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Patterns of lung diseases predict survival in patients with MPO-ANCA-associated vasculitis: a single-center retrospective study

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Abstract

Objectives

This study aimed to explore differences in clinical features and prognosis among patients with varied myeloperoxidase (MPO) antineutrophil cytoplasmic antibody-associated vasculitis (MPO-AAV) associated lung diseases.

Methods

Patients with MPO-AAV-associated lung diseases were enrolled in this retrospective cohort study at a single center. Clinical features and laboratory data at the time of diagnosis were compared among patients with various lung disease patterns. Kaplan–Meier and Cox regression analyses were performed to analyze overall survival.

Results

A total of 155 patients were finally included and categorized into five groups, as follows: 72 had a usual interstitial pneumonia (UIP) pattern, 40 had non-UIP interstitial pneumonia, 18 had bronchiectasis (BR), 13 had necrotizing granuloma (NG), and 12 had diffuse alveolar hemorrhage (DAH). Among the five groups, patients with DAH had higher dyspnea and hemoptysis frequencies, lower PaO2/FiO2 levels, elevated C-reactive protein levels, and the poorest prognosis. The overall survival (OS) in the DAH group (median OS: 3.2 months) was significantly poorer than that in the NG group (median OS: not reached, log rank P < 0.001), the BR group (median OS: not reached, log rank P < 0.001), and the non-UIP IP group (median OS: 61.1 months, log rank P = 0.001). The UIP group had significantly more ex-smokers than the other groups (P < 0.001) and the second poorest survival (median OS: 39.1 months). The NG group tended to have female predominance, a higher incidence of ENT involvement, less severe renal involvement, and the best survival. After adjusting for multi-model Cox regression analysis, DAH and UIP (hazard ratio: 19.301 and 9.940, respectively, compared with NG) were independent predictors of all-cause mortality.

Conclusions

Various patterns of lung disease-associated MPO-AAV may potentially predict patient survival.

Key Point

• The present study described the clinical and prognostic features of various lung diseases-associated MPO-AAV, indicating the potential prediction for the survival of MPO-AAV patients.

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Data availability

The data used and analyzed in this study is included in the article or is available from the corresponding and first authors on reasonable request.

Code availability

Not applicable.

Abbreviations

AAV :

Antineutrophil cytoplasmic antibodies associated vasculitis

AE-UIP :

Acute exacerbation of usual interstitial pneumonia

Alb :

Albumin

ANCA :

Antineutrophil cytoplasmic antibodies

BR :

Bronchiectasis

BVAS :

Birmingham Vasculitis Activity Score

C3 :

Complement 3

C4 :

Complement 4

CHCC2012 :

2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides

CKD :

Chronic kidney disease

CPFE :

Combined pulmonary fibrosis and emphysema

CRP :

C-reactive protein

CTDs :

Connective tissue diseases

DAH :

Diffuse alveolar hemorrhage

eGFR :

Estimated glomerular filtration rate

EGPA :

Eosinophilic granulomatosis with polyangiitis

ELISA :

Enzyme-linked immunosorbent assays

EMA :

European Medicines Agency

ENT :

Ear, nose, and throat

GPA :

Granulomatosis with polyangiitis

HRCT :

High-resolution computed tomography

HRs :

Hazard ratios

IgG :

Immunoglobulin G

IIPs :

Idiopathic interstitial pneumonias

ILDs :

Interstitial lung diseases

IP :

Interstitial pneumonia

IPF :

Idiopathic pulmonary fibrosis

IQR :

Interquartile ranges

MPA :

Microscopic polyangiitis

MPO :

Myeloperoxidase

NETs :

Neutrophil extracellular traps

NG :

Necrotizing granuloma

NSIP :

Nonspecific interstitial pneumonia

OP :

Organic pneumonia

OS :

Overall survival

PR3 :

Proteinase 3

RA :

Rheumatoid arthritis

Scr :

Serum creatinine

UIP :

Usual interstitial pneumonia

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Acknowledgements

We thank all patients and investigators who were involved in this study. We express our thanks to Miss Moyang Xu of University of Michigan—Ann Arbor for polishing language and grammar of the manuscript.

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Authors and Affiliations

  1. Clinical Center for Interstitial Lung Diseases, Beijing Institute of Respiratory Medicine, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, 100020, China

    Shuqiao Yang, Dandan Chai, Yihua Li, Yuanying Wang, Xi Zhan, Liming Zhang, Jing Wang & Qiao Ye

  2. Department of Respiratory and Critical Care Medicine, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, 100020, China

    Shuqiao Yang, Xi Zhan, Liming Zhang & Jing Wang

  3. Department of Occupational Medicine and Toxicology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, 100020, China

    Dandan Chai, Yihua Li, Yuanying Wang & Qiao Ye

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  1. Shuqiao Yang
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Contributions

S Yang was responsible for completing the analysis of data and writing. D Chai, Y Li, and Jing Wang performed all data collection. X Zhan and Y Wang contributed to the follow-up. L Zhang and Q Ye evaluated the chest images. Q Ye also contributed as primary investigator and was responsible for designing the study, recruiting the patients and writing the manuscript. All authors have read and approved the final manuscript.

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Correspondence to Qiao Ye.

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Yang, S., Chai, D., Li, Y. et al. Patterns of lung diseases predict survival in patients with MPO-ANCA-associated vasculitis: a single-center retrospective study. Clin Rheumatol 41, 783–793 (2022). https://doi.org/10.1007/s10067-021-05964-5

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