Glossary of Commonly Used Terms in Auto-Immune Disorders

A:

Actemra; (Tocilizumab) is a biologic medication. It functions by blocking the effects of interleukin 6 a mediator of inflammation. Tocilizumab is given by IV infusion once every four weeks.

Adult Onset Still’s Disease; is a rare form of autoimmune inflammatory arthritis. It presents with high spiking fevers, joint and muscle pain, a salmon colored rash, and other symptoms of systemic inflammation. Its cause is unknown. The diagnosis is based on the presence of specific clinical features. There are no single blood tests that confirm the diagnosis.

Arcalyst; (Rilonacept) is an interleukin inhibitor. It works by binding to interleukin 1 preventing it from activating lymphocytes of the immune system. Rilonacept is given by intravenous infusion weekly.

ANA Test; measures the pattern and amount of autoimmune bodies (antibodies manufactured by the immune system directed against one or more of the individuals proteins). ANA titers of greater than 1:40 may be indicative of autoimmune disease.

Anemia; a condition in which the body does not have the normal levels of healthy red blood cells appropriate for one’s age and sex.

B:

Biologics; a class of medications used for the treatment of autoimmune diseases. Biologic medications are designed to inhibit specific components or actions of the immune system. These components (such as interleukins or capital TNF’s) have critical roles in activating and modulating the inflammatory process.

C:

Canikinumab; (Ilaris) is a biologic medication which is attaches to interleukin 1– B. Ilaris prevents interleukin 1-B from interacting on the interleukin 1 receptor site present on targeted cells of the immune system.

Carpal Ankylosis; a condition in which there is progressive loss of the articular cartilage on the surface of the carpal (wrist) bones resulting in pain and stiffness with motion or at rest. The condition can progress to carpal fusion, which is complete loss of the joint space between the carpal bones.

Cervical Ankylosis; a condition in which there is progressive loss of articular cartilage on the articular surfaces of the cervical vertebrae. This results in cervical arthritis with associated pain, stiffness, possible instability, and possibly nerve impairment

Cholestatic Hepatitis; is a condition in which there is an inability of the hepatocyte (liver cells) to actively secrete bile into the biliary system (bile ducts). The components of bile re-enter into the bloodstream resulting in symptoms that include jaundice and itching.

CRP; C–reactive protein is a protein found in blood that is synthesized by the liver. C–reactive protein levels in the blood increase in inflammatory conditions such as infections, malignancies, tissue injury, and autoimmune conditions.

D:

Dermatographism; is a disorder of the skin in which the skin becomes locally raised and inflamed when stroked, scratched, and/or rubbed. It is thought to result from a disorder of the mast cells with enhanced histamine release.

Diagnosis of Exclusion; a medical condition which is identified by a process of elimination, i.e. its presence cannot be established with complete confidence from physical examination or definitive testing.

DMARDS; (disease modifying anti-rheumatic drugs) are a class of drugs used to slow the progression of joint damage from rheumatoid arthritis. DMARD’S are also referred to as immunosuppressive drugs or slow acting anti-rheumatic drugs (SAARDS).

E:

Effusion; a condition in which excess amounts of food are accumulated between  like biologic layers, i.e., pleural effusion’s in the lung, synovial effusions in the knee, and\or pericardial effusions in the pericardium surrounding the heart

Enbrel; (Etanercept) is a biologic medication used to treat autoimmune diseases. It is classified as a TNF blocker. It is given by a self-administered injection weekly.

Erosive Arthritis; is a condition that results from uncontrolled inflammation involving the lining cells of the joint (synovial cells). This results in the enhanced loss of the cartilage surface which forms the specialized terminations of the bones comprising the joints.

Evanescent Rash; a skin rash present for a short duration, which leaves no permits in alteration

Etanercept; (see Enbrel)

F:

Fibromyalgia; a medical condition in which a person has long-term body wide pain and tenderness in the joints, muscles, tendons and other soft tissues. The cause is unknown. Diagnosis is based on clinical factors including three months of widespread pain and tenderness in at least 11 of 18 designated areas of the body.

G:

H:

Hemochromatosis; a medical condition that results in abnormal high levels of iron in the blood and organs. Hemochromatosis can result from a hereditary basis or arise as an acquired condition secondary to other causes. Hemochromatosis may result in liver failure, diabetes, heart disease, and/or arthritis.

