Schnitzler’s Syndrome
Differential diagnostics, an overview of therapeutic options and description of 5 cases treated with anakinra


Authors: Zdeněk Adam 1;  Anna Šedivá 2;  Renata Koukalová 3;  Zdeněk Řehák 3;  Hana Petrášová 4;  Petr Szturz 1;  Zdenka Adamová 5;  Eva Vetešníková 1;  Luděk Pour 1;  Marta Krejčí 1;  Viera Sandecká 1;  Eva Pourová 6;  Zdeňka Čermáková 7;  Sabina Ševčíková 8;  Zdeněk Král 1;  Jiří Mayer 1
Authors‘ workplace: Interní hematologická a onkologická klinika LF MU a FN Brno, pracoviště Bohunice 1;  Ústav imunologie 2. LF UK a FN v Motole, Praha 2;  Oddělení nukleární medicíny, centrum PET, RECAMO, Masarykova onkologického ústavu Brno 3;  Radiologická klinika LF MU a FN Brno, pracoviště Bohunice 4;  Ambulance pro děti a dorost, Obilní trh 9, Brno 5;  Ambulace praktického lékaře pro dospělé, Pustiměř 6;  Oddělení klinické biochemie FN Brno a Katedra laboratorních metod LF MU Brno 7;  Ústav patologické fyziologie LF MU Brno 8
Published in: Vnitř Lék 2016; 62(9): 713-727
Category: Reviews

Overview

Schnitzler’s syndrome is an acquired auto-inflammatory disease of still unclear origin. The Strasbourg criteria were adopted (non-infectious fever, chronic urticaria, changes in the bone structure, leukocytosis and higher values of inflammatory markers – CRP and presence of monoclonal immunoglobulin mostly of type IgM, very rarely of IgG) to establish this diagnosis. The first-choice therapy for this disease is the blocking of interleukin-1 effects. In practice, the interleukin-1 receptor antagonist, anakinra, is the most commonly used. Currently reports also appear of the use of other medicines blocking the effect of interleukin-1, namely canakinumab and rilonacept. We have been treating 5 patients with anakinra (108, 72, 33, 32 and 1 months) on a long-term basis. In all the patients, we commenced administration of anakinra in a dose of 100 mg once a day. As a result of 100 mg being administered once a day, all symptoms went away completely in 4 patients, while they receded by about 75 % in 1 patient, without disappearing completely. This patient needs an increased dose of 2 ampoules per day on the days of spontaneously intensified medical ailments. After one year of treatment it turned out for one of the four patients whose symptoms had completely disappeared when administered the 100mg daily dose, that he only needed the respective dose of anakinra at 48-hour intervals. However this patient does not tolerate further extension of the intervals between dose administrations. We have not recorded any adverse effects of anakinra in the course of the treatment, and no decline in the efficiency of anakinra has been observed: it acts as effectively now as it did at the beginning of the treatment. The text discusses the differential diagnostics of the Schnitzler syndrome.

Key words:
anakinra – auto-inflammatory diseases – canakinumab – fever of unknown origin – FUO – interleukin 1 – cryopyrin-associated autoinflammatory syndrome (CAPS) – monoclonal gammopathy – rilonacept – Schnitzler’s Syndrome – Adult Still’s disease


Sources

1. Schnitzler L. Lésions urticariennes chroniques permanentes (érythème pétaloïde ?) Cas cliniques, n° 46 B. Journée Dermatologique d‘Angers, 28 octobre 1972.

2. Schnitzler L, Schubert B, Boasson M et al. Urticaire chronique, lésions osseuses, macroglobulinémie IgM: maladie de Waldenström? 2ème présentation. Bull Soc Fr Dermatol Syphil 1974; 81: 363–366.

3. Schnitzler L, Hurez D, Verret JL. Urticaire chronique, ostéocondensation, macroglobulinémie. Cas princeps. Etude sur 20 ans. Ann Dermatol Venereol 1989; 116(8): 547–550.

4. Lipsker D, Veran Y, Grunenberger F et al. The Schnitzler syndrome. Four new cases and review of the literature. Medicine (Baltimore) 2001; 80(1): 37–44.

