No single finding qualifies an individual as having SLE. Instead, the American College of Rheumatology (ACR) has devised certain classification criteria, and four or more of these criteria must be present for a classification of lupus. [The term “classification” is not synonymous with “diagnosis.” “Classification” means that reasonable certainty exists for the diagnosis of lupus for research purposes.] Although, these criteria are currently being updated, they are believed to be about 90% effective. The ACR criteria include malar rash; discoid rash; photosensitivity (development of a rash after sun exposure); oral or nasal ulcers; arthritis of multiple joints; serositis: (inflammation of the lining around the lungs or heart); kidney disease indicated by protein or casts in the urine; neurological disorders such as seizures and psychosis; and blood disorders such as hemolytic anemia, leukopenia, and lymphopenia. Other signs that are common but not included in the classification criteria are hair loss or breaking, especially around the forehead, and Raynaud’s Phenomenon, a two- or three-color change of the fingertips upon cold exposure.

Vasculitis, antiphospholipid antibodies, and renal failure are commonly found in patients with lupus; these conditions greatly increase the risk of developing pulmonary emboli. The diagnosis in a patient with shortness of breath, hemoptysis, and pleuritic chest pain is commonly made with ventilation-perfusion scans or computed tomography (CT) angiography. The CT angiogram demonstrates a filling defect in the left anterior segmental artery (arrow).
A rheumatologic illness marked by fevers, malaise, weight loss, muscle pain, stiffness (esp. of the shoulders and pelvis), and morning stiffness. It occurs primarily, but not exclusively, in white people over 60. The cause of PMR is unknown. Although there is no single diagnostic test for PMR, patients typically have a markedly elevated erythrocyte sedimentation rate (>50 mm/hr) and no evidence of another disease (such as infection, cancer, rheumatoid arthritis, or lupus). Patients obtain rapid and durable relief from corticosteroids but usually require a course of treatment lasting 6 to 18 months. Pathologically, and sometimes clinically, PMR is related to giant cell arteritis. Mild cases may sometimes respond to nonsteroidal anti-inflammatory drugs.
Lupus is an autoimmune disease that takes on several forms, of which systemic lupus erythematosus (SLE) is one. Lupus can affect any part of the body, but it most commonly attacks your skin, joints, heart, lungs, blood cells, kidneys, and brain. Around 1.5 million Americans have some form of lupus, according to the Lupus Foundation of America, with an estimated 16,000 newly diagnosed each year. Anyone at any age can acquire the disease, though most lupus patients are women between the ages of 15 and 45.
Systemic lupus erythematosus (SLE) is a complex multisystemic autoimmune disease resulting, oftentimes, in irreversible damage, diminished quality of life and reduced life expectancy.1–3 Genetic and environmental factors play important roles in its pathogenesis.4–8 Disease manifestations and severity vary according to the patients’ racial/ethnic background and socioeconomic status (SES).1 9 10 Data from Grupo Latino Americano de Estudio del Lupus (GLADEL), Lupus in Minorities: Nature vs Nurture (LUMINA) and the Lupus Family Registry and Repository cohorts have demonstrated that Latin American and North American Mestizo patients (mixed Amerindian and European ancestry), African descendants and Native Americans develop lupus earlier11 12 although diagnostic delays may occur.1 They also experience more severe disease, have higher disease activity levels,1 accrue more organ damage2 and have higher mortality rates,1 succumbing mainly to disease activity and/or infections.1 3 13–15
Flare-ups of lupus can cause acute inflammation and damage to various body tissues and can affect the joints, skin, kidneys, heart, lungs, blood vessels, and brain. Some of the most common symptoms are painful or swollen joints, unexplained fever, kidney problems and extreme fatigue. A characteristic red skin rash – called a “malar” or “butterfly” rash because it roughly mimics the insect’s shape – may appear across the nose and cheeks. Rashes may also occur on the face and ears, upper arms, shoulders, chest, and hands. Because many lupus patients are sensitive to sunlight, skin rashes often develop or worsen after sun exposure.
To unravel which people with positive ANA tests actually have lupus, additional blood work can be done. Doctors look for other potentially troublesome antibodies, so they will test for anti-double-stranded DNA and anti-Smith antibodies. These tests are less likely to be positive unless a patient truly has lupus. However, a person who has negative test results could still have lupus, even though this is not so in the case of ANA tests.