Hemophagocytic Syndrome; (Hemophagocytic Lymphohistiocytosis, HLH) is a medical condition resulting from a disorder of the immune system that results in fever, swelling of the spleen and liver, swelling of the lymph nodes, jaundice, rash, and abnormal high levels of histiocytes (cell in the immune system that under normal conditions attack entities identified as foreign to the body). HLH effects, primarily young infants and children, although it can develop for the first time at any age. HLH may result from hereditary factors or develop secondary to an acquired condition.

Hepatic Enzymes; are proteins found in the liver. Their functions are to break down and use (metabolize) substances to prevent over accumulation in the body. The most common liver enzyme levels that are tested include GGT (gamma–glutamyl transferase), ALT/SGPT (alanine amino transferase), AST/SGOT (aspartate aminotransferase), ALP (alkaline phosphatase) abnormal liver function tests may result from primary liver injury and/or disease states that secondarily effect the liver.

Hepato-Cellular; the cells that collectively comprise the liver.

Hepatomegaly; enlargement of the liver. It is a nonspecific medical sign. It may result from many causes including infection, direct toxicity, malignancy primary or metastatic, metabolic disorder, and autoimmune disease.

HLA-DR4 Gene; is a human gene. The gene is present in the DNA of 60%-70% of those patients of European ancestry diagnosed with rheumatoid arthritis.

I:

Ilaris; (see Canakinumab)

IL-1; (interleukin 1) is a cytokine, a small protein molecule that is produced and released by specific cells of the immune system. Interleukin1 is released into the intracellular environment and binds to receptors sites on cells. Il-1 modulates the immune response. In disease states, levels of interleukin 1 are increased, resulting in fever, increased sensitivity to pain, and increased activity of white blood cells.

IL-6; (interleukin 6) is a cytokine, a small protein molecule formed and released by specific cells of the immune system. Interleukin 6 acts on specific cell receptors to modulate the immune response. The effects of Interleukin 6 include fever, production and enhanced white blood cells function.

Immunosuppressives; are any agent that suppresses the immune response of an individual. Diseases of auto immune origin are treated with immunosuppressive drugs.

Interleukin; (see IL6 and IL1)

Interleukin Inhibitors; are protein molecules either natural or constructed as pharmaceutical agents. They act to block the naturally occurring interleukins from attaching to their target cell receptors.

J:

Juvenile Rheumatoid Arthritis; a condition in which children up to the age of 16 experience inflammation of the joints resulting from an autoimmune process. Juvenile rheumatoid arthritis can occur in three patterns;

Systemic (Still’s disease) in which there is involvement of the at least one joint, multiple organ involvement including lymph nodes, and fever. This pattern may progress into adulthood.

Polyarticular which is characterized by multiple joint involvement. This includes five or more large and small joints of the legs, arms, neck and jaw. Progression into adulthood may occur.

Pauciarticular (oligo-articular) involves four or less joints. Most often, the wrists, knees, and possibly eyes.

K:

Kinaret; is a biologic medication used to treat autoimmune diseases. Its method of action is to bind to the Interleukin 1 type receptor on specific cell surfaces. It is self-administered once a day as a subcutaneous injection.

Koebner Phenomenon; a condition in which skin lesions appear on areas of minor skin trauma such as scratching. Koebner’s Phenomenon is seen in many conditions including; psoriasis, SLE, Still’s Disease, and Kaposi’s sarcoma.

L:

Leukocytosis; a situation in which there is an increase in the white blood cell count. It is a laboratory finding not a separate disease state. It results from multiple medical conditions including infections, stress, malignancy, and autoimmune diseases. Any or all of the cells of the immune blood cell system may be increased over normal values. Also more of the juvenile forms of the white blood cells will be present in the bloodstream. This is referred to as a shift to the left of the white blood cell population found in the bloodstream.

Lymphadenopathy; a condition in which the lymph nodes become enlarged. Underlying causes include infection, autoimmune disease, and malignancy.

M:

Macrophage Activation Syndrome; is a complicated condition that can arise in children affected with systemic inflammatory disorders. MAS results in excessive proliferation and activation of specific white blood cell types; T-lymphocytes and macrophages. MAS results in high fever, organ enlargement, anemia, and difficulty with coagulation of the blood. It is described as a secondary form of HLH (see Hemophagocytic Syndrome).

Maculo-Papular Rash; a skin rash characterized by a flat red area (macules) that is covered with small raised bumps (papules).

