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If mild exacerbations are included, some worsening will occur in passively acquired autoimmune disease resulting from transfer
48% of all pregnant SLE patients, 33% of these in the postpartum of maternal autoantibodies to Ro/SSA, La/SSB, or U1RNP anti-
gens.76 Less than 33% of babies of women positive for these
period. The incidence of flares during the immediate postpar-
tum period can be reduced by giving corticosteroids during antibodies develop neonatal lupus. Approximately 50% of the
labor.13 Termination of pregnancy has no beneficial effects on babies affected will manifest classical skin lesions of lupus and
either the acute exacerbations of lupus or the long-term course approximately 2% will develop congenital complete heart
block.77,78,79 Neonatal lupus is responsible for up to 90% of
of the disease.64
cases of congenital heart block in the neonatal period.80
Rarely, other organ systems are involved in neonatal lupus,
Effect of systemic lupus erythematosus
resulting in hemolytic anemia, thrombocytopenia, leukopenia,
on pregnancy
hepatosplenomegaly, or glomerulonephritis. Most of the mani-
Systemic lupus erythematosus does not affect fertility in the festations of neonatal lupus resolve completely within the first six
absence of active disease, renal failure, treatment with high-dose months of life, with the exception of heart block, for which 50%
steroids, or cyclophosphamide therapy.66,67 The incidence of per- require cardiac pacing.77 Complete heart block is thought to
manent amenorrhea following cyclophosphamide treatment result from either antibody attack on fetal cardiac conductive
ranges from 11“59% and is increased with age and cumulative tissues, immune complex deposition in myocardial tissue, or as
dose.68 However, SLE does lead to increased maternal and fetal a consequence of fetal myocarditis. Complete congenital heart
morbidity and mortality. block is usually diagnosed by fetal echocardiogram after 18
weeks™ gestation. Glucocorticosteroids, NSAIDs, and plasma-
Effect on mother pheresis are ineffective in altering the course of the fetal cardiac
outcome.77 However, there is a suggestion that the early use of
Parturients with SLE have an increased risk of preeclampsia (PET),
which ranges from 5“38% in reported studies.67 Distinguishing dexamethasone may reverse myocarditis and possibly heart
block.81 Prediction of those fetuses at risk for developing com-
between PET and nephritic lupus flare may be difficult, but impor-
tant, as treatment regimes differ (i.e. delivery for PET, immunosup- plete congenital heart block is difficult, as mothers of affected
infants are often asymptomatic at the time of presentation.76,77
pressive therapy for nephritic flare). Both conditions can cause



