Read The Lupus Book: A Guide for Patients and Their Families, Third Edition Online
Authors: Daniel J. Wallace
but can flare it. Infections can be difficult to diagnose. The principal manifes-
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The Management of Lupus Erythematosus
tations of infections include fever, sweats, and shaking chills. If you have a high fever for more than a few days, you should be thoroughly evaluated, especially
if you are also taking NSAIDs or steroids, which lower the body temperature.
I try to take a thorough medical history, noting symptoms and inquiring about
recent travel (especially abroad), exposure to illness, purchase of a new pet, and occupation-related illnesses. I often order a throat, urine, blood, or stool culture in addition to a complete blood count or chest x-ray. Other causes of fevers
such as active lupus, cancer, allergy, or a drug reaction must be considered.
Some physicians find a C-reactive protein (CRP) blood test helpful in differ-
entiating active lupus from infection, but this is controversial. Anybody with a suspected life-threatening infection from an unknown source may have to be
observed and have cultures taken in a hospital where if necessary gallium scan-
ning, bone marrow biopsy, lymph node biopsy, or lung bronchoscopy can be
performed to make a critical diagnosis as safely and quickly as possible and
where intravenous antibiotics are readily available.
HOW ARE INFECTIONS TREATED?
Many rheumatologists have noted that patients with SLE need higher doses of
antibiotics for longer periods of time than healthy people do. Most infections
can be treated on an outpatient basis, but serious bacterial processes and most opportunistic infections necessitate a period of in-hospital intravenous antimi-crobial therapy. Antimicrobial agents fight numerous types of organisms. Anti-
biotics attack bacteria and prevent them from reproducing. Antiviral, antipar-
asitic, antiprotozoan, and antifungal drugs are also available. Although steroids may delay the response to antibiotics, the two can be taken together, since lupus frequently flares up when the patient becomes infected.
CAN INFECTIONS BE PREVENTED?
Some prevention is possible and just requires common sense. For example, a
lupus patient’s exposure to people with colds or other infections should be minimized. Antibiotics are sometimes given to such patients as a preventive measure before, during, or after certain surgical and dental procedures. A patient’s dentist should know that he or she has lupus. Dental procedures in those with heart
murmurs or phospholipid antibodies or endocarditis are managed with at least
2 to 4 grams of ampicillin or amoxicillin 2 hours before and, if necessary, the dose can be divided so that some is given 4 to 6 hours afterwards. If a patient is allergic to penicillin, there are substitutes available. I occasionally encounter lupus patients who develop repeated colds or infections. For these individuals
and others at high risk (e.g., patients who work at day-care centers or are nursery or kindergarten teachers), I boost immunity with a gamma globulin shot every
3 to 4 months. While there is no proof that this is effective, I have found that it decreases the intensity, duration, and number of minor infections. During flu
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season, exposure to a person with influenza usually warrants a 48-hour course
of amantadine (Symmetrel, 100 milligrams twice a day), Tamiflu, 75 mg twice
a day for 2 days or rimantadine (Flumadine, 100 milligrams a day for 2 days)
to prevent the development of symptoms. The key to prevention is to be aware
of the conditions in your environment and make sure your doctors and dentist
know you have lupus so they can take necessary steps to protect you.
WHAT ABOUT VACCINES?
Occasional reports have appeared of healthy persons developing lupus after re-
ceiving a routine vaccination. The concept of vaccinating patients with small
amounts of a provoking substance, with the goal of having them make antibodies
to an infectious agent, dates back to Edward Jenner’s experiments with smallpox in the 1700s. Nearly all individuals with SLE have been vaccinated against a
variety of diseases with little difficulty. Some vaccines use live organisms (e.g., virus) and others do not. Vaccines against measles, mumps, and polio, among
others, use live viruses. Even though there is a theoretical risk in exposing
a lupus patient to a live virus or to a family member who has received a
live-virus vaccine, there has never been a case report of the patient contract-
ing the disease. Passive immunization with nonspecific antibodies such as
gamma globulin poses no problems in patients with SLE. On the other hand, I
have frequently been asked whether patients can develop lupus from a vaccine.
