 5 ]. Our patient was dually positive for anti-MDA5 and anti-SSA 52 kD antibodies, which has an aggressive clinical course and poorer outcomes.…”

Section: Discussionmentioning

confidence: 99%

Dermatomyositis With Dual Positive Anti-melanoma Differentiation-Associated Gene 5 and Anti-Sjogren Syndrome-Related Antigen 52 kD Antibodies: A Case Report and Literature Review on Clinical Characteristics

Parthiban¹,

Arivazhagan²,

Anaji³

et al. 2023

Cureus

View full text Add to dashboard Cite

Anti-melanoma differentiation-associated gene 5 (MDA 5) is one of the subtypes of dermatomyositis associated with rapidly progressive lung disease. MDA 5 carries a high mortality risk due to respiratory failure. The exact pathophysiology is unclear, but it is linked to genetic predisposition and viral triggers with the associated innate response and cytokine production like interleukins IL-1,6,18, tumor necrosis factor-alpha, and interferons. It is usually treated with anti-cytokines, high-dose steroids, immunosuppressants, and plasma exchange. Due to the atypical presentation and rapidity of the disease course, the diagnosis is often delayed. We report a 39-year-old female presenting with rapidly progressive lung disease secondary to an aggressive form of dermatomyositis.

show abstract

The prevalence and clinical characteristics of anti-HMGCR (anti-3-hydroxy-3-methyl-glutaryl-coenzyme A reductase) antibodies in idiopathic inflammatory myopathy: an analysis from the MyoCite registry

et al. 2022

View full text Add to dashboard Cite

Disease characteristics and clinical outcomes of adults and children with anti-MDA-5 antibody-associated myositis: a prospective observational bicentric study

Cited by 13 publications

References 51 publications

Fever, Cutaneous Ulcers, and Arthritis Define the MDA5 Phenotype in Indian Patients with Idiopathic Inflammatory Myositis

Fever, Cutaneous Ulcers, and Arthritis Define the MDA5 Phenotype in Indian Patients with Idiopathic Inflammatory Myositis

Dermatomyositis With Dual Positive Anti-melanoma Differentiation-Associated Gene 5 and Anti-Sjogren Syndrome-Related Antigen 52 kD Antibodies: A Case Report and Literature Review on Clinical Characteristics

The prevalence and clinical characteristics of anti-HMGCR (anti-3-hydroxy-3-methyl-glutaryl-coenzyme A reductase) antibodies in idiopathic inflammatory myopathy: an analysis from the MyoCite registry

Contact Info

Product

Resources

About