American Rheumatism Association Criteria Research Articles

A new diagnostic sign for rheumatoid arthritis: Avicenna sign

AbstractAim: The status of a new sign for diagnosing rheumatoid arthritis is discussed for the first time in this study. It is defined as marked tenderness on the anterior surface of leg (shin) on pressure of about 4 kg by thumb. The sign has been denoted as ‘Avicenna sign’.Methods: All cases were selected by convenient sampling from patients who were referred to two rheumatology clinics in Tabriz, Iran. Sample size was calculated as 618 using Cochran's sampling equation.Diagnosis of rheumatoid arthritis was made according to American Rheumatism Association criteria. Two physicians were asked to examine the sign on all of the selected patients separately. One of them (a general physician) was not informed about methodology and objectives of the study, whereas a rheumatologist was well informed and he collected data using a written questionnaire.Test–retest method and discriminant analysis were used to test reliability and validity of the sign for diagnosing rheumatoid arthritis. The effects of intervening variables including osteoporosis and consumption of some medications were controlled employing Chi‐square test.Results: When the general physician examined patients with Avicenna sign to diagnose rheumatoid arthritis, sensitivity and specificity of the sign were reported as 77.1% and 98.4%, respectively. The corresponding values were 81.3% and 98.4%, respectively, when the rheumatologist examined the patients.Conclusions: This study suggested considerable diagnostic value of the sign for rheumatoid arthritis. Consumption of acetaminophen or NSAIDs had no effect on the status of Avicenna sign while taking gluccocorticoids made it unreliable for diagnostic purposes.

Correlation between serum E-selectin levels and panoramic nailfold capillaroscopy in systemic sclerosis.

E-selectin is expressed by the activated endothelium and its plasma levels are increased in patients with systemic sclerosis. Eighteen patients fulfilling the American Rheumatism Association criteria for systemic sclerosis, 15 females and 3 males, 42-70 years old, 9 with diffuse and 9 with limited forms, were sequentially recruited for this study. Serum E-selectin levels were determined by commercially available ELISA and their association with nailfold capillaroscopic abnormalities was investigated. Nailfold capillaries were analyzed by 16X magnification wide-field capillaroscopy. Two parameters on capillaroscopy were used to correlate to serum E-selectin: deletion and ectasia. Data were analyzed statistically by the Student t-test and Spearman correlation. Two-tailed P values below 0.05 were considered significant. E-selectin range was 38 to 200 ng/ml (80 +/- 39.94). There was a correlation between serum E-selectin levels and the deletion capillaroscopic score (r = 0.50, P < 0.035). This correlation was even stronger within the first 48 months of diagnosis (r = 0.63, P < 0.048). On the other hand, no association was observed between selectin and ectasia. Patients with diffuse disease presented higher serum E-selectin levels than patients with limited disease, although the difference was not statistically significant (96.44 +/- 48.04 vs 63.56 +/- 21.77 ng/dl; P = 0.08). The present study is the first showing a correlation between soluble serum E-selectin levels and alterations in capillaroscopy. The stronger correlation of deletion score in capillaroscopy in early disease suggests that serum E-selectin levels might be a useful biochemical marker of disease activity in systemic sclerosis.

Lp(a) lipoprotein and lipids in patients with rheumatoid arthritis: serum levels and relationship to inflammation

