It also recommends intravenous Ig with/without GCs or RTX plus GCs for patients who are refractory to high-dose GCs, those with life-threatening bleeding, those requiring urgent surgery and those with infections (strong recommendation based on moderate certainty of the evidence). Cost and availability, however, may prompt the use of IS instead of RTX although there are no data to support this assertion (table 4).
Common initial and chronic complaints include fever, malaise, joint pains, muscle pains, and fatigue. Because these symptoms are so often seen in association with other diseases, these signs and symptoms are not part of the diagnostic criteria for SLE. When occurring in conjunction with other signs and symptoms (see below), however, they are considered suggestive.[11]
It also is known that some women with systemic lupus erythematosus can experience worsening of their symptoms prior to their menstrual periods. This phenomenon, together with the female predominance of systemic lupus erythematosus, suggests that female hormones play an important role in the expression of SLE. This hormonal relationship is an active area of ongoing study by scientists.
Another targeted treatment, anifrolumab, is being investigated in clinical trials and appears to be promising, says Stacy Ardoin, MD, a rheumatologist at the Ohio State University Wexner Medical Center in Columbus, citing a study in the February 2017 issue of Arthritis & Rheumatology (7). “I don’t think it will work for everyone, but it’s good to have another treatment option.”

Inflammation of the pleurae known as pleurisy can rarely give rise to shrinking lung syndrome.[25] SLE can cause pleuritic pain and also give rise to shrinking lung syndrome, involving a reduced lung volume.[26] Other associated lung conditions include pneumonitis, chronic diffuse interstitial lung disease, pulmonary hypertension, pulmonary emboli, and pulmonary hemorrhage.
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]
Immunosuppressive Medications Immunosuppressives are medications that help suppress the immune system. Many were originally used in patients who received organ transplants to help prevent their bodies from rejecting the transplanted organ. However, these drugs are now also used for the treatment of certain autoimmune diseases, such as lupus and rheumatoid arthritis.
Hydroxychloroquine (Plaquenil) is an antimalarial medication found to be particularly effective for SLE people with fatigue, skin involvement, and joint disease. Consistently taking Plaquenil can prevent flare-ups of lupus. Side effects are uncommon but include diarrhea, upset stomach, and eye-pigment changes. Eye-pigment changes are rare but require monitoring by an ophthalmologist (eye specialist) during treatment with Plaquenil. Researchers have found that Plaquenil significantly decreased the frequency of abnormal blood clots in people with systemic lupus. Moreover, the effect seemed independent of immune suppression, implying that Plaquenil can directly act to prevent the blood clots. This fascinating study highlights an important reason for people and doctors to consider Plaquenil for long-term use, especially for those SLE people who are at some risk for blood clots in veins and arteries, such as those with phospholipid antibodies (cardiolipin antibodies, lupus anticoagulant, and false-positive venereal disease research laboratory test). This means not only that Plaquenil reduces the chance for re-flares of SLE, but it can also be beneficial in thinning the blood to prevent abnormal excessive blood clotting. Plaquenil is commonly used in combination with other treatments for lupus.
Approval for SC belimumab was based on the BLISS-SC phase III study (n=839), which documented reduction in disease activity at week 52 in patients receiving belimumab plus standard of care, compared with those receiving placebo plus standard of care. SRI response with belimumab versus placebo was 61.4% vs 48.4%, respectively (P = 0.0006). In the belimumab group, both time to and risk of severe flare were improved (median 171 days vs 118 days; P = 0.0004), and more patients were able to reduce their corticosteroid dosage by ≥25% (to ≤7.5 mg/day) during weeks 40-52 (18.2% vs 11.9%; P = 0.0732), compared with placebo. [163]
Belimumab, a type of agent referred to as a B-lymphocyte stimulator (BLyS) protein inhibitor, was approved by the U.S. Food and Drug Administration (FDA) in March 2011 for patients with lupus who are receiving other standard therapies, including those listed above. Given by IV infusion, belimumab may reduce the number of abnormal B cells thought to be a problem in lupus.
