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Calcium pyrophosphate dihydrate crystal deposition disease

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Flares are sudden, severe and without warning. Diet does not appear to cause flares. Overexertion of any exercise, standing too long, shopping, stressful or loud environments, can or may lead to severe flares, which can last from one hour to months. Although, in some patient interviews, alcohol
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within the affected joint, indicating a substantial amount of calcium crystal deposition within the cartilage or ligaments. Ultrasound is a reliable method to diagnose CPPD. Using ultrasound, chondrocalcinosis may be depicted as echogenic foci with no acoustic shadow within the hyaline cartilage or
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Zhang W, Doherty M, Bardin T, Barskova V, Guerne PA, Jansen TL, Leeb BF, Perez-Ruiz F, Pimentao J, Punzi L, Richette P, Sivera F, Uhlig T, Watt I, Pascual E. European League Against Rheumatism recommendations for calcium pyrophosphate deposition. Part I: terminology and diagnosis. Ann Rheum Dis.
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NSAIDs, Colchicine, and methotrexate may provide initial relief. There is currently no treatment for non-invasive removal of these crystals once they are deposited. Attempts to dissolve crystals in situ using enzymes turned up to be a "clinical failure". New, innovative methods using catalytic
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The symptoms can be monoarticular (involving a single joint) or polyarticular (involving several joints). Symptoms usually last for days to weeks, and often recur. Although any joint may be affected, the knees, wrists, and hips are most common.
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on polarized light microscopy, and this method remains the most reliable method of identifying the crystals under the microscope. However, even this method has poor sensitivity, specificity, and inter-operator agreement.
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CPPD is estimated to affect 4% to 7% of the adult populations of Europe and the United States. Previous studies have overestimated the prevalence by simply estimating the prevalence of
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The disease is defined by presence of joint inflammation and the presence of CPPD crystals within the joint. The crystals are usually detected by imaging and/or joint fluid analysis.
186:(ATP; the molecule used as energy currency in all living things), which results in increased pyrophosphate levels in joints, is thought to be one reason why crystals may develop. 338:. In general, NSAIDs are administered in low doses to help prevent CPPD. However, if an acute attack is already occurring, higher doses are administered. If nothing else works, 294:, or removing synovial fluid from the affected joint, is performed to test the synovial fluid for the calcium pyrophosphate crystals that are present in CPPD. When stained with 1389: 326:
Because any medication that could reduce the inflammation of CPPD bears a risk of causing organ damage, treatment is not advised if the condition is not causing pain. For
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X-ray, CT, or other imaging usually shows accumulation of calcium within the joint cartilage, known as chondrocalcinosis. There can also be findings of
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It may cause considerable pain, but it is never fatal. Women are at a slightly higher risk than men, with an estimated ratio of occurrence of 1.4:1.
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or radiographic findings are most prominent. A task force of the European League Against Rheumatism (EULAR) made recommendations on preferred
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CPPD crystals appear as shattered glass under the microscope. When released into the synovial fluid, it causes unbearable pain to the patient.
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may provide relief. Research into surgical removal of calcifications is underway, however, this still remains an experimental procedure.
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Chrondocalcinosis may be extremely common in the population. CPPD flares may also be triggered by joint trauma from previous surgeries.
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Arend CF. Ultrasound of the Shoulder. Master Medical Books, 2013. Free chapter on acromioclavicular chondrocalcinosis is available at
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These two modalities currently define CPPD disease, but lack diagnostic accuracy. Thus, the diagnosis of CPPD disease is potentially
569:(CPPD) is an umbrella term for the various clinical subsets, whose naming reflects an emphasis on particular features. For example, 1683: 279: 1204: 331: 1693: 1281: 1688: 526: 465: 1519: 778:
Tsui FW (April 2012). "Genetics and mechanisms of crystal deposition in calcium pyrophosphate deposition disease".
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Polarized light microscopy of CPPD, showing rhombus-shaped calcium pyrophosphate crystals with positive
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is most commonly affected. The disease is metabolic in origin and its treatment remains symptomatic.