5. de Koning HD, Bodar EJ, van der Meer JW. Schnitzler Syndrome Study Group. Schnitzler syndrome: beyond the case reports: review and follow-up of 94 patients with an emphasis on prognosis and treatment. Semin Arthritis Rheum 2007; 37(3): 137–148.

6. Lipsker D. The Schnitzler syndrome. Orphanet J Rare Dis 2010; 8: 38. Dostupné z DOI: <http://dx.doi.org/10.1186/1750–1172–8-38>.

7. Kyle RA, Therneau TM, Rajkumar SV et al. Long-term follow-up of IgM monoclonal gammopathy of undetermined significance. Blood 2003; 102(10): 3759–3764.

8. Claes K, Bammens B, Delforge M et al. Another devastating complication of the Schnitzler syndrome: AA amyloidosis. Br J Dermatol 2008; 158(1): 182–184.

9. Simon A, Asli B, Braun-Falco M et al. Schnitzler syndrome: diagnosis, treatment and follow up. Alergy 2013; 68(5): 562–568. Dostupné z DOI: <http://dx.doi.org/10.1111/all.12129>.

10. Adam Z, Krejčí M, Pour L et al. Schnitzlerův syndrom – popis čtrnáctiletého průběhu nemoci a přehled informací o této nemoci. Vnitř Lék 2008; 54(12): 1140–1153.

11. Cetkovská P, Benáková N. Autoinflamatorní syndromy s kožními projevy. Čes Dermat 2015; 90(4): 144–155.

12. Tichá M. Terapie autoinflamatorních onemocnění a periodických horeček. Alergie 2015; 17(2): 128–135.

13. Šedivá A. Nové poznatky a pokroky v oblasti primárních imunodeficiencí. Postgraduální medicína 2015; 17(3): 294–303.

14. Blažová K, Horáčková M, Horváth R et al. Kardiorenální syndrom při AA amyloidóze v důsledku kombinace dvou mutací genů pro periodické horečky. Aktuality v nefrologii 2013; 19(4): 127–130.

15. Doležalová P. Syndrom CINCA – nejzávažnější onemocnění ze skupiny kryopyrinopatií. Farmakoterapie 2012; 8(Suppl 1): 8–9.

16. Šedivá A. Autoinflamatorní onemocnění v dermatologii. Čes Dermat 2012; 2(4): 239–243.

17. Šedivá A. Autoinflamatorní onemocnění a familiární středozemní horečka. Alergie 2012; 14(Suppl 2): 26.

18. Šedivá A. Periodické horečky a autoinflamatorní onemocnění – klinický přístup. Postgraduální medicína 2012; 14(2): 174–179.

19. Šedivá A. Periodické horečky AU. Alergie 2009; 11: P1-P16.

20. Šedivá A. Periodické horečky a další syndromy s poruchou regulace zánětlivé odpovědi. Českoslov Ped 2011; 66(3): 187–191.

21. Němcová D, Doležalová D, Brejchová I. Autoinflamatorní onemocnění kostí. Čes Revmat 2010; 18(3): 10–15.

22. Doležalová P, Król P, Němcová D. Autoinflamatorní onemocnění – přehled. Čes Revmat 2010; 18(3): 144–145.

23. Cush JJ. Autoinflamatory syndromes. Dermatol Clin 2013; 31(3): 471–480. Dostupné z DOI: <http://dx.doi.org/10.1016/j.det.2013.05.001>.

24. Yu JR, Leslie KS. Cryopyrin-associated periodic syndrome: an update on diagnosis and treatment response. Curr Allergy Asthma Rep 2011; 11(1): 12–20. Dostupné z DOI: <http://dx.doi.org/10.1007/s11882–010–0160–9>.

25. Kötter I, Schedel J, Kümmerle-Deschner JB. Periodic fever syndrome/autoinflammatory syndrome. Z Rheumatol 2009; 68(2): 137–148.

26. Jesus AA, Goldbach-Mansky R. Il-1 blokade in autoinflamatory syndromes. Annu Rev Med 2014; 65: 233–244. Dostupné z DOI: <http://dx.doi.org/10.1146/annurev-med-061512–150641>.

