Extra Topic 6.2 -- Systemic Lupus Erythematosus (SLE)

Question 1

What is systemic lupus erythematosus (SLE)?

(A 33-year-old, 5’4” tall, 190 pound, G1P0 female at 38 weeks gestation is scheduled for cesarean section. She reports a history of systemic lupus erythematosus (SLE). Blood pressure = 152/96 mmHg; urine protein 3+; CXR = bilateral pulmonary infiltrates.)

Answer 1

SLE is an autoimmune disease resulting in systemic chronic inflammation (i.e. vasculitis) and tissue damage.

Diagnosis is often difficult and is usually made based on the presence of 3 or more of the following criteria:

1. antinuclear antibodies;
2. characteristic rash (i.e. malar rash and/or discoid rash);
3. nephritis;
4. polyarthritis (symmetrical arthritis involving the hands, wrists, elbows, knees, and/or ankles);
5. hematologic disorder (i.e. thrombocytopenia, hemolytic anemia, etc.);
6. serositis (i.e. pericarditis and/or pleuritis);
7. neurologic disorder (i.e. seizures and/or psychosis); and
8. photosensitivity.

Question 2

The patient is positive for the presence of lupus anticoagulant. Would you employ neuraxial anesthesia for the cesarean section?

(A 33-year-old, 5’4” tall, 190 pound, G1P0 female at 38 weeks gestation is scheduled for cesarean section. She reports a history of systemic lupus erythematosus (SLE). Blood pressure = 152/96 mmHg; urine protein 3+; CXR = bilateral pulmonary infiltrates.)

Answer 2

Lupus anticoagulant is a misnomer because this immunoglobulin does not result in clinical coagulopathy but, rather, is a prothrombotic agent that only causes a prolonged aPTT because of a laboratory artifact.

Therefore, given my preference to avoid general anesthesia in an obese pregnant patient, I would provide neuraxial anesthesia as long as she had no true antibodies to specific coagulation factors (which can result in a significant bleeding diathesis) and/or significant thrombocytopenia (secondary to SLE or preeclampsia).

Moreover, if interstitial lung disease, recurrent pulmonary emboli, and/or cardiomyopathy (all may occur with SLE) had resulted in pulmonary hypertension, I would employ a slowly induced epidural anesthetic, recognizing that a rapid sympathectomy could lead to decreased venous return and a precipitous drop in blood pressure.

Question 3

Given this patient’s medical history, what additional concerns do you have?

(A 33-year-old, 5’4” tall, 190 pound, G1P0 female at 38 weeks gestation is scheduled for cesarean section. She reports a history of systemic lupus erythematosus (SLE). Blood pressure = 152/96 mmHg; urine protein 3+; CXR = bilateral pulmonary infiltrates.)

Answer 3

Given her pregnancy, SLE, elevated blood pressure, proteinuria, and pulmonary infiltrates, I would have multiple concerns, including:

1. potentially difficult airway management, secondary to –
   
   • her pregnancy,
   • airway edema (due to preeclampsia or SLE),
   • cricoarytenoid arthritis (may cause hoarseness, stridor, or airway obstruction), or
   • arthritic involvement of the cervical spine (a rare finding);
2. inadequate coagulation (possibly resulting in excessive perioperative bleeding and/or epidural hematoma), secondary to –
   
   • thrombocytopenia (SLE or preeclampsia),
   • thrombocytopathia (preeclampsia and NSAIDs), or
   • the production of antibodies to specific coagulation factors (SLE);
3. thromboembolic complications (i.e. DVT with pulmonary or cerebral embolism), secondary to –
   
   • pregnancy and/or
   • antiphospholipid antibodies;
4. complicated blood type and cross-match, secondary to –
   
   • the presence of atypical blood antibodies (SLE);
5. pulmonary complications, secondary to –
   
   • thromboembolism and
   • the pulmonary effects of SLE, such as –
     
     • frequent infections,
     • pleural effusions,
     • interstitial lung disease, and
     • lupus pneumonitis;
6. cardiac complications, secondary to –
   
   • pulmonary hypertension,
   • systemic hypertension (SLE or preeclampsia), and/or
   • the cardiac manifestations of SLE, such as –
     
     • premature coronary artery arterosclerosis,
     • coronary vasculitis,
     • pericarditis,
     • myocarditis,
     • cardiomyopathy,
     • aseptic endocarditis,
     • pericardial effusions (cardiac tamponade is rare),
     • conduction abnormalities, and
     • valvular abnormalities (i.e. regurgitation, stenosis, and vegetations);
7. worsening renal function, secondary to –
   
   • preeclampsia and/or
   • lupus nephritis (occurs in 50-60% of patients with SLE);
8. patient positioning issues, secondary to –
   
   • polyarthritis and/or
   • previous joint replacement (often occurs due to osteonecrosis of the femoral head);
9. neurologic complications secondary to –
   
   • preeclampsia or
   • SLE, such as –
     
     • cerebral vasculitis,
     • stroke,
     • seizures,
     • cognitive dysfunction,
     • psychosis, and
     • peripheral neuropathy;
10. maternal or neonatal adrenal suppression, recognizing that –
    
    • steroids are often used in the treatment of SLE;
11. hepatic dysfunction, secondary to –
    
    • HELLP or
    • lupus hepatitis;
12. increased risk of infection, secondary to –
    
    • SLE and
    • treatment with immunosuppressant drugs;
13. deleterious effects of immunosuppressant drugs
    
    • (i.e. cyclophosphamide and azathioprine can both prolong the effects of succinylcholine); and
14. neonatal lupus erythematosus,
    
    • which may occur when maternal autoantibodies cross the placenta and bind to fetal tissue
    • (can cause fetal heart block, cutaneous lupus, and thrombocytopenia).

Question 4

While you are still evaluating the patient, she begins to seize. What do you think is the cause?

(A 33-year-old, 5’4” tall, 190 pound, G1P0 female at 38 weeks gestation is scheduled for cesarean section. She reports a history of systemic lupus erythematosus (SLE). Blood pressure = 152/96 mmHg; urine protein 3+; CXR = bilateral pulmonary infiltrates.)

Answer 4

Given this patient’s SLE and possible preeclampsia (her elevated blood pressure, proteinuria, and pulmonary infiltrates are consistent with both preeclampsia and SLE),

I suspect that her seizure is secondary to –

• eclampsia,
• cerebral vasculitis, or
• cerebral embolism
  
  • (the presence of antiphospholipid antibodies often associated with SLE can lead to venous and arterial thrombosis).

Although less likely, her seizure could also be the result of –

• uterine rupture and
• subsequent amniotic fluid embolism.