Systemic lupus erythematosus (SLE), a complex and heterogeneous autoimmune disease, represents a significant challenge for both diagnosis and treatment. Patients with SLE in Latin America face special problems that should be considered when therapeutic guidelines are developed. The objective of the study is to develop clinical practice guidelines for Latin American patients with lupus. Two independent teams (rheumatologists with experience in lupus management and methodologists) had an initial meeting in Panama City, Panama, in April 2016. They selected a list of questions for the clinical problems most commonly seen in Latin American patients with SLE. These were addressed with the best available evidence and summarised in a standardised format following the Grading of Recommendations Assessment, Development and Evaluation approach. All preliminary findings were discussed in a second face-to-face meeting in Washington, DC, in November 2016. As a result, nine organ/system sections are presented with the main findings; an ‘overarching’ treatment approach was added. Special emphasis was made on regional implementation issues. Best pharmacologic options were examined for musculoskeletal, mucocutaneous, kidney, cardiac, pulmonary, neuropsychiatric, haematological manifestations and the antiphospholipid syndrome. The roles of main therapeutic options (ie, glucocorticoids, antimalarials, immunosuppressant agents, therapeutic plasma exchange, belimumab, rituximab, abatacept, low-dose aspirin and anticoagulants) were summarised in each section. In all cases, benefits and harms, certainty of the evidence, values and preferences, feasibility, acceptability and equity issues were considered to produce a recommendation with special focus on ethnic and socioeconomic aspects. Guidelines for Latin American patients with lupus have been developed and could be used in similar settings.

Medications that suppress immunity (immunosuppressive medications) are also called cytotoxic drugs. They are sometimes referred to as chemotherapy because they are also used to treat cancer, generally in much higher doses than those used to treat lupus. Immunosuppressive medications are used for treating people with more severe manifestations of SLE, such as damage to internal organ(s). Examples of immunosuppressive medications include azathioprine (Imuran), cyclophosphamide (Cytoxan), chlorambucil (Leukeran), cyclosporine (Sandimmune), and the disease-modifying drug methotrexate (Rheumatrex, Trexall). All immunosuppressive medications can seriously depress blood-cell counts and increase risks of infection and bleeding. Immunosuppressive medications may not be taken during pregnancy or conceptionbecause of risk to the fetus. Other side effects are specific for each drug. For examples, methotrexate can cause liver toxicity, while cyclosporine can impair kidney function.


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The symptoms involved in CREST syndrome are associated with the generalized form of the disease Systemic sclerosis (scleroderma). CREST is an acronym for the clinical features that are seen in a patient with this disease. The “C” stands for calcinosis, where calcium deposits form under the skin on the fingers or other areas of the body. The “R”, stands for Raynaud’s phenomenon, spasm of blood vessels in the fingers or toes in response to cold or stress. The “E” represents esophageal dysmotility, which can cause difficulty in swallowing. The “S” is for sclerodactyly, tightening of the skin causing the fingers to bend. Finally, the letter “T” is for telangiectasia, dilated vessels on the skin of the fingers, face, or inside of the mouth.
Two working teams on logistics and methodological issues constituted by experienced Latin American rheumatologists and experts in the Grading of Recommendations Assessment, Development and Evaluation (GRADE) guideline system developed a framework for these guidelines. Nine organ/system sections were prepared with the main findings. Special emphasis was placed on reviewing local problems and regional publications.