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Filippou G, Adinolfi A, Iagnocco A, Filippucci E, Cimmino MA, Bertoldi I, et al. (June 2016).
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pseudogout, treatments include intra-articular corticosteroid injection, systemic corticosteroids,
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forms are rare. One genetic study found an association between CPPD and a region of chromosome 8q.
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is involved in crystal-related inflammatory reactions and inorganic phosphate transport.
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When symptomatic, the disease classically begins with symptoms that are similar to a
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Artistic depiction of pseudogout crystals (calcium pyrophosphate dihydrate crystals)
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Baldwin CT, Farrer LA, Adair R, Dharmavaram R, Jimenez S, Anderson L (March 1995).
787: 645: 637: 327: 161: 1448: 1300: 626:"Calcium pyrophosphate crystal deposition is not always 'wear and tear' or aging" 1570: 1355: 1349: 1057:"Crystal arthritis: calcium pyrophosphate deposition-nothing 'pseudo' about it!" 213: 1640: 1422: 1311: 896: 879: 598: 594: 291: 157: 1261: 1257: 1137: 791: 1672: 1427: 1417: 589:, on the other hand, refers to the radiographic evidence of calcification in 307: 249:
fibrocartilage. By x-ray, CPPD can appear similar to other diseases such as
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CPPD crystal deposition disease was originally described over 50 years ago.
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Piast RW, Wieczorek RM, Marzec N, Garstka M, Misicka A (September 2021).
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CPPD crystals are more clearly visualized on light microscopy without a
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disease which is thought to be secondary to abnormal accumulation of
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Rothschild BM, Bruno MA (9 April 2021). Coombs BD, Keats TE (eds.).
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Rothschild BM, Bruno MA (7 June 2022). Coombs BD, Keats TE (eds.).
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The cause of CPPD disease is unknown. Increased breakdown of
415:"Calcium pyrophosphate dihydrate crystal deposition disease" 1509: 1118: 578: 254: 196: 102: 90: 302:"). However, CPP crystals are much better known for their 1560: 756:"Calcium Pyrophosphate Deposition Disease (rheumatology)" 1195:
Longmore M, Wilkinson I, Turmezei T, Cheung CK (2007).
298:, calcium pyrophosphate crystals appears deeply blue (" 682:"Calcium Pyrophosphate Deposition Disease (radiology)" 573:
refers to the acute symptoms of joint inflammation or
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inability to walk or perform everyday tasks or hobbies
