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Polyarteritis nodosa

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initial ACR analysis, suggested their potential usefulness for diagnostic purposes as well. Subsequent studies did not confirm their diagnostic utility, demonstrating a significant dependence of their discriminative abilities on the prevalence of the various vasculitides in the analyzed populations. Recently, an original study, combining the analysis of more than 100 items used to describe patients' characteristics in a large sample of vasculitides with a computer simulation technique designed to test the potential diagnostic utility of the various criteria, proposed a set of eight positively or negatively discriminating items to be used as a screening tool for diagnosis in patients suspected of systemic vasculitis.
83: 353: 55: 465: 193:, which is the most common neurologic sign of PAN. Mononeuritis multiplex develops in more than 70% of patients with polyarteritis nodosa because of damage to arteries supplying large peripheral nerves. Most cases are marked by asymmetric polyneuropathy, but progressive disease can lead to symmetric nerve involvement. Central nervous system involvement may cause 356:
Microscopic findings in polyarteritis nodosa: nodular thickened and branched arteries from small bowel mucosa (Fig. 1), flexor digitorum superficialis artery with early diffuse nuclear proliferation (X155; Fig. 2), nodular thickened and aneurysmal expanded artery: (a) tunica intima, (b) tunica media,
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is used before the leflunomide treatment is begun. Therapy results in remissions or cures in 90% of cases. Untreated, the disease is fatal in most cases. The most serious associated conditions generally involve the kidneys and gastrointestinal tract. A fatal course usually involves gastrointestinal
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PAN may affect nearly every organ system and thus can present with a broad array of signs and symptoms. These manifestations result from ischemic damage to affected organs, often the skin, heart, kidneys, and nervous system. Constitutional symptoms are seen in up to 90% of affected individuals and
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In polyarteritis nodosa, small aneurysms are strung like the beads of a rosary, therefore making this "rosary sign" an important diagnostic feature of the vasculitis. The 1990 ACR criteria were designed for classification purposes only, but their good discriminatory performances, indicated by the
336:. The cause remains unknown in the remaining cases; there may be causal and clinical distinctions between classic idiopathic PAN, the cutaneous forms of PAN, and PAN associated with chronic hepatitis. In children, cutaneous PAN is frequently associated with 406:
A patient is said to have polyarteritis nodosa if he or she has three of the 10 signs known as the 1990 American College of Rheumatology (ACR) criteria, when a radiographic or pathological diagnosis of vasculitis is made:
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The condition affects adults more frequently than children and males more frequently than females. Most cases occur between the ages of 40 and 60. Polyarteritis nodosa is more common in people with hepatitis B infection.
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Henegar, Corneliu; Pagnoux, Christian; Puéchal, Xavier; Zucker, Jean-Daniel; Bar-Hen, Avner; Guern, Véronique Le; Saba, Mona; Bagnères, Denis; Meyer, Olivier; Guillevin, Loïc (1 May 2008).
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Polyarteritis nodosa rarely affects the blood vessels of the lungs and this feature can help to differentiate it from other vasculitides that may have similar signs and symptoms (e.g.,
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No specific lab tests exist for diagnosing polyarteritis nodosa. Diagnosis is generally based on the physical examination and a few laboratory studies that help confirm the diagnosis:
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Giannini, AJ; Black, HR. Psychiatric, Psychogenic and Somatopsychic Disorders Handbook. Garden City, NY. Medical Examination Publishing, 1978. Pp. 219–220.
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In case of remission, about 60% experience relapse within five years. In cases caused by hepatitis B virus, however, recurrence rate is only around 6%.
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In polyarteritis nodosa, small aneurysms are strung like the beads of a rosary, therefore making "rosary sign" a diagnostic feature of the vasculitis.
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in the urine may also be seen. Almost all patients with PAN have renal insufficiency caused by renal artery narrowing, thrombosis, and infarctions.