27. Katra R, Dytrych P, Groh D et al. PFAPA syndrom v ORL oblasti a jeho indikace k tonzilektomii. Otorinolaryngologie a foniatrie 2010; 59(4): 197–201.

28. Beer HD, Contassot E, Frech LE. The inflammasome in autoinflamatory disease with skin involvement. J Investigative Dermatol 2014; 134(7): 1805–1810. Dostupné z DOI: <http://dx.doi.org/10.1038/jid.2014.76>.

29. Valentin MG, Jamilloux Y, Iwaz Y et al. Adult onset Sill´s disease. Autoimmun Rev 2014; 13(7): 708–722. Dostupné z DOI: <http://dx.doi.org/10.1016/j.autrev.2014.01.058>.

30. Malegová J, Koen L, Horák P. Stillova nemoc dospělých, obtížná cesta k diagnóze přes horečku a výpotky nejasné etiologie. Vnitřní Lék 2014; 60(5–6): 520–526.

31. Skácelová, M, Horák P. Stillova nemoc dospělých. Čes Revmat 2012; 20(4): 198–202.

32. Skácelová M, Horák P. Stillova choroba dospělých. Postgraduální medicína 2015; 17(4): 391–394.

33. Houzarová A. Stillova nemoc. Referátový výběr z revmatologie 2007; 47(3): 1214–1216.

34. Fischer-Betz R, Specker C, Schneider M. Successful outcome of two pregnancies in patients with adult-onset Still‘s disease treated with IL-1 receptor antagonist (anakinra). Clin Exp Rheumatol 2011; 29(6): 1021–1023.

35. Jamilloux Y, Gerfaund-Valentin M, Henry T et al. Treatment of adult-onset Still´s disease: a review. Ther Clin Risk Manag 2015; 11: 33–43. Dostupné z DOI: <http://dx.doi.org/10.2147/TCRM.S64951>.

36. Ortiz-Sanjuán F, Blanco R, Riancho-Zarrabeitia L et al. Efficacy of Anakinra in Refractory Adult-Onset Still‘s Disease: Multicenter Study of 41 Patients and Literature Review. Medicine (Baltimore) 2015; 94(39): e1554. Dostupné z DOI: <http://dx.doi.org/10.1097/MD.0000000000001554>.

37. Jamilloux Y, Gerfaud-Valentin M, Henry T et al. Treatment of adult-onset Still‘s disease: a review. Ther Clin Risk Manag 2014; 11: 33–43.

38. Hong D, Yang Z, Han S et al. Interleukin 1 inhibition with anakinra in adult-onset Still disease: a meta-analysis of its efficacy and safety. Drug Des Devel Ther 2014; 8: 2345–2357. Dostupné z DOI: <http://dx.doi.org/10.2147/DDDT.S73428>.

39. Gerfaud-Valentin M, Jamilloux Y, Iwaz J at al. Adult-onset Still‘s disease. Autoimmun Rev 2014; 13(7): 708–722.

40. de Koning HD, van Gijn ME, Stoffels M et al. Myeloid lineage-restricted somatic mosaicism of NLRP3 mutations in patients with variant Schnitzler syndrome. J Allergy Clin Immunol 2015; 135(2): 561–564.

41. Pizzirani C, Falzoni S, Govoni M et al. Dysfunctional inflammasome in Schnitzler‘s syndrome. Rheumatology (Oxford) 2009; 48(10): 1304–1308.

42. Loock J, Lamprecht P, Timmann C et al. Genetic predisposition (NLRP3 V198M mutation) for IL-1-mediated inflammation in a patient with Schnitzler syndrome. J Allergy Clin Immunol 2010; 125(2): 500–502.

43. Szturz P, Šedivá A, Žurek M et al. Léčba anakinrou u Schnitzler-syndromu – výsledky první retrospektivní multicentrické studie šesti pacientů z České republiky. Klin Onkol 2014; 27(2): 111–126.

44. Lamprecht P. Adult-onset Still‘s disease, Schnitzler syndrome, and autoinflammatory syndromes in adulthood. Z Rheumatol 2009; 68(9): 740–746. Dostupné z DOI: <http://dx.doi.org/10.1007/s00393–009–0490-y>.

