Materiały źródłowe

• #1 Amyloidosis – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/amyloidosis/symptoms-causes/syc-20353178

  There are many different types of amyloidosis. Some types are hereditary. Others are caused by outside factors, such as inflammatory diseases or long-term dialysis. Many types affect multiple organs. Others affect only one part of the body. […] AL amyloidosis (immunoglobulin light chain amyloidosis). This is the most common type of amyloidosis in developed countries. AL amyloidosis is also called primary amyloidosis. It usually affects the heart, kidneys, liver and nerves. […] AA amyloidosis. This type is also known as secondary amyloidosis. It’s usually triggered by an inflammatory disease, such as rheumatoid arthritis. It most commonly affects the kidneys, liver and spleen. […] Hereditary amyloidosis (familial amyloidosis). This inherited disorder often affects the nerves, heart and kidneys. It most commonly happens when a protein made by your liver is abnormal. This protein is called transthyretin (TTR).

• #2 Amyloidosis: Symptoms, Types & Treatments

  https://my.clevelandclinic.org/health/diseases/23398-amyloidosis

  Amyloidosis happens when proteins in your body become misshapen and sticky. They make clumps, or fibrils, that land on organs and tissues. Healthcare providers call amyloidosis a protein misfolding disorder. Instead of being neat, long chains that your body can break down, the fibrils get stuck in organs and cause problems. […] Causes of amyloidosis include: Gene changes (mutations). You may inherit a change (mutation) in the gene that makes abnormal amyloid proteins. These mutations can happen during a persons lifetime for unknown reasons. […] Underlying conditions. Amyloidosis may happen because of a separate condition. This is the case with AA amyloidosis. Its more common in people with conditions that cause long-term inflammation, like long-term infections or rheumatoid arthritis.

• #3 Amyloidosis: Causes, Types, Symptoms, Diagnosis, Treatment, and Prognosis

  https://www.webmd.com/cancer/lymphoma/amyloidosis-symptoms-causes-treatments

  Amyloidosis can happen without any known cause, or it can be caused by another disease, such as cancer. Amyloidosis can also be inherited genetically. […] Many different proteins can lead to amyloid deposits, but only a few have been linked to major health problems. […] AL amyloidosis (immunoglobulin light chain amyloidosis). This is the most common type and used to be called primary amyloidosis. AL stands for „amyloid light chains,” which is the type of protein responsible for the condition. Theres no known cause, but it happens when your bone marrow makes abnormal antibodies that cant be broken down. Its linked to a blood cancer called multiple myeloma. […] AA amyloidosis. Previously known as secondary amyloidosis, this condition is the result of another chronic infectious or inflammatory disease such as rheumatoid arthritis, Crohns disease, or ulcerative colitis.

• #4 AL Amyloidosis (Primary Amyloidosis): Symptoms, Treatment & What it Is

  https://my.clevelandclinic.org/health/diseases/15718-amyloidosis-al-amyloid-light-chain

  AL amyloidosis happens when plasma cells making antibodies made of heavy and light protein chains make too many light protein chains. The light chains misfold and clump together, making amyloid fibrils that end up in your organs. […] AL amyloidosis is a plasma cell disorder. Plasma cells are part of your immune system. They make antibodies that fight infection. […] AL amyloidosis typically affects your heart and/or your kidneys, but it may affect your stomach, intestine, nerves and skin. […] AL amyloidosis happens when a single plasma cell makes a clone that begins to multiply uncontrollably.

• #5 4 Things to Know About AL Amyloidosis Causes, Diagnosis & Treatment

  https://www.jnj.com/health-and-wellness/things-we-now-know-about-al-amyloidosis

  AL amyloidosis is caused by an overproduction of protein chains by the immune system. […] In people with AL amyloidosis, however, an increased number of abnormal plasma cells grow in the bone marrow and produce an excess of one part of the immunoglobin, called light chains. These immunoglobin light chains undergo abnormal folding into a form called amyloid. It is the amyloid that deposits in tissues and organs throughout the body. […] When it comes to risk factors, AL amyloidosis is not hereditary. But about 15% of patients with a blood cancer called multiple myeloma will also be diagnosed with AL amyloidosis like AL amyloidosis, multiple myeloma affects the plasma cells inside the bone marrow. […] While there’s still a great deal to learn about AL amyloidosis, research is underway to further advance understanding of the disease and how it’s managed, says Dr. Weiss, adding that there is reason to be hopeful. The traditional treatment for AL amyloidosis has been chemotherapy, but new immunotherapies that specifically target the plasma cells, as well as the amyloid deposits, are being studied as possible treatments for the rare disease.