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PAN is a rare disease. With treatment, five-year survival is 80%; without treatment, five-year survival is 13%. Death is often a consequence of
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The skin may show rashes, swelling, necrotic ulcers, and subcutaneous nodules (lumps). Skin manifestations of PAN include palpable purpura and
1525: 1403: 1297: 121:, therefore making this "rosary sign" an important diagnostic feature of the vasculitis. PAN is sometimes associated with infection by the 383:) - not associated with "classic" polyarteritis nodosa, but is present in a form of the disease affecting smaller blood vessels, known as 251:
Damage to mesenteric arteries can cause abdominal pain, mesenteric ischemia, and bowel perforation. Abdominal pain may also be seen.
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Russell Goodman; Paul F. Dellaripa; Amy Leigh Miller; Joseph Loscalzo (January 2, 2014). "An Unusual Case of Abdominal Pain".
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Polyarteritis nodosa: Macroscopic specimen of the heart with abundant adipose tissue and nodular thickened coronary vessels
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and other internal organs but generally sparing the lungs' circulation. Small aneurysms are strung like the beads of a
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Nerve involvement may cause sensory changes with numbness, pain, burning, and weakness (peripheral neuropathy).
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Guillevin, L.; Lhote, F.; Cohen, P.; Sauvaget, F.; Jarrousse, B.; Lortholary, O.; Noël, L.; Trépo, C. (1995).
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is diagnosed with polyarteritis nodosa after experiencing excruciating chest pain and is treated with
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are occasionally discovered in people affected by PAN. PAN has also been associated with underlying
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Greenson, Joel K.; Montgomery, Elizabeth A.; Polydorides, Alexandros D. (1 September 2009).
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may be helpful. Some patients have entered a remission phase when a four-dose infusion of
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Ebert, Ellen C.; Hagspiel, Klaus D.; Nagar, Michael; Schlesinger, Naomi (2008).
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Testicular pain or tenderness (occasionally, a site biopsied for diagnosis)
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Biopsy of tissue showing the arteritis (typically inflamed arteries): The
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Hepatitis B (not C) virus tests positive (for surface antigen or antibody)
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Treatment involves medications to suppress the immune system, including
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bleeding, infection, myocardial infarction, and/or kidney failure.
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Diastolic blood pressure greater than 90 mmHg (high blood pressure)
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Systemic necrotizing inflammation of medium-sized muscular arteries
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Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007).
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Tissue biopsy (reveals inflammation in small arteries, called
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Perinuclear pattern of antineutrophil cytoplasmic antibodies (
