360:. In June 2020 FDA approved Ilaris (canakinumab) for the treatment of AOSD, this is the first FDA approved treatment for AOSD. Canakinumab is another anti-IL1 drug which selectively binds IL-1β and rilonacept which blocks both IL-1A and IL-1β. The monoclonal anti-IL6 antibody tocilizumab is another treatment option as effective as anakinra.
288:
One set of 21 adult-onset Still's disease patients were divided into four types, according to clinical course patterns. These included monocyclic systemic disease, polycyclic systemic disease, chronic articular monocyclic systemic disease, and chronic articular polycyclic systemic disease. People
156:
is markedly elevated. Patients experiencing a flare-up from adult-onset Still's disease usually report extreme fatigue, swelling of the lymph nodes and, less commonly, fluid accumulation in the lungs and heart. In rare cases, AOSD can cause life-threatening complications, including hemophagocytic
383:
per year is estimated to be 1.6 per 1,000,000 population. The number of people currently affected is estimated at 1.5 cases per 100,000–1,000,000 population. Onset is most common in two age ranges, between ages 16–25 and between ages of 36–46 years.
940:
Sugiura, T; Kawaguchi, Y; Harigai, M; Terajima-Ichida, H; Kitamura, Y; Furuya, T; Ichikawa, N; Kotake, S; Tanaka, M; Hara, M; Kamatani, N (Nov 2002). "Association between adult-onset Still's disease and interleukin-18 gene polymorphisms".
189:. At least seven sets of diagnostic criteria have been devised; however, the Yamaguchi criteria have the highest sensitivity. Diagnosis requires at least five features, with at least two of these being major diagnostic criteria.
1695:
1680:
1321:
880:
Néel, Antoine; Wahbi, Anaïs; Tessoulin, Benoit; Boileau, Julien; Carpentier, Dorothée; Decaux, Olivier; Fardet, Laurence; Geri, Guillaume; Godmer, Pascal; Goujard, Cécile; Maisonneuve, Hervé (2018-04-11).
173:
The cause of adult-onset Still's disease is unknown and it is not heritable, but it presumably involves interleukin-1 (IL-1), since medications that block the action of IL-1β are effective treatments.
1205:
Vastert, Sebastiaan J.; Jamilloux, Yvan; Quartier, Pierre; Ohlman, Sven; Osterling
Koskinen, Lisa; Kullenberg, Torbjörn; Franck-Larsson, Karin; Fautrel, Bruno; de Benedetti, Fabrizio (2019-11-01).
1091:
Yamaguchi M, Ohta A, Tsunematsu T, Kasukawa R, Mizushima Y, Kashiwagi H, Kashiwazaki S, Tanimoto K, Matsumoto Y, Ota T (1992). "Preliminary criteria for classification of adult Still's disease".
97:
Prognosis is usually favorable but manifestations of the disease affecting the lungs, heart, or kidneys may occasionally cause severe life-threatening complications. It is treated first with
356:(IL-1), particularly IL-1β. A randomized, multicenter trial reported better outcomes in a group of 12 patients treated with anakinra than in a group of 10 patients taking other
1795:
1048:
Efthimiou P, Kontzias A, Ward CM, Ogden NS (June 2007). "Adult-onset Still's disease: can recent advances in our understanding of its pathogenesis lead to targeted therapy?".
1909:
1313:
1788:
1644:
1533:
883:"Diagnostic and management of life-threatening Adult-Onset Still Disease: a French nationwide multicenter study and systematic literature review"
508:
Colafrancesco, Serena; Priori, Roberta; Alessandri, Cristiano; Perricone, Carlo; Pendolino, Monica; Picarelli, Giovanna; Valesini, Guido (2012).
1024:
1781:
1997:
1119:
Cush, JJ; Medsger TA Jr; Christy, WC; Herbert, DC; Cooperstein, LA (Feb 1987). "Adult-onset Still's disease. Clinical course and outcome".
