Anesthesia in systemic lupus erythematosus: a review of key considerations and surgical challenges

Abstract

Systemic Lupus Erythematosus (SLE) presents complex challenges in the perioperative setting due to its multisystem involvement, immune-mediated pathology, and heterogeneous presentation. This narrative review explores the pathogenesis of SLE and its surgical implications, emphasizing how immune dysregulation, vascular endothelial dysfunction, and immunosuppressive therapy influence anesthetic management. It discusses the importance of comprehensive preoperative assessment, focusing on cardiovascular, pulmonary, renal, neurological, and hematological systems, to guide individualized anesthetic planning. Special attention is given to pharmacotherapy management, perioperative monitoring, and strategies to prevent disease flares. The role of spinal, epidural, and general anesthesia in surgical procedures and obstetric care for SLE patients is analyzed, highlighting benefits, risks, and precautions. By synthesizing multidisciplinary strategies and tailoring anesthetic approaches to SLE’s variability, this review provides a framework to guide clinicians in optimizing perioperative safety for SLE patients. Future research should focus on standardized protocols and novel monitoring technologies to enhance outcomes.

Introduction

Systemic Lupus Erythematosus (SLE) is a chronic autoimmune disease with a prevalence of 20–150 per 100,000, predominantly affecting women of childbearing age (Lisnevskaia et al. 2014). Characterized by immune dysregulation, autoantibody production, and multisystem inflammation, SLE’s heterogeneous presentation impacts organs such as the kidneys, heart, lungs, and hematologic system, posing significant perioperative challenges (Lisnevskaia et al. 2014; Tsokos 2011). The increasing surgical burden in autoimmune populations, driven by disease-related complications (e.g., lupus nephritis, cutaneous lesions) and unrelated procedures (e.g., elective orthopedic or obstetric surgeries), underscores the need for tailored anesthetic strategies (Stull et al. 2023; Hannon et al. 2021). Complications like renal impairment, cardiovascular disease, pulmonary involvement, and thrombotic risks, particularly in patients with Antiphospholipid Syndrome (APS), necessitate individualized approaches (Ben-Menachem 2010; Gualtierotti et al. 2018). Immunosuppressive therapies, such as corticosteroids, increase infection risk, suppress the adrenocortical axis, and delay wound healing, while surgical stress may exacerbate disease activity (Zisa and Goodman 2022; Wang et al. 2013). Regional anesthesia, such as spinal or epidural techniques, potentially avoids airway manipulation, benefiting patients with rare laryngeal or cervical involvement, though evidence on flare reduction is limited (Vyas et al. 2014). This narrative review synthesizes evidence-based strategies for anesthesia management in SLE, focusing on preoperative evaluation, anesthetic technique selection, pharmacotherapy adjustments, and postoperative monitoring to guide clinicians.

Methods

This narrative review was conducted through a structured search of PubMed, Scopus, Web of Science, and Google Scholar, covering literature from January 2000 to March 2024. Search terms included “Systemic Lupus Erythematosus,” “anesthesia,” “perioperative management,” “regional anesthesia,” “spinal anesthesia,” and “pregnancy.” Inclusion criteria were: (1) peer-reviewed articles in English; (2) clinical trials, observational studies, reviews, and case reports focused on anesthetic practices in adult SLE patients; (3) articles addressing perioperative or intraoperative management. Exclusion criteria were: (1) articles unrelated to anesthesia; (2) studies on pediatric populations or basic immunological mechanisms without clinical correlation; (3) non-English articles. A total of 120 articles were screened, with 56 included after excluding duplicates (n = 32), irrelevant studies (n = 20), and non-English articles (n = 12). No formal quality assessment was performed, but priority was given to high-impact journals and recent publications. Data were manually extracted and categorized into thematic domains: general anesthetic implications, regional and general anesthesia considerations, obstetric challenges, and organ-specific risks. The review aims to provide anesthesiologists with a clinically applicable guide for managing SLE patients.

