Preoperative evaluation and perioperative management of patients with rheumatic diseases

INTRODUCTION — Clinicians caring for patients with rheumatic diseases must be aware that there are specific issues involved in their pre- and postoperative management [1,2].

Areas of special concern for patients with rheumatic diseases who are having surgery will be discussed here. General issues in preoperative medical consultation and perioperative management are presented separately. (See "Preoperative medical evaluation of the healthy adult patient" and "Evaluation of cardiac risk prior to noncardiac surgery" and "Prevention of venous thromboembolic disease in adult nonorthopedic surgical patients" and "Perioperative medication management".)

PREOPERATIVE EVALUATION

History and physical examination — All patients require a careful preoperative history and physical examination, with further evaluation indicated in some individuals. Some particular concerns among patients with rheumatic diseases include the following:

●Several rheumatic diseases are associated with increased risks of coronary artery disease, arrhythmias, heart failure, or cardiac death. (See "Overview of the systemic and nonarticular manifestations of rheumatoid arthritis", section on 'Cardiac disease' and "Coronary heart disease in systemic lupus erythematosus".)

Determining cardiovascular risk may be more difficult because many patients with rheumatic diseases are not physically active. An exercise or pharmacologic stress test may be necessary in some circumstances to adequately assess the risk of perioperative myocardial infarction. (See "Evaluation of cardiac risk prior to noncardiac surgery".)

●Airway management for anesthesia may be difficult in patients with cervical spine or temporomandibular joint disease. Airway manipulation in patients with severe cervical subluxation can result in spinal cord injury or death. (See "Cervical subluxation in rheumatoid arthritis" and "Management of the difficult airway for general anesthesia in adults".)

●Limited range of motion of other joints or joint pain may make positioning for surgery complicated. As an example, patients with shoulder pain may have difficulty lying in the lateral decubitus position for hip surgery.

Laboratory testing — Preoperative laboratory testing should be based on patient-specific factors and the planned surgical procedure. (See "Preoperative medical evaluation of the healthy adult patient", section on 'Laboratory studies' and "Preoperative evaluation for anesthesia for noncardiac surgery", section on 'Clinical evaluation'.)

GENERAL MANAGEMENT PRINCIPLES — Perioperative care in patients with rheumatic diseases requires special attention to the following issues:

●Assessing disease activity and optimizing the medication regimen

●Recognizing the increased cardiovascular risk associated with rheumatic diseases

●Reducing thrombosis risk in patients with antiphospholipid syndrome (APS)

●Minimizing risk of surgical site infections in the setting of immunosuppressive medications

Assessment of disease activity — Prior to elective surgery, patients should be evaluated by their rheumatologist to assess disease activity and identify major organ involvement that may impact the surgery. Ideally, patients should undergo surgery during periods when their disease is well controlled and they are on an optimized medication regimen. Appropriate testing will vary by disease. (See 'Disease-specific issues' below.)

Cardiac risk assessment — Given the increased risk of cardiovascular disease among patients with inflammatory diseases such as rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE), a cardiovascular risk assessment may be required prior to surgery. (See "Coronary artery disease in rheumatoid arthritis: Implications for prevention and management", section on 'Risk estimation' and "Coronary heart disease in systemic lupus erythematosus", section on 'Evaluation' and "Evaluation of cardiac risk prior to noncardiac surgery".)

Management of thrombophilia in antiphospholipid syndrome — Patients with APS may be at increased risk of thrombosis. Management of such patients depends upon their specific drug regimen and whether or not they have APS. The treatment of APS and the management of patients receiving anticoagulants in the perioperative setting are discussed in detail separately. (See "Prevention of venous thromboembolic disease in adult nonorthopedic surgical patients" and "Management of antiphospholipid syndrome", section on 'Primary thrombosis prevention' and "Perioperative management of patients receiving anticoagulants".)

Medication management — The optimal strategy for managing immunosuppressive therapies during the perioperative period is unknown due to the lack of evidence. We discuss our approach to management of the major categories of medications used for the treatment of various rheumatic diseases in the perioperative setting below; it is largely based on low-quality evidence and expert opinion. A rheumatologist should be involved in the medication management around the time of surgery.