411
5 Other disorders


Medical management Obstetric management
The cornerstone to successful management of SLE parturients is to Close vigilance of the parturient with SLE is vital, as these patients
reduce or prevent end-organ damage. This is challenging during are at high risk for associated conditions, in addition to organ
pregnancy as the additional physiological and metabolic demands deterioration. Obstetric management should begin with an
imposed by the fetus may compromise already dysfunctional mater- assessment of baseline clinical state and disease markers to
nal organs. A multidisciplinary approach, involving obstetricians, assess prognosis and to detect any deterioration at a later stage.
anesthesiologists, neonatologists, and rheumatologists is useful to Initial investigations include: full blood count, platelet count,
improve care of these patients. Activity of SLE can be assessed by a urea, electrolytes, uric acid, autoantibody screen (e.g. antiRo,
combination of clinical history, examination, and serological stu- anti La antibodies), anticardiolipin antibody (aCL), lupus anti-
dies.82 Numerous serological markers (e.g. antinuclear antibodies, coagulant (LA), complement levels (C3, C4, CH50), urinalysis, and
anti-double-stranded DNA antibodies, aPLs, extractable nuclear 24-hour urine protein. Platelet count and creatinine should be
antibodies “ anti-Ro, anti-La, complement levels, erythrocyte sedi- measured monthly. Any deterioration of SLE or symptoms of PET
mentation rate, C reactive protein) have been used to assess disease require a full blood count, platelet count, urea, electrolytes, crea-
activity, although they are not necessarily specific or sensitive.83 tinine, uric acid, and urinalysis (plus 24-hour urinary protein).
The essential areas of treatment are described in Table 23.7. Many Close consultation between the obstetrician and rheumatologist
of the drugs used for this condition are also used to treat RA and are is necessary throughout the pregnancy. Anti-double-stranded
described earlier. As NSAIDs may be inappropriate in patients with DNA antibodies may or may not be present during inactive SLE,
renal dysfunction, antimalarial medication can be used successfully but increase markedly prior to flares, and remain elevated for up
to two weeks.53Antiphospholipid and aCLs in SLE patients are
to treat SLE patients. Hydroxychloroquine is considered safe during
pregnancy and continuation of therapy during pregnancy is prob- associated with an increased risk of fetal loss and maternal
ably safer than risking increased SLE exacerbations. Stopping hy- thrombotic complications. Ro/SSA and La/SSB antibodies are
droxychloroquine after pregnancy has been confirmed, will not avoid associated with an increased risk of neonatal lupus and fetal
fetal exposure as the drug has a long elimination half-life. Breast- congenital heart block.
feeding should be used with caution due to slow drug elimination Monitor fetal growth and development from early pregnancy
and the possible accumulation of toxic doses in the neonate.22 through delivery to reduce morbidity and mortality. If the mother
Corticosteroids with or without immunosuppressive drugs are has autoantibodies to Ro/SSA or La/SSB antigens, a pediatric
generally reserved for patients with significant organ involvement cardiology consultation is indicated as early as 20 weeks™ gestation
particularly renal or CNS disease. Sulfonamide antibiotics and to rule out fetal cardiac involvement. Consultation with other
penicillin (but not semisynthetic penicillins) should be avoided specialists is dictated by the specific organ systems affected by
as anecdotal reports suggest that they may increase disease the disease. Clearly, it is prudent to consult with the anesthesiol-
exacerbations. Other drugs, e.g. hydralazine, and procainamide, ogist and neonatologist as the fetus nears 24 weeks™ gestation,
known to cause drug-induced SLE, are safe in the idiopathic form since early emergency delivery may become necessary.
of the disease. Vaginal delivery is preferred, but delivery mode should be tail-
Women with SLE have a nine to tenfold increase in risk for ored to maternal and fetal conditions. Although the ideal timing of
coronary artery disease associated with accelerated atherosclero- delivery is at term, worsening renal function, hypertension unres-
sis. Thus, patients with SLE and traditional risk factors for coro- ponsive to treatment, or fetal distress may necessitate preterm
nary artery disease should be managed aggressively using delivery. Supplementation of corticosteroids may be necessary
medication, diet, and lifestyle modification.84 during labor and delivery in those patients on regular cortico-
steroid treatment. Because of immunosuppression, close vigilance
for signs of infection is important and prompt treatment necessary.
Table 23.7 Treatment for systemic lupus erythematosus
In addition, both mother and neonate should be monitored in the
Nonsteroidal anti-inflammatory drugs (NSAIDs) postpartum period for flares and neonatal lupus respectively.
For symptomatic relief of arthritis and arthralgia
Antimalarial drugs (e.g. hydroxychloroquine)
Anesthetic management
For rashes and arthritis
For flares
Full evaluation of all associated organ disorders is important (see
Glucocorticosteroids
Table 23.8). Although arthritis occurs in approximately 90% of SLE
For severe flare or lower doses for maintenance
patients, the spine and hips are rarely affected and therefore posi-
Immunosuppressive drugs
tioning during labor and regional anesthesia are rarely a technical
For severe flare (often in combination with corticosteroids)
challenge. Cardiac abnormalities occur in over 50% of SLE patients
Additional treatment
and include pericardial, myocardial, valvular, and coronary artery
Antibiotics for infection
disease.85 Signs and symptoms of cardiac disease should be noted
Thromboprophylaxis
and routine electrocardiogram (EKG) performed (plus echocardio-
Antihypertensives if needed
gram, if necessary). A structural valvular disorder is the common-
? Anticonvulsants for cerebral disease
est cardiac abnormality in SLE, with mitral regurgitation being the



