Inflammation of the kidneys caused by an autoimmune disease called systemic lupus erythematosus. The condition can cause hematuria and proteinuria, and it may progress to end-stage renal disease. The most severe form of lupus nephritis, called diffuse proliferative nephritis, can cause scars to form in the kidneys. Scars are permanent, and kidney function often declines as more scars form. Early diagnosis and treatment may help prevent long-lasting damage.
Aggrecan is a type of protein known as a proteoglycan, which means it has several sugar molecules attached to it. It is the most abundant proteoglycan in cartilage, a tough, flexible tissue that makes up much of the skeleton during early development. Most cartilage is later converted to bone (a process called ossification), except for the cartilage that continues to cover and protect the ends of bones and is present in the nose, airways, and external ears. Aggrecan attaches to the other components of cartilage, organizing the network of molecules that gives cartilage its strength. These interactions occur at a specific region of the aggrecan protein called the C-type lectin domain (CLD). Because of the attached sugars, aggrecan attracts water molecules and gives cartilage its gel-like structure. This feature enables the cartilage to resist compression, protecting bones and joints. Although its role is unclear, aggrecan affects bone development.
(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).
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).  Overall, the addition of belimumab to standard therapy reduced SLE disease activity and severe flares, and the medication was well tolerated. 
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.
Symptoms vary but can include fatigue, joint pain, a red rash on the face (also called the "butterfly rash") and fever. These symptoms can periodically get worse (flare-up) and then improve. Lupus flares can range from mild to severe, often resulting in periods in which the disease is relatively quiescent. Currently, no cures exist for lupus, and treatment often involves corticosteroids, other immunosuppressants or organ transplants. But research is providing hope for better diagnosis, treatments and even cures.
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. Other genes which contain risk variants for SLE are IRF5, PTPN22, STAT4, CDKN1A, ITGAM, BLK, TNFSF4 and BANK1. Some of the susceptibility genes may be population specific.
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.
Administer angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor blockers (ARBs) to all patients with lupus nephritis (except pregnant women) who have proteinuria of 0.5 g or more per 24 hours (or equivalent by protein/creatinine ratios on spot urine tests).  This treatment has been reported to not only reduce proteinuria by about 30% but also significantly delay the doubling of serum creatinine and the progression to ESRD (in patients with nondiabetic chronic renal disease). 
Management of systemic lupus erythematosus (SLE) often depends on disease severity and disease manifestations,  although hydroxychloroquine has a central role for long-term treatment in all SLE patients. The LUMINA (Lupus in Minorities: Nature versus Nurture) study and other trials have offered evidence of a decrease in flares and prolonged life in patients given hydroxychloroquine, making it the cornerstone of SLE management. 
Blood clots are more common in people with lupus. Clots often occur in the legs (called deep venous thrombosis or DVT) and lungs (called pulmonary embolus or PE) and occasionally in the brain (stroke). Blood clots that develop in lupus patients may be associated with the production of antiphospholipid (APL) antibodies. These antibodies are abnormal proteins that may increase the tendency of the blood to clot. Blood can be tested for these antibodies.
If this disorder is suspected in people, brain scans are usually required for early detection. These scans can show localized areas of the brain where blood supply has not been adequate. The treatment plan for these people requires anticoagulation. Often, low-dose aspirin is prescribed for this purpose, although for cases involving thrombosis anticoagulants such as warfarin are used.
Systemic lupus erythematosus (S.L.E.), commonly called lupus, is a chronic autoimmune disorder that can affect virtually any organ of the body. In lupus, the body's immune system, which normally functions to protect against foreign invaders, becomes hyperactive, forming antibodies that attack normal tissues and organs, including the skin, joints, kidneys, brain, heart, lungs, and blood. Lupus is characterized by periods of illness, called flares, and periods of wellness, or remission.
Lupus is a chronic autoimmune condition in which the immune system attacks the body’s own healthy tissue and organs. Depending on the specific patient, lupus can cause high levels of persistent inflammation that can negatively affect various parts of the body. Lupus patients often experience tissue damage that affects the heart, joints, brain, kidneys, lungs and endocrine glands (such as the adrenals and thyroid gland). Although it’s not completely known why this happens, lupus risk factors are believed to include: (2)
The 19th century's research into lupus continued with the work of Sir William Osler who, in 1895, published the first of his three papers about the internal complications of erythema exudativum multiforme. Not all the patient cases in his paper had SLE but Osler's work expanded the knowledge of systemic diseases and documented extensive and critical visceral complications for several diseases including lupus. Noting that many people with lupus had a disease that not only affected the skin but many other organs in the body as well, Osler added the word "systemic" to the term lupus erythematosus to distinguish this type of disease from discoid lupus erythematosus. Osler's second paper noted that reoccurrence is a special feature of the disease and that attacks can be sustained for months or even years. Further study of the disease led to a third paper, published in 1903, documenting afflictions such as arthritis, pneumonia, the inability to form coherent ideas, delirium, and central nervous system damage as all affecting patients diagnosed with SLE.
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.
Inflammation of the heart muscle, usually in the U.S. as a consequence of infections (viruses, esp. coxsackie virus, and occasionally as a consequence of bacterial, protozoan or fungal infections); immunological-rheumatological conditions (e.g., systemic lupus erythematosus, ulcerative colitis, hypersensitivity reactions, or transplant rejection); exposure to chemicals or toxins (e.g., cocaine, doxorubicin, methamphetamine); nutritional or metabolic abnormalities (e.g., thiamine deficiency or hypophosphatemia); or radiation. Myocarditis also is occasionally found in pregnancy and with advanced age. The myocardium is infiltrated by leukocytyes, lymphocytes, and macrophages, leading to inflammation, necrosis of muscle cells, and fibrosis. Inflammatory damage to heart muscle fibers may resolve spontaneously or may cause progressive deterioration of the heart with pericarditis, arrhythmias, chronic dilated cardiomyopathy, and heart failure.
Along with nutritional deficiencies, steroid medications can cause significant weight gain and increased cholesterol, blood glucose, and triglycerides, further underscoring the need for patients with SLE who are taking these agents to follow a healthy diet to counter the effects.6 There are also specific things that individuals with SLE should avoid, including alfalfa sprouts and garlic, which can stimulate an already overactive immune system.7
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.  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. 
Today, physicians treat lupus using a wide variety of medicines, ranging in strength from mild to extremely strong. Prescribed medications will usually change during a person’s lifetime with lupus. However, it can take months—sometimes years—before your health care team finds just the right combination of medicines to keep your lupus symptoms under control.
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