Changes in lipid profiles, Lp(a) lipoprotein, and acute phase reactants are associated with early atherosclerosis in rheumatoid arthritis (RA). The associations of Lp(a) levels with atherosclerotic disorders, diabetes, RA, and renal diseases suggest that Lp(a) might be involved in autoimmune reactions. Eighty-seven women with RA diagnosed according to American Rheumatism Association criteria (mean age 45.4+/-9.4 years) were recruited and 50 healthy women (mean age 44+/-10.7 years) included as a control group. Serum Lp(a), total cholesterol (TC), triglyceride (TG), LDL cholesterol (LDL-C), HDL cholesterol (HDL-C), and C-reactive protein levels were analyzed. In the RA and C groups, serum Lp(a) levels were 39.2+/-20.6 mg/dl and 14.8+/-9.7 mg/dl, respectively (P<0.001). The TC levels were 188.4+/-41.8 mg/dl and 185.3+/-19.3 mg/dl (P>0.05), TG levels were 124.5+/-50.1 mg/dl and 94.6+/-24.9 mg/dl (P<0.01), HDL-C levels were 40.0+/-7.4 mg/dl and 52.8+/-4.8 mg/dl (P<0.01), and LDL-C levels were 123.4+/-24.6 mg/dl and 113.3+/-21.1 mg/dl (P>0.05). While serum CRP levels showed a positive correlation with Lp(a), they correlated negatively with HDL-C levels (r=0.83 and P<0.0001, r=-0.49 and P<0.0001, respectively). It was meaningful that Lp(a) correlated negatively with serum HDL-C level (r=-0.36, P<0.001). It is suggested that higher serum Lp(a), lower HDL-C, higher TG level, and a high ratio of TC/HDL-C might show high risk of atherosclerosis. Inflammation in RA may cause changes in HDL-C and Lp(a) metabolisms.

RHEUMATOID ARTHRITIS AMONG WOMEN IN THE AGRICULTURAL HEALTH STUDY: RISK ASSOCIATED WITH FARMING HISTORY AND OCCUPATIONAL EXPOSURES

ISEE-458 Abstract: Several epidemiologic studies have reported increased risk of rheumatoid arthritis (RA) among farmers. However, the exposures accounting for any increase (e.g., pesticides, silica, solvents) have not been extensively investigated. We studied farming activities and exposures as risk factors for RA among women in the Agricultural Health Study (AHS), a cohort of licensed pesticide applicators and their spouses. We attempted to validate diagnoses for 430 women who self-reported that they had RA in the 5-year followup interview. RA diagnosis was confirmed for 140 women (33%) through a combination of self-confirmation upon callback and contact with physicians. Among the remaining women, 29% did not confirm the diagnosis themselves when recontacted, 28% were not confirmed by the physician, and 10% were never reached. Among the 140 ′confirmed’ cases, RA diagnosis was considered confirmed in several ways. A woman was classified as having ’physician-confirmed RA’ if any one of her physicians indicated that she had a diagnosis of RA, or indicated that she satisfied 4 of 7 American Rheumatism Association (ARA) criteria for RA diagnosis. In addition, ′self-confirmed RA’ was classified as self-confirmation of RA diagnosis upon re-interview in the validation effort and return of a signed form consenting physician contact, regardless of whether any physician responded. Five controls for each case were selected from all women in the AHS cohort, matched on birthdate (n=700). Longterm smoking was associated with 80% increased risk for more than 20 pack-years (OR=1.8, 95% CI: 1.0-3.4), consistent with previous reports. The risk of RA was not associated with having ever personally mixed or applied pesticides (of any type), nor did it vary by the number of days or years a woman participated in this activity. However, certain insecticides were associated with increased risk, including organochlorines (OR=1.6; 95% CI: 0.9-2.8) such as DDT (OR=1.5, 95% CI: 0.7-2.9) and lindane (OR=3.7, 95% CI: 1.3-10.9), and organophosphates (OR=1.3, 95% CI: 0.9-1.9) such as malathion (OR=1.4, 95% CI: 0.9-2.1) and DDVP (OR=1.9, 95% CI: 0.8-4.6). Welding on the farm or in another job was associated with increased risk of RA (OR=2.7, 95% CI: 1.1-6.5). These reported associations were generally present in each state, and upon restriction to cases whose diagnosis date was confirmed to follow exposure. Other farming exposures, including herbicides and solvents, were not associated with RA, nor were residential pesticide applications. Because of the severe disability associated with this relatively common disease, further investigation into causes is warranted.

Antinuclear antibody-negative lupus as a distinct diagnostic entity--does it no longer exist?