45. Simon A, Asli B, Braun-Falco M et al. Schnitzler‘s syndrome: diagnosis, treatment, and follow-up. Allergy 2013; 68(5): 562–568. Dostupné z DOI: <http://dx.doi.org/10.1111/all.12129>.

46. Patel S, Sindher S, Jariwala S et al. Chronic urticaria with monoclonal IgG gammopathy: a clinical variant of Schnitzler syndrome? Ann Allergy Asthma Immunol 2012; 109(2): 147–148.

47. Sokumbi O, Drage LA, Peters MS. Clinical and histopathologic review of Schnitzler syndrome: the Mayo Clinic experience (1972–2011). J Am Acad Dermatol 2012; 67(6): 1289–1295.

48. Flórez AF, Gallardo Agromayor E, García-Barredo R et al. Radiological aid to clinical diagnosis of Schnitzler‘s syndrome: multimodality imaging approach. Clin Rheumatol 2008; 27(1): 107–110.

49. Lee KY, Grattan CE. Intracostal neuralgia as a previously undescribed symptom of Schnitzler‘s syndrome. Br J Dermatol 2012; 167(6): 1392–1393. Dostupné z DOI: <http://dx.doi.org/10.1111/j.1365–2133.2012.11057.x>.

50. Terpos E, Asli B, Chistoulas D et al. Increased Angiogenesis and Enhanced Bone Formation in Patients with IgM Monoclonal Gammopathy and Urticarial Skin Rash: New Insight into the Biology of the Schnitzler Syndrome. Haematologica 2012; 97(11): 1699–1703. Dostupné z DOI: <http://dx.doi.org/10.3324/haematol.2012.067306>.

51. Niederhauser BD, Dingli D, Kyle RA et al. Imaging findings in 22 cases of Schnitzler syndrome: characteristic paraarticular osteosclerosis and the hot knees sign differential diagnosis. Skeletal Radiol 2014; 43(7): 905–915. Dostupné z DOI: <http://dx.doi.org/10.1007/s00256–014–1857-y>.

52. Willekens I, Walgraeve N, Goethals L et al. Correlative bone imaging in a case of Schnitzler´s syndrome a briew review of the literatura. Hell J Nucl Med 2015; 18(1): 71–73.

53. Jarůšková M, Bělohlávek O. Role of FDG-PET and PET/CT in the diagnosis of prolonged febrile states. Eur Journal Nucl Med Mol Imaging 2006; 33(8): 913–918.

54. Kotík L. Teploty nejasného původu. Interní med pro praxi 2006; 8(11): 493–495.

55. Ferdová E, Záhlava J, Ferda J. Horečky nejasného původu, význam hybridního zobrazení 18F-FDG-PET/CT. Čes Radiol 2008; 62(1): 23–33.

56. Lachmann HJ. Autoinflammatory syndromes as causes of fever of unknown origin. Clin Med (Lond) 2015; 15(3): 295–298. Dostupné z DOI: <http://dx.doi.org/10.7861/clinmedicine.15–3-295>.

57. Barbosa NS, Schoch JJ, Ringler MD et al. Chronic urticarial eruption associated with monoclonal gammopathy. Am J Hematol 2015; 90(4): 365–366. Dostupné z DOI: <http://dx.doi.org/10.1002/ajh.23873>.

58. Šedivá A, Slíva J, Doležalová P et al. Anakinra. Farmakoterapie 2011; 7(6): 621–629.

59. Jesus AA, Goldbach-Mansky R. IL-1 blockade in autoinflammatory syndromes. Annu Rev Med 2014; 65: 223–44. Dostupné z DOI: <http://dx.doi.org/10.1146/annurev-med-061512–150641>.

60. Pazyar N, Feily A, Yaghoobi R. An overview of interleukin-1 receptor antagonist, anakinra, in the treatment of cutaneous diseases. Curr Clin Pharmacol 2012; 7(4): 271–275.

