The Lupus Book: A Guide for Patients and Their Families, Third Edition (20 page)

spinal tap was consistent with CNS vasculitis based on an elevated protein

and cell count as well as large amounts of antibodies to nerve cells. No

evidence for infection was found. Elyse was transferred to intensive care

and given high doses of intravenous steroids. She worsened for several

days and became practically comatose but eventually began to respond.

She was discharged 3 weeks later and, after convalescing for a month at

home, finally returned to school on 20 milligrams of prednisone a day.

Vasculitis of the CNS is an inflammation of the brain’s blood vessels due to

lupus activity. The most serious of the CNS syndromes associated with lupus,

it was the first to be described and is one of only two CNS syndromes (the other is psychosis) that fulfill the American College of Rheumatology criteria for

defining lupus.

Vasculitis of the CNS usually occurs early in the disease course; over 80

percent of episodes take place within 5 years of diagnosis. The typical patient experiences high fevers, seizures, meningitis-like stiffness of the neck, and may manifest psychotic or bizarre behavior. Ten percent of lupus patients develop

CNS vasculitis. Untreated, their course rapidly deteriorates into stupor and ultimately coma.

How Is CNS Vasculitis Diagnosed?

Vasculitis of the CNS can be definitively diagnosed in several ways. The phy-

sician may order a conventional angiogram (an x-ray study of the vessels of the brain after injecting them with dye), a magnetic resonance angiogram, or if

available, tests to detect high levels of antineuronal antibodies in the serum.

These tests may be negative in CNS vasculitis, but if positive, they usually

correlate with evidence from blood testing for active lupus (elevated sedimen-

tation rates, low complements, high anti-DNA, etc.).

Some of the most helpful diagnostic studies are obtained using a spinal tap.

There is no need to fear this procedure. Also known as a lumbar puncture, it

sounds scarier than it is. Spinal taps are usually only minimally uncomfortable.

It is, however, important to lie flat in bed for at least 8 hours afterwards to avoid a post-spinal tap headache. Spinal fluid, which is obtained from a spinal tap, may indicate increased white blood cells or protein, LE cells, or low sugar levels. I usually order a test known as a MS panel, so called because it was

originally used to diagnose multiple sclerosis. This panel may show data sug-

gesting an immune reaction (e.g., elevations in IgG synthesis rates, Ig levels, or the presence of oligoclonal bands) in the central nervous system. Antineuronal

antibodies are often present in large amounts.

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How Is CNS Vasculitis Treated?

CNS vasculitis is treated with high doses of corticosteroids, which are often

given intravenously. Very high dose or pulse steroid dosing (Chapter 27) may

be instituted. If improvement is not noted fairly quickly, cyclophosphamide is

given intravenously with or without a blood filtering treatment known as apher-

esis (Chapters 27 and 28). The mortality rate of CNS lupus has decreased over

the last 30 years as a result of improved diagnostic testing and greater awareness of this syndrome. In the 1950s, the majority of patients with acute episodes did not have a good chance of surviving; today, 80 percent of patients recover from such episodes. Unfortunately, doctors sometimes treat a condition that is not

present, since CNS vasculitis mimics various syndromes, some of which we

now look at.

The Antiphospholipid Syndrome

Monica thought she had licked lupus. Ten years into the disease, she was

off all medication, felt well, and was working full time as a designer. Glad

to be off Plaquenil and the anti-inflammatory Lodine, Monica had lots of

plans for the future. One day, while braiding her niece’s hair, Monica sud-

denly found herself unable to move her right arm. She saw a doctor at the

clinic she attended and was told that she had had a stroke. A brain MRI

was ordered, which showed several small defects pointing to prior CNS

episodes. The neurologist Monica was referred to confirmed the diagnosis

of stroke and, in view of her history of lupus, started her on 60 milligrams

of prednisone daily to rule out CNS vasculitis. However, the spinal tap

results were negative, as were all blood tests for lupus activity. Monica

then decided to see her rheumatologist in the next town, who stopped her

prednisone and detected, through blood tests, the presence of antiphospho-

lipid antibodies. Monica’s right arm began to return to normal after several

months and, as a long-term preventive measure, her rheumatologist placed

her on lifelong low-dose aspirin.

