47:
238:
In the
Strasbourg criteria, the person must have hives and the presence of monoclonal IgM or IgG. Schnitzler's is diagnosed if the person has IgM and two of the following, or IgG and three of the following: recurrent fevers, abnormalities in bone imaging, with or without bone pain, findings of
351:
Because anakinra is so highly and rapidly effective for inducing complete remission of
Schnitzler syndrome, it has been suggested that in patients who do not respond to anakinra, the diagnosis should be reconsidered. Anakinra is not curative, however; symptoms recur soon after treatment stops.
157:
As of 2017 the cause of the disease was not understood. A 2024 review by Braud and
Lipsker aimed to "describe what is currently known about the pathogenesis of this peculiar disease, as well as to address its diagnosis and management" and concluded that "physiopathology of Schnitzler syndrome
1023:
407:
on the taxonomy and definition of auto-inflammatory diseases, published in 2018, considered the alternative name "late onset gammopathy with recurrent urticaria and fever" but this received little support.
227:
The
Lipsker criteria require hives, the presence of monoclonal IgM, and at least 2 of the following: fever, joint pain or arthritis, bone pain, swollen lymph nodes, enlarged spleen or liver, elevated
919:
de Koning HD, Bodar EJ, van der Meer JW, Simon A (December 2007). "Schnitzler syndrome: beyond the case reports: review and follow-up of 94 patients with an emphasis on prognosis and treatment".
282:
294:
224:
There are two sets of diagnostic criteria, the
Lipsker criteria published in 2001 and the Strasbourg criteria that were produced at a meeting in that city in 2012.
751:
Betrains, A; Staels, F; Vanderschueren, S (August 2020). "Efficacy and safety of canakinumab treatment in schnitzler syndrome: A systematic literature review".
158:
remains elusive" and "the main question regarding the relationship between the autoinflammatory features and the monoclonal gammopathy remains to be answered".
125:. This treatment controls the condition but does not cure it. Around 15% of people develop complications, but the condition generally does not shorten life.
953:
L. Schnitzler, Lésions urticariennes chroniques permanentes (érythème pétaloïde?) Cas cliniques No 46 B, J Dermatol Angers (1972) Abstract 46.
963:
Ben-Chetrit, Eldad; Gattorno, Marco; Gul, Ahmet; Kastner, Daniel L; Lachmann, Helen J; Touitou, Isabelle; Ruperto, Nicolino (November 2018).
365:
1111:
149:, mostly on the torso and limbs, often with recurring fever, joint pain, bone pain, muscle pain, headache, fatigue, and loss of weight.
313:
activity have been the preferred treatment since they emerged in 2005; since 2012 a consensus guideline has recommended treatment with
1116:
348:
which stated that "Anakinra may be used as a first line treatment in patients with a documented diagnosis of
Schnitzler syndrome".
795:
383:
It is a rare condition; as of
September 2014, 281 cases had been reported and as of 2017 around 300 cases had been reported.
360:
Generally treatment with anakinra prevents outbreaks but they resume if treatment is stopped. In around 15-20% of people, a
228:
198:
270:
361:
185:
is raised has been described, which appears to be one-tenth as common. The immunoglobulins may show up in the urine as
392:
178:
375:
The life span in patients with
Schnitzler syndrome has not been shown to differ much from the general population.
298:
326:
206:
134:
114:
1038:
337:
was "an effective long-term treatment with a favorable safety profile in patients with
Schnitzler syndrome".
417:
1121:
1089:
797:
Clinical
Commissioning Policy: Anakinra to treat periodic fevers and autoinflammatory disorders (all ages)
346:
Clinical Commissioning Policy: Anakinra to treat periodic fevers and autoinflammatory disorders (all ages)
254:
965:"Consensus proposal for taxonomy and definition of the autoinflammatory diseases (AIDs): a Delphi study"
560:"Schnitzler Syndrome: Insights into Its Pathogenesis, Clinical Manifestations, and Current Management"
103:
46:
1027:
186:
776:
684:
635:
489:
396:
248:
202:
107:
68:
824:"Schnitzler syndrome: validation and applicability of diagnostic criteria in real-life patients"
1049:
986:
936:
901:
845:
768:
733:
676:
627:
540:
481:
318:
99:
35:
976:
928:
891:
881:
835:
760:
723:
715:
666:
617:
581:
571:
530:
520:
473:
330:
286:
190:
322:
170:
653:
Simon A, Asli B, Braun-Falco M, De Koning H, Fermand JP, Grattan C, et al. (2013).
586:
559:
896:
869:
728:
703:
535:
508:
369:
258:
214:
1032:
1105:
932:
780:
764:
622:
605:
404:
310:
122:
981:
964:
688:
493:
1078:
639:
604:
Lipsker D, Veran Y, Grunenberger F, Cribier B, Heid E, Grosshans E (January 2001).
