Materiały źródłowe

• #1 Calciphylaxis – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/calciphylaxis/symptoms-causes/syc-20370559

  Calciphylaxis (kal-sih-fuh-LAK-sis) is a rare, serious disease. It involves a buildup of calcium in small blood vessels of fat tissues and skin. […] The exact cause of calciphylaxis isn’t clear. But people with the disease usually have kidney failure. […] Calciphylaxis can happen in people without kidney disease too. […] The exact cause of calciphylaxis isn’t known. The disease involves the buildup of calcium in the smallest parts of the arteries in fat tissues and skin. […] Many people who get calciphylaxis also have kidney failure or receive dialysis. It’s not known why people with kidney failure or people receiving dialysis are at higher risk of calciphylaxis. […] For some people, the calcium buildup in calciphylaxis is linked with small organs in the neck called parathyroid glands. If the glands release too many parathyroid hormones, that can cause calcium to collect. But the link isn’t clear. Most people with seriously overactive parathyroid glands don’t get calciphylaxis. And many people with kidney failure and calciphylaxis don’t have overactive parathyroid glands.

• #2 Calciphylaxis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK519020/

  Calciphylaxis, also known as calcific uremic arteriolopathy, is a rare but potentially devastating condition most often observed in patients with end-stage renal disease, although it does occasionally develop in patients without renal failure. […] The cause and mechanisms leading to calciphylaxis remain poorly understood, and its development is likely dependent on multiple factors that lead to medial calcification of arterioles. Elevated calcium x phosphate product, increased parathyroid hormone levels, and administration of activated vitamin D have been associated with the development of calciphylaxis. […] Deficiency of vascular calcification inhibitors such as fetuin-A, osteoprotegerin, and matrix G1a protein may play a role in the development of calciphylaxis.

• #3 Calciphylaxis // Middlesex Health

  https://middlesexhealth.org/learning-center/diseases-and-conditions/calciphylaxis

  Calciphylaxis causes blood clots, painful skin ulcers and may cause serious infections that can lead to death. […] The exact cause of calciphylaxis is unknown, but recent studies have revealed that most people with the condition have abnormalities in blood-clotting factors. […] In addition, people with calciphylaxis have an imbalance in the metabolism of calcium. […] These abnormalities can lead to small blood clots forming more often than they normally would. […] This causes calcium to be deposited in the smallest parts of the arteries (arterioles), which eventually leads to the formation of blood clots in the arterioles. […] Blood clots can cause fat tissues and skin to be deprived of oxygen and nourishment.

• #4 Calciphylaxis – Augusta HealthSearchClose SearchSearch IconSearch IconClose Search IconMobile Menu IconMobile Menu Close IconInstagramFacebookTwitterYoutube

  https://www.augustahealth.com/disease/calciphylaxis/

  Calciphylaxis (kal-sih-fuh-LAK-sis) is a serious, uncommon disease in which calcium accumulates in small blood vessels of the fat and skin tissues. […] The exact cause of calciphylaxis is unknown, but recent studies have revealed that most people with the condition have abnormalities in blood-clotting factors. Blood-clotting factors are substances in your blood that help stop bleeding. These abnormalities can lead to small blood clots forming more often than they normally would. […] In addition, people with calciphylaxis have an imbalance in the metabolism of calcium. This causes calcium to be deposited in the smallest parts of the arteries (arterioles), which eventually leads to the formation of blood clots in the arterioles. Blood clots can cause fat tissues and skin to be deprived of oxygen and nourishment.

• #5 Calciphylaxis | UK Kidney Association

  https://www.ukkidney.org/rare-renal/patient-information-0/calciphylaxis

  Calciphylaxis is a serious condition that can occur in people chronic kidney disease; it is most commonly seen in people receiving dialysis but remains very rare with approximately one case diagnosed in every 600 people, having dialysis, per year. […] The exact cause of calciphylaxis remains unknown; we do not know why some people get the condition and others do not. Possible risk factors (things that may contribute to the development of calciphylaxis) are considered to be: […] The trigger for the disease is not known but may include local injury; for example, people can report their calciphylaxis started with a flee bite, cut or graze to the area. […] What risk factors are associated with development and progression of calciphylaxis?

• #6

  https://journals.lww.com/jasn/abstract/1996/07000/calciphylaxis_in_chronic_renal_failure_.2.aspx

  Calciphylaxis is a rare and life-threatening complication that is estimated to occur in 1% of patients with ESRD each year. […] Although most calciphylaxis patients have abnormalities of the calcium:phosphate axis or elevated levels of parathyroid hormone, these abnormalities do not appear to be fundamental to the pathophysiology of the disorder, and the etiology of calciphylaxis remains unclear. […] Recently, functional protein C deficiency has been hypothesized to cause a hypercoagulable state that could induce thrombosis in small vessels, with resulting skin ischemia, necrosis, and gangrene.

• #7 Calciphylaxis: Symptoms & Causes

  https://my.clevelandclinic.org/health/diseases/22359-calciphylaxis

  Calciphylaxis is a rare, painful disease that happens most often in people with moderate to severe kidney problems. It happens when calcium deposits form in your blood vessels and block blood flow to areas of your skin. This can lead to open wounds that are prone to dangerous (or even deadly) infections. […] The exact cause of calciphylaxis remains unknown, but research suggests it takes more than one factor to cause it. These factors can team up to become calciphylaxis causes. […] These factors include: Too much parathyroid hormone. When you’re having kidney problems, your parathyroid glands try to keep the calcium levels in your blood stable. In doing this, they produce too much of a hormone your body needs (hyperparathyroidism). This can also happen when you aren’t getting enough vitamin D or calcium.

• #8 Calciphylaxis: Symptoms & Causes

  https://my.clevelandclinic.org/health/diseases/22359-calciphylaxis

  Kidney failure. Kidneys that aren’t functioning properly disrupt your body’s chemical balance of handling calcium and phosphorus. […] Too much phosphorus. Your body uses phosphorus in several chemical processes, and your kidneys help control phosphorus levels. […] Certain types of cancer. This is more common with cases of calciphylaxis that have no connection to kidney disease. […] Trauma. This can be due to either injuries or medical procedures like an incision or a needle stick for an intravenous (IV) medication line.

• #9 Calciphylaxis: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1095481-overview

  Disorders associated with the development of calciphylaxis include the following: Common associations – CKD, hypercalcemia, hyperphosphatemia, elevated calcium-phosphate product, hyperparathyroidism, and vascular calcification […] The cause of calciphylaxis remains obscure. Most cases occur in the setting of CKD, abnormal calcium-phosphate homeostasis, and hyperparathyroidism. Both hypercalcemia and hyperphosphatemia may be present, and the calcium-phosphate product frequently exceeds 60-70 mg2/dL2. However, calciphylaxis may occur in the setting of normal, or minimally elevated, calcium and phosphate levels. […] Case reports exist of calciphylaxis occurring in primary hyperparathyroidism, cirrhosis, Crohn disease, malignancy, and rheumatoid arthritis, without renal disease. The pathogenesis of calciphylaxis in these cases is uncertain.

