The Lupus Book: A Guide for Patients and Their Families, Third Edition (21 page)

difference between CNS vasculitis and lupus with fibromyalgia, which he

had diagnosed a year earlier. He showed how fibromyalgia can mimic in-

flammatory (or vasculitic) symptoms and explained that steroids make fi-

bromyalgia worse. Angela was started on Elavil (a tricyclic antidepressant

that, in low doses, promotes sleep, relaxes muscles, and decreases pain

perception) for fibromyalgia, and she was quickly tapered off the steroids.

Many of my lupus patients may complain of difficulty sleeping and cognitive

problems (e.g., decreased ability to concentrate) similar to those observed in the chronic fatigue syndrome as well as lack of stamina and chronic muscle tension

headaches. Fibromyalgia is a syndrome that makes one very sensitive to pain;

it afflicts 6 million Americans. Even though its cause is unknown, 15–40 percent of patients with systemic lupus have a concurrent fibromyalgia syndrome characterized by at least 11 of 18 specific tender points throughout the body and

increased pain in the soft tissues. The administration of steroids, with an ad-

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justment of their doses, induces most of the fibromyalgia I see in lupus patients, although poor coping mechanisms and untreated inflammation leading to a secondary fibromyalgia are also, less commonly, causes of the syndrome.

Since medications used to treat lupus do not help fibromyalgia and cortico-

steroids can worsen its symptoms, I make an effort to rule out active lupus

before assuming that fibromyalgia is causing these symptoms. This can be tricky and difficult. Tricyclic antidepressants, muscle relaxants, and drugs that boost a chemical in the brain called serotonin are used to treat the syndrome, which is reviewed in depth in Chapter 23.

The Peripheral Nervous System

Sarah was an accomplished artist when she was told she had lupus. For-

tunately, it was relatively mild and controlled with antimalarial drugs. One

day, having recently gotten over a cold, she was sitting at her easel in a

sunny spot out of doors. When she tried to stand up, however, she could

not raise her left foot above her ankle. For the past few weeks, Sarah had

noticed intermittent symptoms of numbness and tingling in her left leg and

foot, but she thought nothing of it. Now, she was sent to a neurologist,

who performed a muscle study called an electromyogram (EMG) along

with a nerve conduction study. Her ‘‘foot drop’’ turned out to be consistent

with mononeuritis multiplex resulting from active lupus. Sarah was started

on high doses of steroids for a few weeks and made a complete recovery.

I occasionally come across patients with symptoms of numbness and tingling

with or without a burning sensation or an inability to move part of the body.

These problems fall into the domain of the peripheral nervous system, which

consists of the twelve cranial nerves that are found in the face as well as nerves emanating from the cervical, thoracic, lumbar, and sacral spine. These nerves

are divided into motor and sensory roots. Impairment of motor nerves leads to

problems with movement, ranging from Bell’s palsy in the face to a wrist or

foot drop in the extremities. Sensory defects produce numbness and tingling.

The CNS includes the spinal cord and brain and is distinguished from the pe-

ripheral nervous system for purposes of diagnosis and treatment.

Between 10 and 20 percent of patients with lupus exhibit inflammation of the

peripheral nervous system at some point. Peripheral neuropathies can result from inflammation of the nerves, a consequence of lupus (which is also called
mononeuritis multiplex
), or compressed nerves, which can result from lupus synovitis, as in
carpal tunnel syndrome
. Many other conditions are associated with peripheral neuropathies and must be considered in what physicians term a ‘‘dif-

ferential diagnosis.’’ These include fibromyalgia-induced numbness and tingling (which produces normal EMGs and nerve conduction studies), diabetes, kidney

failure, neuropathy, or a herniated disc.

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Inflammation in peripheral nerves is managed with short courses of moderate-

to high-dose corticosteroids, and compressed nerves may respond to anti-

inflammatories, local injections, splinting, or surgical decompression.

