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Imitators of Severe Pre-Eclampsia
Imitators of Severe Pre-Eclampsia
Baha M. Sibai, MD
There are many obstetric, medial, and surgical disorders that share many of the clinical and
laboratory findings of patients with severe pre-eclampsia– eclampsia. Imitators of severe
pre-eclampsia– eclampsia are life-threatening emergencies that can develop during preg-
nancy or in the postpartum period. These conditions are associated with high maternal and
perinatal mortalities and morbidities, and survivors may face long-term sequelae. The
pathophysiologic abnormalities in many of these disorders include vasospasm, platelet
activation or destruction, microvascular thrombosis, endothelial cell dysfunction, and
reduced tissue perfusion. Some of these disorders include acute fatty liver of pregnancy,
thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, acute exacerbation of
systemic lupus erythematosus, and disseminated herpes simplex and sepsis syndromes.
Differential diagnosis may be difficult due to the overlap of several clinical and laboratory
findings of these syndrome. It is important that the clinician make the accurate diagnosis
when possible because the management and complications from these syndromes may be
different. Because of the rarity of these conditions during pregnancy and postpartum, the
available literature includes only case reports and case series describing these syndromes.
This review focuses on diagnosis, management, and counseling of women who develop
these syndromes based on results of recent studies and my own clinical experience.
Semin Perinatol 33:196-205 © 2009 Elsevier Inc. All rights reserved.
196 0146-0005/09/$-see front matter © 2009 Elsevier Inc. All rights reserved.
doi:10.1053/j.semperi.2009.02.004
Imitators of severe pre-eclampsia 197
ascites and bleeding from severe coagulopathy. Because of Table 1 Imitators of Severe Pre-eclampsia/HELLP Syndrome
these findings, the diagnosis may be initially confused with ● AFLP
pre-eclampsia.1,2,6 Neurologic findings may range from nor- ● TTP
mal to lethargy, agitation, confusion, and even coma.8,9,13,14 ● HUS
● Exacerbation of lupus erythematosus
Laboratory Findings ● Catastrophic antiphospholipid syndrome
The complete blood count usually reveals hemoconcentra- ● Systemic viral sepsis (disseminated herpes)
● Systemic inflammatory response syndrome/septic shock
tion, elevated white blood count, and platelet count that is
● Other conditions (cholestasis of pregnancy, necrotizing
initially normal but may be low.1-6 Coagulation findings re- pancreatitis, etc.)
veal low fibrinogen, prolonged prothrombin time (PT), and
low levels of antithrombin.1-10 These abnormalities are re-
lated to reduced production of these factors by the liver and
are consistent with disseminated intravascular coagulopathy is not possible to be performed once the tissue has been
(DIC). In contrast, the DIC seen in severe pre-eclampsia and submitted to routine paraffin blocks. Liver biopsy should be
abruptio placentae is due to abnormal consumption.15 Serum performed only after correction of coagulopathy. However,
electrolytes will reveal evidence of metabolic acidosis with this is rarely used in clinical practice, and the diagnosis is
elevated creatinine and uric acid values. Blood sugar may be usually made based on clinical and laboratory findings.1-9
normal but is usually low in the postpartum period.1-6,8,9,13 In addition to the other conditions listed in Box I, the
Blood sugars may also be elevated in association with second- differential diagnosis of AFLP should include idiopathic cho-
ary pancreatitis.1,8 Liver enzymes, such as AST, ALT, alkaline lestasis of pregnancy, Budd-Chiari syndrome, adult-onset
phosphatase, and bilirubin, will be elevated. The increase in Reye’s syndrome, and drug-induced hepatic toxicity (acet-
bilirubin is mainly of the conjugated form, with levels usually aminophen overdose, tetracycline-induced toxicity, anticon-
exceeding 5 mg/dL. Ammonia levels are also increased, par- vulsant drugs hypersensitivity, and methyldopa hepati-
ticularly in late stage of the disease. Amylase and lipase values tis).1,4,21
may be elevated in the presence of concomitant pancreati-
tis.1,8 Hepatitis profile for A, B, and C will be negative.