• #10 Calciphylaxis: Symptoms & Causes

  https://my.clevelandclinic.org/health/diseases/22359-calciphylaxis

  Calciphylaxis is a rare, painful disease that happens most often in people with moderate to severe kidney problems. It happens when calcium deposits form in your blood vessels and block blood flow to areas of your skin. This can lead to open wounds that are prone to dangerous (or even deadly) infections. […] The exact cause of calciphylaxis remains unknown, but research suggests it takes more than one factor to cause it. These factors can team up to become calciphylaxis causes. […] These factors include: Too much parathyroid hormone. When you’re having kidney problems, your parathyroid glands try to keep the calcium levels in your blood stable. In doing this, they produce too much of a hormone your body needs (hyperparathyroidism). This can also happen when you aren’t getting enough vitamin D or calcium.

• #11 Calciphylaxis: Symptoms & Causes

  https://my.clevelandclinic.org/health/diseases/22359-calciphylaxis

  Calciphylaxis is a rare, painful disease that happens most often in people with moderate to severe kidney problems. It happens when calcium deposits form in your blood vessels and block blood flow to areas of your skin. This can lead to open wounds that are prone to dangerous (or even deadly) infections. […] The exact cause of calciphylaxis remains unknown, but research suggests it takes more than one factor to cause it. These factors can team up to become calciphylaxis causes. […] These factors include: Too much parathyroid hormone. When you’re having kidney problems, your parathyroid glands try to keep the calcium levels in your blood stable. In doing this, they produce too much of a hormone your body needs (hyperparathyroidism). This can also happen when you aren’t getting enough vitamin D or calcium.

• #12 Calciphylaxis: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1095481-overview

  Disorders associated with the development of calciphylaxis include the following: Common associations – CKD, hypercalcemia, hyperphosphatemia, elevated calcium-phosphate product, hyperparathyroidism, and vascular calcification […] The cause of calciphylaxis remains obscure. Most cases occur in the setting of CKD, abnormal calcium-phosphate homeostasis, and hyperparathyroidism. Both hypercalcemia and hyperphosphatemia may be present, and the calcium-phosphate product frequently exceeds 60-70 mg2/dL2. However, calciphylaxis may occur in the setting of normal, or minimally elevated, calcium and phosphate levels. […] Case reports exist of calciphylaxis occurring in primary hyperparathyroidism, cirrhosis, Crohn disease, malignancy, and rheumatoid arthritis, without renal disease. The pathogenesis of calciphylaxis in these cases is uncertain.

• #13 Update on Calciphylaxis Etiopathogenesis, Diagnosis, and Management | MDedge

  https://blogs.the-hospitalist.org/content/update-calciphylaxis-etiopathogenesis-diagnosis-and-management

  Calciphylaxis is thought to have a multifactorial etiology with the exact cause or trigger unknown.7 A long list of risk factors and triggers is associated with the condition (Table 1). Calciphylaxis primarily affects small arteries (40600 m in diameter) that become calcified due to an imbalance between inhibitors and promoters of calcification.2,11 Fetuin-A and matrix Gla protein inhibit vascular calcification and are downregulated in calciphylaxis.12,13 Dysfunctional calcium, phosphate, and parathyroid hormone regulatory pathways provide an increased substrate for the process of calcification, which causes endothelial damage and microthrombosis, resulting in tissue ischemia and infarction.14,15 Notably, there is growing interest in the role of vitamin K in the pathogenesis of calciphylaxis. Vitamin K inhibits vascular calcification, possibly by increasing the circulating levels of carboxylated matrix Gla protein.16

• #14 Calciphylaxis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK519020/

  Calciphylaxis, also known as calcific uremic arteriolopathy, is a rare but potentially devastating condition most often observed in patients with end-stage renal disease, although it does occasionally develop in patients without renal failure. […] The cause and mechanisms leading to calciphylaxis remain poorly understood, and its development is likely dependent on multiple factors that lead to medial calcification of arterioles. Elevated calcium x phosphate product, increased parathyroid hormone levels, and administration of activated vitamin D have been associated with the development of calciphylaxis. […] Deficiency of vascular calcification inhibitors such as fetuin-A, osteoprotegerin, and matrix G1a protein may play a role in the development of calciphylaxis.

• #15 Case Report: Warfarin-Induced Non-Uremic Calciphylaxis Mimicking Vasculitis – The Rheumatologist

  https://www.the-rheumatologist.org/article/case-report-warfarin-induced-non-uremic-calciphylaxis-mimicking-vasculitis/

  Calciphylaxis, or calcific uremic arteriolopathy, is a rare disease characterized by calcification of the arterioles and capillaries in the dermis and subcutaneous tissue, resulting in thrombus formation and subsequent skin ischemia and necrosis. […] In rare cases, warfarin has been associated with non-ESRD-related calciphylaxis. […] This condition can also occur without kidney disease, termed non-uremic calciphylaxis, and is most commonly associated with derangements in calcium and phosphate homeostasis, such as hyperphosphatemia, hypercalcemia and thyroid disease. […] Additionally, cases of exposure to warfarin as a cause for calciphylaxis have been documented. […] The suspected mechanism by which warfarin induces calciphylaxis is through the inhibition of vitamin K-dependent carboxylation of matrix Gla-protein, a mineral-binding extracellular matrix protein that prevents calcium deposition in arteries. […] Warfarin decreases protein C levels faster than the coagulation factors, therefore increasing thrombus formation.

• #16 Calciphylaxis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK519020/

  Calciphylaxis, also known as calcific uremic arteriolopathy, is a rare but potentially devastating condition most often observed in patients with end-stage renal disease, although it does occasionally develop in patients without renal failure. […] The cause and mechanisms leading to calciphylaxis remain poorly understood, and its development is likely dependent on multiple factors that lead to medial calcification of arterioles. Elevated calcium x phosphate product, increased parathyroid hormone levels, and administration of activated vitamin D have been associated with the development of calciphylaxis. […] Deficiency of vascular calcification inhibitors such as fetuin-A, osteoprotegerin, and matrix G1a protein may play a role in the development of calciphylaxis.