290:
266:
232:
194:
64:
1043:
525:
341:
334:
262:
244:
91:
1054:
477:
274:
240:
166:
83:
40:
1073:
265:
or chronic hives that should be ruled out, include (and are not limited to)
210:
145:
The typical onset is at around 55 years old, and the symptoms are recurrent
87:
79:
990:
940:
905:
886:
849:
772:
737:
719:
680:
631:
544:
485:
309:
As of 2017, no drug was approved to treat Schnitzlers. Drugs that inhibit
1084:
576:
400:
314:
278:
118:
1015:
95:
840:
823:
671:
654:
606:"The Schnitzler syndrome. Four new cases and review of the literature"
189:. Signs of inflammation are often present: these include an increased
464:
Gusdorf L, Lipsker D (August 2017). "Schnitzler Syndrome: a Review".
218:
146:
75:
71:
182:
174:
655:"Schnitzler's syndrome: diagnosis, treatment, and follow-up"
372:
has also been reported in people with Schnitzler syndrome.
1005:
1064:
1009:
509:"Schnitzler syndrome and Schnitzler-like syndromes"
34:
26:
21:
283:monoclonal gammopathy of undetermined significance
704:"Schnitzler syndrome with cold-induced urticaria"
870:"Schnitzler's syndrome: lessons from 281 cases"
295:neonatal onset multisystem inflammatory disease
599:
597:
459:
457:
455:
453:
333:may be effective. A 2020 review reported that
451:
449:
447:
445:
443:
441:
439:
437:
435:
433:
8:
863:
861:
859:
558:Braud, Antoine; Lipsker, Dan (31 May 2024).
235:, and findings of problems in bone imaging.
67:characterised by onset around middle age of
702:Kurian A, Lee JK, Vadas P (December 2010).
1006:
822:Gusdorf, L.; et al. (February 2017).
45:
18:
980:
895:
885:
839:
727:
670:
621:
585:
575:
534:
524:
429:
708:Journal of Dermatological Case Reports
269:or autoinflammatory disorders such as
247:, high levels of white blood cells or
169:show a high concentration of specific
113:Schnitzler syndrome is considered an
7:
921:Seminars in Arthritis and Rheumatism
753:Seminars in Arthritis and Rheumatism
209:. Bone abnormalities can be seen on
133:Schnitzler syndrome is a late-onset
874:Clinical and Translational Allergy
277:, hematological disorders such as
181:of the Îş-type. A variant in which
14:
507:Chu, Cong-Qiu (27 January 2022).
364:as a complication, most commonly
969:Annals of the Rheumatic Diseases
933:10.1016/j.semarthrit.2007.04.001
765:10.1016/j.semarthrit.2020.05.002
623:10.1097/00005792-200101000-00004
233:high levels of white blood cells
982:10.1136/annrheumdis-2017-212515
366:Waldenström's macroglobulinemia
285:(MGUS), other causes of hives,
173:(monoclonal gammopathy) of the
229:erythrocyte sedimentation rate
199:erythrocyte sedimentation rate
117:and is generally treated with
1:
794:NHS England (29 June 2018).
526:10.1097/CM9.0000000000002015
466:Current Rheumatology Reports
362:lymphoproliferative disorder
213:(often increased density or
271:adult-onset Still's disease
177:type. It almost always has
1138:
1112:Autoinflammatory syndromes
478:10.1007/s11926-017-0673-5
399:who first described this
395:the French dermatologist
327:cyclooxygenase inhibitors
207:anemia of chronic disease
135:autoinflammatory disorder
115:autoinflammatory disorder
108:enlarged spleen and liver
74:(urticaria) and periodic
1117:Urticaria and angioedema
513:Chinese Medical Journal
418:List of skin conditions
887:10.1186/2045-7022-4-41
720:10.3315/jdcr.2010.1060
868:de Koning HD (2014).
299:Muckle–Wells syndrome
61:Schnitzler's syndrome
30:Schnitzler's syndrome
577:10.3390/biom14060646
211:radiological imaging
187:Bence Jones proteins
104:swollen lymph glands
57:Schnitzler syndrome
22:Schnitzler syndrome
1065:External resources
397:Liliane Schnitzler
249:C-reactive protein
243:infiltration in a
203:C-reactive protein
141:Signs and symptoms
88:joint inflammation
1099:
1098:
975:(11): 1558–1565.
841:10.1111/all.13035
672:10.1111/all.12129
519:(10): 1190–1202.
319:Immunosuppressant
121:, which inhibits
54:
53:
16:Medical condition
1129:
1007:
995:
994:
984:
960:
954:
951:
945:
944:
916:
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899:
889:
865:
854:
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843:
819:
813:
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810:
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791:
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742:
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731:
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650:
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601:
592:
591:
589:
579:
555:
549:
548:
538:
528:
504:
498:
497:
461:
331:interferon alpha
287:cryoglobulinemia
257:which can cause
255:Other conditions
191:white blood cell
86:(sometimes with
50:
49:
19:
1137:
1136:
1132:
1131:
1130:
1128:
1127:
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1100:
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948:
918:
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749:
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652:
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647:
603:
602:
595:
557:
556:
552:
506:
505:
501:
463:
462:
431:
426:
414:
391:The disease is
389:
381:
358:
323:corticosteroids
307:
259:periodic fevers
205:. There can be
197:) and a raised
171:gamma-globulins
164:
155:
143:
131:
44:
17:
12:
11:
5:
1135:
1133:
1125:
1124:
1119:
1114:
1104:
1103:
1097:
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1081:
1069:
1068:
1066:
1062:
1061:
1058:
1057:
1046:
1035:
1019:
1014:
1013:
1011:
1010:Classification
1003:
1002:External links
1000:
997:
996:
955:
946:
911:
855:
834:(2): 177–182.
