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Glossary

UNDERSTANDING LUPUS
—TERMINOLOGY AND PROCESSES

Lupus is one of the most complex diseases for scientists to decode and for patients to comprehend. The Alliance for Lupus Research (ALR) understands that many people —especially those newly diagnosed with the disease — grapple with the words and terms used to describe lupus and its often multipart and varied manifestations. This is why we have compiled the following glossary of terms associated with lupus. Many of these terms and processes are used to describe the scientific investigations that are funded by the ALR in its quest to find a cure the disease.

Acute Cutaneous Lupus (ACLE)

Acute cutaneous lupus (ACLE) is one of three subsets of the general lupus skin disorder called cutaneous lupus erythematosus.

This condition occurs when lupus is active and is generally characterized by rash and lesions, often localized across the face in a butterfly pattern.  However, ACLE can appear on other parts of the body, as well.  ACLE usually occurs as a result of exposure to ultraviolet light.  Without predictability, symptoms may come and go and severity varies greatly from person to person.

AMP – Accelerating Medicines Partnership
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.
AMP will begin with three to five year pilot projects in three disease areas:
      Alzheimer’s disease
      type 2 diabetes
      autoimmune disorders of rheumatoid arthritis and systemic lupus erythematosus (lupus)

source: NIH
Anti-Cardiolipin Antibodies (ACA)

Anti-cardiolipin antibodies (ACA) are antibodies that are present in autoimmune diseases like lupus to directly attack cardiolipin, a protein that is very common in the body.  As the name implies, cardiolipin is found mostly in the heart, and secondly in skeletal muscles.

Much remains unclear about the presence of ACA.  Possible risks for pathological conditions in patients with lupus include:  thrombosis (clotting), fetal loss, and thrombocytopenia (low platelets) – yet, ACA are also present in individuals with no apparent ill effects.  It appears that more research is needed to grasp a better understanding of this antibody.

Anti-inflammatory

The term anti-inflammatory refers to an agent or mechanism that counteracts or suppresses inflammation. 

Anti-inflammatory drugs help to reduce the inflammatory response and are the most common type of medication used to treat the pain and discomfort of people living with lupus.

Anti-inflammatories are divided into two categories—non-steroidal anti-inflammatory drugs (NSAIDs) and corticosteroids or steroids.

Anti-RNP antibodies

Ribonucleprotein (RNP) is a protein that is structurally associated with DNA.  Anti-RNP antibodies are autoantibodies that are associated with lupus and are detected in nearly 40 percent of lupus patients.  Anti-RNP is also closely associated with mixed connective tissue disease (MCTD), which features signs and symptoms similar to those of lupus. 

RNP is a factor in both lupus and MCTD, which leads to an abnormal immune reaction causing inflammation of and damage to various body parts and can affect joins, skin, kidneys, heart, lungs, blood vessels, and the brain.

Anti-SM

Anti-SM are antibodies closely associated with lupus.  Nearly all individuals with anti-SM antibodies have lupus—but only 20 percent of people with lupus have these antibodies.  Anti-SM is rarely found in people with other rheumatic diseases and its incidence in healthy individuals is less than 1 percent.  The presence of anti-SM is an indication of systemic lupus and is a factor in diagnosing the disease.

Antibody

Antibodies are the cells and molecules of the immune system that detect and attack invaders.  In diseases like lupus, antibodies mistakenly attack a patient’s own tissue and organs.

Anticoagulant

Anticoagulants are substances that hinder blood from clotting by suppressing coagulation.  Among their many benefits, anticoagulants are used in preventing stroke and thrombosis (formation of a blood clot in the heart).  They are also used in drawing blood and flushing catheters. 

Anticoagulant therapy is used to treat lupus patients.  About one-third of people with the disease have antibodies to molecules that make them susceptible to blood clots.

Antigen

In immunology, an antigen is a substance that evokes the production of one or more antibodies. Each antibody binds to a specific antigen by way of an interaction similar to the fit between a lock and a key. The substance may be from the external environment or formed within the body. The immune system will try to destroy or neutralize any antigen that is recognized as a foreign and potentially harmful invader. The term originally came from antibody generator[1][2] and was a molecule that binds specifically to an antibody, but the term now also refers to any molecule or molecular fragment that can be bound by a major histocompatibility complex (MHC) and presented to a T-cell receptor.[3] "Self" antigens are usually tolerated by the immune system, whereas "non-self" antigens can be identified as invaders and can be attacked by the immune system.

Antinuclear antibodies

Antinuclear antibodies are antibodies that target proteins within the nuclei of cells.  In autoimmune diseases like lupus, antinuclear antibodies can signal the body to begin attacking itself.

Most people have autoantibodies, but the presence of large amounts of them may indicate an autoimmune disease.  There is a test that measures pattern and amount of autoantibodies.  More than 95 percent of people with lupus test positive.

Antiphospholipid antibodies

Antiphospholipid antibodies (APLAs) are a type of protein that usually helps the body ward off infection.  However, in lupus, APLAs mistakenly attack phospholipids, which is a type of fat found in in all living cells, including blood and the lining of blood vessels.

APLAs can interfere with blood vessels’ normal function, potentially altering clotting and leading to strokes, blood clots, miscarriages, and low platelet counts.

APLAs are present in about one-third of people with SLE.

Apoptosis

Apoptosis is the natural process of cell death, which is a normal function of the body.  When cells die, the debris is cleared by macrophages, or white blood cells within tissues.  In people with lupus, however, apoptosis may play a role in disease manifestation.

Lupus patients may not be able to effectively clear apoptotic cells, which can evoke the production of cytokines.  The cytokines cause B-cells to release antibodies—and in lupus, antibodies mistakenly attack healthy tissue and organs.

