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.

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.

Although these guidelines consider region limitations, the inclusion of alternative approaches for tailoring treatment did not exclude the task of providing physicians with the state-of-the-art findings in the field. This was a major advantage of the present work since highlighting these advances provides valuable basis for future requirement of government authorisation of new drugs in these countries.
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]
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.
One common early symptom that can be indicative of lupus is a photosensitive rash, meaning a rash that develops in response to sun exposure, particularly on the face and upper arms, says Dr. Kramer. Other early symptoms are unexplained fever and pain, swelling, and stiffness of multiple joints. Complications such as inflammation of the lining surrounding the lungs or heart can also occur early on, he adds.
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.
Landmark research has shown clearly that oral contraceptives do not increase the rate of flares of systemic lupus erythematosus. This important finding is opposite to what has been thought for years. Now we can reassure women with lupus that if they take birth-control pills, they are not increasing their risk for lupus flares. Note: Birth-control pills or any estrogen medications are still be avoided by women who are at increased risk of blood clotting, such as women with lupus who have phospholipid antibodies (including cardiolipin antibody and lupus anticoagulant).
Because some treatments may cause harmful side effects, it is important to report any new symptoms to the doctor promptly. It is also important not to stop or change treatments without talking to the doctor first. In addition to medications for lupus itself, in many cases it may be necessary to take additional medications to treat problems related to lupus such as high cholesterol, high blood pressure, or infection.
Mixed connective tissue disease (MCTD) is a autoimmune disorder that causes overlapping features of three connective tissue disorders: lupus, scleroderma, and polymyositis. MCTD may also have features of rheumatoid arthritis. This condition is most often diagnosed in women in their 20’s and 30’s. Occasionally, children are affected. At this time the cause of this condition is unknown.
The ACR Quality of Care statement [147] recommends annual cardiovascular disease risk assessment; some researchers suggest that the cardiovascular risk for SLE is similar to that for diabetes mellitus. The 10-year coronary event rate is 13-15% in patients with active SLE, which is comparable to the 10-year event rate of 18.8% in patients with known coronary artery disease. [148] African American patients with SLE may be particularly vulnerable to premature cardiovascular disease and related death. [149]
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 following drugs are commonly used to treat the inflammation and symptoms of lupus. Since lupus manifests in different ways in different people, treatment regimens differ from patient to patient. In addition, one patient may experience several different treatment regimens during her/his lifetime. It is important that you understand the medications you are taking and the risks, benefits, and restrictions associated with them. Please remember to take your medications exactly as directed by your physician and to address any questions or concerns upon your next visit.

Note: Ultimately, in patients with kidney disease from systemic lupus erythematosus (lupus nephritis), a kidney biopsy may be necessary to both define the cause of the kidney disease as being lupus-related as well as to determine the stage of the kidney disease in order to optimally guide treatments. Kidney biopsies are often performed by fine-needle aspiration of the kidney under radiology guidance, but in certain circumstances, a kidney biopsy can be done during an open abdominal operation.
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.
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.
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The Accelerating Medicines Partnership (AMP) is a bold new venture between the NIH, 10 biopharmaceutical companies and several non-profit organizations to transform the current model for developing new diagnostics and treatments by jointly identifying and validating promising biological targets of disease. The ultimate goal is to increase the number of new diagnostics and therapies for patients and reduce the time and cost of developing them.
A common neurological disorder people with SLE have is headache,[33] although the existence of a specific lupus headache and the optimal approach to headache in SLE cases remains controversial.[34] Other common neuropsychiatric manifestations of SLE include cognitive dysfunction, mood disorder, cerebrovascular disease,[33] seizures, polyneuropathy,[33] anxiety disorder, psychosis, depression, and in some extreme cases, personality disorders.[35] Steroid psychosis can also occur as a result of treating the disease.[31] It can rarely present with intracranial hypertension syndrome, characterized by an elevated intracranial pressure, papilledema, and headache with occasional abducens nerve paresis, absence of a space-occupying lesion or ventricular enlargement, and normal cerebrospinal fluid chemical and hematological constituents.[36]
For arthritic symptoms, take a natural anti-inflammatory agent, containing ginger and turmeric. Get the right kind of regular exercise; swimming or water aerobics are best for those who have arthritis symptoms. Investigate traditional Chinese medicine and Ayurvedic medicine, both of which often do well with autoimmune conditions. Definitely try one or more mind/body therapies, such as hypnosis or interactive guided imagery.
Similarly, a phase III trial of 819 SLE patients who were positive for either antinuclear antibody or anti–double-stranded DNA at baseline screening found that IV belimumab at 10 mg/kg plus standard therapy resulted in a significantly greater SRI score (43.2%) than placebo (33.5%) at 1 year (those who received belimumab 1 mg/kg plus standard therapy had a 40.6% response rate). [118] Overall, the addition of belimumab to standard therapy reduced SLE disease activity and severe flares, and the medication was well tolerated. [118]

It is important to understand that osteoporosis has no symptoms. There is no pain in the bones where individuals with osteoporosis would hypothetically feel sore, so it is important to speak with your doctor to have a regular bone mass density test performed. Again, there is a high risk of osteoporosis for people with lupus because of often decreased physical activity, vitamin D deficiency, medication side effects, and the additional risk of kidney disease. Listed below are nutritional tips to follow:


Periodic follow-up and laboratory testing, including complete blood counts with differential, creatinine, and urinalyses, are imperative for detecting signs and symptoms of new organ-system involvement and for monitoring response and adverse reactions to therapies. At least quarterly visits are recommended in most cases. [151] Periodic complement levels and dsDNA titers may be used as adjuncts to clinical evaluation for detecting lupus flares.


In the absence of systemic lupus erythematosus, the most common reason for a positive ANA test is the presence of another connective tissue disease. Diseases that often are associated with a positive ANA test include Sjögren's syndrome (68 percent of affected patients), scleroderma (40 to 75 percent), rheumatoid arthritis (25 to 50 percent), and juvenile rheumatoid arthritis (16 percent).20 An ANA test also can be positive in patients with fibromyalgia. In patients with diseases other than systemic lupus erythematosus, ANA titers usually are lower, and the immunofluorescent pattern is different.20
(1) SOC; (2) SOC plus methotrexate (MTX); (3) SOC plus leflunomide (LFN); (4) SOC plus belimumab; (5) SOC plus abatacept (ABT); (6) other options: azathioprine (AZA), mycophenolate mofetil (MMF), cyclosporine A (CsA) or rituximab (RTX) (online supplementary tables S2.1.1, S2.1.4, S2.1.6, S2.1.7, S2.2.11, S2.1.11, S2.1.12, S2.1.14, S2.1.15, S2.1.17, S2.2.1, S2.2.2, S2.2.4, S3.1.1, S3.1.3–S3.1.6, S3.2.1, S3.2.2, S12.2–S12.5, S12.8–S12.10).
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.
Your gut is somewhat permeable to allow very small molecules (micronutrients) pass through the intestinal wall. It’s how you absorb your food. Many factors can damage your gut, including gluten, infections, medications and stress. This damage allows much larger particles such as toxins, microbes, and undigested food particles to “leak through” your gut and enter your bloodstream, triggering an immune response. We know from the research of Dr. Alessio Fasano that leaky gut is a necessary precursor to autoimmunity, meaning if you’re dealing with lupus, you also have a leaky gut. Not to mention, your gut is where nearly 80% of your immune system lives. So in order to overcome lupus, you must first repair your gut.
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.
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.
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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