Sjogren’s syndrome is a disease that causes dryness in your mouth and eyes. It can also lead to dryness in other places that need moisture, such as your nose, throat and skin. Most people who get Sjogren’s syndrome are older than 40. Nine of 10 are women. Sjogren’s syndrome is sometimes linked to rheumatic problems such as rheumatoid arthritis. In Sjogren’s syndrome, your immune system attacks the glands that make tears and saliva. It may also affect your joints, lungs, kidneys, blood vessels, digestive organs and nerves. The main symptoms are:
Heart and Lungs. Heart and lung involvement often is caused by inflammation of the covering of the heart (pericardium) and lungs (pleura). When these structures become inflamed, patients may develop chest pain, irregular heartbeat, and accumulation of fluid around the lungs (pleuritis or pleurisy) and heart (pericarditis). The heart valves and the lung itself can also be affected by lupus, resulting in shortness of breath.
While SLE can occur in both males and females, it is found far more often in women, and the symptoms associated with each sex are different.[5] Females tend to have a greater number of relapses, a low white blood cell count, more arthritis, Raynaud's phenomenon, and psychiatric symptoms. Males tend to have more seizures, kidney disease, serositis (inflammation of tissues lining the lungs and heart), skin problems, and peripheral neuropathy.[12]
Genetic factors increase the tendency of developing autoimmune diseases, and autoimmune diseases such as lupus, rheumatoid arthritis, and autoimmune thyroid disorders are more common among relatives of people with lupus than the general population. Moreover, it is possible to have more than one autoimmune disease in the same individual. Therefore, "overlap" syndromes of lupus and rheumatoid arthritis, or lupus and scleroderma, etc., can occur.
*All images unless otherwise noted are property of and were created by Kaleidoscope Fighting Lupus. To use one of these images, please contact us at info@kflupus.org for written permission; image credit and link-back must be given to Kaleidoscope Fighting Lupus. **All resources provided by us are for informational purposes only and should be used as a guide or for supplemental information, not to replace the advice of a medical professional. The personal views do not necessarily encompass the views of the organization, but the information has been vetted as a relevant resource. We encourage you to be your strongest advocate and always contact your medical provider with any specific questions or concerns.    
A genetic disorder is a disease caused in whole or in part by a change in the DNA sequence away from the normal sequence. Genetic disorders can be caused by a mutation in one gene (monogenic disorder), by mutations in multiple genes (multifactorial inheritance disorder), by a combination of gene mutations and environmental factors, or by damage to chromosomes (changes in the number or structure of entire chromosomes, the structures that carry genes).
Testing for antibody to double-stranded DNA antigen (anti-dsDNA) and antibody to Sm nuclear antigen (anti-Sm) may be helpful in patients who have a positive ANA test but do not meet full criteria for the diagnosis of systemic lupus erythematosus. AntidsDNA and anti-Sm, particularly in high titers, have high specificity for systemic lupus erythematosus, although their sensitivity is low. Therefore, a positive result helps to establish the diagnosis of the disease, but a negative result does not rule it out.46 The CAP guideline recommends against testing for other autoantibodies in ANA-positive patients, because there is little evidence that these tests are of benefit.46
For reasons that doctors still don't understand, the immune system sometimes becomes confused, attacking the body itself, a condition known as autoimmune disease. With lupus,the immune system can attack any organ of the body, including the kidneys and brain, although skin and joints are often most affected, he said. Like many autoimmune diseases, it's more common in women.
The Food and Drug Administration is responsible for protecting the public health by ensuring the safety, efficacy, and security of human and veterinary drugs, biological products, and medical devices; and by ensuring the safety of our nation’s food supply, cosmetics, and products that emit radiation. FDA also has responsibility for regulating the manufacturing, marketing, and distribution of tobacco products to protect the public health and to reduce tobacco use by minors.