412
Chapter 23


most frequent problem.86 In one study, 51% of SLE patients
Table 23.8 Clinical features and anesthetic implications had thickening, 43% vegetations, 28% regurgitation, and 4% steno-
of systemic lupus erythematosus sis of the mitral valve.87 A five-year review found that nearly 25%
of SLE patients had significant cardiovascular complications.87
Organ Disease problem Anesthetic implications
Pericardial involvement is common, but usually is benign,
NS Cognitive dysfunction Consent issues
although almost 50% develop a pericardial effusion. Myocarditis
(20“80%) Differential diagnosis
is uncommon and often asymptomatic, although conduction
Seizures (10“20%) of PET/treatment
abnormalities have been noted in 34“70% of SLE patients.85
Risk of stroke (<15%) Possible need for
Occasionally, myocardial ischemia or infarction resulting from
thromboprophylaxis
coronary artery vasculitis or accelerated atherosclerosis may
(problems with
occur in young women.88 Risk factors for coronary artery disease
regional and insertion)
in association with SLE include: increased age at diagnosis,
Psychiatric conditions
increased duration of SLE, and longer duration of corticosteroid
Peripheral neuro- Neurological
use.89 Prophylactic antibiotics should be given to all parturients
pathy (10“15%) assessment should
with known cardiac structural abnormalities during delivery, and
be made prior to
anesthesia should be tailored to maintain cardiovascular stability.
regional insertion
Neurological and psychiatric (e.g. anxiety, mood disorders,
Skin Epidermal and Minimal
psychosis, cognitive dysfunction) abnormalities occur in
subdermal lesions
10“80% of SLE patients. Approximately 15% of SLE patients
Lungs Pleuritis Pain
have a peripheral neuropathy with sensory, rather than motor
Pneumonia Care with sterility,
nerves, more likely to be affected.90 Peripheral neuropathy is
(increased risk) antibiotics if
usually mild, asymmetric and affects more than one nerve
appropriate
(mononeuritis multiplex).91 Autonomic neuropathy has been
Fibrotic lung disease Respiratory
reported occasionally. Any neurological deficit should be noted
impairment
and documented prior to regional anesthesia. Seizures occur in
Pulmonary hyper- Oxygen/vasodilators
10“20% of SLE patients and it may prove difficult to differentiate
tension (rare)
between SLE seizures and eclampsia.92
Kidney Glomerulonephritis Renal dysfunction/
Radiological investigations of the chest may be utilized to
Lupus nephritis altered drug handling
evaluate the pulmonary complications of SLE, which include inter-
CVS Increased risk of Depends on
stitial pneumonitis, pulmonary hemorrhage, pulmonary hyper-
cardiac disease: abnormality
tension, and pleural effusion. Arterial blood gas analysis is
(pericardial, May require endocar-
indicated if pulmonary or cardiac involvement is suspected.
valvular, ditis prophylaxis
Pulmonary function tests in patients with SLE may show a restric-
myocardial,
tive pattern with decreased vital capacity and diffusion capacity.
coronary artery
Winslow and coworkers found that pulmonary hypertension
disease)
developed in nearly 50% of SLE patients over a five-year period.93
Cardiac abnormal-
The diagnosis of pulmonary hypertension should be considered
ities (50%)
in patients who complain of dyspnea, chest pain, and non-
GIT Dysphagia (<25%) Risk of aspiration
productive cough in addition to confirmatory physical signs (jugular
Risk of mesenteric Risk of acute abdomen
venous distension, prominent A/V wave pulsations, hepato-
vasculitis/infarction
megaly, ascites, peripheral edema). Anesthesia for parturients
Risk of peptic ulcer
with pulmonary hypertension is discussed in Chapter 1.
Hematological Anemia Reduced oxygen
Successful general anesthesia has been reported in a parturient
carrying capacity
with SLE restrictive lung disease and pulmonary hypertension.94
Leukopenia Risk of infections
The parturient with SLE must undergo hematologic evaluation.
Thrombocytopenia Concerns with
Although mild thrombocytopenia (platelet 100“150 ‚ 109/l) has
insertion of regional
been noted in up to 50% of patients, platelet counts < 50 ‚ 109/l
techniques
(precluding the use of neuraxial anesthesia) are found in only 10%
Antibodies to Abnormalities in in vitro
of patients.95 Antibodies to a number of clotting factors, including
clotting factors/ coagulation tests
VIII, IX, XII, and XIII have been reported in SLE patients and may
phospholipids Problems in cross-
result in significant coagulopathy, again precluding the use of
matching blood
regional anesthesia. In patients with ˜˜lupus anticoagulant™™ “ the
term is a misnomer as its presence promotes thrombosis “ there is
NS ¼ nervous system; CVS ¼ cardiovascular system; GIT ¼
an artifactual in-vitro prolongation of the activated partial throm-
gastrointestinal system; PET ¼ preeclampsia
boplastin time (aPTT), despite normal or increased in vivo coagu-
lation. Antibodies to other phospholipids (e.g. aCLs) in moderate



