Clinical features, radiological findings and prognosis of microscopic polyangiitis with interstitial lung disease: a retrospective matched control study.

Autor: Song Q; Department of Respiratory, Ningbo Zhenhai People's Hospital (Ningbo No. 7 Hospital), No. 718, Nan'er West Road, Luotuo Street, Zhenhai, Ningbo, Zhejiang, China., Liu Y; Department of Respiratory, Ningbo Zhenhai People's Hospital (Ningbo No. 7 Hospital), No. 718, Nan'er West Road, Luotuo Street, Zhenhai, Ningbo, Zhejiang, China., Wu T; Department of Respiratory and Critical Care Medicine, Key Laboratory of Respiratory Disease of Ningbo, The First Affiliated Hospital of Ningbo University, Ningbo, Zhejiang, China., Zhang Y; Department of Respiratory and Critical Care Medicine, Key Laboratory of Respiratory Disease of Ningbo, The First Affiliated Hospital of Ningbo University, Ningbo, Zhejiang, China., Yan Y; Department of Respiratory, Ningbo Zhenhai People's Hospital (Ningbo No. 7 Hospital), No. 718, Nan'er West Road, Luotuo Street, Zhenhai, Ningbo, Zhejiang, China., Xiao S; Department of Respiratory, Ningbo Zhenhai People's Hospital (Ningbo No. 7 Hospital), No. 718, Nan'er West Road, Luotuo Street, Zhenhai, Ningbo, Zhejiang, China. xiaoshengwen1990@163.com.
Jazyk: angličtina
Zdroj: BMC pulmonary medicine [BMC Pulm Med] 2024 Dec 09; Vol. 24 (1), pp. 605. Date of Electronic Publication: 2024 Dec 09.
DOI: 10.1186/s12890-024-03423-w
Abstrakt: Background: The association between interstitial lung disease (ILD) and microscopic polyangiitis (MPA) has received increasing attention in recent years. However, there are still no studies comparing clinical characteristics and prognoses between MPA-ILD patients and patients with ANCA-negative ILDs. Therefore, the purpose of this study was to analyse a group of patients presenting MPA-ILD matched with patients exhibiting ANCA-negative ILDs to identify differences in their clinical characteristics and survival.
Methods: This study retrospectively reviewed the data of 60 patients with MPA-ILD and 60 patients with ANCA-negative ILDs who were matched for age, sex, and patterns on chest high-resolution CT (HRCT) images. The baseline clinical information, laboratory parameters and chest CT data were collected and analysed at each patient's initial diagnosis.
Results: Sixty of the 682 (8.8%) ILD patients were diagnosed with MPA-ILD. MPA-ILD patients tended to be older and have higher CRP and ESR levels. ILD antedated MPA in 61.7% (37/60) of the patients, and MPA occurred on average (45.1 ± 33.4) months after the ILD diagnosis. Compared with matched ANCA-negative ILD patients, MPA-ILD patients had higher CRP and serum creatinine levels and lower haemoglobin levels. UIP (63.3%) was the most common chest HRCT pattern, and the proportion of patients with oddly shaped cysts (P = 0.04) was significantly greater in the MPA-ILD group than in the matched ANCA-negative ILD group. The number of MPA-ILD patients was significantly shorter than that of ANCA-negative ILD patients (P = 0.005). Survival analysis revealed that acute exacerbation (AE) of ILD (HR 2.40, 95% CI 1.03-5.59, P = 0.043) and diffuse alveolar haemorrhage (HR 3.42, 95% CI 1.09-10.73, P = 0.036) were independently associated with shorter survival and that receiving glucocorticoids combined with immunosuppressants (HR 0.11, 95% CI 0.03-0.37, P < 0.001) was independently associated with prolonged survival in MPA-ILD patients.
Conclusions: The proportion of MPA-ILD patients with total ILD is not low, and ANCA testing of ILD patients is necessary. Oddly shaped cysts with a UIP pattern may be a characteristic chest imaging manifestation of MPA-ILD. The prognosis of MPA-ILD is poor, especially for patients who are older, have DAH, and have experienced AEs.
Competing Interests: Declarations. Ethics approval and consent to participate: All the studies were conducted in accordance with the Declaration of Helsinki. Written informed consent from study subjects was waived because of the retrospective design. The cohort study was approved by the institutional review board of the First Affiliated Hospital of Ningbo University and Ningbo Zhenhai People’s Hospital. All the methods were carried out in accordance with relevant guidelines and regulations. Consent for publication: Not applicable. Competing interests: The authors declare no competing interests. Clinical trial number: Not applicable.
(© 2024. The Author(s).)
Databáze: MEDLINE
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