Anyone who has antiphospholipid antibodies (Chapter 21) has an added risk

of developing blood clots that can travel to and settle in the brain. Whether

from a Libman-Sacks endocarditis (Chapter 14) or other sources, clots to the

arteries or veins (called arterial emboli or venous thromboses) account for 10

to 35 percent of all sudden CNS complications in lupus. The disease does not

have to be active; sedimentation rates, blood complements, and anti-DNA levels

may all be normal.

Blood clots (called thromboembolic events) to the brain usually occur sud-

denly and are not associated with pain. Patients may find themselves unable to

move an arm or leg; they may develop slurred speech or acute weakness in a

particular part of the body. Eventually, MRI and CT show a focal defect in the

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affected area of the brain. Spinal fluid is usually normal. Some patients are

fortunate in having warnings of these events, which may disappear after seconds, minutes, or hours and are called
transient ischemic attacks
, or
TIAs
.

I recommend that all my patients with certain types of antiphospholipid an-

tibodies take low-dose aspirin on a continuous basis to try to prevent clots. I treat clotting episodes aggressively with anticoagulant medications or drugs that inhibit certain actions of blood platelets that are responsible for clotting blood, and I avoid using steroids unless lupus activity is present.

Other Coagulation and Flow Abnormalities

Complications in the CNS may arise when your blood becomes too thick or too

thin or when it clots too easily. Sjo¨gren’s syndrome is found in up to one-third of patients. Patients with Sjo¨gren’s complain of dry eyes, dry mouth, and arthritis. These patients have particularly high autoantibody levels on blood testing. Very large amounts of antibody, particularly IgM antibodies with or without Sjo¨gren’s, can increase the viscosity (thickness) of blood.

Several autoimmune disorders including lupus may be complicated by a con-

dition known as
hyperviscosity syndrome
, in which blood takes on the quality of sludge and is accompanied by symptoms of confusion, dizziness, and mental

clouding. Occurring as a result of an excessively large number of autoantibodies, it is treated with chemotherapy and blood filtering (apheresis). Similarly, a circulating protein that becomes solid in cold temperatures known as
cryoglobulin
is occasionally found in SLE. Because it ‘‘clogs up the works,’’ this disorder, known as
cryoglobulinemia
, is very similar to hyperviscosity syndrome, sharing many of its symptoms. Often associated with the hepatitis C virus, cryoglobulinemia is more common than hyperviscosity syndrome and is usually looked

for when the skin is ulcerated, has a mottled appearance, or shows vasculitic

skin lesions. Cryoglobulinemia responds to chemotherapy, and, if needed, apher-

esis.

Alterations in blood clotting, which can lead to bleeding from blood vessels

in the brain and therefore can mimic strokes, are observed when platelet counts become very low. Two examples of this are
idiopathic thrombocytopenic purpura
and
thrombotic thrombocytopenic purpura
. The reader is referred to Chapter 20 for more details.

Lupus Headache

Naomi had suffered from headaches for years, but this was the headache

from hell. Her lupus was responding fairly well to treatments and seemed

under control. And even though she told her parents not to worry all the

time, Naomi was a chronic overachiever and her parents couldn’t help but

be concerned. In the past, Fiorinal or an occasional aspirin with codeine

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Where and How Can the Body Be Affected by Lupus?

preparation had stopped Naomi’s headaches. Also, she found that when she

lowered her stress level and practiced biofeedback, her headaches lessened.

But neither medication nor relaxation helped this time. Her doctor gave her

an injection of sumatriptan (Imitrex), which gave Naomi some temporary

relief. When she was in his office, he drew blood and was puzzled to find

her lupus to be more active than usual. Upon seeing these results, Dr.