While there is no specific lupus diet, scientists have found that low-dose diet supplementation with omega-3 fish oils could help patients with lupus by decreasing inflammation and disease activity and possibly decreasing heart-disease risk. It is generally recommended that patients with lupus eat a balanced diet that includes plant-based foods and lean sources of protein.
A. Like Gomez, people with lupus often begin chemotherapy, which helps to suppress the immune system. Gomez has said that she is in remission, which means her disease is not causing her any symptoms. With luck, these remissions can last for years. But about 25% of people with lupus a year experience a "flare," in which symptoms recur. To keep the disease under control, people with lupus need to be treated for the rest of their lives. Most take a drug called hydroxychloroquine, which is also used to fight malaria. People also usually take an immune-suppressing drug, Gilkeson said.
The most serious health risks are cardiovascular disease, kidney disease and stroke. Specifically, people with lupus are at increased risk for atherosclerosis (hardening of the arteries). In some people, inflammation can occur in the heart itself (myocarditis and endocarditis) or the membrane that surrounds it. Endocarditis can damage heart valves, which can result in heart murmurs. When the disease affects the kidneys, patients generally require intensive drug treatment to prevent permanent damage. Lupus also may attack the brain or central nervous system, which can cause seizures or stroke.
The immune response is how your body recognizes and defends itself against bacteria, viruses, and substances that appear foreign and harmful.The immune system protects the body from possibly harmful substances by recognizing and responding to antigens. Antigens are substances (usually proteins) on the surface of cells, viruses, fungi, or bacteria. Nonliving substances such as toxins, chemicals, drugs, and foreign particles (such as a splinter) can also be antigens. The immune system recognizes and destroys substances that contain antigens. Your own body’s cells have proteins that are antigens. These include a group of antigens called HLA antigens. Your immune system learns to see these antigens as normal and usually does not react against them.

Photosensitive systemic lupus erythematosus (SLE) rashes typically occur on the face or extremities, which are sun-exposed regions. Although the interphalangeal spaces are affected, the metacarpophalangeal (MCP) and proximal interphalangeal (PIP) and distal interphalangeal (DIP) joints are spared. Photo courtesy of Dr. Erik Stratman, Marshfield Clinic.
Jump up ^ Smyth, Andrew; Guilherme H.M. Oliveira; Brian D. Lahr; Kent R. Bailey; Suzanne M. Norby; Vesna D. Garovic (November 2010). "A Systematic Review and Meta-Analysis of Pregnancy Outcomes in Patients with Systemic Lupus Erythematosus and Lupus Nephritis". Clinical Journal of the American Society of Nephrology. 5 (11): 2060–2068. doi:10.2215/CJN.00240110. PMC 3001786. PMID 20688887. Archived from the original on 2016-01-26.
Approximately 20% of people with SLE have clinically significant levels of antiphospholipid antibodies, which are associated with antiphospholipid syndrome.[90] Antiphospholipid syndrome is also related to the onset of neural lupus symptoms in the brain. In this form of the disease the cause is very different from lupus: thromboses (blood clots or "sticky blood") form in blood vessels, which prove to be fatal if they move within the blood stream.[79] If the thromboses migrate to the brain, they can potentially cause a stroke by blocking the blood supply to the brain.
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.
Regulatory T cells (Tregs) are a population of CD4+ T cells with a unique role in the immune response. Tregs are crucial in suppressing aberrant pathological immune responses in autoimmune diseases, transplantation, and graft-vs-host disease after allogeneic hematopoietic stem cell transplantation. Tregs are activated through the specific T-cell receptor, but their effector function is nonspecific and they regulate the local inflammatory response through cell-to-cell contact and cytokine secretion. Tregs secrete interleukin (IL)-9 (IL-9), IL-10, and transforming growth factor-beta 1 (TGF-beta 1), which aid in the mediation of immunosuppressive activity.
Photosensitive systemic lupus erythematosus (SLE) rashes typically occur on the face or extremities, which are sun-exposed regions. Although the interphalangeal spaces are affected, the metacarpophalangeal (MCP) and proximal interphalangeal (PIP) and distal interphalangeal (DIP) joints are spared. Photo courtesy of Dr. Erik Stratman, Marshfield Clinic.
