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403
AUTOIMMUNE DISEASES
23
Caroline Grange




Introduction the early postpartum period.6 Women are less likely to develop
the disease during oral contraceptive use or during prolonged
An autoimmune disease represents a pathological condition
breast feeding (>one year).7,8
caused by an immune response directed against an antigen
The diagnosis of RA is usually based on the clinical pattern of
within the body of the host. The most accepted theory suggests
joint involvement, presence of rheumatoid factor, and typical bony
that autoimmunity results from a failure of the normal regulation
erosive x-ray changes (see Table 23.1). The autoantibody rheuma-
of the immune system (which contains many immune cells that
toid factor is found in 80“90% of patients with RA, although RA
recognize self antigens, but are normally suppressed).1 The exact
factor titers do not correlate with disease activity. Rheumatoid
etiology of these diseases remains unclear, although there are a
factor is not exclusive to RA and is found in patients with other
number of factors that are implicated in their development,
autoimmune diseases and in some normal patients.9
including infection, hormonal effect, drug exposure, and human
Drug treatment for RA sufferers includes: analgesics (e.g. aspirin,
leukocyte antigen (HLA) type. The incidence and activity of auto-
acetaminophen, nonsteroidal anti-inflammatory drugs [NSAIDs],
immune diseases are particularly high in young women and
opioids), glucocorticoids (e.g. prednisolone), and antirheumatics
hence their occurrence in parturients is not uncommon. During
(e.g. methotrexate, hydroxychloroquine, sulfasalzine, leflunomide,
pregnancy, mother and fetus produce immunological factors to
gold, D-penicillamine, azathioprine, and cyclosporine). The cur-
limit cell-mediated immunity and prevent fetal rejection, but the
rent treatment rationale employs a combination of agents from
high estrogen environment may enhance immune function
different drug classes to maximize efficacy whilst minimizing side
resulting in these demographic findings.
effects.


Rheumatoid arthritis Effect of pregnancy on rheumatoid arthritis
Rheumatoid arthritis (RA) is a chronic systemic disorder charac- Approximately 75% of patients with RA improve during preg-
nancy, but not all studies confirm this.10,11,12 Clinical character-
terized by symmetrical polyarthritis, resulting in joint destruction
and deformity. Diagnosis depends on an aggregation of clinical istics, such as disease duration, rheumatoid factor titers, and
symptoms, signs, laboratory data, and radiological data (see functional class, do not predict the course of RA during preg-
Table 23.1). The joints primarily affected include the wrists, nancy. However, similar patterns of disease change recur
in individuals during future pregnancies.13,14 In those patients
knees, shoulders, and metacarpal-phalangeal joints, with fre-
quent sparing of the larger joints and spinal column. However, with a reduction in symptoms, the improvement usually starts
RA is a multisystem disease and extra-articular manifestations during the first trimester and continues throughout the course
include lymphadenopathy, fatigue, anemia, weight loss, intersti- of pregnancy. Unfortunately, 90% of RA patients deteriorate to
their antepartum status within three months postpartum.13,15
tial lung disease, pericarditis, subcutaneous nodules (rheumatoid
nodules), vasculitis, neuropathy, renal disease, Sj¨ gren syndrome
o Spontaneous and therapeutic abortions produce similar de-
(parotid and lacrimal hypertrophy, keratoconjunctivitis, vaginitis, terioration following delivery. There may be an increased risk of
xerostomia), and Felty syndrome (see Table 23.2). The disease developing RA in this early postpartum period, especially in first-
time mothers.10 Some authors suggest that breast feeding may be
usually follows a slow progressive course with exacerbations and
a risk factor for developing RA,16,17 but, as breast feeding occurs
remissions, although prognosis is highly variable.
The prevalence of the disease is 1% in the USA with all races at a time when disease activity normally deteriorates, the actual
being affected.2 The overall female-to-male ratio is approxi- effect is difficult to determine. In addition, the physical demands
mately 3:1, increasing to 5:1 in the pregnant population.3 The of caring for an infant may result in worsening fatigue, joint pain,
and depression.18
reason for this female preponderance is in part due to the effects
of estrogen on the immune system (inhibition of T suppressor cell
function, enhancement of T helper cell function).4 In addition,
Effect of rheumatoid arthritis on pregnancy
receptor polymorphism may be associated with the disease as
estrogen receptors have been found on synovial and memory T Rheumatoid arthritis probably does not affect biological fertility;
cells.5 Rheumatoid arthritis is polygenic in inheritance with an however, there is a significant reduction in coital frequency and
libido.19 Fetal morbidity and mortality are not increased in
increased incidence of RA in patients with the HLA-DR4 pheno-
RA parturients,11,20 but one study found a slight increase in
type. Other risk factors include smoking, nulliparity, and being in