In addition to hormonal mechanisms, specific genetic influences found on the X chromosome may also contribute to the development of SLE. Studies indicate that the X chromosome can determine the levels of sex hormones. A study has shown an association between Klinefelter syndrome and SLE. XXY males with SLE have an abnormal X–Y translocation resulting in the partial triplication of the PAR1 gene region.[104]
Therefore, “maintaining good bone health is an area of concern for people with lupus, and a diet rich in calcium and vitamin D can help counteract bone-damaging effects,” Gibofsky explained. These foods might include “milk, light ice cream or frozen yogurt, cottage cheese, pudding, almonds, broccoli, fortified cereal, oranges, yogurt, hard cheese, soybeans and soy milk, navy beans, oysters, sardines, and spinach,” according to experts at Johns Hopkins University School of Medicine.6
Alfalfa seeds and sprouts, green beans, peanuts, soybeans, and snow peas contain a substance that has been shown to trigger lupus flare-ups in some patients (although not all). Negative reactions caused by these foods experienced by lupus patients can include antinuclear antibodies in the blood, muscle pains, fatigue, abnormal immune system function and kidney abnormality. These symptoms are believed to be caused by the amino acid L-canavanine. (7)
Lupus is chronic, complex, and difficult to diagnose. No single lab test can tell if you have lupus. Many lupus symptoms imitate symptoms of other diseases and often come and go. Your primary care doctor or rheumatologist will use your medical history, a physical exam, and many routine as well as special tests to rule out other diseases. Many physicians also use the American College of Rheumatology's "Eleven Criteria of Lupus" to aid in the diagnosis of lupus. The criteria include symptoms as well as specific laboratory findings that provide information about the functioning of a person's immune system. In most cases, the diagnosis of lupus is made when four or more of the criteria have occurred at some time.
Synovitis is an inflammation of the joint lining, called synovium. The symptoms are often of short duration and may change location although when caused by overuse tend to remain in one joint. The pain is usually more severe than expected based on the appearance of the joint on examination. In fact, sometimes there is pain without swelling or even tenderness in the joint, in which case the symptom is called “arthralgias” (literally meaning “joint pain” in Greek). Although synovitis has many different causes, the most common cause in an active healthy person is overuse.

The removal of plasma from a patient (usually to treat an immmunologically mediated illness such as thrombotic thrombocytopenic purpura or myasthenia gravis) and its replacement with normal plasma. Plasma exchange therapy can also be used to replace excessively viscous plasma in patients with Waldenström macroglobulinemia. Pathological antibodies, immune complexes, and protein-bound toxins are removed from the plasma by plasma exchange. Immunoglobulin infusions are an alternative to plasma exchange when treating some immunological illnesses, including Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy.
Mortality rates for systemic lupus erythematosus are particularly high in children. In a retrospective study26 of Brazilian children, overall mortality during 16 years of follow-up was 24 percent. Death occurred because of infection (58 percent), central nervous system disease (36 percent), and renal disease (7 percent). When disease onset was before the age of 15 years, renal involvement and hypertension predicted mortality.
Lupus in children tends to be more aggressive than in adults, says Dr. Pascual. The exact reasons for this are not understood. One theory is that people are born with genetic susceptibility to the disease that may be triggered by environmental factors such as a virus. “Children with the condition may have inherited a more complex set of predisposing genes,” she says. But this theory has yet to be proved.

The symptoms involved in CREST syndrome are associated with the generalized form of the disease Systemic sclerosis (scleroderma). CREST is an acronym for the clinical features that are seen in a patient with this disease. The “C” stands for calcinosis, where calcium deposits form under the skin on the fingers or other areas of the body. The “R”, stands for Raynaud’s phenomenon, spasm of blood vessels in the fingers or toes in response to cold or stress. The “E” represents esophageal dysmotility, which can cause difficulty in swallowing. The “S” is for sclerodactyly, tightening of the skin causing the fingers to bend. Finally, the letter “T” is for telangiectasia, dilated vessels on the skin of the fingers, face, or inside of the mouth.