413
5 Other disorders


or high levels can produce a similar effect. In these circumstances,
Table 23.9 Clinical features of polyarteritis nodosa (Lhote)99
the elevated aPTT does not suggest an increased bleeding tendency
and regional anesthesia is not contraindicated. Frequency
Atypical antibodies can make typing and cross-matching of Clinical feature (%)
blood difficult and time consuming. Therefore, any patient at
Constitutional symptoms (fever, weight loss, fatigue) 70
risk of significant hemorrhage should have early blood typing or
Neuropathy (polyneuropathy, rarely cranial nerves 65
cross-matching.
affected)
Systemic lupus erythematosus patients should be monitored
Cutaneous (livedo reticularis, purpura, ulcers, 50
carefully for signs of PET in the postpartum period. Those who are
bullous/vesicular eruption)
positive for aPLs or aCLs are at increased risk for thrombosis, and
Arthralgias/myalgias 50
should have additional prophylactic thromboprophylaxis meas-
Renal disease (glomerulonephritis, raised creatinine) 50
ures instituted immediately after delivery.
Gastrointestinal symptoms (abdominal pain, rectal 40
bleeding, diarrhea, nausea, vomiting)
Polyarteritis nodosa Hypertension (new onset) 25
Respiratory manifestations (infiltrates, nodules, 25
Polyarteritis nodosa (PAN) is a rare multisystem autoimmune
cavities)
disease affecting about 6 per 100 000 individuals in the United
Central nervous system disease (stroke, confusion) 20
States.57 Unlike other collagen diseases, men are affected two to
Orchitis (testicular pain, swelling) 20
three times as frequently as women with a peak age incidence of
Myocardial manifestations (angina, myocardial 10
between 40“60 years.96 Due to the peak age of onset and the male
infarction, cardiac failure)
preponderance, pregnancy with PAN is a rare occurrence.
Although the etiology of PAN is unknown, it is thought to be
autoimmune in origin, as antibodies and complement compo-
nents have been seen in histochemical analysis of the lesions. signs and symptoms of other organ involvement (see Table 23.9).
Most cases are idiopathic, although there may be a link with hairy Abdominal symptoms occur frequently, including anorexia, nausea,
cell leukemia and hepatitis B virus (HBV) infection.97,98 pain, diarrhea, and bleeding. Cardiac and renal involvement are
Polyarteritis nodosa is characterized by a segmental necrotiz- common, leading to hypertension and myocardial infarction.
ing angiitis affecting mostly small- and medium-sized arteries, Optimal treatment and duration of therapy remains unclear. The
with a predilection for arterial bifurcations.53,96 Affected areas mainstays of therapy include corticosteroids and immunosup-
pressants (cyclophosphamide, azathioprine).101,102 Antiviral therapy
dilate to form small aneurysms. During the acute phase, the
media of the affected areas undergoes fibrinoid necrosis and may be helpful in those patients with HBV-related PAN in order to
infiltration with polymorphonuclear leukocytes and eosinophils. diminish the immunosuppressant enhanced viral replication.
The patient is at risk of thrombosis, aneurysmal rupture, and Otherwise therapy is aimed at normalizing associated hypertension,
infarction during this stage. Edema of the walls of the affected in addition to supportive treatment of affected organ dysfunction.
areas may result in complete occlusion of the vessel. Renal transplantation in PAN patients is associated with a lower
The acute phase gives way to a transitional phase, in which renal survival than in patients with other causes for end-stage renal
monocytes replace the polymorphonuclear leukocytes, and disease.
granulation tissue replaces areas of necrosis. Finally, during the Untreated patients with multisystem disease have a one-year
survival < 50%, and a five-year survival of only 13%.96,103 However,
chronic phase, scar tissue replaces former areas of necrosis,
with current treatment, five-year survival is approximately 80%.101
resulting in thickening of vessel walls and perivascular fibrosis.
This proliferation of scar tissue and fibrosis may result in vessel Poor prognostic features include renal and gastrointestinal invol-
vement,104 with mortality usually related to mesenteric, cerebral,
occlusion. A characteristic feature is the coexistence of acute and
chronic changes, often within adjacent sections of a vessel.96 or myocardial infarction or renal failure.
The most commonly affected systems in PAN are skin, peripheral
nerves, kidney, and gastrointestinal tract, but involvement of the
Impact of pregnancy on polyarteritis nodosa
lungs, CNS, and myocardium also occurs (see Table 23.9).53,96,99
Diagnosis is made using the criteria devised by the Royal College Due to the limited number of case reports of PAN in pregnancy it
of Rheumatology, which include unexplained weight loss (>4 kg), is difficult to draw any valid conclusions about the effects of
testicular pain in men, livedo reticularis, myalgia, mono/polyneuro- pregnancy on PAN. In the first six cases reported, all six women
pathy, new onset hypertension (diastolic blood pressure [BP] died within 33 days of delivery, and the finding of PAN was made
at autopsy.53 The first reported case of maternal survival occurred
> 90 mmHg), raised serum creatinine, evidence of HBV infection,
characteristic arteriographic abnormalities (multiple aneurysms in in 1970 in a woman who was diagnosed eight years before and
larger vessels, occlusion of small penetrating arteries), and charac- had been treated with corticosteroids. Her fetus died in utero and
teristic biopsy of small/medium sized artery of affected organs.100 was delivered by C/S. Of the six patients reported since 1970, one
The clinical presentation of PAN is one of constitutional symp- died 22 weeks post conception, following a spontaneous abortion
toms such as fever, weight loss, arthralgia, and malaise, together with at 20 weeks™ gestation; one died 18 months postpartum; and the



