Why is lupus called 'disease with a thousand faces'?

Lupus, an autoimmune disease, is a rare and incurable ailment that is difficult to manage even when treated in a hospital. It is called the disease with a thousand faces because it has a wide variety of symptoms.

So, why does lupus have such a wide variety of symptoms?

“There are more than 14,000 diseases. If you classify them before dividing them into individual diseases, one of the groups is autoimmune diseases,” said Professor Shim Seung-cheol of the Department of Rheumatology at Chungnam National University Hospital and the chairman of the Korean Lupus Research Foundation on the Korean College of Rheumatology’s YouTube channel “Arthritis TV.”

Professor Shim divided autoimmune diseases into two main groups: those that attack just one organ and those that attack multiple organs, like lupus.

“The body is made up of many organs, and those organs are made up of tissues, and each tissue is made up of individual cells. In lupus, the main problem is a factor that attacks the cells in the body, which is called antinuclear antibodies,” Professor Shim said. “(Antinuclear antibodies) attack individual cells. Our organs are multi-cellular so that they can invade any organ.

So, what are the chances of lupus patients developing problems in other organs?

It has been reported that the chances of each organ being affected are:

ㆍ50 percent for kidneys.

ㆍ10 percent for lungs.

ㆍ10 percent for heart.

ㆍ30-50 percent for pleurisy and pericarditis.

ㆍ20 percent for cranial nerves.

ㆍ90 percent for joints.

“The etymology of lupus comes from a skin disease, but less than half of lupus patients have a skin disease,” Shim said. “The most commonly involved organs are the joints, followed by the kidneys, and inflammation of the lungs or heart occurs in about one-third of patients.”

Because lupus can involve other organs, the most important treatment goal is to prevent future organ damage rather than control current symptoms.

“Just as in hypertension, the goal is to lower blood pressure to prevent the disease from progressing to the point where a heart attack occurs, so in lupus, the goal is to prevent future organ damage rather than controlling outward symptoms,” Professor Shim said.

How should lupus nephritis, which affects half of patients, be treated and managed?

“It's very important to take medication (as prescribed by your doctor) because medication is often protocol-driven,” said Professor Jung Seung-min of the Department of Rheumatology at the Catholic University of Korea St. Vincent's Hospital. “Also, you should avoid eating salty foods, eating too much protein, taking medications you don't need, or eating healthy foods you don't need.”

Professor Shim agreed.

“Limiting protein in the diet is important,” he said. “Not only in lupus but in most kidney diseases. It's even more important in lupus nephritis because a high-protein diet directly worsens kidney filtration in addition to lupus disease. In studies, protein restriction is effective in maintaining kidney function.”

Restricting protein is also important for lupus patients for good reasons.

“There's an additional risk of osteoporosis in lupus, and it's been shown that a higher protein diet than the general population exacerbates osteoporosis,” Professor Shim said. “So we recommend protein restriction in lupus patients for various reasons.”

Shim said that patients with normal kidney function need adequate protein intake for nutrition, adding that not all patients need to restrict all protein.

Many patients do not respond to the initial treatment of lupus nephritis, but this is not a reason to despair.

“The first line of treatment for lupus nephritis is antimalarials and steroids, but about 40 percent of patients don't respond to them,” Professor Shim said. “Recently, a new drug, Benlysta, was approved for active lupus nephritis. In addition, another new drug called Lupkynis was approved, and I think it will soon be available in Korea. These drugs are supposed to be available in the early stages of the disease.”

Besides, patients with lupus nephritis who have certain antibodies are more likely to have adverse kidney events, such as antiphospholipid antibodies.

“In patients with antiphospholipid antibodies, there may be an obstruction in the kidney vessels. If you don't address it further, the nephritis can get worse. Even if you get better with these additional medications, it will relapse if you don't continue the medication for at least three years,” Shim warned.

Are people with lupus nephritis at increased risk for fatal cerebrovascular events such as stroke or myocardial infarction?

Younger patients with lupus nephritis are not uncommonly concerned about cardiovascular disease, as blood pressure usually increases as kidney function declines, and high blood pressure increases the risk of cardiovascular disease.

“In 1950, if you were diagnosed with lupus, the five-year survival rate was less than half,” Shim said. “In 1990, however, the 10-year survival rate was reported to be 91 percent. There is not much difference compared to the general population's 10-year survival rate of 96 percent. In the past, because of poor treatment, most of the deaths were due to kidney damage, but as treatment has improved, the kidneys have become more treatable, so now almost half of the deaths are due to cardiovascular problems.”

In addition to lupus nephritis, there is another group of lupus patients who are at particularly high risk for cardiovascular disease – those with antiphospholipid antibodies.

“There are lupus patients who have anti-phospholipid antibodies, usually about one in four,” Shim said. “Anti-phospholipid antibodies are antibodies against the membrane surrounding the cells, which is an important factor in blocking blood vessels. If you have antiphospholipid antibodies, you have a five- to 10-fold higher risk of cardiovascular disease,” he explains.

For this reason, lupus patients with antiphospholipid antibodies need to take antithrombotic medications and actively prevent hypertension, diabetes, and dyslipidemia (hyperlipidemia).

“Patients with antiphospholipid antibodies should take additional oral medications, such as aspirin or warfarin,” Professor Shim said. “They must use their antiphospholipid antibodies well and do not have conditions that increase their risk of vascular occlusion, such as hypertension, diabetes, or hyperlipidemia,” Shim said. “In addition to vascular occlusions, smoking worsens lupus itself, and the most important drug used in lupus is antimalarials, which are less effective when smoked. The most common source is secondhand smoking, so family members should also be discouraged from smoking.”