词条 | Calciphylaxis |
释义 |
| name = Calciphylaxis | image = Calciphylaxis.png | caption = Calciphylaxis on the abdomen of a patient with end stage kidney disease. Markings are in cm. | | pronounce = | field = | synonyms = | symptoms = | complications = | onset = | duration = | types = | causes = | risks = | diagnosis = | differential = | prevention = | treatment = | medication = | prognosis = 1- and 5-year survival rates are estimated to be 45% and 35%, respectively | frequency = | deaths = }}Calciphylaxis, also known as calcific uremic arteriolopathy (CUA), is a rare painful syndrome of calcification of the small blood vessels located within the fatty tissue and deeper layers of the skin, blood clots, and the death of skin cells due to too little blood flow.[1] It is seen mostly in people with end-stage kidney disease but can occur in the earlier stages of chronic kidney disease and rarely in people with normally functioning kidneys.[1] It results in chronic non-healing wounds and is usually fatal. Calciphylaxis is a rare but serious disease, believed to affect 1-4% of all dialysis patients.[2] Calciphylaxis is one type of extraskeletal calcification. Similar extraskeletal calcifications are observed in some people with high levels of calcium in the blood, including people with milk-alkali syndrome, sarcoidosis, primary hyperparathyroidism, and hypervitaminosis D. Certain medications such as warfarin can also result in calciphylaxis in rare cases. The presence of calciphylaxis generally predicts a poor prognosis with a typical life expectancy of less than one year.[1] Signs and symptomsThe first skin changes in calciphylaxis lesions are mottling of the skin and induration in a livedo reticularis pattern. As tissue thrombosis and infarction occurs, a black, leathery eschar in an ulcer with adherent black slough are found. Surrounding the ulcers is usually a plate-like area of indurated skin.[3] These lesions are always extremely painful and most often occur on the lower extremities, abdomen, buttocks, and penis. Because the tissue has infarcted, wound healing seldom occurs, and ulcers are more likely to become secondarily infected. Many cases of calciphylaxis end with systemic bacterial infection and death.[4] Calciphylaxis is characterized by the following histologic findings:
===Heart of stone=== Severe forms of calciphylaxis may cause diastolic heart failure from cardiac calcification, called heart of stone.[5] CauseThe cause of calciphylaxis is unknown. It does not seem to be an immune type reaction. In other words, calciphylaxis is not a hypersensitivity reaction (i.e., allergic reaction) leading to sudden local calcification. Clearly, additional factors are involved in calciphylaxis. It is also known as calcific uremic arteriolopathy; however, the disease is not limited to patients with kidney failure. The current belief is that in end-stage kidney disease, abnormal calcium and phosphate homeostasis result in the deposition of calcium in the vessels, also known as metastatic calcification. Once the calcium has been deposited, a thrombotic event occurs within the lumen of these vessels, resulting in tissue infarction. It is unknown what the triggers are that cause the thrombotic and ischemic event.[6] Reported risk factors include female sex, obesity, elevated calcium-phosphate product, medications such as warfarin, vitamin D derivatives e.g. calcitriol, calcium-based binders, or systemic steroids, protein C or S deficiency, low blood albumin levels, and diabetes mellitus.[7] DiagnosisThere is no diagnostic test for calciphylaxis. The diagnosis is a clinical one. The characteristic lesions are the ischemic skin lesions (usually with areas of skin necrosis). The necrotic skin lesions (i.e. the dying or already dead skin areas) typically appear as violaceous (dark bluish purple) lesions and/or completely black leathery lesions. They can be extensive. The suspected diagnosis can be supported by a skin biopsy. It shows arterial calcification and occlusion in the absence of vasculitis. Sometimes the bone scintigraphy can show increased tracer accumulation in the soft tissues.[8] In certain patients, anti-nuclear antibody may play a role.[9] TreatmentThe optimal treatment is prevention. Rigorous and continuous control of phosphate and calcium balance most probably will avoid the metabolic changes which may lead to calciphylaxis. There is no specific treatment. Of the treatments that exist, none are internationally recognized as the standard of care. An acceptable treatment could include:
PrognosisUnfortunately, response to treatment is not guaranteed. Also, the necrotic skin areas may get infected, and this then may lead to sepsis in some patients. Overall, the clinical prognosis remains poor. EpidemiologyCalciphylaxis most commonly occurs in patients with end-stage renal disease who are on hemodialysis or who have recently received a renal (kidney) transplant. Yet calciphylaxis does not occur only in end-stage renal disease patients. When reported in patients without end-stage renal disease, it is called non-uremic calciphylaxis by Nigwekar et al.[12] Non-uremic calciphylaxis has been observed in patients with primary hyperparathyroidism, breast cancer (treated with chemotherapy), liver cirrhosis (due to alcohol abuse), cholangiocarcinoma, Crohn's disease, rheumatoid arthritis (RA), and systemic lupus erythematosus (SLE). References1. ^1 2 {{cite journal|last1=Nigwekar|first1=SU|last2=Thadhani|first2=R|last3=Brandenburg|first3=VM|title=Calciphylaxis|journal=New England Journal of Medicine|date=May 2018|volume=378|issue=18|pages=1704–1714|doi=10.1056/NEJMra1505292|pmid=29719190}} 2. ^{{cite journal |last1=Angelis |first1=M |last2=Wong |first2=LM |last3=Wong |first3=LL |last4=Myers |first4=S |title=Calciphylaxis in patients on hemodialysis: A prevalence study |journal=Surgery |date=1997 |volume=122 |issue=6 |pages=1083–1090 |doi=10.1016/S0039-6060(97)90212-9 |pmid=9426423}} 3. ^{{cite journal |author1=Zhou Qian |author2=Neubauer Jakob |author3=Kern Johannes S |author4=Grotz Wolfgang |author5=Walz Gerd |author6=Huber Tobias B | year = 2014 | title = Calciphylaxis| url = | journal = The Lancet | volume = 383 | issue = 9922| page = 1067 | doi = 10.1016/S0140-6736(14)60235-X |pmid=24582472 }} 4. ^{{cite book |last1=Wolff |first1=Klaus |last2=Johnson |first2=Richard |last3=Saavedra |first3=Arturo |title=Fitzpatrick's Color Atlas and Synopsis of Clinical Dermatology |publisher=McGraw Hill |isbn=978-0-07-179302-5 |page=429 |edition=7th|date=2013-03-06 }} 5. ^Heart of Stone - CINDY W. T OM, MD, ANDDEEPAKR. TALREJA, MD. Division of Cardiovascular Diseases, Mayo Clinic College of Medicine, Rochester, Minn 6. ^{{cite journal|last1=Wilmer|first1=William|last2=Magro|first2=Cynthia|title=Calciphylaxis: Emerging Concepts in Prevention, Diagnosis, and Treatment|journal=Seminars in Dialysis|date=2002|volume=15|issue=3|pages=172–186|pmid=12100455|doi=10.1046/j.1525-139X.2002.00052.x}} 7. ^{{cite journal|last1=Arseculeratne|first1=G|last2=Evans|first2=AT|last3=Morley|first3=SM|title=Calciphylaxis – a topical overview|journal=Journal of the European Academy of Dermatology and Venereology|date=2006|volume=20|issue=5|pages=493–502|doi=10.1111/j.1468-3083.2006.01506.x|pmid=16684274}} 8. ^{{cite journal |vauthors=Araya CE, Fennell RS, Neiberger RE, Dharnidharka VR |title=Sodium thiosulfate treatment for calcific uremic arteriolopathy in children and young adults |journal=Clin J Am Soc Nephrol |volume=1 |issue=6 |pages=1161–6 |year=2006 |pmid=17699342 |doi=10.2215/CJN.01520506 |url=http://cjasn.asnjournals.org/cgi/content/full/1/6/1161}} 9. ^{{cite journal |vauthors=Rashid RM, Hauck M, Lasley M | date = Nov 2008 | title = Anti-nuclear antibody: a potential predictor of calciphylaxis in non-dialysis patients | url = | journal = J Eur Acad Dermatol Venereol | volume = 22 | issue = 10| pages = 1247–8 | doi=10.1111/j.1468-3083.2008.02606.x| pmid = 18422539 }} 10. ^{{cite journal |vauthors=Edsell ME, Bailey M, Joe K, Millar I |title=Hyperbaric oxygen therapy in the treatment of skin ulcers due to calcific uraemic arteriolopathy: experience from an Australian hyperbaric unit. |journal=Diving and Hyperbaric Medicine |year=2008 |volume=38 |issue=3 |pages=139–44 |url=http://archive.rubicon-foundation.org/10189 |accessdate=2013-04-02}} 11. ^Cai MM, Smith ER, Brumby C, McMahon LP, Holt SG.Nephrology (Carlton). 2013 Nov;18(11):724-7. {{PMID|24571743}} {{doi|10.1111/nep.12137}}. 12. ^{{cite journal |vauthors=Nigwekar SU, Wolf M, Sterns RH, Hix JK | date = Jul 2008 | title = Calciphylaxis from nonuremic causes: a systematic review | url = | journal = Clin J Am Soc Nephrol | volume = 3 | issue = 4| pages = 1139–43 | doi=10.2215/cjn.00530108| pmid = 18417747 | pmc = 2440281 }} Further reading
External links{{Medical resources| DiseasesDB = 1897 | ICD10 = | ICD9 = {{ICD9|275.49}} | ICDO = | OMIM = | MedlinePlus = | eMedicineSubj = derm | eMedicineTopic = 555 | MeshID = D002115 }}{{Inborn errors of metal metabolism}} 3 : Vascular-related cutaneous conditions|Nephrology|Ailments of unknown cause |
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