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.
Most autoimmune diseases affect one specific system. For example, Rheumatoid Arthritis involves the joints, and Multiple Sclerosis affects the brain and spinal cord. Lupus, on the other hand, affects more than one system simultaneously. No matter what organ or system is being attacked, all autoimmune diseases are similar in that they are an immune response caused by systemic inflammation that leads your body to attack itself.
EULAR recommendations for the management of SLE with neuropsychiatric manifestations support the evaluation and treatment of these symptoms in the same way as they are evaluated and treated in patients without SLE; if symptoms persist, management of these symptoms as an extension of SLE should be considered. [83, 61] For example, in patients with neuropsychiatric manifestations that may have an inflammatory etiology, immunosuppressive agents may be considered. [61]
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.
Discoid Lupus is the most common form of Cutaneous Lupus. People living with Discoid Lupus complain of a red, raised and scaly lesion on the face, scalp or parts of the body. Manifestations on the face form across the cheeks, nose and ears. Over time, these lesions can produce scarring and skin discoloration (darkly colored and/or lightly colored areas). Typically, these lesions occur on areas of the body that are exposed to sunlight or fluorescent lights. If lesions appear in the scalp or involve the hair follicles, areas of hair loss may develop which could be permanent if the hair follicle is completely destroyed. They are often not itchy or painful. 
Disease-modifying antirheumatic drugs (DMARDs). DMARDs do more than just treat the symptoms of lupus. Research has shown that they can modify the course of the disease, prevent progression and slow joint damage. DMARDs are often used with NSAIDs. Hydroxychloriquine commonly is prescribed for people with lupus. It can cause vision changes in some people, so it is important to have regular vision examinations. Hydroxychloriquine is effective in preventing flares.
The ACR recommends ANA testing in patients who have two or more unexplained signs or symptoms listed in Table 2.2,20,21 [Reference2—Evidence level C, consensus/expert guidelines] Because of the high rate of false positive ANA titers, testing for systemic lupus erythematosus with an ANA titer or other autoantibody test is not indicated in patients with isolated myalgias or arthralgias in the absence of these specific clinical signs.45 Under most circumstances, a persistently negative ANA titer (less than 1:40) can be assumed to rule out systemic lupus erythematosus.41

Since SLE patients can have a wide variety of symptoms and different combinations of organ involvement, no single test establishes the diagnosis of systemic lupus. To help doctors improve the accuracy of the diagnosis of SLE, 11 criteria were established by the American Rheumatism Association. These 11 criteria are closely related to the symptoms discussed above. Some people suspected of having SLE may never develop enough criteria for a definite diagnosis. Other people accumulate enough criteria only after months or years of observation. When a person has four or more of these criteria, the diagnosis of SLE is strongly suggested. Nevertheless, the diagnosis of SLE may be made in some settings in people with only a few of these classical criteria, and treatment may sometimes be instituted at this stage. Of these people with minimal criteria, some may later develop other criteria, but many never do.
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
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]
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.
According to the Lupus Foundation of America, approximately 1.5 million people in the U.S. have lupus. People of African, Asian, and Native American descent are more likely to develop lupus than are Caucasians. Although it can occur in both men and women, 90% of people diagnosed with the disease are women. Women of childbearing age (14 to 45 years old) are most often affected and as many as 1 in 250 people may develop lupus.
Many drugs have been known to cause this form of the disease, but several are considered primary culprits. They are mainly anti-inflammatories, anticonvulsants, or drugs used to treat chronic conditions such as heart disease, thyroid disease, hypertension (high blood pressure), and neuropsychiatric disorders. The three drugs mostly to blame for drug-induced lupus are:
ANA screening yields positive results in many connective tissue disorders and other autoimmune diseases, and may occur in normal individuals. Subtypes of antinuclear antibodies include anti-Smith and anti-double stranded DNA (dsDNA) antibodies (which are linked to SLE) and anti-histone antibodies (which are linked to drug-induced lupus). Anti-dsDNA antibodies are highly specific for SLE; they are present in 70% of cases, whereas they appear in only 0.5% of people without SLE.[10] The anti-dsDNA antibody titers also tend to reflect disease activity, although not in all cases.[10] Other ANA that may occur in people with SLE are anti-U1 RNP (which also appears in systemic sclerosis and mixed connective tissue disease), SS-A (or anti-Ro) and SS-B (or anti-La; both of which are more common in Sjögren's syndrome). SS-A and SS-B confer a specific risk for heart conduction block in neonatal lupus.[71]

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.

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.


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.
ANAs are proteins made by the body that can attach to DNA and other substances inside cells. But just because they are present in the body doesn’t necessarily mean they will attack these substances. These antibodies are found in at least 5% of the general population, so there are "many more people walking around with ANAs who are perfectly healthy or have some illness that has nothing to do with lupus," adds Dr. Belmont.

Gluten can also lead to what’s known as molecular mimicry. The gluten protein, gliadin, resembles many of your body’s own tissues, particularly thyroid tissue. If you have Celiac disease, gluten intolerance, or a leaky gut, your immune system releases gliadin antibodies every time you eat gluten. Because gliadin looks so similar to your own tissues, sometimes these antibodies mistakenly attack other organs and systems, from the skin to the thyroid to the brain. This case of mistaken identity often leads to full-blown autoimmune disease.