The Autonomic Nervous System

When you sweat, urinate, or have palpitations, your autonomic nervous system

is at work. These are the body functions we have some control of and rarely

think about—like breathing and the heart rate. The autonomic nervous system

is our ‘‘fight or fright’’ response to any form of stress. This system regulates adrenalin release, the tone of local blood vessels, and muscular contractions.

Rapid or slow pulse rates, sweating, feelings of hot and cold, rapid or slow

bladder and bowel transit times, and burning sensations are counted among our

autonomic responses. Although inadequately studied in lupus, the autonomic

nervous system may also be impaired in many patients. Table 15.2 provides

examples of autonomic-related complaints some lupus patients report.

NERVOUS SYSTEM SYNDROMES THAT AFFECT BEHAVIOR

Cognitive Dysfunction

Neil was an accountant with a large firm and nobody knew he had lupus.

Since having been treated successfully for pleurisy and joint swelling a

year before, Neil would not even think about his illness, and his general

practitioner had him on no treatment other than occasional aspirin. Over

the past month, Neil had encountered difficulty in remembering the names

of his secretary’s husband, the postman, and the parking attendant. If he

tried hard enough, the names came to him, but it took a few minutes. Neil

was surprised to find that none of his coworkers noticed his poor recall; it

was so bad that he had to look at his appointment schedule every hour to

remember what to do next. He returned to the consulting rheumatologist,

who initiated a neurologic workup that uncovered nothing unusual. How-

ever, when Neil was given some psychological tests for memory and other

Table 15.2.
Examples of Autonomic Nervous Dysfunction in Lupus

1.
Raynaud’s phenomenon:
Fingers turn different colors in cold weather 2.
Lupus headache:
A migraine-like headache from dilation of blood vessels 3.
Cognitive impairment:
Constriction of brain blood vessels decreases oxygen flow 4.
Livedo reticularis and palmar erythema:
Lace-like, checkerboard mottling of the skin due to altered autonomic signals to superficial capillaries and increased flow through small, superficial arteries

5.
Mitral valve prolapse:
Palpitations due to release of adrenalin which increases heart rates 6.
Numbness, burning, tingling:
Abnormal vascular tone on nerves which activates their sensors; nerve inflammation, or neuritis, needs to be ruled out

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Where and How Can the Body Be Affected by Lupus?

thinking abilities, some odd results turned up. Neil denied depression or

fibrositic pain. His rheumatologist placed him on Plaquenil and 3 months

later added quinacrine. Although Neil showed a 70 percent improvement,

he still had to take it easy and pace himself in order to function well at

work.

I frequently encounter lupus patients who complain of confusion as well as

profound fatigue, difficulty in articulating thoughts, and memory impairment.

Blood testing may confirm evidence of systemic lupus, but other tests may be

normal. Conventional spinal fluid evaluations and brain imaging frequently show no abnormalities, and these individuals often look well. A superficial examination of mental function will detect no deficiencies, leaving the physician puzzled. When this occurs, some well-meaning physicians may explain to their

patient that they are depressed, stressed, or having difficulty coping. They may have lupus, but the disease could not be causing their symptoms.

How Can I Convince My Doctor This Really Exists?

Even though this scenario is still all too common, studies began to appear in

the early 1980s showing that lupus could be responsible for a whole host of

subtle cognitive difficulties. Behavioral testing of lupus patients revealed that up to 70 percent at times had decreased ability to focus, deficits in attention span and task completion, altered memory, and decreased problem-solving capabilities. Only 20 percent of control subjects had similar difficulties.

The cause of cognitive dysfunction is not known, but it is probably mediated

by two factors. First, circulating chemicals such as cytokines (Chapter 5) may

induce the syndrome. Some of these cytokines work differently in lupus patients, particularly interleukin-1, interleukin-6, and the interferons, and have been

shown to cause cognitive dysfunction when they are administered to patients.

