Abstract:
:Cogan's syndrome (CS) is a rare multisystemic disease characterized by vestibuloauditory dysfunction, inflammatory eye disease and vasculitis. Aortic aneurysms due to aortitis are under-recognized in CS, and are potentially fatal, with two of eight reported cases dying from aneurysm/arterial rupture. The presence of aneurysms was not recognized antemortem in both cases, highlighting the importance of early detection to prevent this potentially fatal outcome. We report a 17-year-old Chinese male with recurrent carotid and aortic aneurysms, bilateral sensorineural hearing loss, and recurrent scleritis who was initially thought to have Marfan syndrome. The diagnosis of CS was made 4.5 years after initial presentation, during which time recurrent aneurysms due to active aortitis (with aneurysmal rupture on one occasion) necessitated four surgical procedures. Treatment with methotrexate and corticosteroids resulted in no recurrence of aneurysms over 2 years of follow-up. This case illustrates (i) the challenges in diagnosing CS when various manifestations occur separately over a relatively long time period, (ii) that detection of aortic aneurysms in young patients, especially if recurrent, should prompt investigations for vasculitis/aortitis to prevent potentially fatal aneurysm rupture, and (iii) that methotrexate and high-dose corticosteroids may be effective in preventing development of further aneurysms in CS patients.
journal_name
Scand J Rheumatoljournal_title
Scandinavian journal of rheumatologyauthors
Low AH,Su JW,Sin KY,Fong KY,Thumboo Jdoi
10.1080/03009740600907923subject
Has Abstractpub_date
2007-01-01 00:00:00pages
71-3issue
1eissn
0300-9742issn
1502-7732pii
777055680journal_volume
36pub_type
杂志文章abstract:OBJECTIVES:Measurement of serum biomarkers at disease onset may improve prediction of disease course in patients with early rheumatoid arthritis (RA). We evaluated the multi-biomarker disease activity (MBDA) score and early changes in MBDA score for prediction of 28-joint Disease Activity Score based on C-reactive prot...
journal_title:Scandinavian journal of rheumatology
pub_type: 杂志文章,随机对照试验
doi:10.1080/03009742.2018.1464206
更新日期:2019-01-01 00:00:00
abstract:OBJECTIVES:Reactive arthritis (ReA) is a sterile joint inflammation triggered by a remote infection and associated with human leucocyte antigen (HLA)-B27. Its pathogenesis is unknown, but abnormal response to microbial structures or endogenous inflammatory mediators may be involved. We studied responses in leucocyte si...
journal_title:Scandinavian journal of rheumatology
pub_type: 杂志文章
doi:10.3109/03009742.2012.664649
更新日期:2012-08-01 00:00:00
abstract:OBJECTIVE:Interleukin-1 (IL-1) has been implicated in the pathogenesis of several rheumatic inflammatory diseases, including adult-onset Still's disease (AOSD) and systemic-onset juvenile idiopathic arthritis (SoJIA). Several clinical trials also suggest that anakinra, a human recombinant interleukin-1 receptor antagon...
journal_title:Scandinavian journal of rheumatology
pub_type: 杂志文章
doi:10.1080/03009740701340081
更新日期:2007-09-01 00:00:00
abstract::With improved disease survival, arterial thromboembolism has become an important cause of morbidity in patients with systemic lupus erythematosus (SLE). A number of conventional and non-conventional risk factors are associated with accelerated atherosclerosis. Regular surveillance and control of traditional risk facto...
journal_title:Scandinavian journal of rheumatology
pub_type: 杂志文章,评审
doi:10.1080/03009740600601526
更新日期:2006-03-01 00:00:00
abstract::In a series of 100 adult patients with definite rheumatoid arthritis of at most 3 years' duration and with no previous penicillamine, gold or systemic corticosteroid treatment, 50 patients were treated with D-penicillamine and 50 with gold for one yar. The dose of penicillamine was 600 mg daily. Sodium aurothiomalate ...
