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Journal ArticleDOI

Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus.

01 Sep 1997-Arthritis & Rheumatism (Arthritis Rheum)-Vol. 40, Iss: 9, pp 1725-1725

TL;DR: In 1992, Piette and colleagues suggested that the ACR revised criteria be reevaluated in light of the above discoveries, and the presence and clinical associations or antiphospholipid antibodies in patients with SLE was suggested.
Abstract: In 1982, the Diagnostic and Therapeutic Criteria Committee of the American College of Rheumatology (ACR)published revised criteria for the classification of systemiclupus erythematosus (SLE) (1). During the ensuing decade several investigators, including Drs. Graham Hughes and Donato Alarcon-Segovia, among others, have described the presence and clinical associations or antiphospholipid antibodies in patients with SLE, as well as the occurrence of theprimary antiphospholipid syndrome (2-5). In 1992, Piette and colleagues suggested that the ACR revised criteria be reevaluated in light of the above discoveries (6).
Topics: Lupus erythematosus (61%), Lupus vasculitis (54%)
Citations
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Journal ArticleDOI
Spiros Miyakis1, Michael D. Lockshin2, Tatsuya Atsumi3, D W Branch4  +11 moreInstitutions (11)
TL;DR: This document appraise the existing evidence on clinical and laboratory features of APS addressed during the forum and proposes amendments to the Sapporo criteria, including definitions on features ofAPS that were not included in the updated criteria.
Abstract: New clinical, laboratory and experimental insights, since the 1999 publication of the Sapporo preliminary classification criteria for antiphospholipid syndrome (APS), had been addressed at a workshop in Sydney, Australia, before the Eleventh International Congress on antiphospholipid antibodies In this document, we appraise the existing evidence on clinical and laboratory features of APS addressed during the forum Based on this, we propose amendments to the Sapporo criteria We also provide definitions on features of APS that were not included in the updated criteria

5,039 citations


Journal ArticleDOI
Jessica J Manson1, Anisur Rahman1Institutions (1)
TL;DR: Systemic lupus erythematosus is a relapsing and remitting disease, and treatment aims are threefold: managing acute periods of potentially life-threatening ill health, minimizing the risk of flares during periods of relative stability, and controlling the less life- threatening, but often incapacitating day to day symptoms.
Abstract: Systemic lupus erythematosus (SLE) is a clinically heterogeneous disease, which is autoimmune in origin and is characterized by the presence of autoantibodies directed against nuclear antigens. It is a multi-system disease, and patients can present in vastly different ways. Prevalence varies with ethnicity, but is estimated to be about 1 per 1000 overall with a female to male ratio of 10:1. The clinical heterogeneity of this disease mirrors its complex aetiopathogenesis, which highlights the importance of genetic factors and individual susceptibility to environmental factors. SLE can affect every organ in the body. The most common manifestations include rash, arthritis and fatigue. At the more severe end of the spectrum, SLE can cause nephritis, neurological problems, anaemia and thrombocytopaenia. Over 90% of patients with SLE have positive anti-nuclear antibodies (ANA). Significant titres are accepted to be of 1:80 or greater. SLE is a relapsing and remitting disease, and treatment aims are threefold: managing acute periods of potentially life-threatening ill health, minimizing the risk of flares during periods of relative stability, and controlling the less life-threatening, but often incapacitating day to day symptoms. Hydroxychloroquine and non-steroidal anti-inflammatory drugs are used for milder disease; corticosteroids and immunosuppressive therapies are generally reserved for major organ involvement; anti-CD20 monoclonal antibody is now used in patients with severe disease who has not responded to conventional treatments. Despite enormous improvements in prognosis since the introduction of corticosteroids and immunosuppressive drugs, SLE continues to have a significant impact on the mortality and morbidity of those affected.

3,850 citations


Cites background from "Updating the American College of Rh..."

  • ...They were further updated in 1997 [2] to reflect a greater underPublished: 27 March 2006...