60:
856:
666:
Vastert SJ, Jamilloux Y, Quartier P, Ohlman S, Osterling
Koskinen L, Kullenberg T, Franck-Larsson K, Fautrel B, de Benedetti F (2019).
462:
Akkara Veetil BM, Yee AH, Warrington KJ, Aksamit AJ Jr, Mason TG (December 2012). "Aseptic meningitis in adult onset Still's disease".
2099:
1264:"Beneficial effect of interleukin 1 inhibition with anakinra in adult-onset Still's disease. An open, randomized, multicenter study"
367:. However, there are obvious similarities between the two conditions, and there is some evidence that they may be closely related.
357:
120:(also known as "juvenile-onset Still's disease"), and there is some evidence that the two conditions are closely related.
90:
may be extremely elevated with this disorder. AOSD may present in a similar manner to other inflammatory diseases and to
157:
lymphohistiocytosis, intervertebral disc calcification (IVDC), fulminant hepatitis, or disabling conditions such as
1925:
759:
Fauter, M.; Gerfaud-Valentin, M.; Delplanque, M.; Georgin-Lavialle, S.; Sève, P.; Jamilloux, Y. (2020-01-07).
1992:
428:
364:
117:
1648:
1573:
G. F. Still. A special form of joint disease met with in children. Doctoral dissertation, Cambridge, 1896.
720:"[It is time to reconcile systemic juvenile idiopathic arthritis and adult-onset Still's disease]"
1958:
1761:
1500:
1442:
Agha-Abbaslou, Mojgan; Bensaci, Ana Maria; Dike, Oluchi; Poznansky, Mark C.; Hyat, Arooj (2017-02-03).
162:
985:
289:
with chronic articular and polyarticular disease were at higher risk to develop disabling arthritis.
44:
1948:
1710:
1444:"Adult-Onset Still's Disease: Still a Serious Health Problem (a Case Report and Literature Review)"
380:
2094:
507:
2089:
2016:
1943:
809:
83:
1750:
984:
Jamilloux, Y; Gerfaud-Valentin, M; Martinon, F; Belot, A; Henry, T; Sève, P (February 2015).
400:
274:
149:
64:
2004:
8:
2056:
2009:
1938:
1896:
1699:
1314:"FDA Approves First Treatment for Adult Onset Still's Disease, a Severe and Rare Disease"
986:"Pathogenesis of adult-onset Still's disease: new insights from the juvenile counterpart"
280:
a somewhat less aggressive pattern, in which the main symptom is chronic joint pain and
2084:
2051:
1971:
1933:
1617:
1584:
1476:
1443:
1370:
1343:
1294:
1239:
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1182:
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1016:
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692:
667:
640:
615:
536:
509:
487:
306:
158:
133:
91:
1704:
305:
are used to treat severe symptoms of Still's. Other commonly used medications include
1904:
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145:
27:
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491:
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1218:
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824:
772:
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687:
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635:
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575:
531:
521:
471:
334:
1411:
1394:
1222:
828:
683:
562:
Gerfaud-Valentin, Mathieu; Jamilloux, Yvan; Iwaz, Jean; Sève, Pascal (July 2014).
1854:
1715:
777:
760:
736:
719:
580:
563:
217:
137:
113:, can be effective treatments when standard steroid treatments are insufficient.
1976:
1583:
Kopeć-Mędrek, Magdalena; Widuchowska, Małgorzata; Kucharz, Eugeniusz J. (2016).
1262:
Nordström D; Knight A; Luukkainen R; van
Vollenhoven R; et al. (Oct 2012).
1261:
758:
510:"IL-18 Serum Level in Adult Onset Still's Disease: A Marker of Disease Activity"
2046:
1828:
1726:
1562:
1557:
939:
393:
174:
153:
98:
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1004:
899:
631:
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908:
836:
786:
589:
404:
353:
310:
106:
79:
1600:
1520:
954:
363:
The condition "juvenile-onset Still's disease" is now usually grouped under
2041:
1886:
1864:
1626:
1529:
1485:
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1379:
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1248:
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745:
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597:
545:
483:
433:
416:
376:
318:
314:
297:
Adult-onset Still's disease is treated with anti-inflammatory medications.