Methotrexate; is a medication classified as a DMARD. Methotrexate is used to treat multiple diseases including autoimmune conditions. It functions by inhibiting folic acid metabolism. It is taken by mouth. (see DMARD’S)

Muckle-Wells Syndrome; is a condition characterized by periodic episodes of skin rash, fever, joint pain, progressive hearing loss and kidney damage. These episodes begin during infancy and/or early childhood and continue through adulthood. Muckle-Wells syndrome is caused by a gene mutation resulting in the production of a defective version of cryopyrin. Cryopyrin is a protein molecule which is functions in the modulation of the inflammatory response.

Myalgia; is a symptom that corresponds to diffuse muscular pain. It may be related to local conditions involving the muscles or more frequently result from a systemic condition (involving the entire body).

N:

Neoplastic Disease; is the growth of an abnormal mass of cellular tissue. Neoplastic tissue is unregulated or lacking in coordination with the local normal tissue surrounding it.

NSAIDs; a class of medications that reduce the inflammatory reaction and do not contain steroids. The method of action is inhibition of the enzyme cyclooxygenase which are required for prostaglandin synthesis. NSAIDs are most often taken orally, but can be available as IV infusions, gels, or creams to be applied topically. NSAIDs use may result in side effects on multiple organ systems. On the gastrointestinal system NSAIDs can result in possible peptic ulcer formation. Examples of NSAIDs include Celebrex, Motrin, and Voltaren.

O:

Organomegaly; abnormal enlargement of an organ.

P:

Pauci-Articular Disease; (see juvenile rheumatoid arthritis, oligo-articular pattern)

Peri-capitate fusion; a condition resulting from the progressive and accelerated loss of articular cartilage on the surface of the capitate (the largest bone of the carpal bones forming the wrist) and its surrounding carpal bones resulting in pain and loss of motion at the wrist. Peri-capitate arthritis is the most common pattern of wrist arthritis seen in Adult Onset Still’s Disease.

Pericarditis; inflammation of the surface cells which form a sack like covering surrounding the heart. The condition produces chest pain and mechanically can limit the function of the heart. Pericarditis can result from a viral infection, kidney failure, autoimmune disease, tuberculosis, following a heart attack, direct chest injury, radiation, or unknown causes. Pericarditis can cause symptoms including chest pain and weakness related to mechanical failure of the heart.

Pleuritis (Pleural Involvement); inflammation of the surface cells, which form a sack like covering of the lungs. The condition produces chest pain worsened by breathing or coughing and can reduce the mechanical function of the lungs. Pleuritis can result from lung inflammation due to infections, exposure to asbestos, neoplasia, direct chest trauma, pulmonary embolus, and autoimmune diseases including rheumatoid arthritis and systemic lupus erythematosus

Pneumonitis (Pneumonic Involvement); inflammation of the lung tissue. It can result from infection, radiation therapy, inhalation of toxic gas, smoking, autoimmune diseases, and inhalation of molds. It can result in shortness of breath, cough, fatigue, loss of appetite, and weight loss.

Polymyalgia Rheumatica; and autoimmune condition effecting primarily adults over the age of 50. It can occur alone, with, or before giant cell arthritis (temporal arteritis). Giant cell arteritis is an inflammation of the arterial blood vessels of the head. Polymyalgia Rheumatica results in pain and stiffness of the shoulders, neck, and hips with associated findings of fatigue. It may also include symptoms such as anemia, loss of appetite, fever, depression, stroke, or sudden blindness.

Prodromal Sore Throat; inflammation in the pharyngeal area preceding the onset of systemic disease. It is usually not related to a productive discharge. Prodromal sore throat may last several days and lacks sensitivity to antibiotics. Prodromal sore throat may result from an autoimmune based inflammation of the lining cells of the back of the mouth and throat.

Q:

Quotidian Fever; a once daily fever of greater than 39°C (102.2°F) with intervening periods of no fever. Fever may occur at the same time each day and be preceded by shaking chills.

R:

Reticulo-Endothelial System; is an older term that refers to the portion of the immune system that consists of phagocytic cells (white blood cells that protect the body by ingesting potentially harmful foreign particles) and the reticular connective tissue that the cells inhabit. These structures include the spleen and lymph nodes.

Respiratory Distress; a medical condition in which one experiences difficulty in breathing, noted as shortness of breath. The condition can be acute or chronic. It can result from primary disease along such as emphysema, infection, and neoplasm. It can also result from a systemic condition such as sepsis and/or autoimmune diseases.

Rheumatic Fever; is an inflammatory disease that can develop after infection with a Group A streptococcal bacteria that initially resulted in strep throat or scarlet fever. Rheumatic fever can affect the heart, joints, skin and brain. It occurs primarily in children ages 5 to 15. Rheumatic fever is usually noted 14 to 28 days following the initial streptococcal infection.

