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.