The complement system is the name of a group of blood proteins that help fight infection. Complement levels, as the name implies, measure the amount and/or activity of those proteins. Working within the immune system, the proteins also play a role in the development of inflammation. In some forms of lupus, complement proteins are consumed (used up) by the autoimmune response. A decrease in complement levels can point toward lupus nephritis, lupus nephritis, kidney inflammation. Normalization of complement levels can indicate a favorable response to treatment.
Osteoarthritis is the most common form of arthritis, affecting millions of people around the world. Often called wear-and-tear arthritis, osteoarthritis occurs when the protective cartilage on the ends of your bones wears down over time. While osteoarthritis can damage any joint in your body, the disorder most commonly affects joints in your hands, neck, lower back, knees and hips. Osteoarthritis gradually worsens with time, and no cure exists. But osteoarthritis treatments can slow the progression of the disease, relieve pain and improve joint function.

Recommendations are applicable to patients showing partial or total remission after induction therapy aiming at sustaining renal remission, preventing relapses and achieving the best long-term outcome. The following interventions were considered: (1) AZA; (2) MMF; (3) CYC; (4) TAC; and (5) CsA (online supplementary tables S1.1.1.7, S1.1.2.1, S1.1.2.2, S1.2.1, S1.2.3, S1.2.4, S1.2.5, S1.2.6, S1.2.7).
Neutrophils, 55% to 70% of all leukocytes, are the most numerous phagocytic cells and are a primary effector cell in inflammation. Eosinophils, 1% to 3% of total leukocytes, destroy parasites and are involved in allergic reactions. Basophils, less than 1% of all leukocytes, contain granules of histamine and heparin and are part of the inflammatory response to injury. Monocytes, 3% to 8% of all leukocytes, become macrophages and phagocytize pathogens and damaged cells, esp. in the tissue fluid. Lymphocytes, 20% to 35% of all leukocytes, have several functions: recognizing foreign antigens, producing antibodies, suppressing the immune response to prevent excess tissue damage, and becoming memory cells.
I recommend having your MTHFR genes tested, as this genetic mutation can impact how you detoxify mercury and other heavy metals. There are multiple options for heavy metal testing, however I’ve found the DMPS “challenge” test to be the gold standard. To assess whether someone is being exposed to mycotoxins, I use the test from Real Time Lab. If either heavy metals or mycotoxins are an issue for you, work on minimizing your exposure to these toxins, and be sure to support your detox pathways while you work to flush them out.

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Blood clots are seen with increased frequency in lupus. Clots often occur in the legs (a vein clot, called deep venous thrombosis), lungs (a lung clot, called pulmonary embolus), or brain (stroke). Blood clots that develop in lupus patients may be associated with the production of antiphospholipid antibodies. These antibodies are abnormal proteins that may increase the tendency of the blood to clot.
Recommendations are applicable to patients showing partial or total remission after induction therapy aiming at sustaining renal remission, preventing relapses and achieving the best long-term outcome. The following interventions were considered: (1) AZA; (2) MMF; (3) CYC; (4) TAC; and (5) CsA (online supplementary tables S1.1.1.7, S1.1.2.1, S1.1.2.2, S1.2.1, S1.2.3, S1.2.4, S1.2.5, S1.2.6, S1.2.7).
The severity of lupus varies from mild to life threatening. Kidney problems and neurologic complications are more dangerous than the rashes, arthritis or other symptoms. After many years of having lupus, patients may develop hypertension (high blood pressure), accelerated atherosclerosis (plaque and fat build-up in the arteries), heart and lung diseases, kidney failure or osteoporosis. With proper treatment, the majority of people diagnosed with lupus have a normal life expectancy, but many will experience disabilities. Each patient will likely have his or her own specific pattern of symptoms and flares, but the disease can change over time.
