The Lupus Book: A Guide for Patients and Their Families, Third Edition (48 page)

The Social Security Administration will review your medical file and often have one of their consulting physicians examine you. Individuals with organ-threatening disease infrequently have a problem being awarded these benefits.

If you are turned down, you have a right to appeal. Ask your doctor write a

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The Management of Lupus Erythematosus

note on your behalf (I have rarely encountered a “consulting physician” for

disability who is a rheumatologist. Most consultants welcome a rheumatologist’s input). At this point, consider hiring an attorney. Disability attorneys don’t get paid by you; if successful, their bill is fixed as a percentage of your benefits for a limited period of time. Vocational rehabilitation is offered by most private

disability carriers and includes job retraining, tuition payments for career-

oriented college classes, and work-function assessments to see what skills you

can offer in the workforce. Infrequently, lupus patients are covered by insurance and disability provided by Worker’s Compensation or automobile, or home-owner’s insurance carriers, which is beyond the scope of this discussion.

32

What’s the Prognosis?

When I first diagnose a patient with systemic lupus, she frequently inquires

about the bottom line. She wants to know outcomes. Will I live? Can I have

children? Is this crippling or deforming? Many lupus patients have excellent

outcomes, live normal lives, and generally feel well. Others have serious im-

pairments. I have to tell my patients that the prognosis, or bottom line, varies from individual to individual, and is dependent on many factors.

WE’VE COME A LONG WAY!

I still come across newly diagnosed patients who are convinced that lupus is a

fatal disease and that they only have 6 months to live. This nonsense is based

on perceptions by some older physicians, who have had limited recent exposure

to lupus patients. It also stems from the many outdated encyclopedias and ref-

erence books that line our family bookshelves. The first lupus survival study

was published in 1939; it stated that half these patients were dead within 2

years. This 50 percent survival figure has constantly been revised. In 1955, half the patients were dead within 4 years, and by 1969, within 10 years.
In fact, at
the present time, more than 90 percent of all lupus patients live more than 10

years
, although—if organ-threatening disease is present—only 60 percent survive 15 to 20 years. (This is a worldwide overall average.)

How can we account for these improvements? First of all, the discovery of

the LE prep in 1948 and ANA in 1957 allowed milder cases to be diagnosed,

which increased the number of people with the disease. This increased the pool

of SLE patients, and those with mild cases would naturally have longer life-

spans. Also, the widespread availability of steroids by 1950, chemotherapy by

1955, and dialysis by 1970 extended lifespans. Though less dramatic, additional factors have also greatly improved survival over the last 20 years: doctors have newer antibiotics to treat a variety of infections, better agents to attack high blood pressure, and a broader experience in managing disease complications.

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OUTCOME AND TYPE OF LUPUS

As you might expect, certain types of lupus have better outcomes than others.

Cutaneous (discoid) lupus and drug-induced lupus are associated with a normal

life expectancy. Patients with non-organ-threatening SLE can expect the same

outcome as that of a person without lupus; more than 95 percent survive at least 10 years.

The ‘‘prognostic variables,’’ as lupus specialists like to call them, depend on several major considerations.
Methods of health-care delivery
have an impact on determining outcome. For example, a Veterans Administration lupus study

would largely restrict itself to males. Several Kaiser-Permanente HMO lupus

surveys limited themselves to analyzing trends among middle class, insurable,

working families. Hospital-based surveys focus on a sicker population of lupus

patients. Publicly funded patients (e.g., Medicaid, county hospital patients)

clearly have worse outcomes. Bluntly stated, if patients can read or write, afford food and medications, have transportation to clinics and access to rheumatologists who know their cases, and have been educated about the disease, their

prognosis improves.
Epidemiologic factors
include race, geographic environment, and age. For example, more Asians (especially Chinese) develop SLE,

but African Americans have a more severe process. Native Americans (Amer-

ican Indians) are also particularly prone to develop lupus. Lupus is rare on the African continent but observed in 1 of 300 African American women. Childhood

SLE is more organ-threatening and severe; lupus is much milder in patients over the age of 60. Environmental considerations reveal the influence of climate,

occupation, diet, and exercise; none of these has been adequately surveyed in

lupus.

Genetic factors
influence outcome. Males make up only 10 percent of all lupus patients but may have more severe disease. Also, a family history of

autoimmune disease increases awareness of lupus, which can lead to earlier

diagnosis and intervention.

Clinical and laboratory variables
—findings from blood and other testing—

suggest that organ-threatening disease has a worse outcome. High blood pres-

sure, low platelet counts, anemia, elevated cholesterols, and renal involvement—

especially with nephrotic syndrome—require more attentive and aggressive care.

The presence of certain autoantibodies such as anti-Ro (SSA), anticardiolipin

antibody, and anti-RNP can be seen as distinguishing different types of lupus

with distinct treatments and outcomes.

Finally,
treatment variables
influence the course of the disease. Many rheumatologists rarely prescribe chemotherapies for kidney disease and restrict their patients to corticosteroids. When these investigators publish survival studies, they must be interpreted differently than those of centers that intervene liberally with azathioprine or cyclophosphamide. There is not right or wrong treatment,

What’s the Prognosis?

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only different outlooks and viewpoints. More subtle ‘‘adjunctive’’ measures—

such as whether or not doctors prescribe birth control pills, immunizations, antimalarials, special diets, and so forth—can also affect quality of life and survival.

PROACTIVE AND PREVENTIVE STRATEGIES FOR

MANAGING LUPUS WITHOUT USING LUPUS MEDICINE

The last decade has been characterized by the development of lupus consortiums

and cohorts. Organizations such as SLICC (the Systemic Lupus International

Coordinating Committee) have spearheaded “outcomes” research, wherein pa-

tients with SLE are followed prospectively for years and a variety of factors are analyzed to ascertain which groups do better. It turns out that the prognosis of lupus can be improved by implementing measures that don’t involve using medicine. These are enumerated below.

