Rheumatic involvement and bone scan features in Schnitzler syndrome: initial and follow-up data from a single-center cohort of 25 patients
| العنوان: | Rheumatic involvement and bone scan features in Schnitzler syndrome: initial and follow-up data from a single-center cohort of 25 patients |
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| المؤلفون: | Claire Bernier, Hélène Aubert, Jean-Marie Berthelot, Antoine Néel, Christian Agard, Sébastien Barbarot, Benoit Le Goff, Mohamed Hamidou, Agathe Masseau, Françoise Kraeber-Bodéré, Christelle Darrieutort-Laffite, Catherine Ansquer |
| المصدر: | Arthritis Research & Therapy, Vol 22, Iss 1, Pp 1-10 (2020) Arthritis Research & Therapy |
| بيانات النشر: | BMC, 2020. |
| سنة النشر: | 2020 |
| مصطلحات موضوعية: | Male, medicine.medical_specialty, lcsh:Diseases of the musculoskeletal system, Interleukin 1 receptor antagonist, Single Center, Cohort Studies, 030207 dermatology & venereal diseases, 03 medical and health sciences, 0302 clinical medicine, Schnitzler syndrome, Musculoskeletal Pain, Internal medicine, medicine, Humans, Bone pain, Retrospective Studies, business.industry, Waldenstrom macroglobulinemia, Retrospective cohort study, Bone scan, medicine.disease, Rash, Rheumatology, Bone lesions, Cohort, Female, medicine.symptom, lcsh:RC925-935, business, 030215 immunology, Research Article, Follow-Up Studies |
| الوصف: | Objective To report on the characteristics and long-term course of rheumatic manifestations in Schnitzler syndrome (SchS). Methods A retrospective cohort study of patients with SchS followed between 2000 and 2020. Inclusion criteria included a diagnosis of SchS (Strasbourg criteria). All available bone scans were reviewed and scored according to the intensity and number of pathological sites. The scintigraphic score was compared with the clinical activity score, CRP level, and treatments. Results Twenty-five patients were included. Median age at diagnosis was 68 years. Eighty patients (72%) had SchS-related rheumatic pain. Most patients had a long-standing isolated rash before constitutional and/or rheumatic symptoms appeared. The monoclonal component level was usually very low (IgMκ in 22/25). Rheumatic pain predominated around the knees. Bone scans revealed abnormal tracer uptake in 15/18 (85%). The scintigraphic score correlated with clinical activity (r = 0.4, p r = 0.47, p p Conclusions Rheumatic manifestations are very prevalent in SchS. However, bone pain can be misleading and contribute to misdiagnosis. Bone scan abnormalities are very prevalent and correlate with disease activity and treatments. IL1-Ra has a dramatic and durable efficacy but may not be required in every patient early on. |
| اللغة: | English |
| تدمد: | 1478-6362 |
| الوصول الحر: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::52bd4540be4dcd843b01445f9f1276c5Test http://link.springer.com/article/10.1186/s13075-020-02318-5Test |
| حقوق: | OPEN |
| رقم الانضمام: | edsair.doi.dedup.....52bd4540be4dcd843b01445f9f1276c5 |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 14786362 |
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