Abstract：
OBJECTIVES:The Schnitzler syndrome is a rare inflammatory disorder, with a chronic urticaria-like rash and an IgM (rarely IgG) monoclonal gammopathy as cardinal features. Interleukin-1 β is regarded as the key mediator and the interleukin-1 receptor antagonist anakinra has been proposed as first-line treatment. This case series of eleven patients is intended to enhance disease awareness and to compare our centre's experience with that of literature. METHODS:We describe the clinical features and disease course of 11 patients with a definite Schnitzler syndrome, according to the Strasbourg diagnostic criteria, encountered in the University Hospital, Leuven, Belgium, between 1995 and 2015. RESULTS:Eleven patients, with a median age of 55 years, were diagnosed with Schnitzler syndrome. All but one were diagnosed during the last decade. Of 6 patients treated with anakinra, 2 had a suboptimal response and 2 had poor tolerance (injection site reaction and neutropenia, respectively). Two of the 11 patients died as a consequence of the disease, culminating in Waldenström's macroglobulinaemia and AA amyloidosis, respectively. CONCLUSIONS:The Schnitzler syndrome is rare, but probably underdiagnosed. In a patient with a chronic urticaria-like dermatosis, minor itch, intermittent fever and bone or joint aches, protein electrophoresis and immunofixation should be ordered. Especially, a finding of a monoclonal IgM kappa fits the diagnosis of Schnitzler syndrome. Anakinra may provide symptomatic relief, although the response is not always spectacular. The outcome is not always benign as fatal complications may occur.
journal_name
Clin Exp Rheumatoljournal_title
Clinical and experimental rheumatologyauthors
Vanderschueren S,van der Veen Asubject
Has Abstractpub_date
2017-01-01 00:00:00pages
69-73issue
1eissn
0392-856Xissn
1593-098Xpii
10598journal_volume
35pub_type
杂志文章abstract：:A meta-analysis showed that methodological differences in prevalence studies such as a sample survey design or census design may be responsible for some of the variance in BS prevalence reported across countries, in addition to a true geographic variation. Efforts towards developing a data driven core set of outcome m...
journal_title：Clinical and experimental rheumatology
pub_type： 杂志文章,评审
doi：
更新日期：2017-11-01 00:00:00
abstract：OBJECTIVE:To document the psychological side effects of methotrexate (MTX) treatment in children with juvenile idiopathic arthritis (JIA) and to explore the usefulness of psychological therapy to ameliorate these side effects. METHODS:The patients included in this study consisted of 29 patients with JIA using MTX. Of ...
journal_title：Clinical and experimental rheumatology
pub_type： 临床试验,杂志文章
doi：
更新日期：2007-05-01 00:00:00
abstract：:Interstitial granulomatous dermatitis with arthritis (IGDA) is a rare idiopathic skin disorder with variable cutaneous expression, typically associated with a seronegative arthritis. Histopathology of this disorder reveals a granulomatous infiltrate with foci of collagen degeneration in the deep reticular dermis. We p...
journal_title：Clinical and experimental rheumatology
pub_type： 杂志文章
doi：
更新日期：2014-03-01 00:00:00
abstract：:Skin inflammation is a physiological reaction to tissue injury, pathogen invasion and irritants. During this process, innate and/or adaptive immune cells are activated and recruited to the site of inflammation to either promote or suppress inflammation. The sequential recruitment and activation of immune cells is modu...
journal_title：Clinical and experimental rheumatology
pub_type： 杂志文章,评审
doi：
更新日期：2015-07-01 00:00:00
abstract：OBJECTIVES:To develop and test a new multidimensional questionnaire for assessment of children with auto-inflammatory disease (AID) such as FMF, PFAPA, HIDS, TRAPS in standard clinical care. METHODS:The juvenile auto-inflammatory disease multidimensional assessment report (JAIMAR) includes 16 parent or patient-centere...
journal_title：Clinical and experimental rheumatology
pub_type： 杂志文章
doi：
更新日期：2016-09-01 00:00:00
abstract：OBJECTIVES:There is no information on how patella cartilage relates to the radiological grade of individual features of patellofemoral osteoarthritis (osteophytes or joint space narrowing) which have been used in most epidemiological and clinical studies. In this study we compared patella cartilage volume as measured b...
journal_title：Clinical and experimental rheumatology
pub_type： 杂志文章
doi：
更新日期：2003-05-01 00:00:00
abstract：:Primary fibromyalgia syndrome (PFS) is a common form of nonarticular rheumatism with chronic and generalized musculoskeletal aching and stiffness, accompanied by tender points at characteristic sites in the absence of an underlying condition. No satisfactory criteria for its diagnosis, based on appropriate controlled ...