Avoid calcium supplements, however, which Johns Hopkins researchers have found to potentially increase the risk of heart damage and arterial plaque buildup. “Due to the risk of accelerated atherosclerosis in lupus, we no longer recommend calcium supplementation and encourage a diet rich in calcium instead,” noted George Stojan, MD, a rheumatologist and assistant professor of medicine at Johns Hopkins.
The panel recommends HCQ plus LMWH plus LDA over HCQ plus LDA or adding GCs or intravenous Ig for pregnant patients with SLE with antiphospholipid antibodies and recurrent pregnancy loss (strong recommendation based on moderate certainty of the evidence (LMWH plus LDA vs other alternatives) and very low certainty of the evidence (GCs and intravenous Ig vs other alternatives), since high certainty of harms related to GCs (increased premature delivery) and intravenous Ig (costs increase, burden related to drug administration) exists).
While the genetics of SLE are not very well understood, there is growing evidence for the involvement of specific genes in this complex autoimmune disease. Part of the complexity of this disease is due to the effects of both environment and genetics factors that may contribute to its development.[49] Further compounding our understanding of the etiology of the disease is the involvement of several organ systems.[50] Genetic studies of the rates of disease in families supports the genetic basis of this disease with a heritability of >66%.[51] Identical (monozygotic) twins were found to share susceptibility to the disease at >35% rate compared to fraternal (dizygotic) twins and other full siblings who only showed a 2–5% concordance in shared inheritance.[51]
Heart: If inflammation affects the heart, it can result in myocarditis and endocarditis. It can also affect the membrane that surrounds the heart, causing pericarditis. Chest pain or other symptoms may result. Endocarditis can damage the heart valves, causing the valve surface to thicken and develop. This can result in growths that can lead to heart murmurs.

Some people with lupus experience occasional heartburn, acid reflux, or other gastrointestinal problems. Mild symptoms can be treated with OTC antacids. If you have frequent bouts of acid reflux or heartburn, try cutting down on the size of your meals, and avoid beverages containing caffeine. Also, don’t lie down right after a meal. If symptoms continue, see your doctor to rule out other conditions.
Thinning hair is often one of the first symptoms of lupus. Hair loss is the result of inflammation of the skin and scalp. Some people with lupus lose hair by the clump. More often, hair thins out slowly. Some people also have thinning of the beard, eyebrows, eyelashes, and other body hair. Lupus can cause hair to feel brittle, break easily, and look a bit ragged, earning it the name “lupus hair.”
Some people find that excluding gluten from their diet gives them more energy. Don’t be tempted to start excluding a lot of foods from your diet; this could lead to serious deficiencies. Avoiding gluten or dairy products will not necessarily prevent flares; food “triggers” vary greatly from person to person.  If you feel that you have problems processing certain foods, talk to your GP and ask for a referral to either a dietician or an allergy specialist within the NHS. There are commercial allergy tests available, but these are not always accurate and could cost you a lot of money but bring you no lasting benefit.
The role of the immune system in causing diseases is becoming better understood through research. This knowledge will be applied to design safer and more effective treatment methods. For example, completely revising the immune system of people with extremely aggressive treatments that virtually temporarily wipe out the immune system is being evaluated. Current studies involve immune eradication with or without replacement of cells that can reestablish the immune system (stem-cell transplantation).
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]

Environment Researchers suspect environmental factors may increase the risk of developing lupus. For example, exposure to sun can cause a lupus rash and some systemic lupus activity, says Stacy Ardoin, MD, a rheumatologist at the Ohio State University Wexner Medical Center in Columbus. Other environmental factors that may contribute to lupus can include some drugs, viral infections, exhaustion, stress, and anything that can cause physical stress to the body (such as surgery, physical harm, injury, pregnancy, or giving birth).


A member of a group of enzymes that can break down proteins, such as collagen, that are normally found in the spaces between cells in tissues (i.e., extracellular matrix proteins). Because these enzymes need zinc or calcium atoms to work properly, they are called metalloproteinases. Matrix metalloproteinases are involved in wound healing, angiogenesis, and tumor cell metastasis.
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.
