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Published on: June 16, 2020
Characteristics and prognosis of microscopic polyangiitis with bronchiectasis
Hiroki Tashiro1, Koichiro Takahashi1, Masahide Tanaka1
1Division of Hematology, Respiratory Medicine and Oncology, Faculty of Medicine, Saga University, Saga, Japan.
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.
Insights
Microscopic polyangiitis (MPA) patients with bronchiectasis (BE) have better short-term survival but worse long-term outcomes. Pulmonary infections require increased vigilance in chronic phases for these patients.
Area of Science:
- Pulmonary Medicine
- Rheumatology
- Internal Medicine
Background:
- Microscopic polyangiitis (MPA) commonly presents with diffuse alveolar hemorrhage (DAH) and interstitial pneumonia (IP).
- Bronchiectasis (BE) has been identified as a pulmonary complication of MPA, but its specific clinical features and prognosis remain unclear.
- This study investigates the characteristics and outcomes of MPA patients with BE.
Purpose of the Study:
- To characterize the clinical features of bronchiectasis in microscopic polyangiitis patients.
- To evaluate the prognosis of microscopic polyangiitis patients with bronchiectasis compared to those without.
- To identify potential management implications for MPA-associated BE.
Main Methods:
- Retrospective study of 45 patients diagnosed with microscopic polyangiitis (MPA).
- Patients were categorized into two groups: those with bronchiectasis (BE) and those without BE.
- Comparison of clinical characteristics, initial treatments, and long-term prognosis between the two groups.
Main Results:
- Pulmonary involvement was observed in 69% of MPA patients, including IP (51%), BE (16%), and DAH (11%).
- No significant differences in clinical characteristics or initial treatments were found between MPA patients with and without BE.
- MPA patients with BE showed better prognosis in the first year but worse prognosis between 1 and 5 years post-diagnosis, with pneumonia causing mortality in two BE patients.
Conclusions:
- Bronchiectasis in MPA may be associated with lower acute-phase mortality but higher chronic-phase mortality compared to other pulmonary manifestations.
- Increased attention to pulmonary infections is crucial for managing MPA patients with BE during the chronic phase.
- Further research is warranted to elucidate the mechanisms underlying the differential prognosis of BE in MPA.
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