title: Diffuse Alveolar Hemorrhage as a Manifestation of Childhood-Onset Systemic Lupus Erythematosus.
abstract: BACKGROUND
  Diffuse alveolar hemorrhage (DAH) is a devastating clinical syndrome characterized by a falling hematocrit, respiratory insufficiency, and radiographic evidence of pulmonary infiltrates. Literature regarding management of DAH in childhood-onset SLE (cSLE) is limited.
  
  
  METHODS
  We reviewed the presentation, management, and outcome of DAH in a pediatric tertiary medical center with one of the largest cSLE cohorts in North America. During a 10 year period 7 of 410 children with cSLE had DAH.
  
  
  RESULTS
  The majority of cSLE patients with DAH were male (71%) and Hispanic (57%). The median age at the time of DAH diagnosis was 14 years (range 3 -15 years). DAH was the presenting manifestation of cSLE in 29% of children; 71% presented with DAH within 3 months of their diagnosis. All patients had cough, 86% had dyspnea, and 29% had hemoptysis. All patients had anemia and 71% had thrombocytopenia. Eighty-six percent had hematuria/proteinuria, and a positive anti-double stranded DNA antibody. Chest imaging showed diffuse ground glass opacities in all events. All patients developed respiratory insufficiency (29% supplemental oxygenation and 71% mechanical ventilation). Transfusions were required in 57% of cases. All patients received corticosteroids and additional immunomodulation to achieve disease control. Eighty-six percent of our DAH/cSLE cohort survived their initial event (median follow-up 2.5 years). No survivor required supplemental oxygen or had a DAH recurrence.
  
  
  CONCLUSIONS
  SLE should be in the hospitalist's differential diagnosis for any child with respiratory insufficiency, cytopenias, and/or urinary abnormalities. Once cSLE is identified, initiation of aggressive immune suppression with multiple agents may enhance outcomes.
authors.affiliation: Division of Allergy, Immunology, and Rheumatology, Texas Children's Hospital, Houston, Texas; Department of Pediatrics, Baylor College of Medicine, Houston, Texas; and emuscal@bcm.edu.
authors.forename: Saimun
authors.initials: S
authors.lastname: Singla
authors.affiliation: Division of Allergy, Immunology, and Rheumatology, Texas Children's Hospital, Houston, Texas; Department of Pediatrics, Baylor College of Medicine, Houston, Texas; and.
authors.forename: Debra L
authors.initials: DL
authors.lastname: Canter
authors.affiliation: Department of Pediatrics, Baylor College of Medicine, Houston, Texas; and Division of Pulmonology, Texas Children's Hospital, Houston, Texas.
authors.forename: Timothy J
authors.initials: TJ
authors.lastname: Vece
authors.affiliation: Division of Allergy, Immunology, and Rheumatology, Texas Children's Hospital, Houston, Texas; Department of Pediatrics, Baylor College of Medicine, Houston, Texas; and.
authors.forename: Eyal
authors.initials: E
authors.lastname: Muscal
authors.affiliation: Division of Allergy, Immunology, and Rheumatology, Texas Children's Hospital, Houston, Texas; Department of Pediatrics, Baylor College of Medicine, Houston, Texas; and.
authors.forename: Marietta
authors.initials: M
authors.lastname: DeGuzman
mesh_terms: D000293:Adolescent; D000359:Aftercare / Q000379:methods / Q000706:statistics & numerical data; D017668:Age of Onset; D001803:Blood Transfusion / Q000379:methods; D005260:Female; D006470:Hemorrhage / Q000175:diagnosis / Q000208:ethnology / Q000503:physiopathology / Q000628:therapy; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use*; D007159:Immunologic Tests / Q000706:statistics & numerical data; D008171:Lung Diseases / Q000175:diagnosis / Q000208:ethnology / Q000503:physiopathology / Q000628:therapy; D008180:Lupus Erythematosus, Systemic / Q000175:diagnosis / Q000453:epidemiology / Q000503:physiopathology; D008297:Male; D008501:Medical Records, Problem-Oriented; D013902:Radiography, Thoracic / Q000379:methods / Q000706:statistics & numerical data; D063189:Symptom Assessment / Q000706:statistics & numerical data; D014481:United States / Q000453:epidemiology
chemical_list: D007155:Immunologic Factors