For each of the subheadings listed below, the panel considered interventions based on experience, availability, affordability and a stepwise therapeutic approach of the different alternatives. Standard of care (SOC) was defined as the use of hydroxychloroquine (HCQ) and, if clinically indicated, low-dose glucocorticoids (GC) (prednisone ≤7.5 mg or equivalent for the shortest time).24 Chloroquine remains an alternative for some of the Latin American countries where HCQ is not available and careful monitoring of eye side effect is recommended. Overarching principles are shown in box 1. Tables summarising the evidence that was considered in the process are shown in online supplementary tables in https://doi.org/10.5061/dryad.bg8452h.
Any of a group of autoantibodies that react against normal components of the cell nucleus. They are present in several immunologic diseases, including systemic lupus erythematosus, progressive systemic sclerosis, Sjögren syndrome, scleroderma, polymyositis, and dermatomyositis, and in some patients taking hydralazine, procainamide, or isoniazid. In addition, ANA is present in some normal people. Tests for ANAs are used in the diagnosis and management of autoimmune diseases.
Studies have shown that lupus multiplies a woman’s risk for osteoporosis five times4 – an alarming figure, no doubt. Osteoporosis is a condition where bone density decreases, leading to an increased risk of skeletal fracture at older age. Like lupus, osteoporosis has no cure, so it's a condition better prevented  than treated, especially for women.
Based on the identified evidence the panel concluded that compared with GCs alone, the addition of other IS (CYC, MMF or TAC) is associated with significant benefits, higher remission rates and lower progression rates to end-stage renal disease (ESRD). Head-to-head comparisons between MMF, TAC and high-dose CYC showed that MMF and TAC are associated with less adverse effects than high-dose CYC. Between low and high-dose CYC the balance favours the former because of better safety profile and comparable efficacy, although this conclusion is based on one trial that included predominantly Caucasians. RTX did not provide additional benefits when combined with MMF.
Decorin is a protein coded for by the DCN gene. This protein is a component of the extracellular matrix, which is the intricate lattice of proteins and other molecules that forms in the spaces between cells. Decorin is found in the extracellular matrix of a variety of connective tissues, including skin, tendon, bone, and cartilage. Connective tissues support the body’s joints and organs. Decorin is involved in the organization of proteins called collagens. Collagens strengthen and support connective tissues throughout the body. Collagens also play an important role in the cornea, which is the clear outer covering of the eye. Bundles of collagen called fibrils must be strictly organized for the cornea to be transparent. Decorin ensures that these collagen fibrils are uniformly sized and regularly spaced.
Systemic sclerosis (SSc): Similar symptoms between SSc and lupus are reflux and Raynaud's disease (when your fingers turn blue or white with cold). One difference between SSc and lupus is that anti-double-stranded DNA (dsDNA) and anti-Smith (Sm) antibodies, which are linked to lupus, don't usually occur in SSc. Another differentiator is that people with SSc often have antibodies to an antigen called Scl-70 (topoisomerase I) or antibodies to centromere proteins.
Lupus nephritis is one of the most common complications of lupus. (13) People with lupus nephritis are at a higher risk of developing end-stage renal disease, requiring dialysis or a transplant, says Kaplan. Symptoms of the condition include high blood pressure; swelling of the hands, arms, feet, legs, and area around the eyes; and changes in urination, such as noticing blood or foam in the urine, needing to go to the bathroom more frequently at night, or pain or trouble urinating.
Inflammation of the heart muscle, usually in the U.S. as a consequence of infections (viruses, esp. coxsackie virus, and occasionally as a consequence of bacterial, protozoan or fungal infections); immunological-rheumatological conditions (e.g., systemic lupus erythematosus, ulcerative colitis, hypersensitivity reactions, or transplant rejection); exposure to chemicals or toxins (e.g., cocaine, doxorubicin, methamphetamine); nutritional or metabolic abnormalities (e.g., thiamine deficiency or hypophosphatemia); or radiation. Myocarditis also is occasionally found in pregnancy and with advanced age. The myocardium is infiltrated by leukocytyes, lymphocytes, and macrophages, leading to inflammation, necrosis of muscle cells, and fibrosis. Inflammatory damage to heart muscle fibers may resolve spontaneously or may cause progressive deterioration of the heart with pericarditis, arrhythmias, chronic dilated cardiomyopathy, and heart failure.
