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Clinical significance of microscopic polyangiitis with interstitial lung disease and bronchiectasis: probability of
Yun Zhang1, Qunli Ding1, Chengna Lv1
1Department of Respiratory, The First Affiliated Hospital of Ningbo University, Ningbo, China.
Background:
The association between pulmonary involvement and microscopic polyangiitis (MPA) has been increasingly recognized in recent years. Whether interstitial lung disease (ILD) and bronchiectasis (BE) are disease manifestations of MPA, preexisting comorbidities or important complications remains unclear. The purpose of this study was to determine the clinical characteristics and prognosis of MPA with pulmonary involvement to further guide clinical management.
Methods:
The data for 97 patients with a definitive diagnosis of MPA were retrospectively reviewed. The MPA diagnosis was based on the 2012 revised Chapel Hill Consensus Conference (CHCC) criteria. The baseline clinical information and laboratory parameters were collected and analysed at each patient's initial diagnosis.
Results:
Forty-seven out of the 97 (48.5%) patients who were diagnosed with MPA presented with pulmonary involvement, including 37 patients with ILD, 12 patients with BE and two patients with diffuse alveolar haemorrhage (DAH). ILD and BE antedated MPA in 56.76% and 75.00% of the patients, respectively. Compared with that in the MPA-BE group, the serum LDH level (222.86 ± 68.19 vs. 171.58 ± 31.43, p = .016) in the MPA-ILD group was significantly higher. In the multivariate Cox analysis, elevated serum creatinine (HR 4.08, confidence interval (CI) 1.38-12.05, p = .011) was an independent risk factor for shorter survival in MPA patients with pulmonary involvement, and treatment with glucocorticoid pulse cyclophosphamide therapy (HR 0.095, 95% CI 0.019-0.47, p = .004) was independently associated with prolonged survival. Among the patients in the MPA-ILD group, acute exacerbations of ILD (HR 4.55 CI 1.16-17.86, p = .029) and elevated serum creatinine (HR 4.95, CI 1.39-17.54, p = .014) were independently associated with a poor prognosis, and treatment with glucocorticoids (HR 0.057, 95% CI 0.012-0.28, p < .001) was independently associated with significant prolongation of survival.
Conclusions:
Patients with MPA have a high prevalence of pulmonary involvement, and ILD is the most common subtype of MPA. ILD and BE can be considered preexisting comorbidities of MPA. Elevated serum creatinine was associated with shorter survival. However, remission induction regimens with glucocorticoids and/or immunosuppressants may improve this outcome.
Insights
Microscopic polyangiitis (MPA) frequently involves the lungs, with interstitial lung disease (ILD) being most common. While ILD and bronchiectasis (BE) may predate MPA, elevated creatinine indicates poorer survival, treatable with immunosuppressants.
Area of Science:
- Rheumatology
- Pulmonology
- Internal Medicine
Background:
- Pulmonary involvement is increasingly recognized in microscopic polyangiitis (MPA).
- The exact nature of interstitial lung disease (ILD) and bronchiectasis (BE) in MPA (manifestation, comorbidity, or complication) remains unclear.
- Understanding these associations is crucial for guiding clinical management.
Purpose of the Study:
- To determine the clinical characteristics of MPA patients with pulmonary involvement.
- To investigate the prognosis of MPA with pulmonary involvement.
- To provide data for improved clinical management strategies.
Main Methods:
- Retrospective review of 97 patients with a definitive MPA diagnosis based on 2012 revised Chapel Hill Consensus Conference (CHCC) criteria.
- Collection and analysis of baseline clinical information and laboratory parameters at initial diagnosis.
- Multivariate Cox analysis to identify risk factors for survival.
Main Results:
- 48.5% of MPA patients had pulmonary involvement (37 ILD, 12 BE, 2 DAH).
- ILD and BE often antedated MPA diagnosis (56.76% and 75.00%, respectively).
- Elevated serum creatinine was an independent risk factor for shorter survival (HR 4.08), while glucocorticoid pulse cyclophosphamide therapy improved survival (HR 0.095).
Conclusions:
- Pulmonary involvement, particularly ILD, is common in MPA and may represent preexisting comorbidities.
- Elevated serum creatinine is linked to reduced survival in MPA patients with pulmonary involvement.
- Aggressive treatment with glucocorticoids and/or immunosuppressants can improve outcomes.
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