Schnitzler Syndrome - Unraveling the Mystery of a Complex Condition
Introduction: This review paper aims to explore the current state of knowledge regarding the underlying mechanisms of Schnitzler syndrome, its clinical manifestations, the diagnostic challenges, and to analyze current treatment approaches, along with potential complications related to the disease....
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| Format: | Article |
| Language: | English |
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Nicolaus Copernicus University in Toruń
2025-04-01
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| Series: | Quality in Sport |
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| Online Access: | https://apcz.umk.pl/QS/article/view/59394 |
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| author | Paulina Grzeszczuk Aleksandra Głowacka Iwona Skorulska Weronika Barbara Grywińska Agnieszka Kalisz Julia Kozakiewicz Aleksandra Okońska Patrycja Długozima Klaudia Mączewska Kamil Kościelecki |
| author_facet | Paulina Grzeszczuk Aleksandra Głowacka Iwona Skorulska Weronika Barbara Grywińska Agnieszka Kalisz Julia Kozakiewicz Aleksandra Okońska Patrycja Długozima Klaudia Mączewska Kamil Kościelecki |
| author_sort | Paulina Grzeszczuk |
| collection | DOAJ |
| description |
Introduction: This review paper aims to explore the current state of knowledge regarding the underlying mechanisms of Schnitzler syndrome, its clinical manifestations, the diagnostic challenges, and to analyze current treatment approaches, along with potential complications related to the disease.
Materials and Methods: A comprehensive review of the literature was conducted using the PubMed and Google Scholar databases using the following keywords: "Schnitzler syndrome", "Schnitzler-like syndrome", "NETosis", "urticaria", "fever", "monoclonal gammopathy", "neutrophilic dermatosis", "anakinra", "treatment".
Summary: Schnitzler syndrome is a rare autoinflammatory disease that typically affects adults and presents with symptoms such as recurrent urticaria, fever, monoclonal gammopathy, and musculoskeletal pain. The exact cause is still not fully understood, but IL-1 antagonists have shown promising results, indicating IL-1's role in the disease's development. Despite the established diagnostic criteria, the disease still presents significant diagnostic challenges, often leading to delays in recognition or even underdiagnosis. Schnitzler syndrome, particularly if left untreated, can lead to complications such as the development of lymphoproliferative disorders, most commonly Waldenström's macroglobulinemia or, in rare cases, AA amyloidosis.
Conclusions: This review emphasizes the importance of a thorough examination, as its symptoms often resemble those of other diseases, which can lead to misdiagnosis. Due to the complexity of the clinical presentation, effective collaboration among dermatologists, rheumatologists and immunologists is essential. Ongoing research is crucial to better understand the disease’s pathogenesis and to develop more effective and personalized treatment strategies.
Keywords: Schnitzler syndrome, neutrophilic urticarial dermatosis, monoclonal gammopathy
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| format | Article |
| id | doaj-art-460e2f177d304aeeb886697176c19cc7 |
| institution | DOAJ |
| issn | 2450-3118 |
| language | English |
| publishDate | 2025-04-01 |
| publisher | Nicolaus Copernicus University in Toruń |
| record_format | Article |
| series | Quality in Sport |
