loading page

Prolonged Remission Following Refractory Pulmonary Capillaritis in a Young Child
  • Jodi Gustave,
  • Abigail Strang
Jodi Gustave
Nemours/ AI duPont Hospital for Children
Author Profile
Abigail Strang
Nemours/Alfred I. duPont Hospital for Children
Author Profile

Abstract

Diffuse alveolar hemorrhage is a serious disorder where bleeding occurs throughout the alveoli that originates from the pulmonary microvasculature. Pulmonary capillaritis is a common cause of DAH but overall is extremely rare albeit potentially deadly in the pediatric population. PC is typically associated with systemic autoimmune disorders and treatment revolves around control of the cause. Isolated pulmonary capillaritis occurs without an underlying association and is extremely uncommon, particularly in children. Because of this, treatment guidelines do not exist but the urgency of treatment remains due to serious sequelae that may occur, including sudden death. Here, an interesting case is presented involving a toddler with diffuse alveolar hemorrhage due to ANCA-negative isolated pulmonary capillaritis with a strong family history of autoimmune disease. After multiple relapses she is currently in remission following a several year treatment regimen that included rituximab, pulse steroids, IVIG, azathioprine and oral corticosteroids. We would like to acknowledge to Dr. Timothy Vece for his assistance in the final treatment protocol for this patient.