Report on vasculitis special interest group.
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Humans↗
Biomedical subjects
D G Scott
Publications and source records attributed to D G Scott.
Blood transfusion, smoking, and obesity as risk factors for the development of rheumatoid arthritis: results from a primary care-based incident case-control study in Norfolk, England.
OBJECTIVE: To examine a range of demographic, social, and clinical risk factors for the development of rheumatoid arthritis (RA). METHODS: Population-based case-control study in Norfolk, England, involving adult patients, ages 18-70, with an inflammatory polyarthritis of <12 months' duration who were recruited from the Norfolk Arthritis Register. Controls, matched for sex and date of birth, were selected from the primary care register of the Norwich Health Authority. Both cases and controls completed identical self-administered questionnaires. Matched analysis of the 165 case-control sets was conducted for the whole group and for the subset in which the cases satisfied the 1987 American College of Rheumatology criteria for RA. RESULTS: The controls were of higher socioeconomic status than the cases. This was probably due to response bias. Having a body mass index > or =30 was associated with an adjusted odds ratio (OR) of 3.74 for developing RA (95% confidence interval [95% CI] 1.14-12.27). RA was also associated with a history of blood transfusion (OR 4.83, 95% CI 1.29-18.07). Even after correcting for social class, a history of having ever smoked was associated with a higher risk of developing RA (OR 1.66, 95% CI 0.95-3.06). There was no difference between cases and controls in previous exposure to childhood infections, certain surgical procedures, or reproductive history variables. CONCLUSION: RA has a number of potential environmental triggers, including smoking, obesity, and blood transfusion.
Adult↗
Classification and epidemiology of the vasculitides.
The systemic vasculitides are rare inflammatory conditions of blood vessel walls. A number of different classification schemes have been published since the first in 1952. The important developments have been the recognition of dominant blood vessel size, the distinction between primary and secondary vasculitis and the incorporation of pathogenic markers such as anti-neutrophil cytoplasmic antibodies. In 1990 the American College of Rheumatology (ACR) published criteria for the diagnosis of polyarteritis nodosa, Churg-Strauss syndrome, Wegener's granulomatosis, hypersensitivity vasculitis, Schönlein-Henoch purpura, giant cell arteritis and Takayasu arteritis. Sensitivity and specificity rates varied considerably: 71.0-95.3% for sensitivity and 78.7-99.7% for specificity. The criteria were not tested against the general population or against patients with other connective tissue diseases or rheumatic conditions. Four years later the Chapel Hill Consensus Conference (CHCC) produced definitions for the major types of vasculitis, however, these have proved controversial. Comparison in unselected patients with systemic vasculitis (in particular polyarteritis nodosa and microscopic polyangiitis) has shown that the ACR criteria and CHCC definitions identify different patients. The systemic vasculitides are somewhat more common than previously believed. The overall annual incidence approaches 40/million adults. The most common form of primary systemic vasculitis is giant cell arteritis; Wegener's granulomatosis, microscopic polyangiitis and Churg-Strauss syndrome have similar incidences. Classical polyarteritis nodosa and Takayasu arteritis are very rare in the UK.
Humans↗
Neoral--new cyclosporin for old?
Cyclosporin A is now well established as an effective second-line drug to treat rheumatoid arthritis. In April 1995, the microemulsion-based formulation of cyclosporin (Neoral) was introduced based on its increased bioavailability at 'no extra cost'. There may have been concerns that with increased bioavailability of Neoral, some patients might experience increased toxicity, particularly if transferring from Sandimmun to Neoral at the same dose. We describe our experience of 51 patients treated with Neoral--39 with rheumatoid arthritis, six with psoriatic arthritis and the remainder with a variety of diseases, including Behçet's, systemic lupus erythematosus and juvenile chronic arthritis. All patients continued their other medication including non-steroidal anti-inflammatory drugs and analgesics. Five continued low dose prednisolone (average 7.5 mg per day) all patients were monitored for safety and efficacy throughout their treatment according to standard protocol. Five patients were enrolled in a study of efficacy and safety where the dose of cyclosporin was reduced to 2.5 mg/kg/day at the time of conversion, i.e. to Neoral 2.5 mg/kg/day; 19 patients were converted dose for dose, cyclosporin A dose range 2.5-4 mg/kg/day converted to Neoral dose range 2.5-4 mg/kg/day and 27 patients started Neoral de novo. We conclude that cyclosporin is a useful disease modifying anti-rheumatic agent, and our experience suggests that the new formulation, Neoral, has a similar safety and efficacy profile to the original preparation (Sandimmun). Neoral was relatively easy to manage and we noted a slight reduction in dose when compared to Sandimmun. With dose adjustments over 18 months the mean dose for patients with RA fell from 3.2 to 2.7 mg/kg/day and of the 27 patients starting Neoral de novo only seven required an increased dose above 2.5 mg/kg/day in order to establish efficacy.
