LINEBURG


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arteriosus
The mainstays of drug therapy for symptoms of RA are aspirin or
Pulmonary
NSAIDs, which are generally safe in pregnancy. Neither of these
hypertension
drug classes is teratogenic in humans; however, these agents
Renal dysfunction
should be avoided by women having conception difficulties as
Glucocorticoids Weight gain ? Cleft lip/palate
they prevent blastocyst implantation in animals.22,23 Both aspirin
Cushingoid features Adrenal
and NSAIDs may increase the incidence and severity of maternal
Increased risk of suppression
anemia, prolonged gestation and labor, and bleeding problems
infection
(e.g. hemorrhage).18,24 In addition, potential adverse fetal effects
Adrenal suppression
include increased bleeding (e.g. neonatal cephalohematoma,
Diabetes mellitus
intracranial hemorrhage in preterm infants), premature closure
Hypertension
of the ductus arteriosus, pulmonary hypertension, impaired renal
Osteoporosis
function, and oligohydramnios. Ideally, aspirin and NSAIDs
Aseptic necrosis of
should be avoided six to eight weeks prior to delivery to reduce
joints
the incidence of these problems. Significant plasma aspirin levels
Depression
have been found in breast-fed babies, raising concerns of possible
Psychosis
metabolic acidosis, altered pulmonary circulation, and Reye
Hydroxychloroquine Retinopathy ? fetal ocular/
syndrome. Therefore, avoid high doses of aspirin in nursing
(antimalarial) ototoxicity
mothers, although normal doses do not appear to cause major
Methotrexate Hepatotoxicity Craniofacial defects
concerns. Nonsteroidal anti-inflammatory drugs are weak acids
Leucopenia Limb defects
and are only minimally transferred to breast milk. Breast-feeding
CNS defects
is considered safe in women on NSAIDs;25 however, as NSAIDs can
Sulfasalazine Blood disorders No fetal defects
displace bilirubin from plasma proteins the nursing mother of a
Neutropenia
jaundiced neonate should avoid them due to the increased risk of
Thrombocytopenia
kernicterus.
Azathioprine Nausea and vomiting ? Intrauterine
Corticosteroid therapy appears to be relatively safe in preg-
Leukopenia growth restriction
nancy, although there may be a slight increased risk of cleft palate
Hepatotoxicity Various fetal
and lip in the newborn, together with an increased risk of pre-
Impairment of defects reported
eclampsia (PET), gestational diabetes, premature rupture of
fertility Teratogenic
membranes, and intrauterine growth restriction (IUGR).26 All
Leflunomide Renal impairment Teratogenic
corticosteroids cross the placenta but the shorter-acting agents
Bone marrow
(prednisone, prednisolone, methyl-prednisolone) are partially
depression
inactivated by placental 11-b-dehydrogenase. When these
Diarrhea
shorter-acting agents are used, the fetus is exposed to minimal
Hepatotoxicity
drug concentrations. Administer the lowest effective dose of
Gold Bone marrow Congenital
corticosteroids and avoid fluorinated preparations (dexametha-
depression abnormalities
sone, betamethasone) to reduce fetal exposure. This policy also
unlikely
minimizes any increased risk of neonatal adrenal suppression
D-Penicillamine Bone marrow Connective tissue
and infection. Breast-feeding is considered safe during maternal
depression abnormalities
consumption of corticosteroids as only small amounts of the drug
Nephrotoxicity Cuta laxa
are seen in the breast milk.
Dislocated hips
Methotrexate and leflunomide are absolutely contraindicated in
Hernias
pregnancy due to teratogenic effects (craniofacial defects, limb