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Journal ArticleDOI
TL;DR: The Systemic Lupus International Collaborating Clinics (SLICC) group revised and validated the American College of Rheumatology (ACR) systemic lupus erythematosus (SLE) classification criteria in order to improve clinical relevance, meet stringent methodology requirements, and incorporate new knowledge regarding the immunology of SLE.
Abstract: Objective The Systemic Lupus International Collaborating Clinics (SLICC) group revised and validated the American College of Rheumatology (ACR) systemic lupus erythematosus (SLE) classification criteria in order to improve clinical relevance, meet stringent methodology requirements, and incorporate new knowledge regarding the immunology of SLE. Methods The classification criteria were derived from a set of 702 expert-rated patient scenarios. Recursive partitioning was used to derive an initial rule that was simplified and refined based on SLICC physician consensus. The SLICC group validated the classification criteria in a new validation sample of 690 new expert-rated patient scenarios. Results Seventeen criteria were identified. In the derivation set, the SLICC classification criteria resulted in fewer misclassifications compared with the current ACR classification criteria (49 versus 70; P = 0.0082) and had greater sensitivity (94% versus 86%; P < 0.0001) and equal specificity (92% versus 93%; P = 0.39). In the validation set, the SLICC classification criteria resulted in fewer misclassifications compared with the current ACR classification criteria (62 versus 74; P = 0.24) and had greater sensitivity (97% versus 83%; P < 0.0001) but lower specificity (84% versus 96%; P < 0.0001). Conclusion The new SLICC classification criteria performed well in a large set of patient scenarios rated by experts. According to the SLICC rule for the classification of SLE, the patient must satisfy at least 4 criteria, including at least one clinical criterion and one immunologic criterion OR the patient must have biopsy-proven lupus nephritis in the presence of antinuclear antibodies or antidouble-stranded DNA antibodies. (Less)

2,869 citations


Journal ArticleDOI
TL;DR: The onset and progression of autoantibody development before the clinical diagnosis of systemic lupus erythematosus is investigated, with a progressive accumulation of specificAutoantibodies before the onset of SLE, while patients are still asymptomatic.
Abstract: Background Although much is known about the natural history of systemic lupus erythematosus (SLE), the development of SLE autoantibodies before the diagnosis of the disease has not been extensively explored. We investigated the onset and progression of autoantibody development before the clinical diagnosis. Methods The Department of Defense Serum Repository contains approximately 30 million specimens prospectively collected from more than 5 million U.S. Armed Forces personnel. We evaluated serum samples obtained from 130 persons before they received a diagnosis of SLE, along with samples from matched controls. Results In 115 of the 130 patients with SLE (88 percent), at least one SLE autoantibody tested was present before the diagnosis (up to 9.4 years earlier; mean, 3.3 years). Antinuclear antibodies were present in 78 percent (at a dilution of 1:120 or more), anti–double-stranded DNA antibodies in 55 percent, anti-Ro antibodies in 47 percent, anti-La antibodies in 34 percent, anti-Sm antibodies in 32 pe...

1,994 citations


Journal ArticleDOI
TL;DR: Global gene expression profiling of peripheral blood mononuclear cells is used to identify distinct patterns of gene expression that distinguish most SLE patients from healthy controls, and identify a subgroup of patients who may benefit from therapies targeting the IFN pathway.
Abstract: Systemic lupus erythematosus (SLE) is a complex, inflammatory autoimmune disease that affects multiple organ systems. We used global gene expression profiling of peripheral blood mononuclear cells to identify distinct patterns of gene expression that distinguish most SLE patients from healthy controls. Strikingly, about half of the patients studied showed dysregulated expression of genes in the IFN pathway. Furthermore, this IFN gene expression “signature” served as a marker for more severe disease involving the kidneys, hematopoetic cells, and/or the central nervous system. These results provide insights into the genetic pathways underlying SLE, and identify a subgroup of patients who may benefit from therapies targeting the IFN pathway.

1,924 citations


References
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Journal ArticleDOI
Eng M. Tan1, Alan S. Cohen, James F. Fries2, Alfonse T. Masi3  +5 moreInstitutions (7)
TL;DR: The 1971 preliminary criteria for the classification of systemic lupus erythematosus (SLE) were revised and updated to incorporate new immunologic knowledge and improve disease classification and showed gains in sensitivity and specificity.
Abstract: The 1971 preliminary criteria for the classification of systemic lupus erythematosus (SLE) were revised and updated to incorporate new immunologic knowledge and improve disease classification. The 1982 revised criteria include fluorescence antinuclear antibody and antibody to native DNA and Sm antigen. Some criteria involving the same organ systems were aggregated into single criteria. Raynaud's phenomenon and alopecia were not included in the 1982 revised criteria because of low sensitivity and specificity. The new criteria were 96% sensitive and 96% specific when tested with SLE and control patient data gathered from 18 participating clinics. When compared with the 1971 criteria, the 1982 revised criteria showed gains in sensitivity and specificity.

