Treatment consensus for management of polymyositis and dermatomyositis among rheumatologists, neurologists and dermatologists

Abstract

Although rheumatologists, neurologists, and dermatologists see patients with polymyositis (PM) and dermatomyositis (DM), their management appears to vary depending on the physician's specialty. The aim of this study was to establish the treatment consensus among specialists of the three fields to standardize the patient care. We formed a research team supported by a grant from the Ministry of Health, Labor, and Welfare, Japan. Clinical questions (CQ) on the management of PM and DM were raised. A published work search on CQ was performed primarily using PubMed. Using the nominal group technique, qualified studies and results in the published work were evaluated and discussed to reach consensus recommendations. They were sent out to the Japan College of Rheumatology, Japanese Society of Neurology, and Japanese Dermatological Association for their approval. We reached a consensus in 23 CQ and made recommendations, and a decision tree for management was proposed. They were officially approved by the three scientific societies. In conclusion, a multidisciplinary treatment consensus for the management of PM and DM was established for the first time.

Disease burden and treatment sequence of polymyositis and dermatomyositis patients in Japan: a real-world evidence study

“…Overall, the patients took a median of 6 months to switch between treatments, suggesting that patients initiating treatment with steroids continued for a long time before switching, thus experiencing a burden (steroid myopathy affecting muscle recovery) due to steroid-related side effects. Thus, a high glucocorticoids (GC) dose is recommended to avoid myositis while keeping the treatment period as short as possible [ 8 ]. This burden could be the reason for the difference in the choice of treatment between two LoTs, which is also reported in previous studies that comorbidities do influence treatment choice and might also further complicate treatment operation for patients who switched multiple therapies in the past [ 26 ].…”

“…This burden could be the reason for the difference in the choice of treatment between two LoTs, which is also reported in previous studies that comorbidities do influence treatment choice and might also further complicate treatment operation for patients who switched multiple therapies in the past [ 26 ]. Other studies have also reported systemic corticosteroids as the most recommended initial therapeutic agent in PM/DM patients [ 8 ] while immunosuppressors being useful in inducing/maintaining remission/experiencing intolerable side effects with steroids in DM patients [ 33 , 34 ]. Few studies have reported cyclosporin A (CsA) administration resulting in 75% reduction in the GC dose [ 35 ] and muscle recovery in JDM patients [ 8 ].…”

“…Other studies have also reported systemic corticosteroids as the most recommended initial therapeutic agent in PM/DM patients [ 8 ] while immunosuppressors being useful in inducing/maintaining remission/experiencing intolerable side effects with steroids in DM patients [ 33 , 34 ]. Few studies have reported cyclosporin A (CsA) administration resulting in 75% reduction in the GC dose [ 35 ] and muscle recovery in JDM patients [ 8 ]. Our study established that switching within drug class was the most common action taken among patients and switches from the first to third LoT are mainly in between different concomitant therapies.…”

“…Majority of patients (69%) experienced PM/DM onset during middle age (40–60 years), with females being more susceptible [ 7 ]. Japanese Ministry of Health, Labor and Welfare (MHLW) has classified PM/DM as an intractable disease due its unknown etiology, lack of effective treatment, and poor prognosis [ 8 ].…”

Complete response to pembrolizumab in a patient with dermatomyositis and MMR deficient ovarian cancer: A case report

“…The treatment for primary DM consists of glucocorticoids and/or glucocorticoid-sparing regimens guided by a rheumatologist. Glucocorticoids are initiated at high doses until improvement of symptoms, which can take weeks, followed by a slow taper over 1 year ( Kohsaka et al, 2019 ). When DM fails to respond to glucocorticoids or individuals have contraindications to steroids, other options include methotrexate, cyclophosphamide, cyclosporin A, rituximab, and IVIG.…”