Preoperative considerations

Pathogenesis and surgical implications

Systemic Lupus Erythematosus (SLE) results from immune dysregulation, leading to autoantibody production and immune complex deposition, causing inflammation and tissue damage (Lisnevskaia et al. 2014). This systemic inflammation impairs wound healing, increases infection risk, and elevates thrombosis propensity, particularly in APS patients (Glueck et al. 1985; Bazzan et al. 2015). Vascular endothelial dysfunction may complicate intraoperative management, affecting tissue perfusion (Moschetti et al. 2022). Surgical stress may exacerbate disease activity, with studies noting flares post-surgery, particularly in patients with high Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) scores (> 8), a validated tool assessing clinical and laboratory disease activity (Katz et al. 2023; Adamichou and Bertsias 2017). The SLEDAI evaluates 24 descriptors (e.g., rash, arthritis, proteinuria, seizures), assigning weighted scores (1–8) based on severity, with higher scores indicating greater disease activity. A more detailed overview of the SLEDAI scoring system, including common severity cutoffs relevant to perioperative risk stratification and flare prevention, is provided in (Table 1). Other scoring systems, such as the British Isles Lupus Assessment Group (BILAG) index, assess organ-specific activity but are less commonly used perioperatively due to complexity (Adamichou and Bertsias 2017). Corticosteroids, which suppress the adrenocortical axis, further increase infection and adrenal insufficiency risks (Wang et al. 2013). Preoperative optimization, including disease stabilization and thromboprophylaxis, is critical (Boyce and Massicotte 2020). A flowchart for preoperative planning is provided (Fig. 1) (Table 2).

Table 1.

Surgical outcomes in systemic lupus erythematosus patients

Outcome	Incidence	Risk Factors	Management	Reference
Disease Flares	Varies post-surgery	High SLEDAI, surgical stress	Preoperative optimization, regional anesthesia	Adamichou and Bertsias 2017; Jindal et al. 2013)
Thrombosis	15–30% in APS	APS, immobility	Anticoagulation, compression devices	Al-Homood 2012)
Infections	Varies	Immunosuppression	Prophylactic antibiotics for high-risk patients	Danza and Ruiz-Irastorza 2013)

Fig. 1.

Preoperative planning and anesthesia choice algorithm for patients with systemic lupus erythematosus

Table 2.

Overview of the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) Scoring System and Severity Cutoffs SLEDAI (most commonly SLEDAI-2 K) is a validated global score comprising 24 weighted clinical and laboratory descriptors assessed over the preceding 10–30 days. Higher scores indicate greater disease activity and correlate with flare risk in the perioperative period (Adamichou and Bertsias 2017). Cutoffs are approximate and should be interpreted together with clinical context and organ-specific evaluation

Total Score Range	Disease Activity Level	Clinical/Perioperative Implications	Key Notes/Examples of Descriptors
0	Inactive/Remission	Minimal flare risk; standard preoperative optimization	No active descriptors
1–5	Mild	Low perioperative risk; routine monitoring sufficient	Rash, arthritis, mild mucocutaneous (weighted 2 points)
6–10	Moderate to High	Increased flare risk post-surgery; consider intensified optimization or delay of elective procedures	Proteinuria, thrombocytopenia, serositis (weighted 4 points); higher scores signal need for multidisciplinary input
≥ 11	Severe/High activity	High risk of flares, organ involvement, and complications; strong consideration for preoperative stabilization	Seizure, psychosis, vasculitis, major organ involvement (weighted up to 8 points); maximum total score = 105

Diagnosis and clinical features relevant to anesthesia

Effective preoperative evaluation requires understanding SLE’s systemic nature. Diagnosis relies on laboratory findings (e.g., Antinuclear Antibody [ANA], anti-double-stranded DNA [anti-dsDNA]) and clinical signs, such as organ-specific involvement (Tables 3) (Ben-Menachem 2010; Aringer 2020). A thorough workup assesses disease activity, organ damage, and perioperative risks. Recommended tests, tailored to symptoms, include complete blood count (CBC), comprehensive metabolic panel (CMP), electrocardiogram (ECG), echocardiography for cardiovascular symptoms, pulmonary function tests (PFTs) for respiratory symptoms, and coagulation studies (e.g., PT/INR, aPTT, lupus anticoagulant) for APS or thrombocytopenia (Ben-Menachem 2010; Nikdoust et al. 2018).

