P0442 - 2-Year-Old Male with Fever, an Urticarial Rash, and Tachypnea

Systemic onset juvenile idiopathic arthritis (SoJIA) is a complex inflammatory disease of childhood associated with significant morbidity. SoJIA has a variety of presentations and can often be difficult to diagnose. Other than serositis, pulmonary manifestations of SoJIA are rare though have been increasingly detected in the last decade. This case report describes pulmonary findings as the initial presentation of SoJIA.

A 2-year-old male with alpha thalassemia presented with fever, cough and increased work of breathing. Chest radiograph was significant for a lower lobe airspace opacity, complicated by pleural effusion on subsequent imaging. The patient was placed on a series of escalating antibiotic therapies for presumed complicated bacterial pneumonia. Due to lack of improvement, persistence of fevers, and intermittent rash, the patient was started on intravenous immunoglobulin and aspirin for incomplete Kawasaki disease. The patient had an acute decline in his respiratory status and was found to have pericardial effusion concerning for tamponade physiology on echocardiography, prompting emergent pericardiocentesis. SoJIA was suspected and systemic steroids were initiated with improvement in his clinical status. The patient developed arthritis of the wrist a few weeks after discharge from the hospital, leading to a diagnosis of SoJIA.

To the best of our knowledge, this is the first case of a patient presenting with lung disease as the initial presentation of SoJIA. Rheumatologic causes should be considered for patients with persistent fever and elevated inflammatory markers. It is important to recognize that patients with SoJIA may also develop lung disease and present with signs of respiratory distress.