- ICH GCP
- US Clinical Trials Registry
- Clinical Trial NCT00933296
Schnitzler Syndrome: Clinical Study, Physiopathological and Search for Genetic Factors
September 7, 2017 updated by: University Hospital, Strasbourg, France
Schnitzler Syndrome: Clinical Study, Physiopathological and Search for Genetic
The Schnitzler syndrome is a rare entity characterized by an urticarial rash and recurrent fever in a patient with a monoclonal IgM component.
Other frequent signs include joint, bone and muscle pain, enlarged spleen, liver and lymph nodes, increased blood sedimentation rate (BSR), elevated neutrophil count and abnormalities on bone morphologic investigations.
In 2001, the investigators proposed criteria to diagnose this syndrome, which are currently admitted in the literature.
The main complications of the Schnitzler syndrome are a difficult-to-control inflammatory anemia, AA-amyloidosis and malignant B lymphoproliferative disorders.
About 15% of patients with a Schnitzler will eventually develop a lymphoproliferative disorder; thus this syndrome allows studying the relationship between lymphomagenesis and inflammation.
By many aspects, the Schnitzler syndrome is reminiscent of auto-inflammatory syndromes.
Though the term auto-inflammatory disease is as to yet restricted to diseases with Mendelian inheritance, some polygenic inflammatory diseases like for example Crohn's disease clearly involve pathogenetic pathways shared with the monogenic auto-inflammatory syndromes.
The investigators stipulate that this could also be the case in the Schnitzler syndrome for the following reasons: (1) this is a recurrent fever of unknown cause; (2) the peculiar eruption, characterized pathologically by a neutrophilic infiltrate very similar to the one observed in the auto-inflammatory cryopyrinopathies (CINCA/NOMID syndrome, Muckle-Wells syndrome and familial cold-urticaria); the investigators recently individualized this particular eruption, significantly associated with systemic inflammatory disease, within the group of neutrophilic urticarias (Kieffer et al.
Medicine, in press); (3) the occurrence of aseptic neutrophilic osteitis, very similar to the one reported in patients with Majeed syndrome, another auto-inflammatory syndrome; (4) a significant increase of neutrophil count, not otherwise explained; (5) a spectacular response to the IL-1 inhibitor, within hours after the first injection, similar to what is reported in the PAPA (pyogenic arthritis, pyoderma gangrenosum and acne) syndrome or the cryopyrinopathies, suggesting a direct pathogenic effect of IL-1.
Study Overview
Status
Completed
Conditions
Study Type
Observational
Enrollment (Actual)
52
Contacts and Locations
This section provides the contact details for those conducting the study, and information on where this study is being conducted.
Study Locations
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BREST Cedex, France, 29609
- Service de DermatologieCHU Morvan5 avenue Foch
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Besancon, France
- CHU de Besançon Service de Dermatologie
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DAX, France
- Centre Hospitalier Général Bd Yves du Manoir
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LYON Cedex 02, France, 69288
- Service de DermatologieHôtel Dieu
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Le Mans Cedex 9, France, 72037
- Service de Rhumatologie Centre Hospitalier du Mans
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Lille, France
- Service de Médecine Interne Hôpital Claude-Huriez
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MONTPELLIER cedex 5, France, 34295
- Service de Dermatologie Hôpital Saint Eloi 80 avenue A Fliche
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Marseille Cedex 09, France, 13274
- Service médecine interne et thérapeutique Hôpital sainte marguerite
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Nantes, France, 44093
- Service de dermatologie CHU Hôtel Dieu1 place Alexis Ricordeau
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Niort, France, 79021
- Unité de médecine interne Centre Hospitalier G. Renon
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PARIS Cedex 20, France, 75970
- Service de médecine interne Hôpital Tenon4, rue de la Chine
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POITIERS Cedex, France, 86021
- Service de dermatologieCHU GUERIN2 rue de la Milétrie
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Paris, France, 75010
- Service d'immuno-hématologie Hôpital St-Louis 1 av Claude Vellefaux
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Toulon, France, 83041
- BOYE Thierry
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Valenciennes, France, 59322
- Service de médecine interne-néphrologie Centre Hospitalier Valenciennes
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Bari, Italy, 70122
- Azienda Ospedaliero Universitaria Policlinico
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Genova, Italy, 716132
- Clinica DermatologicaUnivesité de GênesVialle Benedetto
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Participation Criteria
Researchers look for people who fit a certain description, called eligibility criteria. Some examples of these criteria are a person's general health condition or prior treatments.