241:
234:
230:
141:
32:
1360:
1344:"Review Article: Anti-Interleukin-1 Agents in Adult Onset Still's Disease"
1281:
1263:
1140:
1105:
526:
269:
People with AOSD generally experience one of two patterns in the disease:
1804:
1745:
1459:
1154:
Jamilloux, Y; Gerfaud-Valentin, M; Henry, T; Sève, P (22 December 2014).
1061:
330:
245:
1672:
1172:
2061:
1849:
1118:
461:
346:
338:
322:
302:
211:
129:
102:
75:
983:
1818:
1773:
1647:. Genetic and Rare Diseases Information Center (GARD). Archived from
396:
342:
281:
227:
salmon-colored rash (usually over trunk or extremities while febrile)
718:
Jamilloux Y, Georgin-Lavialle S, Sève P, Belot A, Fautrel B (2019).
616:"Mechanisms, biomarkers and targets for adult-onset Still's disease"
1756:
810:"Complications of adult-onset Still's disease and their management"
326:
298:
224:
186:
110:
87:
1153:
717:
1441:
561:
67:
415:
Researchers are investigating whether levels of a protein named
1684:
1341:
1090:
39:
1582:
1204:
665:
258:
Negative tests for antinuclear antibody and rheumatoid factor
71:
132:, high fevers, a salmon-pink macular or maculopapular rash,
1915:
1047:
403:(1861–1941). The adult-onset version was characterized by
1966:
879:
273:
a debilitating pattern of fevers, joint pain, and other
1454:. International Scientific Information, Inc.: 119–124.
94:, which must be ruled out before making the diagnosis.
1207:"Anakinra in children and adults with Still's disease"
668:"Anakinra in children and adults with Still's disease"
1395:"Biologic treatments for adult-onset Still's disease"
761:"[Adult-onset Still's disease complications]"
1662:
1156:"Treatment of adult-onset Still's disease: a review"
613:
419:could be used to improve diagnosis and monitoring.
203:Fever of at least 39 °C for at least one week
2076:
1501:"Adult – onset Still's disease : A review"
807:
713:
711:
503:
501:
379:and has been described all over the world. The
1585:"Calprotectin in rheumatic diseases: a review"
1789:
1498:
661:
659:
1998:Systemic-onset juvenile idiopathic arthritis
1311:
1041:
708:
498:
457:
455:
453:
451:
449:
1112:
808:Mitrovic, Stéphane; Fautrel, Bruno (2018).
609:
607:
1796:
1782:
1342:Cecilia Giampietro; Bruno Fautrel (2012).
1312:Commissioner, Office of the (2020-06-16).
656:
185:The diagnosis is clinical, not based upon
1616:
1519:
1475:
1410:
1392:
1369:
1359:
1280:
1238:
1181:
1171:
1160:Therapeutics and Clinical Risk Management
1084:
916:
898:
776:
735:
691:
639:
579:
535:
525:
446:
604:
614:Feist E, Mitrovic S, Fautrel B (2018).
105:. Medications that block the action of
2077:
1803:
410:
70:characterized by the classic triad of
1777:
1492:
1348:International Journal of Inflammation
514:International Journal of Inflammation
358:disease-modifying antirheumatic drugs
123:
86:. Levels of the iron-binding protein
1435:
817:Expert Review of Clinical Immunology
557:
555:
128:The disease typically presents with
214:or arthritis for at least two weeks
134:enlargement of the liver and spleen
78:, and a distinctive salmon-colored
13:
168:
14:
2111:
1637:
552:
264:
244:(10,000/microL or greater), with
2100:Diseases named after discoverers
1448:American Journal of Case Reports
142:increased white blood cell count
116:Obvious similarities exist with
1645:"Adult-onset Still's disease |"
1576:
1567:
1550:
1539:from the original on 2018-07-22
1393:Al-Homood, I. A. (2014-01-01).