SLE is regarded as a prototype disease due to the significant overlap in its symptoms with other autoimmune diseases.[49] This means that it is an important area of continued research and study that is utilizing diverse techniques such as GWAS, microarrays, and murine studies.[50] Further genetic studies of multiple ethnic groups and the creation of disease models incorporating environmental influences will help to increase and refine the understanding of specific genes, linkages, as well as the mechanisms underlying the disease.[51]
SLE is undoubtedly a potentially serious illness with involvement of numerous organ systems. However, it is important to recognize that most people with SLE lead full, active, and healthy lives. Periodic increases in disease activity (flares) can usually be managed by varying medications. Since ultraviolet light can precipitate and worsen flares, people with systemic lupus should avoid sun exposure. Sunscreens and clothing covering the extremities can be helpful. Abruptly stopping medications, especially corticosteroids, can also cause flares and should be avoided. People with SLE are at increased risk of infections as SLE-related complications, especially if they are taking corticosteroids or immunosuppressive medications. Therefore, any unexpected fever should be reported to medical professionals and evaluated.
Therefore, “maintaining good bone health is an area of concern for people with lupus, and a diet rich in calcium and vitamin D can help counteract bone-damaging effects,” Gibofsky explained. These foods might include “milk, light ice cream or frozen yogurt, cottage cheese, pudding, almonds, broccoli, fortified cereal, oranges, yogurt, hard cheese, soybeans and soy milk, navy beans, oysters, sardines, and spinach,” according to experts at Johns Hopkins University School of Medicine.6
Two working teams on logistics and methodological issues constituted by experienced Latin American rheumatologists and experts in the Grading of Recommendations Assessment, Development and Evaluation (GRADE) guideline system developed a framework for these guidelines. Nine organ/system sections were prepared with the main findings. Special emphasis was placed on reviewing local problems and regional publications.
There is a wide range of diets advertised to help you lose weight quickly or control various chronic diseases, such as lupus. Many people claim to be experts in nutrition yet have limited knowledge and offer no protection to the public. You should be wary of unqualified practitioners who may be offering unproven techniques to diagnose and treat nutritional problems.
The most commonly sought medical attention is for joint pain, with the small joints of the hand and wrist usually affected, although all joints are at risk. More than 90 percent of those affected will experience joint or muscle pain at some time during the course of their illness.[16] Unlike rheumatoid arthritis, lupus arthritis is less disabling and usually does not cause severe destruction of the joints. Fewer than ten percent of people with lupus arthritis will develop deformities of the hands and feet.[16] People with SLE are at particular risk of developing osteoarticular tuberculosis.[17]
As with all autoimmune conditions, lupus is a disease of the immune system. Your immune system has a very sophisticated mechanism for keeping you safe that it uses to identify the foreign substances that you come into contact with every day, such as allergens, toxins, infections, and even food. If your immune system deems anything dangerous, it will produce antibodies to ward off the harmful intruders.
Immunosuppressive agents/chemotherapy. In advanced cases of lupus, drugs like azathioprine, methotrexate and cyclophosphamide might be used to suppress the immune system. These types of therapies can help prevent organ damage; however, they do cause severe side effects as well as infertility in women. People on immunosuppressive therapies must be closely monitored by a doctor.
To ensure that the person has lupus and not another autoimmune disease, the American College of Rheumatology (ACR) established a list of clinical and immunologic criteria that, in any combination, point to SLE. The criteria include symptoms that the person can identify (e.g. pain) and things that a physician can detect in a physical examination and through laboratory test results. The list was originally compiled in 1971, initially revised in 1982, and further revised and improved in 2009.[120]
Pain is typically treated with opioids, varying in potency based on the severity of symptoms. When opioids are used for prolonged periods, drug tolerance, chemical dependency, and addiction may occur. Opiate addiction is not typically a concern since the condition is not likely to ever completely disappear. Thus, lifelong treatment with opioids is fairly common for chronic pain symptoms, accompanied by periodic titration that is typical of any long-term opioid regimen.
Describes a clinical study in which groups of participants receive one of several combinations of interventions. For example, a two-by-two factorial design involves four groups of participants. Each group receives one of the following pairs of interventions: 1) drug A and drug B, 2) drug A and a placebo, 3) a placebo and drug B, or 4) a placebo and a placebo. So during the trial, all possible combinations of the two drugs (A and B) and placebos are given to different groups of participants.