Arthritis

 

Arthritis is inflammation of one or more joints  and it involves the breakdown of cartilage. Cartilage normally protects a joint, allowing it to move smoothly. Cartilage also absorbs shock when pressure is placed on the joint, such as when you walk. Without the normal amount of cartilage, the bones rub together, causing pain, swelling (inflammation), and stiffness.

Usually the joint inflammation goes away after the cause goes away or is treated. Sometimes it does not. When this happens, you have chronic arthritis. Arthritis may occur in men or women. Osteoarthritis is the most common type.

Source NIH.gov

Assays

An assay is an investigative (analytic) procedure in laboratory medicine, pharmacology, environmental biology, and molecular biology for qualitatively assessing or quantitatively measuring the presence or amount or the functional activity of a target entity (the analyte) which can be a drug or biochemical substance or a cell in an organism or organic sample.

Autoantibody

An autoantibody is an antibody that works against one’s own tissues or cells.

Autoimmune disease

An illness that occurs when the body tissues are attacked by its own immune system. The immune system is a complex organization within the body that is designed normally to "seek and destroy" invaders of the body, including infectious agents. Patients with autoimmune diseases frequently have unusual antibodies circulating in their blood that target their own body tissues.

Autoimmunity

 

Autoimmunity is the failure of an organism in recognizing its own constituent parts as self, which allows an immune response against its own cells and tissues. Any disease that results from such an aberrant immune response is termed an autoimmune disease. Autoimmunity is often caused by a lack of germ development of a target body and as such the immune response acts against its own cells and tissues. Prominent examples include Celiac disease, diabetes mellitus type 1 (IDDM), Sarcoidosis, systemic lupus erythematosus (SLE), Sjögren's syndrome, Churg-Strauss Syndrome, Hashimoto's thyroiditis, Graves' disease, idiopathic thrombocytopenic purpura, Addison's Disease, rheumatoid arthritis (RA) and allergies. Autoimmune diseases are very often treated with steroids.

Source: Wikipedia.org

Autoreactive B cells

B cells are a type of white blood cell that helps the body fight infection.  In lupus this process malfunctions, causing the immune system to attack healthy tissue.  Therefore, B cells are essential for the development and pathogenesis of autoimmune disease.

Autoreactive B cells are generally considered to be the source of autoantibodies, causing the body to secrete pro-inflammatory cytokines.

B cells

Like T cells, B cells are a type of blood cell that belong to a group of white blood cells (WBCs) called lymphocytes, which help the body fight infection.
 
When B cells encounter an antigen (infection or a foreign body), they become activated and produce molecules called antibodies that attach to the surface of the infectious agent.  These antibodies either kill the infection-causing organism or make it vulnerable to attack by other WBCs.  This process guards the body against infection.

These vital cells are called B cells because they are created in bone marrow.  Once matured, B cells are present in the blood and lymph nodes. 

The function of B cells and T cells is vital to the immune system, allowing humans to ward off and better cope with often-hostile bacteria, viruses, and other foreign matter.

B-lymphocyte stimulator

Also known as B-cell activating factor, or BAFF, B-lymphocyte stimulator (BLyS) is a cytokine that is vital for the survival of B cells.  High levels or overproduction of BLyS is linked to lupus.

The BLyS protein is expressed by a wide variety of cell types, including monocytes, activated neutrophils, and T cells.

BAFF

Is the acronym for the term B-cell activating factor.

Belimumab

Belimumab, commonly known as Benlysta, is the first new drug therapy to be approved by the FDA in more than 50 years.  Belimumab is a human antibody that inhibits B-cell activating factor (BAFF). 

Significant decreases in the number of activated B cells have been observed in some patients.  However, in testing, the drug was not effective with all types of lupus and showed little benefit among African Americans.  More testing is planned for the more severe cases of lupus, including people with kidney and brain damage. Belimumab holds the promise of reduced disease manifestation and the sparing use of corticosteroid.

Biologic

Biologic is a process or phenomenon connected with life or living organisms.

Source: Wikipedia.org

Biomarkers

Biomarkers are essentially proteins, enzymes, or other molecules in the body that can be used as tracers of diagnosis or indicators of disease activity.  In the case of lupus, immune cells that are either more common in —or exclusive to — patients with lupus produce biomarkers. 

Today, scientists are looking to at biomarkers as early predictors of lupus flares.  Biomarkers also hold the promise of enhancing diagnostic accuracy, prognosis, and monitoring of treatment response.

BLyS-specific inhibitors

BLyS-specific inhibitors are a class of medications, such as belimumab, that prevent BLyS proteins from stimulating the growth of auto-reactive B cells.

Butterfly rash

Butterfly rash is a red flat facial rash involving the malar region bilaterally and the bridge of the nose. The presence of a butterfly rash is generally a sign of lupus erythematosus (LE), but it can also include a plethora of conditions. The case presented here is of a female with butterfly rash along with typical bright red discoloration of gingiva. The clinical, histopathological and biochemical investigations suggested the presence of rosacea.

Source: NIH.gov

Chronic Cutaneous Lupus (CCLE)

Chronic cutaneous lupus (CCLE) is one of three subsets of the general lupus skin disorder called cutaneous lupus erythematosus. 

The most common type of CCLE is discoid lupus, a painful chronic skin condition characterized by red, inflamed disc-like patches that have a scaling, crusty appearance.  Discoid lupus usually occurs on the scalp, face, and neck.  The condition can strike at all ages and among all ethnic groups.