407
5 Other disorders


defects, anencephaly, hydrocephaly, meningomyelocele). As elective, orthopedic surgery patients who were taking regular pre-
dnisolone.33 There were no detrimental effects, but further studies
methotrexate may cause folate deficiency, supplemental folate
should be given to avoid neural tube defects. Breast feeding is are necessary to clarify this important issue.
not recommended with methotrexate due to the risks of neutro-
penia, immunosuppression, and carcinogenesis. Similarly,
Anesthetic management
azathioprine is best avoided in pregnancy due to its risk of terato-
genicity. Infants exposed to azathioprine are at risk of anemia, The affected organ systems in RA that may influence anesthetic
leukopenia, thrombocytopenia, infections, reduced immunoglo- choice are summarized in Table 23.2. In particular, the preanes-
bulin levels, and thymic atrophy. Sporadic abnormalities asso- thetic assessment should focus on a careful evaluation of the
ciated with azathioprine use include polydactyly, pulmonary airway and cervical spine as RA patients may have mandibular
stenosis, atrial septal defect, and hypospadias, but causation has hypoplasia, temporomandibular joint (TMJ) dysfunction, cri-
not been proven.27 It should be noted that a large number of coarytenoid arthritis, and/or neck problems (see Table 23.4).
patients with renal transplants and inflammatory bowel disease Examine the airway and document all findings even if the patient
have had successful fetal outcomes while taking azathioprine.28 has no overt symptoms of airway involvement, as general
Patients requiring azathioprine should consider delaying preg- anesthesia may become necessary for urgent delivery. Clearly in
nancy until disease improvement permits discontinuation of those patients known to have a difficult airway, an early epidural
their medication. Penicillamine is associated with fetal connective during labor is prudent to provide analgesia and allow instru-
tissue disorders (cuta laxa, hernias, dislocated hips, IUGR) and mental or surgical delivery, if required.
should be avoided in pregnancy.29 Cyclosporine has not been MacArthur and Kleiman34 recommended that all RA patients have
associated with severe fetal adverse effects, but based on a few cervical x-rays prior to elective surgery, as 80% of RA patients have
case reports there may be an increased risk of prematurity and cervical spine involvement. Symptoms of stiffness or discomfort,
spontaneous abortion. Sulfasalazine appears safe in pregnancy as well as limitation of cervical flexion and extension, should be
and in breast-feeding. There are no reports of fetal abnormalities,30 noted. The majority of neck extension occurs at the atlanto-
but since sulfasalazine impairs folate absorption, supplemental occipital joint, which has approximately 358 of extension in the
folate should be given. Gold seems to have limited adverse fetal normal patient. If this value is <238, visualization of the trachea
effects; however, the lack of studies probably should preclude its using conventional laryngoscopy may be difficult or impossible.
use during pregnancy and lactation.24 Hydroxychloroquine and The majority of flexion occurs between C3 and C6, with 408
flexion being normal.35 If the patient is unable to flex or extend
chloroquine are probably safe in pregnancy in normal doses.
These drugs have been used in pregnancy for malarial prophylaxis
and the treatment of rheumatic diseases with only one report of
ocular and ototoxicity in three children of a mother with systemic
Table 23.4 Effect of rheumatoid arthritis on airway access
lupus erythematosus.27,31 If hepatic or renal dysfunction exists,
care should be taken with the total dose. Role in airway
Joint access Effect of RA
Obstetric management Atlanto-occipital Majority of If <238 extension, direct
neck laryngoscopy may be
The parturient with RA should be managed using a multidisciplinary
extension impossible, FOI may
approach, involving obstetricians, anesthesiologists, neonatologists,
be necessary
and rheumatologists. As RA is a multisystem disease, all affected
Temporomandibular Mouth Interincisor gap < 3 cm,
organs should be evaluated. Estrogen, progesterone, and relaxin
opening or inability to protrude
cause ligamentous relaxation, which may place increasing strain
Mandibular the mandibular
on weight-bearing joints in RA parturients. In addition, as pregnancy
protrusion incisors in front of the
progresses, the increasing uterine mass accentuates lumbar lordosis
maxillary incisors is
and compensatory thoracic kyphosis. The importance of correct
highly predictive of a
posture and exercises to relieve discomfort should be emphasized.
difficult intubation.
Vaginal delivery is the preferred method of delivery, and cesarean
section (C/S) should be done for obstetric reasons.32 In rare, severe FOI may be necessary
Cricoarytenoid Narrowest Cricoarytenoiditis may
cases the disease may pose a problem to the mechanics of vaginal
upper result in complete
delivery and severely diseased hips or lower spine may preclude the
airway closure of vocal cords
use of stirrups. Patients who have had hip replacements are at
diameter preventing passage of
greater risk of dislocation and care should be taken to avoid exces-
endotracheal tube (or
sive flexion or rotation of the hips. In patients taking corticosteroids,
air). Tracheostomy
additional doses may be required during labor and delivery, due to
may be required.
possible suppression and atrophy of the hypothalamic-pituitary
adrenal axis. However, this standard approach has been challenged
FOI ¼ fiberoptic intubation
in a study where additional corticosteroids were not given to