Educating the patient
about lupus makes a difference. If you know the side effects of a medication, what symptoms and signs to look for if lupus is flaring, what to do if you develop a fever, how to pace yourself, and how to keep

physically and emotionally fit, the risk of complications is less and your quality of life improves.

Believe it or not, a prescription is actually filled and taken only 70 percent

of the time a doctor prescribes a lupus medication. Even fewer patients take the medicine as prescribed. Increasingly, lupus doctors have become aware that

patient
adherence
is a major impediment to optimal care. Perhaps we don’t explain what we are doing to the patient as well as we should. It is possible

that you get scared by what we have to say and want to “think about it.” Studies have shown that lupus clinics have a high “no show” rate and that adolescents

with lupus take their medicine as prescribed only 50 percent of the time. Indi-

viduals who are not compliant have a worse prognosis.

Access to lupus specialists
is critical. Not all lupus specialists are rheumatologists, and for reasons of personal interest and research 80 percent of lupus is managed by 20 percent of the rheumatologists in the United States. Just as with heart surgery, outcomes improve with larger numbers of lupus patients a rheumatologist treats and has experience with. Ask your primary care doctor, local

lupus society or Arthritis Foundation chapter who is taking new lupus patients

and providing good care for them. Unfortunately, the problem more often is that in a managed care setting, access to a lupus specialist is restricted to a single or occasional visit, or simply not available. Don’t take no for an answer. Be

aggressive, pushy and take the lead to make sure that your lupus care is handled by a competent, caring, knowledgeable physician who can see you whenever

necessary.

Exercise programs
can improve outcomes by diminishing muscle atrophy,

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The Management of Lupus Erythematosus

complications from injuries and bone demineralization. The use of bisphos-

phonates in patients with active lupus or in those on corticosteroids can prevent and treat
osteoporosis
. Fewer fractures lead to greater mobility, independence and self esteem.

There is increasing evidence that lupus patients with cognitive impairment

function better after a program of
biofeedback
and
cognitive behavioral therapy
.

These modalities allow lupus patients to better cope with anxiety and stress,

function better in society, and improve productivity which in turn improves

outcomes.

All lupus patients should be screened for
antiphospholipid antibodies
as well as the lupus anticoagulant. Since 12 percent of lupus patients sustain a thromboembolic event (e.g., stroke, pulmonary embolus) in their lifetime, individuals at risk can usually be identified and prophylaxed with low dose aspirin, antimalarials or other platelet inhibitors as needed.

Half of all lupus patients take alternative medicines on a regular basis, but

many never tell their doctor about it. Some of these medicines contain poten-

tially harmful materials such as phenylbutazone, corticosteroids, Echinacea, sulfa derivatives, alfalfa sprouts, ephedra components as well as impurities which can flare the disease. When your doctor asks what medicines you are taking, please

be sure to list non-prescription ones as well.

Finally, a lot of attention has focused upon preventing heart disease, especially in patients taking corticosteroids. (Lupus patients not on corticosteroids are at a slightly increased risk for atherosclerotic and thromboembolic complications due to chronic inflammation.) Measures which make a difference include blood pressure screening and treatment, cessation of smoking, weight reduction, and reg-

ularly following blood sugar and lipid tests. Individuals who have cardiovascular risk factors should have an annual electrocardiogram, baseline 2-D echocardiogram to screen for pulmonary hypertension, imaging studies to look for ath-

erosclerosis (heart scanning, duplex scanning of the carotids), and periodic stress testing (e.g., treadmill). Implementing the strategies in this section has the potential of decreasing the mortality rate of lupus by at least 50 percent and can do so without ever taking a lupus medicine! See Table 32.1.

DOES LUPUS EVER GO AWAY ON ITS OWN?

Every few months, I come across a newly diagnosed lupus patient who listens

patiently to my 30-minute speech on the disease, followed by specific therapeutic recommendations, and says, ‘‘Doctor, I appreciate everything you are telling

me, but I am going to take vitamins and herbs, eat a well-balanced diet, exercise, and see what happens.’’ What happens to these patients? Can their disease go

away on its own? Spontaneous remissions were reported as early as 1954. Non-

organ-threatening disease has a 2 to 10 percent disappearance rate without med-

What’s the Prognosis?

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Table 32.1.
Preventive Strategies without Using Lupus Medicines which Improve Prognosis
1. Patient education

2. Improved adherence to regimen

3. Exercise programs

4. Osteoporosis preventive and treatment measures

5. Cognitive therapy and biofeedback

6. Antiphospholipid antibody screening with preventive measures

7. Smart use of alternative medications

8. Screening for hypertension, hyperlipidemia, hyperglycemia

9. Smoking cessation

10. Weigh control and dietary measures

11. Cardiac screening measures (e.g., EKG, heart scanning, stress test, 2-D Echo, carotid duplex) ication. These are long-shot odds and I do not recommend taking the chance,

since non-organ-threatening disease also has a 20 percent chance of becoming

organ-threatening within 5 years, and I believe that antimalarial therapies further decrease this risk. The spontaneous disappearance of lupus in the heart, lung,

kidney, liver, or hematologic systems is so rare that when one well-documented

patient with severe lupus prayed to Father Junipero Serra (founder of the Spanish missions in California in the 1700s) and had her organ-threatening disease disappear, this evidence was submitted to the Vatican, where Serra was (and still

is) being considered for sainthood.

In non-organ-threatening disease, lupus tends to burn itself out with time.