journal_title：Clinical and experimental rheumatology
pub_type： 杂志文章
doi：
更新日期：1989-01-01 00:00:00
abstract：OBJECTIVE:To assess the effects of initial treatment with intravenous methylprednisolone (IVMP) on the clinical course and laboratory measurements in patients with juvenile dermatomyositis (JDM), and to report the long-term prognosis of these patients. METHODS:This was a retrospective study of 24 children with JDM, tr...
journal_title：Clinical and experimental rheumatology
pub_type： 杂志文章
doi：
更新日期：1999-09-01 00:00:00
abstract：: ...
journal_title：Clinical and experimental rheumatology
pub_type： 信件
doi：
更新日期：2017-05-01 00:00:00
abstract：OBJECTIVES:The aim of this study is to compare clinical features and treatment of young onset rheumatoid arthritis with late-onset rheumatoid arthritis. METHODS:Nine thousand five hundred forty-one patients with rheumatoid arthritis (RA) enrolled in the national database of the German Collaborative Arthritis Centres i...
journal_title：Clinical and experimental rheumatology
pub_type： 杂志文章
doi：
更新日期：2013-03-01 00:00:00
abstract：PURPOSE AND METHODS:In order to evaluate the prevalence of positive hepatitis C virus (HCV) serology and cryoglobulinemia in human immunodeficiency virus (HIV)-infected patients, the prevalence and the clinical significance of cryoglobulinemia were prospectively studied in a cohort of 86 HIV-infected subjects seen as o...
journal_title：Clinical and experimental rheumatology
pub_type： 杂志文章
doi：
更新日期：1997-09-01 00:00:00
abstract：:Many viruses can be responsible for systemic vasculitis, the most frequent being hepatitis B virus-related polyarteritis nodosa (HBV-PAN), even though its incidence has decreased over the past few decades. Mixed cryoglobulinemia has been shown to be associated with hepatitis C virus (HCV) infection in more than 80% of...
journal_title：Clinical and experimental rheumatology
pub_type： 杂志文章,评审
doi：
更新日期：2006-03-01 00:00:00
abstract：OBJECTIVES:We aimed to assess changes in myositis core set measures and ancillary clinical and laboratory data from the National Institutes of Health's subset of patients enrolled in the Rituximab in Myositis trial. METHODS:Eighteen patients (5 dermatomyositis, 8 polymyositis, 5 juvenile dermatomyositis) completed mor...
journal_title：Clinical and experimental rheumatology
pub_type： 杂志文章,多中心研究,随机对照试验
doi：
更新日期：2014-09-01 00:00:00
abstract：OBJECTIVES:To offer a second opinion on the recently published retrospective diagnosis of Cardinal Carlo de' Medici (1596-1666), a prominent member of the grand ducal family then ruling Tuscany. METHODS:Retrospective diagnosis of historical figures is difficult and at times controversial, even with modern technology. ...
journal_title：Clinical and experimental rheumatology
pub_type： 传,历史文章,杂志文章
doi：
更新日期：2012-01-01 00:00:00
abstract：OBJECTIVES:The anti-inflammatory effect of adenosine is partially mediated via the A3 adenosine receptor (A3AR), a Gi protein associated cell surface receptor. The highly selective A3AR agonist, IB-MECA was earlier shown to prevent the clinical and pathological manifestations of arthritis in experimental animal models ...
journal_title：Clinical and experimental rheumatology
pub_type： 杂志文章
doi：
更新日期：2006-07-01 00:00:00
abstract：:Skin biopsies from 4 systemic sclerosis (SSc) patients, 6 SSc patients treated with D-penicillamine (from 8 to 60 months) and 4 normal subjects were analyzed by light and electron microscopy. By light microscopy, collagen bundles of SSc dermis were thicker and more compact than in age-matched controls; D-penicillamine...
journal_title：Clinical and experimental rheumatology
pub_type： 杂志文章
doi：
更新日期：1989-07-01 00:00:00
abstract：OBJECTIVE:To investigate the composition and expression of sialic acid in the labial salivary glands (LSG) in Sjögren's syndrome (SS). METHODS:LSG of 19 patients with primary SS (n = 11) or secondary SS (n = 8) were studied. Specimens from 7 healthy women served as controls. Computer-assisted microscopy was employed t...
journal_title：Clinical and experimental rheumatology
pub_type： 杂志文章
doi：
更新日期：1999-11-01 00:00:00
abstract：:This paper in short reviews general indications, doses and routes of administration of glucocorticoid therapy in rheumatology. It presents greater detail concerning intralesional and intra-articular glucocorticoid injections. ...
