[Secondary bronchiolitis obliterans organizing pneumonia with primary Sjögren's syndrome, resulting in acute respiratory distress syndrome: a case report]

Hiroaki Mitsushima, Kaneo Kawazoe, Atsushi Takahashi, Kazunori Oishi, Tsuyoshi Nagatake
Nihon Kokyūki Gakkai Zasshi, the Journal of the Japanese Respiratory Society 2004, 42 (3): 261-5
A 71-year-old female who was taking 10 mg/day of prednisolone for Sjögren's syndrome was admitted because of fever and dyspnea with multiple infiltrative shadows on chest radiography and computed tomography (CT), although she had been discharged only 4 days before. On the 1st and 2nd admissions, a BOOP pattern had been suspected, and she was treated by tapering the prednisolone dose from 40 mg/day to 10 mg/day, which resulted in the disappearance of the infiltrative lung shadows. This time we confirmed the BOOP pattern with Sjögren's syndrome, because bronchoalveolar lavage showed an increase of total cells, with a high lymphocyte fraction, and a transbronchial lung biopsy revealed loose fibroblastic plugs in some alveolar ducts and alveoli. Also, there were intra-alveolar accumulations of foamy macrophages. Furthermore, we noticed migration of pulmonary opacity. Although the clinical symptoms of the patient improved, the response to the prednisolone therapy appeared to be poor. At 35 mg of prednisolone (which had been initiated at 40 mg/day), the disease became rapidly exacerbated by a common cold, and developed into ARDS on the 30th hospital day. In spite of intensive care, the patient died. Here we report a rare case in which the BOOP pattern based on Sjögren's syndrome resulted in ARDS. In general, prednisolone is effective against the BOOP pattern, but we need to be aware of the possibility of a poor response to this BOOP pattern in Sjögren's syndrome.

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