Our approach is generally consistent with the guidelines proposed by the American College of Rheumatology and the American Association of Hip and Knee Surgeons for the perioperative management of various immunosuppressive medications in patients undergoing total hip and knee arthroplasty [3,4].

Glucocorticoids — The major concerns with regard to glucocorticoid use in the setting of surgery are the risk of surgical site infections and the risk of hemodynamic instability (secondary to glucocorticoid-induced adrenal insufficiency). Ideally, the lowest possible dose of glucocorticoids should be used in the perioperative setting in order to minimize these risks. If patients require greater than 10 mg prednisone (or equivalent) daily, it would imply that the disease activity is not adequately controlled and that elective surgery should be postponed. Therefore, we suggest that the dose of prednisone is less than or equal to 10 mg per day before surgery is planned. Patients should continue their outpatient glucocorticoid dose perioperatively, which may require parenteral administration (of the equivalent dose) if a patient is taking nothing by mouth.

Limited data suggest that the risk of infection associated with glucocorticoids is lower when the daily dose of prednisone does not exceed 10 to 15 mg. A propensity score-matched study using insurance claims data from 9911 adults with RA undergoing elective total knee or hip arthroplasty found that glucocorticoid use was associated with a dose-dependent increase in postoperative risk for hospitalized infection and prosthetic joint infection [5]. Compared with patients who received no glucocorticoids within 90 days of knee or hip replacement surgery, patients who received more than 10 mg of prednisone equivalent daily had a higher predicted risk for hospitalized infection (13.25 versus 6.78 percent) and a higher predicted one-year cumulative incidence of prosthetic joint infection (3.83 versus 2.09 percent). Another population-based study including 381 knee or hip arthroplasties performed in 259 patients with RA found that glucocorticoid doses over prednisone 15 mg (or equivalent) per day were associated with a 20-fold increase in the risk of post-arthroplasty infection [6]. Being overweight and having coronary artery disease were also risk factors for infection.

There is controversy regarding the role of supraphysiologic (or "stress dose") glucocorticoids in the perioperative setting. There are limited data regarding the optimal approach to managing patients at risk for glucocorticoid-induced adrenal insufficiency, and practices vary among clinicians [1,7,8]. Much of the data come from surgical trials that are not specific to patients with rheumatic diseases. Our approach to glucocorticoid use in the perioperative setting is discussed in detail separately. (See "The management of the surgical patient taking glucocorticoids".)

Prolonged use of glucocorticoids may cause other complications related to surgery, including:

●Impaired wound healing

●Increased friability of skin and superficial blood vessels

●Hematoma or skin ulceration due to mild pressure

●Increased susceptibility of the skin to tears from adhesive tape

●Increased risk of fracture, infections, gastrointestinal hemorrhage, or ulcer

Nonsteroidal antiinflammatory drugs — To avoid the antiplatelet effect of nonsteroidal antiinflammatory drugs (NSAIDs) and aspirin during surgery, these drugs should be stopped prior to surgery. A detailed discussion related to the timing and duration of discontinuation of NSAIDs in the perioperative period can be found elsewhere. (See "Perioperative medication management", section on 'Aspirin' and "Perioperative medication management", section on 'Nonsteroidal antiinflammatory drugs'.)

The cardiovascular risks associated with the use of nonselective and cyclooxygenase (COX)-2 selective NSAIDs are discussed in detail elsewhere. (See "NSAIDs: Adverse cardiovascular effects".)

Nonbiologic DMARDs — Although a reasonable concern exists about the potential of nonbiologic disease-modifying antirheumatic drugs (DMARDs) to increase the risk of infection by affecting the immune response [9,10], stopping DMARDs prior to surgery may result in a flare-up of disease activity, which may adversely affect rehabilitation. In patients taking nonbiologic DMARDs for the treatment of rheumatic diseases, we continue the current dose of methotrexate (MTX), leflunomide, hydroxychloroquine, apremilast, and/or sulfasalazine throughout the perioperative period. In clinical practice, the nonbiologic DMARD dose is often missed for one day and up to three days while the patient is hospitalized.