13,962 citations


Journal ArticleDOI
E. N. Harris1, Boey Ml1, C G Mackworth-Young1, A. E. Gharavi1  +3 moreInstitutions (1)
26 Nov 1983-The Lancet
TL;DR: A new solid-phase radioimmunoassay for the detection of anticardiolipin antibodies is 200-400 times more sensitive than the precipitation method used in the Venereal Disease Reference Laboratory test and appears to have predictive value for thrombosis in SLE and related disorders.
Abstract: A new solid-phase radioimmunoassay for the detection of anticardiolipin antibodies is 200-400 times more sensitive than the precipitation method used in the Venereal Disease Reference Laboratory test. 61% of serum samples from patients with systemic lupus erythematosus (SLE) had high levels of anticardiolipin antibodies of at least one immunoglobulin class. There were strong correlations between raised anticardiolipin levels and the lupus anticoagulant, venous and arterial thrombosis, and thrombocytopenia, but no correlation with anti-DNA antibody levels. Of the 15 patients with the highest anticardiolipin titres, 6 had a history of venous thrombosis, 5 cerebral thrombosis, 5 thrombocytopenia, and 2 each pulmonary hypertension and multiple abortions. This simple immunoassay appears to have predictive value for thrombosis in SLE and related disorders.

1,312 citations



Journal ArticleDOI
01 Nov 1989-Medicine
TL;DR: The group of patients presented appears to be closely related, but distinctly separate from SLE, with a history of deep vein thromboses and a family history of SLE or a familial clotting tendency in a minority.
Abstract: The antiphospholipid syndrome--the association of venous and/or arterial thromboses, often accompanied by thrombocytopenia in the presence of the antiphospholipid antibodies ("lupus anticoagulant" antibodies to cardiolipin)--is seen mainly in patients with systemic lupus erythematosus (SLE) and the closely related "lupus-like" disease, i.e., lupus patients not conforming to the 1982 revised American Rheumatism Association classification for SLE. It is also seen in a group of patients who do not manifest any of the major clinical or serologic features of SLE, the majority of whom do not appear to progress to classical lupus. A multicenter study of 70 of these patients is documented and their major clinical and serologic characteristics examined: They have been characterized as suffering from a "primary" antiphospholipid syndrome and present typically with a history of deep vein thromboses, often accompanied by pulmonary thromboembolism, which in a few is complicated by thromboembolic pulmonary hypertension, arterial occlusions (most commonly strokes), or fetal loss. The events are often recurrent and may be accompanied by hemocytopenias (thrombocytopenia and less frequently Coombs positivity and/or hemolytic anemia). They are often antinuclear antibody-negative and are always negative for antibodies to dsDNA and to ENA, typical serologic features of SLE. There may be a family history of SLE or a familial clotting tendency in a minority. The group of patients presented appears to be closely related, but distinctly separate from SLE.

961 citations


Journal Article
TL;DR: This study shows that properly performed ELISA or SRIA assays can be used to provide an accurate, reproducible, and quantitative measure of IgG and IgM aCL concentration in serum samples.
Abstract: Thirty laboratories from institutions in Britain, France, Italy, The Netherlands, New Zealand, Sweden and the USA participated in a workshop to evaluate the anti-cardiolipin (aCL) test. Participants were asked to measure IgG and IgM aCL in seven samples on each of three separate days. The seven samples were prepared so that IgG and IgM aCL concentrations were known before distribution. Twenty-three of 30 laboratories measuring IgG aCL had significant regression slopes (P less than 0.001) when optical absorbance readings or counts per minute were compared with IgG aCL concentration. Twenty-four of 28 laboratories measuring IgM aCL had significant regression slopes (P less than 0.001). Coefficient of determination (R2) ranged from 81.1% to 98.7% for laboratories with valid IgG aCL assays and from 48.0% to 96.7% for valid IgM aCL assays. Valid assays had in common the use of 10% fetal calf or 10% adult bovine serum in PBS. Assays that were not valid had in common the use of PBS, PBS-Tween, or 0.3% gelatin as diluents. All laboratories with valid assays defined samples with high and moderate aCL levels as positive but there was no consensus about low positive samples. This study shows that properly performed ELISA or SRIA assays can be used to provide an accurate, reproducible, and quantitative measure of IgG and IgM aCL concentration in serum samples.

703 citations


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