Chronic disease

Chronic diseases are noncommunicable illnesses that are prolonged in duration, do not resolve spontaneously, and are rarely cured completely.

Although chronic diseases are more common among older adults, they affect people of all ages and are now recognized as a leading health concern of the nation. Growing evidence indicates that a comprehensive approach to prevention can save tremendous costs and needless suffering.

Source: CDC.gov

Clinical trial

Clinical trial is a prospective biomedical or behavioral research study of human subjects that is designed to answer specific questions about biomedical or behavioral interventions (drugs, treatments, devices, or new ways of using known drugs, treatments, or devices). Clinical trials are used to determine whether new biomedical or behavioral interventions are safe, efficacious, and effective.

Source: NIH.gov

Cohorts

A group of people used in a study who have something (such as lupus) in common.

Connective tissue

Connective tissue is the material inside your body that supports many of its parts. It is the "cellular glue" that gives your tissues their shape and helps keep them strong. It also helps some of your tissues do their work. Cartilage and fat are examples of connective tissue.

There are over 200 disorders that impact connective tissue. Some, like cellulitis, are the result of an infection. Injuries can cause connective tissue disorders, such as scars. Others, such as Ehlers-Danlos syndrome, Marfan syndrome, and osteogenesis imperfecta, are genetic. Still others, like scleroderma, have no known cause. Each disorder has its own symptoms and needs different treatment.

Source: NIH.gov

Corticosteroids

Corticosteroids are drugs that closely resemble cortisol, a hormone that is naturally produced in the adrenal glands.

In one of the most common treatments for lupus, corticosteroids—or steroids as they are more commonly termed—are used to reduce the production of inflammation and reduce the activity of the immune system in order to minimize tissue damage.  A physician’s decision to prescribe steroids is based on the potential benefits and risks, which vary from patient to patient.

CRP (C-Reactive Protein)

CRP is a blood protein that is produced by the liver.  High levels of CRP are caused by inflammation and infections.  Levels of CRP can dramatically elevate in a period of hours and are usually present in people with SLE.  Measuring the amount of CRP in the body is a screen for infectious and inflammatory diseases like lupus.

Cutaneous lupus erythematosus

Lupus is a serious disease that can affect multiple organs and systems of the body — including the skin, which may lead to eruptions of hives and lesions.  There are three lupus-specific lesions and non-specific lesions — and cutaneous lupus may be divided into three categories:  chronic cutaneous lupus (CCLE), subacute cutaneous lupus (SCLE) and acute cutaneous lupus (ACLE).  All three subsets are aggravated by exposure to sunlight.

 

  • Chronic cutaneous lupus (CCLE):  The most common form of CCLE is discoid lupus.  It is a painful chronic skin condition characterized by red, inflamed disc-like patches that have a scaling, crusty appearance.  Discoid lupus usually occurs on the scalp, face, and neck.  The condition can strike at any age among people of all ethnic groups.

Discoid lupus lesions can be induced or exacerbated by exposure to the sun.  Early diagnosis and treatment and precautionary measures greatly improve outcomes.  Untreated, discoid lupus can lead to scarring and permanent hair loss.

 

  • Sub-acute cutaneous lupus erythematosus (SCLE):  Characterized by lesions that appear on parts of the body that are exposed to sun, SCLE makes up approximately 10 percent of all lupus cases.  Lesions from SCLE do not cause permanent scarring.

 

  • Acute cutaneous lupus (ACLE):  This skin condition occurs when lupus is active and is generally characterized by rash and lesions.  Often localized across the bridge of the nose and spreading to both sides of the face, it appears in a butterfly pattern.

But ACLE is not restricted to the face.  It can appear on other parts of the body, as well.  ACLE usually occurs as a result of exposure to ultraviolet light.

Cyclophosphamide

Cyclophosphamide is an immunosupressive medication used to treat cancer, severe SLE and lupus nephritis.  In lupus, cyclophosphamide is used when other drugs prove ineffective or are too invasive.  However, cyclophosphamide can cause an adverse drug reactions.

Cytokines

Cytokines are chemicals produced by T-cells in response to the presence of an antigen.  Cytokines cause B-cells to release antibodies.

Dendritic cells

Dendritic cells play a critical role in the regulation of the immune response in humans.  They are antigen-presenting cells, meaning that their main function is to process antigen material and present it on the surface of other cells of the immune system.

These cells can be found in tissue that is exposed to the external environment, such as skin, the inner lining of the nose, lungs, stomach, and intestines.

The part that dendritic cells play in the manifestation of lupus is uncertain, which is why the ALR has funded several studies to grasp a better understanding of how these cells work.

Discoid lupus

Discoid lupus is the most common manifestation of the skin disorder chronic cutaneous lupus.  It is characterized characterized by red, inflamed disc-like patches that have a scaling, crusty appearance.  Discoid lupus usually occurs on the scalp, face, and neck.  The condition can strike at any age among people of all ethnic groups.

Discoid lupus lesions can be induced or exacerbated by exposure to the sun.  Early diagnosis and treatment and precautionary measures greatly improve outcomes.  Untreated, discoid lupus can lead to scarring and permanent hair loss.

Discoid rash

Discoid rash is red, scaly patches on skin that cause scarring.

Source: Wikipedia.org

Diuretic

Diuretics are sometimes called “water pills” because they increase the rate of the body’s urine output.  They are prescribed to treat congestive heart failure, high blood pressure, edema (water retention), and certain kinds of kidney or liver disease. 

For lupus patients, diuretics are used to treat nephritis, or inflammation of the kidneys.

Drug-Induced Lupus (DIL)

Drug-induced lupus (DIL) is an autoimmune disorder similar to systemic lupus erythematosus (SLE).  As with SLE, the body mistakenly attacks healthy tissue.  DIL is caused by an overreaction to a medication.

