In the Journals

Calcinosis linked to greater disease severity, duration in juvenile dermatomyositis

Amir B. Orandi

Among patients with juvenile dermatomyositis, calcinosis was associated with longer active disease duration, disease severity and clinical features such as lipodystrophy and joint contractures, according to data published in Pediatric Rheumatology.

“This study is important because calcinosis is a very poorly understand complication of juvenile dermatomyositis,” Amir B. Orandi, MD, from the division of pediatric rheumatology at the Mayo Clinic, told Healio Rheumatology. “Our prior work has shown that many pediatric rheumatologists have only rarely treated calcinosis in juvenile dermatomyositis. As the overall treatment and survival has dramatically improved, calcinosis is becoming a serious cause of morbidity. More knowledge is needed about which patients are at risk — and why — so that potential interventions can be made to lessen the morbidity.”

To better understand the links between patients with juvenile dermatomyositis and calcinosis, Orandi and colleagues studied data from a multicenter registry for pediatric rheumatologic diseases, developed by the Childhood Arthritis and Rheumatology Research Alliance (CARRA). The registry includes 654 patients with juvenile dermatomyositis, enrolled from May 30, 2010, through Oct. 31, 2014 from 55 CARRA centers. Patients were younger than 21 years at enrollment, and had been diagnosed prior to age 18 years.

 
Among patients with juvenile dermatomyositis, calcinosis was associated with longer active disease duration and disease severity, according to data.
Source: Shutterstock

After excluding 23 patients for lacking information on calcinosis, the researchers included 631 in their final analysis. All patient data were kept in a centralized database by CARRA. Following study approval, the information was transmitted as a coded, deidentified dataset to the study team. Orandi and colleagues used univariate and multivariate logistic regression to compare demographic characteristics, clinical disease features and treatment histories of those with and without calcinosis.

According to the researchers, 13% of the 631 included patients had a current or prior history of calcinosis. These patients were statistically more likely to have longer disease duration before diagnosis and treatment. They were also more likely to demonstrate lipodystrophy and joint contractures, and to have been treated with IV immune globulin or rituximab (Rituxan, Genentech).

“Prolonged disease activity in juvenile dermatomyositis is associated with many adverse effects and efforts to institute prompt and effective treatment are paramount,” Orandi said in an interview. “Patients with select clinical features, including presentation, should be carefully monitored for development of calcinosis. At the time this data was collected, less than half of patients received a biologic medication and the vast majority who did, received [intravenous immunoglobulin]. More investigation of newer biologic drugs and earlier use of those drugs may improve outcomes.” – by Jason Laday

Disclosure: Orandi reports no relevant financial disclosures. Please see the study for all other authors’ relevant financial disclosures.

Amir B. Orandi

Among patients with juvenile dermatomyositis, calcinosis was associated with longer active disease duration, disease severity and clinical features such as lipodystrophy and joint contractures, according to data published in Pediatric Rheumatology.

“This study is important because calcinosis is a very poorly understand complication of juvenile dermatomyositis,” Amir B. Orandi, MD, from the division of pediatric rheumatology at the Mayo Clinic, told Healio Rheumatology. “Our prior work has shown that many pediatric rheumatologists have only rarely treated calcinosis in juvenile dermatomyositis. As the overall treatment and survival has dramatically improved, calcinosis is becoming a serious cause of morbidity. More knowledge is needed about which patients are at risk — and why — so that potential interventions can be made to lessen the morbidity.”

To better understand the links between patients with juvenile dermatomyositis and calcinosis, Orandi and colleagues studied data from a multicenter registry for pediatric rheumatologic diseases, developed by the Childhood Arthritis and Rheumatology Research Alliance (CARRA). The registry includes 654 patients with juvenile dermatomyositis, enrolled from May 30, 2010, through Oct. 31, 2014 from 55 CARRA centers. Patients were younger than 21 years at enrollment, and had been diagnosed prior to age 18 years.

 
Among patients with juvenile dermatomyositis, calcinosis was associated with longer active disease duration and disease severity, according to data.
Source: Shutterstock

After excluding 23 patients for lacking information on calcinosis, the researchers included 631 in their final analysis. All patient data were kept in a centralized database by CARRA. Following study approval, the information was transmitted as a coded, deidentified dataset to the study team. Orandi and colleagues used univariate and multivariate logistic regression to compare demographic characteristics, clinical disease features and treatment histories of those with and without calcinosis.

According to the researchers, 13% of the 631 included patients had a current or prior history of calcinosis. These patients were statistically more likely to have longer disease duration before diagnosis and treatment. They were also more likely to demonstrate lipodystrophy and joint contractures, and to have been treated with IV immune globulin or rituximab (Rituxan, Genentech).

“Prolonged disease activity in juvenile dermatomyositis is associated with many adverse effects and efforts to institute prompt and effective treatment are paramount,” Orandi said in an interview. “Patients with select clinical features, including presentation, should be carefully monitored for development of calcinosis. At the time this data was collected, less than half of patients received a biologic medication and the vast majority who did, received [intravenous immunoglobulin]. More investigation of newer biologic drugs and earlier use of those drugs may improve outcomes.” – by Jason Laday

Disclosure: Orandi reports no relevant financial disclosures. Please see the study for all other authors’ relevant financial disclosures.