473:
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,
517:
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
152:
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
472:
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:
529:
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.
1208:
1193:
950:
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).
481:
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.,
361:
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:
317:
313:
1031:
Giannini, AJ; Black, HR. Psychiatric, Psychogenic and
Somatopsychic Disorders Handbook. Garden City, NY. Medical Examination Publishing, 1978. Pp. 219–220.
1520:
521:
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%.
468:
In polyarteritis nodosa, small aneurysms are strung like the beads of a rosary, therefore making "rosary sign" a diagnostic feature of the vasculitis.
227:
in the urine may also be seen. Almost all patients with PAN have renal insufficiency caused by renal artery narrowing, thrombosis, and infarctions.
132:
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
1535:
1530:
175:
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.
797:
Russell
Goodman; Paul F. Dellaripa; Amy Leigh Miller; Joseph Loscalzo (January 2, 2014). "An Unusual Case of Abdominal Pain".
675:
627:
582:
305:
62:
Polyarteritis nodosa: Macroscopic specimen of the heart with abundant adipose tissue and nodular thickened coronary vessels
1449:
117:
and other internal organs but generally sparing the lungs' circulation. Small aneurysms are strung like the beads of a
1464:
1428:
1290:
1036:
952:"A paradigm of diagnostic criteria for polyarteritis nodosa: Analysis of a series of 949 patients with vasculitides"
1389:
482:
1097:"Polyarteritis nodosa related to hepatitis B virus. A prospective study with long-term observation of 41 patients"
1459:
1433:
185:
Nerve involvement may cause sensory changes with numbness, pain, burning, and weakness (peripheral neuropathy).
373:
1095:
Guillevin, L.; Lhote, F.; Cohen, P.; Sauvaget, F.; Jarrousse, B.; Lortholary, O.; Noël, L.; Trépo, C. (1995).
1510:
1283:
329:
1454:
1355:
1050:"Polyarteritis nodosa when applying the Chapel Hill nomenclature--a descriptive study on ten patients"
549:
is diagnosed with polyarteritis nodosa after experiencing excruciating chest pain and is treated with
1420:
505:. When present, underlying hepatitis B virus infection should be immediately treated. In some cases,
833:
332:
are occasionally discovered in people affected by PAN. PAN has also been associated with underlying
1408:
1223:
486:
384:
82:
1322:
337:
1337:
1365:
1314:
796:
190:
1263:
1138:
1083:
938:
1515:
1489:
234:
186:
137:
664:
Greenson, Joel K.; Montgomery, Elizabeth A.; Polydorides, Alexandros D. (1 September 2009).
1484:
1327:
949:
367:
513:
may be helpful. Some patients have entered a remission phase when a four-dose infusion of
8:
1306:
1212:
920:
771:
333:
1150:
1126:
416:
398:
176:
1217:
1234:
1118:
1113:
1096:
1071:
1032:
1014:
973:
902:
861:
853:
814:
752:
671:
623:
578:
325:
158:
67:
1130:
1080:
951:
572:
1350:
1135:
1108:
1061:
1004:
963:
892:
845:
806:
744:
540:
502:
166:
100:
1066:
1049:
1228:
665:
832:
Ebert, Ellen C.; Hagspiel, Klaus D.; Nagar, Michael; Schlesinger, Naomi (2008).
357:(c) tunica adventitia, (d) newly formed connective tissue and fat (Fig. 3; X155)
1239:
1009:
992:
603:
321:
282:
133:
1202:
849:
748:
598:
1504:
857:
278:
238:
897:
1374:
1075:
1018:
977:
906:
865:
818:
810:
756:
506:
422:
Testicular pain or tenderness (occasionally, a site biopsied for diagnosis)
286:
242:
216:
212:
114:
76:
1122:
454:
Biopsy of tissue showing the arteritis (typically inflamed arteries): The
441:
Hepatitis B (not C) virus tests positive (for surface antigen or antibody)
215:, which supplies the kidneys with highly oxygenated blood, often leads to
1258:
546:
510:
455:
444:
309:
220:
126:
122:
497:
Treatment involves medications to suppress the immune system, including
498:
435:
352:
261:
208:
106:
72:
54:
1275:
1185:
993:"Low-Dose Methotrexate Controls a Severe Form of Polyarteritis Nodosa"
968:
880:
881:"Evolution of paediatric-specific vasculitis classification criteria"
550:
514:
448:
419:(a mottled purplish skin discoloration over the extremities or torso)
391:
224:
518:
bleeding, infection, myocardial infarction, and/or kidney failure.
431:
Diastolic blood pressure greater than 90 mmHg (high blood pressure)
341:
292:
162:
103:
16:
Systemic necrotizing inflammation of medium-sized muscular arteries
573:
Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007).
257:
198:
1397:
1383:
1197:
464:
380:
273:
194:
141:
118:
110:
1047:
390:
Tissue biopsy (reveals inflammation in small arteries, called
379:
Perinuclear pattern of antineutrophil cytoplasmic antibodies (
1048:
Selga, D.; Mohammad, A.; Sturfelt, G.; Segelmark, M. (2006).
831:
663:
154:
878:
667:
Diagnostic
Pathology: Gastrointestinal: Published by Amirsys
1094:
939:
http://www.medicinenet.com/polyarteritis_nodosa/article.htm
434:
Elevated kidney blood tests (BUN greater than 40 mg/dL or
735:
Forbess, L; Bannykh, S (2015). "Polyarteritis Nodosa".
189:
are often affected, and this most commonly presents as
834:"Gastrointestinal Involvement in Polyarteritis Nodosa"
659:
657:
617:
233:
Involvement of the arteries of the heart may cause a
1175:
618:
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?
601:
600:
594:
586:
580:
576:
569:
567:
562:
554:
552:
548:
544:
542:
531:
522:
519:
516:
512:
508:
504:
500:
490:
488:
484:
474:
466:
457:
453:
450:
446:
443:
440:
437:
433:
430:
427:
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418:
415:
412:
411:
410:
409:
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400:
396:
393:
389:
386:
382:
378:
375:
372:
369:
366:
365:
364:
363:
362:
354:
345:
343:
339:
335:
331:
327:
323:
319:
315:
311:
307:
294:
290:
288:
284:
280:
279:Heart failure
277:
275:
272:
271:
268:Complications
265:
263:
259:
256:
252:
250:
246:
244:
240:
239:heart failure
236:
232:
228:
226:
222:
218:
214:
210:
206:
205:Renal system:
202:
200:
196:
192:
188:
184:
180:
178:
174:
170:
168:
164:
160:
156:
145:
143:
139:
135:
130:
128:
124:
120:
116:
112:
108:
105:
102:
98:
94:
84:
78:
74:
71:
69:
65:
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1315:Large vessel
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264:are common.
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782:24 December
547:James Mason
511:leflunomide
456:sural nerve
445:Arteriogram
310:hepatitis B
291:Intestinal
262:joint aches
127:hepatitis C
123:hepatitis B
104:necrotizing
30:Other names
1505:Categories
1235:DiseasesDB
1160:2021-07-16
557:References
499:prednisone
436:creatinine
376:(elevated)
73:Immunology
1443:Ungrouped
858:1542-3565
776:eMedicine
683:19 August
551:cortisone
515:rituximab
493:Treatment
449:aneurysms
397:Elevated
392:arteritis
348:Diagnosis
68:Specialty
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978:18438816
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599:synd/764
342:serology
293:necrosis
209:death of
199:seizures
163:weakness
153:include
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1123:7565065
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