Ultrasonography of the liver may reveal the presence of Maternal and Perinatal Complications
increased echogenicity in severe cases; however, it is less AFLP is associated with an increased risk of maternal mortal-
sensitive than computed tomography (CT) and magnetic res- ity and morbidities. In the past, the rate of maternal death was
onance imaging (MRI).16-20 In addition, quantitation of liver close to 70%; however, recent data indicate a mortality rate of
density by ultrasound is subjective and operator-dependent. ⬍10%, but maternal complications remain high. Table 1 descri-
CT scan of the liver may show decrease or diffuse attenuation bes maternal complications in recent series of AFLP.1-11,14,22,23
in the liver. However, none of these techniques are suffi- Recently, Davidson and coworkers reported on three women
ciently sensitive to exclude a diagnosis of AFLP.2,20 with triplet gestation who had AFLP proven by liver biopsy
Liver biopsy is the gold standard for confirming the diag- who survived. They suggested that women with triplets are at
nosis of AFLP. Histopathologic findings in AFLP reveal swol- increased risk for AFLP because of potential for increased
len, pale hepatocytes with central nuclei.21 The diagnosis can production of fatty acid metabolites by three fetuses. It has
be made only on a frozen section liver biopsy with special been suggested that the improved maternal survival in recent
stains for fat, such as oiled red (O).1,4 Plans to perform this years was related to the supportive care and aggressive man-
stain should be made before the biopsy procedure because it agement of serious maternal complications by a multidisci-
plinary group of physicians from various specialties.2-9
Recently, Knight, and coworkers11 reported a prospective
national study of AFLP in the UK. The participants included
57 women with AFLP diagnosed mostly by clinical criteria;
55 had abdominal ultrasound scan, but only 12 (27%) had
either ascites and/or abnormal liver findings. Forty-two
(74%) were diagnosed antepartum and 15 (26%) postpartum
(within 4 days of delivery). In general, most of these patients
had a biochemical diagnosis of AFLP (mild prolongation of
PT, PTT, normal platelets), only 8 (16%) had acute renal
failure, and 4 (7%) required ventilatory support. There was
only 1 maternal death (1.8%) in a patient who received a liver
transplant.14
Both perinatal mortality and morbidities are increased in
patients of AFLP.2-9,20 The perinatal mortality among 160
births (142 pregnancies) in recent case series was 13.1%.9,22
Figure 1 Liver biopsy findings in herpes hepatitis. (Color version of In addition, neonatal morbidity remains high because of the
figure is available online.) high rate of preterm delivery (74%) among reported cases.
198 B.M. Sibai
The average gestational age at delivery was 34 weeks (range, can be used to maintain blood sugars above 60 mg/dL. Ane-
25-42 weeks).2-9,22 mia and DIC should be treated as needed by a packed red
In the study by Knight and coworkers,11 the perinatal mor- blood cells, platelets, and fresh frozen plasma. It is important
tality was 10.4% among 67 infants (6 stillbirths and 1 neo- to aggressively treat maternal hypotension to avoid further
natal death). injury to the liver, kidneys, and other organs. Invasive hemo-
dynamic monitoring may be necessary in some patients to
assess fully the intravascular volumes and maintain cardiac
Management of AFLP output, and in patients who develop acute respiratory dis-
The clinical course of women with AFLP is usually character- tress syndrome (ARDS).
ized by progressive and sometimes sudden deterioration in Pancreatitis is a potentially lethal complication of AFLP,
maternal and fetal conditions.9 Therefore, patients in whom and thus all patients with AFLP should undergo serial screen-
AFLP is considered require hospitalization in a labor and ing of serum lipase and amylase for several days after the
delivery unit. Fetal heart rate monitoring and/or performing onset of hepatic dysfunction.1,8 Abnormalities in these en-
of a biophysical profile should be performed concurrently zymes typically appear after hepatic and renal dysfunction.