Several studies of RA patients undergoing elective orthopedic surgery have found that continued use of MTX through the perioperative period is safe [11-13]. As an example, a systematic review including four studies with RA patients undergoing elective orthopedic surgery evaluated the effects of continuing MTX versus stopping MTX in the perioperative period [13]. Continued MTX therapy was safe perioperatively and was associated with a reduced risk of flares. There was no evidence to suggest that stopping MTX preoperatively reduced the incidence of infection or improved wound healing. However, in all of the studies, the mean dose of MTX was less than 15 mg per week. Limited data on the use of leflunomide during the perioperative period are conflicting [14,15]. In one study, there were significantly more wound complications in patients taking leflunomide at the time of elective orthopedic surgery compared with patients on MTX [15]. However, another small study compared patients who continued leflunomide for four weeks prior to arthroplasty versus those who withheld leflunomide and found no difference between the groups [14]. There are also limited data suggesting it is safe to continue hydroxychloroquine and sulfasalazine in the perioperative period. In a retrospective study of 367 orthopedic surgeries among 204 RA patients, two-thirds of whom were receiving nonbiologic DMARDs including hydroxychloroquine and sulfasalazine, there was no increased infection associated with nonbiologic DMARD use [16].

Biologic DMARDs — In patients taking biologic DMARDs for rheumatic diseases, we withhold the biologic medication and plan the elective surgery at the end of the dosing cycle for that specific medication. These agents should not be restarted until external wound healing is complete, which is typically around two weeks postoperatively.

As an example, patients taking weekly etanercept should aim to schedule the surgery in the second week after the first withheld dose. Patients taking adalimumab in two-week intervals should plan the surgery in the third week after the first withheld dose. In a similar manner, patients on monthly intravenous abatacept should schedule the surgery in the fifth week after the first withheld dose. Patients taking rituximab should wait until month 7 after the last dose to schedule the surgery, presumably when B cells have returned to the circulation. However, we do not delay nonelective procedures in patients who have been more recently treated. Additional information regarding the use of these agents for the treatment of rheumatic diseases can be found elsewhere. (See "Overview of biologic agents in the rheumatic diseases".)

There is relatively little evidence available regarding the optimal timing for use of biologic DMARDs in the perioperative period. Our approach is largely based on indirect evidence suggesting that biologic DMARDs are associated with an increased risk of infection in general [17-19] (see "Overview of biologic agents in the rheumatic diseases"). However, limited data have not demonstrated a clear risk of complications with these agents in the perioperative period.

●Risk of postoperative complications – Many [20-24], but not all [25,26], retrospective studies suggest that the use of biologic DMARDs such as tumor necrosis factor (TNF) inhibitors does not increase the risk of postoperative complications (eg, infection or impaired wound healing). As examples:

•Findings from a meta-analysis of cohort studies including data from 7344 patients on biologic DMARDs for systemic rheumatic diseases who were undergoing orthopedic surgery suggested that continuing biologic DMARDs did not appear to be associated with an increased risk of surgical site infections (odds ratio [OR] 1.11, 95% CI 0.82-1.49) and wound complications (OR 2.16, 95% CI 0.48-9.85); however, stopping biologics prior to surgery was associated with a higher risk of disease flares (26 versus 7 percent) [27]. These findings, however, are limited by low-quality evidence from observational studies.

•In a subsequent retrospective review that included 1464 patients followed for an average of five years after hip replacement, the need for revision surgery was not significantly different among patients who had received perioperative TNF inhibitors (2.5 percent) versus perioperative conventional synthetic DMARDs (3 percent) [28]. There were also no differences in the risk of perioperative infection or aseptic prosthetic joint loosening.

