Abstract:
:Hereditary deficiencies of early complement components have usually been associated with the development of rheumatic diseases like systemic lupus erythematosus (SLE), while terminal component deficiency is well known to predispose to recurrent neisserial infection. In contrast, only recently have patients been reported with rheumatic disease and hereditary deficiency of a terminal component. The clinical syndrome in these patients has been characterised as 'SLE-like'. We describe here a third patient with complete C6 deficiency and a systemic rheumatic illness characterised by fever, anaemia, lymphadenopathy, hepatosplenomegaly, episcleritis, and asymmetric arthritis. After blood transfusion her serum became anticomplementary; IgG antibody to human C6 was found to be the cause of anticomplement activity. Persistent absence of C6 in this patient and production of anti-C6 antibody after antigenic challenge indicate hereditary C6 deficiency. This case supports an association between hereditary deficiency of a terminal complement protein and the development of systemic rheumatic disease.
journal_name
Ann Rheum Disjournal_title
Annals of the rheumatic diseasesauthors
Wisnieski JJ,Naff GB,Pensky J,Sorin SBdoi
10.1136/ard.44.10.716subject
Has Abstractpub_date
1985-10-01 00:00:00pages
716-22issue
10eissn
0003-4967issn
1468-2060journal_volume
44pub_type
杂志文章abstract::A case of pachydermoperiostosis characterised by the presence of finger clubbing, periostosis, sweating of hands and feet is described. Modifications of capillaroscopic pattern and of arteriovenous anastomoses are reported. The periungual border and finger tip tissue showed diffuse endothelial hyperplasia, hyalinosis,...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.48.3.240
更新日期:1989-03-01 00:00:00
abstract::The aetiology of Reiter's syndrome (RS) is unknown. In order to evaluate the role of immunological mechanisms in this disease we performed synovial biopsies on 12 patients with RS looking for deposition of immunoglobulins and complement components in synovial tissue. By immunofluorescent techniques 11 synovia were fou...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.40.3.281
更新日期:1981-06-01 00:00:00
abstract::The modulation of interleukin 1 (IL-1) effects on proteoglycan metabolism in intact murine patellar cartilage by transforming growth factor beta (TGF-beta) was investigated in vitro and in vivo. In vitro TGF-beta (400 pmol/l) had no effect on basal proteoglycan degradation. Proteoglycan degradation induced by IL-1, ho...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.52.3.185
更新日期:1993-03-01 00:00:00
abstract::The viscosity of plasma (PV) was investigated in 27 outpatients with classical or definite rheumatoid arthritis (RA) according to the American Rheumatism Association (ARA) criteria. The measurements showed a significantly raised PV in patients with RA (p less than 10(-5)) compared with a control group. There was a pos...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.46.8.601
更新日期:1987-08-01 00:00:00
abstract:OBJECTIVE:To estimate the cost of illness in an incidence based cohort of patients with juvenile idiopathic arthritis. METHODS:Direct costs (healthcare and non-healthcare costs) and indirect costs (productivity loss due to sick leave and work disability) were measured in 215 JIA patients, assessed on an average of 17 ...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.2003.008516
更新日期:2004-07-01 00:00:00
abstract:BACKGROUND:Reaching the therapeutic target of remission or low-disease activity has improved outcomes in patients with rheumatoid arthritis (RA) significantly. The treat-to-target recommendations, formulated in 2010, have provided a basis for implementation of a strategic approach towards this therapeutic goal in routi...
journal_title:Annals of the rheumatic diseases
pub_type: 共识发展会议,杂志文章,评审
doi:10.1136/annrheumdis-2015-207524
更新日期:2016-01-01 00:00:00
abstract::Arthralgias have previously been reported in association with acne fulminans, but the following case in the first report of osseous lesions associated with this disease. The destructive bony lesion was rapid in onset but self-limited. ...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.39.4.403
更新日期:1980-08-01 00:00:00
abstract:BACKGROUND:We report on a workshop on ethical and legal constraints on data sharing between countries in multinational epidemiologic research in Europe that was held in January 2007 in Potsdam, Germany. The participants were experienced epidemiologic and clinical researchers from eight European countries. The aim of th...
