Interstitial pneumonitis and air leakage in juvenile dermatomyositis

Fatal acute fibrinous and organizing pneumonia in a child with juvenile dermatomyositis

“…2,3 Several case reports describe the occurrence of interstitial pneumonia (IP) and/or pneumothorax in JDM. [4][5][6] The IP in these cases was not well defined, and pneumothorax often dominated the presentation, sometimes with acute respiratory failure. In 1975, Park and Nyhan 7 described a 12-year-old boy who had respiratory symptoms 4 months after the onset of JDM.…”

The Role of Microvascular Injury in the Evolution of Idiopathic Pulmonary Fibrosis

“…[36][37][38][39][40] Patients with CTD in whom fibrosing pneumonitis develops characteristically have anti-ENA antibodies, especially anti-Ro/SSA or anti-RNP and/or one of the myositis-associated antibodies such as anti-Jo-1. [41][42][43][44][45] The basis of the microangiopathy has been postulated to reflect an anti-EC antibody with C5b-9 as the effector mechanism. It is suggested that endothelia demonstrate up-regulated ENA expression owing to the displacement of nuclear or cytoplasmic ENA to the cell surface, 30 a classic trigger being accelerated apoptosis.…”

Juvenile amyopathic dermatomyositis complicated by progressive interstitial pneumonia

“…Pulmonary involvement is rarely reported in juvenile DM. Furthermore, spontaneous air leakage (pneumothorax, pneumomediastinum, and subcutaneous emphysema) has been reported rarely in both adult and pediatric patients with DM 3,13 . The exact mechanism responsible for the development of air leakage in DM is unknown 13 .…”