408
Chapter 23


her neck adequately, alternative techniques such as flexible, Cricoarytenoid joint involvement has been reported in
26“86% of RA patients.45 Acute cricoarytenoid synovitis should
fiberoptic laryngoscopy are indicated.
Cervical joint destruction (atlantoaxial and subaxial “ below be suspected if the patient has symptoms of hoarseness or
C1“2) may result in instability and subluxation in 25“50% of throat discomfort/fullness that increases with phonation,
patients with longstanding disease.36 Other risk factors for sub- coughing, or swallowing, pain or tenderness over the larynx, or
luxation include increased age at onset of disease, rapidly progres- inspiratory stridor. Patients with chronic cricoarytenoid arthritis
sive erosive peripheral joint disease, increased activity of synovitis, may be asymptomatic at rest, but several rapid, deep inspira-
presence of rheumatoid factor, and elevated C-reactive protein tions may reveal occult stridor. Airway decompensation can
level.37,38 Atlantoaxial subluxation can be demonstrated if the occur with infection or laryngeal manipulation. Flexible fiber-
distance from the anterior arch of the atlas to the odontoid process optic pharyngo-laryngoscopy can be used to evaluate cricoary-
is >3 mm on lateral cervical x-ray (in flexion). Neurological injury tenoid joint mobility but caution is warranted as the joints may
may arise if movement causes the odontoid process to compress be red and swollen, and pregnancy may cause additional edema
the spinal cord or the vertebral arteries. An increased incidence of and narrowing of the airway. Traumatic examination may result
in acute, complete airway obstruction.46,47 The use of a smaller
cord compression occurs when the distance between C1 and C2 is
>9 mm, the posterior atlantodental distance is <14 mm or if the than usual endotracheal tube is recommended with mild to
space available for the spinal cord is <13 mm anywhere in the moderate cricoarytenoid involvement; however, with severe lar-
cervical region.39 Extreme care should be taken during intubation yngeal disease, consultation with an otorhinolaryngologist is
to minimize further displacement of the odontoid process by necessary. If a regional anesthetic technique is not possible,
excessive movement of the neck. The ˜˜sniffing the morning air™™ the patient may require a tracheostomy to secure her airway
position is widely recommended as the standard head and neck prior to surgery.
One report48 described awake, fiberoptic intubation in a par-
position (extension at occipitoatlantoaxial region and flexion at
subaxial region) for conventional direct laryngoscopy. There is a turient with Still disease following failed regional anesthesia for
report of worsening of anterior atlantoaxial subluxation with the C/S (see Table 23.5). Additional personnel, capable of assisting in
sniffing position in an RA patient. Although no neurological con- the management of failed intubation, and special airway equip-
sequences occurred, the authors recommended caution in using ment should be available for these patients. It may be better to
the sniffing position.40 Concerns regarding application of cricoid secure the airway via a primary tracheostomy in patients with
pressure in patients with cervical subluxation are unfounded. severe cricoarytenoid arthritis, rather than risk laryngeal trauma
Campbell and colleagues41 questioned the routine use of pre- after several failed intubation attempts. An otorhinolaryngologist
operative cervical x-rays in RA patients. In their study there was a should be informed of the patient™s status and be on standby, if
5.5% incidence of unsuspected C1“2 subluxation in 128 RA necessary. After extubation, monitor for symptoms of glottic
patients undergoing elective surgery. Twenty patients had docu- obstruction, secondary to cricoarytenoid inflammation.
mented craniocervical instability and, despite different airway About 10% of RA patients have Sj¨ gren syndrome (keratocon-
o
management techniques, there were no reports of neurological junctivitis sicca due to lacrimal dysfunction and reduced tear
damage. In order to diagnose cervical instability, the following formation), and require special attention to prevent corneal inju-
x-rays are needed: lateral views of the neck in flexion and exten- ries, especially if a general anesthetic is required. Similarly, dys-
sion, an antero-posterior view of the cervical spine in addition to function of the salivary glands can result in a dry mouth, making
frontal ˜˜open mouth™™ view of the odontoid process of C2. These the mucosa more vulnerable to damage after instrumentation.
images may be difficult to interpret in advanced disease and Patients with RA are particularly susceptible to injuries caused
magnetic resonance imaging (MRI) scans may be needed. by improper positioning. Arrangements should be made for addi-
Sagittal and axial images in both flexion and extension (functional tional supports and/or padding before beginning any anesthetic.
MRI) are useful for demonstrating atlantoaxial and subaxial sub- The neck and lower back should be well padded and supported,
luxation and the presence of cord compression.42 and extensive hip flexion or rotation avoided.
The TMJ can be assessed by noting any clicking, locking, or
limitation of movement. Mouth opening involves two distinct
Table 23.5 Problems with awake, fiberoptic intubation
motions of the TMJ: firstly, a hinge type action, allowing the
in parturients with rheumatoid arthritis
mouth to be opened halfway, and secondly, a forward gliding of
the condylar surface on the articular surface enabling full opening
1. Fetal side effects of maternal sedative drugs
of the mouth. Normal mandibular opening in an adult requires
2. Risk of aspiration of stomach contents (especially as pregnant
both motions with normal values for interincisor gap > 3 cm.43
patients known to be at risk of regurgitation) following topical local
Evaluation of the second motion of the TMJ may be accomplished
anesthesia
by having the patient try to protrude the mandibular incisors in
3. Risk of severe hemorrhage (due to nasal engorgement during
front of the maxillary incisors. The combination of an interincisor
pregnancy) if nasal fiberoptic intubation used
gap of <4“5 cm and the inability to protrude the mandibular
4. Coughing following airway manipulation/topical local anesthesia.
incisors in front of the maxillary incisors is highly indicative of
Potential neurological consequences in patient with cervical
critically limited TMJ mobility44 and the need for a different
subluxation
technique to secure the airway.



