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Long-Term Prognostic Factors in Patients With Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: A 15-Year
Qian-Qian Liao1, Ya-Fei Ren2, Ke-Wei Zhu3,4
1Department of Pharmacy, People's Hospital of Guilin, Guilin, China.
Background:
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a multisystem autoimmune disease with small-vessel involvement. In AAV, microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) are major clinicopathologic variants. In addition, myeloperoxidase (MPO) and proteinase 3 (PR3) are major target antigens. The objective of the study was to explore the predictive factors for long-term survival in AAV patients.
Materials And Methods:
A multicenter retrospective study was carried out on 407 patients between 2005 and 2020. Clinical parameters were obtained from laboratory tests including the ANCA types, antinuclear antibody (ANA), extractable nuclear antigen (ENA), anti-streptolysin O (ASO), glomerular filtration rate (GFR), and the laboratory examinations for the blood routine, liver function, renal function, and immunity, etc. The data for clinical parameters were collected from electronic medical records (EMRs), and the data for patient survival were acquired through regular follow-up. The association of clinical parameters with overall survival (OS) along with 3-year and 5-year survival rates was analyzed, and the nomogram as a predictive model was established according to the analysis results.
Results:
In the present study, 336 (82.6%) patients and 46 (11.3%) patients were diagnosed with MPA and GPA, respectively. The mean and median OS for all the patients were 2,285 and 2,290 days, respectively. The 1-year, 3-year, 5-year, and 10-year cumulative survival rates for all the patients were 84.2%, 76.3%, 57.2%, and 32.4%, respectively. Univariate and multivariate survival analyses indicated that the independent prognostic factors included age, pathological categories (MPA, GPA, and other types), serum ANCA types (negative or positive for MPO and/or PR3), ANA, ASO, GFR, lymphocyte, neutrophil-to-lymphocyte ratio (NLR), and C-reactive protein (CRP), and these clinical parameters except for ASO were used to construct a nomogram. The nomogram for 3-year and 5-year survival rates had a C-index of 0.721 (95% CI 0.676-0.766). The calibration curves showed that the predicted values of the nomogram for 3-year and 5-year survival rates were generally consistent with practical observed values, and decision curve analysis (DCA) further demonstrated the practicability and accuracy of the predictive model.
Conclusion:
Laboratory tests at diagnosis have great significance in the prediction of long-term survival in AAV patients.
Insights
This study identified key predictors of long-term survival in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) patients. Laboratory test results at diagnosis are crucial for predicting survival outcomes in AAV.
Area of Science:
- Rheumatology
- Immunology
- Internal Medicine
Background:
- Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a multisystem autoimmune disease affecting small vessels.
- Microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) are primary clinicopathologic variants of AAV.
- Myeloperoxidase (MPO) and proteinase 3 (PR3) are key target antigens in AAV pathogenesis.
Purpose of the Study:
- To investigate predictive factors for long-term survival in patients diagnosed with AAV.
- To develop a predictive model for estimating survival rates in AAV patients.
Main Methods:
- A multicenter retrospective study involving 407 AAV patients from 2005 to 2020.
- Collected clinical and laboratory data including ANCA types, autoantibodies (ANA, ENA), GFR, and inflammatory markers.
- Utilized survival analysis and constructed a nomogram to predict 3-year and 5-year survival rates.
Main Results:
- The study analyzed 407 patients, with MPA and GPA being the most common variants (82.6% and 11.3%, respectively).
- Identified independent prognostic factors for overall survival: age, pathological type, ANCA status (MPO/PR3), ANA, GFR, lymphocyte count, NLR, and CRP.
- The developed nomogram demonstrated good predictive accuracy for 3-year and 5-year survival rates (C-index: 0.721).
Conclusions:
- Laboratory test results obtained at the time of diagnosis are highly significant for predicting long-term survival in AAV patients.
- The established nomogram provides a practical and accurate tool for predicting survival in AAV, aiding clinical decision-making.
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