• #17 Calciphylaxis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2989826/

  Calciphylaxis is a complex disorder with a multifactorial etiology. The exact pathogenesis of calciphylaxis is unclear. […] In humans, most of the cases are associated with chronic renal failure and end-stage renal disease (ESRD). […] Excess aluminum greater than 25ng/mL confers a four-fold increased risk and is thought to play a role in the pathogenesis of calciphylaxis. […] Diabetes, obesity, corticosteroid use, immunosuppressive drugs, warfarin use, female sex, and protein C or S deficiency have been reported as risk factors for the development of calciphylaxis. […] Additionally, obesity confers a four-fold increased risk for calciphylaxis. […] In humans, vascular calcification is an active process and is not sufficient to produce skin necrosis. […] Activation of nuclear factor kB (NFkB) is thought to be the final common pathway leading to vascular calcification. […] Calciphylaxis is a complex disorder with a multifactorial etiology. The exact cause is unknown, but it is seen more commonly in patients with renal failure.

• #18 Article: Acenocoumarol as a risk factor for calciphylaxis: a feature clinicians should be aware of (full text) – May 2017 – NJM

  https://www.njmonline.nl/article_ft.php?a=1845&d=1222&i=205

  Calciphylaxis is a rare disease that consists of calcifications in the blood vessels, which lead to secondary ischaemia and painful necrotic lesions of the skin. […] In contrast with uraemic calciphylaxis, causes of and risk factors for non-uraemic calciphylaxis are relatively unknown to clinicians and have yet to become fully established. […] A systematic review assessing the different causes of non-uraemic calciphylaxis indicated primary hyperparathyroidism as the most common cause (28%). […] The suggested risk factors for non-uraemic calciphylaxis are numerous, including white race, female sex, obesity, diabetes mellitus, use of a vitamin K antagonist, liver disease, malignancy, systemic corticosteroid use, and protein C and S deficiency. […] Of these, we found the use of a vitamin K antagonist of particular interest, as it is the only one of the mentioned risk factors that can immediately be adjusted.

• #19 Calciphylaxis: Causes, Symptoms, and Management — DermNet

  https://dermnetnz.org/topics/calciphylaxis

  Calciphylaxis is a condition characterised by necrosis (cellular death) of the skin and fatty tissue. It is seen mainly in patients with end-stage kidney disease. […] The cause of calciphylaxis is not properly understood. The primary event is occlusion of the small blood vessels in the skin by a thrombus (blood clot), which results in spreading ischaemia and skin necrosis. It is thought that the clots occur because of calcification within the walls of the blood vessels. […] Calciphylaxis can also occur in patients with normal kidney function. Causes of non-uraemic calciphylaxis include: Primary hyperparathyroidism, Malignancy, Alcoholic liver disease, Connective tissue disease, Diabetes mellitus. […] High levels of matrix metalloproteinases have been described, and one theory suggests chemically altered elastin protein allows deposition of calcium on small vessels.

• #20

  https://scholars.duke.edu/individual/pub1382429

  Calciphylaxis, or calcific uremic arteriolopathy, is a well-described entity in end-stage kidney disease and renal transplant patients; however, little systematic information is available on calciphylaxis from nonuremic causes. […] Primary hyperparathyroidism, malignancy, alcoholic liver disease, and connective tissue disease were the most common reported causes. […] Nonuremic calciphylaxis is reported most often in white women. Mineral abnormalities that are invoked as potential causes in calcific uremic arteriolopathy are often absent, suggesting that heterogeneous mechanisms may contribute to its pathogenesis. […] Nonuremic calciphylaxis is associated with high mortality, and there is no known effective treatment.

• #21 Calciphylaxis: What Causes it and Is it Fatal?

  https://www.webmd.com/skin-problems-and-treatments/what-is-calciphylaxis

  Calciphylaxis is a rare disease that mostly affects people who suffer from kidney failure. […] The cause of calciphylaxis is currently unknown. Researchers are working to discover a reason why calciphylaxis happens by studying the biology and risk factors of the disease. […] Most people who get calciphylaxis are going through end-stage kidney failure. However, there are some other known factors that can put you at risk for calciphylaxis: […] A history of kidney problems or transplant. This is a major risk factor. A history of kidney malfunction, dialysis, or kidney transplant can make you vulnerable to this disease. […] Trouble metabolizing calcium. A calcium imbalance or trouble metabolizing the mineral calcium can lead to this condition. […] Hormonal imbalance. Parathyroid hormone (PTH) regulates calcium levels in your body. If the balance of this hormone changes, it can put you at risk. […] Autoimmune disease. In rare cases, calciphylaxis isn’t caused by kidney problems. Sometimes, autoimmune diseases like Lupus, multiple sclerosis, and rheumatoid arthritis can lead to calciphylaxis.

• #22 Calciphylaxis: Causes, Symptoms, and Management — DermNet

  https://dermnetnz.org/topics/calciphylaxis

  Calciphylaxis is a condition characterised by necrosis (cellular death) of the skin and fatty tissue. It is seen mainly in patients with end-stage kidney disease. […] The cause of calciphylaxis is not properly understood. The primary event is occlusion of the small blood vessels in the skin by a thrombus (blood clot), which results in spreading ischaemia and skin necrosis. It is thought that the clots occur because of calcification within the walls of the blood vessels. […] Calciphylaxis can also occur in patients with normal kidney function. Causes of non-uraemic calciphylaxis include: Primary hyperparathyroidism, Malignancy, Alcoholic liver disease, Connective tissue disease, Diabetes mellitus. […] High levels of matrix metalloproteinases have been described, and one theory suggests chemically altered elastin protein allows deposition of calcium on small vessels.

• #23

  https://scholars.duke.edu/individual/pub1382429

  Calciphylaxis, or calcific uremic arteriolopathy, is a well-described entity in end-stage kidney disease and renal transplant patients; however, little systematic information is available on calciphylaxis from nonuremic causes. […] Primary hyperparathyroidism, malignancy, alcoholic liver disease, and connective tissue disease were the most common reported causes. […] Nonuremic calciphylaxis is reported most often in white women. Mineral abnormalities that are invoked as potential causes in calcific uremic arteriolopathy are often absent, suggesting that heterogeneous mechanisms may contribute to its pathogenesis. […] Nonuremic calciphylaxis is associated with high mortality, and there is no known effective treatment.

• #24 Case Report: Warfarin-Induced Non-Uremic Calciphylaxis Mimicking Vasculitis – The Rheumatologist

  https://www.the-rheumatologist.org/article/case-report-warfarin-induced-non-uremic-calciphylaxis-mimicking-vasculitis/

  Calciphylaxis, or calcific uremic arteriolopathy, is a rare disease characterized by calcification of the arterioles and capillaries in the dermis and subcutaneous tissue, resulting in thrombus formation and subsequent skin ischemia and necrosis. […] In rare cases, warfarin has been associated with non-ESRD-related calciphylaxis. […] This condition can also occur without kidney disease, termed non-uremic calciphylaxis, and is most commonly associated with derangements in calcium and phosphate homeostasis, such as hyperphosphatemia, hypercalcemia and thyroid disease. […] Additionally, cases of exposure to warfarin as a cause for calciphylaxis have been documented. […] The suspected mechanism by which warfarin induces calciphylaxis is through the inhibition of vitamin K-dependent carboxylation of matrix Gla-protein, a mineral-binding extracellular matrix protein that prevents calcium deposition in arteries. […] Warfarin decreases protein C levels faster than the coagulation factors, therefore increasing thrombus formation.