1215: 577:: red, tender, and swollen joints that may resemble 365:, which is found in many other conditions as well. 89:dihydrate crystals within joint soft tissues. The 1670: 1050: 1048: 1046: 873: 871: 753: 697: 679: 1003:"Identification of crystals in synovial fluid" 358:The condition is more common in older adults. 1383: 1095: 1054: 1043: 749: 747: 745: 623: 1592:Systemic-onset juvenile idiopathic arthritis 1188: 868: 743: 741: 739: 737: 735: 733: 731: 729: 727: 725: 700:"Update on calcium pyrophosphate deposition" 675: 673: 671: 669: 619: 617: 1168: 1166: 819: 817: 1390: 1376: 1000: 934: 827: 773: 771: 769: 507:. Please do not remove this message until 1172: 1146: 1136: 1072: 1026: 996: 994: 905: 895: 851: 722: 666: 649: 614: 527:Learn how and when to remove this message 466:Learn how and when to remove this message 1163: 814: 581:arthritis (a similar condition in which 553:are associated with a range of clinical 503:Relevant discussion may be found on the 278: 259: 212: 171: 138:gnawing/chewing sensations in the joints 53: 766: 605:refers to several of these situations. 22:Calcium pyrophosphate dihydrate disease 1671: 1397: 1185:This topic last updated: Jul 24, 2018. 991: 704:Clinical and Experimental Rheumatology 1371: 624:Wright GD, Doherty M (October 1997). 334:(NSAIDs), or, on occasion, high-dose 332:non-steroidal anti-inflammatory drugs 96: 1197:Oxford Handbook of Clinical Medicine 1173:Rosenthal AK (2021). Post TW (ed.). 777: 693: 691: 477: 404:adding citations to reliable sources 375: 1102:Journal of Musculoskeletal Medicine 13: 1055:Rosenthal AK, Ryan LM (May 2011). 979:. American College of Rheumatology 940: 840:American Journal of Human Genetics 585:are deposited within the joints). 14: 1705: 688: 267:showing crystal deposition in an 1007:Annals of the Rheumatic Diseases 630:Annals of the Rheumatic Diseases 601:features are the most apparent. 567:calcium pyrophosphate deposition 482: 380: 35: 1112: 1098:"All about gout and pseudogout" 1089: 965: 548:Calcium pyrophosphate dihydrate 391:needs additional citations for 353: 228:Medical imaging, consisting of 63:Calcium pyrophosphate dihydrate 1684:Inflammatory polyarthropathies 1096:Emkey GR, Reginato AM (2009). 922: 698:Abhishek A, Doherty M (2016). 542: 119:swelling of one or more joints 1: 1587:Juvenile idiopathic arthritis 1001:Dieppe P, Swan A (May 1999). 608: 350:peptides are in development. 1061:Nature Reviews. Rheumatology 884:Osteoarthritis and Cartilage 780:Current Rheumatology Reports 321: 205: 7: 1694:Crystal deposition diseases 1616:Adult-onset Still's disease 1553:Connective tissue disorders 943:"calcinosis_cutis_2_060122" 509:conditions to do so are met 10: 1710: 897:10.1016/j.joca.2016.01.136 371: 71:crystal deposition disease 1689:Musculoskeletal disorders 1628: 1579: 1551: 1518: 1489: 1466: 1457: 1436: 1405: 1321: 1219: 1138:10.3390/molecules26195777 792:10.1007/s11926-011-0230-6 603:Pyrophosphate arthropathy 583:monosodium urate crystals 105:attack (thus the moniker 79:pyrophosphate arthropathy 43: 34: 26: 21: 1543:Enteropathic arthropathy 1183:. Waltham, MA: UpToDate. 306:shape and weak positive 167: 153:may be a known trigger. 1199:. Oxford. p. 841. 1074:10.1038/nrrheum.2011.50 977:Rheumatology Image Bank 164:count is often raised. 1611:Palindromic rheumatism 1538:Ankylosing spondylitis 1481:Tuberculosis arthritis 288: 276: 251:ankylosing spondylitis 225: 184:adenosine triphosphate 179: 128:feeling of malaise or 59: 710:(4 Suppl 98): 32–38. 