| spelling | doaj-art-460e2f177d304aeeb886697176c19cc72025-08-20T03:06:09ZengNicolaus Copernicus University in ToruńQuality in Sport2450-31182025-04-014010.12775/QS.2025.40.59394Schnitzler Syndrome - Unraveling the Mystery of a Complex ConditionPaulina Grzeszczuk0https://orcid.org/0009-0003-3522-1567Aleksandra Głowacka1https://orcid.org/0009-0000-9389-7731Iwona Skorulska2https://orcid.org/0009-0006-6326-2464Weronika Barbara Grywińska3https://orcid.org/0009-0004-8609-9626Agnieszka Kalisz4https://orcid.org/0009-0001-2996-7691Julia Kozakiewicz5https://orcid.org/0009-0002-5435-3007Aleksandra Okońska6https://orcid.org/0009-0001-9036-7150Patrycja Długozima7https://orcid.org/0009-0001-4513-2256Klaudia Mączewska8https://orcid.org/0009-0007-3426-6666Kamil Kościelecki9https://orcid.org/0009-0001-5516-7540University Clinical Hospital In BialystokIndependent Public Complex of Health Care Facilities in Ostrow Mazowiecka ul. S. Dubois 68, 07-300 Ostrów MazowieckaUniversity Clinical Hospital in Bialystok ul. M. C. Skłodowskiej 24a, 15-276 Białystok, PolandIndependent Public Complex of Health Care Facilities in Wyszkow ul. KEN 1, 07-200 WyszkówUniversity Clinical Hospital in Bialystok ul. M. C. Skłodowskiej 24a, 15-276 Białystok, PolandUniversity Clinical Hospital in BialystokUniversity Clinical Hospital in Bialystok ul. M. C. Skłodowskiej 24a, 15-276 Białystok, PolandUniversity Clinical Hospital in BialystokUniversity Clinical Hospital in Bialystok ul. M. C. Skłodowskiej 24a, 15-276 Białystok, PolandVoivodeship Integrated Hospital named after Jedrzej Sniadecki in Bialystok, ul. M. C. Skłodowskiej 26, 15-278 Białystok Introduction: This review paper aims to explore the current state of knowledge regarding the underlying mechanisms of Schnitzler syndrome, its clinical manifestations, the diagnostic challenges, and to analyze current treatment approaches, along with potential complications related to the disease. Materials and Methods: A comprehensive review of the literature was conducted using the PubMed and Google Scholar databases using the following keywords: "Schnitzler syndrome", "Schnitzler-like syndrome", "NETosis", "urticaria", "fever", "monoclonal gammopathy", "neutrophilic dermatosis", "anakinra", "treatment". Summary: Schnitzler syndrome is a rare autoinflammatory disease that typically affects adults and presents with symptoms such as recurrent urticaria, fever, monoclonal gammopathy, and musculoskeletal pain. The exact cause is still not fully understood, but IL-1 antagonists have shown promising results, indicating IL-1's role in the disease's development. Despite the established diagnostic criteria, the disease still presents significant diagnostic challenges, often leading to delays in recognition or even underdiagnosis. Schnitzler syndrome, particularly if left untreated, can lead to complications such as the development of lymphoproliferative disorders, most commonly Waldenström's macroglobulinemia or, in rare cases, AA amyloidosis. Conclusions: This review emphasizes the importance of a thorough examination, as its symptoms often resemble those of other diseases, which can lead to misdiagnosis. Due to the complexity of the clinical presentation, effective collaboration among dermatologists, rheumatologists and immunologists is essential. Ongoing research is crucial to better understand the disease’s pathogenesis and to develop more effective and personalized treatment strategies. Keywords: Schnitzler syndrome, neutrophilic urticarial dermatosis, monoclonal gammopathy https://apcz.umk.pl/QS/article/view/59394schnitzler syndromeneutrophilic urticarial dermatosismonoclonal gammopathy |
| spellingShingle | Paulina Grzeszczuk Aleksandra Głowacka Iwona Skorulska Weronika Barbara Grywińska Agnieszka Kalisz Julia Kozakiewicz Aleksandra Okońska Patrycja Długozima Klaudia Mączewska Kamil Kościelecki Schnitzler Syndrome - Unraveling the Mystery of a Complex Condition Quality in Sport schnitzler syndrome neutrophilic urticarial dermatosis monoclonal gammopathy |
| title | Schnitzler Syndrome - Unraveling the Mystery of a Complex Condition |
| title_full | Schnitzler Syndrome - Unraveling the Mystery of a Complex Condition |
| title_fullStr | Schnitzler Syndrome - Unraveling the Mystery of a Complex Condition |
| title_full_unstemmed | Schnitzler Syndrome - Unraveling the Mystery of a Complex Condition |
| title_short | Schnitzler Syndrome - Unraveling the Mystery of a Complex Condition |
| title_sort | schnitzler syndrome unraveling the mystery of a complex condition |
| topic | schnitzler syndrome neutrophilic urticarial dermatosis monoclonal gammopathy |
| url | https://apcz.umk.pl/QS/article/view/59394 |
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