|Year : 2016 | Volume
| Issue : 1 | Page : 91-94
Perioperative considerations of systemic lupus erythematosus and antiphospholipid syndrome
Jyotsna Satish Paranjpe, Ravikiran Jangonda Thote
Department of Anesthesia, Bharati Vidyapeeth Deemed University Medical College, Sangli, Maharashtra, India
|Date of Web Publication||22-Dec-2015|
Jyotsna Satish Paranjpe
Department of Anesthesia, Bharati Vidyapeeth Deemed University Medical College, Sangli, Maharashtra
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Paranjpe JS, Thote RJ. Perioperative considerations of systemic lupus erythematosus and antiphospholipid syndrome. Med J DY Patil Univ 2016;9:91-4
|How to cite this URL:|
Paranjpe JS, Thote RJ. Perioperative considerations of systemic lupus erythematosus and antiphospholipid syndrome. Med J DY Patil Univ [serial online] 2016 [cited 2020 Aug 9];9:91-4. Available from: http://www.mjdrdypu.org/text.asp?2016/9/1/91/172444
Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune connective tissue disorder with heterogeneous presentation. SLE is not a rare condition; its estimated prevalence is 1 per 1000 population with male to female ratio of 1:9. , It primarily affects women of childbearing age, also having an ethnic component, black women being affected 3 times more than whites.  Multiple organ dysfunction associated with SLE is caused by autoantibodies and immune complex-mediated tissue destruction.  Although SLE is largely attributed to autoimmune processes, exposure to certain drugs (phenothiazines, procainamide, quinidine, phenytoin, hydralazine) may induce a lupus-like illness or exacerbate SLE. Drug-induced SLE presents only as arthralgias and serositis, which resolves after discontinuation of the drug. ,
Considerable variation exists in the clinical presentation of SLE, ranging from acute features with the classical malar, erythematous "butterfly rash" to a progressive fatal illness most commonly caused by complications of renal, cardiovascular, pulmonary, and central nervous system pathologies. Disruption of normal immunity, chronic inflammation, and immunosuppressive therapy make these patients particularly susceptible for infections, which play an important role in the morbidity and mortality in SLE. 
The American College of Rheumatology and Systemic Lupus International Collaborating Clinics have established criteria for the diagnosis of SLE. A patient must exhibit at least 4 of the 11 features [Table 1]. ,,,,
These standard criteria confer 95% specificity and 85% sensitivity for SLE diagnosis.  Positive antinuclear antibody (ANA) is the most sensitive and optimal test for SLE screening. However, ANA is commonly seen in other autoimmune disorders, while anti-double stranded DNA and anti-Smith antibodies are more specific to SLE. ,, Risk of thromboembolism increases by 53% when antiphospholipid (aPL) antibodies, anticardiolipin (aCL) antibodies, or lupus anticoagulant (LAC) antibodies are detected in SLE patients.  Rising anti-dsDNA titers may predict lupus flares, especially when coupled with decreasing C3/C4 complement levels. ,, LAC activity is caused by autoantibodies to: Beta 2 glycoprotein-I perform coagulation screen to detect prolongation in phospholipid-dependent coagulation assay (activated partial thromboplastin time [aPTT]).  However, a normal aPTT does not exclude LAC. Despite advances, it must be recognized that there is no definitive laboratory test for the diagnosis or monitoring of SLE.
Patients with mild SLE, defined by musculoskeletal and cutaneous involvement, generally are treated with antimalarials, glucocorticoids, and nonsteroidal anti-inflammatory agents. Patients in whom there is major organ involvement, including renal, hematologic, pulmonary, cardiac, and nervous systems, are considered to have moderate to severe SLE. These patients benefit from more intense treatment with immunosuppressive, cytotoxic, and biologic agents with appropriate monitoring for toxicity. In addition, a new drug belimumab, a monoclonal antibody that inhibits B lymphocyte differentiation and autoreactivity, shows promising results in patients with active disease. 
Most of these patients are on long-term steroids and other immunosuppressant drugs like Rituximab, which should be continued preoperatively. Abrupt cessation of glucocorticoids or the stress response associated with surgery could precipitate an addisonian crisis; since they can have hypothalamic pituitary axis (HPA) suppression.  Integrity of HPA axis can be checked by plasma cortisol level and the 250 μg adrenocorticotropic hormone stimulation test. 
Perioperatively, SLE can present major challenges to the anesthesiologist because of unanticipated difficult airway, accrued organ damage, coagulation defects, and complex management regimes. SLE necessitates extensive preoperative assessments of patients, including obtaining detailed histories and physical examinations. Careful anesthetic planning tailored to the individual patient and intraoperative monitoring of all affected organ systems-particularly renal, pulmonary and cardiovascular function are required. Renal or hepatic involvement may affect the metabolism and efficacy of common drugs, including IV and inhaled anesthetics, analgesics, neuromuscular inhibitors, cholinesterase inhibitors, and muscarinic antagonists.  Patients treated with cyclophosphamide may prolong effects of succinylcholine due to its inhibitory effect on cholinesterase. , Patients with SLE may have mucosal ulceration, cricoarytenoid arthritis, laryngeal pathology including recurrent laryngeal nerve palsy, or temporomandibular joint dysfunction that results in a difficult intubation. , In cases of extreme end-organ damage, the use of remifentanil and cisatracurium-both metabolized via processes that are end organ-independent-is indicated.  SLE predisposes to accelerated atherosclerosis and the relative risk of myocardial infarction (MI) is 52 times more in SLE females aged between 35 and 44 years.  aPL antibodies cause MI with normal coronary arteries. Stroke and transverse myelitis are important neuromanifestations presenting acutely or subacutely. 
