ANCA-Associated Vasculitis (AAV)

In the course of disease, patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) may require surgery. Proper evaluation, including the identification of risk and prognosis, should be completed to assess the benefits and liabilities of each procedure on a case-by-case basis. Before choosing surgery, a patient’s team of physicians should: assess the nature, severity, and stability of all comorbidities; evaluate the risks associated with anesthesia and surgery; identify possible postoperative complications; and make an optimal treatment plan, including patient preventative practices. Comprehensive perioperative management is vital to ensure optimal patient outcomes and promote patient-centered care. 1

Preoperative Evaluation in All Patients With AAV

Evaluation of Cardiovascular Risk

The perioperative management of all patients with vasculitis involves a careful cardiovascular risk assessment. In patients with ischemic heart disease, risk is determined by a combination of factors, including disease severity and stability, the patient’s age, functional capacity, comorbidities, and the type of surgery to be performed. These determinants may help predict the occurrence of postoperative myocardial infarction, congestive heart failure (CHF), and mortality in patients undergoing surgery. An accurate estimation of these factors can determine the appropriate level of perioperative cardiac monitoring and management and mitigate the risk of adverse cardiovascular events.1 

Major predictors of increased perioperative risk include myocardial infarction within the past 30 days, unstable or severe angina, poorly compensated CHF, significant arrhythmias, and severe valvular disease. Intermediate determinants include mild angina, a history of prior myocardial infarction or the presence of pathologic Q waves, compensated CHF or prior CHF, and diabetes mellitus.1 

To reduce cardiac risk in noncardiac surgery, a range of strategies is available. These may include preoperative medical therapy or invasive cardiac interventions, modifications to anesthetic techniques, and the proactive management of hemodynamic changes during and after surgery. For the internist-rheumatologist evaluating a patient before surgery, the focus of management is primarily therapeutic, involving the use of β-blockers, antiplatelet agents, and statin therapy. Special consideration must be given to the management of antiplatelet therapy in patients with cardiac stents, because this can present unique challenges. In some cases, coronary revascularization before noncardiac surgery may also be considered as an option to reduce risk.1 

Read more about AAV risk factors

Evaluation of Pulmonary Risk

Pulmonary involvement is common in patients who have granulomatosis with polyangiitis (GPA) or eosinophilic granulomatosis with polyangiitis (EPA), and postoperative pulmonary complications are important adverse events to consider.2,3 The effect of chronic pulmonary dysfunction on perioperative risk varies significantly depending on the what type of surgery is required. Minor procedures may be well tolerated by patients with severe lung impairment, even under general anesthesia. However, major intra-abdominal or intrathoracic surgeries pose a high risk of atelectasis or pneumonia. In contrast, peripheral limb surgery, such as hip or knee surgery, carry a low risk of pneumonia, even in patients with chronic lung disease.1

When regional anesthesia for extremity surgery is being considered, it is essential to note that an interscalene block may temporarily paralyze the ipsilateral diaphragm and decrease forced vital capacity (FVC) by 30% to 40%. Therefore, preoperative pulmonary function studies are necessary when patients with lung disease may use an interscalene block for shoulder surgery. For patients with severely impaired pulmonary function (forced expiratory volume in 1 second [FEV1] <1 L), the interscalene block should not be used at all.1 

Patients on long-term bronchodilator therapy should take their standard dose the night before surgery, and then this therapy should be restarted postoperatively, typically via nebulizer. In addition, the use of incentive spirometry at least 10 times daily and early mobilization can help prevent postoperative atelectasis.1

Read more about AAV guidelines

Special Considerations for Patients With GPA

Respiratory Complications of Surgery

In patients with GPA, localized granulomatous inflammation characteristically affects the upper respiratory tract (otitis media, sinusitis, mastoiditis, crusting rhinitis, sinusitis) and lower respiratory tract (alveolar hemorrhage, lung nodules).2 Involvement of the larynx is not uncommon and may complicate the preoperative intubation process. Anesthesiologists should anticipate the possibility of a difficult airway in a patient with GPA, and it is recommended that an indirect laryngoscopy be performed preoperatively to assess for narrowing of the laryngeal lumen. The immediate postoperative period following tracheal extubation is critical because airway edema and obstruction are likely to develop.4

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Renal Complications of Surgery

Kidney involvement in GPA is common. As a result, the anesthetic management of these patients requires careful drug selection and dosing adjustments. The accumulation of anesthetic drugs that rely on renal excretion can have adverse effects. The loading doses of inducing agents depend primarily on redistribution rather than elimination, but maintenance doses of highly protein-bound agents should be reduced by approximately 30% to 50%.4 

Commonly used anesthetic agents, such as vecuronium, Pavulon® (pancuronium), Atropen® (atropine), glycopyrrolate, and Bloxiverz® (neostigmine), depend predominantly on renal excretion and should be used with caution. Drugs that produce active or toxic metabolites that are excreted renally, including midazolam, Valium® (diazepam), and morphine, should also be used judiciously.4 

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Cardiac Complications of Surgery

In addition to careful drug selection and dosing, close monitoring of the hemodynamic status of patients with renal involvement is essential because fluctuations in the heart rate and blood pressure can lead to myocardial ischemia or infarction. For patients with a reduced ejection fraction, dose titration of induction and maintenance agents may be required to maintain a stable hemodynamic profile. Furthermore, vasculitis in peripheral veins and arteries, which can result in digital infarcts, should be anticipated, and the number of arterial and venous punctures should be minimized accordingly.4

Read more about AAV prognosis


1. MacKenzie CR, Paget SA. Perioperative management of the patient with rheumatic disease. In: Imboden JB, Hellmann DB, Stone JH, eds. Current Diagnosis & Treatment: Rheumatology. 3e. McGraw-Hill;2013;62. Accessed March 8, 2023.

2. Garlapati P, Qurie A. Granulomatosis with polyangiitis. StatPearls [Internet]. Updated December 5, 2022. Accessed March 14, 2023.

3. Gioffredi A, Maritati F, Oliva E, Buzio C. Eosinophilic granulomatosis with polyangiitis: an overview. Front Immunol. 2014;5:549. doi:10.3389/fimmu.2014.00549

4. Sharma J, Lal J, Gehlaut P, Dhawan G, Yadav A. Wegener’s granulomatosis and anaesthetic implications: a case report.  Int J Med Res Pro. 2018;4(1):479-481. doi:10.21276/ijmrp

Reviewed by Debjyoti Talukdar, MD, on 3/17/2023.