Systemic lupus erythematosus (SLE) is a multi-system autoimmune disorder with protean clinical presentations. Historically, Biett reported the first case of lupus erythematosus in 1833, and Kaposi later described the systemic form in 1872,1 but the varied presentation of this illness has made it a challenging diagnosis for clinicians. The difficulties in the diagnosis of SLE were considerably eased by the demonstration of antinuclear antibodies (ANA) as a marker of lupus, initially in the form of LE cells2 and later by indirect immunofluorescence.3 Since then, ANA positivity has become an important diagnostic criterion for the diagnosis of SLE and officially recognised as such by its inclusion in the American College of Rheumatology's criteria for the classification of this disorder.4,,5 The concept of ANA-negative lupus was first mooted by Koller et al. in 1976, with their description of five patients who were ANA-negative despite having clinical features consistent with SLE.6 Cutaneous features, particularly photosensitivity, appeared to be a predominant feature of this ANA negative group. A succession of reports over the next 10 years suggested that approximately 5% of patients with lupus were ANA-negative. Fessel7 corroborated that finding when he reported 10 cases of persistently ANA-negative SLE (as assessed on human granulocyte substrate) over a period of 10 years. More recently, in 1982 McHardy, Horne and Rennie,8 investigating a cohort of SLE patients in Aberdeenshire, suggested a prevalence of 8.9% for ANA-negative SLE. These findings were based on patients assessed by the modified American Rheumatism Association criteria (later known as the American College of Rheumatology or ACR criteria) for SLE, with ANA determinations performed on rat liver substrate. McHardy et al. justified the alteration of the ACR criteria on the basis that the criteria were conceived to allow comparison of patient groups in …

Balneotherapy for rheumatoid arthritis and osteoarthritis.

Balneotherapy (hydrotherapy or spa therapy) for patients with arthritis is one of the oldest forms of therapy. One of the aims of balneotherapy is to soothe the pain and as a consequence to relieve patients' suffering and make them feel well. To perform a systematic review to assess the effects of balneotherapy for rheumatoid arthritis and osteoarthritis. Using the Cochrane search strategy, studies were found by screening: 1) The Medline CD-ROM database from 1966 to June 1999 and 2) the database from the Cochrane Field 'Rehabilitation and Related Therapies', which contains also studies published in journals not covered by Medline. Also, 3) reference checking and 4) personal communications with authors was carried out to retrieve eligible studies. To perform an adequate assessment of the methodological quality the languages of the publications had to be: Dutch, English, French or German. Date of the most recent literature search: June, 1999 Studies were eligible if they were randomized controlled trials (RCT) comparing balneotherapy with any intervention or with no intervention. Patients included had rheumatoid arthritis (RA), osteoarthritis (OA) or some other form of arthritis. Trials incorporating patients with definite or classical rheumatoid arthritis (RA) as defined by the American Rheumatism Association Criteria (ARA) (Ropes 1958) (these criteria have changed over time) or by the criteria of Steinbrocker (1949) were regarded as a separate group. At least one of the WHO/ILAR core set of endpoints for RA clinical trials had to be the main outcome measures. A criteria list used to assess the methodological quality was the one developed at the Department of Epidemiology at the Maastricht University, called "the Maastricht list". The quality scores and data abstraction of the studies were carried out independently by two reviewers (HdV, RdB). Disagreements were solved by consensus. Ten trials with 607 patients were included in this review. Most trials reported positive findings, but were methodologically flawed to some extent. A 'quality of life' outcome was reported by two trials. Just one of the randomized trials mentioned an intention-to-treat analysis and only three performed a comparison of effects between groups. Pooling of the data was not performed, because of heterogeneity of the studies, multiple outcome measurements, and, apart from two studies, the overall data presentation was too scarce to enable pooling of the data. One cannot ignore the positive findings reported in most trials. However the scientific evidence is weak because of the poor methodological quality, the absence of an adequate statistical analysis, and the absence, for the patient, of most essential outcome measures (pain, quality of life), Therefore, the noted "positive findings" should be viewed with caution. Because of the methodological flaws an answer about the efficacy of balneotherapy cannot be provided at this time. Flaws found in the reviewed studies could be avoided in future trials.