Symptoms vary but can include fatigue, joint pain, a red rash on the face (also called the "butterfly rash") and fever. These symptoms can periodically get worse (flare-up) and then improve.  Lupus flares can range from mild to severe, often resulting in periods in which the disease is relatively quiescent. Currently, no cures exist for lupus, and treatment often involves corticosteroids, other immunosuppressants or organ transplants. But research is providing hope for better diagnosis, treatments and even cures.
Inflammation of the lining of the lungs (pleuritis) with pain aggravated by deep breathing (pleurisy) and of the heart (pericarditis) can cause sharp chest pain. The chest pain is aggravated by coughing, deep breathing, and certain changes in body position. The heart muscle itself rarely can become inflamed (carditis). It has also been shown that young women with SLE have a significantly increased risk of heart attacks due to coronary artery disease.

Ms. Everett then discussed some important general nutrition guidelines of which individuals with lupus should be aware. Some key guidelines include diets low in fat, cholesterol, and sodium; low in refined sugars like soda and concentrated juices; and high in fiber. It is important to be aware of high protein diets which can often stress the kidneys. Most importantly, Ms. Everett stresses the importance of keeping a well-balanced diet.
Your primary care doctor should coordinate care between your different health care providers and treat other problems as they come up. Your doctor will develop a treatment plan to fit your needs. You and your doctor should review the plan often to be sure it is working. You should report new symptoms to your doctor right away so that your treatment plan can be changed if needed.
Other diseases and conditions that can accompany lupus include fibromyalgia, coronary heart disease, nonbacterial valvular heart disease, pancreatitis, esophagus disease with difficulty swallowing (dysphagia), swollen lymph nodes (lymphadenopathy), liver disease (lupoid hepatitis), infections, and a tendency to spontaneous blood clotting and thrombosis.
A. Chemotherapy can cause a variety of difficult side effects, such as nausea and vomiting. But the chemo drugs used to treat lupus are often gentler than those used to treat cancer, so the side effects aren't as severe. But any type of chemo can harm a woman's fertility by putting her in early menopause, Gilkeson said. While cancer patients sometimes bank their sperm or eggs in order to preserve their future fertility, people with lupus may not have this option, because doctors often want to begin chemo as quickly as possible.
ANA = antinuclear antibody; CNS = central nervous system; ds-DNA = double-stranded DNA; ELISA = enzyme-linked immunoassay; ENA = extractable nuclear antigen; Ig = immunoglobulin; p-ANCA = perinuclear antineutrophil cytoplasmic antibody; RBCs = red blood cells; RNP = ribonucleic protein; SLE = systemic lupus erythematosus; Sm = Smith; SSA = Sjögren syndrome A; SSB = Sjögren syndrome B.

Kidney inflammation in SLE (lupus nephritis) can cause leakage of protein into the urine, fluid retention, high blood pressure, and even kidney failure. This can lead to further fatigue and swelling (edema) of the legs and feet. With kidney failure, machines are needed to cleanse the blood of accumulated waste products in a process called dialysis.
Outcomes research seeks to understand the end results of particular health care practices and interventions. End results include effects that people experience and care about, such as change in the ability to function. In particular, for individuals with chronic conditions—where cure is not always possible—end results include quality of life as well as mortality.
Most people who have SLE have low levels of vitamin D and should take a vitamin D supplement regularly. Vitamin D is essential for proper function of the immune system and several studies have shown that people who have more severe lupus tend to have lower levels of vitamin D compared to those who have milder disease.  It is advised to talk with your consultant or GP about your vitamin D levels as you may already be prescribed calcium supplements which may contain vitamin D. Some dietary sources of vitamin D can be found HERE. It is important to bear in mind that most vitamin D is usually synthesised from sunlight on the skin, but with lupus you should be protecting yourself from exposure to UV.
Neonatal lupus is a rare form of temporary lupus affecting a fetus or newborn. It's not true lupus: It occurs when the mother’s autoantibodies are passed to her child in utero. These autoantibodies can affect the skin, heart, and blood of the baby. Fortunately, infants born with neonatal lupus are not at an increased risk of developing SLE later in life.

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