journal_title:Scandinavian journal of rheumatology
pub_type: 临床试验,杂志文章
doi:10.3109/03009747809095648
更新日期:1978-01-01 00:00:00
abstract::Familial Mediterranean fever (FMF) is characterized by recurrent attacks of fever and serositis. Typical attacks of FMF last 3 to 5 days. Fever and myalgia are not always improved by colchicine therapy and sometimes require steroid therapy. We present two cases of severe prolonged febrile myalgia where steroid therapy...
journal_title:Scandinavian journal of rheumatology
pub_type: 杂志文章
doi:10.1080/030097400447615
更新日期:2000-01-01 00:00:00
abstract::YKL-40, also called human cartilage glycoprotein-39, is a major secretory protein of human chondrocytes in cell culture. YKL-40 mRNA is expressed by cartilage from patients with rheumatoid arthritis, but is not detectable in normal human cartilage. The aim was to investigate the distribution of YKL-40 in osteoarthriti...
journal_title:Scandinavian journal of rheumatology
pub_type: 杂志文章
doi:10.1080/03009749950154257
更新日期:1999-01-01 00:00:00
abstract::Pain intensity, muscle strength, static muscle endurance, pressure pain threshold, and pain tolerance at tender points and control points were assessed in 31 patients with fibromyalgia (FM), before and after intravenous administration of morphine (9 patients), lidocaine (11 patients), and ketamine (11 patients). The t...
journal_title:Scandinavian journal of rheumatology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.3109/03009749509095181
更新日期:1995-01-01 00:00:00
abstract::The aim of the present study was to assess the T cell cytokines IFN-gamma, IL-2, IL-4 and IL-5 in labial salivary glands (LSG) in Sjögren's syndrome (SS) and healthy controls using RT-PCR and immunohistochemistry. IFN-gamma is always or almost always produced in SS and in healthy controls. IL-2 was also found in some ...
journal_title:Scandinavian journal of rheumatology
pub_type: 杂志文章
doi:10.1080/030097499442577
更新日期:1999-01-01 00:00:00
abstract::Antibodies against interleukin-1 alpha (aAb-IL-1 alpha) have previously been demonstrated in sera of healthy adults and in patients with inflammatory diseases. In the present investigation the occurrence of aAb-IL-1 alpha was examined by second antibody precipitation technique in sera from umbilical cords (n = 11), ch...
journal_title:Scandinavian journal of rheumatology
pub_type: 杂志文章
doi:10.3109/03009749609080008
更新日期:1996-01-01 00:00:00
abstract:OBJECTIVE:To determine if calprotectin is predictive for outcome in patients with rheumatoid arthritis (RA). METHODS:Fifty six RA in-patients with variable disease duration were prospectively followed for five years. Clinical and laboratory data were collected to assess disease activity. Health Assessment Questionnair...
journal_title:Scandinavian journal of rheumatology
pub_type: 杂志文章
doi:10.1080/030097402320817077
更新日期:2002-01-01 00:00:00
abstract:OBJECTIVES:Rheumatoid arthritis (RA) is a systemic, inflammatory disease. Renal involvement worsens the course of RA and increases mortality. It is suggested that chronic inflammatory processes may contribute to renal impairment. The aim of this study was to investigate the impact of chronic inflammation and RA activit...
journal_title:Scandinavian journal of rheumatology
pub_type: 杂志文章
doi:10.3109/03009742.2011.571219
更新日期:2011-01-01 00:00:00
abstract::Perinatal outcome in infants of women with rheumatic disease notified between 1967 95 to the Medical Birth Registry of Norway was compared to women without such disease. Logistic regression provided odds ratios for associations between rheumatic disease and perinatal outcome for 3 time periods: 1967-76, 1977-86, and 1...
journal_title:Scandinavian journal of rheumatology
pub_type: 杂志文章
doi:10.1080/03009749950155337
更新日期:1999-01-01 00:00:00
abstract::A vast accumulation of clinical experience and controlled studies justifies the use of gold compounds in selected cases of rheumatoid arthritis. The striking therapeutical efficacy observed in some patients has stimulated active research to clarify the pharmacokinetics and mechanisms of action of gold compounds. Based...