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Selga, D.; Mohammad, A.; Sturfelt, G.; Segelmark, M. (2006).
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Diagnostic Pathology: Gastrointestinal: Published by Amirsys
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http://www.medicinenet.com/polyarteritis_nodosa/article.htm
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Elevated kidney blood tests (BUN greater than 40 mg/dL or
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Forbess, L; Bannykh, S (2015). "Polyarteritis Nodosa".
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are often affected, and this most commonly presents as
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Involvement of the arteries of the heart may cause a
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Kumar, Vinay; K. Abbas, Abul; C. Aster, Jon (2015).
654: 447:(angiogram) showing the arteries that are dilated ( 324:-mediated cause in that subset. Infection with the 320:complexes in affected blood vessels, indicating an 451:) or constricted by the blood vessel inflammation 1502: 308:, but about 30% of people with PAN have chronic 241:, and inflammation of the sac around the heart ( 207:Kidney involvement is common and often leads to 129:virus. The condition may be present in infants. 1139:Entry in Polyarteritis Nodosa Follow-up article 1084:Entry in Polyarteritis Nodosa Follow-up article 620:Robbins and Cotran: Pathologic Basis of Disease 370:(may demonstrate an elevated white blood count) 879:Sarah Ringold; Carol A Wallace (May 1, 2010). 734: 730: 728: 726: 724: 722: 720: 718: 716: 714: 1404:Eosinophilic granulomatosis with polyangiitis 1291: 990: 712: 710: 708: 706: 704: 702: 700: 698: 696: 694: 984: 943: 991:Boehm, Ingrid; Bauer, R (1 February 2000). 1521:Steroid-responsive inflammatory conditions 1298: 1284: 737:Rheumatic Disease Clinics of North America 691: 641: 639: 613: 611: 568: 566: 107:inflammation of blood vessels (vasculitis) 81: 53: 1112: 1065: 1008: 967: 896: 769: 476: 428:Nerve disease (either single or multiple) 872: 838:Clinical Gastroenterology and Hepatology 463: 425:Muscle pain, weakness, or leg tenderness 413:Weight loss greater than/equal to 4.5 kg 351: 344:is included in the diagnostic criteria. 211:parts of the kidney. Involvement of the 1305: 636: 622:(9th ed.). Elsevier. p. 509. 608: 563: 1503: 458:is a frequent location for the biopsy. 306:anti-neutrophil cytoplasmic antibodies 1536:Hepatitis C virus-associated diseases 1531:Vascular-related cutaneous conditions 1279: 670:. Lippincott Williams & Wilkins. 147: 1526:Systemic connective tissue disorders 545:the protagonist character played by 790: 13: 1450:Acute hemorrhagic edema of infancy 14: 1547: 1465:Cutaneous small-vessel vasculitis 1429:Cutaneous small-vessel vasculitis 1171: 921:"Log in | BMJ Best Practice" 169:, and unintentional weight loss. 1390:Granulomatosis with polyangiitis 1114:10.1097/00005792-199509000-00002 885:Annals of the Rheumatic Diseases 772:"Infantile Polyarteritis Nodosa" 770:Person, A, Donald (2006-06-15). 645: 483:granulomatosis with polyangiitis 267: 109:affecting medium-sized muscular 1460:Bullous small vessel vasculitis 1143: 1088: 1041: 1025: 931: 913: 524: 825: 763: 650:. W. B. Saunders. p. 243. 591: 1: 556: 340:, and positive streptococcal 219:in about one-third of cases. 