Mortality in children with juvenile dermatomyositis: two decades of experience from a single tertiary care centre in North India

Abstract

Survival and outcomes have improved considerably among patients with juvenile dermatomyositis (JDM) in the west. However, mortality continues to be high in the developing world. There is paucity of literature on this aspect of JDM from developing countries. We reviewed case files of all patients with JDM registered in the Pediatric Rheumatology Clinic, Advanced Pediatrics Centre at the Post Graduate Institute of Medical Education and Research, Chandigarh, during the period 1993-2013. Seventy-six children were diagnosed to have inflammatory myopathy during this period. Of these, 63 had JDM, 3 had polymyositis while 10 had an overlap syndrome. We had reported 2 deaths out of 33 (8.3 %) patients with JDM in 2004, and over the last 9 years, we have encountered five more deaths in this group, thereby accounting for a mortality rate of 11.1 % (7/63) over two decades of follow-up. In these five children now being described, the mean duration between onset of symptoms and institution of appropriate therapy was 9.2 months. Four children (80 %) had severe muscle weakness needing nasogastric tubes at the onset, three (60 %) had cutaneous ulcers and three (60 %) had superadded infections. Two children (40 %) had gastrointestinal vasculitis and one of these developed an intestinal perforation. Three patients (60 %) had progressive pulmonary disease and air leak was identified in two of them. Although the prognosis for survival in JDM has steadily improved, in our experience the disease remains a serious illness and still carries significant mortality in the context of a developing country.

Profile of Pediatric Idiopathic Inflammatory Myopathies from a Tertiary Care Center of Eastern India

“…The common causes of death are progressive pulmonary disease, gastrointestinal problems (hemorrhage, perforation, vasculopathy), multisystem and idiopathic [7,29]. In spite of best efforts of the authors 2 of the JDM and 1 of the OM patients died (overall n = 3; 27%).…”

“…Higher age at diagnosis has been reported from other parts of India [5-7, 10, 11]. Most of the earlier Indian studies have shown a male predilection in the incidence of JDM [5][6][7][8][9][10]. The exact reason is unclear and it is difficult to comment regarding true epidemiological variation of sex ratio in different parts of India or a gender bias bringing more boys for the treatment than the girls [5].…”

“…The mean time from the onset of symptoms to diagnosis was 5.2 mo (range 1 mo to 18 mo) which is lower than other parts of India [5][6][7][8][9][10][11]. Early diagnosis is documented in western data due to prompt recognition and referral to the specialized center [13,16].…”

“…The scenario has changed over the recent times. The prognosis, in terms of mortality and morbidity has improved with the advent of modern therapy [7,30]. However, in developing countries the disease still carries a risk for significant mortality due to limited regional data, delay in diagnosis along with resource constrains [7].…”

“…Studies on IIM from India are scarce in literature and only a few studies have been published from various parts of India that include only JDM [5][6][7][8][9][10][11]. A scarcity of reported data on IIM from the eastern part of India prompted the authors to carry out this retrospective study in a tertiary referral center.…”