with maternal evaluation. Evidence of fetal compromise may The development of pseudocysts with secondary infections
be present even in those with stable maternal conditions. or hemorrhagic pancreatitis with resultant retroperitoneal
Nonreassuring fetal testing may be secondary to maternal bleeding increases the risk for maternal death.8
acidosis and/or reduced uteroplacental blood flow.1-6 The In general, most patients with AFLP will start to improve
presence of maternal acidosis may be reflected in reduced- 2-3 days after delivery. In some cases, however, deterioration
to-absent fetal movement, absent fetal breathing, or tone dur- in liver function tests, renal function, mental status, and co-
ing biophysical profile testing.1 agulopathy may continue for about 1 week. In such cases,
The next step in management is to confirm or exclude the some authors suggest using plasmapheresis to improve ma-
diagnosis of AFLP according to the clinical findings and re- ternal outcome. This method of treatment was recently re-
sults of blood tests.1,23 The presence of bleeding and/or severe ported in six such cases with no maternal death. However,
coagulopathy requires transfusion with fresh frozen plasma the value of such therapy remains unclear. In rare cases, a
and other blood products as needed. The ultimate treatment patient will progress into fulminant hepatic failure, requiring
for this condition is maternal stabilization and delivery be- liver transplantation.21
cause there are no reports of women recovering from AFLP
spontaneously. The presence of AFLP is not an indication for
delivery by cesarean section because of the risks of bleeding
Counseling of Women with AFLP
complications in the presence of coagulopathy.2-6,10 The de- Several case reports and case series22-26 have noted an asso-
cision to perform cesarean delivery should be based on fetal ciation between the development of AFLP and/or HELLP
gestational age, fetal condition, and presence of labor. Induc- syndrome and a deficiency of long-chain 3-hydroxyacyl-co-
tion of labor with an attempt for vaginal delivery within 24 enzyme A dehydrogenase (LCHAD) in infants born to
hours is a reasonable approach. Because of the associated women with the above complications. This disorder of mito-
coagulopathy, most anesthesiologists will avoid epidural an- chondrial fatty acid oxidation might lead to significant in-
algesia. Maternal analgesia during labor can be provided by crease in maternal fatty acid levels that are highly toxic to the
intermittent use of small doses of systemic opioids. The use of liver. Based on these findings, some authors suggest that
pudendal block should be avoided because of the risk of women with AFLP as well as their partners and children
bleeding and hematoma formation into this area. Caution should undergo molecular testing for Glu 474 Gln mutation
should be exercised to avoid vaginal trauma and lacerations in the LCHAD.22,24 Screening for this mutation would allow
during vaginal delivery.1 early diagnosis and treatment in newborns of affected moth-
In case of cesarean delivery, the patient should receive ers and would allow counseling about subsequent pregnan-
general anesthesia. It is advisable to avoid incisions that re- cies.22,25 The risk of recurrence is increased in women who
quire extensive dissection, such as the Pfannenstiel incision, are carriers for this mutation, particularly if the fetus is also
and meticulous attention should be given to secure hemosta- affected during a subsequent pregnancy. There are few case
sis. It is advisable to perform a midline incision, to use a reports describing recurrent AFLP in women without the
subfascial drain, and to keep the skin incision open for at above mutation2,4; however, the risk of this recurrence re-
least 48 hours to avoid hematoma formation.1,23 mains unknown because of the limited number of pregnan-
In the postpartum period, the patient should be monitored cies reported following AFLP in these women.
very closely with careful evaluation of vital signs, intake–
output, and bleeding. Some of these patients may develop
acute refractory hypotensive shock in the immediate postpar-
Thrombotic Microangiopathies
tum period. Serial measurements of hematologic, hepatic, Thrombotic thrombocytopenic purpura (TTP) and hemo-
and renal function should be performed and recorded in an lytic uremic syndrome (HUS) are two microangiopathic dis-
organized fashion. Blood sugars should be monitored every orders that are extremely rare during pregnancy/postpartum.