• #25 Warfarin and Calciphylaxis — A Rare but Serious Adverse Event

  https://www.medsafe.govt.nz/profs/PUArticles/September2017/WarfarinAndCalciphylaxis.htm

  Calciphylaxis is a very rare but serious condition most commonly observed in patients with known risk factors such as end-stage renal disease. […] Calciphylaxis has been reported in patients taking warfarin, including those with normal renal function. […] Calciphylaxis is generally seen in patients with end-stage renal disease on dialysis or in those with known risk factors such as protein C or S deficiency, hyperphosphataemia, hypercalcaemia or hypoalbuminaemia. […] The exact pathogenesis of calciphylaxis is unknown. […] The mechanism by which warfarin causes calciphylaxis may be mediated through the matrix Gla protein, which is a vitamin-K-dependent protein that prevents calcium deposition in arteries. Warfarin inhibits Gla protein and may therefore promote vascular calcification in susceptible individuals. […] The authors of a recent review suggest that warfarin-associated calciphylaxis is distinct from classic calciphylaxis in pathogenesis, course and particularly outcome as the survival rate of nonuremic patients was remarkably high (83%).

• #26 Calciphylaxis: treatment, symptoms, causes and diagnosis

  https://www.kidneyresearchuk.org/conditions-symptoms/calciphylaxis/

  Calciphylaxis is a rare and severe condition mostly seen in people with kidney problems, especially those receiving dialysis. It happens when calcium products build up in the small blood vessels in and around the skin, blocking the flow of blood – this is a process known as ‘calcification’. As a result, the skin and underlying tissues do not receive enough blood flow, stopping adequate oxygen from reaching the skin. This leads to painful skin ulcers that are prone to serious infection. […] The exact cause of calciphylaxis isn’t fully understood; it remains unknown why some people get the condition and others do not. In addition to kidney disease, possible risk factors (things that increase the chances of a person developing the condition) are: Being overweight, Use of certain medications, such as warfarin, Female gender: women are more commonly affected by calciphylaxis than men, Hyperparathyroidism (high parathyroid hormone level in the blood): an overactive parathyroid gland can disrupt the balance of calcium and phosphate in the body, High levels of calcium and/or phosphate in the blood.

• #27 Calciphylaxis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2989826/

  Calciphylaxis is a complex disorder with a multifactorial etiology. The exact pathogenesis of calciphylaxis is unclear. […] In humans, most of the cases are associated with chronic renal failure and end-stage renal disease (ESRD). […] Excess aluminum greater than 25ng/mL confers a four-fold increased risk and is thought to play a role in the pathogenesis of calciphylaxis. […] Diabetes, obesity, corticosteroid use, immunosuppressive drugs, warfarin use, female sex, and protein C or S deficiency have been reported as risk factors for the development of calciphylaxis. […] Additionally, obesity confers a four-fold increased risk for calciphylaxis. […] In humans, vascular calcification is an active process and is not sufficient to produce skin necrosis. […] Activation of nuclear factor kB (NFkB) is thought to be the final common pathway leading to vascular calcification. […] Calciphylaxis is a complex disorder with a multifactorial etiology. The exact cause is unknown, but it is seen more commonly in patients with renal failure.

• #28 Calciphylaxis epidemiology, risk factors, treatment and survival among French chronic kidney disease patients: a case-control study | BMC Nephrology | Full Text

  https://bmcnephrol.biomedcentral.com/articles/10.1186/s12882-020-01722-y

  Calcific uremic arteriolopathy (CUA), also called calciphylaxis, is a rare but devastating disease involving patients with end stage renal disease (ESRD). CUA causes painful skin lesions that evolve to ulcerative lesions at risk of superinfection and sepsis, with a poor prognosis. Reported risk factors of CUA are female sex, obesity, diabetes mellitus, vitamin K antagonists (VKA) and ESRD. Dysregulation of calcium-phosphate metabolism also participates to its development. Risks factors of developing CUA among dialysis patients were obesity, VKA, weight loss, serum albumin decrease or high serum phosphate in the 6 months before lesion onset. CUA involved mainly obese patients under VKA. Malnutrition and inflammation preceded the onset of skin lesions and could be warning signs among dialysis patients at risk. The association of CUA and VKA therapy has already been reported. By decreasing carboxylated matrix Gla Protein (cMGP), VKA are suspected to contribute to vascular calcification and therefore promote calciphylaxis. Thrombophilia is also a known risk factor of calciphylaxis. We were surprised to identify so many non-dialyzed patients with calciphylaxis. The frontier between uremic calciphylaxis and non-uremic calciphylaxis is difficult to define. Calciphylaxis seems to be the conjunction of multiple conditions (obesity, VKA, bone mineral disease abnormalities, uremia, inflammation) with a broad spectrum of variations. The mortality rate was particularly high in our study. Ulcerative lesions and proximal-type CUA, known as poor prognosis factors, were predominant.

• #29

  https://journals.lww.com/cjasn/fulltext/2008/07000/calciphylaxis_from_nonuremic_causes__a_systematic.33.aspx

  Calciphylaxis, or calcific uremic arteriolopathy, is a well-described entity in end-stage kidney disease and renal transplant patients; however, little systematic information is available on calciphylaxis from nonuremic causes. […] This systematic review was designed to characterize etiologies, clinical features, laboratory abnormalities, and prognosis of nonuremic calciphylaxis. […] Primary hyperparathyroidism, malignancy, alcoholic liver disease, and connective tissue disease were the most common reported causes. […] Preceding corticosteroid use was reported for 61% patients. […] Mineral abnormalities that are invoked as potential causes in calcific uremic arteriolopathy are often absent, suggesting that heterogeneous mechanisms may contribute to its pathogenesis. […] Nonuremic calciphylaxis is associated with high mortality, and there is no known effective treatment.