The natural history and prognosis of rheumatoid arthritis: association of radiographic outcome with process variables, joint motion and immune proteins

SummaryObjective: The purposes of the present study were: 1) to investigate how the long‐term course of outcome and inflammatory variables could be described in individual patients and suitably summarized in groups of patients; 2) to investigate the associations between outcome and inflammatory variables on the basis of the defined summary measures; and 3) to investigate new prognostic aspects of RA by means of frozen sera and DNA specimens.Patients and methods: During the period 1966–78, 685 Danish Caucasian patients with RA, classified according to the 1958 American Rheumatism Association (ARA) criteria, were admitted to the Department of Rheumatology of Aarhus University Hospital. For scientific purposes all patients went through the same examination programme, including biochemical variables, clinical evaluation of 68 diarthrodial joints, and radiographic evaluation of 46 diarthrodial joints. Since 1987, data from these patients have been organized in a database. The data are arranged according to onset of disease. This thesis is based on about 600 000 data‐points from 257 patients.Results: The thesis is based on six studies. The first study shows that early symptomatic improvement of RA during gold treatment was stable over several years, but when evaluated radiographically, the condition continued to deteriorate. In the second study, six main types of radiographic progression were identified: (a) a rare type with no radiographic progression at all (<1%); (b) a type with a slow or moderate onset, but an increasing progression rate (exponential growth type) (9%); (c) a linear type (30%); (d) a type with a moderate to fast onset, and a stable progression rate (the square root type) (11%); (e) a type with a fast onset, but a later decreasing progression rate (the first order kinetics type) (30%) and (f) a type characterized by slow onset, then acceleration and later deceleration (the sigmoid type) (20%). The fact that there was a systematic progression was used to define a system of radiographic events, which could be used as outcome measures in prediction models of the long‐term course of RA. The third study shows that low serum levels of the complement‐activating serum lectin, mannan (mannose) binding protein (lectin) (MBP=MBL), are associated with a higher erythrocyte sedimentation rate (ESR) (p=0.006), joint swelling score (JS score) (p=0.019), limitation of joint motion score (LM score) (p=0.027), and annual increase in radiographic destruction score (R score) (p=0.053). The fourth study demonstrated a highly significant association between summary measures of inflammatory variables and radiographic outcome, as defined in the second study, indicating that the degree of inflammation is important for the development of destructive joint damage in RA. The fifth study showed that MBL‐insufficient patients (two defective structural MBL alleles, or one defective allele combined with a low‐expression variant of the normal allele) had a relative risk of a severe radiographic event of 3.1 compared with the MBL competent group (p<0.0001). The sixth study showed that the relative risk (RR) of early interleukin (IL)‐1α auto‐antibodies (aAb) positive patients developing serious radiographic joint destruction was significantly lower than for IL‐1α aAb‐negative patients, RR=0.29 (p=0.04). In rheumatoid factor (RF) positive patients RR was only 0.18 (p=0.02). Patients who seroconverted >2 years after the onset of RA showed the most aggressive development of joint erosion, with RR of serious radiographic joint destruction of 2.56 (p=0.048). Other factors investigated in subgroups of the patients were HLA‐DR4, chemokine receptor 5 (CCR 5) genotypes, IL‐6 aAb, vascular endothelial growth factor (VEGF) aAb, and interferon (IFN)‐γ aAb. About 80% of the patients were HLA‐DR4 positive, indicating the importance of HLA‐DR4 as a predisposing factor for RA. There was no association between IL‐6 aAb and radiographic outcome, or CCR5 genotypes and radiographic outcome. VEGF aAb and IFN‐γ aAb were quantitatively unimportant.Conclusion: In spite of a general improvement in single measures of inflammatory variables, and a general deterioration in radiographic outcome of RA, there is a highly significant association between summary measures of inflammatory variables and radiographic outcome. The progression of radiographic damage in RA follows mathematical patterns. A new method of evaluating the long‐term radiographic outcome by means of Kaplan–Meier plots is demonstrated. It is shown that MBL and IL‐1α aAb are predictors of the prognosis of RA and may play important roles in the pathogenesis of RA.

