Limitations of the test: Like CRP, the ESR is not specific to lupus. Because there are many causes for a positive result, including infection, the test is not diagnostic for lupus. Nor can it distinguish a lupus flare from an infection. Also, the level doesn't directly correlate with lupus disease activity. So it isn't necessarily useful for monitoring disease activity.
The clearance of early apoptotic cells is an important function in multicellular organisms. It leads to a progression of the apoptosis process and finally to secondary necrosis of the cells if this ability is disturbed. Necrotic cells release nuclear fragments as potential autoantigens, as well as internal danger signals, inducing maturation of dendritic cells (DCs), since they have lost their membranes' integrity. Increased appearance of apoptotic cells also stimulates inefficient clearance. That leads to maturation of DCs and also to the presentation of intracellular antigens of late apoptotic or secondary necrotic cells, via MHC molecules. Autoimmunity possibly results by the extended exposure to nuclear and intracellular autoantigens derived from late apoptotic and secondary necrotic cells. B and T cell tolerance for apoptotic cells is abrogated, and the lymphocytes get activated by these autoantigens; inflammation and the production of autoantibodies by plasma cells is initiated. A clearance deficiency in the skin for apoptotic cells has also been observed in people with cutaneous lupus erythematosus (CLE).[67]
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]
Intravenous immunoglobulins may be used to control SLE with organ involvement, or vasculitis. It is believed that they reduce antibody production or promote the clearance of immune complexes from the body, even though their mechanism of action is not well understood.[87] Unlike immunosuppressives and corticosteroids, IVIGs do not suppress the immune system, so there is less risk of serious infections with these drugs.[88]
A nonspecific laboratory test used as a marker of inflammation. In this test, the speed at which erythrocytes settle out of unclotted blood is measured. Blood to which an anticoagulant has been added is placed in a long, narrow tube, and the distance the red cells fall in 1 hr is the erythrocyte sedimentation rate (ESR). Normally it is less than 10 mm/hr in men and slightly higher in women. The speed at which the cells settle depends on how many red blood cells clump together. Clumping is increased by the presence of acute-phase proteins released during inflammation.
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.

Jump up ^ Johanneson, Bo; Lima, Guadalupe; von Salomé, Jenny; Alarcón-Segovia, Donato; Alarcón-Riquelme, Marta E.; Collaborative Group on the Genetics of SLE, The BIOMED II Collaboration on the Genetics of SLE and Sjögrens syndrome (2002-11-01). "A major susceptibility locus for systemic lupus erythemathosus maps to chromosome 1q31". American Journal of Human Genetics. 71 (5): 1060–1071. doi:10.1086/344289. ISSN 0002-9297. PMC 385085. PMID 12373647.
In addition to the 11 criteria, other tests can be helpful in evaluating people with SLE to determine the severity of organ involvement. These include routine testing of the blood to detect inflammation (for example, the erythrocyte sedimentation rate, or ESR, and the C-reactive protein, or CRP), blood-chemistry testing, direct analysis of internal body fluids, and tissue biopsies. Abnormalities in body fluids (joint or cerebrospinal fluid) and tissue samples (kidney biopsy, skin biopsy, and nerve biopsy) can further support the diagnosis of SLE. The appropriate testing procedures are selected for the patient individually by the doctor.
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).
The aim of this review is to provide an up-to-date overview of treatment approaches for systemic lupus erythematosus (SLE), highlighting the multiplicity and heterogeneity of clinical symptoms that underlie therapeutic decisions. Discussion will focus on the spectrum of currently available therapies, their mechanisms and associated side-effects. Finally, recent developments with biologic treatments including rituximab, epratuzumab, tumor necrosis factor (TNF) inhibitors, and belimumab, will be discussed.

Although it is known that chronically low complement levels and functional asplenia may result in a low level of susceptibility to infection, it is not known to what degree. [128, 129] Overall, it is likely that the primary reason patients with SLE die of infections is immunosuppressive medications.Stress-dose steroid protocols should be used in patients who are receiving maintenance corticosteroids when they are admitted with infectious or perioperative stress.

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.
A biopsy is a procedure that removes a small piece of living tissue from your body. The tissue is examined with a microscope for signs of damage or disease. Biopsies can be done on all parts of the body. A biopsy is the only test that can tell for sure if a suspicious area is cancer. But biopsies are performed for many other reasons too. There are different ways to do a biopsy. A needle biopsy removes tissue with a needle passed through your skin to the site of the problem. Other kinds of biopsies require surgery.
In recent years, mycophenolate mofetil (CellCept) has been used as an effective medication for lupus, particularly when it is associated with kidney disease. CellCept has been helpful in reversing active lupus kidney disease (lupus renal disease) and in maintaining remission after it is established. Its lower side-effect profile has advantage over traditional immune-suppression medications.
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.

Antibodies produced by a single clone of cells; A type of protein made in the laboratory that can bind to substances in the body, including cancer cells. There are many kinds of monoclonal antibodies. A monoclonal antibody is made so that it binds to only one substance. Monoclonal antibodies are being used to treat some types of cancer. They can be used alone or to carry drugs, toxins, or radioactive substances directly to cancer cells.

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 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]
Because the antibodies and accompanying cells of inflammation can affect tissues anywhere in the body, lupus has the potential to affect a variety of areas. Sometimes lupus can cause disease of the skin, heart, lungs, kidneys, joints, and/or nervous system. When only the skin is involved by rash, the condition is called lupus dermatitis or cutaneous lupus erythematosus. A form of lupus dermatitis that can be isolated to the skin, without internal disease, is called discoid lupus erythematosus. When internal organs are involved, the condition is referred to as systemic lupus erythematosus (SLE).
In addition to hormonal mechanisms, specific genetic influences found on the X chromosome may also contribute to the development of SLE. Studies indicate that the X chromosome can determine the levels of sex hormones. A study has shown an association between Klinefelter syndrome and SLE. XXY males with SLE have an abnormal X–Y translocation resulting in the partial triplication of the PAR1 gene region.[104]
Corticosteroids. Prednisone and other types of corticosteroids can counter the inflammation of lupus. High doses of steroids such as methylprednisolone (A-Methapred, Medrol) are often used to control serious disease that involves the kidneys and brain. Side effects include weight gain, easy bruising, thinning bones (osteoporosis), high blood pressure, diabetes and increased risk of infection. The risk of side effects increases with higher doses and longer term therapy.

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