Table 3.

diagnostic methods and clinical features of systemic lupus erythematosus relevant to preoperative anesthesia planning

Diagnostic Method	Feature Identified	Relevance to Anesthesia	Challenges	Recommendations	Reference
Serological Tests	ANA, anti-dsDNA	Confirms autoimmune activity	Non-specific in stable disease	Correlate with clinical findings (e.g., rash, joint pain)	Ben-Menachem 2010; Aringer 2020)
Imaging Studies	Pulmonary/cardiac involvement (e.g., chest CT, echocardiography)	Identifies organ risks	May miss active inflammation	Combine with PFTs or ECG	Alghareeb et al. 2022; Depascale et al. 2021)
Biopsy	Lupus nephritis	Assesses renal damage	Invasive	Use for ambiguous renal symptoms (e.g., proteinuria without clear cause)	Vorobjeva and Pinegin 2014)
Clinical Examination	Rash, joint pain, limited mouth opening, neck mobility, airway edema, Raynaud’s	Indicates disease activity, airway/vascular access issues	Subjective	Assess airway (Mallampati score), use warming for Raynaud’s	Ben-Menachem 2010; Santacruz et al. 2022)
ESR	Inflammation	Signals systemic activity	Non-specific	Monitor trends; ESR > 30 mm/h may indicate active disease	Aringer 2020)
Complement Levels	C3, C4	Reflects immune activity	Variable correlation	Assess in active disease	Aringer 2020)

Organ-specific considerations

SLE’s multisystem involvement requires tailored preoperative evaluation to address organ activity versus damage (Bultink and Lems 2015). Key considerations include:

• Cardiovascular System: Active pericarditis, myocarditis, dysrhythmias, or vasculitis increase arrhythmia and ischemic risks (Alghareeb et al. 2022; Doria et al. 2005). Echocardiography is recommended for symptomatic patients; invasive monitoring (e.g., arterial lines) ensures stability in severe cases (Nikdoust et al. 2018).

• Pulmonary System: Interstitial Lung Disease (ILD) impairs gas exchange, complicating general anesthesia (Depascale et al. 2021; Hannah and D’Cruz 2019). PFTs (spirometry, diffusion capacity [DLCO]) guide low tidal volume (6–8 mL/kg) and positive end-expiratory pressure (PEEP) ventilation strategies to prevent barotrauma (Bevacqua 2015).

• Renal System: Lupus nephritis reduces Glomerular Filtration Rate (GFR), affecting drug choice and dosing (Vorobjeva and Pinegin 2014). Fluid titration and nephrotoxic drug avoidance are essential (Goren and Matot 2015).

• Hematologic System: Thrombocytopenia or APS presents bleeding or thrombotic risks. Platelet counts < 50,000/μL contraindicate regional anesthesia; APS requires bridging anticoagulation (e.g., Low-Molecular-Weight Heparin [LMWH]) (Bauer et al. 2021; Tektonidou et al. 2019). Hematological tests (CBC, PT/INR, aPTT) guide management (Tektonidou et al. 2019).

• Neurological System: Neuropsychiatric Systemic Lupus Erythematosus (NPSLE), including seizures, may increase sensitivity to anesthetics (e.g., propofol); peripheral neuropathy requires ultrasound-guided regional blocks to avoid nerve injury (Hanly 2014) (Table 4).

Table 4.