642:10.1136/ard.56.10.586 282: 263: 216: 177:Calcium pyrophosphate 175: 87:calcium pyrophosphate 57: 1599:Rheumatoid arthritis 1019:10.1136/ard.58.5.261 400:improve this article 1533:Psoriatic arthritis 496:of this section is 316:epiphenomenological 269:intervertebral disc 1566:systemic sclerosis 1528:Reactive arthritis 1399:Diseases of joints 1322:External resources 953:on 5 February 2007 340:hydroxychloroquine 289: 277: 226: 180: 109:). These include: 97:Signs and symptoms 60: 1666: 1665: 1624: 1623: 1499:Chondrocalcinosis 1365: 1364: 1206:978-0-19-856837-7 587:Chondrocalcinosis 537: 536: 529: 476: 475: 468: 450: 363:chondrocalcinosis 246:chondrocalcinosis 222:chondrocalcinosis 52: 51: 16:Medical condition 1701: 1651:Bouchard's nodes 1604:Felty's syndrome 1476:Septic arthritis 1464: 1463: 1392: 1385: 1378: 1369: 1368: 1217: 1216: 1211: 1210: 1192: 1186: 1184: 1170: 1161: 1160: 1150: 1140: 1116: 1110: 1109: 1093: 1087: 1086: 1076: 1052: 1041: 1040: 1030: 998: 989: 988: 986: 984: 969: 963: 962: 960: 958: 949:. Archived from 938: 932: 926: 920: 919: 909: 899: 875: 866: 865: 855: 831: 825: 821: 812: 811: 775: 764: 763: 751: 720: 719: 695: 686: 685: 677: 664: 663: 653: 621: 565:. Accordingly, 532: 525: 521: 518: 512: 486: 485: 478: 471: 464: 460: 457: 451: 449: 408: 384: 376: 162:white blood cell 73:, also known as 39: 19: 18: 1709: 1708: 1704: 1703: 1702: 1700: 1699: 1698: 1669: 1668: 1667: 1662: 1646:Heberden's node 1629:Noninflammatory 1620: 1575: 1547: 1514: 1485: 1453: 1449:Joint stiffness 1432: 1401: 1396: 1366: 1361: 1360: 1317: 1316: 1228: 1214: 1207: 1193: 1189: 1171: 1164: 1117: 1113: 1094: 1090: 1053: 1044: 999: 992: 982: 980: 971: 970: 966: 956: 954: 939: 935: 927: 923: 876: 869: 832: 828: 824:2011;70(4):563. 822: 815: 776: 767: 752: 723: 696: 689: 678: 667: 636:(10): 586–588. 622: 615: 611: 545: 533: 522: 516: 513: 502: 487: 483: 472: 461: 455: 452: 409: 407: 397: 385: 374: 356: 324: 220:of a knee with 208: 170: 99: 17: 12: 11: 5: 1707: 1697: 1696: 1691: 1686: 1681: 1664: 1663: 1661: 1660: 1659: 1658: 1653: 1648: 1641:Osteoarthritis 1638: 1632: 1630: 1626: 1625: 1622: 1621: 1619: 1618: 1613: 1608: 1607: 1606: 1596: 1595: 1594: 1583: 1581: 1577: 1576: 1574: 1573: 1568: 1563: 1557: 1555: 1549: 1548: 1546: 1545: 1540: 1535: 1530: 1524: 1522: 1516: 1515: 1513: 1512: 1507: 1501: 1495: 1493: 1487: 1486: 1484: 1483: 1478: 1472: 1470: 1461: 1455: 1454: 1452: 1451: 1446: 1440: 1438: 1434: 1433: 1431: 1430: 1425: 1423:Oligoarthritis 1420: 1415: 1409: 1407: 1403: 1402: 1395: 1394: 1387: 1380: 1372: 1363: 1362: 1359: 1358: 1338: 1326: 1325: 1323: 1319: 1318: 1315: 1314: 1303: 1292: 1278: 1264: 1245: 1229: 1224: 1223: 1221: 1220:Classification 1213: 1212: 1205: 1187: 1162: 1111: 1088: 1067:(5): 257–258. 1042: 1013:(5): 261–263. 990: 964: 933: 930:ShoulderUS.com 921: 890:(6): 973–981. 867: 846:(3): 692–697. 826: 813: 786:(2): 155–160. 