1205 - Pulmonary Alveolar Proteinosis in an 8 Year-Old Female with Systemic Onset Juvenile Idiopathic Arthritis: A Case Report

Navin R. Pinto, Karen B. Onel, Charles Spencer, Oren J. Lakser. University of Chicago Comer Children's Hospital, Chicago, IL
Presentation Number: 1205

Abstract.
We are reporting an 8-year-old child with a previous diagnosis of systemic-onset juvenile idiopathic arthritis (SOJIA) who developed progressive shortness of breath, exercise intolerance and clubbing. She was found to have pulmonary alveolar proteinosis by thorascopic lung biopsy. The child showed some improvement with a reduction in her immunosuppressive medications.

Case Report
An 8-year-old girl with a 5 year history of severe SOJIA presented to her primary rheumatologist with a one-month history of progressively worsening shortness of breath, fever, weight loss, and new-onset clubbing. Pulse oximetry was found to be 92% on room air and a chest x-ray showed diffuse extensive airspace disease bilaterally. Her medications included: thalidomide 100 mg PO qday, dexamethasone 30 mg IV qweek, anakinra 100 mg SQ qday, cyclosporine 50 mg PO BID, prednisone 5 mg PO qday, and methotrexate 15 mg IV qweek. Blood, urine, stool, sputum and bronchoalveolar cultures were negative. BAL was unrevealing and high-resolution CT showed diffuse non-specific airspace disease. The decision was made to pursue a thorascopic lung biopsy. Multiple specimens were consistent with the diagnosis of pulmonary alveolar proteinosis (PAP). After diagnosis, the patient’s medications were reduced to include only prednisone, anakinra and cyclosporine. She was treated with IVIG and she was discharged home with nighttime supplemental oxygen.

Conclusion
Pulmonary alveolar proteinosis (PAP) is a rare lung disorder characterized by alveolar obstruction with surfactant phospholipids and proteinaceous debris. Three forms of this disease are recognized: primary (idiopathic); secondary (due to lung infections; hematologic malignancies; and immunodeficiency); and congenital (abnormal or absent surfactant-associated protein B. This is the first case attributed to immunomodulatory agents used to treat JIA. This case reinforces the need for intensive investigation of any new pulmonary symptoms in chronically ill children with SOJIA.

 N.R. Pinto, None.