• #6 AL Amyloidosis | Amyloidosis Foundation

  https://amyloidosis.org/facts/al

  AL amyloidosis is caused by a bone marrow disorder. […] For AL amyloidosis, it is the light chains that become misfolded, and the abnormal, misfolded result is the forming of amyloid. […] While the abnormal light chain is responsible for the deposits and organ damage in the disease, it is believed that the plasma cell they come from is the real underlying cause of disease. […] AL amyloidosis is not contagious, is not secondary to other diseases, and is not considered to be hereditary. […] However, there are very rare families that have an increased incidence of blood diseases including myeloma, lymphoma, MGUS (monoclonal gammopathy of undetermined significance), and AL amyloidosis. […] Approximately 15% of those diagnosed with multiple myeloma will also acquire AL amyloidosis. […] When these light chain proteins assemble incorrectly, they are called abnormal, or misfolded, protein.

• #7 Amyloidosis – Leukaemia Foundation

  https://www.leukaemia.org.au/blood-cancer/types-of-blood-cancer/amyloidosis/

  Amyloidosis is the general term given to a relatively rare and serious group of disorders in which an abnormal protein known as amyloid is produced. […] At this time it is not known what triggers the initial formation of the amyloid protein and why this happens in such a small proportion of the population. Amyloidosis can be acquired (occurs spontaneously) or hereditary (occurs due to a faulty gene). […] Research is going on all the time into possible causes of amyloidosis and certain factors have been identified that may put some people at an increased risk. These include: patients with myeloma may also develop AL amyloidosis (15-20% of myeloma patients), patients with a long-term chronic infectious or inflammatory disease are at risk of developing AA amyloidosis, people who inherit a certain gene mutation may develop hereditary amyloidosis, patients who require kidney dialysis for a long period of time may be at increased risk of dialysis-associated amyloidosis, although this is rare with modern dialysis techniques.

• #8 Secondary systemic amyloidosis: MedlinePlus Medical EncyclopediaLock

  https://medlineplus.gov/ency/article/000585.htm

  Secondary systemic amyloidosis is a disorder in which abnormal proteins build up in tissues and organs. Clumps of the abnormal proteins are called amyloid deposits. […] Secondary means it occurs because of another disease or situation. For example, this condition usually occurs due to long-term (chronic) infection or inflammation. […] The exact cause of secondary systemic amyloidosis is unknown. You are more likely to develop secondary systemic amyloidosis if you have a long-term infection or inflammation. […] This condition may occur with: Ankylosing spondylitis — a form of arthritis that mostly affects the bones and joints in the spine, Bronchiectasis — a disease in which the large airways in the lungs are damaged by chronic infection, Chronic osteomyelitis — a long-standing bone infection, Cystic fibrosis — a genetic disease that causes thick, sticky mucus to build up in the lungs, digestive tract, and other areas of the body, leading to chronic infection of the lungs, Familial Mediterranean fever — an inherited disorder of repeated fevers and inflammation that often affects the lining of the abdomen, chest, or joints, Gout, Hairy cell leukemia — a type of blood cancer, Hodgkin disease — cancer of the lymph tissue, Inflammatory bowel disease, Juvenile idiopathic arthritis — arthritis that affects children, Multiple myeloma — a type of blood cancer, Psoriatic arthritis, Reactive arthritis — a group of conditions that causes swelling and inflammation of the joints, eyes, and urinary and genital systems, Rheumatoid arthritis, Systemic lupus erythematosus — an autoimmune disorder, Tuberculosis, Vasculitis (inflammation of the blood vessels).