409
5 Other disorders


Regional anesthesia is not contraindicated in RA parturients.
Table 23.6 Diagnosis of systemic lupus erythematosus51,52
A paramedian approach to the spinal canal may be easier if the
interspinous ligament is calcified or osteophytes are present. Criterion Definition

Malar rash Fixed erythema over malar eminences, often
Postpartum management with sparing of nasolabial folds
Discoid rash Erythematosus raised patches with
Approximately 90% of RA patients deteriorate in the early post-
adherent keratotic scaling and follicular
partum period so antirheumatic medications may have to be
plugging
reintroduced or dosages increased.15 The choice of medication
Photosensitivity Skin rash as a result of unusual reaction to
has to be balanced against risks associated with breast feeding.
sunlight, by patient history or physician
Prednisone and NSAIDs are probably the best choices as these are
observation
considered safe in breast feeding. Despite an increased preva-
Oral ulcers Oral or nasopharyngeal ulceration, usually
lence of depression in RA patients compared with the general
painless, observed by physician
population, there is no evidence of increased postpartum depres-
Nonerosive arthritis involving !2 peripheral
Arthritis
sion.49 However, additional support for the mother is important
joints, characterized by tenderness, swelling,
as reactivation of the disease, in addition to the demands of a new
or effusion
baby, may cause considerable problems.
Serositis Pleuritis “ convincing history of pleuritic pain or
rub heard by a physician or evidence of pleural
Systemic lupus erythematosus (SLE) effusion
or
Systemic lupus erythematosus is a chronic inflammatory disease
Pericarditis “ documented by EKG, rub, or
of unknown etiology. Patients with SLE show a great diversity of
evidence of pericardial effusion
signs and symptoms making diagnosis from similar disorders
Persistent proteinuria > 500 mg per day (or >3 þ
Renal disorder
difficult. Prevalence is reported as 40“50 cases per 100 000 of the
on dip stick if quantization not performed)
population and this figure is thought to be increasing, probably
or
related to improvements in diagnosis.2 Systemic lupus erythema-
Cellular casts (may be red cell, hemoglobin,
tosus has a mean age of onset of 25 years and is commoner in
granular, tubular or mixed)
nonCaucasians with an adult female to male preponderance of
Neurological Seizures or psychosis (in the absence of
10“15:1, making it the commonest of the collagen vascular dis-
offending drugs or known metabolic
eases seen in pregnancy.50 Although the etiology of SLE is
derangements “ uremia, ketoacidosis, or
unknown, it is clearly multifactorial, with hormonal (e.g. estro-
electrolyte imbalance)
gen), genetic (e.g. HLA B8, DR2, DR3 haplotypes), immunolo-
Hematological Hemolytic anemia (with reticulocytosis) or
gical, and environmental (viruses, ultraviolet light) factors
Leukopenia (<4 000/mm3 total on two or more
contributing to the initiation and progression of the disease.
occasions) or
Clinical effects are mediated directly or indirectly by antibody
Lymphopenia (<1 500/mm3 on two or more
formation and/or the deposition of immune complexes. The
occasions) or
American College of Rheumatology proposed a list of criteria to
Thrombocytopenia (<100 000/mm3 in the
establish the diagnosis of SLE,51,52 where four or more of the
absence of offending drugs)
criteria are necessary to meet the entry requirements for clinical
Immunological Positive antiphospholipid antibody or
studies. However, only one of the classical clinical signs plus
AntiDNA (antibody to native DNA in abnormal
immunological findings (antinuclear and anti DNA antibody
titer) or
measurements) is sufficient for the clinical diagnosis of SLE.53
AntiSm (presence of antibody to Sm nuclear
Antinuclear antibodies, anti-double-stranded DNA antibodies,
antigen) or
and antibodies to extractable antigens (e.g. antiRo, antiLa anti-
False positive serological test for syphilis known
bodies) are found in 90%, 80%, and 30% of SLE patients respect-
to be positive for at least six months and
ively. The commonest disease pattern includes a mixture of
confirmed by Treponema pallidum
constitutional complaints (e.g. fatigue, fever, weight loss)
immobilization or fluorescent treponemal
together with musculoskeletal (e.g. arthralgia, arthritis), skin,
antibody absorption test
mild hematological, and serological involvement. However,
Antinuclear An abnormal titer of antinuclear antibody by
some patients experience predominantly renal, central nervous
antibody immunofluorescence or an equivalent assay at
system (CNS), or hematological manifestations (see Table 23.6).
any point in time and in the absence of drugs
The clinical course of the disease is highly variable and charac-
known to be associated with ˜˜drug induced
terized by relapses and remissions. Exacerbations may be precipi-
lupus™™ syndrome
tated by ultraviolet light, infections, drugs, or pregnancy. Prognosis
is related to the severity of the disease and the degree of organ EKG ¼ electrocardiogram
involvement. Factors associated with increased morbidity and