few hours with a bedside glucometer because of the risk of They are usually reported as case reports or small case se-
hypoglycemia in the postpartum period. Glucose infusions ries.1,26-35 Thus, their expected development during preg-
Imitators of severe pre-eclampsia 199
develop exacerbations or relapse after plasma exchange is APAs (lupus anticoagulant and/or anticardiolipin antibod-
stopped.37,41,42 ies) are present in 30-40% of patients with systemic lu-
The treatment of HUS is similar to that of TTP. However, pus.59-61 These patients are at increased risk for thrombotic
the response to plasma infusions is not as favorable, and most events. Patients with lupus and APA are at risk for tissue
patients will require dialysis. ischemia secondary to thromboembolic events and throm-
Delivery is the only cure for patients with HELLP syn- botic microangiopathy, resulting in a clinical picture similar
drome or AFLP. In contrast, pregnancy should be continued to that seen in HELLP syndrome, eclampsia, TTP, and
in patients with TTP or HUS who develop the condition HUS.61-67 Thrombocytopenia is seen in about 40-50% of
remote from term in the absence of fetal compromise and in these cases and hemolytic anemia in 14-23%.61 Cerebral le-
those who respond to plasma infusions and/or plasma ex- sions and symptoms will develop because of cerebral vascu-
change. In rare cases, plasmapheresis results in improve- litis and/or cerebral vaso-occlusive disease. In such patients,
ments in fetal heart rate tracing.50 However, theses patients the clinical and laboratory findings are similar to those with
require close observation of laboratory and clinical findings eclampsia.61 In patients with renal involvement, hyperten-
because of the risk of relapse. Some of the managements used sion and proteinuria are the rule with findings identical to
in such patients have included corticosteroids, antiplatelet severe pre-eclampsia.
agents, weekly plasma infusions, and serial plasma exchange The catastrophic antiphospholipid syndrome occurs in
or dialysis.1,26,33-35 ⬍1% of patients with APA syndrome.60 It is characterized by
acute thrombotic microangiopathy affecting small vessels of
multiple organs (at least three). The most common affected
Counseling of Women with TTP
organs are the kidneys and central nervous system.60,61 Liver
Women who develop TTP during pregnancy should be aware involvement will result in cellular necrosis and infarcts, lead-
of the potential of relapse after delivery as well as of risk of ing to elevated liver enzymes.64-66 The clinical and laboratory
relapse in subsequent pregnancies.28,30,32,34,35,38 Therefore, findings may also be similar to other microvascular angiopa-
these women should be instructed about the symptoms of thies.60-67
early relapse and to report these symptoms immediately.
There are few case reports describing recurrent TTP/HUS in
subsequent pregnancies28,32,34,35,38; however, the risk of this Maternal–Perinatal Outcome
recurrence remains unknown because of limited data. Pregnancy outcome is usually favorable in patients with SLE
who were in remission before pregnancy and who do not
develop a flare during pregnancy.51,57 In addition, the out-
Systemic Lupus Erythematosus come is favorable in those without lupus nephritis and/or
absent antiphospholipid antibodies.52,55-57 However, mater-
Systemic lupus erythematosus (SLE) is an autoimmune dis-
nal morbidities and perinatal mortality and morbidity are
order that is characterized by deposits of antigen–antibody
increased in those with lupus nephritis central nervous sys-
complexes in capillaries and various visceral structures. Most
tem disease and in those with antiphospholipid antibod-
patients are female and of reproductive age (26-40 years
ies.51-55,59-62 These latter pregnancies are associated with high
old).51 The clinical findings may be mild or severe amd may
rates of miscarriage, fetal death (4-19%), intrauterine growth
affect multiple organ systems, including the kidneys (nephri-
restriction, and preterm delivery (38-54%). This high rate of
tis), lungs (pleuritis or pneumonitis), liver (hepatitis), and
fetal loss and perinatal complications is related to decidual
brain.51-59 In patients with lupus nephritis, the clinical and
vascular thrombosis and placental infarctions and hemor-
laboratory findings are similar to those of severe pre-eclamp-
rhage.59-62 Maternal complications include a high rate of early
sia.51-57 Such patients will have hypertension, proteinuria,
onset pre-eclampsia and complications related to thrombo-
and microscopic hematuria. In some of these women, partic-
embolism and microangiopathy.57-61 Maternal morbidities
ularly during an acute exacerbation, patients will have
are substantially increased in those with APA syndrome.58-67
thrombocytopenia. The thrombocytopenia is usually mild to
Maternal mortality is almost 50% in patients who develop the
moderate (⬎50,000/mm3).51-59 Most patients with lupus
catastrophic APA syndrome.61
have skin lesions (typical discoid or malar rash), and joint
symptoms and fever are very common during an acute flare.
