Systemic lupus erythematosus (SLE) is a multisystem, autoimmune disease in which the immune system attacks one’s own tissues and organs1. Lupus can vary in severity from mild disease, requiring little treatment, to severe cases, which can be life threatening. It has many different possible manifestations because different systems of the body may be involved. There are several different types of lupus, some of which are limited to skin involvement, but the systemic form is the more severe and well-known form.
What are the symptoms of lupus?
The most common symptoms experienced by lupus sufferers are fatigue, joint pain, skin rashes and alopecia (hair loss). Other frequent symptoms include fevers, generalized weakness, oral ulcers, and Raynaud’s phenomenon. Since lupus may affect many body systems, additional symptoms depend upon the region affected. For example, there may be nervous system involvement, which may cause burning and tingling of peripheral nerves, loss of sensation or strength in a limb, headaches, or even confusion, seizures and psychiatric problems in some cases. If the blood system is affected, anemia or clotting problems arise, leading to easy bruising and bleeding. One of the most important and worrisome problems for lupus patients is the potential for kidney involvement. “Lupus nephritis “may cause severe leg swelling and can sometimes lead to kidney failure and the need for hemodialysis or a kidney transplant2.
In what ways can a person contract lupus?
SLE is not contagious, nor is it an infection. However, the actual cause of lupus is unknown. There is clearly a genetic component. It is estimated that about 25% of the risk for developing lupus is due to hereditary factors, while unknown environmental events seem to trigger disease development. Certain environmental factors are known to influence or aggravate lupus, such as exposure to UV ( sun) light or silica dust. Hormonal factors may also be important. Some medications can also cause “drug-induced” lupus.3
How Common is lupus?
Fortunately, SLE is not common. In the US, the prevalence of lupus is about 100 cases per 100,000 population. African-Americans are affected about 3 times more often than Caucasians. Women are stricken about 8 times more frequently than men, which is one reason that hormones are believed to play an important role in the disease4.
Lupus also may afflict young persons. 65% of patients with SLE are between age 16 and 55 and childhood lupus is a serious condition. Most often, women are diagnosed with SLE in their late 30’s and early 40’s.
Is lupus a life-threatening disease?
It can be. Lupus manifests itself with great variety. Some people have a relatively mild and benign illness with a good long-term prognosis. Others have severe disease, involving multiple organ systems which may be progressive and fulminant, in which organ failure and death may occur. Overall, it is estimated that lupus patients have 2 to 5 times greater mortality than unaffected persons. Despite a 90% 5-year survival rate, SLE ranks in the top 20 causes of death for women under age 64.
What are the different types of lupus?
There are 2 main types of lupus: systemic lupus erythematosus ( SLE) and cutaneous lupus. Cutaneous lupus, of which there are several subtypes (i.e. discoid lupus and subacute cutaneous lupus) is a skin disease without systemic manifestations. There are several types of rashes seen in lupus patients. The most common is the “malar rash” which is a mask-like butterfly rash across the face and nose. Discoid lupus rash is a potentially deep and scarring plaque-like rash which may occur anywhere on the body. Exposure to ultraviolet light can trigger emergence of a lupus rash, which is often found on sun exposed regions of the body. In order to distinguish a lupus rash from other form of dermatitis, a skin biopsy is often required. Skin involvement in lupus is common and many patients with systemic disease-ie, SLE, will have skin involvement, but also will manifest a variety of organ system disease, as well.
Another important type of SLE is drug-induced lupus. Several medications that are known to cause a lupus-like syndrome. Procainamide, hydralazine, minocycline, and some types of sulfa medications are among known causes. In these cases, discontinuation of the medication leads to resolution of symptoms.
What is the best method to diagnose lupus?
Diagnosing lupus can sometimes be difficult, in part because it has so many different potential manifestations and each SLE patient is unique. Consequently, the diagnosis is made on the basis of the presence of a number of different clinical and laboratory criteria. A thorough history and physical examination, followed by blood and urine testing, and sometimes tissue biopsy is required. Rheumatologists, specialists in arthritic and autoimmune disorders are usually consulted in order to establish the diagnosis. The presence of a number of signs and symptoms, such as rash, constitutional symptoms ( fatigue malaise, fevers, aches and pains), arthritis, swollen glands, chest or abdominal pains, difficulty breathing, neurologic symptoms are just a few of the clinical features to consider. The presence of several of these symptoms, without other explanation, combined with certain laboratory findings can establish the diagnosis.
