Lupus is an autoimmune disease characterized by acute and chronic inflammation of various tissues of the body. Autoimmune diseases are illnesses that occur when the body's tissues are attacked by its own immune system. The immune system is a complex system within the body that is designed to fight infectious agents, such as bacteria and other foreign microbes. One of the ways that the immune system fights infections is by producing antibodies that bind to the microbes. People with lupus produce abnormal antibodies in their blood that target tissues within their own body rather than foreign infectious agents. These antibodies are referred to as autoantibodies.
There is no cure for SLE.[1] Treatments may include NSAIDs, corticosteroids, immunosuppressants, hydroxychloroquine, and methotrexate.[1] Alternative medicine has not been shown to affect the disease.[1] Life expectancy is lower among people with SLE.[5] SLE significantly increases the risk of cardiovascular disease with this being the most common cause of death.[4] With modern treatment about 80% of those affected survive more than 15 years.[3] Women with lupus have pregnancies that are higher risk but are mostly successful.[1]
Lupus is often missed or misdiagnosed because the symptoms are vague and can match those of other conditions. Generally, a doctor will review your medical history and your family history, and look for signs of systemic inflammation. Because lupus can involve the internal and external organs, a doctor will rely on observation as well as laboratory testing in order to make a lupus diagnosis. There is no one test for lupus–generally, many different criteria will need to come together, and it can take years to reach a diagnosis.
The diagnosis of lupus is best made by an experienced clinician who fully understands the disease and other diseases with similar features that can mimic lupus. The diagnosis is made when a patient has several features of the disease (including symptoms, findings on examination and blood test abnormalities). The American College of Rheumatology has devised criteria to assist clinicians in making the correct diagnosis of lupus.
Since a large percentage of people with SLE have varying amounts of chronic pain, stronger prescription analgesics (painkillers) may be used if over-the-counter drugs (mainly nonsteroidal anti-inflammatory drugs) do not provide effective relief. Potent NSAIDs such as indomethacin and diclofenac are relatively contraindicated for people with SLE because they increase the risk of kidney failure and heart failure.[83]
Lupus is diagnosed when a person has several features of the disease (including symptoms, findings on examination, and blood test abnormalities). The American College of Rheumatology has devised criteria to assist doctors in making the correct diagnosis of lupus. A person should have at least four of the following 11 criteria, either at the same time or one after the other, to be classified as having lupus. These criteria include:
Other tests for lupus depend on the symptoms patients are experiencing, says Kaplan. For example, chest X-rays and echocardiograms may be necessary to investigate fluid around the lungs and the heart. If doctors suspect nephritis is present, the patient may need a kidney biopsy. Early diagnosis and treatment can help to avoid complications, he adds.
Hormonal mechanisms could explain the increased incidence of SLE in females. The onset of SLE could be attributed to the elevated hydroxylation of estrogen and the abnormally decreased levels of androgens in females. In addition, differences in GnRH signalling have also shown to contribute to the onset of SLE. While females are more likely to relapse than males, the intensity of these relapses is the same for both sexes.[12]
Certain foods, including garlic and alfalfa sprouts, should be avoided by people with lupus. [For a more complete list of items to be avoided, please see the article “Things to Avoided” in the Lupus 101 section.] Recently controversy has also arisen over whether aspartame induces lupus. However, scientists have concluded that there is no evidence to suggest that aspartame causes lupus.

