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ANCA-Associated Vasculitis (AAV)

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Comorbidities

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) describes a group of rare autoimmune conditions characterized by multisystemic inflammation and the destruction of blood vessels throughout the body. The group comprises granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA).1 

Cardiovascular Comorbidities

Cardiovascular comorbidities are among the leading causes of morbidity and mortality among patients with ANCA-associated vasculitis, especially during active phases of the disease.2,3 Aspects of the pathogenesis of ANCA-associated vasculitis, including systemic inflammation that drives platelet activation and endothelial dysfunction, elevate the risk of cardiovascular events.4 

The risk of venous thrombotic events, including pulmonary embolism and deep venous thrombosis, is increased in patients with ANCA-associated vasculitis. Most of these events occur during treatment for active disease, with an incidence rate between 8% and 10%.2 

Older patients with ANCA-associated vasculitis are at greater risk for cardiovascular disease, demonstrating an increased incidence of myocardial infarction and ischemic heart disease within the first 1 to 2 years after diagnosis.2 Studies reported an almost 2-fold rise in the incidence of both myocardial infarction (relative risk [RR], 1.86) and ischemic heart disease (RR, 1.9) among patients with GPA.2,5,6 

Other studies revealed an increased incidence of comorbidities such as coronary artery disease (calculated comparative morbidity figure [CMF], 4.22) and ischemic stroke (CMF, 4.65).2,7

Advanced age, a low estimated glomerular filtration rate (eGFR), chronic kidney disease requiring dialysis dependency, smoking history, and hypercholesterolemia increase the risk of cardiovascular disease in this patient population.2,8

A comprehensive cardiac workup is recommended for individuals with a diagnosis of ANCA-associated vasculitis, including lipid serological testing, echocardiography, electrocardiography, cardiac magnetic resonance imaging, and if necessary, endomyocardial biopsy and coronary artery assessment.2

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Respiratory Comorbidities

ANCA-associated vasculitis has been found to be associated with bronchiectasis,3,9 upper airway disease,9 and pulmonary infection,9 particularly in patients with GPA. In patients with MPA and unclassified ANCA-associated vasculitis, fibrotic interstitial lung disease and emphysema are more prevalent comorbidities.9 

The presence of pulmonary infection predicted mortality (hazard ratio [HR], 2.64; P =.04) and chance of remission [HR, 1.58; P =.03) among patients with MPO-ANCA associated vasculitis. Concurrent comorbidities of fibrotic interstitial lung disease and pulmonary infection predicted worse survival in this patient population.9

Another pulmonary comorbidity and serious complication of ANCA-associated vasculitis is diffuse alveolar hemorrhage, which also contributes to morbidity and mortality in these patients.10

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Renal Comorbidities

End-stage renal disease11 and ANCA-associated necrotizing crescentic glomerulonephritis are some of the most serious complications of ANCA-associated vasculitis. Patients with these comorbidities may require a kidney transplant.12

Renal-limited vasculitis, a fourth rare form of ANCA-associated vasculitis, affects only the kidneys and is associated with elevated serum creatinine, proteinuria, and hematuria. Inflammation in the capillaries of the glomeruli causes tissue scarring and damage that ultimately leads to kidney failure and a need for dialysis or transplant. 13

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Other Comorbidities

Osteoporosis may develop as a treatment-related comorbidity following the long-term use of glucocorticoids.1,3,14 Other treatment-related comorbidities may include malignancy, bone marrow failure, chemical cystitis, diabetes, and infertility due to gonadal failure.1

Thyroid disease, bronchiectasis, osteoporosis,3 and peripheral neuropathy including mononeuritis multiplex15 are other potential comorbidities of ANCA-associated vasculitis. These conditions can lead to a marked decrease in health-related quality of life, including either physical or mental status with concurrent increases in fatigue, anxiety, mood disorders, and sleep disturbances.3 