61. Neel A, Henry B, Barbarot S et al. Long-term effectiveness and safety of interleukin-1 receptor antagonist (anakinra) in Schnitzler´s syndrome. A French multicenter study. Autoimmune Rev 2014; 13(10): 1035–1041. Dostupné z DOI: <http://dx.doi.org/10.1016/j.autrev.2014.08.031>.

62. Martinez-Taboada VM, Fontalba A et al. Successful treatment of refractory Schnitzler syndrome with anakinra: comment on the article by Hawkins et al. Arthritis Rheum 2005; 52(7): 2226–2227.

63. de Koning HD, van der Meer JW, Simon A Comment on: Schnitzlers syndrome-exacerbation after anti-TNF treatment. Rheumatology (Oxford) 2007; 46(11): 1741.

64. Besada E, Nossent H. Dramatic response to IL-1-RA treatment in longstanding multidrug resistant Schnitzler‘s syndrome: a case report and literature review. Clin Rheumatol 2010; 29(5): 567–571. Dostupné z DOI: <http://dx.doi.org/10.1007/s10067–010–1375–9>.

65. Billey T, Beldjerd M, Popa L et al. Schnitzler syndrome: a dramatic improvement with anakinra. Presse Med 2010; 39(12): 1338–1339. Dostupné z DOI: <http://dx.doi.org/10.1016/j.lpm.2010.07.009>.

66. Cascavilla N, D‘Arena G, Dell‘OLio M. Schnitzler syndrome. Br J Haematol 2008; 143(2): 152. 10.1111/j.1365–2141.2008.07287.x

67. Cascavilla N, Bisceglia M, D‘Arena G. Successful treatment of Schnitzler‘s syndrome with anakinra after failure of rituximab trial. Int J Immunopathol Pharmacol 2010; 23(2): 633–636.

68. Crouch R, Akhras V, Sarkany R. Schnitzler‘s syndrome: successful treatment with anakinra. Australas J Dermatol 2007; 48(3): 178–181.

69. Devlin LA, Wright G, Edgar JD. A rare cause of a common symptom Anakinra is effective in the urticaria of Schnitzler Syndrome: a case report. Cases J 2008; 1(1): 348. Dostupné z DOI: <http://dx.doi.org/10.1186/1757–1626–1-348>.

70. Dybowski F, Sepp N, Bergerhausen HJ et al. Successful use of anakinra to treat refractory Schnitzler‘s syndrome. Clin Exp Rheumatol 2008; 26(2): 354–357.

71. Eiling E, Möller M, Kreiselmaier I et al. Schnitzler syndrome: treatment failure to rituximab but response to anakinra. J Am Acad Dermatol 2007; 57(2): 361–364.

72. Eiling E, Schröder JO, Gross WL et al. The Schnitzler syndrome: chronic urticaria and monoclonal gammopathy – an autoinflammatory syndrome? J Dtsch Dermatol Ges 2008; 6(8): 626–631. Dostupné z DOI: <http://dx.doi.org/10.1111/j.1610–0387.2008.06627.x>.

73. Frischmeyer-Guerrerio PA, Rachamalla R, Saini SS. Remission of Schnitzler syndrome after treatment with anakinra. Ann Allergy Asthma Immunol 2008; 100(6): 617–619. Dostupné z DOI: <http://dx.doi.org/10.1016/S1081–1206(10)60064–6>.

74. Gilson M, Abad S, Larroche C et al. Treatment of Schnitzler syndrome with anakinra. Clin Exp Rheumatol 2007; 25(6): 931.

75. Klemmer N, Lenain P, Balguerie X et al. Effectiveness of anti-IL1 in Schnitzler‘s syndrome. Joint Bone Spine 2007; 74(5): 509–510.

76. Kluger N, Rivière S, Guillot B et al. Efficacy of interleukin 1 receptor antagonist (anakinra) on a refractory case of Schnitzler‘s syndrome. Acta Derm Venereol 2008; 88(3): 287–288. Dostupné z DOI: <http://dx.doi.org/10.2340/00015555–0404>.

77. Larocca CA, McEvoy JW, Ellis CL et al.. Schnitzler‘s syndrome associated with pancreatitis: a disease of IL-1 dysregulation. Clin Rheumatol 2012; 31(1): 169–174. Dostupné z DOI: <http://dx.doi.org/10.1007/s10067–011–1804–4>.

