ArticleClinical rheumatology2012
Schnitzler's syndrome associated with pancreatitis: a disease of IL-1 dysregulation.
Article in Clinical rheumatology, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 6 papers.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
6 citing papers in PubMed, 13 citations in OpenAlex.
- Schnitzler Syndrome: Insights into Its Pathogenesis, Clinical Manifestations, and Current Management.Biomolecules · 2024Review
- Monoclonal gammopathies of clinical significance (MGCS): In pursuit of optimal treatment.Frontiers in immunology · 2022Review
- An expanding role for interleukin-1 blockade from gout to cancer.Molecular medicine (Cambridge, Mass.) · 2014Review
- Schnitzler's syndrome: lessons from 281 cases.Clinical and translational allergy · 2014Review
- Treating inflammation by blocking interleukin-1 in humans.Seminars in immunology · 2013Review
- Diagnosis and treatment of urticaria and angioedema: a worldwide perspective.The World Allergy Organization journal · 2012Article
Corrections and comments
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Authors and funding
7 authors at 3 institutions in 1 country.
Funding
Abstract
Schnitzler's syndrome (SS) is a rare inflammatory disease of unknown origin characterized by chronic urticaria and monoclonal gammopathy (usually IgM) associated with at least two of the following components: fever, arthralgia or arthritis, bone pain, hepato- and/or splenomegaly, lymphadenopathy, elevated erythrocyte sedimentation rate, leukocytosis, and/or abnormal findings on bone morphological investigations. To date, about 100 cases have been described with only 4 being reported in the USA. The mean time to diagnosis from the onset of disease is 5.4 years, given the varied symptoms with which patients may present. The pathogenesis of SS remains unknown but likely involves dysregulation of the IL-1 pathway. We describe here a 48-year-old woman with a monoclonal IgM gammopathy and a 3-year history of chronic pruritic urticarial dermatosis, unexplained fevers, chronic polyarthritis, lymphadenopathy, leukocytosis, hepatomegaly, and weight loss. She also had a history of chronic pancreatitis as well as a family history of recurrent pancreatitis. The diagnosis of Schnitzler's syndrome was made, and she was successfully treated with the IL-1 receptor antagonist, anakinra.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.