410
Chapter 23


earlier onset of organ damage include Hispanic ethnicity, greater proteinuria, hypertension, and worsening renal function,
disease activity, and a history of thrombotic events.54 Poor survival although the presence of active urinary sediment (white cell, red
prognostic factors in SLE patients include renal disease (particu- cell, or granular casts), reduction of C3/C4, and disease activity in
other organs, favor the diagnosis of a lupus nephritic flare.67 Risk
larly diffuse proliferative glomerulonephritis), male sex, hyperten-
sion, poor socioeconomic class, black race, high disease activity, factors for developing PET in these patients include renal disease,
presence of antiphospholipid antibodies (aPLs), and secondary aPLs, preexisting hypertension, diabetes mellitus, and preexisting
antiphospholipid syndrome (APS).55,56 Approximately 6% of thrombocytopenia.69 Other potentially lethal complications of
women with SLE will have a coexisting autoimmune disease, SLE include severe lupus nephritis, severe serositis, cerebritis,
most commonly secondary APS. adult respiratory distress syndrome, and pulmonary hemorrhage.
Although pulmonary hemorrhage occurs in only 2% of patients
with SLE, young women, and hence the parturient population,
Effect of pregnancy on systemic lupus
are at increased risk.70 Mortality and morbidity from this compli-
erythematosus
cation remain high and largely result from respiratory failure and
Although pregnancy does not seem to affect the long-term out- major blood loss. The efficacy of available therapeutic options has
come of SLE, its impact on acute exacerbations remains uncer- not been fully evaluated due to lack of randomized controlled
tain57 because of the normal spontaneous fluctuations of the trials. Keane and coworkers71 described alveolar hemorrhage in a
condition. However, the majority of studies suggest the disease parturient with SLE which was refractory to treatment with ster-
deteriorates in pregnancy.20 Two prospective studies showed oids and azothiaprine and necessitated the use of cyclophospha-
a significant increase in the rate of flares in pregnancy, with mide. Despite treatment, respiratory failure and recurrent
mostly constitutional, articular, and cutaneous manifesta- alveolar hemorrhage only resolved after termination and delivery
tions.58,59 In contrast, another study found no detrimental effect of a nonviable fetus.
on the disease.60 Rates of exacerbations of SLE during and
after pregnancy range from 13.5“65%.59,61,62 Flare frequency Effect on fetus
increased with greater disease activity at time of conception, Patients with SLE have an increased rate of fetal loss (sponta-
lower serum albumin, proteinuria, and the use of prednisolone neous abortion, stillbirth) compared to the normal population.
and hydroxychloroquine.62,63 Clearly, these risk factors may Reported fetal loss has ranged from 20“30%62 and increases in
represent patients with more severe disease. Patients who con- patients with aPLs, low levels of pregestational serum albumin,
and high disease activity.62,72,73 Other fetal risks include IUGR
ceive during active SLE or in the presence of lupus nephritis have
and prematurity.62,73,74 Long-term effects seen in children born
a 50% probability of serious exacerbations during their pregnan-
cies.64,65 Approximately 10% of SLE patients with preexisting to SLE mothers include an increased risk of learning disabilities in
renal involvement will develop permanent renal dysfunction.13 male offspring.75 As well there is a risk of neonatal lupus, a

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