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.
Only one population-based screening study13 of systemic lupus erythematosus was identified. This study reported a prevalence of 200 cases per 100,000 women (18 to 65 years of age) in England. One review14 estimated the overall U.S. prevalence of definite systemic lupus erythematosus plus incomplete systemic lupus erythematosus (disease meeting only some diagnostic requirements for systemic lupus erythematosus) to be 40 to 50 cases per 100,000 persons.
It can be very scary to receive a lupus diagnosis, have your life disrupted and cause you to become uncertain about the future. The good news is that strides are continually being made in the discovery of better diagnostic tools and more effective medications. With the combination of correct treatment, medication, and living a healthy lifestyle, many people with lupus can look forward to a leading a long and productive life.
***Please note that this article is written for informational purposes only and should not be a substitute for professional medical advice or treatment. Do not delay seeking or disregard medical advice based on information here. Always seek the advice of your local family physician or other qualified health professional before starting any new treatment or making any changes to existing treatment. It is also advisable to consult a medical professional before making any changes to diet or starting alternative remedies, which may interact with other medications.***
Inflammation associated with lupus can cause stiffness, swelling, pain, and warmth of the joints, most commonly in the fingers, hands, elbows, ankles, and toes. (8) Most people with lupus will experience joint inflammation at some point, says Caricchio. For many people, joint pain is one of the first symptoms of the disease that they’ll notice and report.
The severity of lupus varies from mild to life threatening. Kidney problems and neurologic complications are more dangerous than the rashes, arthritis or other symptoms. After many years of having lupus, patients may develop hypertension (high blood pressure), accelerated atherosclerosis (plaque and fat build-up in the arteries), heart and lung diseases, kidney failure or osteoporosis. With proper treatment, the majority of people diagnosed with lupus have a normal life expectancy, but many will experience disabilities. Each patient will likely have his or her own specific pattern of symptoms and flares, but the disease can change over time.
Not necessarily. The antinuclear antibody (ANA) test is positive in most people who have lupus, but it also may be positive in many people who are healthy or have another autoimmune disease. Therefore, a positive ANA test alone is not adequate for the diagnosis of lupus. There must be at least three additional clinical features from the list of 11 features for the diagnosis to be made.
The panel judged the observed reduction in pregnancy loss with the addition of heparin to LDA as a large benefit. This intervention was not associated with significant harms. The addition of GCs or intravenous Ig to heparin plus LDA was associated with large harms (significant increase in premature delivery) without relevant benefits. Regarding heparin administration, the panel considered the reduction in pregnancy loss with low molecular weight heparin (LMWH) in comparison with unfractionated heparin (UFH) as a large benefit without significant adverse effects. No additional benefits were observed with LMWH-enoxaparin 80 mg compared with 40 mg.
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.
Neutrophils, 55% to 70% of all leukocytes, are the most numerous phagocytic cells and are a primary effector cell in inflammation. Eosinophils, 1% to 3% of total leukocytes, destroy parasites and are involved in allergic reactions. Basophils, less than 1% of all leukocytes, contain granules of histamine and heparin and are part of the inflammatory response to injury. Monocytes, 3% to 8% of all leukocytes, become macrophages and phagocytize pathogens and damaged cells, esp. in the tissue fluid. Lymphocytes, 20% to 35% of all leukocytes, have several functions: recognizing foreign antigens, producing antibodies, suppressing the immune response to prevent excess tissue damage, and becoming memory cells.
B cells are essential for the development and pathogenesis of both systemic and organ-specific autoimmune diseases. Autoreactive B cells are typically thought of as sources of autoantibody, but their most important pathogenetic roles may be to present autoantigens to T cells and to secrete proinflammatory cytokines. A rate-limiting step in the genesis of autoimmunity then is the activation of autoreactive B cells. Here, mechanisms are discussed that normally prevent such activation and how they break down during disease. Integrating classic work with recent insights, emphasis is placed on efforts to pinpoint the precursor cells for autoantibody-secreting cells and the unique stimuli and pathways by which they are activated.
Steroids Synthetic cortisone medications are some of the most effective treatments for reducing the swelling, warmth, pain, and tenderness associated with the inflammation of lupus. Cortisone usually works quickly to relieve these symptoms. However, cortisone can also cause many unwelcome side effects, so it is usually prescribed only when other medications—specifically NSAIDs and anti-malarials—are not sufficient enough to control lupus.
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.”
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).