• #30 Calciphylaxis epidemiology, risk factors, treatment and survival among French chronic kidney disease patients: a case-control study | BMC Nephrology | Full Text

  https://bmcnephrol.biomedcentral.com/articles/10.1186/s12882-020-01722-y

  Calcific uremic arteriolopathy (CUA), also called calciphylaxis, is a rare but devastating disease involving patients with end stage renal disease (ESRD). CUA causes painful skin lesions that evolve to ulcerative lesions at risk of superinfection and sepsis, with a poor prognosis. Reported risk factors of CUA are female sex, obesity, diabetes mellitus, vitamin K antagonists (VKA) and ESRD. Dysregulation of calcium-phosphate metabolism also participates to its development. Risks factors of developing CUA among dialysis patients were obesity, VKA, weight loss, serum albumin decrease or high serum phosphate in the 6 months before lesion onset. CUA involved mainly obese patients under VKA. Malnutrition and inflammation preceded the onset of skin lesions and could be warning signs among dialysis patients at risk. The association of CUA and VKA therapy has already been reported. By decreasing carboxylated matrix Gla Protein (cMGP), VKA are suspected to contribute to vascular calcification and therefore promote calciphylaxis. Thrombophilia is also a known risk factor of calciphylaxis. We were surprised to identify so many non-dialyzed patients with calciphylaxis. The frontier between uremic calciphylaxis and non-uremic calciphylaxis is difficult to define. Calciphylaxis seems to be the conjunction of multiple conditions (obesity, VKA, bone mineral disease abnormalities, uremia, inflammation) with a broad spectrum of variations. The mortality rate was particularly high in our study. Ulcerative lesions and proximal-type CUA, known as poor prognosis factors, were predominant.

• #31 Calciphylaxis: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1095481-overview

  Patients at an increased risk appear to be those who are obese and those who have been exposed to immunosuppressive agents, including glucocorticoids. […] Hypercoagulable conditions, including protein C and protein S deficiencies, vitamin K deficiency, and the presence of a circulating anticoagulant have been described in a number of patients. […] Vascular calcification is a constant finding in cases of calciphylaxis. Theoretically, various pathologic roles may be attributed to this vascular calcification, including the following: First, calcification of the vascular endothelium may alter the local interaction of procoagulant and anticoagulant factors, predisposing to a microenvironment of hypercoagulability […] The exact role of PTH is uncertain because calciphylaxis may occur after total parathyroidectomy, in the absence of measurable PTH levels.

• #32 Calciphylaxis – Calcific Uremic Arteriolopathy (CUA) Or “Grey Scale”

  https://www.icliniq.com/articles/kidney-and-urologic-diseases/calciphylaxis

  Increase in Blood Phosphorus Levels: Calcium and phosphorus together form crystals of calcium phosphate that can get deposited in parts of the body other than the bones. […] Certain Cancer Types: Calciphylaxis without kidney disease may have a cancer connection. […] Triggering factors that can lead to calciphylaxis include the following: Metal-Based Compounds: Metals like lead, aluminum, chromium, iron, or titanium can initiate calciphylaxis. […] Trauma: Injuries, medical or surgical procedures can trigger the condition. […] Calciphylaxis is characterized by painful skin lesions which are red, purple, or black and have a net-like appearance. These lesions can become hard and leathery. Some lesions can develop into wounds that are star-shaped and have a foul smell. […] Calciphylaxis is mainly due to deposits of calcium in the medial layer of blood vessels which leads to inflammation and a hypercoagulable state (increased blood clots), and the blood flow is reduced due to injury to the vessel walls and blood clot formation. Therefore all these events lead to the formation of ulceration, ischemia, and necrosis. This pathology depends on many factors such as increased level of calcium x phosphate, increased level of parathyroid hormone level, vitamin D levels, warfarin treatment, obesity, diabetes, and prolonged dialysis.

• #33 Calciphylaxis: Causes, Symptoms and Treatment – WoW Health

  https://www.mywowhealth.com/calciphylaxis-causes-symptoms-and-treatment/

  Calciphylaxis is a rare condition that can be deadly. It occurs when calcium builds up in the blood vessels, causing them to harden and narrow. This can lead to blockages and eventually death. There are several potential causes of calciphylaxis, including: Kidney failure: When the kidneys are not able to remove excess calcium from the blood, it can build up and cause calciphylaxis. Certain medications: Some medications, such as those used to treat osteoporosis, can increase the risk of calciphylaxis. Medical conditions: Conditions that affect calcium metabolism, such as hyperparathyroidism, can also lead to calciphylaxis. Injury or surgery: Any injury or surgery that damages blood vessels can increase the risk of calciphylaxis. […] Calciphylaxis is a rare condition that can cause death from a cut or bite. The condition is caused by the build-up of calcium in the blood vessels, which can lead to blockages and eventually death.

• #34 Calciphylaxis | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/calciphylaxis?lang=us

  Calciphylaxis, also known as calcific uremic arteriolopathy, is a rare condition that manifests as subcutaneous vascular calcification and cutaneous necrosis (small blood vessels of the fat tissue and the skin). Some authors describe it as a syndrome of vascular calcification, thrombosis and skin necrosis. […] It is a complex disorder with a multifactorial etiology. The exact pathogenesis of calciphylaxis is unclear. Medial calcification and intimal fibrosis of the cutaneous arterioles combined with thrombotic occlusion leading to ischemic skin necrosis is seen in calciphylaxis.

• #35 Atypical Wounds – Calciphylaxis | AccessMedicine Network

  http://www.accessmedicinenetwork.com/users/253399-rose-hamm/posts/atypical-wounds-calciphylaxis

  In the absence of end-stage renal disease, the disorder is classified as non-uremic calciphylaxis. […] The pathogenesis of calciphylaxis involves progressive narrowing of the cutaneous blood vessels as a result of calcification within the media layer of the vessel walls and proliferation of endothelial cells with fibrosis under the intima of the vessel. […] The vascular calcification is actually like ectopic bone formation in the vessel walls, which is related to hyperphosphatemia, hypercalcemia, and hyperglycemia. […] These same conditions that cause vessel calcification are the conditions that exist in the above mentioned risk factors. […] In summary, calciphylaxis is a disorder that causes calcification and thrombosis of the cutaneous blood vessels, resulting in painful ischemic skin and subcutaneous wounds that require a multidisciplinary approach for medical and wound management.

• #36 Calciphylaxis | 5-Minute Clinical Consult

  https://www.unboundmedicine.com/5minute/view/5-Minute-Clinical-Consult/816114/2.0/Calciphylaxis

  Pathogenesis is a two-step process involving initial obliterative medial wall calcification, followed by ischemic, occlusion, and tissue necrosis. […] Local trauma, hypotension, thrombosis, procoagulant states may trigger process. […] ESRD patients commonly develop vascular calcification in absence of calciphylaxis; mechanisms involve: […] Disturbances in Ca/P/parathyroid hormone (PTH) homeostasis have been implicated but are inconclusive. […] Obesity is an important etiologic factor.

• #37 Calciphylaxis | 5-Minute Clinical Consult

  https://www.unboundmedicine.com/5minute/view/5-Minute-Clinical-Consult/816114/2.0/Calciphylaxis

  Pathogenesis is a two-step process involving initial obliterative medial wall calcification, followed by ischemic, occlusion, and tissue necrosis. […] Local trauma, hypotension, thrombosis, procoagulant states may trigger process. […] ESRD patients commonly develop vascular calcification in absence of calciphylaxis; mechanisms involve: […] Disturbances in Ca/P/parathyroid hormone (PTH) homeostasis have been implicated but are inconclusive. […] Obesity is an important etiologic factor.

