Abstract:

Interstitial lung disease is a rare complication of Juvenile Dermatomyositis (JDM). We present a five years male child of Asian origin with Bronchiolitis Obliterans Organizing Pneumonia (BOOP) associated with Dermatomyositis. Symptoms of progressive weakness of lower limbs, erythematous facial rash and breathing difficulty persisted for 18 months prior reference to our center. High resolution computed tomography (HR CT) chest was suggestive of BOOP but no definitive treatment was instituted at the local hospital. At presentation he had heliotrope rash, generalized finger and toe clubbing, tachypnea and bilateral proximal muscle weakness of lower limbs. Elevated muscle enzymes and muscle biopsy confirmed JDM. S. IgE was markedly elevated. Child is in clinical remission with oral prednisolone and methotrexate therapy. Repeat CT chest done 5 months after treatment suggests improvement. We believe that this is the youngest male child with JDM associated with BOOP in literature. Presence of facial rash, proximal muscle weakness, and elevated serum IgE in a child with respiratory symptoms may represent JDM with BOOP and warrant aggressive treatment for resolution of pulmonary lesions and improvement of muscle function.

RCIAPCON 2005 - Conference Abstracts.Pediatric Oncall [serial online] 2006 [cited 15 June 2006(Supplement 6)];3. Available from:
http://www.pediatriconcall.com/fordoctor/Conference_abstracts/
juvenile_dermatomyositis.asp