Moderate use of alcohol is usually not a problem for people with lupus, but alcohol can lower the effectiveness of some medications, cause new health problems, and/or can make existing problems worse. For example, non-steroidal anti-inflammatory drugs -- such as aspirin, ibuprofen (Motrin®), naproxen (Naprosyn®), and celecoxib (Celebrex®) -- can cause ulcers and bleeding in the stomach and intestines at any time during treatment; the chance of developing an ulcer or internal bleeding increases with alcohol use. Also, anticoagulant medicines such as warfarin (Coumadin®) and the chemotherapy drug, methotrexate, may not be as effective if you are drinking alcohol.
Corticosteroids and immune suppressants: Patients with serious or life-threatening problems such as kidney inflammation, lung or heart involvement, and central nervous system symptoms need more “aggressive” (stronger) treatment. This may include high-dose corticosteroids such as prednisone (Deltasone and others) and drugs that suppress the immune system. Immune suppressants include azathioprine (Imuran), cyclophosphamide (Cytoxan), and cyclosporine (Neoral, Sandimmune). Recently mycophenolate mofetil has been used to treat severe kidney disease in lupus – referred to as lupus nephritis.
Normally, our immune system produces proteins called antibodies that protect the body from these foreign invaders. When you have lupus, your immune system cannot tell the difference between these foreign invaders and your body’s healthy tissues, so autoantibodies are made that damage and destroy healthy tissue (auto means self and anti means against, so autoantibody means against self). These autoantibodies cause inflammation, pain, and damage in various parts of the body.
The body’s tolerance of the antigens present on its own cells, i.e., autoantigens or self-antigens. It is theorized that autoreactive T lymphocytes are destroyed in the thymus by negative selection or in peripheral blood. Autoreactive T cells that escape destruction in the thymus may become tolerant because they are exposed to thousands of autoantigens as they circulate in the blood.
The history of SLE can be divided into three periods: classical, neoclassical, and modern. In each period, research and documentation advanced the understanding and diagnosis of SLE, leading to its classification as an autoimmune disease in 1851, and to the various diagnostic options and treatments now available to people with SLE. The advances made by medical science in the diagnosis and treatment of SLE have dramatically improved the life expectancy of a person diagnosed with SLE.[105]
If you have lupus, you may experience dry mouth. Your eyes may feel gritty and dry, too. That’s because some people with lupus develop Sjogren’s disease, another autoimmune disorder. Sjogren’s causes the glands responsible for tears and saliva to malfunction, and lymphocytes can accumulate in the glands. In some cases, women with lupus and Sjogren’s may also experience dryness of the vagina and skin.
Lupus antibodies can be transferred from the mother to the fetus and result in lupus illness in the newborn ("neonatal lupus"). This includes the development of low red cell counts (hemolytic anemia) and/or white blood cell counts (leucopenia) and platelet counts (thrombocytopenia) and skin rash. Problems can also develop in the electrical system of the baby's heart (congenital heart block). Occasionally, a pacemaker for the baby's heart is needed in this setting. Neonatal lupus and congenital heart block are more common in newborns of mothers with SLE who carry specific antibodies referred to as anti-Ro (or anti-SSA) and anti-La (or anti-SSB). (It is helpful for the newborn baby's doctor to be made aware if the mother is known to carry these antibodies, even prior to delivery. The risk of heart block is 2%; the risk of neonatal lupus is 5%.) Neonatal lupus usually clears after 6 months of age, as the mother's antibodies are slowly metabolized by the baby.

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Take a good multivitamin/multimineral supplement with recommended dosages of antioxidants. To help address inflammation, increase intake of omega-3 fatty acids by eating sardines or other oily fish (salmon, herring, mackerel) three times a week or supplementing with fish oil. Freshly ground flaxseeds (grind two tablespoons a day and sprinkle over cereals or salads) can also help decrease inflammation. Other dietary strategies include avoiding polyunsaturated vegetable oils (safflower, sunflower, corn, etc.), margarine, vegetable shortening, and all products made with partially hydrogenated oils. Eat a low-protein, plant-based diet that excludes all products made from cows’ milk, be sure to eat plenty of fresh fruits and vegetables (with the exception of alfalfa sprouts, which contain the amino acid L-canavanine that can worsen autoimmunity.)