However, the symptoms of cognitive dysfunction are often intermittent.

Recent work has suggested that blood-flow abnormalities (termed hypoper-

fusion) may play a role. For example, on a newer form of brain imaging known

as PET (positron emission tomography) scanning, lupus patients with cognitive

dysfunction display specific areas of the brain that do not receive enough oxy-

gen, and this correlates with their symptoms. This $5000 test is usually not

medically necessary in diagnosing the syndrome, since it can be detected by

routine neuropsychological testing (paper-and-pencil tests) or a cruder form of PET called SPECT (single-photon-emission computed tomography), and fMRI

or functional MR Imaging.

Cognitive dysfunction must be differentiated from depression, fibromyalgia,

behavioral alterations due to medication, infections, strokes, or other brain disorders. In certain patients with cognitive symptoms, we sometimes find increased
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antibody levels that react to nerve cells in the spinal fluid and evidence of active lupus on blood testing.

Can Anything Help Cognitive Dysfunction?

The treatment of cognitive dysfunction, which may come and go on its own, is

often unsatisfactory. Emotional support and reassurance are important. Corti-

costeroids are a two-edged sword, and there is little evidence that these drugs alleviate the syndrome when other evidence for active lupus is not also present.

I prescribe antimalarial drugs, particularly hydroxychloroquine (Plaquenil), for active lupus and quinacrine for profound fatigue. Cognitive behavioral therapy, EEG and regular biofeedback are quite useful. Tricyclic antidepressants or specific serotonin reuptake inhibitors (e.g., Prozac, Zoloft, Lexapro), and modafanil (Provigil) may also be useful. Dehydroepiandrosterane (DHEA), St. John’s

Wort, counseling and gingko biloba are complementary medicine alternatives.

Organic Brain Syndrome

Charlene barely survived an episode of CNS vasculitis when she was 20.

At that time, she had high fevers, seizures, and psychotic behavior, and

lapsed into a coma for 3 weeks. Now 40 years of age, Charlene is slightly

retarded and picks up supplies for Goodwill Industries. Her lupus has not

been active in over ten years. Charlene moved to the country to be with

her boyfriend. She had not had a seizure in 2 years; therefore, when her

epilepsy prescription lapsed, Charlene figured that it was no longer nec-

essary. Last week Charlene had a grand mal seizure at the Goodwill loading

dock. Her new doctor looked at her chart and noticed a long-standing his-

tory of lupus. He placed her on 60 milligrams of prednisone a day for

presumed CNS vasculitis with seizures, but Charlene immediately became

agitated and displayed psychotic tendencies. Her old rheumatologist was

called on the telephone and explained to her doctor that she had a chronic

organic brain syndrome and probably had a seizure due to scarring in brain

tissue because of the earlier episode of CNS vasculitis. Charlene was taken

off prednisone and resumed her anticonvulsant medication.

In patients who have had a previous stroke because of antiphospholipid an-

tibodies and in those with a history of CNS vasculitis, brain lesions heal with scarring. This results in permanent motor and mental deficits as well as seizures.

These patients have what is called
organic brain syndrome
or clots. They do not have active lupus but have scars from previous inflammation related to lupus.

Therefore, steroids do not help them and will only increase brain atrophy. Since blood tests and spinal fluid usually reveal that the lupus is inactive, a patient’s history is very important. Organic brain syndrome is managed with emotional

support and, if needed, psychotropic medications or anticonvulsants.

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Psychosis

Sometimes lupus patients may demonstrate symptoms of psychosis. Psychosis

is defined as an inability to judge reality, marked by disordered thinking and

bizarre ideas, often including delusions and hallucinations. It usually results in an inability to carry out the ordinary demands of living. The incidence of acute (and fortunately, temporary) psychosis is between 10 and 15 percent during the

course of systemic lupus.

Most psychotic episodes occur with CNS vasculitis, but others occur as a