1386:
1335:
1324:from the original on 2020-06-17
1305:
1255:
1198:
1147:
1030:from the original on 2022-05-17
977:
933:
862:from the original on 2023-05-29
392:Still's disease is named after
375:Adult-onset Still's disease is
370:
140:, and a neutrophil-predominant
873:
801:
752:
251:Abnormal liver function tests
82:. The disease is considered a
1:
1993:Juvenile idiopathic arthritis
1499:Owlia MB, Mehrpoor G (2009).
829:10.1080/1744666X.2018.1465821
564:"Adult-onset Still's disease"
439:
429:Juvenile idiopathic arthritis
365:juvenile rheumatoid arthritis
177:is expressed at high levels.
118:juvenile rheumatoid arthritis
778:10.1016/j.revmed.2019.12.003
765:La Revue de MĂ©decine Interne
737:10.1016/j.revmed.2019.06.001
620:Nature Reviews. Rheumatology
581:10.1016/j.autrev.2014.01.058
292:
180:
7:
2022:Adult-onset Still's disease
1959:Connective tissue disorders
1412:10.1093/rheumatology/ket250
1223:10.1093/rheumatology/kez350
684:10.1093/rheumatology/kez350
422:
53:Adult-onset Still's disease
22:Adult-onset Still's disease
10:
2116:
1217:(Supplement_6): vi9–vi22.
387:
163:sensorineural hearing loss
2034:
1985:
1957:
1924:
1895:
1872:
1863:
1842:
1811:
1736:
1666:
1005:10.1007/s12026-014-8561-9
900:10.1186/s13054-018-2012-2
724:Revue de MĂ©decine Interne
632:10.1038/s41584-018-0081-x
476:10.1007/s00296-010-1529-8
352:Newer medications target
152:are usually negative and
65:systemic autoinflammatory
45:Sir George Frederic Still
38:
26:
21:
1949:Enteropathic arthropathy
1121:Arthritis and Rheumatism
1050:Nat Clin Pract Rheumatol
144:in the blood. Tests for
1601:10.5114/reum.2016.64907
1521:10.4103/0019-5359.53169
955:10.1038/sj.gene.6363922
150:anti-nuclear antibodies
2017:Palindromic rheumatism
1944:Ankylosing spondylitis
1887:Tuberculosis arthritis
1133:10.1002/art.1780300209
84:diagnosis of exclusion
1282:10.3899/jrheum.111549
678:(Suppl 6): vi9–vi22.
401:George Frederic Still
2005:Rheumatoid arthritis
1460:10.12659/ajcr.901846
1062:10.1038/ncprheum0510
993:Immunologic Research
568:Autoimmunity Reviews
1939:Psoriatic arthritis
1361:10.1155/2012/317820
1173:10.2147/TCRM.S64951
527:10.1155/2012/156890
411:Research directions
381:number of new cases
138:swollen lymph nodes
92:autoimmune diseases
1972:systemic sclerosis
1934:Reactive arthritis
1805:Diseases of joints
1737:External resources
1354:(317820): 317820.
943:Genes and Immunity
307:hydroxychloroquine
159:aseptic meningitis
124:Signs and symptoms
2072:
2071:
2030:
2029:
1905:Chondrocalcinosis
1771:
1770:
893:(1). London: 88.
470:(12): 4031–4034.