Dozens of medications have been reported to trigger SLE. However, more than 90% of cases of "drug-induced lupus" occurs as a side effect of one of the following six drugs: hydralazine (Apresoline) is used for high blood pressure; quinidine (Quinidine Gluconate, Quinidine Sulfate) and procainamide (Pronestyl; Procan-SR; Procanbid) are used for abnormal heart rhythms; phenytoin (Dilantin) is used for epilepsy; isoniazid (Nydrazid, Laniazid) is used for tuberculosis; and d-penicillamine (used for rheumatoid arthritis
A substance (generally a protein, polypeptide, or peptide) that stimulates the differentiation, division, development, and maintenance of cells and the tissues they make up. Growth factors are signaling molecules released by certain groups of cells, e.g., lymphocytes, to influence the activities of other cells. Growth factors can be divided into families, e.g., platelet-derived GFs, transforming GFs, and angiogenic GFs. They are released normally during fetal and embryonic development, wound healing, and tissue maturation. Massive releases of GFs are characteristic of some types of cancer cells. Artificial GFs, e.g., granulocyte colony-stimulating factor, are used in health care to restore depressed levels of cells to normal values, e.g., in patients who have received chemotherapy.
In healthy people, eosinophils comprise approximately 1 to 6 percent of white blood cells. The body may produce more of these cells in response to parasitic and fungal infections. Certain allergic diseases, skin conditions, autoimmune disorders, cancers, and bone marrow diseases also may result in elevated eosinophil counts. Many people with eosinophilic disorders have high numbers of eosinophils in their blood or tissues over a long period of time. Sometimes, the presence of excess eosinophils in tissue, called “eosinophilic inflammation,” can result in tissue damage.
SLE is an autoimmune disease involving multiple organ systems, a clinical pattern of flares and remissions, and the presence of anti-nuclear autoantibodies. Whereas early symptoms most frequently involve the skin and joints, disease morbidity and mortality are usually associated with cardiovascular events and damage to major organs, particularly the kidneys. Many of the current therapeutic options are considered to be inadequate because of toxicities, accrual of organ damage, and insufficient control of the underlying disease pathology. Improved understanding of SLE pathogenesis and immunology has led to the identification of new treatment targets. Current interest is mainly focused on the targeted immunosuppressive actions provided by biologic therapy. Although the potential long-term beneficial or harmful effects of the new molecular treatments are unclear, their precise molecular targeting may reveal key relationships within the immune system and advance the cause of individualized molecular medicine.
Another new B-cell-suppressing treatment is belimumab (Benlysta). Belimumab blocks the stimulation of the B cells (a B-lymphocyte stimulator or BLyS-specific inhibitor) and is approved for the treatment of adults with active autoantibody-positive systemic lupus erythematosus who are receiving standard therapy. It is important to note that the efficacy of belimumab has not been evaluated in patients with severe active lupus nephritis or severe active central nervous system lupus. Belimumab has not been studied in combination with other biologic therapies or intravenous cyclophosphamide.
Lupus, a chronic autoimmune disorder that causes inflammation, creates a wide range of signs and symptoms. Systemic lupus erythematosus, the most common form of the condition, can potentially involve any major organ system of the body, says Neil Kramer, MD, co-medical director at the Institute for Rheumatic and Autoimmune Diseases at Overlook Medical Center in Summit, New Jersey. “Therefore, the first signs and symptoms vary from patient to patient.”
Cognitive impairment is defined as any difficulty with normal thought functions or processes, such as thinking, learning, or remembering. Cognitive impairment can occur as a neurological symptom of lupus. However, cognitive impairment is a common occurrence that happens to everyone at some time, not just lupus patients, and it can affect one single thought process or many thought processes either temporarily or permanently.
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.
Heart and Lungs. Heart and lung involvement often is caused by inflammation of the covering of the heart (pericardium) and lungs (pleura). When these structures become inflamed, patients may develop chest pain, irregular heartbeat, and accumulation of fluid around the lungs (pleuritis or pleurisy) and heart (pericarditis). The heart valves and the lung itself can also be affected by lupus, resulting in shortness of breath.
In addition to the oral antimalarial hydroxychloroquine, doctors may prescribe topical steroids for lupus rash. Steroids or antimalarials may also be injected directly into rash lesions. (8) Topical creams containing tacrolimus or pimecrolimus that modulate the skin’s immune response may help manage lupus rash. Oral thalidomide, which affects the immune response, may be prescribed if other therapies don’t work. Doctors may also recommend that people with lupus rash avoid the sun and other ultraviolet light sources and wear sunscreen.
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