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AB0078 Pulse methylprednisolone and cyclophosphamide therapy in three systemic lupus erythematosus patients with bronchiolitis obliterans organising pneumonia as the initial manifestation
  1. JC Tseng,
  2. HH Cheng,
  3. RJ Hu,
  4. LY Lu
  1. Allergy, Immunology and Rheumatology, Veterans General Hospital, Kaohsiung, Taiwan, R.O.C

Abstract

Background Diverse pleuropulmonary manifestations are common in patients with systemic lupus erythematosus (SLE). Bronchiolitis obliterans organising pneumonia (BOOP) is a pathologic entity characterised by the formation of fibrous plugs within terminal bronchioles and alveolar ducts. Although BOOP has been associated with several connective tissue disorders, there are rare reports of BOOP in patients with SLE as the initial manifestation.

Objectives We describe three SLE patients with open lung biopsy-proved BOOP as the initial manifestation.

Methods All three cases presented with acute/subacute chest symptoms of fever, cough and dyspnea. Chest radiographs and CT scan of lung revealed bilateral pulmonary infiltrates. BOOP was confirmed by open lung biopsy. Pulmonary vasculitis was also demonstrated in lung histopathology in one case. The pulmonary lesions and respiratory illnesses responded to pulse intravenous methylprednisolone and cyclophosphamide therapy.

SexAgeANAanti-ENAanti-dsDNAAnticardiolipin antibodyDuration of pulse cyclophosphamide therapy
Case 1 Female431:160, speckledSS-A, SS-BNegativePositive3 months
Case 2 Male561:320, homogeneousNegative69 IU/mlPositive3 months
Case 3 Male471:320, homogeneousNegative>400 IU/mlNegative2 months
  • Clinical and laboratory data.

  • Abstract AB0078 Table 1

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