Tumor necrosis factor inhibitor (anti-TNF) agents may be a viable therapeutic option for juvenile dermatomyositis (JDM) patients previously unresponsive to existing treatments, according to the results of a study.
Researchers evaluated 66 patients with JDM, recruited from the UK JDM Cohort and Biomarker Study, who met the Bohan-Peter criteria and were actively treated with anti-TNF agents for at least three months. The study was presented at the European League Against Rheumatism Annual Congress (EULAR) in London on June 10, 2016
As part of the investigation, the Childhood Myositis Assessment Scale (CMAS), Manual Muscle Testing (MMT8), muscle enzymes and Physicians Global Assessment (PGA) were recorded. In addition, skin disease was assessed using modified skin Disease Activity Score (DAS), the authors noted.
Of the 66 patients included in the investigation, the authors noted that 41 patients (62%) were female. At enrollment, the mean age of the patients was 16.8+5.6 years old, with an average disease duration of 9.6+4.6 years.
The overall mean duration at the beginning of anti-TNF treatment was 3.49+2.80 years and the average duration of anti-TNF therapy was 2.76+2.09 years.
Muscle, skin improvement
Findings showed improvements in muscle and skin involvement, as well as in overall disease activity, the authors noted.
Data showed significant changes in the median values of two standard muscle measurements including CMAS and MMMT (p<0.0001 and p=0.0097 respectively). In addition, there were also significant improvements in skin involvement assessed using the modified skin DAS (p<0.0001). The PGA also improved significantly (p<0.0001).
Reported data also revealed that 16 of the JDM patients were switched from anti-TNF treatment, for reasons including therapy failure, adverse events and patient preference.
Of 21 adverse reactions registered, seven were considered severe (anaphylactic reactions on infliximab infusion). Three-quarters of the mild to moderate adverse reactions were due to infection; four of the patients were switched to another TNF antagonist, while in the remaining patients, temporarily withholding the drug proved sufficient. No cases of TB were registered.
“High levels of the cell signalling protein TNF have been reported in JDM patients with a long disease course, suggesting this immune cell regulator may play a significant role in refractory disease,” said Dr. Raquel Campanilho-Marques, lead author of the study who was quoted in a press release.
“There are no published clinical trials (only case reports) of this therapy, but some are in progress. Our study is one of the largest to explore the efficacy and safety of anti-TNF therapy in a large independent cohort of JDM patients,” explained Dr. Campanilho-Marques of the Institute of Child Health, University College London.