Anesthetic considerations and management strategies for organ-specific manifestations in systemic lupus erythematosus

Organ System	Manifestation	Anesthetic Consideration	Complications	Management Strategy	Reference
Cardiovascular	Pericarditis, myocarditis, dysrhythmias, vasculitis	Arrhythmia risk	Hemodynamic instability	Echocardiography for symptoms, invasive monitoring if severe	Alghareeb et al. 2022; Doria et al. 2005)
Pulmonary	ILD	Impaired gas exchange	Hypoxia	PFTs, low tidal volume ventilation	Depascale et al. 2021; Hannah and D’Cruz 2019; Bevacqua 2015)
Renal	Lupus nephritis	Altered drug clearance	Fluid overload	Fluid titration, avoid nephrotoxins	Vorobjeva and Pinegin 2014; Goren and Matot 2015)
Hematologic	APS, thrombocytopenia	Thrombosis/bleeding risk	Hematoma	Coagulation profile (CBC, PT/INR, aPTT), bridging therapy	Bauer et al. 2021; Tektonidou et al. 2019)
Neurological	NPSLE, peripheral neuropathy	Altered anesthetic response	Nerve injury	Tailored drug dosing, ultrasound-guided blocks	Hanly 2014)

Management of pharmacotherapy and monitoring

Perioperative pharmacotherapy balances disease control and surgical safety (Boyce and Massicotte 2020; Saunders et al. 2021). A multidisciplinary approach adjusts medications based on disease activity and surgical risk (Tables 5).

Table 5.

Perioperative pharmacotherapy management for systemic lupus erythematosus patients

Medication	Perioperative Strategy	Anesthetic Implication	Risks	Reference
Corticosteroids	Continue; stress-dose hydrocortisone (50 mg for moderate, 100 mg for major surgeries, e.g., abdominal, thoracic, orthopedic) if cortisol < 10 μg/dL	Prevents adrenal insufficiency	Hypotension	Wang et al. 2013; Saunders et al. 2021)
Hydroxychloroquine	Continue	Minimal anesthetic impact	None significant	Boyce and Massicotte 2020)
Methotrexate/Biologics	Discontinue 1–2 weeks pre-major surgery, resume post-healing	Reduces infection risk	Flare risk	Saunders et al. 2021)
Antibiotics	Prophylactic cefazolin for high-risk patients (e.g., on immunosuppressives)	Reduces infection	Allergic reactions	Danza and Ruiz-Irastorza 2013)

Infection risk

Immunosuppression increases infection risk, varying by patient and surgery type. Prophylactic antibiotics (e.g., cefazolin) and sterile techniques are standard for high-risk patients (e.g., on immunosuppressives, recent infections) (Danza and Ruiz-Irastorza 2013). Preoperative CBC assesses infection risk in patients on immunosuppressives, typically once unless clinical changes occur (Danza and Ruiz-Irastorza 2013).

Flare prevention

High SLEDAI scores (> 8) indicate flare risk; preoperative optimization (e.g., low-dose prednisone) is recommended (Adamichou and Bertsias 2017). Regional anesthesia may reduce systemic stress, with limited evidence on flare reduction (Vyas et al. 2014).

Monitoring

Lupus nephritis patients require urine output (> 0.5 mL/kg/h) and creatinine monitoring every 6–12 h; arterial lines are used for cardiovascular instability. Postoperative infection surveillance includes daily wound inspections, fever monitoring, and CBC for 48–72 h (Danza and Ruiz-Irastorza 2013).

Intraoperative management

Benefits and precautions in regional and general anesthesia

Regional anesthesia (spinal/epidural) offers effective pain management and potentially avoids airway manipulation, benefiting patients with rare laryngeal edema or cervical limitations (Vyas et al. 2014; Jindal et al. 2013). It promotes hemodynamic stability in patients with pericarditis or myocarditis (Weidner et al. 2021). However, thrombocytopenia (< 50,000/μL) or anticoagulation (e.g., LMWH) increases epidural hematoma risk, requiring a comprehensive coagulation workup (CBC, PT/INR, aPTT, lupus anticoagulant) (Bauer et al. 2021). Vertebral deformities from corticosteroids may complicate placement (Bultink and Lems 2015).