Any of the plasma proteins whose concentration increases or decreases by at least 25% during inflammation. Acute-phase proteins include C-reactive protein, several complement and coagulation factors, transport proteins, amyloid, and antiprotease enzymes. They help mediate both positive and negative effects of acute and chronic inflammation, including chemotaxis, phagocytosis, protection against oxygen radicals, and tissue repair. In clinical medicine the erythrocyte sedimentation rate or serum C-reactive protein level sometimes is used as a marker of increased amounts of acute-phase proteins.

Systemic lupus erythematosus (SLE), commonly known as "lupus," is an autoimmune illness. The immune system, which normally protects the body from foreign invaders and infection, malfunctions and instead attacks a person's own healthy body tissues. Its cause is unknown, but most scientists believe that genetics, combined with outside triggers (such as infections, medications or other environmental factors) lead people to develop lupus. Lupus is a lifelong condition, but symptoms tend to cycle in alternate periods of "flares" (or "flares-ups") and remissions. Lupus affects women much more than men. There is no known cure, but numerous treatments are available.
At least half of people with lupus experience fatigue. (4) Fatigue may be brought on by the disease itself or from associated depression, anxiety, lack of exercise, and problems with sleep. ( 5) Because people with lupus need to avoid sun exposure, they may have low levels of vitamin D, which can contribute to fatigue. Lupus treatments may also play a role.
Lupus can cause problems with the blood, too, including anemia, or low red blood cell count. Anemia can cause symptoms such as weakness and fatigue. (14) Thrombocytopenia is another blood disorder that may develop, resulting in lower platelet counts. (Platelets are the blood cells that help the blood clot.) Symptoms of thrombocytopenia can include bruising easily, nosebleeds, and petechiae, when the blood appears as red pinpoints under the skin. (15)
The monoclonal antibody belimumab (Benlysta), a B-lymphocyte stimulator–specific inhibitor, has been found to reduce disease activity and possibly decrease the number of severe flares and steroid use in patients with SLE when used in combination with standard therapy. [114] In March, 2011, the US Food and Drug Administration (FDA) approved the use of belimumab in combination with standard therapies (including steroids, nonbiologic DMARDS [eg, hydroxychloroquine, azathioprine, methotrexate]) to treat active autoantibody-positive SLE. [115]  In July 2017, a subcutaneous (SC) formulation was approved that allows patients to self-administer a once-weekly dose. [162]
Of note, problems faced by Latin American countries are shared by several developing nations. Therefore, it is expected that these guidelines will also be very useful for them. Furthermore, due to ever increasing globalisation and the increase of migratory movements of people from countries with more susceptible SLE groups in terms of frequency and disease severity both in terms of race/ethnicity (Mestizos, Asians, Africans) and low SES to countries with better life opportunities, we consider that these guidelines may be used by physicians anywhere in the world, even in developed countries, where such individuals may migrate to and seek care for their lupus.
Cardiac tamponade is pressure on the heart that occurs when blood or fluid builds up in the space between the heart muscle (myocardium) and the outer covering sac of the heart (pericardium). This prevents the heart ventricles from expanding fully. The excess pressure from the fluid prevents the heart from working properly. As a result, the body does not get enough blood.
Corticosteroids and immune suppressants: Patients with serious or life-threatening problems such as kidney inflammation, lung or heart involvement, and central nervous system symptoms need more “aggressive” (stronger) treatment. This may include high-dose corticosteroids such as prednisone (Deltasone and others) and drugs that suppress the immune system. Immune suppressants include azathioprine (Imuran), cyclophosphamide (Cytoxan), and cyclosporine (Neoral, Sandimmune). Recently mycophenolate mofetil has been used to treat severe kidney disease in lupus – referred to as lupus nephritis.

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