275:systemic symptoms
262:
261:
146:rheumatoid factor
50:
49:
16:Medical condition
2107:
2057:Bouchard's nodes
2010:Felty's syndrome
1882:Septic arthritis
1870:
1869:
1798:
1791:
1784:
1775:
1774:
1664:
1663:
1659:
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1656:
1631:
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1620:
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1544:
1538:
1523:
1508:Indian J Med Sci
1505:
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1414:
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1309:
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1202:
1196:
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1185:
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1045:
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1038:
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1029:
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868:
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805:
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756:
750:
749:
739:
715:
706:
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663:
654:
653:
643:
611:
602:
601:
583:
559:
550:
549:
539:
529:
505:
496:
495:
459:
335:cyclophosphamide
192:
191:
19:
18:
2115:
2114:
2110:
2109:
2108:
2106:
2105:
2104:
2075:
2074:
2073:
2068:
2052:Heberden's node
2035:Noninflammatory
2026:
1981:
1953:
1920:
1891:
1859:
1855:Joint stiffness
1838:
1807:
1802:
1772:
1767:
1766:
1732:
1731:
1675:
1654:
1652:
1643:
1640:
1635:
1634:
1581:
1577:
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1568:
1555:
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1542:
1540:
1536:
1503:
1497:
1493:
1440:
1436:
1391:
1387:
1340:
1336:
1327:
1325:
1310:
1306:
1275:(10): 2008–11.
1260:
1256:
1203:
1199:
1152:
1148:
1117:
1113:
1089:
1085:
1046:
1042:
1033:
1031:
1027:
988:
982:
978:
938:
934:
878:
874:
865:
863:
859:
812:
806:
802:
757:
753:
730:(10): 635–636.
716:
709:
664:
657:
626:(10): 603–618.
612:
605:
560:
553:
506:
499:
460:
447:
442:
425:
413:
390:
373:
295:
267:
218:Lymphadenopathy
198:Minor criteria
183:
171:
169:Pathophysiology
126:
99:corticosteroids
61:Still's disease
59:) is a form of
17:
12:
11:
5:
2113:
2103:
2102:
2097:
2095:Medical triads
2092:
2087:
2070:
2069:
2067:
2066:
2065:
2064:
2059:
2054:
2047:Osteoarthritis
2044:
2038:
2036:
2032:
2031:
2028:
2027:
2025:
2024:
2019:
2014:
2013:
2012:
2002:
2001:
2000:
1989:
1987:
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1982:
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1969:
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1867:
1861:
1860:
1858:
1857:
1852:
1846:
1844:
1840:
1839:
1837:
1836:
1831:
1829:Oligoarthritis
1826:
1821:
1815:
1813:
1809:
1808:
1801:
1800:
1793:
1786:
1778:
1769:
1768:
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1734:
1733:
1730:
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1718:
1707:
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1676:
1671:
1670:
1668:
1667:Classification
1661:
1660:
1639:
1638:External links
1636:
1633:
1632:
1595:(6): 306–309.
1575:
1566:
1549:
1491:
1434:
1385:
1334:
1304:
1254:
1197:
1146:
1127:(2): 186–194.
1111:
1083:
1040:
999:(1–2): 53–62.
976:
932:
872:
823:(5): 351–365.
800:
771:(3): 168–179.
751:
707:
655:
603:
574:(7): 708–722.
551:
497:
444:
443:
441:
438:
437:
436:
431:
424:
421:
412:
409:
405:E. G. Bywaters
389:
386:
372:
369:
294:
291:
286:
285:
278:
266:
265:Classification
263:
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238:
237:
228:
221:
220:
215:
208:
207:
204:
200:
199:
196:
195:Major criteria
182:
179:
175:Interleukin-18
170:
167:
154:serum ferritin
125:
122:
48:
47:
42:
36:
35:
30:
24:
23:
15:
9:
6:
4:
3:
2:
2112:
2101:
2098:
2096:
2093:
2091:
2090:Rare diseases
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2018:
2015:
2011:
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1868:
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1856:
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1851:
1848:
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1845:
1841:
1835:
1834:Polyarthritis
1832:
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212:Arthralgias
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2062:Osteophyte
1874:Infectious
1850:Joint pain
1722:DiseasesDB
1655:2017-06-08
1543:2019-09-04
1328:2020-06-21
1034:2022-04-21
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440:References
347:infliximab
339:adalimumab
323:etanercept
303:prednisone
130:joint pain
109:, such as
103:prednisone
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343:rituximab
293:Treatment
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181:Diagnosis
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28:Specialty
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