Anti-tissue transglutaminase antibodies in arthritic patients: a disease-specific finding?

Tissue transglutaminase (tTG), widely distributed in human organs, is a multifunctional enzyme involved in the cross-linking of extracellular matrix proteins, fibrogenesis, and wound healing (1). Recently, tTG has been proposed as the autoantigen of anti-endomysial antibodies (EMAs) (2), a serologic marker of celiac disease (CD) (3). Use of anti-tTG antibodies has been advocated in the diagnostic work-up of CD (4), although positive results have been reported in patients with other intestinal disorders, such as inflammatory bowel disease (5)(6)(7). The aim of the present investigation was to evaluate the occurrence of anti-tTG-positive results in a cohort of arthritic patients, in whom the target organ is located at a distance from the intestine. Changes in anti-tTG antibodies were also investigated in patients with rheumatoid arthritis (RA) during treatment with methotrexate (MTX), a drug previously shown to decrease rheumatoid factor, soluble interleukin-2 receptor, and interleukin-6 (8). In this retrospective study, we enrolled 203 patients [121 males and 82 females; mean (SD) age, 51.4 (14.1) years; range, 17–76 years] attending our Rheumatological Unit in 1998–2000 and presenting without clinical signs and symptoms suggestive of CD. Of these patients, 183 belonged to three different groups: 74 had RA according to the American Rheumatism Association criteria (9); 67 had psoriatic arthritis (PsA) according to the European Spondyloarthropathy Study Group criteria (10); and 42 had ankylosing spondylitis (AS) according to modified New York criteria (11). The remaining 20, having knee, hand, or hip osteoarthritis (OA) according to the American College of Rheumatology criteria (12)(13)(14), were considered as disease controls. Sixty untreated CD patients [25 males and 35 females; mean (SD) age, 42.5 (11.2) years; range, 19–70 years], diagnosed according to the current criteria (15), were selected as disease controls. Fifty-four blood donors [26 males and 28 females; …

Hla class II antigens assoiated with lupus nephritis in italian SLE patients

Human leukocyte antigen DR2 (HLA-DR2), namely the allelic variant HLA-DR15, have been associated with lupus nephritis (LN) in Caucasians. The study investigated the relationships between HLA class II alleles and lupus nephritis in Italian patients. Two hundred forty-four patients fulfilling the American Rheumatism Association criteria for systemic lupus erythematosus (SLE) were typed for HLA-DRB1*, -DQA1*, -DQB1*, and -DPB1* alleles by polymerase chain reaction–sequence-specific oligonucleotide and polymears chain reaction–single-strand polymorphism; 71 patients had renal damage assessed by renal biopsy. Glomerulonephritis was classified using WHO criteria. Significance was tested by X 2 on 2×2 tables. HLA-DQA1*0101 was strongly associated with LN (OR = 2.72 [1.43–5.19]; p = 0.002), whereas HLA-DRB1*1501 was only marginally associated (OR = 1.94 [0.88–4.26]; p = not significant). HLA-DQA1*0102 demonstrated a significant protective effect (OR = 0.31 [0.14–0.86]; p = 0.002). On analyzing the distribution of HLA-DRB1*1501 bearing haplotypes in our SLE patients we found that the HLA-DRB1*1501 greatly enhanced the risk of developing LN conferred by the DQA1*0101 allele (OR = 65.96 [9.35–1326.25]), whereas DQA1*0102 suppressed the nephritogenic effect of DRB1*1501. At renal biopsy, 80% of DRB1*15 positive patients were classified as having class IV LN with the remaining 20% having class III LN. The figures were 19% and 21%, respectively, among the HLA-DR15 negative patients. In the Italian population HLA-DQA and HLA-DR alleles interact in conferring susceptibility to or protection against lupus nephritis, the diffuse proliferative glomerulonephritis ( i.e., the most severe form of nephritis) is associated with the HLA-DR15 bearing haplotypes.

