DISCUSSION
COPA syndrome is a rare mutation of the coatomer associated protein subunit alpha gene (COPα) causing adaptive immune dysregulation. The cases in the literature describe ILD, pulmonary hemorrhage, arthritis, and nephropathy. This autosomal dominant entity is likely presenting as a de novo mutation in this young child as no other family members are affected. The initial lack of pulmonary hemorrhage and arthritis, as well as her age represent a novel presentation of the disease and speak to the important contribution genetic testing has made in the diagnosis of ILD. Current literature describes molecular mechanisms of the syndrome however due to twenty-one case reports described,5, 7,8 clinical progression and surveillance methods are not yet described. Inflammatory markers such as ESR, C-reactive protein, complete blood count, ANA, ANCA, along with imaging and bronchoscopy with lavage cell counts have been useful markers of disease activity in this patient. However additional research is needed to better define disease markers, treatment options, and long-term prognosis.