Adolescent↗
Presence of psoriasis does not influence the presentation or short-term outcome of patients with early inflammatory polyarthritis.
OBJECTIVE: To determine whether the presence of psoriasis influences the presentation and early outcome of disease in a primary case based inception cohort of patients with early inflammatory polyarthritis. METHODS: In total, 966 patients with early inflammatory polyarthritis referred to the Norfolk Arthritis Register were studied. The clinical and demographic variables of patients with and without psoriasis were compared. RESULTS: Fifty-one patients (5.3%) had psoriasis on examination. Compared to other patients with inflammatory polyarthritis, those with psoriasis were significantly more likely to be male (49 vs 34%) and less likely to be seropositive for rheumatoid factor (RF) (13 vs 31%). The pattern of joint involvement was similar, as was the outcome at one year. Fewer patients with psoriasis developed radiological erosions (22 vs 39%). Multivariate analysis suggested that this was due to RF acting as a confounder. CONCLUSION: Among patients with early inflammatory polyarthritis there are few strong differences between patients with and without psoriasis, some of which may be accounted for by the absence of RF.
Adolescent↗
Differences in the urinary metabolites of the tobacco-specific lung carcinogen 4-(methylnitrosamino)-1-(3-pyridyl)-1-butanone in black and white smokers.
Incidence and mortality rates for lung cancer in the United States are significantly greater in blacks than in whites. This disparity cannot be explained by differences in smoking behavior. We hypothesize that the observed racial differences in risk may be due to differences in the metabolic activation or detoxification of the tobacco-specific lung carcinogen 4-(methylnitrosamino)-1-(3-pyridyl)-1-butanone (NNK). To test this, different biomarkers of NNK exposure and metabolism, including the urinary metabolite 4-(methylnitrosamino)-1-(3-pyridyl)-1-butanol (NNAL) and the presumed detoxification product [4-(methylnitrosamino)-1-(3-pyridyl)but-1-yl]-beta-O-D-glucosiduronic acid (NNAL-Gluc), were examined along with questionnaire data on lifestyle habits and diet in a metabolic epidemiological study of 34 black and 27 white healthy smokers. Results demonstrated that urinary NNAL-Gluc:NNAL ratios, a likely indicator of NNAL glucuronidation and detoxification, were significantly greater in whites than in blacks (P < 0.02). In addition, two phenotypes were apparent by probit analysis representing poor (ratio < 6) and extensive (ratio > or = 6) glucuronidation groups. The proportion of blacks falling into the former, potentially high-risk group was significantly greater than that of whites (P < 0.05). The absolute levels of urinary NNAL, NNAL-Gluc, and cotinine were also greater in blacks than in whites when adjusted for the number of cigarettes smoked. None of the observed racial differences could be explained by dissimilarities in exposure or other sociodemographic or dietary factors. Also, it is unlikely that the dissimilarities are due to racial differences in preference for mentholated cigarettes, because chronic administration of menthol to NNK-treated rats did not result in either increases in urinary total NNAL or decreases in NNAL-Gluc:NNAL ratios. Altogether, these results suggest that racial differences in NNAL glucuronidation, a putative detoxification pathway for NNK, may explain in part the observed differences in cancer risk.
Adult↗
Disodium pamidronate has beneficial effect in Paget's disease of bone.
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Diphosphonates↗
Effect of classification on the incidence of polyarteritis nodosa and microscopic polyangiitis.