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A group of people who review, approve, and monitor the clinical study protocol. Their role is to protect the rights and welfare of human research subjects participating in a study. The group typically includes people with varying backgrounds, including a community member, to make sure that research activities conducted by an organization are completely and adequately reviewed. Also known as an institutional review board (IRB) or ethics committee.
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.
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.
Sources:  (1.) American College of Rheumatology. 1997 Update of the 1982 American College of Rheumatology revised criteria for classification of systemic lupus erythematosus. Available at: http://tinyurl.com/zrfsuhs Accessed: September 19, 2016 [94] ; (2.) Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. Sep 1997;40(9):1725. [5]
Gene regulation is the process of turning genes on and off. During early development, cells begin to take on specific functions. Gene regulation ensures that the appropriate genes are expressed at the proper times. Gene regulation can also help an organism respond to its environment. Gene regulation is accomplished by a variety of mechanisms including chemically modifying genes and using regulatory proteins to turn genes on or off.
Arthritis is inflammation of one or more joints and it involves the breakdown of cartilage. Cartilage normally protects a joint, allowing it to move smoothly. Cartilage also absorbs shock when pressure is placed on the joint, such as when you walk. Without the normal amount of cartilage, the bones rub together, causing pain, swelling (inflammation), and stiffness. Usually the joint inflammation goes away after the cause goes away or is treated. Sometimes it does not. When this happens, you have chronic arthritis. Arthritis may occur in men or women. Osteoarthritis is the most common type.
Drugs used to treat lupus include nonsteroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen and naproxen, alone or combined with other drugs for pain, swelling, and fever. Drugs that work inside cells, including antimalarial drugs such as hydroxychloroquine (Plaquenil) are used for fatigue, joint pain, skin rashes, and inflammation of the lungs. Continuous treatment with antimalarials may prevent lupus flare up from recurring.
Erythrocyte Sedimentation Rate:  This is a blood test that is used to determine the rate at which red blood cells settle to the bottom of a tube in one hour’s time.  If the rate is faster than normal, it may be an indication of a systemic disease, like lupus.  It is important to note that this sedimentation rate, or rate of settling, does not specifically indicate lupus, but can be elevated if other inflammatory conditions are present like cancer or an infection.
In 2007, the European League Against Rheumatism (EULAR) released recommendations for the treatment of SLE. [61] In patients with SLE without major organ manifestations, glucocorticoids and antimalarial agents may be beneficial. [61] NSAIDs may be used for short periods in patients at low risk for complications from these drugs. Consider immunosuppressive agents (eg, azathioprine, mycophenolate mofetil, methotrexate) in refractory cases or when steroid doses cannot be reduced to levels for long-term use. [106]
People with lupus have a higher risk of CAD. This is partly because people with lupus have more CAD risk factors, which may include high blood pressure, high cholesterol, and type 2 diabetes. The inflammation that accompanies lupus also increases the risk of developing CAD. People with lupus are often less active because of fatigue, joint problems, and/or muscle pain, and this also puts them at risk.
The term undifferentiated connective tissue diseases is used to define conditions characterized by the presence of signs and symptoms suggestive of a systemic autoimmune disease that do not satisfy the classificative criteria for defined connective tissue diseases (CTD) such as systemic lupus erythematosus (SLE), Sjögren’s syndrome (SS), rheumatoid arthritis (RA) and others. A small percentage of patients presenting with an undifferentiated profile will develop during the first year follow up of a full blown CTD, however an average of 75% will maintain an undifferentiated clinical course. These patients may be defined as having a stable undifferentiated connective tissue diseases (UCTD). The most characteristic symptoms of UCTD are represented by arthritis and arthralgias, Raynaud’s phenomenon, leukopenia, while neurological and kidney involvement are virtually absent. Eighty percent of these patients have a single autoantibody specificity, more frequently anti-Ro and anti-RNP antibodies. Stable UCTD are considered as distinct clinical entities and therefore it has been proposed to define those conditions as UCTD. Classificative criteria have also been proposed and a work to better define them is still under way.
SLE may cause pericarditis—inflammation of the outer lining surrounding the heart, myocarditis—inflammation of the heart muscle, or endocarditis—inflammation of the inner lining of the heart. The endocarditis of SLE is non-infectious, and is also called (Libman–Sacks endocarditis). It involves either the mitral valve or the tricuspid valve. Atherosclerosis also occurs more often and advances more rapidly than in the general population.[23][24]
The panel concluded that long-term IS agents during maintenance therapy prolong stable renal function, reduce proteinuria, extend renal survival and minimise the toxicity of GCs. AZA, CYC, MMF and CsA seem to be equivalent regarding efficacy but MMF and AZA have a better safety profile, particularly regarding gonadal toxicity and blood pressure control. We found very low certainty of the evidence for TAC as maintenance therapy, with studies mostly restricted to Asian populations.
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.
For resistant skin disease, other antimalarial drugs, such as chloroquine (Aralen) or quinacrine, are considered and can be used in combination with hydroxychloroquine. Alternative medications for skin disease include dapsone and retinoic acid (Retin-A). Retin-A is often effective for an uncommon wart-like form of lupus skin disease. For more severe skin disease, immunosuppressive medications are considered as described below.
Neuropsychiatric syndromes can result when SLE affects the central or peripheral nervous system. The American College of Rheumatology defines 19 neuropsychiatric syndromes in systemic lupus erythematosus.[30] The diagnosis of neuropsychiatric syndromes concurrent with SLE (now termed as NPSLE),[31] is one of the most difficult challenges in medicine, because it can involve so many different patterns of symptoms, some of which may be mistaken for signs of infectious disease or stroke.[32]
Lupus is often missed or misdiagnosed because the symptoms are vague and can match those of other conditions. Generally, a doctor will review your medical history and your family history, and look for signs of systemic inflammation. Because lupus can involve the internal and external organs, a doctor will rely on observation as well as laboratory testing in order to make a lupus diagnosis. There is no one test for lupus–generally, many different criteria will need to come together, and it can take years to reach a diagnosis.