Laboratory testing is very important. The ANA test (anti-nuclear antibody) is positive in 95% of SLE patients, but because it may be positive in other conditions, its presence alone does not confirm a diagnosis of lupus. Autoantibodies are identified in the blood of patients with a variety of autoimmune diseases. An analysis of the presence and pattern of autoantibodies can be very useful in establishing a diagnosis. In SLE, there are several autoantibodies that are specific for lupus, meaning that if present, the diagnosis of lupus is probable. Examples of these are antibodies to double stranded DNA and the Sm antibody. Since they are not present in all lupus patients, however, their absence does not rule out SLE.
Additional laboratory studies are important to assess for the presence of anemia, kidney or liver problems. The sedimentation (“sed rate”) and C-reactive protein indicates systemic inflammation. These are just some of the tests that are important are part of an overall assessment
Can drinking alcohol worsen lupus?
Generally no, although alcohol should always be consumed in moderation for a variety of health reasons! Some of the medications used in lupus patients may interact adversely with alcohol, so this remains an important consideration.
Can lupus lead to weight gain?
Some patients with SLE will develop a great deal of fluid accumulation and edema and as a result, weight gain. This is commonly seen lupus nephritis. Otherwise, many patients with SLE actually lose weight, since they feel poorly, often lose appetite and may lose weight and muscle mass.
On the other hand, prednisone and other corticosteroid drugs are often used to treat SLE and these medications are notorious for causing weight gain, among other adverse effects. SO, in fact, many treated SLE patients will experience weight gain due to their treatment.
Do symptoms of lupus worsen with age?
Not necessarily. Actually, patients who first develop SLE at an older age tend to have a milder course than younger individuals.
Lupus is a chronic illness with fluctuations in disease severity and manifestations over time. It is common to experience “flare-ups” which require interventions and treatment modifications, but these can often be managed and subdued. If the condition can be controlled with good personal care and close follow up and monitoring along with one’s rheumatologist, things need not worsen. It is critical that SLE patients are regularly followed up with examinations and laboratory testing.
Why is lupus more common in women than men?
It has been postulated that the reason for this has to do with sex hormone differences between men and women. Some studies have suggested that patients on birth control pills may have a higher risk and, in some patients, estrogen containing medications do seem to aggravate the disease. However, other studies have not found this to be the case, so the matter is more complicated. Estrogen and progesterone do have effects on immune cell function, so it is likely that the hormone story is important in lupus. Other theories suggest that the female preponderance is related to genetic factors on the X chromosome. So, it is really not understood why women have so much greater risk.5
What dietary changes would you recommend for a person suffering from lupus?
There is no single nutritional approach that has been shown to impact SLE. That said, all patients with chronic inflammatory diseases should consider their diet, as a number of foods have been shown to be inflammatory. A Mediterranean diet, rich in fruits and vegetables, fish and olive oil has been shown to help patients with inflammatory arthritis. Avoidance of added sugars, high fructose corn syrups and processed foods is important, as these act as inflammatory triggers in most people. In some people gluten can be aggravating, while in others it may be dairy, corn, soy or legumes. It can be a challenge to determine which foods an individual may find inflammatory, since there is no test to make an accurate determination. I typically recommend a whole foods diet, and advise patients to compose a food diary to help them reflect on which foods may result in disease flares. It is often worth a trial without gluten and dairy products , since these seem to be frequent culprits in impacting inflammation.
Can lupus increase your chances of developing cancer?
Yes. There appears to be an increased risk of malignancies in patients with chronic inflammatory and autoimmune diseases. Moreover, some of the medications used to treat SLE, such as azathioprine and cyclophosphamide are know to increase the risk of cancer.6
Does lupus increase the chances of infertility?