People with DIL may have symptoms that affect the joints, heart, and lungs.  Lupus nephritis and other symptoms associated with SLE are rare in people with DIL.

Once the medication that caused DIL is stopped, symptoms may go away over time.  Treatment usually involves nonsteroidal anti-inflammatory drugs, corticosteroids, or antimalarial drugs.

Ex Vivo

Means that which takes place outside an organism. In science, ex vivo refers to experimentation or measurements done in or on tissue from an organism in an external environment with the minimum alteration of natural conditions. Ex vivo conditions allow experimentation on an organisms cells or tissue under more controlled conditions than is possible in in vivo experiments (in the intact organism), at the expense of altering the "natural" environment.

Examples of ex vivo specimen use include: assays; realistic models for surgical procedure development; investigations into the interaction of different energy types with tissues;

FDA

The FDA (or Food and Drug Administration) is an agency of the United States Department of Health and Human Services, one of the United States federal executive departments. The FDA is responsible for protecting and promoting public health through the regulation and supervision of food safety, tobacco products, dietary supplements, prescription and over-the-counter pharmaceutical drugs (medications), vaccines, biopharmaceuticals, blood transfusions, medical devices, electromagnetic radiation emitting devices (ERED), and veterinary products.

Source: Wikipedia.org

Fibromyalgia

Fibromyalgia is a disorder of unknown etiology characterized by widespread pain, abnormal pain processing, sleep disturbance, fatigue and often psychological distress. People with fibromyalgia may also have other symptoms; such as

  • Headaches
  • Sleep disturbances
  • Cognitive problems with thinking and memory (sometimes called "fibro fog")
  • Problems with thinking and memory (sometimes called "fibro fog")

Source: CDC.gov

Flares

Flares can refer to either the recurrence of symptoms or an onset of more severe symptoms.

Function of B-cells and T-cells

B-cells and T-cells work together.  When a T-cell recognizes an antigen, it will produce chemicals known as cytokines that cause B-cells to multiply and release many immune proteins (antibodies).  Circulating widely in the bloodstream, antibodies recognize foreign particles and trigger inflammation to help the body rid itself of the virus or bacteria.

B-cells and T-cells play a vital role in protecting the immune system.

Glomerulonephritis

 

Glomerulonephritis is a type of kidney disease in which the part of your kidneys that helps filter waste and fluids from the blood is damaged.

Damage to the glomeruli causes blood and protein to be lost in the urine.

A quarter of people with chronic glomerulonephritis have no history of kidney disease.

Source: NIH.gov

Hematologic disorders

 

Hematolic disorders affect one or more parts of the blood and prevent your blood from doing its job. They can be acute or chronic. Many blood disorders are inherited. Other causes include other diseases, side effects of medicines, and a lack of certain nutrients in your diet.

Types of blood disorders include

  • Platelet disorders, excessive clotting, and bleeding problems, which affect how your blood clots
  • Anemia, which happens when your blood does not carry enough oxygen to the rest of your body
  • Cancers of the blood, such as leukemia and myeloma
  • Eosinophilic disorders, which are problems with one type of white blood cell.

Source: NIH.gov

IgG (Immunoglobulin G)

IgG is a type of antibody.  Found in all body fluids — and the main antibody found in blood — IgG protects against bacterial and viral infections.

Measuring immunoglobulin levels is useful in evaluating patients for autoimmune conditions such as lupus, rheumatoid arthritis, and celiac disease.

Immune complex

Immune complex occurs when an antibody binds to an antigen, such as toxins, microorganisms, and proteins that are foreign to the body.  Immune complex molecules help fight disease—but in lupus, individuals form autoantibodies that attack the person’s own tissue and organs.

Immune system

The human immune system is a complicated organization of cells and pathways within the body that seeks and responds to foreign invaders such as bacteria, viruses, and other infectious agents — as well as foreign material such as pollen.  Composed of numerous and varied cells—each with a specific task — the immune system is the body’s defense mechanism.

Immunologic disorder

 

An immunologic disorder is a dysfunction of the immune system. These disorders can be characterized in several different ways:

  • By the component(s) of the immune system affected
  • By whether the immune system is overactive or underactive
  • By whether the condition is congenital or acquired

It has been suggested that most people have at least one primary immunodeficiency. Due to redundancies in the immune system, though, many of these are never detected.

Source: Wikipedia.org

Immunomodulation

Alteration of the immune system or of an immune response by agents that activate or suppress its function.

Immunosuppressive

Immunosuppressive is a term that refers to the lowering of the body’s normal immune response.  It can occur naturally through the aging process, as the side effect of medical therapy, or it can be deliberately induced to treat diseases like lupus.

In lupus, immunosuppressive drugs are sometimes used to address the hyperactivity of the immune system, which triggers the inflammatory response and can cause severe pain in the joints, skin, and kidneys of patients with the disease.

Immunosuppressive drugs are also used to prevent the body from rejecting a transplanted organ.  Along the same lines, this therapy is used in lupus to prevent the from body attacking or rejecting healthy tissue.

Inflammation

 

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.

Source: NIH.gov

Interferon

Interferons (IFNs) are proteins made and released by host cells in response to the presence of pathogens such as viruses, bacteria, parasites or tumor cells. They allow for communication between cells to trigger the protective defenses of the immune system that eradicate pathogens or tumors.

Interferon Regulatory Factor 5 (IRF 5)

IRF5 is one of the interferon regulatory factors, a group of transcription factors with diverse roles within the body.  Among its many functions, IRF-5 influences virus-mediated activation of interferon, the modulation of cell growth, differentiation, apoptosis, and immune system activity.