journal_title:Scandinavian journal of rheumatology
pub_type: 杂志文章
doi:10.3109/03009747709095455
更新日期:1977-01-01 00:00:00
abstract:OBJECTIVES:To study the link between metabolic syndrome (MetS), endothelial injury, and atherosclerosis in patients with systemic lupus erythematosus (SLE). METHODS:Consecutive SLE patients without a history of arterial thrombosis were screened for atherosclerosis at the carotid and coronary arteries by B-mode ultraso...
journal_title:Scandinavian journal of rheumatology
pub_type: 杂志文章
doi:10.3109/03009740903046668
更新日期:2010-01-01 00:00:00
abstract::The lupus anticoagulant (LAC) is associated with the occurrence of thromboembolic complications. Assuming that thrombosis may underlie manifestations of the central nervous system (CNS) in patients with systemic lupus erythematosus (SLE), we studied 20 patients with SLE and CNS manifestations for the presence of LAC. ...
journal_title:Scandinavian journal of rheumatology
pub_type: 杂志文章
doi:10.3109/03009748609102086
更新日期:1986-01-01 00:00:00
abstract:OBJECTIVE:To investigate the feasibility of magnetic resonance imaging (MRI)-guided high power focused ultrasound (FUS) to perform synovectomy noninvasively. METHODS:Five New Zealand white male rabbit knees with experimentally induced arthritis underwent MRI-guided thermal surgery by high power (60 W/10 s) sonication....
journal_title:Scandinavian journal of rheumatology
pub_type: 杂志文章
doi:10.1080/03009749950155607
更新日期:1999-01-01 00:00:00
abstract::Antibodies to soluble ribonucleoprotein (sRNP) are by definition a prerequisite for the diagnosis mixed connective tissue disease (MCTD). They are also found in 30--40% of other rheumatic conditions with a high titred speckled IFL reaction. The same is true for the most characteristic clinical features of MCTD, viz. R...
journal_title:Scandinavian journal of rheumatology
pub_type: 杂志文章
doi:10.3109/03009747809095661
更新日期:1978-01-01 00:00:00
abstract::Objective: To evaluate clinical associations of anti-hydroxy-3-methylglutaryl-coenzyme A reductase (anti-HMGCR) antibody (Ab) and statin exposure in necrotizing myopathy (NM) patients. Methods: NM without a known myositis-specific autoantibody (MSA) was ascertained from a large single-centre myositis database between ...
journal_title:Scandinavian journal of rheumatology
pub_type: 杂志文章
doi:10.1080/03009742.2019.1672782
更新日期:2020-09-01 00:00:00
abstract::IgM and IgG rheumatoid factors (RFs) were purified by affinity chromatography and gel filtration. The preparations thus obtained were used as standards in a radio-immunoassay for RF detection. In this assay, RFs were reacted with immunoprecipitates, and the RFs were detected with radiolabelled (F(ab')2 fragments speci...
journal_title:Scandinavian journal of rheumatology
pub_type: 杂志文章
doi:10.3109/03009748409100395
更新日期:1984-01-01 00:00:00
abstract::To obtain data on the incidence of Reiter's syndrome (RS) as well as on possible simultaneously appearing signs and symptoms of RS in sexual couples we have made interview reports of randomly selected 530 persons: 219 males and 311 females, aged 15 to 54. None had RS. Six males (2.7%) and seven females (2.3%) had a hi...
journal_title:Scandinavian journal of rheumatology
pub_type: 杂志文章
doi:10.3109/03009748209098181
更新日期:1982-01-01 00:00:00
abstract::We have recently shown that synoviocytes and extracellular matrices exhibit distinct patterns of carbohydrate expression. Their biological relevance is however not known. The purpose of the present study was to find out whether human synovial tissue would also show a specific receptor pattern for complex sugar molecul...