●Comparative risk of biologic DMARDs – Limited data suggest that the comparative infectious risk across biologic DMARDs is similar. Conclusions regarding perioperative safety are largely based on trials in nonoperative patients showing that the infection rate was similar to that in patients receiving placebo [29]. In addition:

•A propensity score-matched study using insurance claims data from 9911 adults with RA undergoing elective total knee or hip arthroplasty who had a recent infusion of or prescription for abatacept, adalimumab, etanercept, infliximab, rituximab, or tocilizumab found that risks for hospitalized infection, prosthetic joint infection, and readmission after arthroplasty were similar among the biologic agents [5].

•The infectious risks of abatacept are similar to those of TNF inhibitors and other biologic agents, but there are no trials that have examined its safety perioperatively [17,30]. A case series described eight uncomplicated surgeries in seven RA patients on abatacept [31].

•Similarly, there is no direct evidence regarding the safety of the interleukin 1 (IL-1) receptor inhibitor anakinra in the perioperative period. (See "Overview of biologic agents in the rheumatic diseases", section on 'Abatacept' and "Overview of biologic agents in the rheumatic diseases", section on 'Anakinra'.)

Janus kinase inhibitors — In patients taking a Janus kinase (JAK) inhibitor (such as tofacitinib, baricitinib, and upadacitinib), we withhold the JAK inhibitor for at least three days prior to surgery. JAK inhibitors are used in the management of several forms of inflammatory arthritis and other inflammatory disorders. The approach is based on the short half-life of the JAK inhibitors as well as limited data suggesting a rapid increase in disease activity after interrupting therapy, suggesting a reversal of the immunosuppressive effects [32]. However, given the known increased risk of infection associated with tofacitinib [33], withholding JAK inhibitors for a longer period of time (eg, seven days) prior to surgery, particularly in a patient with a history of infections or prior to a prosthetic joint replacement, may be preferable. Information regarding the efficacy and safety of JAK inhibitors is presented separately. (See "Treatment of rheumatoid arthritis in adults resistant to initial biologic DMARD therapy", section on 'JAK inhibitors' and "Overview of the Janus kinase inhibitors for rheumatologic and other inflammatory disorders".)

Other SLE-specific medications — There is uncertainty regarding the optimal perioperative medication management in patients with SLE given the lack of data. Medications other than glucocorticoids typically used for SLE patients include hydroxychloroquine, belimumab, MTX, mycophenolate mofetil, azathioprine, cyclosporine, and tacrolimus. Given the clinical spectrum of SLE disease severity and organ involvement, the decision to withhold medications prior to surgery should be made on an individual basis. Thus, for patients with severe SLE and multiorgan involvement in which discontinuation of the medication may result in a disease flare, it is reasonable to continue the medications through the surgical period. This is based on indirect evidence from organ transplant patients that supports continuing anti-rejection therapy during the time of surgery [34,35]. However, if a patient has mild SLE, withholding medications seven days prior to surgery is also reasonable. More data are needed to help guide perioperative medication management in SLE patients.

DISEASE-SPECIFIC ISSUES — Certain problems related to particular rheumatic diseases can be anticipated, such as possible atlantoaxial instability in severe, chronic rheumatoid arthritis (RA). Some disease manifestations that can impact perioperative management in patients with Sjögren's disease, RA, juvenile idiopathic arthritis (JIA), ankylosing spondylitis, psoriatic arthropathy, hemophilic arthropathy, systemic lupus erythematous (SLE), and systemic sclerosis (SSc) are described below. Many of these manifestations will not be present in patients whose disease is mild or well controlled with treatment.

Sjögren's disease — Ocular and oral dryness usually accompany Sjögren's disease. Issues surrounding the management of this condition in the patient undergoing surgery include the following:

●Pilocarpine should not be given in the perioperative period to avoid the risk of bronchospasm, bradycardia, involuntary urination, vomiting, hypotension, and tremor; such manifestations may be confused with postoperative complications.

●Lubricating gel and artificial tears are recommended during and after anesthesia to prevent corneal drying and abrasion.

●To prevent exacerbating ocular and oral dryness, the use of anticholinergic medications should be minimized.

Rheumatoid arthritis — RA is a multisystem disease with wide-ranging implications for perioperative care.