General anesthesia is indicated for major surgeries (e.g., abdominal, thoracic) or when regional anesthesia is contraindicated (e.g., severe thrombocytopenia). It requires preoperative airway assessment (e.g., Mallampati score, neck mobility) due to potential difficulties from rare laryngeal involvement (Kravets et al. 2025). Ventilation strategies (low tidal volume, PEEP) mitigate pulmonary risks in ILD patients (Bevacqua 2015).

Spinal and epidural anesthesia in labor and pregnancy

Regional anesthesia is preferred in SLE patients during labor for pain relief and reduced systemic stress (Jindal et al. 2013). It potentially avoids airway manipulation and maintains hemodynamic stability (Weidner et al. 2021). APS increases thrombosis risk, requiring LMWH cessation 24 h before epidural placement for surgical safety; thrombocytopenia (< 50,000/μL) contraindicates regional techniques (Bauer et al. 2021). Multidisciplinary planning with obstetricians and rheumatologists ensures maternal and fetal safety, with continuous fetal monitoring and use of fetal-safe medications (e.g., fentanyl over NSAIDs) (Saulescu et al. 2022) (Table 6).

Table 6.

Anesthetic implications and precautions for managing systemic lupus erythematosus during pregnancy and labor

Parameter	Detail	Anesthetic Implications	Risks	Precautions	Reference
Autoimmune Activity	Flares in pregnancy	Increased anesthetic complexity (e.g., altered drug response)	Newborn distress	SLEDAI monitoring	Adamichou and Bertsias 2017; Jindal et al. 2013)
Corticosteroids	Perioperative use	Adrenal insufficiency risk	Hypotension	Stress-dose hydrocortisone	Wang et al. 2013; Saunders et al. 2021)
Antimalarials	Continued use	Minimal impact	None significant	Maintain therapy	Boyce and Massicotte 2020)
Coagulation	APS	Thrombosis risk	Hematoma	Coagulation workup, LMWH cessation	Bauer et al. 2021; Al-Homood 2012)
Fetal Well-being	Hypoxia risk	Drug selection (e.g., fentanyl over NSAIDs)	Preterm labor	Continuous fetal monitoring	Saulescu et al. 2022; Dao and Bermas 2022)

Postoperative management and recovery

Postoperative care focuses on pain, infection, thrombosis, and organ function (Katzap et al. 2013). Regional anesthesia (e.g., epidural catheters) with acetaminophen minimizes systemic effects; Non-Steroidal Anti-Inflammatory Drugs (NSAIDs) are avoided in lupus nephritis due to nephrotoxicity (Chitnis et al. 2020). Prophylactic antibiotics (e.g., cefazolin) reduce infection risk in high-risk patients (e.g., on immunosuppressives) (Danza and Ruiz-Irastorza 2013). APS patients receive LMWH and compression devices; coagulation profiles (CBC, PT/INR, aPTT) guide therapy (Al-Homood 2012). Renal function is monitored via urine output (> 0.5 mL/kg/h) and creatinine every 6–12 h; cardiovascular monitoring (ECG for 24–48 h) is used for symptomatic patients (Lin et al. 2015). Psychosocial support addresses postoperative anxiety, enhancing recovery (Warchol-Biedermann et al. 2022; Williams et al. 2017).

Perioperative workflow

Description: The flowchart begins with a preoperative assessment box that includes SLEDAI scoring, organ-specific evaluations (e.g., echocardiography, PFTs), and coagulation studies. It then branches based on thrombocytopenia (< 50,000/μL) and APS status. The APS-positive node has clearly labeled “Yes” and “No” arrows. For APS-positive patients, anticoagulation status is assessed; neuraxial techniques are considered only when coagulation parameters are normal after appropriate holding of anticoagulation. The “contraindications to regional anesthesia” node under lower-body/obstetric surgery now has two clear outcome arrows leading to specific choices. All decision paths terminate in explicit recommendations: “Regional Anesthesia” or “General Anesthesia.” An additional tip “Adjust Ventilation for ILD if needed” is placed under the General Anesthesia branch. The postoperative management section is presented as a symmetric box at the bottom, mirroring the preoperative box at the top. Regional anesthesia is preferred for lower-body/obstetric surgeries if coagulation is normal; general anesthesia is selected for major surgeries or contraindications to regional techniques.