It acts as a molecular switch that controls whether macrophages will promote or inhibit inflammation.  The ALR has funded investigations to better grasp an understanding of RF5 in regard to lupus manifestation.

Intravenous (IV) infusion

Intravenous infusion or IV infusion is a medical term that describes the way certain kinds of medicines or other substances, such as nutrition, are delivered to the body.  This method is often used when the substance cannot be taken orally.  Sometimes the therapy must bypass the gut or go directly into the veins.

Many therapies are delivered via IV infusion—including intravenous steroids and chemotherapeutic agents for people with lupus.

(JAK) Janus Kinase Inhibitors

Janus kinase inhibitors also known as JAK inhibitors are a type of medication that functions by inhibiting the activity of one or more of the Janus kinase family of enzymes (JAK1, JAK2, JAK3, TYK2), thereby interfering with the JAK-STAT signaling pathway. These inhibitors have therapeutic application in the treatment of cancer and inflammatory diseases.

Kinase inhibitor

A protein kinase inhibitor is a type of enzyme inhibitor that blocks the action of one or more protein kinases. Protein kinases are enzymes that add a phosphate (PO4) group to a protein or other organic molecule. Phosphate groups can turn a protein off.

Lesion

A lesion is any abnormality in the tissue of an organism (in layman's terms, "damage"), usually caused by disease or trauma. Lesion is derived from the Latin word laesio which means injury.

Source: Wikipedia.org

Leukopenia

Leukopenia is an abnormal reduction of circulating white blood cells, especially the granulocytes. The term leukopenia is often used interchangeably with neutropenia. It may result from reduced production of white blood cells or increased utilization and destruction, or both. Infection, drugs, malignancy, megaloblastosis, hypersplenism and immunoneutropenia are responsible for most cases of neutropenia. Primary neutropenia is very rare. Sometimes, particularly in children, primary neutropenia is hereditary and may be associated with other developmental defects. The major danger of neutropenia is the risk of infection. Management requires identification of the cause and effective antimicrobial therapy, especially when serious systemic infection is present.        

Source: NIH.gov

Lupus

Lupus is one of many disorders of the immune system known as autoimmune diseases. In autoimmune diseases, the immune system turns against parts of the body it is designed to protect. This leads to inflammation and damage to various body tissues. Lupus can affect many parts of the body, including the joints, skin, kidneys, heart, lungs, blood vessels, and brain. Although people with the disease may have many different symptoms, some of the most common ones include extreme fatigue, painful or swollen joints (arthritis), unexplained fever, skin rashes, and kidney problems.

Lymph nodes

Lymph nodes are small organs of the immune system spread throughout the body.  They behave as filters or traps for foreign particles.  Lymph nodes contain cells that become lymphocytes and are important in the proper functioning of the immune system. 

Lymphocytes

A lymphocyte is a type of white blood cell that is responsible for immune responses.

Macrophages

The role of microphages is not completely known, but scientific research has shown that these cells attack foreign substances and infectious microbes through destruction and ingestion.  Derived from Greek, macrophages translates as “big eaters.”

Recent research suggests that macrophages in people with lupus may have intrinsic defects that hamper its normal function.

MicroRNAs

Found in plants and mammals, microRNAs (miRNA) are small RNA molecules, which increase or decrease the production of specific gene protein or RNA.  Aberrant expression of miRNA has been seen in numerous disease states.

Mixed connective tissue disease

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.

Source: NIH.gov

Monoclonal antibodies

Naturally occurring in the human body, antibodies are a “sticky” protein that targets a specific antigen. These antibodies circulate in the body, find and attach to the antigen, and recruit other parts of the immune system to destroy the cells containing the antigen.

A monoclonal antibody is a man-made, laboratory-produced protein that is designed to target a certain antigen.  The advantage of infection-fighting proteins is that they are highly specific, identical, and can be produced in large quantities.

Monoclonal antibodies are now used as therapies for a number of diseases.

Myositis

 

Myositis is inflammation of your skeletal muscles, which are also called the voluntary muscles. These are the muscles you consciously control that help you move your body. An injury, infection or autoimmune disease can cause myositis.

The diseases dermatomyositis and polymyositis both involve myositis. Polymyositis causes muscle weakness, usually in the muscles closest to the trunk of your body. Dermatomyositis causes muscle weakness, plus a skin rash. Both diseases are usually treated with prednisone, a steroid medicine, and sometimes other medicines

Sources: NIH.gov

Neonatal lupus

Neonatal lupus is a rare condition – about 1 to 2 percent of babies born to mothers who have autoimmune disease or carry the antibodies to SSA/Ro and/or SSB/La [1,2] will develop neonatal lupus.  The disease is presumed to be the result of these antibodies crossing the placenta in pregnancy from mother to her developing baby.

Neonatal lupus is usually benign and the signs — such as skin rash, liver problems, and low blood cell counts —are generally short lived.  Symptoms usually disappear within months after birth, when infants begin to develop their own immune system — and, the mother’s antibodies leave the baby’s body.

Nephritis

Lupus nephritis is kidney inflammation caused by systemic lupus erythematosus (SLE or lupus). SLE is an autoimmune disease—a disorder in which the body’s immune system attacks the body’s own cells and organs. Up to 60 percent of people with SLE are diagnosed with lupus nephritis, which can lead to significant illness and even death.

Source: NIH.gov

NETosis

When, in the living body of a plant or animal, NETs are released during a form of pathogen-induced cell death.