A large randomized trial that compared induction therapy consisting of oral mycophenolate mofetil with cyclophosphamide therapy in patients with lupus nephritis showed that mycophenolate mofetil was not inferior to cyclophosphamide. [132] The investigators suggested that mycophenolate mofetil was associated with both a trend toward greater complete remissions and a greater safety profile. [132] This study’s findings were confirmed with the large, international Aspreva Lupus Management Study (ALMS) trial. [133]
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).
Medical historians have theorized that people with porphyria (a disease that shares many symptoms with SLE) generated folklore stories of vampires and werewolves, due to the photosensitivity, scarring, hair growth, and porphyrin brownish-red stained teeth in severe recessive forms of porphyria (or combinations of the disorder, known as dual, homozygous, or compound heterozygous porphyrias).[121]
The American College of Rheumatology (ACR) established eleven criteria in 1982,[73] which were revised in 1997[74] as a classificatory instrument to operationalise the definition of SLE in clinical trials. They were not intended to be used to diagnose individuals and do not do well in that capacity. For the purpose of identifying people for clinical studies, a person has SLE if any 4 out of 11 symptoms are present simultaneously or serially on two separate occasions.
JAMES M. GILL, M.D., M.P.H., is director of the Health Services Research Group and associate program director of the family practice residency program at Christiana Care Health Services, Wilmington, Del. Dr. Gill received a medical degree from the University of Medicine and Dentistry of New Jersey–Robert Wood Johnson Medical School, Piscataway, and a master of public health degree from Johns Hopkins University, Baltimore....

These foods are not helpful and most of them contribute to raising the risk of coronary heart disease; there is an increased risk of this in people with lupus, so you will protect yourself by reducing the amount of these you consume. The recommended daily amount of salt should not be more than six grams, which is approximately one teaspoonful; many processed foods are highly salted which means that it’s really easy to exceed this amount. Instead of seasoning your food with salt, try using lemon juice or herbs to enhance its flavour.

A chronic inflammation of large arteries, usually the temporal, occipital, or ophthalmic arteries, identified on pathological specimens by the presence of giant cells. It causes thickening of the intima, with narrowing and eventual occlusion of the lumen. It typically occurs after age 50. Symptoms include headache, tenderness over the affected artery, loss of vision, and facial pain. The cause is unknown, but there may be a genetic predisposition in some families. Corticosteroids are usually administered.
Patients with SLE should be educated to avoid triggers for flare. Persons with SLE should avoid ultraviolet light and sun exposure to minimize worsening of symptoms from photosensitivity. Diet modification should be based on the disease activity. A balanced diet is important, but patients with SLE and hyperlipidemia, for example, should be placed on a low-fat diet. Many patients with SLE have low levels of vitamin D because of less sun exposure; therefore, these patients should take vitamin D supplements. Exercise is important in SLE patients to avoid rapid muscle loss, bone demineralization, and fatigue. Smoking should also be avoided.
Drug-induced lupus erythematosus is a (generally) reversible condition that usually occurs in people being treated for a long-term illness. Drug-induced lupus mimics SLE. However, symptoms of drug-induced lupus generally disappear once the medication that triggered the episode is stopped. More than 38 medications can cause this condition, the most common of which are procainamide, isoniazid, hydralazine, quinidine, and phenytoin.[54][10]
Any of a group of immunoglobulin autoantibodies that react with phospholipids, which are one of the primary components of the cell membrane (the other components are glycolipids and steroids). These antibodies are found in patients with a variety of connective tissue and infectious disorders, including systemic lupus erythematosus, the antiphospholipid antibody syndrome, syphilis, and malaria. They cause abnormal blood clotting, thrombocytopenia; and in women of childbearing age, repeated miscarriages. The anticardiolipin antibodies are one type of antiphospholipid antibody.
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.

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.


An inflammatory response (inflammation) occurs when tissues are injured by bacteria, trauma, toxins, heat, or any other cause. The damaged cells release chemicals including histamine, bradykinin, and prostaglandins. These chemicals cause blood vessels to leak fluid into the tissues, causing swelling. This helps isolate the foreign substance from further contact with body tissues.

An inflammatory response (inflammation) occurs when tissues are injured by bacteria, trauma, toxins, heat, or any other cause. The damaged cells release chemicals including histamine, bradykinin, and prostaglandins. These chemicals cause blood vessels to leak fluid into the tissues, causing swelling. This helps isolate the foreign substance from further contact with body tissues.


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.

If you are a young woman with lupus and wish to have a baby, carefully plan your pregnancy. With your doctor’s guidance, time your pregnancy for when your lupus activity is low. While pregnant, avoid medications that can harm your baby. These include cyclophosphamide, cyclosporine, and mycophenolate mofetil. If you must take any of these medicines, or your disease is very active, use birth control. For more information, see Pregnancy and Rheumatic Disease.

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