During the active phase of SLE exacerbation, laboratory Management
findings will show pancytopenia, thrombocytopenia, hemo- Management of SLE flare during pregnancy will depend on
lytic anemia, and an increase in anti-DNA antibodies. Serum the presence of the organ systems involved, laboratory find-
complement levels may be normal or depressed. Severe lupus ings (thrombocytopenia, APAs), and the presence or absence
flares occur in 25-30%, and it may develop for the first time of nephritis. Treatment usually includes the use of cortico-
during pregnancy or in the postpartum period.51,55 In pa- steroids, low-dose aspirin, hydroxychloroquine immuno-
tients with lupus nephritis who develop active flare during suppressive drugs, and heparin.51-68 The usual dose of ste-
pregnancy, the clinical and laboratory findings are similar to roids is 40-80 mg/day of prednisone, and for aspirin it is 81
those with severe pre-eclampsia and HELLP syndrome.53-56,58 mg/day. Prednisone therapy is usually used in patients with
The exact diagnosis may be difficult, particularly in those lupus nephritis, whereas combined regimens of prednisone
with associated antiphospholipid antibodies (APAs).58-63 and low-dose aspirin are recommended in patients with
202 B.M. Sibai
APAs.59-61,68 Alternative regimens have included the use of nant women. The onset is usually in the third trimester (av-
heparin plus low-dose aspirin. For patients with severe erage onset of 30 weeks) with clinical presentation of fever,
thrombocytopenia that does not respond to the above regi- abdominal pain, and upper respiratory symptoms.69-72 Some
mens, intravenous ␥ globulin may be beneficial.61 Recent patients will have hepatitis without jaundice and encephali-
data also suggested that the use of other agents, such as aza- tis.69,71,72 Hepatitis is a common finding in fulminant sep-
thioprine, cyclosporin, and hydroxychloroquine, may be re- sis.70,72 Hypertension and proteinuria are absent. Laboratory
quired in management of some of these patients.54,59,68 In findings will reveal leukopenia, thrombocytopenia, hemoly-
patients with catastrophic APA syndrome, treatment includes sis, DIC, markedly elevated levels of AST (⬎2000 IU/L), and
full anticoagulation with heparin, steroids, plus plasma- severe elevations in LDH levels. In fulminant cases, the am-
pheresis.60,61,66 monia levels may be elevated; however, bilirubin values are
In summary, the clinical presentations of AFLP, TTP, HUS, usually normal or slightly elevated, which is different from
and exacerbation of SLE can be easily confused with those the usual finding in patients with AFLP. The diagnosis is
of severe pre-eclampsia– eclampsia HELLP syndrome. Al- usually made in the presence of vesicular lesions in the skin,
though these conditions share a range of signs and symptoms perineum, or the cervix. Liver biopsy will show the typical
(Table 4) and laboratory tests (Table 5) with HELLP syn- intranuclear inclusions (Cowdry type A inclusions) with as-
drome, each has some distinguishing clinical findings or lab- sociation with cell necrosis and hemorrhage (Fig. 2). The CT
oratory results. Making the right diagnosis is extremely im- scan may show the typical mottled appearance in the liver
portant regarding decisions about need for delivery as well as (multiple low-density areas that do not enhance with contrast
treatment and complications. injection), which is different from the CT scan findings in
patients with HELLP syndrome, AFLP, or liver infarction.1
Disseminated herpes infection is associated with high ma-
Systemic Viral Sepsis ternal and perinatal mortality if not treated promptly.69-72 In
Disseminated herpes simplex is a rare complication during a review of 24 cases of herpes simplex hepatitis in pregnancy
pregnancy. It is usually seen in immunocompromised preg- up to the year 1998, Kanga and Graves70 found an overall