Neurologic disorders

Neurologic disorders are diseases of the brain, spinal cord and nerves throughout your body. Together they control all the workings of the body. When something goes wrong with a part of your nervous system, you can have trouble moving, speaking, swallowing, breathing or learning. You can also have problems with your memory, senses or mood.

There are more than 600 neurologic diseases. Major types include

  • Diseases caused by faulty genes, such as Huntington's disease and muscular dystrophy
  • Problems with the way the nervous system develops, such as spina bifida
  • Degenerative diseases, where nerve cells are damaged or die, such as Parkinson's disease and Alzheimer's disease
  • Diseases of the blood vessels that supply the brain, such as stroke
  • Injuries to the spinal cord and brain
  • Seizure disorders, such as epilepsy
  • Cancer, such as brain tumors
  • infections, such as meningitis

Source: NIH.gov

Neuropathy

 

Peripheral neuropathy describes damage to the peripheral nervous system, the vast communications network that transmits information from the brain and spinal cord (the central nervous system) to every other part of the body. Peripheral nerves also send sensory information back to the brain and spinal cord, such as a message that the feet are cold or a finger is burned. Damage to the peripheral nervous system interferes with these vital connections. Like static on a telephone line, peripheral neuropathy distorts and sometimes interrupts messages between the brain and the rest of the body.

Source: NIH.gov

Neutrophil extracellular traps (NETs)

Neutrophil extracellular traps (NETs) are chromatin structures loaded with antimicrobial molecules. They can trap and kill various bacterial, fungal and protozoal pathogens, and their release is one of the first lines of defense against pathogens.

Neutrophils

The most abundant type of white blood cells in humans, neutrophils are important for the defense against infection.  Neutrophils are fast acting, arriving at the site of infection within an hour.  They can ingest and kill bacteria and are the main component of pus.

More than 50 percent of all white blood cells occurring in the human body are neutrophils.

With ALR funding Michael Denny, PhD, at Temple University in Philadelphia, PA, worked to broaden the understanding of the relationship between neutrophils and lupus.  Dr. Denny and his team identified an abnormal pool of neutrophils in people with the disease.  “These cells may contribute to disease manifestation like skin rashes,” explained Dr. Denny.

Non-Steroidal Anti-Inflammatory Drugs (NSAIDs)

The muscle and joint pain associated with lupus—as well as other chronic conditions where inflammation is present—are most often treated with non-steroidal anti-inflammatory drugs (NSAIDs).  Examples of over-the-counter NSAIDs are aspirin and ibuprofen.

NSAIDs work by preventing an enzyme called cyclooxygenase from functioning.  This enzyme protects the stomach lining, which is why NSAIDs can cause stomach upset and bleeding.

Nucleic Acid Binding Polymers (NABPs)

Nucleic acids are molecules that are essential to the structure of DNA, RNA, and all forms of life.  A polymer is a large molecule composed of many smaller molecules lined together.

NABPs inhibit toll-like receptor (TLRs) activation and subsequent cytokine production of dendritic cells and B cells.  In lupus, TLRs play a critical role in innate and adaptive immune responses by responding to pathogenic nucleic acids.

With funding from the Alliance for Lupus Research, David Pisetsky, MD, PhD, from Duke University Medical Center, NC is looking at ways that NABPs can be used in lupus treatment. “These polymers can bind tightly to DNA and RNA and prevent the formation of immune complexes and the resulting stimulation of internal receptors. The hope is that the polymers will be able to block the ability of extracellular nucleic acids to stimulate immune system activity that can underlie autoimmunity,” said Dr. Pisetsky.

Oral ulcers

Oral ulcers are sores or open lesions in the mouth.

Source: NIH.gov

Peer Review

Pericardial effusion

 

Pericardial effusion is the buildup of fluid in the sac

Source: NIH.gov

Pericarditis

Pericarditis is a condition in which the sac-like covering around the heart (pericardium) becomes inflamed. The cause of pericarditis is often unknown or unproven, but is often the result of an infection such as:

  • Viral infections that cause a chest cold or pneumonia, such as the echovirus or coxsackie virus (which are common in children), as well as influenza
  • Infections with bacteria (much less common) and some fungal infections (even more rare)

Other causes include:

  • Heart attack
  • Heart surgery or trauma to the chest, esophagus, or heart
  • Certain medications and some drugs used to treat cancer or suppress the immune system
  • Swelling or inflammation of the heart muscle

Source: NIH.gov

PGA

PGA, short for “Physician’s Global Assessment,” is a term that refers to the scale of a patient’s well being based on the impression of the clinician.  Ratings are made on a scale from 0 to 3, measuring the severity of disease (0 = inactive, 1 = mild, 2 = moderate, 3 = severe).

Phenotype

The term phenotype refers to the physical or biochemical characteristics of an organism as determined by its genetic makeup.

Phlebitis

Phlebitis is an inflammation of a vein, usually in the legs.

When phlebitis is associated with the formation of blood clots (thrombosis), usually in the deep veins of the legs, the condition is called thrombophlebitis. These clots can travel to the lungs, causing pulmonary embolisms that can be fatal.

Source: Wikipedia.org

Photosensitivity

Photosensitivity is an abnormal reaction to sunlight

Source: NIH.gov

Pleiotropy

The phenomenon of one gene being responsible for or affecting more than one phenotypic characteristic.

Pleural effusion

A pleural effusion is a buildup of fluid between the layers of tissue that line the lungs and chest cavity.

Your body produces pleural fluid in small amounts to lubricate the surfaces of the pleura, the thin tissue that lines the chest cavity and surrounds the lungs. A pleural effusion is an abnormal, excessive collection of this fluid.

