title: Chronic respiratory disfunction due to diffuse alveolar hemorrhage in patients with systemic lupus erythematosus and primary vasculitis.
abstract: BACKGROUND
  Pulmonary hemorrhage (PH) occurs in 2-5% of SLE patients, and is associated with a high mortality rate (79-90%). Diagnostic criteria for this complication include: 1) Pulmonary infiltrates, with at least ¾ of lung tissue involved in a chest x ray, 2) Acute respiratory failure, 3) A decrease of 3g/dL or more in hemoglobin levels. PH might lead to organized pneumonia, collagen deposition, and pulmonary fibrosis which in time might cause changes in pulmonary function tests with either restrictive or obstructive patterns.
  
  
  OBJECTIVE
  To evaluate the existence of abnormalities in pulmonary function tests after a PH episode.
  
  
  METHODS
  We included patients with SLE and primary vasculitis that developed PH. During the acute episode, we measured SLEDAI in SLE patients, five factor score in microscopic polyangiitis (MPA) and Birmingham Vasculitis Activity Store (BVAS) in granulomatosis with polyangiitis (GPA) (Wegener). We determined the number of PH events, treatment, and ventilator assistance requirements and correlated its association with abnormal pulmonary function tests.
  
  
  RESULTS
  We included 10 patients, 7 with SLE, 2 with MPA and 1 with GPA (Wegener). The mean activity measures were: SLEDAI 20.4 ± 7.5, FFS 2, and BVAS 36. Treatment consisted in methylprednisolone (MPD) in 3 patients, MPD plus cyclophosphamide (CY) in 6 patients, and MPD, CY, IV immunoglobulin, and plasmapheresis in one patient. Five patients required ventilatory support. We found abnormalities in pulmonary function tests in 8 patients, three had an obstructive pattern and five a restrictive pattern; 2 patients did not show any change. We did not find a significant association with any of the studied variables.
  
  
  CONCLUSIONS
  PH might cause abnormalities in pulmonary function tests and prolonged immunosuppressive treatment could be required.
authors.affiliation: Departamento de Reumatología, Centro Médico Nacional Siglo XXI IMSS, México DF, México.
authors.forename: Eva
authors.initials: E
authors.lastname: Pérez Aceves
authors.forename: Mario
authors.initials: M
authors.lastname: Pérez Cristóbal
authors.forename: Gerardo A
authors.initials: GA
authors.lastname: Espinola Reyna
authors.forename: Raul
authors.initials: R
authors.lastname: Ariza Andraca
authors.forename: Daniel
authors.initials: D
authors.lastname: Xibille Fridmann
authors.forename: Leonor A
authors.initials: LA
authors.lastname: Barile Fabris
mesh_terms: D000293:Adolescent; D000328:Adult; D002908:Chronic Disease; D005260:Female; D006470:Hemorrhage / Q000209:etiology*; D006801:Humans; D008171:Lung Diseases / Q000175:diagnosis / Q000209:etiology*; D008180:Lupus Erythematosus, Systemic / Q000150:complications*; D008297:Male; D008875:Middle Aged; D010991:Plethysmography; D011650:Pulmonary Alveoli; D012120:Respiration Disorders / Q000209:etiology*; D012189:Retrospective Studies; D013147:Spirometry; D014657:Vasculitis / Q000150:complications*; D055815:Young Adult
keywords: Alveolar hemorrhage; Disfunción pulmonar; Hemorragia alveolar; Lupus; Pulmonary dysfunction; Vasculitis