journal_title：Clinical and experimental rheumatology
pub_type： 杂志文章,评审
doi：
更新日期：2011-09-01 00:00:00
abstract：:Lymphocyte subpopulations and functions were examined in the salivary (parotid) gland lymphocytes (SGL) obtained as a cell suspension from a patient with Sjögren's syndrome associated with rheumatoid arthritis, in comparison with peripheral blood lymphocytes (PBL). Serial studies on the lymphocyte subsets in PBL using...
journal_title：Clinical and experimental rheumatology
pub_type： 杂志文章
doi：
更新日期：1984-01-01 00:00:00
abstract：:In patients with SLE in remission pregnancy does not confer a special risk to either the mother or foetus, and immunoregulatory drugs should not be withdrawn prior to a pregnancy where these are required for disease control. Likewise therapy to treat disease flares during pregnancy should not be withheld as the foetus...
journal_title：Clinical and experimental rheumatology
pub_type： 杂志文章,评审
doi：
更新日期：1988-04-01 00:00:00
abstract：OBJECTIVES:To validate the diagnostic benefit of dual-energy computed tomography (DECT) and synovial fluid aspiration in suspected gout. METHODS:A total of 43 patients with suspected gout underwent aspiration and DECT (320-row CT; Canon Medical Systems, Japan). The patients were assessed (gout vs. non-gout) based on t...
journal_title：Clinical and experimental rheumatology
pub_type： 杂志文章
doi：
更新日期：2018-11-01 00:00:00
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journal_title：Clinical and experimental rheumatology
pub_type： 杂志文章
doi：
更新日期：1996-11-01 00:00:00
abstract：OBJECTIVE:To describe or determine reference values for the intracapsular cavity and its joint recess of the hip, knee and joints of the hand and for the tendon sheaths of fingers, examined with B-mode gray-scale ultrasonography (US) and power Doppler (PD) in healthy children. METHODS:Sixty healthy children (31 boys a...
journal_title：Clinical and experimental rheumatology
pub_type： 杂志文章
doi：
更新日期：2007-11-01 00:00:00
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journal_title：Clinical and experimental rheumatology
pub_type： 杂志文章
doi：
更新日期：2007-11-01 00:00:00
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journal_title：Clinical and experimental rheumatology
pub_type： 杂志文章,随机对照试验
doi：
更新日期：2012-03-01 00:00:00
abstract：:In recent years, there has been a dramatic rise in the number of trials using monoclonal antibodies, especially those directed against the CD4 molecule, in the treatment of RA. Thus far, the numbers of patients treated in the individual studies have generally been small, and the study designs sometimes not comparable....
journal_title：Clinical and experimental rheumatology
pub_type： 杂志文章,评审
doi：
更新日期：1993-03-01 00:00:00
abstract：:There is a clear need to assess patients presenting with a new onset of inflammatory peripheral arthritis and/or back pain early. Indeed, the clinical presentation of rheumatoid arthritis (RA) is not always characteristic but its early diagnosis is crucial to prevent irreversible structural damage. Likewise low back p...
journal_title：Clinical and experimental rheumatology
pub_type： 杂志文章
doi：
更新日期：2009-07-01 00:00:00
abstract：OBJECTIVES:Autonomic dysfunction (AD) has been described in various chronic inflammatory diseases. Studies of AD in patients with familial Mediterranean fever (FMF) are inconclusive. We aimed to assess AD in a cohort of FMF patients. METHODS:Signs and symptoms of AD were investigated in patients with FMF and compared ...
journal_title：Clinical and experimental rheumatology
pub_type： 杂志文章
doi：
更新日期：2019-11-01 00:00:00
abstract：OBJECTIVES:This study was intended to verify the likelihood of homing of intra-articularily injected mesenchymal stem cells (MSCs) and its involvement in the healing process of experimentally induced, acute and chronic, partial chondral defects in dogs. METHODS:Partial thickness chondral defects were created on the la...
journal_title：Clinical and experimental rheumatology
pub_type： 杂志文章
doi：
更新日期：2011-03-01 00:00:00
abstract：OBJECTIVES:In 1966, Goldbloom et al. described two children who developed a peculiar clinical picture characterized by intermittent daily bone pain in the lower limbs, fever spikes, increased acute phase reactants and dysproteinaemia. The syndrome occurred two weeks after a group A β-haemolytic streptococcus infection....
journal_title：Clinical and experimental rheumatology
pub_type： 杂志文章,评审
doi：
更新日期：2017-05-01 00:00:00