●The systemic and nonarticular manifestations of RA, including atherosclerotic coronary and peripheral vascular disease, heart failure, and pulmonary parenchymal disease, may affect the plans for the management of anesthesia, intraoperative monitoring, and postoperative care. These issues are discussed separately. (See "Overview of the systemic and nonarticular manifestations of rheumatoid arthritis", section on 'Cardiac disease' and "Overview of pleuropulmonary diseases associated with rheumatoid arthritis".)

●Some patients with RA have an increased risk of atlantoaxial subluxation and neurologic damage. Risk factors associated with C1-C2 instability include glucocorticoid use, seropositivity, nodular disease, and erosive peripheral joint disease. A soft collar will not stabilize the cervical spine, but it usually reminds the staff to be gentle, particularly when the patient is sedated or unconscious. Lateral cervical radiographs with flexion and extension views of the cervical spine may detect significant subluxation and should be obtained prior to surgery in patients with neck pain or neurologic symptoms or those without symptoms if at high risk [36,37]. (See "Cervical subluxation in rheumatoid arthritis".)

●Cricoarytenoid joint disease may cause difficulty with intubation or airway obstruction after extubation. The physical symptoms of cricoarytenoid arthritis are dyspnea, hoarseness, dysphagia, odynophagia, sense of fullness in the throat, pain with speech, and radiation of pain to the ears. Investigation may be necessary and is discussed elsewhere. (See "Overview of pleuropulmonary diseases associated with rheumatoid arthritis", section on 'Upper airway obstruction'.)

●If multiple joints require replacement, careful timing is necessary to allow optimal rehabilitation. As an example, upper-extremity stabilization may be necessary to enable crutch ambulation prior to lower-extremity surgery. (See "Total joint replacement for severe rheumatoid arthritis".)

●Neutropenia also occurs in those with Felty syndrome. Most commonly, such patients do well without any intervention; however, if neutrophil counts are extremely low and result in recurrent infection, granulocyte-colony stimulating factor (G-CSF) can be administered. (See "Drug therapy in Felty syndrome".)

●Patients with RA who undergo total hip or knee replacement are at increased risk of prosthetic joint infection, which is further increased in the setting of revision arthroplasty and a previous prosthetic joint infection [38]. These findings highlight the importance of perioperative prophylactic measures and vigilance during the postoperative period [39]. (See "Total joint replacement for severe rheumatoid arthritis", section on 'Infection'.)

Juvenile idiopathic arthritis — Since children are skeletally immature, growth disturbances can occur after operation among those with JIA.

●Joint replacement arthroplasty is usually delayed until patients are skeletally mature.

●Because life expectancy of young patients is greater than that of the prostheses, surgeons may recommend osteotomy as an alternative procedure to joint replacement for those who have a stable joint with a useful functional range of movement, good muscle strength, and some remaining articular cartilage.

●Cervical spine involvement and micrognathia associated with temporomandibular joint involvement can make endotracheal intubation difficult.

●Patients with JIA have higher short-term complication rates after orthopedic surgery when compared with other inflammatory arthritides [40]. This highlights the importance of perioperative optimization and close monitoring for perioperative complications.

Ankylosing spondylitis — Problems related to spinal and costovertebral ankylosis may include the following:

●A substantial risk of operative and perioperative complications if spinal involvement is more than minimal.

●An increased risk of aortic regurgitation and possibly cardiovascular disease. (See "Clinical manifestations of axial spondyloarthritis (ankylosing spondylitis and nonradiographic axial spondyloarthritis) in adults", section on 'Cardiovascular disease'.)

●Pulmonary disease, including restrictive lung disease related to diminished chest wall and spine mobility, and parenchymal disease. (See "Clinical manifestations of axial spondyloarthritis (ankylosing spondylitis and nonradiographic axial spondyloarthritis) in adults", section on 'Pulmonary disease'.)

●Endotracheal intubation may be difficult; flexible scope intubation may be required for severely affected patients. (See "Flexible scope intubation for anesthesia".)

