Characteristics and prognosis of microscopic polyangiitis with bronchiectasis.

BACKGROUND

Major pulmonary manifestations associated with microscopic polyangiitis (MPA) include diffuse alveolar hemorrhage (DAH) and interstitial pneumonia (IP).We previously showed bronchiectasis (BE) was one of the pulmonary complications of MPA. However, clinical features of BE patients with MPA are not fully understood. We investigated the characteristics and prognosis of BE patients with MPA.

METHODS

Forty-five MPA patients were retrospectively studied. The patients were divided into two groups: patients with BE and those without BE.

RESULTS

Thirty-one of 45 patients (69%) had pulmonary involvement including IP (23/45, 51%), BE (7/45, 16%), and DAH (5/45, 11%). There were no differences between the patients with BE versus those without with regard to clinical characteristics and initial treatments. However, the prognosis for patients with BE was better than those without BE during the first year after diagnosis, but it was worse between 1 and 5 years, which was statistically significant. Two BE patients died between 1 and 5 years as a result of pneumonia.

CONCLUSIONS

BE as a complication of MPA might be related to lower mortality in the acute phase and higher mortality in the chronic phase compared to other pulmonary manifestations. More attention to pulmonary infection is needed for patients with BE during the chronic phase.