Clinical Review

Management of lupus flare

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References

The intravenous route is preferred because of its rapid response, though long-term outcome is probably not altered.

Without warning, a catastrophe

Even a healthy lupus patient who fulfills all the accepted criteria for a safe pregnancy can take a disastrous turn

A 28-year-old G0 with SLE since age 11 presented for preconception consultation. She was on no medications, with normal blood pressure and no evidence of disease activity for more than 2 years. Physical examination and laboratory findings were normal, including serum creatinine 0.7 mg/dL; less than 30 mg protein in a 24-hour urine collection; creatinine clearance 110 mL/min; and lupus anticoagulant, anti-cardiolipin antibodies, anti-Ro, and anti-La were negative.

Green light

One year later, she returned for follow-up and to inform her obstetrician that she was getting married and wished to conceive. She had no SLE activity since her last visit. Repeat laboratory studies were unchanged. She was given medical clearance to attempt conception, and told that she met all the criteria that would make her a suitable candidate for pregnancy.

7 weeks All findings normal

Three months later, a single intrauterine gestation of approximately 7 weeks was confirmed. Laboratory studies and physical examination were normal, and she reported no SLE-related symptoms.

11 weeks Lupus flare

Four weeks later, at her next prenatal visit, a 3+ proteinuria and blood pressure of 140/90 mm Hg were noted. Her rheumatologist made a diagnosis of lupus flare with probable nephritis. Oral prednisone was begun, with rapid taper. Clinical response was good. She remained on prednisone, 10 mg/day.

14 weeks Recurrence

A recurrence of lupus flare with probable nephritis was diagnosed and her oral prednisone dose was increased. One week later the patient seemed to worsen. She was admitted for steroid pulse therapy. Initially, she improved, but then continued to worsen.

16 weeks Cyclophosphamide therapy

After counseling, she was begun on cyclophosphamide, but her condition continued to deteriorate. Renal function worsened and the patient, now with nephrotic proteinuria, was profoundly edematous and hypoalbuminemic with a rising serum creatinine.

18 weeks Dilatation and evacuation

Ultrasound evaluation of the fetus revealed evidence of early growth restriction. After much discussion, the patient underwent dilatation and evacuation.

Cerebral infarct and anticoagulation

Her lupus flare did not abate. More aggressive medical therapy ensued. Transfer to the intensive care unit with intubation was necessary. She subsequently had an ischemic cerebral infarct requiring anticoagulation.

The next 7 weeks Lupus remission

Over the next several weeks, the patient improved. She had some residual sequelae from the cerebral infarct, but was doing well, with her lupus flare in remission. She was responding well to rehabilitation therapy.

Fatal cerebrovascular accident

One day before anticipated discharge to home, she had a massive cerebrovascular accident and died.

A vivid reminder

This case vividly illustrates the difficulties we must be prepared to manage in lupus pregnancies.

The foremost concern is that even the best candidates for pregnancy can have unfavorable outcomes when this highly unpredictable disease flares.

These women can become severely ill. Ideally, their care should be provided at a facility where expertise in maternal-fetal medicine, anesthesiology, rheumatology, neonatology, and critical care medicine can be readily mobilized to deal with the occasionally catastrophic complications. Even with all of this expertise available, maternal and fetal mortality will not be preventable in all cases.

Pregnancy termination does not necessarily result in amelioration of the lupus flare or its sequelae.

Patients with SLE must be informed of the unpredictability of this disease during pregnancy. The entire family, where appropriate and desired, should be included in the information-sharing process.

A team approach, both pre- and postconception, will help to maximize (but not guarantee) the likelihood of a successful outcome for mother and child.

Refractory flares: Focus on damage control

In pregnancy, most lupus flares involve nephritis and the systemic effects of nephritis, such as hypertension, proteinuria, and renal failure. In some cases, however, steroid pulse therapy fails to suppress these sequelae, or recurrent flares seem to become refractory to steroid pulse therapy.

Any evidence for pregnancy termination? In such cases it is essential that appropriate medical decisions be made on behalf of the mother. No conclusive data suggest that pregnancy termination ameliorates a lupus flare, although some anecdotes suggest this possibility.

The mainstay of management is to aggressively treat the lupus flare before irreparable maternal harm occurs.8

Early delivery: When and how

In advanced pregnancies it may be worthwhile considering early delivery so that the fetus may be spared any adverse consequences of maternal cytotoxic therapy, which would be the next step in management.

Amniocentesis for gestations earlier than 34 weeks may assist in guiding the decision for betamethasone administration for the purpose of accelerating maturation of fetal lungs.

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