Obstetric Anesthesia and Uncommon Disorders, eds. David R. Gambling, M. Joanne Douglas and Robert S. F. McKay. Published by Cambridge University Press.
# Cambridge University Press 2008.
5 Other disorders


spontaneous abortion.21 However, these studies may be biased as
Table 23.1 Clinical features for diagnosis of rheumatoid parturients with severe disease may choose not to have children
arthritis due to their reduced functional capacity.
A major concern in RA parturients is the potential risk from
Morning stiffness for !one hour and present !six weeks
drug therapy (see Table 23.3). For commercial and ethical rea-
Swelling of wrist, metacarpophalangeal, or proximal interphalangeal
sons, many drugs currently used to treat RA are not approved for
joints !six weeks
use in pregnancy creating a dilemma for the obstetrician and
Swelling of three or more joints for !six weeks
anesthesiologist. Fortunately, improvement in RA symptoms
Symmetric joint swelling
during pregnancy may permit a reduction in medication dose
X-ray changes to hand “ must include erosions or bony decalcification
and revision of combination therapy, reducing maternal and fetal
Rheumatoid nodules
side effects. Therapy during pregnancy should be directed at
Rheumatoid factor
using the lowest effective dose and avoiding those drugs known
to affect the fetus adversely. Clearly, where the evidence is incon-
clusive, the benefits of the drug should significantly outweigh any
potential risks or a relatively safe alternative should be chosen.




Table 23.2 Anesthetic implications of rheumatoid arthritis

Organ Disease process Anesthetic implication

Airway Mandibular hypoplasia Possible difficult intubation
Temporomandibular joint dysfunction
Cricoarytenoid arthritis
Laryngeal deviation
Cervical spine Subluxation Avoidance of excessive manipulation during GA
Use of alternative to direct laryngoscopy
Joints Joint destruction/deformity Care with positioning
Additional padding
Possible difficulty with IV placement
Risk of TE (decreased mobility)
Lumbar spine Calcification of ligaments Regional techniques may be difficult
Osteophytes Paramedian approach may be easier
CVS Pericarditis Limited cardiac reserve
Pericardial effusions Conduction disturbances
Myocarditis Antibiotic endocarditis prophylaxis may be necessary
Myocardial nodules Increased risk of end-organ damage
Endocardial vegetations
Atherosclerosis
Vasculitis
RS Pleural effusion Limited respiratory reserve
Pulmonary fibrosis
Pulmonary nodules
Costochrondritis
Neurological Peripheral nerve root compression Awareness of neurological abnormalities prior to neuraxial anesthesia
Cervical nerve root compression
Vasculitis/neurovascular disease
Hematological Anemia Reduced oxygen transport
Felty syndrome Increased risk of infectious complications
(RA and neutropenia, may be associated with Increased risk of spinal hematoma
anemia, thrombocytopenia, enlarged spleen)
Eye Scleritis Taping/padding during GA to avoid damage
Episcleritis

GA ¼ general anesthesia; IV ¼ intravenous; TE ¼ thromboembolism; CVS ¼ cardiovascular system; RS ¼ respiratory system




406
Chapter 23


Cyclosporine Hypertension
Table 23.3 Drug therapy for rheumatoid arthritis in pregnancy
Nephrotoxicity/
Drug Maternal effects Fetal effects
hepatotoxicity
Hirsutism
Aspirin Prolonged gestation/ Bleeding
Paresthesias/
labor Metabolic acidosis
tremors
Bleeding Premature closure
Anemia
of ductus
Gum hyperplasia
arteriosus
Alopecia
Pulmonary
hypertension
CNS ¼ central nervous system; NSAIDS ¼ nonsteroidal
NSAIDs Prolonged gestation/ Bleeding
anti-inflammatory drugs
labor Premature closure
Bleeding of ductus

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