• #38 Calciphylaxis: A Review | JCAD – The Journal of Clinical and Aesthetic Dermatology

  https://jcadonline.com/calciphylaxis-a-review/

  Vascular calcification and thrombosis are both required to produce lesions of calciphylaxis. […] The key is to prevent patients with known risk factors from developing calciphylaxis. […] Calciphylaxis has a dismal prognosis with up to 80-percent mortality. […] The exact cause is unknown, but it is seen more commonly in patients with renal failure.

• #39 Calciphylaxis Prevention and Treatment Strategies- Clinical Advisor

  https://www.clinicaladvisor.com/features/calciphylaxis-prevention-treatment-strategies/

  A component of the pathogenesis may be dysregulation in the pathway involved in both bone and vascular calcification that includes receptor activator of nuclear factor-B, receptor activator of nuclear factor-B ligand, and osteoprotegerin. More research is needed to determine the exact pathway of calciphylaxis. […] Calciphylaxis appears to be multifactorial with unknown pathogenesis and, therefore, no serologic or hematologic confirmatory test is available. However, diagnostic tests that can be included in the initial workup to identify abnormalities include complete blood count, urea and creatinine, corrected calcium, phosphate, calcium-phosphorus index, PTH, coagulation profile, and thrombophilia screen. Identification and treatment of hypercoagulable states may be a method to reduce calciphylaxis incidence. A case-control study including 38 patients and 104 controls indicated that patients with calciphylaxis were significantly more likely to have lupus anticoagulant, protein C deficiency, or combined thrombophilia, compared with controls, and were also significantly more likely to have been exposed to warfarin. These findings indicate that the listed hypercoagulable states may contribute to the pathogenesis of calciphylaxis, and could be a possible screening tool for patients with chronic kidney disease and/or ESRD.

• #40 Calciphylaxis: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1095481-overview

  Patients at an increased risk appear to be those who are obese and those who have been exposed to immunosuppressive agents, including glucocorticoids. […] Hypercoagulable conditions, including protein C and protein S deficiencies, vitamin K deficiency, and the presence of a circulating anticoagulant have been described in a number of patients. […] Vascular calcification is a constant finding in cases of calciphylaxis. Theoretically, various pathologic roles may be attributed to this vascular calcification, including the following: First, calcification of the vascular endothelium may alter the local interaction of procoagulant and anticoagulant factors, predisposing to a microenvironment of hypercoagulability […] The exact role of PTH is uncertain because calciphylaxis may occur after total parathyroidectomy, in the absence of measurable PTH levels.

• #41 FF #325 Uremic Calciphylaxis – Palliative Care Network of Wisconsin

  https://www.mypcnow.org/fast-fact/uremic-calciphylaxis/?print=print

  Calciphylaxis is a poorly understood disorder in which calcification of small blood vessels causes painful ischemic skin and visceral lesions most often in patients with end-stage renal disease (ESRD). […] Risk factors include: female sex; Caucasian race; obesity; diabetes mellitus; hyperparathyroidism; albumin < 3; hypercoagulable states; and exposure to certain medications such as warfarin, iron, vitamin D, and corticosteroids. [...] Uremia, calcium products, and reactive oxygen species (ROS) associated with ESRD are thought to increase vascular calcium deposition and fibrosis, leading to calciphylaxis. [...] The one-year mortality rate for calciphylaxis is estimated to be 45-80%, which may be even higher when ulcerative skin lesions are present. [...] Ischemic complications and difficult to treat infections given incomplete antibiotic penetrance and poorly perfused tissues are potential mechanisms for the increased mortality risk.

• #42 Calciphylaxis: Causes, Symptoms, and Management — DermNet

  https://dermnetnz.org/topics/calciphylaxis

  Calciphylaxis is a condition characterised by necrosis (cellular death) of the skin and fatty tissue. It is seen mainly in patients with end-stage kidney disease. […] The cause of calciphylaxis is not properly understood. The primary event is occlusion of the small blood vessels in the skin by a thrombus (blood clot), which results in spreading ischaemia and skin necrosis. It is thought that the clots occur because of calcification within the walls of the blood vessels. […] Calciphylaxis can also occur in patients with normal kidney function. Causes of non-uraemic calciphylaxis include: Primary hyperparathyroidism, Malignancy, Alcoholic liver disease, Connective tissue disease, Diabetes mellitus. […] High levels of matrix metalloproteinases have been described, and one theory suggests chemically altered elastin protein allows deposition of calcium on small vessels.

• #43 Calciphylaxis – Inozyme

  https://www.inozyme.com/patients-and-families/calciphylaxis/

  Calciphylaxis is a rare disease linked to disruptions in the PPi-Adenosine Pathway, predominantly affecting patients with end-stage kidney disease (ESKD). […] Calciphylaxis, also known as calcific uremic arteriopathy, is a rare disease with a high mortality rate that mostly affects people with end-stage kidney disease (ESKD). It is characterized by painful sores and wounds that are hard to heal. […] In calciphylaxis, there is an imbalance in the molecules that control mineralization. One of these critical molecules is PPi. Low PPi contributes to hydroxyapatite crystal buildup inside the small blood vessels (arterioles) near the skin and adipose tissue. Overgrowth of smooth muscle cells due to low adenosine can further block the blood vessels. These issues lead to poor blood flow, blood clots, painful skin ulcers, serious infections, and in many cases, death.

• #44 Calciphylaxis – Inozyme

  https://www.inozyme.com/patients-and-families/calciphylaxis/

  Approximately 50% of calciphylaxis patients die within a year of diagnosis. […] Recent studies show that most people with calciphylaxis have abnormalities in blood-clotting factors. These abnormalities can lead to small blood clots forming more often than they normally would. Hydroxyapatite crystals are deposited in the smallest parts of the arteries, which also leads to the formation of blood clots. […] Serious infections occur due to wounds on the skin that spread and do not heal, which can lead to major complications including sepsis (blood infection), the most common cause of death in people with calciphylaxis.

• #45 Calciphylaxis | 5-Minute Clinical Consult

  https://www.unboundmedicine.com/5minute/view/5-Minute-Clinical-Consult/816114/2.0/Calciphylaxis

  Pathogenesis is a two-step process involving initial obliterative medial wall calcification, followed by ischemic, occlusion, and tissue necrosis. […] Local trauma, hypotension, thrombosis, procoagulant states may trigger process. […] ESRD patients commonly develop vascular calcification in absence of calciphylaxis; mechanisms involve: […] Disturbances in Ca/P/parathyroid hormone (PTH) homeostasis have been implicated but are inconclusive. […] Obesity is an important etiologic factor.