journal_title:Annals of the rheumatic diseases
pub_type: 共识发展会议,杂志文章
doi:10.1136/ard.2007.080598
更新日期:2008-07-01 00:00:00
abstract::Rheumatoid arthritis (RA) is diagnosed based on phenotypic characteristics. The 2010 ACR/EULAR criteria were derived with the aim of classifying RA earlier in the disease course than the 1987 ACR criteria. When reviewing thus far published validation studies, it is clear that the 2010 criteria can be fulfilled earlier...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/annrheumdis-2012-201426
更新日期:2012-10-01 00:00:00
abstract:OBJECTIVE:To assess the incremental diagnostic value of spine MRI evaluated separately from and combined with sacroiliac joint (SIJ) MRI in non-radiographic axial spondyloarthritis (nr-axSpA) compared with SIJ MRI alone. METHODS:The study sample comprised two independent cohorts A/B of 130 consecutive patients aged ≤5...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/annrheumdis-2013-203887
更新日期:2015-06-01 00:00:00
abstract:BACKGROUND:Several studies suggest a role of the growth hormone (GH)/insulin-like growth factor-1 (IGF-1) axis in the pathophysiology of primary osteoarthritis (OA). A common polymorphism of the GH receptor (exon 3 deletion, d3-GHR) is associated with increased GH/IGF-1 activity. OBJECTIVE:To study associations betwee...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/annrheumdis-2012-202713
更新日期:2014-02-01 00:00:00
abstract::Three patients are presented who sustained bilateral rupture of the Achilles tendon while on systemic steroid therapy for chest disease; a fourth patient with polymyalgia rheumatica on steroids is also presented. This is further evidence that tendon rupture can be a direct complication of steroid treatment. The Englis...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.42.6.652
更新日期:1983-12-01 00:00:00
abstract::Arthro-osteitis at the anterior chest wall was found in 12 (9.4%) out of 128 consecutive patients with pustulosis palmaris et plantaris. This finding indicates that the concomitance of arthro-osteitis with PPP is not incidental but is based on some common aetiological factor. We propose a term ;pustulotic arthro-ostei...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.40.6.554
更新日期:1981-12-01 00:00:00
abstract:BACKGROUND:Despite the importance of timely management of patients with inflammatory arthritis (IA), delays exist in its diagnosis and treatment. OBJECTIVE:To perform a systematic literature review to identify strategies addressing these delays to inform an American College of Rheumatology (ACR)/European League Agains...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章,评审
doi:10.1136/annrheumdis-2011-201063
更新日期:2013-01-01 00:00:00
abstract:PURPOSE:To assess risk of cartilage loss in the tibiofemoral joint in relation to baseline damage severity, and to analyse the association of nearby pathologic findings on the risk of subsequent cartilage loss. METHODS:The Multicenter Osteoarthritis Study is a longitudinal study of individuals with or at high risk for...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章,多中心研究
doi:10.1136/annrheumdis-2012-201810
更新日期:2013-06-01 00:00:00
abstract::The fluid and cellular phases of inflammatory resonse have been measured using a technique employing subcutaneous implantation of polyurethane foam cubes impregnated with heat-killed Mycobacterium tuberculosis. Phenylbutazone, azathioprine, aspirin, cyclophosphamide, and prednisolone suppressed fluid response, whereas...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.34.4.326
更新日期:1975-08-01 00:00:00
abstract::Peripheral and axial bone mass and fracture incidence were studied in a group of 104 postmenopausal patients with rheumatoid arthritis (RA). Patients were divided into non-corticosteroid and low dose corticosteroid treated groups after elimination of patients with concomitant disease or therapy which might affect bone...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.45.10.852
更新日期:1986-10-01 00:00:00
abstract::105 consecutive patients who presented to a rheumatologist because of joint disease and who also had evidence of deposition of calcium pyrophosphate dihydrate (CPD) were studied clinically and radiologically. There were 76 women (mean age 73) and 29 men (mean age 62). Of only 18 patients below the age of 60 at present...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.41.4.371
更新日期:1982-08-01 00:00:00
abstract:OBJECTIVES:To investigate the presence and functionality of oestrogen receptor alpha (ERalpha) in interleukin (IL)1beta-treated rabbit articular chondrocytes in culture, and to determine the mechanisms of 17beta oestradiol (E2) effects on IL1beta-induced inducible nitric oxide synthase (iNOS) expression. METHODS:The p...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.2006.059550