• #9 AA amyloidosis: Causes and diagnosis – UpToDate

  https://www.uptodate.com/contents/causes-and-diagnosis-of-aa-amyloidosis-and-relation-to-rheumatic-diseases

  AA amyloidosis (previously known as secondary [AA] amyloidosis) is a disorder characterized by the extracellular tissue deposition of fibrils that are composed of fragments of and/or intact serum amyloid A protein (SAA), a hepatic acute phase reactant. AA amyloidosis may complicate any chronic inflammatory condition, including rheumatoid arthritis (RA), juvenile idiopathic arthritis (JIA), ankylosing spondylitis (AS), inflammatory bowel disease, familial periodic fever syndromes, chronic infections, and certain neoplasms (table 1). […] Multiple chronic inflammatory conditions, among them rheumatologic, autoinflammatory, chronic infectious, and other disorders, have been associated with the development of AA amyloid. The most common organ system involved in this form of amyloidosis is the kidney, although other organ systems are often also affected.

• #10 AA (Inflammatory) Amyloidosis: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/335559-overview

  Amyloidosis comprises a heterogeneous group of diseases in which normally soluble plasma proteins are deposited in the extracellular space in an abnormal, insoluble, fibrillar form. […] AA amyloidosis occurs in the course of chronic inflammatory diseases (infectious and noninfectious), hereditary periodic fevers, and with certain neoplasms, such as Hodgkin lymphoma and renal cell carcinoma. […] A wide range of infectious and noninfectious diseases, hereditary periodic fevers, immune deficiencies, and neoplasms have been associated with AA amyloidosis. […] Chronic infectious diseases that have been associated with AA amyloidosis include the following: Tuberculosis, Leprosy, Bronchiectasis, Chronic osteomyelitis, Chronic pyelonephritis, COVID-19. […] The overall incidence in autopsies in Western countries is estimated at 0.5-0.86%, where the most frequent underlying diseases are RA (23-51%), juvenile idiopathic arthritis (7-48%), and ankylosing spondylitis (0-12%).

• #11 AA amyloidosis: Causes and diagnosis – UpToDate

  https://www.uptodate.com/contents/aa-amyloidosis-causes-and-diagnosis

  AA amyloidosis (previously known as secondary [AA] amyloidosis) is a disorder characterized by the extracellular tissue deposition of fibrils that are composed of fragments of and/or intact serum amyloid A protein (SAA), a hepatic acute phase reactant. […] AA amyloidosis may complicate any chronic inflammatory condition, including rheumatoid arthritis (RA), juvenile idiopathic arthritis (JIA), ankylosing spondylitis (AS), inflammatory bowel disease, familial periodic fever syndromes, chronic infections, and certain neoplasms (table 1). […] Multiple chronic inflammatory conditions, among them rheumatologic, autoinflammatory, chronic infectious, and other disorders, have been associated with the development of AA amyloid. […] The most common organ system involved in this form of amyloidosis is the kidney, although other organ systems are often also affected.

• #12 AA (Inflammatory) Amyloidosis: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/335559-overview

  RA is the most common rheumatic cause of AA amyloidosis. However, most patients with RA do not develop AA amyloidosis. Prolonged duration of disease, continuous disease activity, and inadequate treatment are risk factors for AA amyloidosis. […] Other inflammatory disorders associated with AA amyloidosis include the following: Inflammatory bowel disease (0.4-2%), Behet syndrome in Turkey (1-2%), Reactive arthritis in adults (0.3%), Psoriatic arthritis (3-13%), Multiple sclerosis, Familial Mediterranean fever, Chronic juvenile arthritis. […] Renal cell carcinoma (RCC) is the most frequent solid tumor associated with AA amyloidosis; lung lesions and basal cell carcinoma of the skin were also associated with AA amyloidosis more frequently than other solid tumors. […] Among other noninfectious chronic inflammatory diseases, AA amyloidosis has been reported in systemic lupus erythematosus, polymyositis, and polymyalgia rheumatica and has been observed in temporal artery biopsy samples of such patients. […] SAA production is mediated through inflammatory cytokines, primarily IL-6, and AA deposition has been noted in other disorders associated with increased IL-6 production. […] As many as 6% of patients with AA amyloidosis have no clinically overt inflammatory disease.

