TY - CHAP M1 - Book, Section TI - Pulmonary Vasculitis Syndromes A1 - Papadopoulou, Charalampia A1 - Eleftheriou, Despina A2 - Kline, Mark W. Y1 - 2018 N1 - T2 - Rudolph's Pediatrics, 23e AB - Pulmonary vasculitis syndromes are a heterogeneous group of primary or secondary diseases. They are characterized by inflammation in the pulmonary vessels that may lead to progressive destruction of the pulmonary microvasculature. Primary or idiopathic pulmonary vasculitis is extremely rare in children; in most cases, pulmonary vasculitis is seen in the context of a systemic vasculitis syndrome. The systemic inflammatory diseases with the highest likelihood of pulmonary involvement are juvenile systemic lupus erythematosus, systemic sclerosis, juvenile dermatomyositis, and mixed connective tissue disease. The primary systemic vasculitides most commonly associated with pulmonary involvement are granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, and microscopic polyangiitis (MPA). In addition, a new autoinflammatory disease has been described recently that is associated with gain-of-function mutations in the TMEM173 gene encoding the stimulator of interferon genes (STING) referred to as STING-associated vasculopathy with onset in infancy (SAVI). SAVI is characterized by severe pulmonary vasculitic involvement. This chapter reviews the diseases associated with pulmonary involvement and discusses common clinical presentation features, diagnostic workup, and recommended therapies. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/29 UR - accesspediatrics.mhmedical.com/content.aspx?aid=1182917626 ER -