Positive Options for Living with Lupus (4 page)

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Lupus Occurs Unevenly

In the total world population it has been calculated that between forty and fifty people out of every thousand will have lupus at some time in their lives. These estimates have nearly tripled in the last forty years. This is probably not because the disease is actually on the increase, but rather because sophisticated immunological tests introduced in recent years, combined with improved diagnostic criteria, have led to mild cases of the disease being recorded. The estimates have been increased to allow for these cases.

In North America, South America, and Europe, where statistics are the most detailed, the record of new cases (incidence) ranges from 2 to 8 per 1,000 each year. A study in Great Britain has calculated that up to 20,000 people may have lupus. Estimates in the United States range from 275,000 (from a study researching women only) to a massive 1.7 million. This last figure comes from a survey commissioned by the Lupus Foundation of America and reported in 1994. It was obtained by calling people on the telephone and asking them if they had ever been told they have lupus. The researchers admit that estimating prevalence “by unsubstantiated claim” yields a figure higher than previously expected. They write,

“Self-reporting studies are notoriously inaccurate as the criteria for the disease are not verified, indicating that the numbers derived from this study may not be true.” Not counting this extreme estimate, it is likely that the majority of available prevalence statistics conceal a lupus “iceberg,” with many more having the illness than get counted. However, national and global prevalence figures conceal massive differences in distribution between the sexes; between age, racial, and socioeconomic groups; and, to some extent, accord-ing to where people live.

Gender

Ninety percent of people who get lupus are women. Some estimates put this number even higher. It is predominantly a disease that strikes women of childbearing years (see “Age,” below). This combi-POL text Q6 good.qxp 8/12/2006 7:39 PM Page 17

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nation of characteristics suggests that vulnerability to lupus may be related to the reproductive hormone
estrogen
. Men also get lupus, as do children and women beyond reproductive age, but in every age group or other grouping, women always outnumber men. In children, in whom hormonal effects are presumably minimal, for each male, three girls get lupus. In adults, the ratio ranges from ten to fifteen women for each man. In older people (women beyond menopause, when the production of reproductive hormones is reduced) the ratio is approximately eight women for every man.

Age

As I have said, lupus strikes mostly women during their reproductive years. Sixty-five percent of patients first experience symptoms between the ages of sixteen and fifty-five. Of the remaining cases, 20 percent are affected between ages twelve and sixteen—by which age most women are sexually mature—and 15 percent after age fifty-five. Lupus does strike the very young and very old, but not sufficiently to make a statistical contribution.

Race and Geography

Different racial groups are more or less susceptible. This phenomenon is probably and principally related to the genetic differences between peoples. Tiny parts of the human
genome—
the information handed down from parent to child that programs the growth and development of the body—vary from person to person, and some genetic differences are more common in some races than others, making certain races more or less prone to certain diseases.

In every continent, there are more cases of lupus among people of African, Hispanic, and Asian descent than among Caucasians.

In France, immigrants from Spain, Portugal, North Africa, and Italy are more susceptible than native Frenchmen and -women. In New Zealand, both the prevalence and mortality of lupus are higher among Polynesians than Caucasians. The vulnerability of immigrant communities may be compounded by a general tendency for lupus to be more common in urban than rural communities.

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This tendency to affect immigrant communities disproportion-ately is something lupus shares with rheumatoid arthritis. Africans living in Africa appear less susceptible than people of African descent who live elsewhere, and certainly there is a general tendency for lupus to be more common among immigrants than among the same racial groups in their homelands, and in immigrants who move to cooler rather than tropical climes. Some researchers have suggested that this could be because immigration nearly always involves a move to cooler climes from hotter ones, and that the reduction in the amount of sunlight could play a part (see Chapter 3).

Ethnic Variables

In the 1990s the National Institution of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), the agency in the United States concerned with rheumatic disorders, started recruiting several hundred lupus patients from various groups who were between the ages of twenty and fifty. Recruits were asked to take part in a study of all aspects of the disease, from clinical characteristics to psychological factors to genetics, including the contribution of ethnic origin to the incidence of the disease. The study, called LUMINA (which stands for LUpus in MInorities: NAture Versus Nurture), is ongoing as of this writing.

Is It Serious, Doctor?

Differences in the disease’s severity and long-term outcome also occur between different groups. For example, black or dark-skinned people—people originating in tropical countries—have a poorer prognosis. Some studies also suggest that lupus is worse among those with less education and those from lower socioeconomic groups, though this may reflect the fact that such groups often have poorer access to health care or may fail to follow treatment and health guidelines (known to doctors as poor
compliance).

But these days, can lupus be fatal? The short answer is very rarely. A 1955 survey showed a five-year survival rate of only 50

percent, but we now know that the study dealt with a very small, POL text Q6 good.qxp 8/12/2006 7:39 PM Page 19

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seriously ill, largely untreated group of people with the disease. With today’s broader picture, it is possible to say with some confidence that lupus is only rarely fatal. Survival rates are measured over five, ten, and twenty years from diagnosis. In the mid-1990s, survival at five and ten years was close to 95 percent, and even after twenty years it was over 85 percent. If you have lupus now, with all the improvements in treatment that are available, it is highly unlikely to be life-threatening.

Lupus in Young Children

Lupus is rare in children under twelve, the age around when girls usually start their periods. Below the age of five it is exceedingly rare, although specialist physicians see a small number of cases between that age and adolescence.

