In-hospital Mortality Associated with Granulomatosis with Polyangiitis and Microscopic Polyangiitis Complicated with Respiratory Failure
Keywords:
ANCA-associated Vasculitis, Granulomatosis with polyangiitis, Microscopic polyangiitis, National Inpatient Sample database, Respiratory FailureAbstract
Abstract.
Background and aim: Our study aimed to determine the in-hospital mortality rate among granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) hospitalizations complicated with acute respiratory failure (ARF) in the US and determine the independent predictors of in-hospital mortality.
Method
We performed a retrospective cohort analysis utilizing the National In-patient Sample (NIS) database to identify hospitalizations of patients with GPA or MPA associated with ARF from 2016 to 2021. The outcomes included in-hospital mortality, requirement of invasive procedures, and hospital length of stay. Univariable and multivariable analysis were used to identify independent predictors associated with in-hospital mortality.
Results
We identified 4,283 unweighted hospitalizations with diagnosis of GPA or MPA associated with ARF. 78.3% of those were diagnosed with GPA, 19.2% with MPA, and 2.5% with both ICD-10 codes. The mean age was 63.7 years, 53.6% were female, and 73.3% were White. The rate of hemodialysis was 21.5%, plasmapheresis 7.5%, and extracorporeal membrane oxygenation in 0.5% of hospitalizations. The overall in-hospital mortality was 15.4%, compared to 35.9% in the subgroup requiring IMV. Independent predictors associated with increased in-hospital mortality were higher age, acute kidney injury (AKI), sepsis, requirement of non-invasive mechanical ventilation, interstitial lung disease (ILD), history of stroke, shock, and requirement of IMV.
Conclusion
GPA or MPA hospitalizations complicated with ARF were found to be associated with a high in-hospital mortality rate. Higher age, AKI, sepsis, shock, history of ILD, history of stroke, and requirement of mechanical ventilation were found to be independent predictors associated with increased in-hospital mortality.
References
1. Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. 2012 Revised International Chapel Hill consensus conference nomenclature of vasculitides. In: Arthritis and Rheumatism. 2013.
2. Grayson PC, Ponte C, Suppiah R, Robson JC, Craven A, Judge A, et al. 2022 American college of rheumatology/European alliance of associations for rheumatology classification criteria for eosinophilic granulomatosis with polyangiitis. Ann Rheum Dis. 2022;81(3).
3. Chung SA, Langford CA, Maz M, Abril A, Gorelik M, Guyatt G, et al. 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Antineutrophil Cytoplasmic Antibody–Associated Vasculitis. Arthritis and Rheumatology. 2021;73(8):1–18.
4. Banerjee P, Jain A, Kumar U, Senapati S. Epidemiology and genetics of granulomatosis with polyangiitis. Vol. 41, Rheumatology International. 2021. p. 2069–89.
5. Kitching AR, Anders HJ, Basu N, Brouwer E, Gordon J, Jayne DR, et al. ANCA-associated vasculitis. Nat Rev Dis Primers. 2020 Aug 27;6(1):71–99.
6. Wludarczyk A, Biedron G, Wójcik K, Zdrojewski Z, Masiak A, Czuszynska Z, et al. ANCA-associated vasculitis patients treated in Polish intensive care units - Retrospective characteristics based on the POLVAS registry. Anaesthesiol Intensive Ther. 2021;52(4).
7. Gibelin A, Dumas G, Valade S, de Chambrun MP, Bagate F, Neuville M, et al. Causes of acute respiratory failure in patients with small-vessel vasculitis admitted to intensive care units: a multicenter retrospective study. Ann Intensive Care. 2021;11(1):1.
8. Demiselle J, Auchabie J, Beloncle F, Gatault P, Grangé S, Du Cheyron D, et al. Patients with ANCA-associated vasculitis admitted to the intensive care unit with acute vasculitis manifestations: a retrospective and comparative multicentric study. Ann Intensive Care. 2017;7(1):1–9.
9. Zhang Y, Guo J, Zhang P, Zhang L, Duan X, Shi X, et al. Predictors of Mortality in Critically Ill Patients With Antineutrophil Cytoplasmic Antibody-Associated Vasculitis. Front Med (Lausanne). 2021;8:1.