Kidney involvement in people with lupus is potentially life threatening and may occur in up to half of lupus patients. Kidney problems may become apparent when lupus patients feel ill with arthritis, have a rash, fever and weight loss. Less often, kidney disease may occur when there are no other symptoms of lupus. Kidney disease itself usually does not produce symptoms until it is in the advanced stages. It is important that kidney disease be diagnosed early and treated appropriately. The earliest signs of kidney disease are apparent from a urinalysis.


The discovery of the LE cell led to further research and this resulted in more definitive tests for lupus. Building on the knowledge that those with SLE had auto-antibodies that would attach themselves to the nuclei of normal cells, causing the immune system to send white blood cells to fight off these "invaders", a test was developed to look for the anti-nuclear antibody (ANA) rather than the LE cell specifically. This ANA test was easier to perform and led not only to a definitive diagnosis of lupus but also many other related diseases. This discovery led to the understanding of what are now known as autoimmune diseases.[119]
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.
Opportunistic infections can develop, most often in patients receiving chronic immunosuppressive therapy. Another less-common complication is osteonecrosis, especially of the hips and knees after prolonged high-dose corticosteroid usage. More commonly, premature atherosclerotic disease and myocardial infarction are indolent complications of chronic inflammation and steroids.

Blood—hematologic disorder—hemolytic anemia (low red blood cell count), leukopenia (white blood cell count<4000/µl), lymphopenia (<1500/µl), or low platelet count (<100000/µl) in the absence of offending drug; sensitivity = 59%; specificity = 89%.[75] Hypocomplementemia is also seen, due to either consumption of C3[76] and C4 by immune complex-induced inflammation or to congenitally complement deficiency, which may predispose to SLE.
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.
Blood and urine tests. The antinuclear antibody (ANA) test can show if your immune system is more likely to make the autoantibodies of lupus. Most people with lupus test positive for ANA. But, a positive ANA does not always mean you have lupus. If you test positive for ANA, your doctor will likely order more tests for antibodies that are specific to systemic lupus erythematosus (SLE).
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.

