. "\u0160ediv\u00E1, Anna" . . . "\u0158eh\u00E1k, Zden\u011Bk" . . "\u0160tork, Ji\u0159\u00ED" . . "Schnitzler-syndrom je velmi vz\u00E1cn\u00E9 idiopatick\u00E9 onemocn\u011Bn\u00ED charakterizovan\u00E9 p\u0159edev\u0161\u00EDm tri\u00E1dou: chronick\u00E1 kop\u0159ivka, monoklon\u00E1ln\u00ED gamapatie (typu imunoglobulinu M nebo G) a proz\u00E1n\u011Btliv\u00FD stav organismu. D\u00E1le jsou sou\u010D\u00E1st\u00ED diferenci\u00E1ln\u00ED diagnostiky hore\u010Dky nejasn\u00E9ho p\u016Fvodu, an\u00E9mie chronick\u00FDch chorob, bolesti muskuloskelet\u00E1ln\u00EDho syst\u00E9mu a lymfadenopatie. S ohledem na rekurentn\u00ED charakter z\u00E1n\u011Btliv\u00E9 odpov\u011Bdi organismu, predispozici pro nadm\u011Brnou stimulovanou sekreci interleukinu-1\u00DF a ur\u010Dit\u00E9 podobnosti s kryopyrinopatiemi (neutrofiln\u00ED urtikari\u00E1ln\u00ED dermat\u00F3za v ko\u017En\u00ED biopsii, t\u00E9m\u011B\u0159 univerz\u00E1ln\u00ED l\u00E9\u010Debn\u00FD efekt receptorov\u00E9ho antagonisty interleukinu-1 - anakinry, snad i mutace genu NLRP3) je Schnitzler-syndrom st\u00E1le \u010Dast\u011Bji \u0159azen mezi z\u00EDskan\u00E1 autoinflamatorn\u00ED onemocn\u011Bn\u00ED. Tato pr\u00E1ce zd\u016Fraz\u0148uje multidisciplin\u00E1rn\u00ED p\u0159\u00EDstup k pacient\u016Fm pohl\u00ED\u017Eej\u00EDc na diagn\u00F3zu Schnitzler-syndromu o\u010Dima dermatologa, hematologa, onkologa, internisty, revmatologa, imunologa a patofyziologa. S ohledem na potenci\u00E1ln\u011B \u017Eivot ohro\u017Euj\u00EDc\u00ED komplikace (riziko p\u0159echodu do lymfoproliferativn\u00EDho onemocn\u011Bn\u00ED nebo rozvoje sekund\u00E1rn\u00ED amyloid\u00F3zy) si v\u0161ak klademe za c\u00EDl oslovit co mo\u017En\u00E1 nej\u0161ir\u0161\u00ED l\u00E9ka\u0159skou obec. Za t\u00EDmto \u00FA\u010Delem je \u010Dl\u00E1nek dopln\u011Bn obrazovou dokumentac\u00ED zahrnuj\u00EDc\u00ED jak n\u00E1lezy klinick\u00E9, tak i v\u00FDsledky zobrazovac\u00EDch metod a laboratorn\u00EDho testov\u00E1n\u00ED, stejn\u011B jako histopatologick\u00FD rozbor ko\u017En\u00ED biopsie."@cs . "Schnitzler-syndrom - paradigma multidisciplin\u00E1rn\u00EDho p\u0159\u00EDstupu u velmi vz\u00E1cn\u00E9 diagn\u00F3zy"@cs . . "Mayer, Ji\u0159\u00ED" . . "Schnitzler-syndrom - paradigma multidisciplin\u00E1rn\u00EDho p\u0159\u00EDstupu u velmi vz\u00E1cn\u00E9 diagn\u00F3zy" . . "1" . . "RIV/00216208:11130/14:10289926" . "Szturz, Petr" . "Schnitzler-syndrom je velmi vz\u00E1cn\u00E9 idiopatick\u00E9 onemocn\u011Bn\u00ED charakterizovan\u00E9 p\u0159edev\u0161\u00EDm tri\u00E1dou: chronick\u00E1 kop\u0159ivka, monoklon\u00E1ln\u00ED gamapatie (typu imunoglobulinu M nebo G) a proz\u00E1n\u011Btliv\u00FD stav organismu. D\u00E1le jsou sou\u010D\u00E1st\u00ED diferenci\u00E1ln\u00ED diagnostiky hore\u010Dky nejasn\u00E9ho p\u016Fvodu, an\u00E9mie chronick\u00FDch chorob, bolesti muskuloskelet\u00E1ln\u00EDho syst\u00E9mu a lymfadenopatie. S ohledem na rekurentn\u00ED charakter z\u00E1n\u011Btliv\u00E9 odpov\u011Bdi organismu, predispozici pro nadm\u011Brnou stimulovanou sekreci interleukinu-1\u00DF a ur\u010Dit\u00E9 podobnosti s kryopyrinopatiemi (neutrofiln\u00ED urtikari\u00E1ln\u00ED dermat\u00F3za v ko\u017En\u00ED biopsii, t\u00E9m\u011B\u0159 univerz\u00E1ln\u00ED l\u00E9\u010Debn\u00FD efekt receptorov\u00E9ho antagonisty interleukinu-1 - anakinry, snad i mutace genu NLRP3) je Schnitzler-syndrom st\u00E1le \u010Dast\u011Bji \u0159azen mezi