414
Chapter 23



Table 23.10 Clinical features and anesthetic implications of polyarteritis nodosa

Organ Disease problem Anesthetic implications

NS Mononeuritis multiplex Full neurological evaluation needed prior to regional insertion
Asymmetric polyneuropathy
Occasionally cranial nerve neuropathy
Increased risk of ischemic CVA, intracerebral hemorrhage Avoid cardiovascular instability, ameliorate pressor responses
Skin Livedo reticularis, skin ulcers, bullous or vesicular eruptions, Careful handling of patients
purpuric/petechial lesions (vasculitis) Caution with regional if purpuric/petechial lesions
Severe manifestations including infarction of fingers/toes
Lungs Interstitial lung disease Respiratory impairment
Kidney Glomerulonephritis Renal dysfunction/altered drug handling
Renal arterial aneurysms Hypertension
CVS Increased risk of IHD Depends on severity
GIT Abdominal pain, nausea/vomiting Risk of aspiration
Diarrhea, GIT bleeding Dehydration
Anemia (reduced oxygen carrying capacity)
Risk of mesenteric vasculitis/infarction Risk of acute abdomen
ENT Epistaxis Avoid nasal intubation if possible
Sore throat Careful airway management

NS ¼ nervous system; CVS ¼ cardiovascular system; GIT ¼ gastrointestinal system; ENT ¼ ears, nose, and throat; CVA ¼ cerebral vascular accident;
IHD ¼ ischemic heart disease