• #46 Calciphylaxis | UK Kidney Association

  https://www.ukkidney.org/rare-renal/patient-information-0/calciphylaxis

  Calciphylaxis is a serious condition that can occur in people chronic kidney disease; it is most commonly seen in people receiving dialysis but remains very rare with approximately one case diagnosed in every 600 people, having dialysis, per year. […] The exact cause of calciphylaxis remains unknown; we do not know why some people get the condition and others do not. Possible risk factors (things that may contribute to the development of calciphylaxis) are considered to be: […] The trigger for the disease is not known but may include local injury; for example, people can report their calciphylaxis started with a flee bite, cut or graze to the area. […] What risk factors are associated with development and progression of calciphylaxis?

• #47 Calciphylaxis: Symptoms & Causes

  https://my.clevelandclinic.org/health/diseases/22359-calciphylaxis

  Calciphylaxis is a rare, painful disease that happens most often in people with moderate to severe kidney problems. It happens when calcium deposits form in your blood vessels and block blood flow to areas of your skin. This can lead to open wounds that are prone to dangerous (or even deadly) infections. […] The exact cause of calciphylaxis remains unknown, but research suggests it takes more than one factor to cause it. These factors can team up to become calciphylaxis causes. […] These factors include: Too much parathyroid hormone. When you’re having kidney problems, your parathyroid glands try to keep the calcium levels in your blood stable. In doing this, they produce too much of a hormone your body needs (hyperparathyroidism). This can also happen when you aren’t getting enough vitamin D or calcium.

• #48 Calciphylaxis and Hypotension: Report of a Case and Review of the Literature

  https://www.heraldopenaccess.us/openaccess/calciphylaxis-and-hypotension-report-of-a-case-and-review-of-the-literature

  Thus, beyond the more obvious decrease in local cutaneous perfusion, hypotension contributes to calciphylaxis progression through contribution to vascular calcification as well. […] This multifactorial effect of hemodynamic instability becomes particularly relevant in patients such as ours with end stage renal disease on hemodialysis who may develop post-dialysis hypotension. […] The exact pathogenesis of calciphylaxis remains unclear and is the topic of continued research and debate. As such, new theories continue to emerge, with a recurrent theme of hypotension speckling the literature.

• #49 Calciphylaxis | Kidney Care UK

  https://kidneycareuk.org/kidney-disease-information/kidney-conditions/calciphylaxis/

  Calciphylaxis (also known as calcific uraemic arteriolopathy) is a rare condition that can develop in people receiving dialysis for kidney failure. […] The exact cause of calciphylaxis is currently unknown. It is sometimes triggered by a small injury such as an insect bite or a cut. Other risk factors that appear to make people more likely to develop calciphylaxis include: being overweight, having high levels of calcium and/or phosphate in the blood, being on dialysis for a long period of time, having diabetes, taking warfarin (a medication to thin the blood). […] Calciphylaxis is not a genetic condition, so it does not run in families.

• #50 Calciphylaxis | UK Kidney Association

  https://www.ukkidney.org/rare-renal/patient-information-0/calciphylaxis

  Calciphylaxis is a serious condition that can occur in people chronic kidney disease; it is most commonly seen in people receiving dialysis but remains very rare with approximately one case diagnosed in every 600 people, having dialysis, per year. […] The exact cause of calciphylaxis remains unknown; we do not know why some people get the condition and others do not. Possible risk factors (things that may contribute to the development of calciphylaxis) are considered to be: […] The trigger for the disease is not known but may include local injury; for example, people can report their calciphylaxis started with a flee bite, cut or graze to the area. […] What risk factors are associated with development and progression of calciphylaxis?

• #51 Calciphylaxis epidemiology, risk factors, treatment and survival among French chronic kidney disease patients: a case-control study | BMC Nephrology | Full Text

  https://bmcnephrol.biomedcentral.com/articles/10.1186/s12882-020-01722-y

  Calcific uremic arteriolopathy (CUA), also called calciphylaxis, is a rare but devastating disease involving patients with end stage renal disease (ESRD). CUA causes painful skin lesions that evolve to ulcerative lesions at risk of superinfection and sepsis, with a poor prognosis. Reported risk factors of CUA are female sex, obesity, diabetes mellitus, vitamin K antagonists (VKA) and ESRD. Dysregulation of calcium-phosphate metabolism also participates to its development. Risks factors of developing CUA among dialysis patients were obesity, VKA, weight loss, serum albumin decrease or high serum phosphate in the 6 months before lesion onset. CUA involved mainly obese patients under VKA. Malnutrition and inflammation preceded the onset of skin lesions and could be warning signs among dialysis patients at risk. The association of CUA and VKA therapy has already been reported. By decreasing carboxylated matrix Gla Protein (cMGP), VKA are suspected to contribute to vascular calcification and therefore promote calciphylaxis. Thrombophilia is also a known risk factor of calciphylaxis. We were surprised to identify so many non-dialyzed patients with calciphylaxis. The frontier between uremic calciphylaxis and non-uremic calciphylaxis is difficult to define. Calciphylaxis seems to be the conjunction of multiple conditions (obesity, VKA, bone mineral disease abnormalities, uremia, inflammation) with a broad spectrum of variations. The mortality rate was particularly high in our study. Ulcerative lesions and proximal-type CUA, known as poor prognosis factors, were predominant.

• #52 Calciphylaxis and Hypotension: Report of a Case and Review of the Literature

  https://www.heraldopenaccess.us/openaccess/calciphylaxis-and-hypotension-report-of-a-case-and-review-of-the-literature

  Thus, beyond the more obvious decrease in local cutaneous perfusion, hypotension contributes to calciphylaxis progression through contribution to vascular calcification as well. […] This multifactorial effect of hemodynamic instability becomes particularly relevant in patients such as ours with end stage renal disease on hemodialysis who may develop post-dialysis hypotension. […] The exact pathogenesis of calciphylaxis remains unclear and is the topic of continued research and debate. As such, new theories continue to emerge, with a recurrent theme of hypotension speckling the literature.

• #53 Calciphylaxis: Symptoms, causes, and treatment

  https://www.medicalnewstoday.com/articles/calciphylaxis

  The mortality rate for calciphylaxis is around 30% after 6 months and 50% at 12 months. Research suggests that septicemia, or blood poisoning, due to wound infection is the leading cause of death in people with calciphylaxis. […] Medical professionals may monitor and attempt to control the mineral and calcium levels in someone with end stage kidney disease to avoid calciphylaxis.