Vesiculobullous systemic lupus erythematosus: A case with circulating IgG and IgA autoantibodies to type VII collagen

We report a case of a 17-year-old Japanese boy with vesiculobullous systemic lupus erythematosus. He had a facial “butterfly” erythema and blistering eruption develop. Antinuclear, RNP, and Sm antibodies were positive. The American Rheumatism Association criteria for a diagnosis of systemic lupus erythematosus were met. A skin biopsy specimen showed papillary microabscesses of neutrophils and subepidermal blisters and deposits of IgG, IgA, IgM, and C3 by direct immunofluorescence. Indirect immunofluorescence on salt-split skin showed linear IgG, IgA, and C3 deposits on the dermal side. IgG and IgA autoantibodies in his serum reacted with the 290-kd proteins of type VII collagen from human dermal extracts and the recombinant fusion proteins from the noncollagenous domain of type VII collagen by Western immunoblot. To our knowledge, this is the first report of vesiculobullous systemic lupus erythematosus with circulating IgG and IgA autoantibodies to type VII collagen. (J Am Acad Dermatol 2002;47:S283-6.)

Paniculitis lúpica asociada a lupus eritematoso sistémico

Abstract.—Lupus panniculitis or lupus erythematosus profundus in a mixed panniculitis with a predilection for the face, proximal extremities, trunk or buttocks. It occurs in 2% of lupus erythematosus patients. The serum antinuclear antibody is ussally positive in about 70% of case, but only 25%- 50% fulfil the American Rheumatism Association criteria for systemic lypus erythematosus.We report a 54-year-old women who developed firm, erythematous nodules located mainly on the face, trunk and buttocks. Histological examination showed a mixed panniculitis with hyaline fat necrosis and calcification. Laboratory tests revealed lymphopenia and anemia. ANA and anti-DNA were positive. For several months the patient had also been suffering from arthralgia with synovitis of the wrist and several interphalangeal joins. Overall, these clinical and serology findings were consistent with the diagnosis of lupus panniculitis associated with mild systemic lupus erythematosus. Treatment consisted of synthetic antipaludian medication and low dose systemic corticosteroids, under which the lesions regressed except one of them, which remained as a solitary plaque, on buttocks, with an important calcification.

Association entre un lupus érythémateux disséminé et une maladie de Crohn

Introduction. – Association of Crohn’s disease and systemic lupus erythematosus is infrequent. We report an observation of Crohn’s disease that appeared in an 18-year-old woman followed-up for systemic lupus erythematosus for four years. Exegesis. – The patient had all the clinical, biological and histological criteria of Crohn’s disease and systemic lupus erythematosus was diagnosed according to the American Rheumatism Association criteria. On the base of this observation, we discuss the digestive manifestation of systemic lupus erythematosus and extradigestive manifestations of Crohn’s disease. Conclusion. – The immunological background of both diseases was proposed to explain the mechanism of this rare association.

What is the factor that most influences QOL among rheumatoid arthritis patients?

We attempted to elucidate the factors which affect the quality of life (QOL) among patients with rheumatoid arthritis (RA). Ninety-five patients who satisfied the American Rheumatism Association criteria for RA were asked to fill in a modified arthritis impact measurement scale, version 2 (AIMS2) and complete a Lorish's face scale (FS) test. The same questionnaire and FS test were completed by 75 healthy persons as controls. We used Lorish's FS for our assessment of QOL. The investigation was undertaken to analyze the relationship between FS and each item on the questionnaire. For average FS score, there was no significant difference between the RA group and the controls. However, RA group scores covered a wider range than those of the controls. From the correlation analysis, physical stress, pain factors, and some of the activities of daily living (ADL) factors showed a strong correlation with FS. ADL factors which strongly correlated with FS were those related to activities of the lower limbs. Other ADL factors were moderately correlated with FS. Socioeconomic factors were not significantly correlated with FS. There was no significant difference between the QOL of RA patients and that of healthy controls. The QOL was correlated with pain and stress factors rather than with ADL factors among patients with RA.

The ability of the Cochin rheumatoid arthritis hand functional scale to detect change during the course of disease.