Yes. In women, premature ovarian failure may accompany lupus. More commonly, the stress related to the illness contributes to anovulatory cycles and difficulty with conception. However, many women with lupus are able to become pregnant without much difficulty. In fact, a frequent concern is the best means for birth control for the lupus patient.
The reason for this is that in the lupus patient, pregnancy is a high-risk matter. Many of the drugs used to treat lupus are contraindicated in pregnancy and should be discontinued prior to a planned pregnancy. An unexpected pregnancy can be traumatic in the patent with active disease or on potent medications. Some women with lupus experience significant disease flares during pregnancy. Pre-eclampsia is more common in SLE patients and the rate of miscarriage is substantially higher than in healthy woman.
This does not mean that a woman with SLE who desires a child cannot experience a successful pregnancy. In fact, many women with lupus are happy mothers! But in the lupus patient, a pregnancy should be planned well in advance, the illness well-controlled and an experienced high-risk obstetrician involved in the prenatal care. 7
What are the best treatment options for lupus?
In mild cases, lupus can be treated with anti-inflammatory medications such a ibuprofen (Advil, Motrin) or naproxen(Aleve, Naprosyn) and topical cortisone creams if rashes are present. In more moderate cases, anti-malarial medications such as hydroxychloroquine (Plaquenil) may be used. This is quite effective and generally safe and well tolerated in most patients, although rarely, eye toxicity can occur and all patients on this medication should be monitored regularly by an eye doctor.
Corticosteroids, such as prednisone are often added next and can be very effective in controlling disease. But they come at a cost, since there are many side effects associated with their use, including weight gain, osteoporosis, diabetes, cataracts, to name just a few. Many lupus patients need prednisone in order to control their condition, though, so we try to keep the dose as low as possible.
In severe cases, or when high doses of prednisone are needed to control disease, potent medications, such as immune-suppressants are required. The treatment depends upon the systems involved and their severity and very much depends upon the individual needs of the patient. Many patients require multiple medications for disease control. Examples of medications used include azathioprine (Imuran), methotrexate, mycophenolate mofetil ( CellCept), belimumab (Benlysta), and cyclophosphamide (Cytoxan). Some patients with SLE have blood coagulation problems and are at risk for blood clots, pulmonary embolism, and stroke. They typically require blood thinners. Patients with lupus nephritis often must be treated very aggressively to prevent kidney failure, while others require treatment guided largely by symptoms.8
Please share some important points to remember when dealing with lupus.
Lupus can be a very serious condition and frightening for patients and their families. It is most important to learn as much about the condition as possible and establish a good relationship with a physician familiar with SLE, usually a rheumatologist. Emotional, social and family support is most important. Personal care and lifestyle modifications are critical: proper rest, stress management, regular exercise and good nutrition are essential. It is important to take advantage of resources, such as the Lupus Foundation and the Arthritis Foundation, which provide information for patients, referrals, and support groups.
References:
- Hormonal, environmental, and infectious risk factors for developing systemic lupus erythematosus.
Cooper GS, Dooley MA, Treadwell EL, St Clair EW, Parks CG, Gilkeson GS
Arthritis Rheum. 1998;41(10):1714.
- Prevalence of adult systemic lupus erythematosus in California and Pennsylvania in 2000: estimates obtained using hospitalization data.AU
Chakravarty EF, Bush TM, Manzi S, Clarke AE, Ward MM
Arthritis Rheum. 2007;56(6):2092.
5.
Reproductive and menopausal factors and risk of systemic lupus erythematosus in women.
Costenbader KH, Feskanich D, Stampfer MJ, Karlson EW
Arthritis Rheum. 2007;56(4):1251.
6.
The association of malignancy with rheumatic and connective tissue diseases.
Carsons S Semin Oncol. 1997;24(3):360.
7.
EULAR recommendations for women’s health and the management of family planning, assisted reproduction, pregnancy and menopause in patients with systemic lupus erythematosus and/or antiphospholipid syndrome.
Ann Rheum Dis. 2017;76(3):476.
8.
2019 update of the EULAR recommendations for the management of systemic lupus erythematosus.
Ann Rheum Dis. 2019;78(6):736. Epub 2019 Mar 29.