• #54 Calciphylaxis Prevention and Treatment Strategies- Clinical Advisor

  https://www.clinicaladvisor.com/features/calciphylaxis-prevention-treatment-strategies/

  A component of the pathogenesis may be dysregulation in the pathway involved in both bone and vascular calcification that includes receptor activator of nuclear factor-B, receptor activator of nuclear factor-B ligand, and osteoprotegerin. More research is needed to determine the exact pathway of calciphylaxis. […] Calciphylaxis appears to be multifactorial with unknown pathogenesis and, therefore, no serologic or hematologic confirmatory test is available. However, diagnostic tests that can be included in the initial workup to identify abnormalities include complete blood count, urea and creatinine, corrected calcium, phosphate, calcium-phosphorus index, PTH, coagulation profile, and thrombophilia screen. Identification and treatment of hypercoagulable states may be a method to reduce calciphylaxis incidence. A case-control study including 38 patients and 104 controls indicated that patients with calciphylaxis were significantly more likely to have lupus anticoagulant, protein C deficiency, or combined thrombophilia, compared with controls, and were also significantly more likely to have been exposed to warfarin. These findings indicate that the listed hypercoagulable states may contribute to the pathogenesis of calciphylaxis, and could be a possible screening tool for patients with chronic kidney disease and/or ESRD.

• #55 Update on Calciphylaxis Etiopathogenesis, Diagnosis, and Management | MDedge

  https://blogs.the-hospitalist.org/content/update-calciphylaxis-etiopathogenesis-diagnosis-and-management

  The limited understanding of the etiopathogenesis of calciphylaxis and the lack of data on its management are reflected in the limited treatment options for the disease (Table 3).2,5,9 There are no formal algorithms for the treatment of calciphylaxis. Therapeutic trials are scarce, and most of the current treatment recommendations are based on small retrospective reports or case series. Sodium thiosulfate has been the most widely used treatment option since 2004, when its use in calciphylaxis was first reported.31 Sodium thiosulfate chelates calcium and is thought to have antioxidant and vasodilatory properties.32 There are a few promising clinical trials and large-scale studies (Table 4) that aim to evaluate the efficacy of existing treatments (eg, sodium thiosulfate) as well as novel treatment options such as lanthanum carbonate, SNF472 (hexasodium phytate), and vitamin K.33-36

• #56 Update on Calciphylaxis Etiopathogenesis, Diagnosis, and Management | MDedge

  https://blogs.the-hospitalist.org/content/update-calciphylaxis-etiopathogenesis-diagnosis-and-management

  The limited understanding of the etiopathogenesis of calciphylaxis and the lack of data on its management are reflected in the limited treatment options for the disease (Table 3).2,5,9 There are no formal algorithms for the treatment of calciphylaxis. Therapeutic trials are scarce, and most of the current treatment recommendations are based on small retrospective reports or case series. Sodium thiosulfate has been the most widely used treatment option since 2004, when its use in calciphylaxis was first reported.31 Sodium thiosulfate chelates calcium and is thought to have antioxidant and vasodilatory properties.32 There are a few promising clinical trials and large-scale studies (Table 4) that aim to evaluate the efficacy of existing treatments (eg, sodium thiosulfate) as well as novel treatment options such as lanthanum carbonate, SNF472 (hexasodium phytate), and vitamin K.33-36

• #57 Case Report: Refractory Calciphylaxis in Lupus – The Rheumatologist

  https://www.the-rheumatologist.org/article/case-report-refractory-calciphylaxis-in-lupus/?singlepage=1

  Unfortunately, none of these models explain calciphylaxis in patients without any evidence of calcium homeostasis imbalance. […] To adequately treat calciphylaxis, clinicians must perform an evaluation for reversible calciphylaxis causes. Workups should include checking calcium, phosphate and PTH levels. Also perform a thorough evaluation for malignancies and connective tissue diseases. […] The most widely used therapy is sodium thiosulfate. Its thought that sodium thiosulfate works by removing calcium through chelation. […] In patients with hyperparathyroidism, calcimimetics, such as cinacalcet, have shown much success in treating this rare, deadly illness. […] Calciphylaxis remains poorly understood with poor outcomes. Cases of non-uremic calciphylaxis remain rare. Rarer still are reports of calciphylaxis in patients with an underlying connective tissue disease.

• #58 Update on Calciphylaxis Etiopathogenesis, Diagnosis, and Management | MDedge

  https://blogs.the-hospitalist.org/content/update-calciphylaxis-etiopathogenesis-diagnosis-and-management

  The limited understanding of the etiopathogenesis of calciphylaxis and the lack of data on its management are reflected in the limited treatment options for the disease (Table 3).2,5,9 There are no formal algorithms for the treatment of calciphylaxis. Therapeutic trials are scarce, and most of the current treatment recommendations are based on small retrospective reports or case series. Sodium thiosulfate has been the most widely used treatment option since 2004, when its use in calciphylaxis was first reported.31 Sodium thiosulfate chelates calcium and is thought to have antioxidant and vasodilatory properties.32 There are a few promising clinical trials and large-scale studies (Table 4) that aim to evaluate the efficacy of existing treatments (eg, sodium thiosulfate) as well as novel treatment options such as lanthanum carbonate, SNF472 (hexasodium phytate), and vitamin K.33-36

• #59 Seeking a Treatment for Calciphylaxis | National Kidney Foundation

  https://www.kidney.org/seeking-treatment-calciphylaxis

  Calciphylaxis is a type of vascular calcification generally seen in patients with kidney failure. The condition causes skin lesions and severe pain and usually has a high mortality rate. Typically, patients diagnosed with calciphylaxis live about six months. […] „As we map the biology, risk factors, prevention and eventually, treatment of calciphylaxis, we believe it will lead to a number of advancements in understanding the mechanisms of vascular calcification.” […] Vitamin K has been shown to have calcification-inhibitory properties. However, dialysis patients are usually vitamin K deficient due to recommended dietary restrictions aimed at restricting potassium and sodium-rich foods that are also rich sources of vitamin K. […] „Once we learn the biology, this information will inform treatments for other types of vascular calcifications in blood vessels found in the brain, heart and other parts of the body,” Dr. Nigwekar said.

• #60

  https://www.kidney.org.uk/calciphylaxis-information

  Calciphylaxis is a serious condition that can occur in people chronic with kidney disease; it is most commonly seen in people receiving dialysis but remains very rare with approximately one case diagnosed in every 600 people, having dialysis, per year. […] The exact cause of calciphylaxis remains unknown; we do not know why some people get the condition and others do not. Possible risk factors (things that may contribute to the development of calciphylaxis) are considered to be: […] The trigger for the disease is not known but may include local injury; for example, people can report their calciphylaxis started with a flea bite, cut or graze to the area. […] There is no specific treatment as yet that is shown to work. Your doctor will try and individualise your care to reduce your risk factors. This may include improving your calcium and phosphate levels or stopping medications such as warfarin. […] What risk factors are associated with development and progression of calciphylaxis? […] The rarity of the condition has limited the ability to do large research studies.

Zobacz więcej