Perioperative management must be tailored to the individual patient. Spinal block can be given with platelet count >50,000/cumm and epidural block can be administered with platelet count >1,00,000/cumm. American Society of Anesthesiologists task force II recommends transfusing platelets, if platelet count is <20,000/cumm and clinical signs of bleeding are present. 
Strict asepsis should be maintained for invasive procedures such as central line cannulation, arterial line insertion, and intrathecal blocks due to an increased risk of infections.  Maintenance of normothermia by use of warm fluids and covering of the exposed parts of the body decreases the impact of Raynaud's phenomenon.  Use of calcium channel blockers or nitroglycerin which relax the walls of the blood vessels reduce the frequency or severity of attacks.
Antiphospholipid syndrome occurring secondary to SLE needs a special mention and is characterized clinically by recurrent fetal loss (90%), venous thromboembolism (VTE) (deep venous thrombosis, pulmonary embolism), arterial occlusions (stroke, transient ischemic attack).  Diagnosis requires documented vascular thrombosis or recurrent adverse pregnancy outcomes with laboratory evidence of LAC antibodies or aPL (aCL) antibodies, IgG and IgM measured on 2 or more occasions at least 6 weeks apart.  Heparin inhibits complement and protects pregnancy. 
The perioperative management of patients who are receiving antithrombotic therapy is based on (1) an assessment of patient risk for thromboembolism and (2) an assessment of risk for perioperative bleeding [Table 2]. 
Addressing these issues will determine whether antithrombotic therapy is interrupted around the time of the surgery or procedure and if so, whether bridging anticoagulation is considered [Figure 1]. 
| Bridging Anticoagulation|| |
Though there is no universally accepted regimen, bridging strategy for anticoagulation is the administration of a short-acting anticoagulant, consisting of subcutaneous (SC) low-molecular-weight heparin (LMWH) or intravenous (IV) unfractionated heparin (UFH), for 10-12 day period during interruption of Vitamin K antagonist (VKA) therapy when the international normalized ratio (INR) is not within a therapeutic range. If patients have an INR >1.5 preoperatively, it is to be corrected with low-dose (1 mg) oral Vitamin K. 
A high-dose (therapeutic-dose) heparin bridging regimen involves administering an anticoagulant dose that is similar to that used for the treatment of acute VTE or an acute coronary syndrome (e.g., enoxaparin) 1 mg/kg bid or 1.5 mg/kg daily, or IV UFH to attain an aPTT 1.5-2 times the control aPTT. Protamine can be used to reverse effects of UFH monitored by activated clotting time.
A low-dose (prophylactic-dose) heparin regimen involves administering a dose that is used, typically, to prevent postoperative VTE (e.g., enoxaparin 30 mg bid or 40 mg daily, dalteparin 5,000 IU daily, UFH 5,000-7,500 IU bid). Although low-dose LMWH or UFH is effective to prevent VTE after surgery, evidence is lacking about whether such low-dose heparin regimens are effective in preventing arterial thromboembolism, including stroke. 
Low-molecular-weight heparin or UFH should not be resumed at a fixed time after a surgery or procedure without consideration of the anticipated bleeding risk or adequacy of postoperative hemostasis. If therapeutic-dose bridging is used in patients at high risk for postoperative bleeding, its initiation should be delayed for 48-72 h after surgery when adequate surgical hemostasis has been achieved. If bleeding continues beyond 72 h, options include a low-dose heparin bridging regimen or VKA resumption alone without postoperative bridging. UFH may be of particular use in, for example, patients with severe renal insufficiency or dependency on dialysis in whom LMWHs should be avoided. Bridging anticoagulation with UFH may be considered for out-of-hospital administration using a fixed-dose, weight-based, SC regimen (250 IU/kg bid) that does not require aPTT monitoring. 
Since data from randomized controlled trials are lacking, a considerable variability exists in the type of anticoagulant (LMWH or UFH), intensity of anticoagulation (therapeutic dose, low dose or intermediate dose), and timing of perioperative administration.
A close patient follow-up during early postoperative period is necessary since potential thromboembolic or bleeding complications occur during the initial 2 weeks after surgery while patient is at home. In patients who are having a major surgical or other major invasive procedure, interruption of antithrombotic therapy is typically required to minimize the risk for perioperative bleeding. Continuation of VKA therapy or aspirin in the perioperative period confers an increased risk for bleeding. 
Prognosis of SLE has steadily improved, with longer survival resulting in more patients presenting for surgery. Given the heterogeneity of this disease and its ability to affect any organ in the body, anesthetic and perioperative management remains dependent on clinical acumen and understanding of the medical issues at play in these patients.
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[Table 1], [Table 2]