• #13

  https://link.springer.com/article/10.1007/s11926-024-01147-8

  A substantial proportion of cases (up to 20%) appear to develop AA amyloidosis in the absence of overt inflammation and thus labeled idiopathic. […] Obesity was suggested as a cause or susceptibility factor, but yet undefined genetic and/or environmental causes might be involved in the pathogenesis of both obese and idiopathic cases. […] The causes of AA amyloidosis are detailed in Table 1. […] Pathogenesis of AA amyloidosis revolves around a cascade of events triggered by sustained inflammation and increased production of SAA, cleavage of the signal peptide, aberrant aggregation, SAA-derived fibril formation, and deposition of amyloid fibrils. […] SAA is an apolipoprotein of high-density lipoprotein synthesized especially by hepatocytes as an acute-phase reactant, primarily under the influence of pro-inflammatory cytokines such as interleukin-1 (IL-1), IL-6, and tumor necrosis factor-alpha (TNF-).

• #14 Amyloidosis: Causes, Types, Symptoms, Diagnosis, Treatment, and Prognosis

  https://www.webmd.com/cancer/lymphoma/amyloidosis-symptoms-causes-treatments

  Familial (or hereditary) amyloidosis. This is a rare form passed down through families. It often affects the liver, nerves, heart, and kidneys. Many genetic defects are linked to a higher chance of amyloid disease. For example, an abnormal protein such as transthyretin (TTR) is usually the cause. […] Wild-type amyloidosis (formerly senile systemic amyloidosis). This is caused by deposits of normal TTR in the heart and other tissues. It happens most commonly in older men. […] Though some types of amyloid deposits have been linked to Alzheimer’s disease, the brain is rarely affected by amyloidosis that happens throughout your body.

• #15 About Amyloidosis – What causes Amyloidosis?

  https://www.myattrroadmap.com/about/what-causes-amyloidosis.html

  Hereditary ATTR amyloidosis is passed down through family members (genetically inherited). In hereditary ATTR amyloidosis, a mutation in the TTR gene results in an abnormal, misfolded TTR protein leading to amyloid accumulation in the body. If one parent has hereditary ATTR amyloidosis, there is a 50% chance their child will inherit the mutation. However, not everyone who inherits the mutation will go on to develop symptoms of ATTR amyloidosis. […] Wild-type ATTR amyloidosis is not inherited, but is instead caused when TTR proteins become unstable, often as a result of age, causing abnormal TTR.

• #16 About Amyloidosis – Amyloidosis Research Consortium

  https://arci.org/about-amyloidosis/

  Amyloidosis is the term for a group of rare diseases in which abnormal proteins deposit as amyloid in tissues and organs. […] Amyloid is produced when abnormal proteins in the body “misfold” and collect together in various tissues and organs. As the amyloid builds up, it starts to cause organ damage and impair quality of life. […] Amyloidosis is caused by abnormal proteins that misfold and deposit in various tissues and organs. The abnormal protein involved depends on what type of amyloidosis you have. In ATTR amyloidosis, for example, it is the TTR protein that misfolds and collects into amyloid fibrils. […] There are two types of TTR amyloidosis, wild-type and hereditary. Hereditary TTR amyloidosis, like the name suggests, is a disease caused by a genetic mutation. hATTR is inherited in an autosomal dominant pattern. This means that inheriting only one copy of the mutated gene can cause the condition.

• #17 The APOA1 p.Leu202Arg variant potentially causes autosomal recessive cardiac amyloidosis | Human Genome Variation

  https://www.nature.com/articles/s41439-024-00288-7

  The incomplete and age-dependent penetrance of the APOA1 variant could account for the presence of unaffected family members, especially the heterozygous older brother. […] The p.Leu202Arg variant reported here causes an amino acid substitution in the C-terminal region. […] The region in which the amyloidogenic variant is located may be responsible for the genotypic differences. […] Currently, amyloid production or deposition in hereditary ApoA-I amyloidosis cannot be suppressed using any established treatment.