492: 387:or leukocytoclastic angiitis 347: 304:PAN has no association with 7: 1455:Cryoglobulinemic vasculitis 1067:10.1093/rheumatology/kel091 10: 1552: 1356:Thromboangiitis obliterans 1010:10.1001/archderm.136.2.167 956:Arthritis & Rheumatism 538:In the 1956 American film 533: 113:, typically involving the 1477: 1442: 1421:Type III hypersensitivity 1419: 1373: 1364: 1336: 1313: 1249: 1179: 850:10.1016/j.cgh.2008.04.004 749:10.1016/j.rdc.2014.09.005 575:Dermatology: 2-Volume Set 299: 66: 61: 52: 29: 24: 1409:Microscopic polyangiitis 1155:The Criterion Collection 487:microscopic polyangiitis 385:microscopic polyangiitis 338:streptococcal infections 312:and deposits containing 249:Gastrointestinal system: 997:Archives of Dermatology 937:Shiel, Jr., William C, 898:10.1136/ard.2009.127886 438:greater than 1.5 mg/dL) 255:Musculoskeletal system: 115:arteries of the kidneys 811:10.1056/NEJMcps1215559 477:Differential diagnosis 469: 358: 231:Cardiovascular system: 191:mononeuritis multiplex 46:Kussmaul-Maier disease 467: 355: 221:deposition of protein 179:in some individuals. 138:myocardial infarction 1485:Goodpasture syndrome 1346:Polyarteritis nodosa 1328:Giant cell arteritis 1323:Takayasu's arteritis 925:bestpractice.bmj.com 577:. St. Louis: Mosby. 93:Polyarteritis nodosa 38:Periarteritis nodosa 25:Polyarteritis nodosa 1511:Disorders of fascia 1307:Systemic vasculitis 334:hairy cell leukemia 217:high blood pressure 34:Panarteritis nodosa 1490:Sneddon's syndrome 1250:External resources 1151:"Bigger Than Life" 470: 417:Livedo reticularis 399:C-reactive protein 359: 183:Neurologic system: 177:livedo reticularis 148:Signs and symptoms 1498: 1497: 1473: 1472: 1273: 1272: 1060:(10): 1276–1281. 969:10.1002/art.23470 743:(1): 33–46, vii. 677:978-1-931884-26-6 648:Surgery, Volume 5 629:978-1-4557-2613-4 584:978-1-4160-2999-1 326:hepatitis C virus 187:Peripheral nerves 90: 89: 19:Medical condition 1543: 1371: 1370: 1351:Kawasaki disease 1300: 1293: 1286: 1277: 1276: 1177: 1176: 1165: 1164: 1162: 1161: 1147: 1141: 1134: 1116: 1092: 1086: 1079: 1069: 1045: 1039: 1029: 1023: 1022: 1012: 988: 982: 981: 971: 962:(5): 1528–1538. 947: 941: 935: 929: 928: 917: 911: 910: 900: 876: 870: 869: 829: 823: 822: 794: 788: 787: 785: 783: 767: 761: 760: 732: 689: 688: 686: 684: 661: 652: 651: 643: 634: 633: 615: 606: 595: 589: 588: 570: 541:Bigger Than Life 503:cyclophosphamide 167:loss of appetite 86: 85: 57: 42:Kussmaul disease 22: 21: 1551: 1550: 1546: 1545: 1544: 1542: 1541: 1540: 1501: 1500: 1499: 1494: 1469: 1438: 1415: 1360: 1332: 1309: 1304: 1274: 1269: 1268: 1245: 1244: 1188: 1174: 1169: 1168: 1159: 1157: 1149: 1148: 1144: 1093: 1089: 1046: 1042: 1030: 1026: 989: 985: 948: 944: 936: 932: 919: 918: 914: 877: 873: 830: 826: 795: 791: 781: 779: 768: 764: 733: 692: 682: 680: 678: 662: 655: 646:Keen, William. 644: 637: 630: 616: 609: 596: 592: 585: 571: 564: 559: 536: 527: 495: 479: 350: 302: 295:and perforation 281:resulting from 270: 150: 80: 20: 17: 12: 11: 5: 1549: 1539: 1538: 1533: 1528: 1523: 1518: 1513: 1496: 1495: 1493: 1492: 1487: 1481: 1479: 1475: 1474: 1471: 1470: 1468: 1467: 1462: 1457: 1452: 1446: 1444: 1440: 1439: 1437: 1436: 1434:IgA vasculitis 1431: 1425: 1423: 1417: 1416: 1414: 1413: 1412: 1411: 1406: 1394: 1393: 1392: 1379: 1377: 1368: 1362: 1361: 1359: 1358: 1353: 1348: 1342: 1340: 1334: 1333: 1331: 1330: 1325: 1319: 1317: 1311: 1310: 1303: 1302: 1295: 1288: 1280: 1271: 1270: 1267: 1266: 1254: 1253: 1251: 1247: 1246: 1243: 1242: 1231: 1220: 1205: 1189: 1184: 1183: 1181: 1180:Classification 1173: 1172:External links 1170: 1167: 1166: 1142: 1107:(5): 238–253. 1087: 1040: 1024: 983: 942: 930: 912: 871: 844:(9): 960–966. 