Clinical Characteristics and Prognostic Analysis of Microscopic Polyangiitis With Diffuse Alveolar Hemorrhage

Abstract

<jats:sec><jats:title>Objective</jats:title><jats:p> To analyse the clinical features and prognostic factors of microscopic polyangiitis (MPA) with diffuse alveolar haemorrhage (DAH). </jats:p></jats:sec><jats:sec><jats:title>Methods</jats:title><jats:p> We conducted a retrospective study of 92 patients diagnosed with MPA with DAH at the First Affiliated Hospital of Chongqing Medical University between March 1, 2012, and March 12, 2018. The cumulative survival rate was analyzed by the Kaplan-Meier method, and survival curves were drawn. A Cox hazard model was used to determine the prognostic factors for survival by univariate and multivariate analysis. </jats:p></jats:sec><jats:sec><jats:title>Results</jats:title><jats:p> The mean age at the onset of MPA with DAH was 66.32 years. Among the 92 MPA with DAH patients who were followed up, 41 (44.57%) were critical illness and 79 (85.87%) had pulmonary and renal involvement. The cumulative survival rates of the 92 patients at 1, 3, and 5 years were 63.7%, 51.2%, and 47.3%, respectively, and the median survival time was 46 months. In the multivariate analysis, age&gt;65 years (HR=4.303, 95% CI: 1.940~9.547), sCr&gt;500 μmol/L (HR=2.043, 95% CI: 1.051~3.972), PaO2/FiO2&lt;300 mmHg (HR=4.095, 95% CI: 1.966~8.525), and lung involvement area≥50% (HR=2.928, 95% CI: 1.399~6.129) were independent prognostic factors (P&lt;0.05). </jats:p></jats:sec><jats:sec><jats:title>Conclusion</jats:title><jats:p> The incidence and mortality of DAH are high in MPA patients. Age&gt;65 years, sCr&gt;500 μmol/L, PaO2/FiO2&lt;300 mmHg and lung involvement area≥50% are independent prognostic factors for MPA with DAH.</jats:p></jats:sec>

The Survival of Patients With Alveolar Hemorrhage Secondary to Antineutrophil Cytoplasmic Antibody–associated Vasculitis

“…Other factors with a potential effect on reported outcomes of DAH in AAV are undoubtedly influenced by access to health- care, availability of intensive care, cultural attitudes toward intensive care in elderly patients with multiorgan failure, and local practice patterns. These factors lead to differences in time from disease onset to diagnosis (a median of 3.5 months in the Tang, et al study 11 ) or initiation of definitive therapy, as well as differences in intensive care unit admission, which were not consistently reported in all the published cohorts. The comparability of the different reports on DAH in AAV is further complicated by different entry criteria applied across studies.…”

“…In this issue of The Journal, Tang and colleagues 11 report on their analysis of clinical features at presentation and the main prognostic factors of DAH in a single-center cohort of 92 patients with MPA. This is the largest cohort of DAH in patients with MPA published so far.…”

“…In contrast to most previous reports from Western countries, the patients comprising this report are Chinese, and the different genetic background of these patients might have influenced the disease phenotype, the response to treatment, and ultimately the course of the disease 12 . All patients included in this study by Tang and colleagues 11 had a diagnosis of MPA, whereas most other reported cohorts comprised a mix of MPA, GPA, and eosinophilic GPA (EGPA). Only one previous cohort included just patients with MPA, yet the 1-year and 5-year survival was 82% and 68%, respectively 13 (Table 1).…”

“…The comparability of the different reports on DAH in AAV is further complicated by different entry criteria applied across studies. For instance, the study by Tang, et al 11 did not require the performance of bronchoscopy with bronchoalveolar lavage to confirm alveolar hemorrhage, as it was for a few of the previous studies on DAH 13,14 . Since 25-50% of patients with DAH do not have hemoptysis at the time of presentation 11,14 , it is conceivable that patients with different severity and acuity of DAH make up the various different cohorts.…”

“…Treatment is also heterogeneous across studies. In the Tang, et al 11 study, the majority of patients were treated with GC and CYC; no patient received RTX, and approximately 18% of patients were treated with intravenous immunoglobulin (IVIG), which is not a generally accepted remission-induction treatment 9,21 . One could speculate that the use of IVIG to induce the remission of DAH in patients with AAV instead of CYC or RTX might be associated with an increased mortality.…”