maternal mortality of 39% and perinatal mortality of 39%. penia, renal insufficiency, altered mental status, and pulmo-
Maternal mortality was 0% among 13 women treated with nary insufficiency. The clinical picture can mimic HELLP
acyclovir and 20% among 4 women treated with vidarabine, syndrome, AFLP, TTP and HUS, and an SLE flare. It can be
whereas it was 67% among 15 women receiving no antiviral the primary cause or it can be a complication of any one of the
therapy.70 In addition, the perinatal mortality was 18% in above syndromes. The clinical findings include a tempera-
those receiving acyclovir, 67% among those receiving vidara- ture ⬎38°C or ⬍36°C, tachycardia (pulse ⬎100 bpm),
bine, and 44% among those receiving no therapy. Therefore, tachypnea (respiratory rate ⬎24/min), and hypotension (sys-
accurate and urgent diagnosis is important because early tolic blood pressure ⬍90 mm Hg). Cardiac output is initially
therapy with acyclovir improves survival. The usual dose of elevated, systemic vascular resistance is low, and venous re-
intravenous acyclovir is 10-15 mg/kg of body weight every 8 turn is reduced. With reduced tissue perfusion, oxygen de-
hours to be continued for at least 10 days.69-72 These patients livery is impaired, resulting in increased lactate levels sec-
should be managed in an intensive care facility. There is no ondary to anerobic cellular metabolism. Both hypotension
need for early delivery in the absence of fetal indications.70 and poor tissue perfusion will ultimately lead to multiple
organ failure.73-78
Systemic
Maternal and Perinatal
Inflammatory Response
Outcome in Septic Shock
Syndrome (SIRS)/Septic Shock Mabie and coworkers73 reported the etiology, management,
SIRS describes a systemic inflammatory process that can be and outcome in 18 patients with septic shock during preg-
generated by infection or by noninfectious causes, such as nancy. The etiology of shock were pyelonephritis in 6
pancreatitis, burns, and major trauma.73-78 It is characterized women, chorioamnionitis in 3, postpartum endometritis in
by a hyperdynamic state, endothelial cell injury, leukocyto- 3, toxic shock syndrome in 2, and 1 each of septic abortion,
sis, neutrophil activation, and tissue hypoperfusion with appendicitis, ovarian abscess, necrotizing fasciitis, and bac-
multiorgan dysfunction.71,73-78 Sepsis is SIRS due to infection terial endocarditis. Five women (28%) died, and 15 (82%)
that is associated with hypoperfusion or hypotension with had multiorgan dysfunction. The perinatal survival was 50%
organ dysfunction. Septic shock is a subset of sepsis defined (10 of 20, one set of triplets). Five of the 10 losses were ⱕ20
as sepsis-induced hypotension despite adequate fluid resus- weeks’ gestation, and 5 were stillbirths.73 Afessa and cowork-
citation along with the presence of perfusion abnormalities, ers74 reported on 44 obstetric patients who developed SIRS; 2
such as lactic acidosis, renal dysfunction (oliguria, acute tu- patients had septic shock and 18 had severe sepsis. Two
bular necrosis), hepatic dysfunction, and acute changes in women (4.5%) died, and 36 (82%) had multiorgan failure.
mental status.73-78 Laboratory findings include leukocytosis
or leukopenia (white blood count ⬍4000), thrombocytope-
nia, hemolysis, DIC, elevated liver enzymes, and elevated Management of Septic Shock
bilirubin. Treatment of septic shock consists of resuscitation with flu-
Some authors suggested that the maternal syndrome of ids, vasopressors, antibiotics, hemodynamic monitoring, re-
pre-eclampsia share many of the clinical and laboratory find- moval of source of infection (surgery or percutaneous drain-
ings of patients with SIRS.79,80 Both syndromes are character- age of abscesses), and modifying of inflammatory mediators.