remaining four survived.53,96,105 Friedman and coworkers53 baseline clotting studies (prothrombin time, aPTT, fibrinogen
speculated that patients with disease in partial or total remission level, platelet count) and uric acid levels. Epidural analgesia is
at the onset of pregnancy have a better prognosis than those not contraindicated unless the patient presents with extensive
whose disease first occurs during pregnancy or the early puer- purpura or has a coagulopathy. Careful documentation of any
perium. It is important to point out, however, that their analysis neurological deficit is important prior to any regional technique.
includes those six patients reported prior to 1970, in which the Wide fluctuations of BP should be avoided as these patients may
diagnosis was not made until autopsy. Only one of those patients have multiple small aneurysms as well as areas of diminished
received corticosteroids. blood flow secondary to intimal fibrosis and occlusion. Look for
signs and symptoms of myocardial ischemia and evaluate com-
plaints of chest pain or dyspnea. Supplementation with cortico-
Impact of polyarteritis nodosa on the neonate
steroids may be necessary during labor and delivery in those
It is remarkable that nine of the thirteen (69%) reported pregnan- patients on regular corticosteroid treatment. As most of the
cies resulted in surviving neonates. There was only one intrauter- deaths in PAN patients result from cardiorespiratory failure,
ine fetal death, two induced abortions, and one spontaneous renal failure, or gastrointestinal hemorrhage, it is sensible to
abortion (at 20 weeks™ gestation).53,96,105 All neonates, except focus postpartum monitoring on these areas.
one who had vasculitis, were healthy.106
Termination of pregnancy does not appear to ameliorate dis-
Scleroderma (systemic sclerosis)
ease activity and is not indicated for maternal PAN. The impli-
cations of maternal drug treatment (e.g. corticosteroids, Scleroderma is a diverse, progressive, multisystem condition
linked by the presence of thickened, sclerotic skin lesions.107
azathioprine, cyclophosphamide) on the fetus and neonate are
discussed in the section on RA. Clinical manifestations occur in the skin, musculoskeletal, ner-
vous, cardiovascular, pulmonary, renal, and gastrointestinal sys-
tems. Prevalence ranges from 4 to 253 cases per million
Anesthetic management
individuals with a female-to-male preponderance of 3:1.2 As the
There are no case reports addressing the issue of anesthetic peak age of onset occurs in the fourth decade, scleroderma is
management of PAN patients. Clearly it is important to involve relatively uncommon in pregnancy. However, the incidence of
other specialists, such as cardiologists and nephrologists depend- parturients with the disease is increasing as greater numbers of
ing on the clinical picture (see Table 23.10). Baseline investiga- women opt to have children later in life.
tions such as full blood count, urea, electrolytes, EKG, 24-hour The etiology and pathogenesis of scleroderma is not fully
urinary protein, and creatinine clearance are useful. The clinical understood, although genetic and environmental factors may
picture may be similar to that of PET, so it is prudent to obtain play a role. Most theories suggest a complex interplay between



415
5 Other disorders


immunological events and vascular changes resulting in activa- cavities. Cardiac disease results from fibrosis and sclerosis of the
tion of fibroblasts. Excessive collagen and other extracellular myocardial/conducting tissue and coronary vessels, in addition to
matrix constituents are produced leading to obliteration and indirect effects from pulmonary and systemic hypertension.
fibrosis within the skin and other target organs.108 Patients with symptomatic cardiac involvement have a 75% five-
year mortality rate.109 Life threatening or severe ˜˜scleroderma
Scleroderma can be classified into three main subgroups and
diagnosis is based on the presence of typical skin lesions, extra- related renal crises™™ occur in 10“15% of patients and consist of a
cutaneous manifestations, and characteristic antibodies (see sudden onset of severe hypertension, progressive renal insuffi-
Table 23.11). The combination of skin lesions plus one or more ciency, and microangiopathic hemolysis. Pulmonary involvement
of the following “ hypertension (acute onset), renal deficiency, is also a major cause of morbidity and results from interstitial
heartburn/dysphagia (new onset), telangiectasias (face, lip, pulmonary fibrosis, which may lead to pulmonary hypertension.
hand), diarrhea with malabsorption, dyspnea on exertion (with Peripheral and cranial neuropathy may result from compression of
radiological interstitial pulmonary changes), or pulmonary thickened adjacent connective tissue. Disease manifestations in
hypertension “ strongly suggests the diagnosis. the musculoskeletal system are edema, arthralgia, and myalgia.
The most common feature of scleroderma is Raynaud phenom- A multifaceted approach should be used in the treatment of the
enon, which is due to arterial vasoconstriction and results in char- disease. Immunomodulatory and antifibrotic methods do not
acteristic sequential color changes to the hand (white, blue, and alter long-term progression of the disease, although gluco-
red). Permanent arterial insufficiency may result from structural corticoids and cyclophosphamide may be useful in patients
damage to the vessel wall. Skin manifestations include edema, with alveolitis, myositis, arthritis, and serositis. Recombinant
hyperpigmentation, skin thickening/hardening, skin tightening human relaxin (a heterodimer protein secreted by the corpus

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