10. Khera R, Krumholz HM. With Great Power Comes Great Responsibility: Big Data Research from the National Inpatient Sample. Circ Cardiovasc Qual Outcomes. 2017;10(7).
11. Etchegaray-Morales I, Mendoza-Pinto C, Barrera-Hernández S, Martinez-Barragan YM, Munguía-Realpozo P, Pelaez-Ballestas I, et al. Hospitalisation and mortality trends in ANCA-associated vasculitis in Mexico: results from a nationwide retrospective registry analysis. BMJ Open. 2025 Sep 28;15(9):e105133.
12. Craven A, Robson J, Ponte C, Grayson PC, Suppiah R, Judge A, et al. ACR/EULAR-endorsed study to develop Diagnostic and Classification Criteria for Vasculitis (DCVAS). Vol. 17, Clinical and Experimental Nephrology. 2013.
13. Krause ML, Cartin-Ceba R, Specks U, Peikert T. Update on Diffuse Alveolar Hemorrhage and Pulmonary Vasculitis. Vol. 32, Immunology and Allergy Clinics of North America. 2012.
14. HCUP Databases: Healthcare cost and utilization project (HCUP). Rockville, MD; 2023.
15. HCUP National Inpatient Sample (NIS). Healthcare Cost and Utilization Project (HCUP). 2018-2020. Agency for Healthcare Research and Quality, Rockville, MD. www.hcup-us.ahrq.gov/nisoverview.jsp.
16. Khera R, Angraal S, Couch T, Welsh JW, Nallamothu BK, Girotra S, et al. Adherence to methodological standards in research using the National Inpatient Sample. JAMA - Journal of the American Medical Association. 2017;318(20).
17. Agency for Healthcare Research and Quality, Rockville, MD. [Internet]. 2025 [cited 2025 Sep 24]. HCUP National Estimates Tutorial - Accessible Version. Healthcare Cost and Utilization Project (HCUP). Available from: hcup-us.ahrq.gov/tech_assist/nationalestimates/508_course/508course_2023.jsp.
18. Kimmoun A, Baux E, Das V, Terzi N, Talec P, Asfar P, et al. Outcomes of patients admitted to intensive care units for acute manifestation of small-vessel vasculitis: A multicenter, retrospective study. Crit Care. 2016;20(1):1–11.
19. Frausova D, Brejnikova M, Hruskova Z, Rihova Z, Tesar V. Outcome of thirty patients with ANCA-associated renal vasculitis admitted to the intensive care unit. Ren Fail. 2008;30(9).
20. Quartuccio L, Bond M, Isola M, Monti S, Felicetti M, Furini F, et al. Alveolar haemorrhage in ANCA-associated vasculitis: Long-term outcome and mortality predictors. J Autoimmun. 2020;108:1.
21. Qi M, Tang Y, Zhou H, Wang M, Yi Q, Liang Z, et al. Comprehensive characteristics of pulmonary antineutrophil cytoplasmic antibody-associated vasculitis and the development of a predictive nomogram for mortality. Int Immunopharmacol. 2025 Feb 6;147:1–8.
22. Kotani T, Matsuda S, Okazaki A, Nishioka D, Watanabe R, Gon T, et al. Risk prediction model for mortality in microscopic polyangiitis: multicentre REVEAL cohort study. Arthritis Res Ther. 2023;25(1):1.
23. Tufan MA, Tekkarışmaz N. Predictive factors of mortality in granulomatosis with polyangiitis: A single-center study. Arch Rheumatol. 2021;36(3):435–44.
24. Holguin F, Ramadan B, Gal AA, Roman J. Prognostic factors for hospital mortality and ICU admission in patients with ANCA-related pulmonary vasculitis. American Journal of the Medical Sciences. 2008;336(4):321–6.
25. Khan SA, Subla MR, Behl D, Specks U, Afessa B. Outcome of patients with small-vessel vasculitis admitted to a medical ICU. Chest. 2007;131(4):972–6.
26. Befort P, Corne P, Filleron T, Jung B, Bengler C, Jonquet O, et al. Prognosis and ICU outcome of systemic vasculitis. BMC Anesthesiol. 2013 Oct;13(1):1.
27. Guillevin L, Pagnoux C, Seror R, Mahr A, Mouthon L, Toumelin P Le. The Five-Factor Score Revisited. Medicine. 2011 Jan;90(1):19–27.