Eligibility Criteria
Ages Eligible for Study
18 years and older (Adult, Older Adult)
Accepts Healthy Volunteers
No
Genders Eligible for Study
All
Sampling Method
Non-Probability Sample
Study Population
University hospitals
Description
Inclusion Criteria:
- patients with the Schnitzler syndrome according to criteria established by Lipsker D et al. Medicine (Baltimore) 2001;80:37-44
Exclusion Criteria:
- children and pregnancy
Study Plan
This section provides details of the study plan, including how the study is designed and what the study is measuring.
How is the study designed?
Design Details
- Observational Models: Case-Control
- Time Perspectives: Prospective
Cohorts and Interventions
Group / Cohort |
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A Patients with the Schnitzler syndrome
Patients with the Schnitzler syndrome
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B Control subjects:
B1 healthy B2 other diseases
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Collaborators and Investigators
This is where you will find people and organizations involved with this study.
Investigators
- Principal Investigator: Dan Lipsker, Pr, HUS
Study record dates
These dates track the progress of study record and summary results submissions to ClinicalTrials.gov. Study records and reported results are reviewed by the National Library of Medicine (NLM) to make sure they meet specific quality control standards before being posted on the public website.
Study Major Dates
Study Start
July 1, 2009
Primary Completion (Actual)
September 1, 2017
Study Completion (Actual)
September 1, 2017
Study Registration Dates
First Submitted
July 3, 2009
First Submitted That Met QC Criteria
July 6, 2009
First Posted (Estimate)
July 7, 2009
Study Record Updates
Last Update Posted (Actual)
September 8, 2017
Last Update Submitted That Met QC Criteria
September 7, 2017
Last Verified
September 1, 2017
More Information
Terms related to this study
Additional Relevant MeSH Terms
Other Study ID Numbers
- 4350 (PCORI)
This information was retrieved directly from the website clinicaltrials.gov without any changes. If you have any requests to change, remove or update your study details, please contact register@clinicaltrials.gov. As soon as a change is implemented on clinicaltrials.gov, this will be updated automatically on our website as well.
Clinical Trials on Schnitzler Syndrome
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Radboud University Medical CenterNovartisCompleted
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Karoline KrauseRoche Pharma AGCompleted
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Olatec Therapeutics LLCRadboud University Medical CenterTerminated
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Universitaire Ziekenhuizen KU LeuvenCompleted
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Charite University, Berlin, GermanyNovartis PharmaceuticalsCompleted
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Radboud University Medical CenterUnknownAutoinflammatory Syndromes | HIDS | TRAPS | Schnitzler's SyndromeNetherlands
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R-Pharm Overseas, Inc.Worldwide Clinical TrialsNot yet recruitingSchnitzler Syndrome | Urticarial Vasculitis With Monoclonal Immunoglobulin M Component, Schnitzler (Disorder)United States
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Charite University, Berlin, GermanyCompletedSchnitzler Syndrome | Muckle-Wells SyndromeGermany
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University of SienaRecruitingBehcet Syndrome | Uveitis | Hereditary Autoinflammatory Diseases | Schnitzler Syndrome | Scleritis | PFAPA Syndrome | Still Disease | Autoinflammatory Syndrome, Unspecified | Vexas SyndromeEgypt, Spain, Turkey, Italy, Saudi Arabia, Greece, Belgium, United States, Algeria, Brazil, Germany, Ghana, Iran, Islamic Republic of, Lebanon, Morocco, Poland, Portugal, Romania, Taiwan
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Neumedicines Inc.Department of Health and Human ServicesCompletedHematopoietic Syndrome Due to Acute Radiation SyndromeUnited States