• #17 The APOA1 p.Leu202Arg variant potentially causes autosomal recessive cardiac amyloidosis | Human Genome Variation

  https://www.nature.com/articles/s41439-024-00288-7

  ApoA-I amyloidosis is an extremely rare form of systemic amyloidosis that commonly involves the heart, kidneys, and liver. ApoA-I amyloidosis is caused by amyloidogenic variants of APOA1 that are inherited in an autosomal dominant manner. […] Hereditary amyloidosis is caused by genetic variants in amyloid precursor proteins, such as transthyretin (TTR) and apolipoprotein A-I (ApoA-I). […] Genetically, ApoA-I amyloidosis is caused by amyloidogenic variants of APOA1 inherited in an autosomal dominant fashion. After identification of the p.Gly50Arg variant in 1990, more than 20 causative variants have been identified. […] The amyloidogenic phenotype is related to altered structural features, including protein conformation and lipid binding, protein flexibility and stability, susceptibility to proteases, and aggregation propensity.

• #18 Neuromuscular Amyloidosis

  https://practicalneurology.com/diseases-diagnoses/neuromuscular/neuromuscular-amyloidosis/31815/

  Amyloidosis is a rare group of disorders caused by misfolding and extracellular deposition of proteins as amyloid fibrils. […] AL amyloidosis is a plasma cell disorder characterized by production of monoclonal light chain of kappa or lambda type that misfolds and deposits on any tissue other than the central nervous system. […] Transthyretin is a tetrameric transport protein that carries thyroxine and retinol and can become unstable, dissociate into monomers, misfold, and form amyloid deposits. […] ATTRv is caused by more than 130 missense mutations in the TTR gene, and is autosomal dominant with variable penetrance. […] ATTRwt amyloidosis is presumably caused by age-related instability of wild-type transthyretin. […] Other very rare systemic amyloidosis can affect the neuromuscular system, like gelsolin amyloidosis, -2 microglobulin amyloidosis, and AA amyloidosis.

• #19 Hereditary ATTR Amyloidosis – Amyloidosis Research Consortium

  https://arci.org/about-amyloidosis/hereditary-attr-amyloidosis/

  Hereditary ATTR amyloidosis is caused by a fault or mutation in the transthyretin (TTR) gene which is inherited (i.e. runs in families). The mutation results in an abnormal TTR protein that is unstable and readily misfolds, forming aggregates which deposit as amyloid in various organs and tissues in the body. […] There are over 120 different mutations in the TTR gene but, as research continues, it is possible that more may be identified. Each different mutation causes a different pattern of disease onset, symptoms and outlook. […] Hereditary ATTR amyloidosis is confirmed if a blood test shows a genetic mutation in the TTR gene. The test will be able to determine which variant it is.

• #20 Types of Amyloidosis | Amyloidosis Center

  https://www.bu.edu/amyloid/what/types/

  AA amyloidosis is caused by a chronic infection or an inflammatory disease such as rheumatoid arthritis, familial Mediterranean fever (FMF), osteomyelitis, or granulomatous ileitis. […] There are several types of inherited amyloidoses, the most common of which is caused by a mutation in the transthyretin (TTR) gene that produces abnormal transthyretin protein. […] More than 100 different mutations in transthyretin are known and most cause amyloidosis. […] Beta-2 microglobulin amyloidosis is caused by chronic renal failure and often occurs in patients who are on dialysis for many years. […] Localized amyloid deposits in the airway (trachea or bronchus), eye, or urinary bladder are often caused by local production of immunoglobulin light chains, not originating in the bone marrow.