Discussion

Perioperative management of SLE requires addressing its heterogeneity and treatment-related risks. Regional anesthesia reduces systemic stress and potentially avoids airway manipulation (Vyas et al. 2014). General anesthesia requires careful airway planning due to potential laryngeal involvement (Khokhar et al. 2015). APS treatment complicates regional anesthesia due to anticoagulation, necessitating a coagulation workup (Bauer et al. 2021). Infection risk varies by patient, requiring tailored antibiotic prophylaxis (Danza and Ruiz-Irastorza 2013). Thrombosis rates in APS patients (15–30%) underscore anticoagulation needs (Al-Homood 2012). Variability in institutional practices across countries, due to differences in healthcare systems and guideline adoption, complicates standardized care (Goodman and Bass 2018). Current guidelines lack consensus on optimal SLEDAI thresholds for surgical deferral or specific monitoring protocols, highlighting the need for global standardization (Zisa and Goodman 2022). Gaps remain in standardized protocols due to SLE’s variability, warranting future longitudinal studies to explore flare predictors (e.g., SLEDAI) and novel monitoring technologies.

Conclusion

Perioperative management of Systemic Lupus Erythematosus (SLE) demands tailored strategies to address its multisystem complexities. This review provides a framework for preoperative assessment (e.g., SLEDAI, tailored testing), preference for regional anesthesia where feasible, and vigilant postoperative monitoring to mitigate infections and thrombosis. A multidisciplinary approach helps optimize outcomes. Future research should focus on standardized protocols and longitudinal studies to refine management and address gaps in this challenging clinical domain.

Limitations

SLE’s heterogeneity limits generalizability. Reliance on case reports for rare complications (e.g., NPSLE) weakens evidence. Variability in treatment regimens and institutional practices across countries complicates standardized guidelines. Limited data on long-term surgical outcomes, especially in patients with significant organ involvement, highlights the need for large, multicenter studies to evaluate novel anesthetic techniques and develop evidence-based protocols. Additionally, as a narrative review, this work does not include a formal systematic literature search or quantitative meta-analysis, which limits the strength of evidence synthesis.

Future prospects

Precision medicine, including genomic profiling and biomarker discovery, could improve risk stratification and perioperative care. Novel immunomodulatory therapies and enhanced imaging may reduce complications. Multidisciplinary research on anesthesia-immune interactions could mitigate surgical stress. Standardized protocols and large-scale registries will inform evidence-based practices, enhancing long-term outcomes for SLE patients.

Abbreviations

ANA

Antinuclear Antibody

APS

Antiphospholipid Syndrome

CV

Cardiovascular

GFR

Glomerular Filtration Rate

ILD

Interstitial Lung Disease

IV

Intravenous

LMWH

Low-Molecular-Weight Heparin

NPSLE

Neuropsychiatric Systemic Lupus Erythematosus

NSAIDs

Non-Steroidal Anti-Inflammatory Drugs

PFTs

Pulmonary Function Tests

SLE

Systemic Lupus Erythematosus

SLEDAI

Systemic Lupus Erythematosus Disease Activity Index

VTE

Venous Thromboembolism

Authors’ contributions

A.M wrote the main manuscript text and V.F Supervised the writing process. All authors reviewed the manuscript.

Funding

None.

Data availability

No datasets were generated or analysed during the current study.

Declarations

Ethics approval and consent to participate

Not applicable. This narrative review article did not involve primary human or animal research.

Consent for publication

Not applicable.

Competing interests

The authors declare no competing interests.