●Positioning for surgery should be planned preoperatively for patients with severe kyphotic deformities, with preparation for the necessary positioning supports and padding. (See "Patient positioning for surgery and anesthesia in adults", section on 'Trial positioning'.)

●Extensive ligamentous calcification and heterotopic ossification may make regional anaesthesia difficult.

●Postoperative heterotopic ossification may limit total hip arthroplasty success [41]. (See "Treatment of axial spondyloarthritis (ankylosing spondylitis and nonradiographic axial spondyloarthritis) in adults", section on 'Surgery' and "Complications of total hip arthroplasty", section on 'Heterotopic ossification'.)

Psoriatic arthritis — Issues in psoriatic arthritis are principally related to skin disease or associated spondylitis:

●There is a risk of flare of psoriatic skin disease at the operative site (Koebner's phenomenon)

●Stress of surgery may increase the risk of a generalized flare of psoriasis

Systemic lupus erythematosus — Since many patients with SLE have multiorgan involvement, an increased risk of postoperative complications, such as wound infection, renal insufficiency, and pulmonary embolus, may be observed. In addition, hip arthroplasty in patients with SLE is associated with a higher risk of postoperative mortality compared with the general population or patients with RA [42-44].

Cytopenias are common among patients with SLE but often do not require treatment. Additional information regarding appropriate management of cytopenias can be found elsewhere. (See "Hematologic manifestations of systemic lupus erythematosus", section on 'Cytopenias'.)

Antiphospholipid syndrome — Patients with antiphospholipid syndrome (APS) are generally treated with anticoagulation. The treatment of patients with APS and the management of anticoagulation during surgery or an invasive procedure are discussed elsewhere. (See "Management of antiphospholipid syndrome" and "Perioperative management of patients receiving anticoagulants".)

Systemic sclerosis (scleroderma) — A number of the manifestations of SSc may increase perioperative risk and/or may affect intraoperative and postoperative management.

●Patients with SSc are at increased risk of pulmonary hypertension, which is associated with a high risk of perioperative complications. Preoperative echocardiography may be indicated for moderate- to high-risk procedures. (See "Evaluation of perioperative pulmonary risk", section on 'Pulmonary hypertension' and "Treatment and prognosis of pulmonary arterial hypertension in adults (group 1)", section on 'Surgical or periprocedural care'.)

These patients are also at increased risk of heart failure, conduction block, and arrhythmias, all resulting from fibrosis, and myocardial ischemia, due to non-epicardial coronary artery disease. (See "Clinical manifestations and diagnosis of systemic sclerosis (scleroderma) in adults", section on 'Gastrointestinal involvement'.)

●Since lung involvement is associated with increased postoperative mortality, preoperative pulmonary function tests and arterial blood gases may be indicated, depending on the complexity of the planned surgery. (See "Clinical manifestations, evaluation, and diagnosis of interstitial lung disease in systemic sclerosis (scleroderma)".)

●Scleroderma renal disease may cause hypertension or renal crisis. (See "Kidney disease in systemic sclerosis (scleroderma), including scleroderma renal crisis".)

●Endotracheal intubation may be difficult due to decreased mouth opening and limited neck extension.

●Patients with SSc are at risk of aspiration with induction of anesthesia, due to esophageal disease and problems with gastric motility. The risk of aspiration must be balanced with the risk of difficult intubation when deciding whether to perform rapid sequence induction and intubation. (See "Clinical manifestations and diagnosis of systemic sclerosis (scleroderma) in adults", section on 'Gastrointestinal involvement'.)

●There may be a prolonged response to local anesthetic agents when used for peripheral nerve blocks [45].

●Intravenous access may be difficult due to skin thickening.

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Preoperative evaluation and perioperative management of patients with rheumatic diseases" and "Society guideline links: Rheumatoid arthritis".)