• #21 Amyloidosis – Endocrine and Metabolic Disorders – MSD Manual Professional Edition

  https://www.msdmanuals.com/professional/endocrine-and-metabolic-disorders/amyloidosis/amyloidosis

  AA amyloidosis: Caused by aggregation of an acute phase reactant, serum amyloid A. […] Amyloidosis caused by aggregation of beta-2-microglobulin can occur in patients on long-term hemodialysis, but the incidence has declined with use of high-flow dialysis membranes. […] Localized forms of amyloidosis appear to be caused by local production and deposition of an amyloidogenic protein (most often immunoglobulin light chains) within the affected organ rather than by deposition of circulating proteins. […] Hereditary amyloidosis is caused by inheritance of a gene encoding a mutated aggregation-prone serum protein, usually a protein abundantly produced by the liver. […] Serum proteins that can cause hereditary amyloid include transthyretin (TTR), apolipoprotein A-I, apolipoprotein A-II, lysozyme, fibrinogen, gelsolin, and cystatin C.

• #22 Amyloidosis – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/amyloidosis/symptoms-causes/syc-20353178

  Wild-type amyloidosis. This variety has also been called senile systemic amyloidosis. It occurs when the TTR protein made by the liver is normal but produces amyloid for unknown reasons. Wild-type amyloidosis tends to affect men over age 70 and often targets the heart. It can also cause carpal tunnel syndrome. […] Localized amyloidosis. This type of amyloidosis often has a better prognosis than the varieties that affect multiple organ systems. Typical sites for localized amyloidosis include the bladder, skin, throat or lungs. Correct diagnosis is important so that treatments that affect the entire body can be avoided.

• #23 Cardiac Amyloidosis: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1967220-overview

  Primary amyloidosis (AL) is a type of plasma cell dyscrasia and is the most common type involving the heart. […] Secondary systemic amyloidosis seen in chronic inflammatory conditions rarely involves the heart. Organ dysfunction is usually reversible with resolution of the underlying inflammatory disorder. […] Familial or hereditary amyloidosis occurs because of a mutation in the transthyretin (TTR) gene located on the chromosome 18. Most patients with ATTR have heterozygous mutation and autosomal dominant inheritance. […] Senile systemic amyloidosis, also known as wild-type transthyretin (wt-TTR), is common in people over 70 years of age and has a better prognosis.

• #24 Amyloidosis – Hormonal and Metabolic Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/hormonal-and-metabolic-disorders/amyloidosis/amyloidosis

  AA amyloidosis may develop in response to various diseases that cause persistent infection or inflammation (such as tuberculosis, rheumatoid arthritis, and familial Mediterranean fever) and certain types of cancer. […] Mutated transthyretin, a protein produced by the liver, is the most frequent cause of hereditary amyloidosis. […] ATTRwt amyloidosis is caused by abnormal folding of normal (wild type, not mutated) transthyretin protein. It is not known what causes amyloid to build up in the heart. […] Localized amyloidosis occurs when amyloid is deposited in specific organs or tissues. For example, amyloid accumulates in the brain of people with Alzheimer disease and is thought to play a role in causing Alzheimer disease.

• #25 Amyloidosis > Fact Sheets > Yale Medicine

  https://www.yalemedicine.org/conditions/amyloidosis

  AA amyloidosis (serum amyloid A amyloidosis). This form of amyloidosis affects people who have long-term inflammatory conditions, such as rheumatoid arthritis or Crohns disease, or certain chronic infections, such as tuberculosis or osteomyelitis. […] Localized amyloidosis. Sometimes referred to as organ-specific amyloidosis, localized amyloidosis affects a single organ. […] Because amyloidosis is caused by misfolded proteins, this approach stabilizes proteins so that they are less likely to misfold, and thus less likely to clump together into amyloid fibrils.

• #26 What Causes Amyloidosis? Genetics, Sex, Age, and Other Risk Factors | MyAmyloidosisTeam

  https://www.myamyloidosisteam.com/resources/what-causes-amyloidosis

  The exact causes of amyloidosis are not well understood. This rare condition occurs when abnormal proteins, called amyloids, build up in different organs, leading to a variety of symptoms. Several types of proteins can contribute to amyloidosis, resulting in different forms of the disease. Both genetic and environmental risk factors can play a role in the development of amyloidosis. […] There are several types of amyloidosis, and each is caused by a different misfolded protein. […] Hereditary (or familial) amyloidosis is the only form of the disease caused directly by gene changes. […] These gene variations are often inherited, but not everyone with a mutation will develop amyloidosis. […] Amyloidosis risk usually increases with age. […] Men are more likely to be affected by this disease than women, according to Mayo Clinic.