There are two different types:

  • Transudative pleural effusions are caused by fluid leaking into the pleural space. This is caused by increased pressure in the blood vessels or a low blood protein count. Congestive heart failure is the most common cause.
  • Exudative effusions are caused by blocked blood vessels or lymph vessels, inflammation, lung injury, and tumors.

Source: NIH.gov

Pleurisy

 

Pleurisy is inflammation of the lining of the lungs and chest (the pleura) that leads to chest pain (usually sharp) when you take a breath or cough.

Pleurisy may develop when you have lung inflammation due to infections such as pneumonia or tuberculosis. This inflammation also causes the sharp chest pain of pleurisy.

It may also occur with:

  • Asbestos-related disease
  • Certain cancers
  • Chest trauma
  • Pulmonary embolus
  • Rheumatoid arthritis
  • Lupus

Source: NIH.gov

Proliferative lupus nephritis

The term for severe stages of lupus nephritis — or inflammation of the kidneys due to lupus – is proliferative lupus nephritis.  Lupus nephritis is a serious condition, which occurs when autoantibodies affect the filtering structure of the kidneys.  Proliferative lupus nephritis may lead to blood or protein in the urine, impaired kidney function, or even kidney failure.

At any stage of lupus nephritis, early diagnosis and treatment can greatly improve outcomes.

Proteinuria

 

Proteinuria-also called albuminuria or urine albumin-is a condition in which urine contains an abnormal amount of protein. Albumin is the main protein in the blood. Proteins are the building blocks for all body parts, including muscles, bones, hair, and nails. Proteins in the blood also perform a number of important functions. They protect the body from infection, help blood clot, and keep the right amount of fluid circulating throughout the body.

Proteinuria is a sign of chronic kidney disease (CKD). If CKD progresses, it can lead to end-stage renal disease (ESRD), when the kidneys fail completely. A person with ESRD must receive a kidney transplant or regular blood-cleansing treatments called dialysis.

Sources: NIH.gov

Pulmonary hypertension
 

Pulmonary hypertension occurs when the blood pressure in the pulmonary arteries, which carry oxygen and blood from the heart to the lungs, is much higher than normal.

  • Pulmonary hypertension usually occurs along with another disease or condition, such as pregnancy, heart and blood vessel diseases, lung diseases, liver diseases, sleep apnea, connective tissue diseases such as lupus and scleroderma, thyroid diseases, HIV infection, or use of certain diet medicines or illicit drugs.
  • In 2002, pulmonary hypertension led to 15,668 deaths and 260,000 hospital visits in the United States.
  • Pulmonary hypertension can affect men and women of all ages and racial/ethnic groups. However, the majority of people who have this condition are older women.

Source: CDC.gov

Raynaud's phenomenon

Raynaud's Phenomenon is a rare disorder that affects the arteries. Arteries are blood vessels that carry blood from your heart to different parts of your body.

Raynaud's sometimes is called a disease, syndrome, or phenomenon. The disorder is marked by brief episodes of vasospasm (VA-so-spazm), which is a narrowing of the blood vessels.

Vasospasm of the arteries reduces blood flow to the fingers and toes. In people who have Raynaud's, the disorder usually affects the fingers. In about 40 percent of people who have Raynaud's, it affects the toes. Rarely, the disorder affects the nose, ears, nipples, and lips.

Source: NIH.gov

Renal disorder

 

Renal disorder, or nephropathy, means damage to or disease of a kidney. Nephrosis is non-inflammatory nephropathy. Nephritis is inflammatory kidney disease.

Source: Wikipedia.org

Rheumatism

Rheumatism or rheumatic disorder is a non-specific term for medical problems affecting the joints and connective tissue. The study of, and therapeutic interventions in, such disorders is called rheumatology.

Source: Wikipedia.org

Rheumatoid arthritis

 

Rheumatoid arthritis (RA) is a form of arthritis that causes pain, swelling, stiffness and loss of function in your joints. It can affect any joint but is common in the wrist and fingers. You might have the disease for only a short time, or symptoms might come and go. The severe form can last a lifetime.

Rheumatoid arthritis is different from osteoarthritis, the common arthritis that often comes with older age. RA can affect body parts besides joints, such as your eyes, mouth and lungs.

No one knows what causes rheumatoid arthritis. Genes, environment and hormones might contribute. Treatments include medicine, lifestyle changes and surgery.

Source: NIH.gov

Scleroderma


Scleroderma
is a connective tissue disease that involves changes in the skin, blood vessels, muscles, and internal organs. It is a type of autoimmune disorder, a condition that occurs when the immune system mistakenly attacks and destroys healthy body tissue.

The cause of scleroderma is unknown. People with this condition have a buildup of a substance called collagen in the skin and other organs. This buildup leads to the symptoms of the disease.

The disease usually affects people 30 to 50 years old. Women get scleroderma more often than men do. Some people with scleroderma have a history of being around silica dust and polyvinyl chloride, but most do not.

Widespread scleroderma can occur with other autoimmune diseases, including systemic lupus erythematosus and polymyositis. In such cases, the disorder is referred to as mixed connective disease.

Source: NIH.gov

Serositis

Serositis refers to inflammation of the serous tissues of the body, the tissues lining the lungs (pleura), heart (pericardium), and the inner lining of the abdomen (peritoneum) and organs within. It is commonly found with fat wrapping or creeping fat.

Source: Wikipedia.org

Sjogren's syndrome

Sjogren's syndrome is a disease that causes dryness in your mouth and eyes. It can also lead to dryness in other places that need moisture, such as your nose, throat and skin. Most people who get Sjogren's syndrome are older than 40. Nine of 10 are women. Sjogren's syndrome is sometimes linked to rheumatic problems such as rheumatoid arthritis.

