title: Primary antiphospholipid syndrome-associated diffuse alveolar hemorrhage.
abstract: OBJECTIVE
  Diffuse alveolar hemorrhage (DAH) is an uncommon complication of primary antiphospholipid syndrome (APS). We aimed to describe the clinical characteristics, treatment, and outcomes of primary APS-associated DAH in a single center.
  
  
  METHODS
  We conducted a retrospective review of all adults with primary APS-associated DAH evaluated at Mayo Clinic over a 15-year period. DAH was defined as bilateral pulmonary infiltrates and bronchoalveolar lavage (BAL) fluid documenting progressively bloody returns and/or the presence of >20% hemosiderin-laden macrophages. Patients with other causes of DAH were excluded.
  
  
  RESULTS
  Eighteen patients were identified (median age 43 years). Capillaritis was present in surgical lung biopsy samples of 3 patients. BAL differential cell counts revealed predominantly neutrophils. All patients were treated initially with glucocorticoids. Cyclophosphamide (CYC) was used in 8 patients; complete remission was achieved in 3 patients treated with CYC alone and in 1 patient receiving combination therapy with rituximab (RTX). RTX was used in 9 patients; 2 patients achieved remission with RTX alone, whereas 3 patients required combination therapy with CYC or mycophenolate mofetil (MMF). No patient achieved complete remission while receiving single therapy with MMF, azathioprine, or plasma exchange. Intravenous gamma globulin therapy was administered in 5 patients; no patient achieved control of the disease. Six patients died, all because of complications related to uncontrolled DAH or its therapy.
  
  
  CONCLUSIONS
  We present the largest case series of primary APS-associated DAH reported in the literature. DAH carries a very poor prognosis and therapeutic options are limited. Immunosuppression with either CYC or RTX is associated with the highest likelihood of remission induction and should be considered early.
authors: Mayo Clinic, Rochester, Minnesota.
authors: Rodrigo
authors: R
authors: Cartin-Ceba
authors: Tobias
authors: T
authors: Peikert
authors: Aneel
authors: A
authors: Ashrani
authors: Karina
authors: K
authors: Keogh
authors: Mark E
authors: ME
authors: Wylam
authors: Steven
authors: S
authors: Ytterberg
authors: Ulrich
authors: U
authors: Specks
mesh_terms: D000328:Adult; D016736:Antiphospholipid Syndrome / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy / Q000401:mortality; D001706:Biopsy; D001992:Bronchoalveolar Lavage Fluid / Q000166:cytology; D002196:Capillaries / Q000473:pathology; D018450:Disease Progression; D004359:Drug Therapy, Combination; D005260:Female; D006470:Hemorrhage / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology* / Q000401:mortality / Q000628:therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008171:Lung Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology* / Q000401:mortality; D008297:Male; D008875:Middle Aged; D008910:Minnesota; D011650:Pulmonary Alveoli / Q000098:blood supply*; D012074:Remission Induction; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents