Learning objectives

• Describe systemic lupus erythematosus
• Understand the potential complications associated with systemic lupus erythematosus
• Anesthetic management of a patient with systemic lupus erythematosus

Definition and mechanisms

• Systemic lupus erythematosus (SLE) is a chronic autoimmune connective tissue disorder with a heterogeneous presentation and systemic involvement in which tissues and multiple organs are damaged by pathogenic autoantibodies and immune complexes
• There are often periods of illness (flares) and periods of remission during which there are few symptoms

Signs and symptoms

• Fatigue
• Fever
• Joint pain, stiffness, and swelling
• Butterfly-shaped rash on the face that covers the cheeks and bridge of the nose or rashes elsewhere on the body
• Skin lesions that appear or worsen with sun exposure
• Fingers and toes that turn white or blue when exposed to cold or during stressful periods
• Shortness of breath
• Chest pain
• Dry eyes
• Photosensitivity
• Headaches, confusion, and memory loss
• Hair loss
• Mouth ulcers
• Swollen lymph nodes

Risk factors

• Gender: Female > male 
• Age: 15-45 years
• Race: More common in African Americans, Hispanics, and Asian Americans

Causes

SLE is probably caused by a genetic susceptibility coupled with an environmental trigger, resulting in a defect in the immune system 

• Genetics: Mutations in HLA-DR2/HLA-DR3 genes, complement genes, cytokine genes, etc.
• Environmental: UV-light (UV-A, UV-B), viruses (e.g., EBV, CMV, retroviruses), drugs (e.g., minocycline, hydralazine, procainamide), hormones (e.g., estrogen, prolactin), heavy metals

Complications

• Dermatologic: Malar rash, chronic discoid lesions
• Brain and central nervous system: Headaches, dizziness, behavior changes, vision problems, strokes or seizures
• Cardiovascular: Symptomatic pericarditis, pericardial tamponade, myocarditis, Libman-Sacks endocarditis, valvular dysfunction, Raynaud’s phenomenon
• Pulmonary: Pleuritis, pneumonitis, diffuse alveolar hemorrhage, pulmonary arterial hypertension
• Renal: Lupus nephritis, end-stage renal disease
• Hematology: Anemia of chronic disease, autoimmune hemolytic anemia, autoimmune thrombocytopenia
• Gastrointestinal: Oral ulcers, Sjogren’s syndrome, dysphagia, acute abdominal pain, abnormal liver function tests, autoimmune hepatitis
• Musculoskeletal: Arthritis, osteoporosis, fractures, asymptomatic atlantoaxial subluxation
• Infection: SLE and treatment can weaken the immune system
• Pregnancy complications: Increased risk of miscarriage, high blood pressure during pregnancy, and preterm birth

Pathophysiology

Treatment

Management

Preoperative management

• History: Review disease activity index, accrued organ damage, and drug history
• Examination: Thorough examination of cardiovascular, respiratory, and neurological systems, including testing for atlantoaxial subluxation symptoms and signs
• Full blood count: Test for anemia, thrombocytopenia, and leucopenia; consider further testing for hemolysis if anemia is present
• Serum electrolytes, creatinine, urea: Any abnormality requires further investigation for lupus nephritis
• Liver function tests: Abnormalities should prompt review for autoimmune or drug hepatotoxicity
• Coagulation studies: Elevated aPTT requires investigation for the presence of lupus anticoagulant
• Anti-dsDNA, complement levels: May reflect lupus activity after comparison with previous baseline measurements
• Urinalysis: Proteinuria, red cells, white cells, and cellular casts may indicate clinically silent disease and prompt further investigation
• ECG: Silent ischemia, myocarditis, pericarditis, and conduction abnormalities may be identified
• Chest radiograph: Pleural effusion, interstitial pneumonitis, pericardial effusion, or subglottic stenosis may be seen

Intraoperative management

• 5-lead ECG: Accelerated coronary artery disease, conduction abnormalities
• Intra-arterial blood pressure monitoring: Case-dependent, consider in the presence of myocarditis, conduction abnormalities, valvular abnormalities, or autonomic dysfunction
• Laryngeal mask airway if appropriate: Minimize airway manipulation due to the risk of inflammation and post-extubation airway edema
• Difficult airway precautions with immediate access to smaller-size endotracheal tubes: Vocal cord paralysis, subglottic stenosis, or laryngeal edema may make intubation difficult
• Standard antibiotic prophylaxis: Innate susceptibility to infection and immunosuppressive therapy predispose to infection risk
• Caution with muscle relaxants: Azathioprine and cyclophosphamide may interact with muscle relaxants
• Renal protective strategies: Maintain urine output, avoid hypoperfusion and hypotensive states, and use nephrotoxic drugs cautiously due to the possibility of subclinical lupus nephritis
• Careful patient positioning: Predisposition to peripheral neuropathies and osteoporosis
• Antithrombotic prophylaxis: Institute mechanical and pharmacological measures early, especially in the presence of antiphospholipid antibodies
• Eye protection and artificial tears/lubrication: Sjogren’s syndrome may predispose to corneal abrasions despite adequate eye taping
• Temperature monitoring: Hypothermic state may induce vasospasm in patients with Raynaud’s phenomenon
• Pain management: Consider side effects of systemic analgesics; regional techniques may be helpful if neuropathies, myelitis, and coagulopathies are excluded
• Corticosteroid cover: Adrenal suppression may have resulted from long-term corticosteroid therapy with the need for a “stress dose” perioperatively

Postoperative management

• Pain management: Minimize systemic side effects

Keep in mind

• A thorough preanesthetic evaluation is mandatory for safe anesthesia
• The anesthetic plan must be individualized based on the degree of involvement of various systems, current medications, and laboratory results

Suggested reading

• Erez BM. Systemic Lupus Erythematosus: A Review for Anesthesiologists. Anesthesia & Analgesia. 2010;111(3):665-676.