78. Loock J, Lamprecht P, Timmann C et al. Genetic predisposition (NLRP3 V198M mutation) for IL-1-mediated inflammation in a patient with Schnitzler syndrome. J Allergy Clin Immunol 2010; 125(2): 500–502. Dostupné z DOI: <http://dx.doi.org/10.1016/j.jaci.2009.10.066>.

79. Ryan JG, de Koning HD, Beck LA et al. IL-1 blockade in Schnitzler syndrome: ex vivo findings correlate with clinical remission. J Allergy Clin Immunol 2008; 121(1): 260–262.

80. Saiz E, Gálvez J, Mora A et al. Anakinra and Schnitzler‘s syndrome. Med Clin (Barc) 2008; 130(9): 358–359.

81. Schneider SW, Gaubitz M, Luger TA et al. Prompt response of refractory Schnitzler syndrome to treatment with anakinra. J Am Acad Dermatol 2007; 56(5 Suppl): S120-S122.

82. Schuster C, Kränke B, Aberer E et al. Schnitzler syndrome: response to anakinra in two cases and review of the literature. Int J Dermatol 2009; 48(11): 1190–1194. Dostupné z DOI: <http://dx.doi.org/10.1111/j.1365–4632.2009.04151.x>.

83. Sokumbi O, Drage LA, Peters MS. Clinical and histopathologic review of Schnitzler syndrome: the Mayo Clinic experience (1972–2011). J Am Acad Dermatol 2012; 67(6): 1289–1295. Dostupné z DOI: <http://dx.doi.org/10.1016/j.jaad.2012.04.027>.

84. Thonhofer R, Uitz E, Graninger W. Schnitzler‘s syndrome–exacerbation after anti-TNF treatment. Rheumatology (Oxford) 2007; 46(6): 1041–1042.

85. Tinazzi E, Puccetti A, Patuzzo G et al. Schnitzler syndrome, an autoimmune-autoinflammatory syndrome: report of two new cases and review of the literature. Autoimmun Rev 2011; 10(7): 404–409. Dostupné z DOI: <http://dx.doi.org/10.1016/j.autrev.2011.01.003>.

86. Treudler R, Kauer F, Simon JC. Striking effect of the IL-1 receptor antagonist Anakinra in chronic urticarial rash with polyclonal increase in IgA and IgG. Acta Derm Venereol (Stockh) 2007; 87(3): 280–281.

87. van Deuren M, Kroot JJ, Swinkels DW. Time-course analysis of serum hepcidin, iron and cytokines in a C282Y homozygous patient with Schnitzler‘s syndrome treated with IL-1 receptor antagonist. Haematologica 2009; 94(9): 1297–1300. Dostupné z DOI: <http://dx.doi.org/10.3324/haematol.2009.005975>.

88. Vandenhende MA, Bentaberry F, Morlat P et al. Anakinra: an effective treatment in the Schnitzler syndrome. Joint Bone Spine 2011; 78(6): 636–637. Dostupné z DOI: <http://dx.doi.org/10.1016/j.jbspin.2011.03.021>.

89. Volz T, Wölbing F, Fischer J et al. Dermal Interleukin-1 Expression and Effective and Long-lasting Therapy with Interleukin-1 Receptor Antagonist Anakinra in Schnitzler Syndrome. Acta Derm Venereol 2012; 92(4): 393–394. Dostupné z DOI: <http://dx.doi.org/10.2340/00015555–1307>.

90. Wastiaux H, Barbarot S, Gagey-Caron V et al. Schnitzler syndrome: a dramatic improvement with anakinra. J Eur Acad Dermatol Venereol 2009; 23(1): 85–87. Dostupné z DOI: <http://dx.doi.org/10.1111/j.1468–3083.2008.02708.x>.

91. Pesek R, Fox R. Successful treatment of Schnitzler syndrome with canakinumab. Cutis 2014; 94(3): E11-E12.

92. de Koning HD, Schalkwijk J, van der Ven-Jongekrijg J et al. Sustained efficacy of the monoclonal anti-interleukin-1 beta antibody canakinumab in a 9-month trial in Schnitzler‘s syndrome. Ann Rheum Dis 2013; 72(10): 1634–1638. Dostupné z DOI: <http://dx.doi.org/10.1136/annrheumdis-2012–202192>.