Unfortunately, there are no widely accepted diagnostic criteria for SLE. However, many doctors use the American College of Rheumatology (ACR) 11 common criteria. These criteria were designed to identify subjects for research studies, so they are very stringent. If you currently have four or more of these criteria or if you've had them in the past, chances are very high that you have SLE. However, having less than four doesn't rule out SLE. Again, additional testing may be necessary to inform a formal diagnosis. These criteria include:


“NHS dieticians seem to specialise in those struggling to lose (rather than gain) weight in my experience. On my initial consultation I was given a booklet with advice based on eating a full English breakfast, then snacks like doughnuts and pork pies. My sons would be thrilled to get medical advice to eat like that! The nutritional supplements they offer taste extremely artificial to me. I can only eat a little and very slowly, so get to ‘savour’ every sip of it. I’m trying protein shakes I buy myself, which taste better, but just one of those is very filling.”
Conventional lupus treatment usually involves a combination of medications used to control symptoms, along with lifestyle changes — like dietary improvements and appropriate exercise. It’s not uncommon for lupus patients to be prescribed numerous daily medications, including corticosteroid drugs, NSAID pain relievers, thyroid medications and even synthetic hormone replacement drugs. Even when taking these drugs, it’s still considered essential to eat an anti-inflammatory lupus diet in order to manage the root causes of lupus, along with reducing its symptoms.

Most people with lupus have symptoms in only a few organs. If you have not already been diagnosed, the following table may alert you to the possibility of lupus. If you have already been diagnosed, these symptoms may indicate increased activity of the disease, known as a "flare." You may also have periods of remission when few or no symptoms are present. For most people, lupus can be managed and will affect only a few organs. Others may face serious, sometimes life-threatening problems.


SLE is associated with defects in apoptotic clearance, and the damaging effects caused by apoptotic debris. Early apoptotic cells express “eat-me” signals, of cell-surface proteins such as phosphatidylserine, that prompt immune cells to engulf them. Apoptotic cells also express “find-me” signals, to attract macrophages and dendritic cells. When apoptotic material is not removed correctly by phagocytes, they are captured instead by antigen-presenting cells, which leads to development of antinuclear antibodies.[4]
Subacute Cutaneous Lupus can cause skin lesions on any part of the body. These lesions often form red, ring-shaped, scaly patches on the skin. These lesions do not itch and often appear on the chest as well as the upper back and neck; however, they may also be seen on the face and arms. Typically, these lesions occur on areas of the body that are exposed to sunlight or fluorescent lights. Furthermore, it is not uncommon for patients with SCLE to have associated joint disease.
Patients with SLE exhibit a variety of symptoms depending on the severity of their disease. In some cases, the onset of SLE is sudden, with patients developing fever and a general feeling of malaise (that can be mistaken for an acute infection), whereas other patients experience less acute episodes of fever and feeling unwell over many months and years.
The first mechanism may arise genetically. Research indicates SLE may have a genetic link. SLE does run in families, but no single causal gene has been identified. Instead, multiple genes appear to influence a person's chance of developing lupus when triggered by environmental factors. HLA class I, class II, and class III genes are associated with SLE, but only classes I and II contribute independently to increased risk of SLE.[45] Other genes which contain risk variants for SLE are IRF5, PTPN22, STAT4,[46] CDKN1A,[47] ITGAM, BLK,[46] TNFSF4 and BANK1.[48] Some of the susceptibility genes may be population specific.[46]
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.

Fertility rates in women with systemic lupus erythematosus (SLE) may be similar to those in the general population. However, the incidence of spontaneous abortion, premature labor, early preeclampsia/eclampsia, fetal growth restriction, and intrauterine death are somewhat higher in women with SLE, [61, 138] especially in those with SSA(Ro)/SSB(La) antibodies, antiphospholipid antibodies, [88] or lupus nephritis. [139] One study suggested that women with SLE have fewer live births than the general population. [140] In this study, decreased live births were associated with exposure to cyclophosphamide and high SLE disease activity.


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.

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