title: Antiphospholipid antibodies-associated diffuse alveolar hemorrhage.
abstract: OBJECTIVE
  To describe the clinical features and outcomes of 17 patients with primary antiphospholipid syndrome (PAPS) or antiphospholipid antibodies (aPL) and diffuse alveolar hemorrhage (DAH).
  
  
  METHODS
  We reviewed the medical records of all patients diagnosed with PAPS-associated DAH and aPL-associated DAH between January 1, 1997, and December 31, 2013, for clinical features, laboratory and radiographic findings, management, and outcomes.
  
  
  RESULTS
  A total of 17 patients met the criteria for DAH and had aPL and 10 patients met the criteria for PAPS. The mean age at DAH diagnosis was 57.6 years. Secondary causes of DAH were ruled out. Surgical lung biopsy was performed in 6 cases, 5 of whom had bland hemorrhage. Pulmonary capillaritis was present in only 1 case. Four patients (3 with aPLs and 1 with PAPS) achieved complete remission despite receiving no treatment. The majority of patients treated received initial corticosteroids. Additionally, cyclophosphamide (2 cases), rituximab (1 case), plasma exchange (2 cases), methotrexate (1 case), azathioprine (1 case), and hydroxychloroquine (2 cases) were used. In total, 10 patients (59%) achieved complete and sustained remission with a median length of follow-up of 48 months. Four patients (23%) died (2 with PAPS and 2 with aPLs), all from uncontrolled DAH. Three patients (18%) relapsed after achieving complete remission.
  
  
  CONCLUSIONS
  DAH is a rare complication of PAPS that can also arise de novo in aPL-positive individuals. Lung pathology shows either bland hemorrhage or capillaritis. Recognition of this unusual but known complication is important, since early diagnosis and therapy could potentially affect outcomes.
authors.affiliation: Department of Rheumatology, Marshfield Clinic, Marshfield, 1000 N Oak Ave, WI 54449. Electronic address: yachoui.ralph@marshfieldclinic.org.
authors.forename: Ralph
authors.initials: R
authors.lastname: Yachoui
authors.affiliation: Department of Rheumatology, Marshfield Clinic, Marshfield, 1000 N Oak Ave, WI 54449.
authors.forename: Rahul
authors.initials: R
authors.lastname: Sehgal
authors.affiliation: Department of Internal Medicine, Marshfield Clinic, Marshfield, WI.
authors.forename: Barkha
authors.initials: B
authors.lastname: Amlani
authors.affiliation: Department of Rheumatology, Marshfield Clinic, Marshfield, 1000 N Oak Ave, WI 54449.
authors.forename: Jerry W
authors.initials: JW
authors.lastname: Goldberg
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000368:Aged; D017152:Antibodies, Antiphospholipid / Q000276:immunology*; D016736:Antiphospholipid Syndrome / Q000150:complications / Q000276:immunology* / Q000628:therapy; D001379:Azathioprine / Q000627:therapeutic use; D001706:Biopsy; D015331:Cohort Studies; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D006470:Hemorrhage / Q000209:etiology / Q000276:immunology* / Q000473:pathology / Q000628:therapy; D006801:Humans; D006886:Hydroxychloroquine / Q000627:therapeutic use; D008168:Lung / Q000473:pathology; D008171:Lung Diseases / Q000209:etiology / Q000276:immunology* / Q000473:pathology / Q000628:therapy; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use; D008875:Middle Aged; D010951:Plasma Exchange; D012189:Retrospective Studies; D000069283:Rituximab / Q000627:therapeutic use
chemical_list: D000305:Adrenal Cortex Hormones; D017152:Antibodies, Antiphospholipid; D000069283:Rituximab; D006886:Hydroxychloroquine; D003520:Cyclophosphamide; D001379:Azathioprine; D008727:Methotrexate
keywords: Antiphospholipid antibodies; Diffuse alveolar hemorrhage; Primary antiphospholipid syndrome