Metzger prescribed 20 milligrams of prednisone daily for a week, and the

headache finally disappeared.

At least once a day, one of my patients calls me and explains that his or her

head is going to ‘‘split open.’’ Compared with the general population, lupus

patients are perhaps twice as likely to suffer from migraine-like headaches.

These headaches seem to coincide with dilation of the cerebral blood vessels,

but we still don’t know their cause. Many patients also have antiphospholipid

antibodies, while others display Raynaud’s phenomenon (see Chapter 12),

which, interestingly, is caused by restriction of the blood supply to the hands and feet. This instability in the tone (ability to dilate or constrict) of the blood vessels, which allows them to be easily altered, may result from a defect in

local autonomic nervous system control.

The diagnosis of lupus headache involves a careful consideration and ruling

out of other causes of headache, including high blood pressure, osteoarthritis of the neck, fibromyalgia-associated muscular tension headaches, lupus medications that can cause headaches (e.g., methotrexate, omeprazole, indomethacin),

brain infections, or other cerebral pathology, such as an aneurysm, tumor, or

malformation of brain vessels present from birth. The physician can rule out

these possibilities by taking a history, performing a physical examination, and, if necessary, ordering an MRI scan of the brain.

Lupus headache is managed much like conventional migraine in that pain-

killers (analgesics) such as Fiorinal, sumatriptan (Imitrex) injections, anti-

inflammatories like naproxen (Naprosyn, Aleve), and vasoconstrictors such as

ergot derivatives (DHE-45, Migranal, Cafergot) are used for acute attacks, while beta-blockers, tricyclic antidepressants, or calcium channel blockers offer a degree of prevention and may sometimes be taken all the time. Lupus headache,

however, is different from most migraines, since patients may dramatically re-

spond to a 1 week trial of 20 to 60 milligrams of prednisone daily, which is

occasionally useful to migraine sufferers. Any lupus patient with a headache

that does not respond to routine measures deserves a neurologic workup.

Lupus Myelitis

This rare but serious complication of lupus can include paralysis or weakness

that ranges from difficulty in moving one limb to quadriplegia. In lupus myelitis,
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the sac encasing the spinal cord is inflamed or blood clots are formed in the

spinal arteries. Half of all lupus myelitis stems from antiphospholipid antibodies and half is from active vasculitis. The physician will probably first administer steroids to treat any possible inflammation. Anticoagulant drugs, such as heparin, are frequently added.
Chronic inflammatory demyelinating polyneuropathy, or
CIDP
, and post-infectious Guillain-Barre have an increased prevalence in SLE

and may be responsive to intravenous immunoglobulin (gamma globulin) ther-

apy.

Fibromyalgia

Angela was a shy woman without a great deal of self-confidence. When

she was diagnosed with systemic lupus, her self-esteem plummeted to an

all-time low and she had a terrible time coping. Angela read everything

she could about lupus and was almost obsessive in her desire to control

her disease. She consulted four rheumatologists before she found one who

was responsive to her needs. She called Dr. Jones three times a day about

every new development she thought he should know about. Her most trou-

bling complaint was memory loss. Over the past few months, Angela had

had difficulty remembering dates and suffered from severe headaches and

muscle spasms. She read in medical textbooks about severe CNS disorders

and became convinced that she had vasculitis. On the basis of her symp-

toms, Dr. Jones ran a blood panel that revealed a minimally elevated sed-

imentation rate and a slight increase in DNA antibody. He then prescribed

40 milligrams of prednisone a day, and she felt a bit better the first week.

In time, however, her skin became so sensitive that it could not be touched.

Also, Angela gained 20 pounds over the next few weeks. Her sister, who

worked in a medical office, insisted that Angela see a physician who had

treated her earlier in the year. At this office visit, Dr. Wolfe explained the