Read more about AAV signs and symptoms

References

  1. Qasim A, Patel JB. ANCA positive vasculitis. StatPearls [Internet]. Updated May 29, 2022. Accessed February 23, 2023.
  2. Kronbichler A, Leierer J, Gauckler P, Shin JI. Comorbidities in ANCA-associated vasculitis. Rheumatology (Oxford). 2020;59(Suppl 3):iii79-iii83. doi:10.1093/rheumatology/kez617
  3. Mercuzot C, Letertre S, Daien CI, et al. Comorbidities and health-related quality of life in patients with Antineutrophil Cytoplasmic Antibody (ANCA) – associated vasculitis. Autoimmun Rev. 2021;20(1):102708. doi:10.1016/j.autrev.2020.102708
  4. Floyd L, Morris AD, Woywodt A, Dhaygude A. Cardiovascular disease and ANCA-associated vasculitis: are we missing a beat? Clin Kidney J. 2022;15(4):618-623. doi:10.1093/ckj/sfac009
  5. Aviña-Zubieta JA, Mai A, Amiri N, et al. Risk of myocardial infarction and stroke in patients with granulomatosis with polyangiitis (Wegener’s): a population-based study. Arthritis Rheumatol. 2016;68(11):2752-2759. doi:10.1002/art.39762
  6. Faurschou M, Mellemkjaer L, Sorensen IJ, Thomsen BS, Dreyer L, Baslund B. Increased morbidity from ischemic heart disease in patients with Wegener’s granulomatosis. Arthritis Rheum. 2009;60(4):1187-1192. doi:10.1002/art.24386
  7. Mourguet M, Chauveau D, Faguer S, et al. Increased ischemic stroke, acute coronary artery disease and mortality in patients with granulomatosis with polyangiitis and microscopic polyangiitis. J Autoimmun. 2019;96:134-141. doi:10.1016/j.jaut.2018.09.004
  8. Morgan MD, Turnbull J, Selamet U, et al. Increased incidence of cardiovascular events in patients with antineutrophil cytoplasmic antibody-associated vasculitides: a matched-pair cohort study. Arthritis Rheum. 2009;60(11):3493-3500. doi:10.1002/art.24957
  9. Ono N, Inoue Y, Miyamura T, et al. The association of airway comorbidities with the clinical phenotypes and outcomes of patients with antineutrophil cytoplasmic autoantibody–associated vasculitis. J Rheumatol. 2021;48(3):417-425. doi:10.3899/jrheum.190373
  10. West S, Arulkumaran N, Ind PW, Pusey CD. Diffuse alveolar haemorrhage in ANCA-associated vasculitis. Intern Med. 2013;52(1):5-13. doi:10.2169/internalmedicine.52.8863
  11. Moiseev S, Novikov P, Jayne D, Mukhin N. End-stage renal disease in ANCA-associated vasculitis. Nephrol Dial Transplant. 2017;32(2):248-253. doi:10.1093/ndt/gfw046
  12. Sharmeen S, Cassol C, Kato H. ANCA-associated necrotizing glomerulonephritis overlapping with mesangial proliferative lupus nephritis refractory to plasmapheresis, steroid pulse therapy, and a combination of mycophenolate mofetil and rituximab. Case Rep Rheumatol. 2018;2018:3076806. doi:10.1155/2018/3076806
  13. Almaani S, Fussner LA, Brodsky S, Meara AS, Jayne D. ANCA-associated vasculitis: an update. J Clin Med. 2021;10(7):1446. doi:10.3390/jcm10071446
  14. Rosen HN, Saag KG. Prevention and treatment of glucocorticoid-induced osteoporosis. UpToDate. Accessed February 23, 2023.
  15. Eindhoven S, Levels J, Huisman M, de Winter KR, Dalm V, Alwani R. MPO-ANCA associated vasculitis with mononeuritis multiplex following influenza vaccination. Allergy Asthma Clin Immunol. 2017;13:49. doi:10.1186/s13223-017-0222-9

Reviewed by Debjyoti Talukdar, MD, on 2/28/2023.

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