Rheumatoid Factor Test; a blood study which quantitates the level of an antibody associated with the medical condition rheumatoid arthritis. The test will be positive in 70% to 80% of patients with rheumatoid arthritis. The test may also be positive in other conditions, including Systemic Lupus risk Erythematosus, Sjogren’s syndrome, hepatitis, and/or tuberculosis.

Rilonacept; (see Arcalyst)

S:

Salicylate Hepatotoxicity; damage that occurs to the cells of the liver secondary to the presence of aspirin (salicylates). Salicylate hepatotoxicity may occur in the lower levels of blood salicylate in autoimmune diseases, including juvenile rheumatoid arthritis, systemic lupus erythematosus, and/or Reiter’s Disease.

Schnitzler Syndrome; a medical condition characterized by chronic hives, fever, discomfort originating from bone, joint pain, weight loss, malaise, lymphadenopathy, and/or organomegaly. It is considered to be an autoimmune disorder related to faulty increase production of an abnormal protein from plasma cells (a cell line originating in the immune system).

SED Rate; is a blood test that measures how quickly red blood cells settle in a test tube in one hour. The faster the red blood cells settle to the bottom of the test to the higher the sedimentation rate is. High sedimentation rates are correlated with clinical conditions of inflammation which include infection, autoimmune disease, anemia, and/or pregnancy.

Serositis; a condition in which there is inflammation of the surface lining of the tissues. It may occur in individual organs alone or in all areas of the human body that have lining tissue including the lungs (pleuritis), the heart (pericarditis), and/or in the inner lining of the abdomen (peritonitis). It may occur in infection, autoimmune conditions; including Systemic Lupus Erythematosus, rheumatoid arthritis, juvenile rheumatoid arthritis and/or Adult Onset Still’s Disease

Serum S-100; a class of proteins that regulate intracellular processes as well as extracellular processes similar in activity to cytokines. To date, 20 proteins have been identified in this group. Each member of the S–100 protein family exhibits a unique expression pattern in normal cells. In disease states such as Alzheimer’s disease, melanoma, heart failure, systemic lupus erythematosus and/or rheumatoid arthritis there is a change in the pattern of S-100 proteins. This change can be detected by blood tests and indicate the presence and activity of the disease. Successful treatment of the disease can result in return of the S-100 protein levels to non-disease levels.

Splenomegaly; a condition in which there is enlargement of the spleen. It can be related to conditions, local to the spleen such as trauma, infection, neoplasia, secondary to systemic conditions such as metabolic disease, mononucleosis, and/or autoimmune diseases. Splenomegaly can result in symptoms such as chest pain, abdominal pain and/or anemia.

Systemic; a condition which is spread throughout the body, resulting in disruption and dysfunction of multiple organs and bodily functions.

T:

Tarsal Ankylosis; a condition in which the articular surfaces of the tarsal bones of the foot undergo accelerated erosion. It results in reduced motion, localized pain, and possible deformity. Tarsal Ankylosis can result from Adult Onset Still’s Disease, juvenile rheumatoid arthritis, and/or adult rheumatoid arthritis.

Tinnitus; is the perception of sound within the human ear in the absence of external sound. It is a condition that can result from a wide range of causes including neurological damage, recurrent ear infections, trauma to the head or ear, and/or side effects of medications (seen secondary to aspirin, antibiotics, and/or quinidine).

TNF’s; (tumor necrosis factors) a group of 19 identified cytokines (small cell–signaling protein molecules) that, when activated, can cause cell death, modulate the inflammatory response, inhibit tumor formation, and inhibit viral replication. TNF’s are produced by specialized cells of the immune system. TNF inhibitors such as Infliximab (Remicade), Adalimumab (Humira), and Enbrel (Etanercept) prevent binding of the TNF proteins to targeted cell receptors.

Z:

Zygoapophyseal Joints; the synovial lined space constituting the joint between the superior articular process of one vertebrae and the inferior articular process of the vertebrae below it. These joints are at every level of the vertebral column. These joints can undergo accelerated wear in autoimmune disease as a result of synovial hypertrophy and resultant inflammation. Degeneration of the zygoapophyseal joints of the vertebral column can result in pain, limited motion, instability, and possibly progress to loss of neurologic function. This process can occur at any level of the vertebral column, but is more common in the cervical and lumbar portions of the spine.

Harry Steinman, MD

© 2013

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