Skin features of<unk>  cutis dyschromica. Hyperpigmented and hypopigmented macules on (A) lower legs, (B) back and waist, (C) waist. (D) Individual blisters on upper arm The presentation of<unk>  is broad and depends on the site of amyloid accumulation. The kidney and heart are the most common organs involved. Kidneys Amyloid deposition in the kidney often involve the glomerular capillaries and mesangial regions, affecting the organ’s ability to filter and excrete waste and retain plasma protein. This can lead to high levels of protein in the urine (proteinuria) and nephrotic syndrome. Several types of<unk> , including the<unk>  and AA types, are associated with nephrotic syndrome. Approximately 20% and 40-60% of people with<unk>  and AA<unk>  respectively progress to end-stage kidney disease requiring dialysis. Heart Amyloid deposition in the heart can cause both diastolic and systolic heart failure. EKG changes may be present, showing low voltage and conduction abnormalities like atrioventricular block or sinus node dysfunction. On echocardiography, the heart shows a restrictive filling pattern, with normal to mildly reduced systolic function. AA<unk>  usually spares the heart. Cardiac<unk>  can present with symptoms of heart failure including shortness of breath, fatigue, and edema. As cardiac<unk>  progresses, the amyloid deposition can affect the heart's ability to pump and fill blood as well as its ability to maintain normal rhythm, which leads to worsening heart function and decline in people's quality of life. Nervous System People with<unk>  do not get central nervous system involvement but can develop sensory and autonomic neuropathies. Sensory neuropathy develops in a symmetrical pattern and progresses in a distal to proximal manner. Autonomic neuropathy can present as orthostatic hypotension but may manifest more gradually with nonspecific gastrointestinal symptoms like constipation, nausea, or early satiety. Neuropathic presentation can depend on the etiology of<unk> . People with<unk>  may experience dysfunction in various organ systems depending on the location and extent of nervous system involvement. For example, peripheral neuropathy can cause erectile dysfunction, incontinence and constipation, pupillary dysfunction, and sensory loss depending on the distribution of<unk> <unk> ong different peripheral nerves.<unk>  of the central nervous system can have more severe and systemic presentations that may include life-threatening arrhythmias, cardiac failure, malnutrition, infection, or death. Gastrointestinal and Accessory Organs Accumulation of amyloid proteins in the gastrointestinal system may be caused by a wide range of amyloid disorders and have different presentations depending on the degree of organ involvement. Potential symptoms include weight loss, diarrhea, abdominal pain, heartburn (gastrointestinal reflux), and GI bleeding.<unk>  may<unk> so affect accessory digestive organs including the liver, and may present with jaundice, fatty stool, anorexia, fluid buildup in the abdomen, and spleen enlargement. Accumulation of amyloid proteins in the liver can lead to elevations in serum aminotransferases and<unk> kaline phosphatase, two biomarkers of liver injury, which is seen in about one third of people. Liver enlargement is common. In contrast, spleen enlargement is rare, occurring in 5% of people. Splenic dysfunction, leading to the presence of Howell-Jolly bodies on blood smear, occurs in 24% of people with<unk> . Malabsorption is seen in 8.5% of<unk> <unk>  and 2.4% of AA<unk> . One suggested mechanism for the observed malabsorption is that amyloid deposits in the tips of intestinal villi (fingerlike projections that increase the intestinal area available for absorption of food), begin to erode the functionality of the villi, presenting a sprue-like picture. Glands Both the thyroid and adrenal glands can be infiltrated. It is estimated that 10–20% of people with<unk>  have hypothyroidism. Adrenal infiltration may be harder to appreciate given that its symptoms of orthostatic hypotension and low blood sodium concentration may be attributed to autonomic neuropathy and heart failure. "Amyloid deposits occur in the pancreas of people who<unk> so have diabetes mellitus,<unk> though it is not known if this is functionally important. The major component of pancreatic amyloid is a 37-amino acid residue peptide known as islet amyloid polypeptide or 'amylin.' This is stored with insulin in secretory granules in B cells and is co secreted with insulin." (Rang and Dale's Pharmacology, 2015.) Musculoskeletal system Amyloid proteins deposit most commonly inside the knee, followed by hands, wrists, elbow, hip, and ankle, causing joint pain. In males with advanced age (>80 years), there is significant risk of wild-type transthyretin amyloid deposition in synovial tissue of knee joint, but predominantly in old age deposition of wild type transthyretin is seen in cardiac ventricles. ATTR deposits have been found in ligamentum flavum of patients that underwent surgery for lumbar spinal stenosis. In beta 2-microglobulin<unk> , males have high risk of getting carpal tunnel syndrome. Aβ2MG<unk>  (Hemodialysis associated<unk>  tends to deposit in synovial tissue, causing chronic inflammation of the synovial tissue in knee, hip, shoulder and interphalangeal joints. Amyloid light chains deposition in shoulder joint causes enlarged shoulders,<unk> so known as "shoulder pad sign". Amyloid light chain depositions can<unk> so cause bilateral symmetric polyarthritis. The deposition of amyloid proteins in the bone marrow without causing plasma cell dyscrasias is called amyloidoma. It is commonly found in cervical, lumbar, and sacral vertebrae. Those affected may be presented with bone pain due to bone lysis, lumbar paraparesis, and a variety of neurological symptoms. Vertebral fractures are<unk> so common. Eyes A rare development is amyloid purpura, a susceptibility to bleeding with bruising around the eyes, termed "raccoon-eyes". Amyloid purpura is caused by amyloid deposition in the blood vessels and reduced activity of thrombin and factor X, two clotting proteins that lose their function after binding with amyloid. Oral cavity Amyloid deposits in tissue can cause enlargement of structures. Twenty percent of people with<unk> <unk>  have an enlarged tongue, that can lead to obstructive sleep apnea, difficulty swallowing, and<unk> tered taste. Tongue enlargement does not occur in ATTR or AA<unk> . Deposition of amyloid in the throat can cause hoarseness.
Amyloidosis
Sjögren syndrome  Sjögren syndrome  1
Gamasoidosis
Melioidosis
Sarcoidosis
Amyloid cardiomyopathy
Neurosarcoidosis
Cardiac amyloidosis