28. Wallace ZS, Lu N, Miloslavsky E, Unizony S, Stone JH, Choi HK. Nationwide Trends in Hospitalizations and In-Hospital Mortality in Granulomatosis With Polyangiitis (Wegener’s). Arthritis Care Res (Hoboken). 2017;69(6):3685–9.
29. Wallace ZS, Fu X, Harkness T, Stone JH, Zhang Y, Choi H. All-cause and cause-specific mortality in ANCA-associated vasculitis: overall and according to ANCA type. Rheumatology (Oxford). 2020 Sep 1;59(9):2308–15.
30. Arulkumaran N, Periselneris N, Gaskin G, Strickland N, Ind PW, Pusey CD, et al. Interstitial lung disease and ANCA-associated vasculitis: a retrospective observational cohort study. Rheumatology (Oxford). 2011;50(11).
31. Fernandez Casares M, Gonzalez A, Fielli M, Caputo F, Bottinelli Y, Zamboni M. Microscopic polyangiitis associated with pulmonary fibrosis. Clin Rheumatol. 2015;34(7):1273–7.
32. Tzelepis GE, Kokosi M, Tzioufas A, Toya SP, Boki KA, Zormpala A, et al. Prevalence and outcome of pulmonary fibrosis in microscopic polyangiitis. European Respiratory Journal. 2010;36(1).
33. Zhou P, Ma J, Wang G. Impact of interstitial lung disease on mortality in ANCA-associated vasculitis: A systematic literature review and meta-analysis. Vol. 18, Chronic Respiratory Disease. 2021.
34. Ali SF, Smith EE, Bhatt DL, Fonarow GC, Schwamm LH. Paradoxical Association of Smoking With In‐Hospital Mortality Among Patients Admitted With Acute Ischemic Stroke. J Am Heart Assoc. 2013 May 20;2(3):1–8.
35. Paradossi U, De Caterina AR, Trimarchi G, Pizzino F, Bastiani L, Dossi F, et al. The enigma of the ‘smoker’s paradox’: Results from a single-center registry of patients with STEMI undergoing primary percutaneous coronary intervention. Cardiovascular Revascularization Medicine. 2024 Dec;69:42–9.
36. Fonarow GC, Abraham WT, Albert NM, Stough WG, Gheorghiade M, Greenberg BH, et al. A smoker’s paradox in patients hospitalized for heart failure: findings from OPTIMIZE-HF. Eur Heart J. 2008 Jul 10;29(16):1983–91.
37. Adebile T V., Asifat OA, Dey S, Nwaonumah EC, Yu L. Effect of Smoking on Mortality in Individuals With COPD in the United States. Respir Care. 2025 May 1;70(5):559–65.
38. Garg A, Saleemi A, Asfaw M, Aldaoud N, Chalasani P, Lavu VK, et al. The outcomes of the obesity paradox in pulmonary embolism: a study of the national inpatient sample database from 2016 to 2020. Ann Hematol. 2025 Feb 22;104(2):1187–93.
39. Rozen G, Elbaz-Greener G, Margolis G, Marai I, Heist EK, Ruskin JN, et al. The Obesity Paradox in Real-World Nation-Wide Cohort of Patients Admitted for a Stroke in the U.S. J Clin Med. 2022;11(6):1.
40. Oliveros H, Villamor E. Obesity and mortality in critically ill adults: A systematic review and meta-analysis. Vol. 16, Obesity. 2008. p. 515–21.
41. Li S, Zhang W, Liu H. Association between lipid levels and all-cause and cause-specific mortality in critically ill patients. Sci Rep. 2023 Mar 29;13(1):5109.
42. Chen R, Zhong G, Ji T, Xu Q, Liu H, Xu Q, et al. Serum cholesterol levels predict the survival in patients with idiopathic pulmonary fibrosis: A long-term follow up study. Respir Med. 2025 Feb;237:107937.
43. Leavitt RY, Fauci AS, Bloch DA, Michel BA, Hunder GG, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of wegener’s granulomatosis. Arthritis Rheum. 1990;33(8).
44. Suppiah R, Robson JC, Grayson PC, Ponte C, Craven A, Khalid S, et al. 2022 American college of rheumatology/European alliance of associations for rheumatology classification criteria for microscopic polyangiitis. Ann Rheum Dis. 2022;81(3).
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