z\u00EDskan\u00E1 autoinflamatorn\u00ED onemocn\u011Bn\u00ED. Tato pr\u00E1ce zd\u016Fraz\u0148uje multidisciplin\u00E1rn\u00ED p\u0159\u00EDstup k pacient\u016Fm pohl\u00ED\u017Eej\u00EDc na diagn\u00F3zu Schnitzler-syndromu o\u010Dima dermatologa, hematologa, onkologa, internisty, revmatologa, imunologa a patofyziologa. S ohledem na potenci\u00E1ln\u011B \u017Eivot ohro\u017Euj\u00EDc\u00ED komplikace (riziko p\u0159echodu do lymfoproliferativn\u00EDho onemocn\u011Bn\u00ED nebo rozvoje sekund\u00E1rn\u00ED amyloid\u00F3zy) si v\u0161ak klademe za c\u00EDl oslovit co mo\u017En\u00E1 nej\u0161ir\u0161\u00ED l\u00E9ka\u0159skou obec. Za t\u00EDmto \u00FA\u010Delem je \u010Dl\u00E1nek dopln\u011Bn obrazovou dokumentac\u00ED zahrnuj\u00EDc\u00ED jak n\u00E1lezy klinick\u00E9, tak i v\u00FDsledky zobrazovac\u00EDch metod a laboratorn\u00EDho testov\u00E1n\u00ED, stejn\u011B jako histopatologick\u00FD rozbor ko\u017En\u00ED biopsie." . . "Schnitzler-syndrom - paradigma multidisciplin\u00E1rn\u00EDho p\u0159\u00EDstupu u velmi vz\u00E1cn\u00E9 diagn\u00F3zy"@cs . . "12"^^ . "biologic medication; anakinra; interleukins; cytokines; monoclonal immunoglobulin; Schnitzler syndrome"@en . . "\u010Cerm\u00E1kov\u00E1, Zde\u0148ka" . . . . "Koukalov\u00E1, Renata" . . "1"^^ . "Schnitzler syndrome a paradigm of multidisciplinary approach in a very rare diagnosis"@en . "CZ - \u010Cesk\u00E1 republika" . . . "Schnitzler syndrome a paradigm of multidisciplinary approach in a very rare diagnosis"@en . "11130" . "Schnitzler-syndrom - paradigma multidisciplin\u00E1rn\u00EDho p\u0159\u00EDstupu u velmi vz\u00E1cn\u00E9 diagn\u00F3zy" . . "Vok\u00E1\u010Dov\u00E1, Alena" . "Fojt\u00EDk, Zden\u011Bk" . "Schnitzler syndrome is a very rare idiopathic disease characterized above all by: chronic urticaria, monoclonal gammopathy (immunoglobulin M or G types) and proinflammatory state of the organism. Moreover, it should be considered in differential diagnosis of fever of unknown origin, chronic disease anemia, pains of the musculoskeletal system as well as lymphadenopathy. Due to recurring nature of inflammatory response of the organism, predisposition for excessive stimulated interleukin-1\u00DF secretion and certain similarities with cryopyrinopathies (neutrophilic urticarial dermatosis in skin biopsy, almost universal treatment efficacy of interleukin-1 receptor antagonist - anakinra, perhaps also NLRP3 gene mutation), Schnitzler syndrome is being increasingly classified as an acquired autoinflammatory disorder. In our work, we stress a multidisciplinary approach to Schnitzler syndrome from dermatologist's, hematologist's, oncologist's, internist's, rheumatologist's, immunologist's and pathophysiologist's points of view. Regarding potentially life-threatening complications however (risks of transformation into a lymphoproliferation and development of secondary amyloidosis), we aim to address as many medical specialties as possible. With this objective in mind, we have added to the article an illustrated supplementary material including clinical findings, results from imaging methods and laboratory tests as well as a histopathological examination of a skin biopsy."@en . . "Postgradu\u00E1ln\u00ED medic\u00EDna" . "7"^^ . "P(NT13190), S, V" . "Steyerov\u00E1, Petra" . "RIV/00216208:11130/14:10289926!RIV15-MSM-11130___" . "[A071F0D5DEC7]" . "Adam, Zden\u011Bk" . "1212-4184" . "Gottwaldov\u00E1, Jana" . "16" . "43895" .