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 9years, 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 9.2months. 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.