• #27 Types and Causes of Amyloidosis by BKLYN Cardio

  https://bklyncardio.com/blog/what-causes-amyloidosis/

  Wild-type amyloidosis (ATTRwt) happens in some older adults as a result of the aging process, with symptoms typically affecting the heart. […] Several factors increase the risk of developing amyloidosis: […] Chronic inflammatory diseases: RA and IBD increase the risk of AA amyloidosis. […] Kidney dialysis: Long-term dialysis may lead to dialysis-related amyloidosis.

• #28 Facts about Amyloidosis | Fred Hutchinson Cancer Center

  https://www.fredhutch.org/en/diseases/amyloidosis/facts-resources.html

  There are many different causes of amyloidosis. In the most common type, light chain amyloidosis, plasma cells in your bone marrow become abnormal and secrete an abnormal protein into your blood that can build up in your heart, kidneys, nerves and other organ systems. […] Amyloidosis is more common after age 40 and more common in men than women. In some people it is linked with another disease, such as multiple myeloma. […] The familial form runs in families. […] Most people with amyloidosis have no known risk factors.

• #29 Amyloidosis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK470285/

  Amyloidosis is a heterogeneous acquired or hereditary disease that results from the abnormal deposition of beta-sheet fibrillar protein aggregates in various tissues. […] Amyloidosis can be classified according to systemic, hereditary, central nervous system, ocular, and localized etiology. However, the most common types are AL, AA, ATTR (amyloid transport protein transthyretin), and dialysis-related amyloidosis (beta2M type). […] The most common causes of amyloidosis are the immunoglobulin-light-chain relate amyloidosis (AL), ATTR amyloidosis, and reactive amyloidosis (AA) due to chronic inflammatory diseases like chronic infections and rheumatoid arthritis. AL amyloidosis is acquired and is caused by a small plasma cell clone that produces misfolded amyloidogenic light chains that deposit in different organs and tissues. […] AA amyloidosis is associated with various chronic inflammatory conditions, chronic or local microbial infections, and rarely with neoplasms. AA is the most common type of systemic amyloidosis. However, its incidence varies in different ethnic groups.

• #30 Cardiac Amyloidosis > Fact Sheets > Yale Medicine

  https://www.yalemedicine.org/conditions/cardiac-amyloidosis

  Disease caused by the buildup of abnormal proteins in the heart […] Cardiac amyloidosis is caused by the buildup of clumps of abnormally folded protein (known as amyloid fibrils) in the heart muscle. This impairs its ability to function normally. […] Proteins are found throughout the body, where they perform a range of critical functions. Proper functioning requires that the proteins retain their specific shape. If the protein misfolds or changes from its normal shape, it does not function properly. In amyloidosis, certain proteins misfold, binding together to form amyloid clumps or fibrils. […] When amyloid fibrils accumulate in the heart, they cause a disease known as cardiac amyloidosis. This causes the heart muscle to thicken and become stiff, which diminishes its ability to pump blood effectively and efficiently through the body, leading to cardiac complications.

• #31 Amyloidosis: Causes, Symptoms And Treatment

  https://www.netmeds.com/health-library/post/amyloidosis-causes-symptoms-and-treatment?srsltid=AfmBOorvlBpoUwhLANDvyIoykm00iAlzDgKuQs8umxzhICFJ3wyHqmhr

  Amyloidosis is caused due to accumulation of amyloid in the organs and how the amyloid build-up depends on the type of condition a person may have. […] In one type of amyloidosis, white blood cells in the bone marrow produce an abnormal protein called amyloid, which folds and forms masses and makes it harder for the system to break down. […] This type of amyloidosis occurs after an infection like tuberculosis or any disease that causes inflammation such as rheumatoid arthritis or inflammatory bowel disease. […] This is a rare type caused due to mutation in a gene that runs in families. […] Amyloidosis interferes with the hearts electrical system and makes it tough for the heart to function and beat properly. […] When abnormal protein molecule starts to accumulate in the kidneys it may injure filters inside the kidneys making it hard for the excretory system to eliminate waste from the blood. […] When amyloid builds up in nerves you may feel numbness and tingling sensation in your fingers and toes.