824: 789: 762: 690: 676: 653: 635: 628: 607: 590: 583: 561: 560: 558: 555: 535: 532: 526: 523: 494: 491: 478: 475: 462: 461: 460: 459: 452: 442: 439: 432: 429: 426: 423: 420: 414: 404: 403: 402: 401: 395: 388: 377: 371: 349: 346: 322:immune complex 301: 298: 297: 296: 289: 283:cardiomyopathy 276: 269: 266: 149: 146: 134:kidney failure 88: 87: 70: 64: 63: 59: 58: 50: 49: 31: 27: 26: 18: 15: 9: 6: 4: 3: 2: 1548: 1537: 1534: 1532: 1529: 1527: 1524: 1522: 1519: 1517: 1514: 1512: 1509: 1508: 1506: 1491: 1488: 1486: 1483: 1482: 1480: 1476: 1466: 1463: 1461: 1458: 1456: 1453: 1451: 1448: 1447: 1445: 1441: 1435: 1432: 1430: 1427: 1426: 1424: 1422: 1418: 1410: 1407: 1405: 1402: 1401: 1400: 1399: 1395: 1391: 1388: 1387: 1386: 1385: 1381: 1380: 1378: 1376: 1372: 1369: 1367: 1363: 1357: 1354: 1352: 1349: 1347: 1344: 1343: 1341: 1339: 1338:Medium vessel 1335: 1329: 1326: 1324: 1321: 1320: 1318: 1316: 1312: 1308: 1301: 1296: 1294: 1289: 1287: 1282: 1281: 1278: 1265: 1261: 1260: 1256: 1255: 1252: 1248: 1241: 1237: 1236: 1232: 1230: 1226: 1225: 1221: 1219: 1215: 1214: 1210: 1206: 1204: 1200: 1199: 1195: 1191: 1190: 1187: 1182: 1178: 1156: 1152: 1146: 1140: 1136: 1132: 1128: 1124: 1120: 1115: 1110: 1106: 1102: 1098: 1091: 1085: 1081: 1077: 1073: 1068: 1063: 1059: 1055: 1051: 1044: 1038: 1037:0-87488-596-5 1034: 1028: 1020: 1016: 1011: 1006: 1002: 998: 994: 987: 979: 975: 970: 965: 961: 957: 953: 946: 940: 934: 926: 922: 916: 908: 904: 899: 894: 891:(5): 785–86. 890: 886: 882: 875: 867: 863: 859: 855: 851: 847: 843: 839: 835: 828: 820: 816: 812: 808: 804: 800: 793: 777: 773: 766: 758: 754: 750: 746: 742: 738: 731: 729: 727: 725: 723: 721: 719: 717: 715: 713: 711: 709: 707: 705: 703: 701: 699: 697: 695: 679: 673: 669: 668: 660: 658: 649: 642: 640: 631: 625: 621: 614: 612: 605: 604:Who Named It? 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WebMD 534:Culture 195:strokes 159:fatigue 99:) is a 1398:p-ANCA 1384:c-ANCA 1264:001438 1129:  1121:  1074:  1035:  1017:  976:  905:  864:  856:  817:  755:  674:  626:  581:  381:p-ANCA 300:Causes 274:Stroke 258:Muscle 142:stroke 119:rosary 79:  1478:Other 1240:10220 1218:446.0 1203:M30.0 1127:S2CID 318:HBsAb 314:HBsAg 225:blood 173:Skin: 155:fever 140:, or 44:, or 1224:MeSH 1213:9-CM 1119:PMID 1072:PMID 1033:ISBN 1015:PMID 974:PMID 903:PMID 862:PMID 854:ISSN 815:PMID 784:2009 753:PMID 685:2013 672:ISBN 624:ISBN 579:ISBN 501:and 328:and 285:and 260:and 1209:ICD 1194:ICD 1109:doi 1062:doi 1005:doi 1001:136 964:doi 893:doi 846:doi 807:doi 803:370 745:doi 602:at 509:or 489:). 485:or 374:ESR 368:CBC 330:HIV 245:). 223:or 197:or 125:or 97:PAN 1507:: 1262:: 1238:: 1227:: 1216:: 1201:: 1198:10 1153:. 1137:, 1125:. 1117:. 1105:74 1103:. 1099:. 1082:, 1070:. 1058:45 1056:. 1052:. 1013:. 999:. 995:. 972:. 960:58 958:. 954:. 923:. 901:. 889:69 887:. 883:. 860:. 852:. 840:. 836:. 813:. 801:. 774:. 751:. 741:41 739:. 693:^ 656:^ 638:^ 610:^ 565:^ 553:. 237:, 201:. 165:, 161:, 157:, 144:. 136:, 75:, 40:, 36:, 1299:e 1292:t 1285:v 1211:- 1196:- 1186:D 1163:. 1133:. 1111:: 1078:. 1064:: 1021:. 1007:: 980:. 966:: 927:. 909:. 895:: 868:. 848:: 842:6 821:. 809:: 786:. 759:. 747:: 687:. 632:. 587:. 543:, 394:) 316:- 95:( 48:,

Index


Specialty
Immunology
rheumatology
Edit this on Wikidata
systemic
necrotizing
inflammation of blood vessels (vasculitis)
arteries
arteries of the kidneys
rosary
hepatitis B
hepatitis C
kidney failure
myocardial infarction
stroke
fever
fatigue
weakness
loss of appetite
livedo reticularis
Peripheral nerves
mononeuritis multiplex
strokes
seizures
death of
renal artery
high blood pressure
deposition of protein
blood

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