更新日期:2007-03-01 00:00:00
abstract::A case is reported of polytenosynovitis in a 31-year-old male during the course of a severe bacteraemic illness caused by Haemophilus influenzae type b. The clinical presentation was similar to tenosynovitis caused by bacterial or viral agents. As the management of the H. influenzae tenosynovitis would differ from tha...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.38.6.561
更新日期:1979-12-01 00:00:00
abstract::Antibody production in response to the primary immunogen bacteriophage phi X174 was investigated in 14 patients with previous yersinia arthritis (YA) and in 15 controls. HLA-B27 occurred in 10 patients with YA and in three controls. After primary and secondary immunisation the antibody responses were essentially simil...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.46.12.883
更新日期:1987-12-01 00:00:00
abstract:BACKGROUND:Vascular damage is a key pathological process in systemic sclerosis (SSc) and accounts for significant disease-related morbidity. To determine the clinical burden of severe digital vasculopathy (SDV), we have reviewed hospital-based treatment for this important complication of SSc in a large single centre co...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.2007.072686
更新日期:2008-01-01 00:00:00
abstract:OBJECTIVE:To study the long term effectiveness of intravenous immunoglobulin and plasmapheresis associated with prednisone and cyclophosphamide in Churg-Strauss syndrome. SUBJECTS:and methods: We studied 18 subjects with new onset Churg-Strauss syndrome. All received the "standard" treatment based on prednisone (1 mg/...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.2003.015453
更新日期:2004-12-01 00:00:00
abstract:OBJECTIVE:To score systemic activity at diagnosis and correlate baseline activity with survival in a large cohort of patients with primary Sjögren syndrome (SS). PATIENTS AND METHODS:We include 1045 consecutive patients who fulfilled the 2002 classification criteria for primary SS. The clinical and immunological chara...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/annrheumdis-2014-206418
更新日期:2016-02-01 00:00:00
abstract:OBJECTIVES:Millions of patients worldwide are treated with therapeutic monoclonal antibodies. These biological therapeutics can be immunogenic, resulting in anti-drug antibody formation which leads to loss of response. Fully human biological agents, such as the anti-tumour necrosis factor α (anti-TNFα) antibody adalimu...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/annrheumdis-2012-201445
更新日期:2013-01-01 00:00:00
abstract:BACKGROUND:The heteropolymer technology was developed to remove pathogens from the circulation. OBJECTIVES:To evaluate the safety and tolerability of a single administration and to establish proof of principle for ETI-104 in normal healthy volunteers (NHV) and patients with systemic lupus erythematosus (SLE) METHODS: ...
journal_title:Annals of the rheumatic diseases
pub_type: 临床试验,杂志文章
doi:10.1136/ard.2003.016691
更新日期:2004-09-01 00:00:00
abstract::It is postulated that the mobile inflamed joint may be subject to cyclical ischaemic reperfusion injury. Xanthine oxidoreductase is an enzyme thought to contribute to oxidative reperfusion injury, and the detection of this activity in human synovium is described. Three normal and five rheumatoid tissues were assayed w...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.46.11.843
更新日期:1987-11-01 00:00:00
abstract:OBJECTIVE:High serum interferon α (IFNα) activity is a heritable risk factor for systemic lupus erythematosus (SLE). Auto-antibodies found in SLE form immune complexes which can stimulate IFNα production by activating endosomal Toll-like receptors and interferon regulatory factors (IRFs), including IRF5. Genetic variat...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/annrheumdis-2011-200463
更新日期:2012-03-01 00:00:00
abstract:OBJECTIVES:To identify predictors of clinical remission as well as of no x-ray progression in a cohort of early rheumatoid arthritis (ERA) treated with a tight-control protocol. METHODS:A total of 121 consecutive patients with ERA were treated to reach European League Against Rheumatism (EULAR) and/or American College...
journal_title:Annals of the rheumatic diseases
pub_type: 临床试验,杂志文章
doi:10.1136/ard.2010.142729
更新日期:2011-07-01 00:00:00
abstract::Patients with Sjögren's syndrome alone (Ss), Sjögren's syndrome with rheumatoid arthritis (Ss-RA), and Sjögren's syndrome with Raynaud's phenomenon (Ss-RP) were typed for the HLA DR and MT antigens. Ss-RA patients had higher frequencies of HLA DR4 than did Ss patients. HLA-DR4 was also increased in frequency in patien...
journal_title:Annals of the rheumatic diseases
pub_type: 杂志文章
doi:10.1136/ard.42.5.533
更新日期:1983-10-01 00:00:00