Systemic lupus erythematosus

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

Drug	Indication	Anesthetic implications
Aspirin/NSAIDs (ibuprofen, naproxen sodium)	Antiphospholipid syndrome SLE arthritis	Peptic ulceration Platelet inhibition Renal impairment Fluid retention/electrolyte disturbance Hepatic dysfunction Bronchospasm
Antimalarial drugs (hydroxychloroquine)	Cutaneous SLE Pleuritis/pericarditis Arthritis Reduced renal flares	Retinotoxicity Neuromyotoxicity Cardiotoxicity
Corticosteroids (prednisone, methylprednisone, topical preparation)	Cutaneous SLE Nephritis Pleuritis/pericarditis Diffuse alveolar hemorrhage Neuropsychiatric SLE Mesenteric vasculitis SLE pancreatitis	Hyperglycemia Hypercholesterolemia Hypertension Osteoporosis
Immunosuppressants
Cyclophosphamide	Nephritis Neuropsychiatric SLE	Myelosuppression Pseudocholinesterase inhibition Cardiotoxicity Leucopenia Hemorrhagic cystitis
Azathioprine	Arthritis	Myelosuppression Hepatotoxicity
Methotrexate	Arthritis Cutaneous SLE	Myelosuppression Hepatic fibrosis/cirrhosis Pulmonary infiltrates/fibrosis
Mycophenolate mofetil	Nephritis Hemolytic anemia, thrombocytopenia	GI upset Pancytopenia

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