To assess changes measured with the Cochin rheumatoid arthritis (RA) hand functional disability scale during the course of the disease. A cohort study evaluating outcome measure responsiveness in RA was conducted in a referral center. Ambulatory or hospitalized patients with RA according to the 1987 American College of Rheumatology (formerly the American Rheumatism Association) criteria were evaluated twice. Clinical measures included Cochin's scale, Revel's functional index, hand functional index, visual analog scale of patient-perceived handicap, visual analog scale of pain in hands and wrists, total score of swelling, total score of tenderness, and morning stiffness duration. Responsiveness was assessed by the effect size (ES) and the standardized response mean (SRM). The nonparametric Spearman rank correlation coefficient (r) was used to assess the correlation between two quantitative variable changes. Fifty-five patients (44 women) were evaluated twice at an interval of 15.4 +/- 1.4 months (mean +/- SD) (range 13-18 months). The Cochin scale total score had worsened at the second visit (95% confidence interval for mean differences -5.16, 0.73). Its SRM and ES values were -0.20 and -0.15, respectively. Changes in the score had the highest correlation (r = 0.58) with changes in the patient-perceived handicap, while it had only fair or little correlation with changes in the disease activity measures. The factor 2 scale subscore significantly worsened and had the highest values of SRM and ES (SRM = -0.40 and ES = -0.31). The Cochin scale can detect small but meaningful changes in RA hand disability.

A case of systemic lupus erythematosus associated with superior vena cava syndrome

We report on a case of systemic lupus erythematosus associated with superior vena cava syndrome. A 46-year-old woman developed polyarthralgia in December 1994. She was treated with nonsteroidal anti-inflammatory drugs. In February 1995, she was admitted to our hospital with systemic convulsion and disturbance of consciousness (III-300/Japan coma scale). Severe facial edema was also present. Laboratory studies revealed the presence of anti-nuclear antibody, anti-DNA antibody, anti-Sm antibody, and proteinuria. An X-ray film of the chest showed pericardial effusion and bilateral pleural effusions. Computed tomography of the chest showed a severe swelling of mediastinal lymph nodes. A diagnosis of systemic lupus erythematosus was made according to the American Rheumatism Association criteria. Initial treatment with intravenous dexamethasone improved the level of consciousness and decreased the facial edema, mediastinal lymphadenopathy, and the effusions on computed tomography of the chest. We believe that the most likely explanation for the facial edema is superior vena cava syndrome due to severe mediastinal lymphadenopathy.

Pregnancy outcome and family size in systemic lupus erythematosus: a case-control study.

To establish pregnancy outcomes and family size in a geographically defined population of systemic lupus erythematosus (SLE) patients. One hundred and thirty-eight SLE patients (all women satisfying at least four American Rheumatism Association criteria) and 276 age-matched female controls, from the Nottingham area, were interviewed by a single investigator. Demographic details and maternity histories were obtained, and the data collected were analysed statistically to calculate odds ratios (ORs) for risk of fetal loss (through miscarriage, stillbirth and abortion). Family size was also determined in White and non-White cases and controls. Women with SLE are at greater risk of spontaneous fetal loss than their healthy counterparts (OR = 2.21, 95% CI 1.46-3.35, P < 0.01) and they are more likely than controls to have a surgical abortion (OR 2.44, 95%, CI 1.22-4.87, P = 0.01). The excess risk of both of these outcomes exists both before and after diagnosis of SLE. The median number of children in White and non-White families of cases and controls is the same, i.e. two. White women with SLE, however, appear less likely than controls to have more than two children, whereas non-White lupus women tend to retain their propensity to have larger families, i.e. more than two children. We confirm that lupus women who have, or later develop, SLE are at greater risk of pregnancy loss by spontaneous or surgical means. We have also shown that race, and the inherent differences in social and cultural influences, appears to be an important determinant of ultimate family size; White women with SLE have fewer children than controls, whilst non-White lupus women tend to have larger families.

Prevalence of rheumatoid arthritis in Dublin, Ireland: a population based survey.

