Systemic Lupus Erythematosus Management
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Direct answer
Hydroxychloroquine belongs in every lupus prescription: it reduces flares, damage accrual and mortality, and it continues straight through pregnancy. Mild disease (skin and joints) adds sun protection, NSAIDs used with care, and short steroid bursts; moderate disease adds methotrexate, azathioprine or mycophenolate; organ-threatening disease — proliferative nephritis, CNS involvement, alveolar haemorrhage — gets pulse methylprednisolone 500–1000 mg plus an induction agent: mycophenolate 2–3 g daily or low-dose intravenous cyclophosphamide (the Euro-Lupus regimen of 500 mg fortnightly to a 3 g total), with belimumab or voclosporin as validated add-ons. Maintenance is mycophenolate or azathioprine with prednisolone tapered to 5 mg daily or less, ideally to zero.
What you must remember
- Classification: EULAR/ACR 2019 — ANA at least 1:80 as the mandatory entry criterion, then 10 or more weighted points with at least one clinical domain; SLICC 2012 — four criteria including one clinical and one immunological, or biopsy-proven lupus nephritis with positive ANA or anti-dsDNA.
- Serology with meaning: anti-dsDNA tracks activity and nephritis; anti-Sm is highly specific; anti-Ro links subacute cutaneous lupus, neonatal lupus and congenital heart block; low C3 and C4 signal active immune-complex disease.
- Lupus nephritis histology: ISN/RPS class III and IV (proliferative) demand induction therapy; class V (membranous) presents as nephrotic syndrome.
- Euro-Lupus regimen: cyclophosphamide 500 mg intravenously two-weekly for six doses — as effective as high-dose regimens in the trial population and far kinder to fertility, which matters in young Indian women.
- Voclosporin (AURORA) and belimumab (BLISS) are validated add-ons to mycophenolate for proliferative nephritis.
- Pregnancy-safe list: hydroxychloroquine, azathioprine up to 2 mg/kg, tacrolimus, cyclosporine and low-dose aspirin; mycophenolate, cyclophosphamide and methotrexate must stop.
- Anti-Ro pregnancies need foetal echocardiography between 16 and 26 weeks for congenital heart block.
- Drug-induced lupus (anti-histone positive): hydralazine, procainamide, minocycline and TNF inhibitors — kidney and CNS are spared, and it resolves on withdrawal.
Worked example: proliferative lupus nephritis
A 24-year-old woman presents with periorbital oedema, blood pressure 150/95, urine dipstick showing 3+ protein and dysmorphic erythrocytes, low C3 and a high-titre anti-dsDNA. Step one: quantify — urine protein-to-creatinine ratio and serum creatinine. Step two: biopsy, because the class changes both the plan and its intensity. Step three: induce — class IV with crescents gets mycophenolate 2–3 g daily (fertility concerns in a young woman favour it over cyclophosphamide), pulse methylprednisolone followed by oral prednisolone tapering toward 5 mg by three to six months, with voclosporin or belimumab added for higher-risk histology or slow response. Step four: define success early — proteinuria falling below about 0.7–0.8 g per day within twelve months predicts good long-term renal survival, and failure at that checkpoint prompts re-biopsy or escalation. Step five: maintain for years on mycophenolate or azathioprine (azathioprine if she conceives), hydroxychloroquine throughout, with vigilance for the two great Indian companions of chronic immunosuppression — tuberculosis and hepatitis B reactivation.
Where students slip
Cyclophosphamide is reached for in mild lupus; it is reserved for organ-threatening disease, and using it for joint symptoms alone is an exam wrong-answer. Hydroxychloroquine is stopped at conception, when the correct move is to continue it, since it reduces maternal flares and neonatal rash. Serology is treated as the disease itself: rising anti-dsDNA in a clinically quiet patient is "serologically active, clinically quiescent" — observe, do not escalate. And anti-dsDNA-negative lupus exists; the antibody is specific, not obligatory. Sun protection, vitamin D and inactivated vaccines complete the supportive answers examiners reward.
Frequently asked questions
Which class of lupus nephritis needs immunosuppressive induction?
ISN/RPS class III and IV proliferative nephritis; class V membranous disease with nephrotic-range proteinuria also merits immunosuppression.
What is the Euro-Lupus cyclophosphamide regimen?
500 mg intravenously every two weeks for six doses (3 g total), followed by mycophenolate or azathioprine maintenance.
What maintenance prednisolone dose should be targeted?
5 mg daily or less, with complete withdrawal the aim, per current EULAR guidance.
Which drugs are safe across an SLE pregnancy?
Hydroxychloroquine, azathioprine up to 2 mg/kg, tacrolimus, cyclosporine and low-dose aspirin; mycophenolate and cyclophosphamide are not.
Which antibody predicts congenital heart block?
Anti-Ro (SSA), with or without anti-La — screen with foetal echocardiography between 16 and 26 weeks of gestation.