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).
In one study41 that used patients with connective tissue diseases as the control group, the revised ACR diagnostic criteria for systemic lupus erythematosus were found to have an overall sensitivity of 96 percent and a specificity of 96 percent. Other studies21,32,43 have reported sensitivities ranging from 78 to 96 percent and specificities ranging from 89 to 100 percent. The ACR criteria may be less accurate in patients with mild disease.21
Jump up ^ Henderson, LA; Loring, SH; Gill, RR; Liao, KP; Ishizawar, R; Kim, S; Perlmutter-Goldenson, R; Rothman, D; Son, MB; Stoll, ML; Zemel, LS; Sandborg, C; Dellaripa, PF; Nigrovic, PA (March 2013). "Shrinking lung syndrome as a manifestation of pleuritis: a new model based on pulmonary physiological studies". The Journal of Rheumatology. 40 (3): 273–81. doi:10.3899/jrheum.121048. PMC 4112073. PMID 23378468.
*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.    
Fatigue is different from drowsiness. Drowsiness is feeling the need to sleep. Fatigue is a lack of energy and motivation. Drowsiness and apathy (a feeling of not caring about what happens) can be symptoms that go along with fatigue. Fatigue can be a normal and important response to physical activity, emotional stress, boredom, or lack of sleep. Fatigue is a common symptom, and it is usually not due to a serious disease. But it can be a sign of a more serious mental or physical condition. When fatigue is not relieved by enough sleep, good nutrition, or a low-stress environment, it should be evaluated by your doctor.
A skeletal disorder characterized by compromised bone strength predisposing to an increased risk of fracture. Bone strength reflects the integration of two main features: bone density and bone quality. Bone density is expressed as grams of mineral per area or volume and in any given individual is determined by peak bone mass and amount of bone loss. Bone quality refers to architecture, turnover, damage accumulation (e.g., microfractures) and mineralization. A fracture occurs when a failure-inducing force (e.g., trauma) is applied to osteoporotic bone. Thus, osteoporosis is a significant risk factor for fracture, and a distinction between risk factors that affect bone metabolism and risk factors for fracture must be made.
The cause of lupus remains unknown, but there is solid evidence that genetics, epigenetics (changes in chromosomes that affect gene activity), environmental factors, viruses and infections play a role. Further study of these variables is expected to improve our understanding of causes, which should lead to improved diagnosis, prognosis, prevention, and treatment.
Patients with class III or IV disease, as well as those with a combination of class V and class III or IV disease, generally undergo aggressive therapy with glucocorticoid drugs and immunosuppressants. [96] Immunosuppressive therapy consists of induction and maintenance therapy. Induction therapy involves potent immunosuppressive drugs (eg, mycophenolate mofetil, cyclophosphamide) to achieve remission; these drugs are generally used for 3 months to 1 year, with an average of 6 months’ treatment having been shown to be more efficacious and safer than long-term therapy. [131]
A healthy diet is important in general, but perhaps even more so for the roughly 1 million Americans and 3 million people worldwide who suffer from systemic lupus erythematosus (SLE).1 Because of the multifaceted challenges presented by the disease, consuming adequate amounts of the right nutrients — and limiting others — can help relieve symptoms and improve outcomes.
People with SLE have intense polyclonal B-cell activation, with a population shift towards immature B cells. Memory B cells with increased CD27+/IgD—are less susceptible to immunosuppression. CD27-/IgD- memory B cells are associated with increased disease activity and renal lupus. T cells, which regulate B-cell responses and infiltrate target tissues, have defects in signaling, adhesion, co-stimulation, gene transcription, and alternative splicing. The cytokines B-lymphocyte stimulator (BLys), interleukin 6, interleukin 17, interleukin 18, type I interferons, and tumor necrosis factor α (TNFα) are involved in the inflammatory process and are potential therapeutic targets.[4][60][61]
The prognosis for those with lupus often depends on the amount of organ involvement. In other words, is the disease targeting organs rather than skin and joints? Survival for lupus patients with central nervous system symptoms, major organ involvement, and/or kidney disease, is likely to be shorter than those with only skin and/or joint disease related to lupus. The most common cause of death associated with lupus is an infection due to immunosuppression, caused by medications used to manage the disease, especially early in ​the disease.

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