814:
786:
759:(4): 636–642.
743:
694:
645:
593:
550:
499:
428:
427:
425:
422:
421:
420:
413:
410:
388:
385:
380:
377:
370:AA amyloidosis
357:
354:
321:drugs such as
306:
303:
215:osteosclerosis
163:
160:
154:
151:
142:
139:
130:
129:Classification
127:
52:
51:
38:
32:
31:
28:
24:
23:
15:
13:
10:
9:
6:
4:
3:
2:
1134:
1123:
1122:Rare diseases
1120:
1118:
1115:
1113:
1110:
1109:
1107:
1091:
1087:
1086:
1082:
1080:
1076:
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1036:
1034:
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1021:
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992:
988:
983:
978:
974:
970:
966:
959:
956:
950:
947:
942:
938:
934:
930:
927:(3): 137–48.
926:
922:
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898:
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888:
883:
879:
875:
871:
864:
862:
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611:
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546:
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532:
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518:
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487:
483:
479:
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386:
384:
378:
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373:
371:
367:
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355:
353:
349:
347:
343:
340:In June 2018
338:
336:
332:
328:
324:
320:
316:
312:
311:interleukin 1
304:
302:
300:
296:
292:
288:
284:
280:
276:
272:
268:
264:
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256:
252:
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176:
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168:
161:
159:
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148:
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136:
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126:
124:
123:interleukin 1
120:
116:
111:
109:
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101:
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93:
89:
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81:
77:
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39:
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33:
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1022:
972:
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924:
920:
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877:
873:
831:
827:
817:
805:. Retrieved
803:. p. 18
796:
789:
756:
752:
746:
711:
707:
697:
665:(5): 562–8.
662:
658:
648:
616:(1): 37–44.
613:
609:
567:
564:Biomolecules
563:
553:
516:
512:
502:
469:
465:
405:Delphi study
390:
382:
379:Epidemiology
374:
368:, develops.
359:
350:
345:
344:published a
339:
308:
291:mastocytosis
263:paraproteins
253:
237:
226:
223:
195:leukocytosis
179:light chains
165:
156:
144:
132:
112:
65:rare disease
60:
56:
55:
714:(4): 50–3.
403:in 1972. A
393:named after
342:NHS England
335:canakinumab
245:skin biopsy
167:Blood tests
92:weight loss
27:Other names
1106:Categories
1050:DiseasesDB
570:(6): 646.
424:References
293:, chronic
275:angioedema
267:autoimmune
241:neutrophil
84:joint pain
41:Immunology
1074:eMedicine
781:219398134
472:(8): 46.
305:Treatment
162:Diagnosis
80:bone pain
36:Specialty
1085:Orphanet
1079:derm/489
991:30100561
941:17586002
906:25905009
850:27564982
773:32502728
738:21886751
689:12831354
681:23480774
632:11204501
610:Medicine
587:11202231
545:35089885
494:13780498
486:28718061
412:See also
401:syndrome
356:Outcomes
315:anakinra
279:lymphoma
119:anakinra
1044:D019873
897:4405827
828:Allergy
807:11 July
729:3157821
659:Allergy
640:6023980
536:9337259
387:History
193:count (
100:fatigue
96:malaise
69:chronic
989:
939:
904:
894:
880:: 41.
848:
779:
771:
736:
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543:
533:
492:
484:
219:biopsy
217:) or
43:
1090:37748
1055:31345
1033:273.1
801:(PDF)
777:S2CID
685:S2CID
636:S2CID
490:S2CID
153:Cause
147:hives
76:fever
72:hives
63:is a
1039:MeSH
1028:9-CM
987:PMID
937:PMID
902:PMID
846:PMID
809:2018
769:PMID
734:PMID
677:PMID
628:PMID
541:PMID
482:PMID
201:and
106:and
82:and
1024:ICD
977:doi
929:doi
892:PMC
882:doi
836:doi
761:doi
724:PMC
716:doi
667:doi
618:doi
582:PMC
572:doi
531:PMC
521:doi
517:135
474:doi
317:.
297:or
281:or
183:IgG
175:IgM
90:),
59:or
1108::
1088::
1077::
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1042::
1031::
985:.
973:77
971:.
967:.
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925:37
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900:.
890:.
876:.
872:.
858:^
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832:72
830:.
826:.
775:.
767:.
757:50
755:.
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722:.
710:.
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683:.
675:.
663:68
661:.
657:.
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626:.
614:80
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608:.
596:^
580:.
568:14
566:.
562:.
539:.
529:.
515:.
511:.
488:.
480:.
470:19
468:.
432:^
329:,
325:,
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289:,
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231:,
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669::
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476::
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