National registry of patients with juvenile idiopathic inflammatory myopathies in Hungary—Clinical characteristics and disease course of 44 patients with juvenile dermatomyositis

Abstract

Idiopathic inflammatory myopathies (IIMs) are systemic autoimmune diseases characterized by chronic muscle inflammation resulting in progressive weakness and frequent involvement of internal organs, mainly the pulmonary, gastrointestinal and cardiac systems which considerably contribute to the morbidity and mortality of the IIMs. Aim of this study was to present clinical characteristics, disease course, frequency of relapses and survival in patients with juvenile dermatomyositis (DM). A national registry of patients with juvenile IIMs was elaborated by the authors in Hungary. We have summarized data of the register according to signs and symptoms, disease course, frequency of relapses and survival of patients with juvenile IIM. Analysis was performed using data of 44 patients with juvenile DM diagnosed between 1976 and 2004 according to Bohan and Peter's criteria. Survival probability was calculated by Kaplan-Meier method. Data of patients with juvenile DM were compared with data of 66 patients with adult DM. The most frequent cutaneous features were facial erythema and heliotrope rash. Extramuscular and extraskeletal manifestations of the disease were more frequent in adult patients. The most common extramuscular feature was arthralgia in both groups of patients with juvenile or adult DM. Cardiac manifestation of the disease was not observed in juvenile patients. Respiratory muscle involvement and interstitial lung disease (ILD) were more frequent among adult DM patients than cardiac manifestation of the myositis. In view of the disease course, the authors found that frequency of polycyclic and monophasic subtypes of the disease were mainly similar. The hazard of relapse was found higher during the first year after the remission. None of the juvenile patients died. Among adult patients four disease-specific deaths occurred. There was no correlation between relapse free survival and initial therapeutic regimen. Many of our patients had polycyclic or chronic disease. As relapses can occur after a prolonged disease-free interval, patients should be followed up for at least 2 years. Although we found favourable survival probability, further investigations are needed to assess functional outcome.

Pulmonary outcome in juvenile dermatomyositis: a case-control study

“…Serious or fatal pulmonary complications in JDM have been described in case reports 4 5. Also, pulmonary involvement in JDM has been described in studies not primarily addressing this aspect; asymptomatic significant restrictive impairment being found in 14/17 patients6 and ILD in 7% to 19% 7–9…”

Interstitial Lung Disease in Children Older Than 2 Years

“…[20][21][22] Previously, the most commonly described ILD found in dermatomyositis/polymyositis was usual interstitial pneumonia. 23 However, as in systemic sclerosis, recent reviews now have found that the most commonly described pathologic fi nding is nonspecifi c interstitial pneumonia.…”

The treatment and prognosis of dermatomyositis: An updated review

“…One-year survival has varied from 83% to 95%, whereas 5-year survival has ranged from 63% to 95%, with 9-year or greater survival rates ranging from 53% to 100%, depending on the disease subtype. 24,26,34,35,43,151,152 All of these survivals are better than the 50% mortality for DM that Bohan and Peter 4 cited as the natural disease course before corticosteroid therapy.…”

“…Today, these are much lower, as newer studies found mortality at 1.5% 39 and even 0%. 34 DM has a bimodal age distribution, with patients with JDM not having a cancer association, and adults having associated malignancy at variable rates. One series of 120 patients with JDM found no cancers.…”

“…Relapse rates, however, have varied from study to study with monophasic disease (patients with only one flare of myositis and no relapses) varying from 8% to 51.4%. 25,27,[33][34][35] In JDM, monophasic disease has ranged from 25% to 59.1%, 34,[36][37][38][39] and these patients use corticosteroids for shorter times, 32,36,37,[40][41][42] with one study finding corticosteroid treatment necessary for an average of only 14 months. 42 Another study 36 suggested that low-dose maintenance therapy does little to prevent relapse and may be harmful because of the side effects of corticosteroids.…”