SLE

Infections in SLE :

- Patients with SLE may be functionally asplenic and at risk from encapsulated bacterial infections like meningococcemia.
- Immunosuppred patients are at increased risk of sepsis
- Infection can mimic a lupus flare
- ESR and CRP may be raised. CRP changes more acutely, and the ESR lags behind disease changes.
- Measurement of complement may be useful as C3 and C4 levels are reduced in patients with active SLE due to consumption by immune complex–induced inflammation.

SLE - Challenges in management in pregnancy

1. Differentiating signs and symptoms of normal pregnancy from those of exacerbations SLE
2. Differentiate malar rash from chloasma
3. Differentiate proteinuria secondary to preeclampsia from proteinuria due to lupus nephritis. Lupus nephritis is often associated with proteinuria and cellular casts whereas only proteinuria is seen in patients with preeclampsia.
4. Differentiating renal disease secondary to an exacerbation of SLE from preeclampsia.
5. Differentiating thrombocytopenia in pregnancy from thrombocytopenia of SLE exacerbation

SLE

Pulmonary features of SLE

• Pleurisy with pleuritic chest pain +/- pleural effusions is the commonest feature
• Pulmonary embolism, lupus pneumonitis, chronic lupus interstitial lung disease, pulmonary hypertension, alveolar hemorrhage and infection are other complications

GI features

• Abdominal pain in active SLE may be due to peritonitis, pancreatitis, mesenteric vasculitis or bowel infarction

Cardiac features

• Pericarditis is the most common cardiac feature, presenting with positional chest pain that is relieved on leaning forward.
• Myocarditis and pulmonary hypertension can present with breathlessness and chest pain
• Coronary artery disease presents with angina

SLE - Rx & effects

Maternal effects :
- Treatment with cyclophosphamide may lead to premature ovarian failure and infertility

- Increased risk of hypertensive disorders: Pre-eclampsia occurs in ~15% of women and may be difficult to distinguish from lupus nephritis

- Increased risk of UTI

- Increased risk of gestational diabetes

- VTE – risk especially increased in women with anti-phospholipid antibodies. 

1. Women already on warfarin should be switched to therapeutic LMWH as soon as the pregnancy is recognized. 
2. Women with antiphospholipid syndrome but no previous VTE should be treated with prophylactic LMWH and low-dose aspirin during subsequent pregnancies. 
3. Women with SLE + antiphospholipid antibodies but no past morbidity can be managed with low-dose aspirin +/- hydroxychloroquine.

SLE - preconceptional counselling

Preconception management :

1. Assess disease activity from history, examinations and investigations

2. Risk assessment by checking for antiphospholipid antibodies (for a risk of fetal loss) and for anti-Ro and anti-La antibodies (for a risk of neonatal lupus) should be performed before pregnancy.

3. If active disease, advise to defer pregnancy and continue effective contraception

4. Discuss effect of SLE on pregnancy management and outcome. 

5. Women with anti-phospholipid antibody syndrome will require more frequent monitoring than those with SLE alone

6. Discuss effect of pregnancy on SLE

7. Discuss terratogenic effects of drugs such as methotrexate, cyclophosphamide and mycophenolate. 
Due to long half-lives, these drugs should be stopped at least 3 months before the planned conception.

8. Women should use contraception while on immunosuppressive disease-modifying drugs such as mycophenolate and cyclophosphamide. 
 Cyclophosphamide is absolutely contraindicated during pregnancy.

9. Discuss drugs that need to be stopped as soon as pregnancy is detected, such as warfarin.

10. Discuss general measures including folic acid supplementation, screening for rubella immunity

SLE - Diagnosis

Diagnosis of SLE :

SLE is diagnosed if the patient has biopsy-proven lupus nephritis with anti-nuclear antibodies or anti-dsDNA antibodies, or the patient has four of the criteria below, including at least one clinical and one immunologic criterion.

Clinical criteria :

•     Serositis – pleurisy or pericarditis

•     Oral or nasopharyngeal ulcers

•     Arthritis - two or more peripheral joints with tenderness or swelling

•     Photosensitivity

•     Blood disorders - Leukopenia, lymphopenia, thrombocytopenia, hemolytic anemia

•     Renal involvement - Proteinuria (>0.5 g/24h) or cellular casts

•     Neurologic disorder - seizures or psychosis in the absence of other causes

•     Malar rash

•     Discoid rash

SLE in pregnancy - maternal risks

MATERNAL RISKS :

• Tendency for disease to relapse during pregnancy

• Increased risk of hypertensive disorders

• Increased risk of VTE especially in women with anti-phospholipid antibodies

• Tendency for renal disease to worsen during pregnancy, especially proteinuria. 
   Long-term prognosis of renal disease is not altered by pregnancy

• Increased risk of placental abruption

• Psychosis may be the presenting symptom

• Chorea is a very rare complication of SLE in pregnancy

• There is no evidence that prednisolone is terratogenic in humans. Hydralazine and methyldopa used over a prolonged period can induce an SLE-like syndrome but are not contra-indicated.

SLE in pregnancy - Fetal risks

FETAL RISKS :

• Increased risk of miscarriage

• Increased risk of fetal death in-utero

• Increased risk of IUGR

• Increased risk of pre-term delivery although the risk of pre-term labour is not increased

• Neonatal lupus erythrematosus occurs in ~1:20,000 neonates and is associated with heart block, skin and haematological abnormalities