765: 721: 687: 665: 612: 610: 607: 599:osteoarthritis 595:fibrocartilage 544: 541: 535: 534: 490: 488: 481: 474: 473: 388: 386: 379: 373: 370: 355: 352: 323: 320: 292:Arthrocentesis 207: 204: 169: 166: 158:osteoarthritis 143: 142: 139: 136: 133: 132:-like symptoms 126: 123: 122:severe fatigue 120: 117: 114: 98: 95: 50: 49: 41: 40: 32: 31: 28: 24: 23: 15: 9: 6: 4: 3: 2: 1706: 1695: 1692: 1690: 1687: 1685: 1682: 1680: 1677: 1676: 1674: 1657: 1654: 1652: 1649: 1647: 1644: 1643: 1642: 1639: 1637: 1634: 1633: 1631: 1627: 1617: 1614: 1612: 1609: 1605: 1602: 1601: 1600: 1597: 1593: 1590: 1589: 1588: 1585: 1584: 1582: 1578: 1572: 1569: 1567: 1564: 1562: 1559: 1558: 1556: 1554: 1550: 1544: 1541: 1539: 1536: 1534: 1531: 1529: 1526: 1525: 1523: 1521: 1517: 1511: 1508: 1505: 1502: 1500: 1497: 1496: 1494: 1492: 1488: 1482: 1479: 1477: 1474: 1473: 1471: 1469: 1465: 1462: 1460: 1456: 1450: 1447: 1445: 1442: 1441: 1439: 1435: 1429: 1428:Polyarthritis 1426: 1424: 1421: 1419: 1418:Monoarthritis 1416: 1414: 1411: 1410: 1408: 1404: 1400: 1393: 1388: 1386: 1381: 1379: 1374: 1373: 1370: 1357: 1354: 1351: 1348: 1344: 1343: 1339: 1337: 1333: 1332: 1328: 1327: 1324: 1320: 1313: 1309: 1308: 1304: 1302: 1298: 1297: 1293: 1291: 1288: 1284: 1283: 1279: 1274: 1273: 1269: 1265: 1263: 1259: 1255: 1254: 1250: 1246: 1244: 1240: 1239: 1235: 1231: 1230: 1227: 1222: 1218: 1208: 1202: 1198: 1191: 1182: 1181: 1176: 1169: 1167: 1158: 1154: 1149: 1144: 1139: 1134: 1130: 1126: 1122: 1115: 1107: 1103: 1099: 1092: 1084: 1080: 1075: 1070: 1066: 1062: 1058: 1051: 1049: 1047: 1038: 1034: 1029: 1024: 1020: 1016: 1012: 1008: 1004: 997: 995: 978: 974: 968: 952: 948: 944: 937: 931: 925: 917: 913: 908: 907:11392/2365664 903: 898: 893: 889: 885: 881: 874: 872: 863: 859: 854: 849: 845: 841: 837: 830: 820: 818: 809: 805: 801: 797: 793: 789: 785: 781: 774: 772: 770: 761: 757: 750: 748: 746: 744: 742: 740: 738: 736: 734: 732: 730: 728: 726: 717: 713: 709: 705: 701: 694: 692: 683: 676: 674: 672: 670: 661: 657: 652: 647: 643: 639: 635: 631: 627: 620: 618: 613: 606: 604: 600: 596: 592: 588: 584: 580: 576: 572: 568: 564: 560: 556: 552: 549: 540: 531: 528: 520: 510: 506: 500: 499: 495: 489: 480: 479: 470: 467: 459: 448: 445: 441: 438: 434: 431: 427: 424: 420: 417: –  416: 412: 411:Find sources: 405: 401: 395: 394: 389:This section 387: 383: 378: 377: 369: 366: 364: 359: 351: 347: 345: 341: 337: 333: 329: 319: 317: 312: 309: 308:birefringence 305: 301: 297: 296:H&E stain 293: 286: 281: 274: 273:H&E stain 270: 266: 262: 258: 256: 252: 247: 243: 239: 235: 231: 223: 219: 215: 211: 203: 200: 198: 193: 191: 187: 185: 178: 174: 165: 163: 159: 154: 150: 147: 140: 137: 134: 131: 127: 124: 121: 118: 115: 112: 111: 110: 108: 104: 94: 92: 88: 84: 83:rheumatologic 80: 76: 72: 68: 64: 56: 47: 46:birefringence 42: 38: 33: 29: 25: 20: 1636:Hemarthrosis 1520:Seronegative 1506:(Pseudogout) 1503: 1459:Inflammatory 1353:orthoped/382 1340: 1329: 1305: 1294: 1280: 1266: 1247: 1232: 1196: 1190: 1178: 1131:(19): 5777. 1128: 1124: 1114: 1105: 1101: 1091: 1064: 1060: 1010: 1006: 981:. 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Index


birefringence

rheumatologic
calcium pyrophosphate
knee joint
gout
flu
osteoarthritis
white blood cell

Calcium pyrophosphate
adenosine triphosphate
Familial
ANKH

X-ray
chondrocalcinosis
x-ray
CT
MRI
ultrasound
chondrocalcinosis
ankylosing spondylitis
gout

Micrograph
intervertebral disc
H&E stain

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