While this is theoretically possible, it is a very rare occurrence and probably happens in no more than one out of several thousand genetically predisposed
persons.
Difficulties have been encountered with some types of immunizations in lupus
patients. Some investigators have observed that certain patients who received
tetanus or flu vaccines, for example, also made antibodies to DNA or other
lupus autoantigens. It seems that flu vaccines do not work as well if the patient has active SLE; antibodies achieve only half the desired levels for half as long.
Additionally, up to 20 percent of those with SLE may feel sick or achy for a
few days, which is double the incidence in the general population. These patients should consult their physicians before they receive any vaccine. Most rheumatologists give their lupus patients flu shots; others chose to prevent influenza with antiviral antibiotics when their patients are exposed. As a general practice, I give routine flu shots to patients with stable, relatively inactive lupus. In potentially serious circumstances, however, rheumatologists rarely hesitate to give necessary vaccinations.
ARE LUPUS PATIENTS MORE LIKELY
TO HAVE ALLERGIES?
Generalized allergies or increased sensitivities to environmental chemicals or
drugs are observed in 10 percent of the general population and in 20 to 25
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The Management of Lupus Erythematosus
percent of those with SLE. Patients with lupus are more liable to have drug,
insect, and skin allergies as well as asthma. What does this mean for the patient?
First of all, allergic lupus patients have no difficulty using the antihistamines, inhalers, nasal sprays, psendoephedrine-containing decongestants, or steroids
that many otherwise healthy allergy-prone patients take from time to time. On
the other hand, sulfa antibiotics can make them more sun-sensitive and cause
more allergic reactions; they should be avoided if possible. (These antibiotics are arylamine sulfas; most lupus patients do fine with nonarylamine sulfa-based medicines.) Also, a minority of lupus patients who receive
allergy shots (im-munotherapy)
make more autoantibodies and experience disease flareups. For this reason, in 1989, the World Health Organization recommended that patients
with autoimmune diseases should
not
routinely receive allergy shots. Nonspecific allergy shots might cause them to make more anti-DNA and other lupus-
related antibodies in addition to making antibodies against the offending allergen.
WHAT IS OSTEOPOROSIS?
With the passage of time, all of us are likely to have some thinning of our
bones. Manifested by a loss of calcium in bone mineral, this can lead to frac-
tures, bone pain, and shorter stature. The consequence can be an inability to live independently. Lupus patients are especially susceptible to this demineralization process, or osteoporosis.
We have two types of bone: cortical (as in the hips) and trabecular (as in the
vertebrae of the spine). With age, men and women become osteoporotic, or lose
calcium in their cortical bones. Additionally, the onset of menopause selectively demineralizes trabecular bone. Women are more susceptible to osteoporosis in
general. Persons who are Caucasian, have thin builds, abuse tobacco or alco-
hol—as well as those with a genetic predisposition—are also at increased risk.
Inflammation from lupus and the use of corticosteroids accelerate this process, which is why osteoporosis is so common in SLE. Further complicating this
picture is the general hesitancy of doctors to prescribe hormones to lupus pa-
tients and evidence that certain chemotherapies can bring on premature meno-
pause.
How Is Osteoporosis Managed in Systemic Lupus?
Despite all the gloomy risk factors detailed above, an intelligent woman with
lupus who works with her health-care team can frequently prevent the serious
complications induced by osteoporosis. I usually order a
bone mineralization
study
in women at risk to help me decide what management is optimal. These studies go under several names, such as QDRs, QCTs, or dual-energy x-ray
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[241]
absorptiometry (DEXAs). They are inexpensive, painless, take 15 minutes to
perform, and provide a lot of information. I usually order them at 1- to 2-year intervals in high-risk patients, patients who will be on steroids for at least several months, and at the onset of menopause. One has osteopenia if their base mineralization is 1–2.5 standard deviations below the mean, and osteoporosis if
greater than 2.5.