93. Vanderschueren S, Knockaert D. Canakinumab in Schnitzler syndrome. Semin Arthritis Rheum 2013; 42(4): 413–416. Dostupné z DOI: <http://dx.doi.org/10.1016/j.semarthrit.2012.06.003>.

94. de Koning HD, Schalkwijk J, van der Meer JW et al. Successful canakinumab treatment identifies IL-1β as a pivotal mediator in Schnitzler syndrome. J Allergy Clin Immunol 2011; 128(6): 1352–1354. Dostupné z DOI: <http://dx.doi.org/10.1016/j.jaci.2011.05.023>.

95. Krause K, Weller K, Stefaniak R et al. Efficacy and safety of the interleukin-1 antagonist rilonacept in Schnitzler syndrome: an open-label study. Allergy 2012; 67(7): 943–950. Dostupné z DOI: <http://dx.doi.org/10.1111/j.1398–9995.2012.02843.x>.

96. Krause K, Feist E, Fiene M et al. Complete remission in 3 of 3 anti-IL-6-treated patients with Schnitzler syndrome. J Allergy Clin Immunol 2012; 129(3): 848–850. Dostupné z DOI: <http://dx.doi.org/10.1016/j.jaci.2011.10.031>.

97. Asli B, Bienvenu B, Cordoliani F et al. Chronic urticaria and monoclonal IgM gammopathy (Schnitzler syndrome): report of 11 cases treated with pefloxacin. Arch Dermatol 2007; 143(8): 1046–1050.

98. Kastritis E, Katoulis A, Terpos E et al. Schnitzler‘s syndrome: increased levels of bone formation and angiogenesis factors are reduced after successful pefloxacin treatment. Clin Lymphoma Myeloma 2008; 8(6): 359–362. Dostupné z DOI: <http://dx.doi.org/10.3816/CLM.2008.n.053>.

99. Aouba A, Pressiat C, Pricopi M et al. Complete remission of Schnitzler syndrome and Waldenström macroglobulinemia under rituximab-cyclophosphamide-dexamethasone. Dermatology 2015; 230(1): 18–22. Dostupné z DOI: <http://dx.doi.org/10.1159/000368349>.

100. Król P, Doležalová P. Horečka jako hlavní projev nemoci. Pediatrie pro praxi 2011; 12(2): 111–114.

101. Doležalová P, Król P. Recidivující horečky u dětí. Vox pediatriae 2010; 10(3): 16–20.

102. Tomíčková D. Horečka neznámého původu. Lékařské listy 2009; 58(8): 9–11.

103. Křivanová A, Adam Z, Mayer J. Teplota nejasné etiologie: příčiny a diagnostický postup. Vnitř Lék 2007; 53(2): 169–178.

104. Toplak N, Dolezalova P, Constantin T et al. [Eastern/Central European autoinflammatory collaborating group for the Paediatric Rheumatology International Trials Organization (PRINTO) and Eurofever Project]. Periodic fever syndromes in Eastern and Central European countries: results of a pediatric multinational survey. Pediatr Rheumatol Online J 2010; 8: 29. Dostupné z DOI: <http://dx.doi.org/10.1186/1546–0096–8-29>.

105. Król P, Böhm M, Sula V et al. PFAPA syndrome: clinical characteristics and treatment outcomes in a large single-centre cohort. Clin Exp Rheumatol 2013; 31(6): 980–9877.

106. Toplak N, Frenkel J, Ozen S et al. An international registry on autoinflammatory diseases: the Eurofever experience. Ann Rheum Dis 2012; 71(7): 1177–1182. Dostupné z DOI: <http://dx.doi.org/10.1136/annrheumdis-2011–200549>.

Labels
Diabetology Endocrinology Internal medicine
Login
Forgotten password

Don‘t have an account?  Create new account

Forgotten password

Enter the email address that you registered with. We will send you instructions on how to set a new password.

Login

Don‘t have an account?  Create new account