Very occasionally, newborn babies develop a lupus-like rash in the first weeks of life. This is not true lupus and only occurs because the mother has lupus and some of the antibodies that cause the disease have crossed the placenta from her blood into the baby’s. As the maternal antibodies die down, the rash also subsides. This condition is known as
neonatal
(newborn) lupus and is discussed in more detail in Chapter 10.

Most authorities claim that there is essentially no difference between how lupus affects young children and how it affects adults.

Children who have lupus constitute a small group, so significant statistics are difficult to gather. However, some recent studies suggest that their symptoms may often be more severe than adults’. A study from the European Working Party on Systemic Lupus Erythematosus found that the pattern of symptoms in young children—

and also in old people and men—differed from the pattern most typical in women sufferers. Children were less likely to have
rheumatoid factor
(an antibody that is a frequent marker of inflammation in arthritis) in their bloodstream but more likely to suffer from the butterfly rash (also known as the
malar rash),
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problems were also the symptoms most likely to bring child lupus patients to the attention of a doctor.

Lupus in Men vs. Women

There are small differences in how lupus affects men compared with women. Men tend to be diagnosed at a later age, and the mortality rate one year after diagnosis (infinitesimally low in the treated population) is slightly higher. The study from the European Working Party on Systemic Lupus Erythematosus found that men were less likely to suffer from arthritis and photosensitivity than women, and that pleurisy and pericarditis were more frequently their presenting symptoms (the ones that made them consult a doctor).

Lupus in Older People

In the context of lupus, “older” is rather broadly interpreted by the statisticians as over age fifty-five—the age by which most women have passed menopause. Including men, some 15 percent of lupus cases do not appear until this age. The European Working Party on Systemic Lupus Erythematosus found that at this age new patients were less likely to present with the butterfly rash, arthritis, or kidney problems than children or younger adults. These symptoms continued to feature less (about half as often) during the course of the illness, as did photosensitivity and thrombosis. However, sufferers of late-onset lupus were twice as likely to suffer from dry eyes and mouth
(sicca syndrome).
Other studies found that the discoid rash—

hard, raised plaques that sometimes leave scarring—was more common in patients who developed lupus late in their lives.

In developed countries, of course, older people are the ones most likely to be taking medication for conditions other than lupus.

This introduces a group of people who, independent of age and sex, develop lupus as a consequence of taking certain drugs, a variation known as “drug-induced lupus.” These are probably the only cases where the cause is emphatically certain and the cure obvious. The POL text Q6 good.qxp 8/12/2006 7:39 PM Page 21

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drug causing the problem must be stopped or changed. Drug-induced lupus is considered in more detail in Chapter 9.

Lupus in History

The name “lupus” for a skin disease has been around for more than seven centuries.

Thirteenth century. The Italian physician Rogerius describes a disease characterized by lesions and calls it “lupus.” In medieval Latin the word for “wolf”—
lupus
—was also used to mean “ulcerated,”

perhaps because sores or ulcers that eat into the face look rather like a wolf bite.

Seventeenth century. Philosopher/physicians Paracelsus and Sen-nert provide clear descriptions of “lupus” skin lesions.

1828. French dermatologist Laurent-Théodore Biett identifies three types of lupus and coins the term “lupus erythemadoides” for the distinctive butterfly rash. His teachings are published by his pupil Pierre Cazenave in
Practical Summary of Skin Diseases
.

1873. Moritz Kaposi, professor of dermatology at the medical school at the University of Vienna, Austria, publishes a series of articles on lupus erythematosus, noting that patients with the rash also have other symptoms—in other words, that it is systemic. He writes, “Lupus erythematosus . . . may be attended by altogether more severe pathological changes . . . and even dangerous constitutional symptoms may be intimately associated with the process in question, and that death may result from conditions which must be considered to arise from the local malady.”

1890. Thomas Payne, a physician at St. Thomas’ Hospital, London, is the first to recognize that antimalarial drugs, long used to treat fever, may have more general healing powers for symptoms like joint pain and fatigue in lupus.

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1895–1903. In a series of papers, the celebrated U.S. physician William Osler describes other organs involved in lupus—heart, kidneys, and other “mucous surfaces”—and defines the condition as both systemic and chronic (relapsing and remitting).

1941. On the basis of numerous postmortem studies of damaged organs in lupus patients, Paul Klemperer, at the Mt. Sinai Hospital, New York, proposes that lupus is a “collagen vascular” disease. This term remains in use for fifty years or more.

1948. The diagnosis of lupus moves into a new phase: Malcolm Hargraves, of the Mayo Clinic, in Rochester, Minnesota, identifies an odd-looking white blood cell, first in the bone marrow, then in the blood of people with acute lupus. It becomes known as the LE

(lupus erythematosus) cell. As a result, the first blood test for lupus is devised and the number of people diagnosed rises steadily.

1954–1972. Several other anomalies are detected in the blood of people with lupus. Chief among these is an antibody that works specifically against the body’s own DNA (deoxyribonucleic acid—

the genetic raw material of living systems). A test for this antinuclear antibody—ANA—replaces the LE as the gold standard for detecting lupus, and lupus becomes located firmly in the family of autoimmune diseases. (Details of these sophisticated diagnostic tests are included in Chapter 4.)

1983. A group led by Graham Hughes, at St. Thomas’ Hospital, identifies the antibody associated with the artery and vein thrombosis, strokes, and miscarriages that had made pregnancy so risky for lupus sufferers. The condition for which this antibody is the culprit is renamed Hughes’ syndrome. (In the United States, it is more commonly known as antiphospholipid syndrome, or APS.) Treatment to counteract the effect of the antiphospholipid antibody is devised (more about this in Chapter 10).