Women with early SLE should initiate preventive measures such as taking 1
to 1.5 grams of calcium by mouth daily in divided doses. Along with a well-
balanced diet, preparations such as Citrical, Tums EX, Os-Cal, and Posture are
easily obtainable without a prescription. A regular exercise program, as reviewed in Chapter 24, also decreases demineralization rates. The role of postmenopausal replacement estrogen therapy is reviewed in Chapter 28. These agents are well
tolerated in lupus. The American College of Rheumatology issued guidelines
suggesting that all patients on 5 mg of prednisone a day or more should also
take a bisphosphonate. The weekly administration of alendronate (Fosamax) or
risedronate (Actonel) or yearly, intravenous infusions of zoledronic acid (Zo-
meta) is appropriate for lupus patients on corticosteroids, those with osteopo-
rosis, or active disease with osteopenia. Patients with severe osteoporosis or
recurrent fractures should consider parathyroid hormone injections (teriparatide—
Forteo) for two years without a bisphosphonate. Raloxifene (Evista) or nasal
calcitonin are weak agents which may increase trabecular mineralization and are occasionally useful. Table 29.1 summarizes available approaches. The most important thing one can do regarding osteoporosis is to be aware of the risk of
this condition and try to prevent it.
Table 29.1
The Management of Osteoporosis in SLE
1. Try to eat foods with high calcium contents (the average U.S. diet contains 600 mg a day) Milk, 8 oz, 300 mg
Hard cheese, 1 oz, 200 mg
Ice cream, 1 cup, 176 mg
Oysters, 1/2 cup, raw, 113 mg
Broccoli, 1 cup, 136 mg
Sardines, canned, 3 oz, 372 mg
One large orange, 78 mg
Spinach, 1/2 cup raw, 111 mg
Yogurt, 8 oz, 400 mg
2. Oral calcium supplements including examples of calcium products. Never taken in over 600
mg at a time. The body does not absorb more. Reserve some calcium for bedtime.
Calcium carbonate: OsCal, Tums, Titrilac, Maalox, Mylanta
Calcium citrate: Citracal, Caltrate
Calcium lactate: Store brands
Calcium gluconate: Store brands
3. Vitamin D improves the absorption of calcium by the gastrointestinal tract. The easiest way to derive enough Vitamin D is by taking two multivitamin tablets a day.
4. If appropriate, Calcitonin, estrogen replacement therapy or Evista can be prescribed and have modest effects on bone mineralization.
5. Bisphosphonates are the agents of choice for lupus associated demineralization.
6. Forteo injections without bisphosphonates are used to manage severe osteoporosis.
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The Management of Lupus Erythematosus
Summing Up
Lupus is complicated by an increased susceptibility to infection, and this is
associated with a greater risk of disease-related complications. A rheumatologist or family physician should be consulted (at least by telephone) before a patient with SLE takes an antibiotic or receives a vaccine. Prudent preventive measures help prevent problems later. When there is a question of serious, life-threatening infection, careful evaluation (and perhaps hospitalization) is essential. Patients with lupus are more vulnerable to allergies, drugs, insects, and chemicals. Extreme caution should be exercised before taking allergy shots or sulfa antibiotics.
Patients who have risk factors for osteoporosis should arrange to have them-
selves tested and, if necessary, treated, since this condition is more common
with lupus.
Most women with SLE want to have children. This is certainly understandable,
since 90 percent of patients with lupus are female and 90 percent develop the
disease during their reproductive years. Unfortunately, many of my colleagues
advise lupus patients not to become pregnant on the basis of incorrect or out-
dated information. In addition, doctors are notorious for lacking a special sensitivity that is often needed when pregnancy is ill advised. The good news is
that the overwhelming majority of lupus patients can have normal babies. On