Clinical Review

Management of lupus flare

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References

Whether or not pregnancy increases the incidence of lupus flare is a continuing controversy, stemming from variable definitions of “flare.” Universally accepted criteria have been lacking in published studies.5 Consensus indicates, however, that lupus flares are more common in pregnancy than in nonpregnant controls.

Some studies suggest that SLE flares are more common in the second and third trimesters, but the data are not clear on this point.6 This variability is due in part to differences in disease activity of the patients when they entered the studies.

One may conclude that for any given patient it is impossible to accurately predict whether she will experience a lupus flare, and if she does, when it will occur, and to what level of severity it will rise.

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

Nephritis is the most serious of lupus manifestations, and if not aggressively treated, can lead to nephrotic syndrome, edema, and end-stage renal disease in more than 50% of patients within 2 to 3 years

Potential adverse outcomes

Predicting who will have a lupus flare and its degree of severity may be difficult if not impossible, but there is little doubt that a significant percentage of women with SLE will experience a flare of some degree. How a lupus flare will affect the pregnancy is uncertain, as well. SLE activity in pregnancy has been linked to adverse outcomes ranging from increased risk of miscarriage to preterm delivery.

Diagnosis and initial management

Preventive treatments

Steroids. SLE flares being somewhat more common in pregnancy than in the nonpregnant patient has led to the belief in some centers that prophylactic administration of steroids to prevent flares would have beneficial pregnancy effects. To date, however, no conclusive evidence supports this approach. In fact, steroid use in particular has been associated in some series with higher rates of premature rupture of membranes (both term and preterm), preeclampsia, and gestational diabetes.

Hydroxychloroquine. It has been suggested that SLE patients whose disease has been controlled with hydroxychloroquine need not discontinue this therapy due to the pregnancy.

The risks of this agent in pregnancy—which are not thought to be significant—are far outweighed by the potential maternal and fetal benefits of averting a lupus flare.

The differential diagnosis

It is imperative, before starting a management strategy, to determine if in fact a lupus flare is the correct diagnosis. Many features of a lupus flare can be confused with features of normal pregnancy, or pregnancy associated complications such as preeclampsia (TABLE 2). The differential diagnosis includes most commonly preeclampsia, but diagnoses such as immune thrombocytopenia, poststreptococcal glomerulonephritis, and hemolyticuremic syndrome must also be considered.

TABLE 2

Clinical features of preeclampsia vs lupus flare*

FEATUREPREECLAMPSIALUPUS FLARE
HypertensionPresentPresent
ProteinuriaPresentPresent
EdemaPresentPresent
Malar rashAbsentPresent
ArthritisAbsentPresent
PhotophobiaAbsentPresent
Oral ulcersAbsentPresent
SerositisAbsentPresent
SeizuresPresentPresent
*Denoting presence or absence does not suggest absolute presence or absence, but rather, the likely compatibility with the diagnosis.

Is it lupus flare or preeclampsia?

The most frequent cause for uncertainty is whether the diagnosis is lupus flare or preeclampsia. It is important to find their distinguishing features, because therapy for these 2 conditions is radically different.

A few easy tests can help (TABLE 3), but the most important are:

  • positive ANA screen,
  • active urinary sediment,
  • hypocomplementemia (C3, C4, or CH 50—the latter is an assay of total serum hemolytic complement), and
  • high titers of anti-ds-DNA.
Additional tests for anticardiolipin antibody, anti-Ro and anti-La, and lupus anticoagulant may be of some prognostic importance, but do not help differentiate a lupus flare from other similar entities.7

TABLE 3

Tests that help tell lupus flare from preeclampsia

TESTPREECLAMPSIALUPUS FLARE
PTTUsually normalMay be abnormal
Platelet countNormal or reducedNormal or reduced
UrinalysisNormal sedimentActive sediment
ANAUsually negativeUsually positive
Anti-ds-DNAUsually negativeUsually positive
ASTMay be abnormalMay be abnormal
ALTMay be abnormalMay be abnormal
Lupus anticoagulantNegativeMay be positive
HemolysisMay be presentUsually absent
Complement levelsUsually normalUsually reduced
WBCIncreasedDecreased
SFlt-1*IncreasedNormal
*Investigational and not widely available for clinical use.

Aggressive drug therapy is imperative

Management of lupus flare depends on aggressive drug therapy. The choice of therapy is determined by whether the patient is currently on an immunosuppressive regimen, and if so, the types and doses of medications, and whether this is her first flare during the pregnancy.

The usual initial therapy is glucocorticoids, or the so-called steroid “pulses,” typically consisting of very high doses of steroids administered either orally or intravenously over a 3-day period. This strategy has had some success in ameliorating lupus flares, particularly lupus nephritis.

Dosage. Methylprednisolone, 1,000 mg/day intravenously, for 3 days followed by oral prednisone, 0.5 to 1.0 mg/kg per day, provides better survival than lower steroid doses in patients with diffuse proliferative glomerulonephritis.

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