The prevalence of Rheumatoid Arthritis (RA) in Ireland has never been established. Studies from different countries show varying rates, being almost 100 per cent greater in the highlands of Scotland (10/1,000) than in rural Lesotho (6/1,000). A recent study also suggests a fall in the prevalence of RA among women in the London urban area. Given these variations the validity of extrapolating prevalence rates established for other countries to Ireland is questionable. This study aimed to establish a prevalence rate for RA in a defined Dublin population. A self-administered questionnaire was sent to 2,500 people chosen at random from the electoral register. The questionnaire was designed to select out both undiagnosed patients and those with definite arthritis. Respondents whose replies indicated an arthritic process, but in whom no diagnosis had been made, were asked to attend for further assessment and investigations as appropriate. Those who responded that they had been diagnosed with arthritis were asked for consent to inspect their hospital or general practitioner records. A diagnosis of RA was based on American Rheumatism Association (ARA) criteria. Valid responses were received from 1,227 people surveyed (response rate = 49 per cent). Six cases of RA were identified including 2 previously undiagnosed cases. A prevalence rate of 5/1,000 has been estimated based on these findings.

PREVALENCE OF INFECTIVE ENDOCARDITIS IN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS

Compared with the general population, patients with systemic lupus erythematosus, or SLE, have an increased prevalence of functionally impaired cardiac valves due to the presence of Libman-Sacks lesions. These lesions may place patients with SLE at risk of developing infective endocarditis, or IE. The authors performed a retrospective chart review to determine the association between SLE with valvulopathy and IE. They reviewed the records of 361 patients from two health care facilities who had the diagnostic code of SLE. Of the 275 records that met the 1982 revised American Rheumatism Association criteria for SLE, 51 (18.5 percent) were for patients who had a clinically detectable heart murmur that resulted in echocardiography being performed. Nine (3.3 percent) of the 275 patients had a clinically significant valvular abnormality, three (1.1 percent) had a potentially significant valvular abnormality, and one (0.4 percent) had a history of IE that was diagnosed two years before her diagnosis of SLE was made. The findings suggest that 18.5 percent of this cohort of patients with SLE had a clinically detectable heart murmur that would require further investigation to determine its significance. Furthermore, between 3.3 and 4.4 percent of the study population had cardiac valve abnormalities that potentially required antibiotic prophylaxis before certain dental procedures. However, the authors identified no cases that demonstrated an association between IE and diagnosed SLE. Dentists should query their patients with SLE about their cardiac status and consult with the patient's physician if the cardiac status is unknown. Patients with confirmed valvular abnormalities should receive antibiotic prophylaxis for designated bacteremia-producing dental procedures.

Review: dynamic exercise increases aerobic capacity and muscle strength in rheumatoid arthritis

(1998) Br J Rheumatol 37, 677; van den Ende CH, Vliet Vlieland TP, Munneke M . , et al. . Dynamic exercise therapy in rheumatoid arthritis: a systematic review. ....

Integrin signalling defects in T-lymphocytes in systemic lupus erythematosus.

To establish the relationship between T cell responses to integrin coreceptor stimulation and B cell hyperreactivity as measured by pathologic autoantibody production. Peripheral blood mononuclear cells from 42 patients with SLE according to the American Rheumatism Association criteria were examined for their ability to adhere to plate-immobilised fibronectin. Co-stimulation assays were performed on the same cells using anti-CD3 antibody alone or co-immobilised with an anti-beta1-integrin antibody. Proliferative responses were measured by 3[H]thymidine pulsing on day 3 and activation was determined using a commercial protein kinase C assay, the protocol being established by our group in association with Promega. Beta-integrin expression was established by FACS analysis. An impaired PKC response to integrin-mediated activation was found in T-lymphocytes from 6/21 (29%) SLE patients, which correlated significantly with an absence of anti-dsDNA antibody in patient sera, irrespective of prednisolone treatment. Integrin co-stimulation of TcR/CD3-induced proliferation and T cell adhesion to fibronectin were also impaired among 5/21 (24%) and 6/15 (40%) patients studied, respectively. We hypothesise that the integrity of beta1-integrin signalling pathways may influence pathological antibody production in SLE by affecting T-lymphocyte activation and interactions between T- and B-lymphocytes.