journal_title:Scandinavian journal of rheumatology
pub_type: 杂志文章
doi:10.3109/03009749109096796
更新日期:1991-01-01 00:00:00
abstract::Primary cultures of neonatal dermal fibroblasts from two strains of mice (A/J and C57BL6/J) were utilized as an in vitro system to investigate the effects of glucocorticoids on cell growth and collagen synthesis. Protein and DNA synthesis were lower in untreated (control) A/J fibroblasts than in C57BL6/J fibroblasts. ...
journal_title:Scandinavian journal of rheumatology
pub_type: 杂志文章
doi:10.3109/03009748309099741
更新日期:1983-01-01 00:00:00
abstract::Presenting symptoms in polymyalgia rheumatica (PMR) may be atypical. We report herein two old females who developed a bilateral carpal tunnel syndrome several months before the typical symptoms of PMR appeared. In both patients the diagnosis of PMR was overlooked and a surgical release of the median nerve was performe...
journal_title:Scandinavian journal of rheumatology
pub_type: 杂志文章
doi:10.3109/03009749709065686
更新日期:1997-01-01 00:00:00
abstract::Objective: Psoriatic arthritis (PsA) is a heterogeneous inflammatory disease associated with psoriasis. Underlying genetic factors are considered important for disease expression and prognosis of PsA. Interleukin-1β-regulating protein complexes called inflammasomes are associated with several inflammatory diseases, e....
journal_title:Scandinavian journal of rheumatology
pub_type: 杂志文章
doi:10.1080/03009742.2020.1834611
更新日期:2020-12-10 00:00:00
abstract::The distribution of messenger RNA coding for substance P receptor (NK1) in rheumatoid synovia and the relationship between signal intensity of NK1 mRNA and clinical parameters of disease activity were investigated. The synovia from 10 patients with rheumatoid arthritis (RA) and 7 patients with osteoarthritis was inves...
journal_title:Scandinavian journal of rheumatology
pub_type: 杂志文章
doi:10.1080/030097498441010
更新日期:1998-01-01 00:00:00
abstract::Fifty-one patients with different connective tissue diseases in either active or clinically inactive phase were studied to detect the incidence of chronic or subclinical consumption coagulopathy in these forms. Every patient received intravenously 20 mu Ci of [125I] fibrinogen and the clearance of the labeled fibrinog...
journal_title:Scandinavian journal of rheumatology
pub_type: 杂志文章
doi:10.3109/03009748009112361
更新日期:1980-01-01 00:00:00
abstract::The degrees of anaemia and microcytosis in 100 patients with juvenile chronic arthritis (JCA) were analysed according to the pattern of disease and its activity. Microcytosis was common (40% of patients had a mean corpuscular volume (MCV) of less than 75 fl), sometimes severe (MCV less than 65 fl in 14 patients), and ...
journal_title:Scandinavian journal of rheumatology
pub_type: 杂志文章
doi:
更新日期:1987-01-01 00:00:00
abstract::219 metatarsal (MTP) and 69 metacarpal (MCP) capitulae obtained during surgery from patients with definite rheumatoid arthritis (RA) were histologically evaluated. This evaluation, focussing on primary pathways of joint destruction by tumor-like proliferated synovial cell masses revealed 3 pathways of aggression: Path...
journal_title:Scandinavian journal of rheumatology
pub_type: 杂志文章
doi:10.3109/03009749209095055
更新日期:1992-01-01 00:00:00
abstract::Objectives: IgG4-related disease (IgG4-RD) may present as 'idiopathic' retroperitoneal fibrosis (IRPF). We aimed to determine the occurrence of IgG4-retroperitoneal fibrosis (IgG4-RPF) in a nationwide study on patients with newly diagnosed IRPF, and to compare histopathological, imaging, and clinical features in the I...
journal_title:Scandinavian journal of rheumatology
pub_type: 杂志文章
doi:10.1080/03009742.2018.1551963
更新日期:2019-07-01 00:00:00