SUMMARY AND RECOMMENDATIONS

●We perform a thorough preoperative evaluation, including a careful history and physical examination, with targeted laboratory studies and imaging procedures, depending upon the underlying medical conditions or planned surgery. As examples, patients with rheumatoid arthritis (RA) should be evaluated for cervical spine involvement and cardiovascular disease, and patients undergoing elective joint replacement surgery should receive special attention to potential sources of infection. Preoperative laboratory testing should be based on patient-specific factors and the planned surgical procedure. (See 'Preoperative evaluation' above and 'History and physical examination' above and 'Laboratory testing' above.)

●Perioperative care in patients with rheumatic disease requires special attention to the following issues (see 'General management principles' above):

•Assessing disease activity and optimizing the medication regimen (see 'Assessment of disease activity' above)

•Recognizing the increased cardiovascular risk associated with rheumatic diseases (see 'Cardiac risk assessment' above)

•Addressing states of altered coagulation in patients with antiphospholipid syndrome (APS) (see 'Management of thrombophilia in antiphospholipid syndrome' above)

•Minimizing risk of surgical site infections in the setting of immunosuppressive medications (see 'Medication management' above)

●The optimal strategy for managing immunosuppressive therapies during the perioperative period is unknown due to the lack of evidence. Management of the major categories of medications used for the treatment of various rheumatic diseases in the perioperative setting is largely based on low-quality evidence and expert opinion. A rheumatologist should be involved in the medication management around the time of surgery. (See 'Medication management' above.)

•The major concerns with regard to glucocorticoid use in the setting of surgery are: (1) the risk of surgical site infections and (2) the risk of hemodynamic instability (secondary to glucocorticoid-induced adrenal insufficiency). Ideally, the lowest possible dose of glucocorticoids should be used in the perioperative setting in order to minimize these risks. (See 'Glucocorticoids' above and "The management of the surgical patient taking glucocorticoids".)

•To avoid the antiplatelet effect of nonsteroidal antiinflammatory drugs (NSAIDs) and aspirin during surgery, these drugs should be stopped prior to surgery. (See 'Nonsteroidal antiinflammatory drugs' above and "Perioperative medication management", section on 'Aspirin' and "Perioperative medication management", section on 'Nonsteroidal antiinflammatory drugs'.)

•In patients taking nonbiologic disease-modifying antirheumatic drugs (DMARDs) for the treatment of rheumatic diseases, we continue the current dose of methotrexate (MTX), leflunomide, hydroxychloroquine, apremilast, and/or sulfasalazine throughout the perioperative period. (See 'Nonbiologic DMARDs' above.)

•In patients taking biologic DMARDs for rheumatic diseases, we withhold the biologic medication and plan elective surgery at the end of the dosing cycle for that specific medication. As an example, patients taking weekly etanercept should schedule the surgery in the second week after the first withheld dose. These agents should not be restarted until external wound healing is complete, which is typically around two weeks. (See 'Biologic DMARDs' above.)

•In patients taking a Janus kinase (JAK) inhibitor, we withhold the JAK inhibitor for at least three days prior to surgery. (See 'Janus kinase inhibitors' above.)

•There is uncertainty regarding the optimal perioperative medication management in patients with systemic lupus erythematosus (SLE) given the lack of data. Medications typically used for SLE patients include mycophenolate mofetil, azathioprine, cyclosporine, and tacrolimus. Given the clinical spectrum of SLE disease severity and organ involvement, the decision to withhold medications prior to surgery should be made on an individual basis. (See 'Other SLE-specific medications' above.)

●Attention should be given to the approaches needed to address the particular disease-related risks associated with the following disorders:

•Sjögren's disease (see 'Sjögren's disease' above)

•RA (see 'Rheumatoid arthritis' above)

•Juvenile idiopathic arthritis (JIA) (see 'Juvenile idiopathic arthritis' above)

•Ankylosing spondylitis (see 'Ankylosing spondylitis' above)

•Psoriatic arthritis (see 'Psoriatic arthritis' above)

•SLE (see 'Systemic lupus erythematosus' above)

•APS (see 'Antiphospholipid syndrome' above)

•Systemic sclerosis (SSc; scleroderma) (see 'Systemic sclerosis (scleroderma)' above)