ized by systemic inflammation, neutrophilia, and neutrophil Patients with SIRS or septic shock require admission to an
activation. Indeed, many of the clinical and laboratory find- intensive care unit to monitor volume replacement with crys-
ings of women with HELLP syndrome, such as hemoly- talloids or blood components, to monitor vital signs, urine
sis, hepatocellular necrosis, renal dysfunction, pulmonary output, oxygenation, and mental status.73-78 Fluid resuscita-
edema, ARDS, and thrombocytopenia, are similar to those tion will include 3-4 L of crystalloid over 60 minutes, to
seen in patients with severe sepsis and septic shock.79,80 A correct hypotension and to achieve a central venous pressure
recent report by Afessa and coworkers74 found that 59% of of 8-12 mm Hg. If hypotension persists, then vasoactive
obstetric patients admitted to ICU developed SIRS. agents are used. The first drug of choice is dopamine, titrated
In general, patients with severe sepsis or septic shock will up to 20 mg/kg/min to achieve a MAP of at least 65 mm Hg
have fever, but hypertension and proteinuria are absent. (MAP needed to keep adequate tissue perfusion, particularly
However, hypertension or proteinuria may be absent in 10- cerebral perfusion).73-78 Other vasoactive drugs, such as do-
15% of patients with HELLP syndrome. In addition, leuko- butamine or norepinephrine, can be used as needed. If the
cytosis and fever may be present in women with pre-eclamp- patient does not show good response to broad spectrum an-
sia because of endomyometritis, particularly after cesarean tibiotic therapy, attention should be given to identify a source
delivery. Table 1 compares clinical and laboratory findings of infection (retained products of conception, microabscesses
between those with disseminated herpes and SIRS/sepsis in in uterine wall, pelvic abscess, etc.) that require surgical in-
pregnancy. tervention.71,73-76 In general, delivery is required if there is
Septic shock is rare during pregnancy or postpartum.73-76 evidence of an infected fetus, amniotic fluid, or placental
It should be considered in the differential diagnosis of pa- tissues. On the other hand, delivery is not indicated in the
tients with fever, microangiopathic hemolysis, thrombocyto- absence of infection in the uterine cavity. A detailed descrip-
204 B.M. Sibai
tion of pathophysiology and management of sepsis has been ric surveillance system (UKOSS). A prospective national study of acute
described.76-80 fatty liver of pregnancy in the UK. Gut 57:951-956, 2008
12. Suzuki S, Watanalie S, Araki T: Acute fatty liver of pregnancy at 23
weeks of gestation. Br J Obstet Gynaecol 108:223-224, 2001
13. de Nagy J, Strittmatter CA, Lee RV: Acute fatty liver of pregnancy
Conclusions presenting as hepatic encephalopathy at 28 weeks’ gestation. J Obstet
Gynaecol 28:100-102, 2008
Several microangiopathic disorders can occur any time dur-
14. Martin JN Jr, Briery CM, Rose CH, et al: Postpartum plasma exchanges
ing pregnancy or during the postpartum period. The inci- adjunctive therapy for severe acute fatty liver of pregnancy. J Clin
dence of these disorders is extremely low; however, the ma- Apher 23:138-143, 2008
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clinical presentation and laboratory findings in these disor- drome. Obstet Gynecol 103:981-991, 2004
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ders can mimic those seen in pre-eclampsia and eclampsia.
liver of pregnancy. Am J Obstet Gynecol 158:142-145, 1988
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fore, it is important that managing physicians be aware of the 35:815-817, 1990
etiology, diagnostic findings, and management of such dis- 18. Farine D, Newhouse J, Owen J, et al: Magnetic resonance imaging and
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orders. Finally, a rapid diagnosis and close consultation with
pregnancy. Am J Perinatol 7:316-318, 1990
an interdisciplinary team of physicians, such as maternal– 19. Clements D, Young W, Thornton J, et al: Imaging in acute fatty liver of
fetal medicine, nephrologist, hematologist, critical care, etc., pregnancy. Br J Obstet Gynaecol 97:631-633, 1990
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Box I Imitators of Severe 21. Knox TA, Olans LB: Liver disease in pregnancy. Review article. N Engl
J Med 335:569-576, 1996
Pre-eclampsia/Hellp Syndrome 22. Zang Z, Yamada J, Zhao Y, et al: Prospective screening for pediatric
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● AFLP by liver disease. JAMA 288:2163-2166, 2002
● TTP 23. Rajasri AG, Sretha R, Mitchell J: Acute fatty liver of pregnancy (AFLP):
● HUS An overview. J Obstet Gynaecol 27:237-240, 2007
● Exacerbation of lupus erythematosus 24. Tyni T, Ekholm E, Pihko H: Pregnancy complications are frequent in
long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency.
● Catastrophic antiphospholipid syndrome
Am J Obstet Gynecol 178:603-608, 1998
● Systemic viral sepsis (disseminated herpes) 25. Ibdah JA, Bennett MJ, Rinaldo P, et al: A fetal fatty-acid oxidation
● SIRS/septic shock disorder as a cause of liver disease in pregnant women. N Engl J Med
● Other conditions (cholestasis of pregnancy, necrotizing 340:1723-1731, 1999
pancreatitis, etc.) 26. Martin JN Jr, Bailey AP, Rehberg JF, et al: Thrombotic thrombocytope-
nic purpura in 166 pregnancies, 1955-2006. Am J Obstet Gynecol
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