• #32 Amyloidosis – USZ

  https://www.usz.ch/en/disease/amyloidosis/

  Some amyloidoses can be traced back to a fault in the genes. Parents can then pass the disease on to their children. One example is familial ATTR amyloidosis. However, hereditary factors do not play a role in the majority of amyloidoses. The cause of AL amyloidosis is often a bone marrow or lymph gland disease. AA amyloidosis, on the other hand, is triggered by long-standing inflammatory diseases, such as rheumatoid arthritis or the chronic inflammatory bowel diseases Crohns disease and ulcerative colitis. […] The causes of amyloidosis vary. This can be caused by certain cancers, chronic inflammation or hereditary factors. What they all have in common, however, is that abnormally folded proteins accumulate and are deposited in the tissue. These deposits consist of insoluble protein fibers, the so-called amyloid fibrils. The body produces them faster than it can break them down again. The abnormal proteins disrupt the metabolism and the function of various organs.

• #33 AA amyloidosis: Causes and diagnosis – UpToDate

  https://www.uptodate.com/contents/aa-amyloidosis-causes-and-diagnosis

  The disorders most often identified as underlying AA amyloidosis have varied over time and geographically, reflecting the prevalent chronic inflammatory conditions. […] During the 20th century, AA amyloidosis has become less common and the contribution of AA amyloid to large series of amyloidosis has gradually decreased. […] The underlying causes of chronic inflammation have also changed. […] Over time, inflammatory arthritides (eg, rheumatoid arthritis [RA]) became the most common underlying disease, replacing chronic infections, particularly tuberculosis and osteomyelitis, which had historically been predominant causes. […] With widespread availability of highly effective antimicrobial agents, the infectious causes have become less common in much of the world. […] In addition, advances in the treatment of rheumatologic and other inflammatory disorders with highly effective biologic agents have resulted in fewer patients developing amyloidosis.

• #34 AA amyloidosis: Causes and diagnosis – UpToDate

  https://www.uptodate.com/contents/causes-and-diagnosis-of-aa-amyloidosis-and-relation-to-rheumatic-diseases

  The disorders most often identified as underlying AA amyloidosis have varied over time and geographically, reflecting the prevalent chronic inflammatory conditions. During the 20th century, AA amyloidosis has become less common and the contribution of AA amyloid to large series of amyloidosis has gradually decreased. The underlying causes of chronic inflammation have also changed. Over time, inflammatory arthritides (eg, rheumatoid arthritis [RA]) became the most common underlying disease, replacing chronic infections, particularly tuberculosis and osteomyelitis, which had historically been predominant causes. With widespread availability of highly effective antimicrobial agents, the infectious causes have become less common in much of the world, in addition, advances in the treatment of rheumatologic and other inflammatory disorders with highly effective biologic agents have resulted in fewer patients developing amyloidosis.

• #35 OP0323 CAUSES OF AA AMYLOIDOSIS: ANALYSIS OF 952 PATIENTS SEEN AT A REFERENCE CENTRE OVER 34 YEARS | Annals of the Rheumatic Diseases

  https://ard.bmj.com/content/83/Suppl_1/8.1

  In systemic AA amyloidosis (AAA) the amyloid fibrils are derived from serum Amyloid A protein (SAA) and potential underlying causes include almost any condition that can induce persistent systemic inflammation. […] The change in distribution of underlying diseases appears to reflect the impact of biologics in successful treatment of rheumatoid arthritis and juvenile arthritis although there has been less effect in AAA complicating seronegative arthritis or inflammatory bowel disease. […] Since 2015 more patients presented with AAA complicating inflammation of unknown aetiology or obesity than any diagnosed chronic inflammatory disorder, followed by AAA in PWUD. […] The rise in AAA complicating inflammation of unknown cause appears to be genuine. The increasing BMI in this group supports AAA as a rare complication of the low-grade inflammation resulting from adipocyte production of IL-6 which can be seen in some obese individuals.

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