In Sjogren's syndrome, your immune system attacks the glands that make tears and saliva. It may also affect your joints, lungs, kidneys, blood vessels, digestive organs and nerves. The main symptoms are:

  • Dry eyes
  • Dry mouth

Treatment focuses on relieving symptoms.

Source: NIH.gov

SLEDAI

A disease activity index for lupus patients- Find here

Steroids

Steroids is the shortened term for corticosteroids, a drug commonly used to reduce inflammation in lupus.  The term steroids is not to be confused with the male hormone-related steroid compounds designed to enhance athletic performance.

Stratification

Classification of a mass of data (obtained from research or survey) into categories and sub-categories on the basis of one or more chosen criteria.

Sub-acute Cutaneous Lupus Erythematosus (SCLE)

Sub-acute cutaneous lupus (SCLE) is one of three subsets of the general lupus skin disorder called cutaneous lupus erythematosus. 

Characterized by lesions that appear on parts of the body that are exposed to sun, SCLE makes up approximately 10 percent of lupus cases.  Lesions from SCLE do not cause permanent scarring.

Synovitis


 

Synovitis is the medical term for inflammation of the synovial membrane. This membrane lines joints which possess cavities, known as synovial joints. The condition is usually painful, particularly when the joint is moved. The joint usually swells due to synovial fluid collection.

Synovitis may occur in association with arthritis as well as lupus, gout, and other conditions. Synovitis is more commonly found in rheumatoid arthritis than in other forms of arthritis, and can thus serve as a distinguishing factor, although it can be present to a lesser degree in osteoarthritis. Long term occurrence of synovitis can result in degeneration of the joint.

Source: Wikipedia.org

Systemic

Systemic refers to something that is spread throughout, system-wide, affecting a group or system such as a body, economy, market or society as a whole.

Source: Wikipedia.org

T cells

T cells are a type of blood cell that belong to a group of white blood cells (WBCs) called lymphocytes, which help the body fight infection.  T cells play a major role in protecting the immune system by identifying, directly attacking, and destroying infectious agents. 

Like B cells, T cells are produced in bone marrow.  But unlike B cells, they mature and develop in the thymus, an organ in the chest — hence the name, T cells.  When fully matured, T cells are present in the blood and lymph nodes.

The function of B cells and T cells is vital to the immune system, allowing humans to ward off and better cope with often-hostile bacteria, viruses, and other foreign matter.

T Follicular Helper cells (TFH)

T follicular helper cells (TFH) are a type of helper T cells that direct the development of antibodies. TFH cells normally function
 to direct B cells to make antibodies against pathogens.

However, an increase in their number or function is associated with lupus, and their accelerated development or persistence is believed to drive the production of those dangerous autoantibodies.  The ALR is currently funding a study to better understand the function of TFH.

Tolerogenic

Capable of producing immunological tolerance.

Toll-Like Receptors

Toll- like receptors (TLR) play a critical role in early immune response to invading pathogens.  The signaling by TLRs result in a variety of cellular responses including the production of interferons and pro-inflammatory cytokines that direct immune response.

Tregs

Playing a unique role in the immune system, Tregs — or Regulatory T cells — are crucial in suppressing aberrant pathological immune responses in autoimmune diseases like lupus.  These cells help the immune system from attacking healthy tissue.

Acting as a system of checks and balances, Tregs prevent excessive reactions within the body.

Undifferentiated connective tissue diseases

The term undifferentiated connective tissue diseases is used to define conditions characterized by the presence of signs and symptoms suggestive of a systemic autoimmune disease that do not satisfy the classificative criteria for defined connective tissue diseases (CTD) such as systemic lupus erythematosus (SLE), Sjögren's syndrome (SS), rheumatoid arthritis (RA) and others. A small percentage of patients presenting with an undifferentiated profile will develop during the first year follow up of a full blown CTD, however an average of 75% will maintain an undifferentiated clinical course. These patients may be defined as having a stable undifferentiated connective tissue diseases (UCTD). The most characteristic symptoms of UCTD are represented by arthritis and arthralgias, Raynaud's phenomenon, leukopenia, while neurological and kidney involvement are virtually absent. Eighty percent of these patients have a single autoantibody specificity, more frequently anti-Ro and anti-RNP antibodies. Stable UCTD are considered as distinct clinical entities and therefore it has been proposed to define those conditions as UCTD. Classificative criteria have also been proposed and a work to better define them is still under way.

Source: NIH.gov

Uremia

Uremia is a term used to loosely describe the illness accompanying kidney failure (also called renal failure), in particular the nitrogenous waste products associated with the failure of this organ.This is not to be confused with uricemia, or hyperuricemia, a build up of uric acid in the blood.

In kidney failure, urea and other waste products, which are normally excreted into the urine, are retained in the blood. Early symptoms include anorexia and lethargy, and late symptoms can include decreased mental acuity and coma. Other symptoms include fatigue, nausea, vomiting, cold, bone pain, itch, shortness of breath, and seizures. It is usually diagnosed in kidney dialysis patients when the glomerular filtration rate, a measure of kidney function, is below 50% of normal.

Source: Wikipedia.org

Vasculitis

Vasculitis is a condition that involves inflammation in the blood vessels. The condition occurs if your immune system attacks your blood vessels by mistake. This may happen as the result of an infection, a medicine, or another disease or condition.

Vasculitis can affect any of the body's blood vessels. These include arteries, veins, and capillaries. Arteries carry blood from your heart to your body's organs. Veins carry blood from your organs and limbs back to your heart. Capillaries connect the small arteries and veins.

Source: NIH.org


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