OBJECTIVE: Polyarteritis nodosa (PAN) has been used as a generic term for systemic vasculitis. The distinction between classic PAN and microscopic polyangiitis (MPA) has not always been made. The aims of this study were to compare the American College of Rheumatology (ACR) criteria for PAN with the Chapel Hill Consensus Conference (CHCC) definitions of classic PAN and MPA, and to estimate the annual incidence of PAN and MPA. METHODS: The 1990 ACR criteria and CHCC definitions for systemic vasculitis were applied to an unselected cohort of 130 patients with systemic vasculitis attending a single district hospital in the UK between February 1, 1988 and January 31, 1994. RESULTS: Eight patients who met the ACR criteria for PAN and who also met the CHCC definition of MPA but not classic PAN were identified. A further 5 patients met the CHCC definition of MPA but not the ACR criteria for any other type of systemic vasculitis. No patient who met the CHCC definition of classic PAN was identified. The annual incidence of MPA was calculated to be 3.6/million (95% confidence interval 1.7-6.9), and the annual incidence of PAN (ACR criteria) was 2.4/million (95% confidence interval 0.9-5.3). CONCLUSION: Classic PAN as defined by the CHCC is rare, because small vessel involvement is excluded from this definition.
Antibodies, Antineutrophil Cytoplasmic↗
Wegener's granulomatosis--increased incidence or increased recognition?
We aimed to calculate the annual incidence of Wegener's granulomatosis (WG) in Norfolk (UK) and to compare the clinical spectrum of disease to that seen in tertiary centres in both the UK and USA. We also aimed to examine the seasonal onset of symptoms. This was a prospective study of all patients presenting with WG to a district hospital with a well-defined stable population (515,000) during the period 1988-1993. The annual incidence for WG in the adult population is 8.5/million (95% CI 5.2-12.9). These are the first incidence figures for a well-defined population and are higher than previously published. The clinical spectrum of disease in Norfolk was similar to that seen in tertiary centres and there is supportive evidence for a seasonal variation in the onset of symptoms (highest in winter).
Adult↗
Report of Special Interests Group held at the British Society of Rheumatology meeting in Glasgow, April 1995.
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Clinical Trials as Topic↗
Inflammatory polyarthritis in the community is not a benign disease: predicting functional disability one year after presentation.
OBJECTIVE: To predict which patients with early inflammatory polyarthritis presenting to primary care will be functionally disabled one year after presentation, in order to inform treatment and referral decisions. METHODS: The study population consisted of 381 patients notified to the Norfolk Arthritis Register, a primary care based inception cohort of patients with inflammatory polyarthritis. Patients were regarded as functionally disabled if they had a Health Assessment Questionnaire (HAQ) score of one or more. Clinical, laboratory, and demographic variables easily measured at baseline were analyzed for their ability to predict future disability. Recursive partitioning was used to create a simple decision tree to predict those patients who would be disabled at one year. A logistic regression model was generated on a sample of 277 patients and tested on an independent sample of 104 patients. This was compared with other models, one of which consisted of the 1987 ARA criteria. RESULTS: 112 (29%) patients had a HAQ score of at least 1 at one year. The strongest predictors of future disability were a high baseline HAQ, large joint involvement, female sex, and longer disease duration. The decision tree predicted disability accurately in 67% of patients. CONCLUSION: It is possible to predict functional outcome at one year among patients with early inflammatory polyarthritis presenting to primary care using simple clinical variables measured at baseline. Satisfying the 1987 ARA criteria could not be used to predict future disability.
Adult↗
ABC of rheumatology. Rashes and vasculitis.
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Arthritis↗
Epidemiology of systemic vasculitis: changing incidence or definition?
The epidemiology of the systemic vasculitides is poorly documented. Many studies have been conducted from tertiary referral centers, with resulting problems of referral bias and uncertainty of denominator population, or have involved small populations. We have estimated the incidence of the major forms of systemic vasculitis in a stable, ethnically homogeneous population of 414,000 adults from 1988 to 1994. The overall annual incidence of systemic vasculitis (excluding giant cell arteritis) is 39/million (95% confidence intervals; ranging from 31 to 47). The annual incidence of Wegener's granulomatosis is 8.5/million (range, 5.2 to 12.9), Churg-Strauss syndrome 2.4/million (0.9 to 5.3), microscopic polyangiitis 2.4/million (0.9 to 5.3), adult Henoch-Schonlein purpura 1.2/million (0.3 to 3.5), and systemic rheumatoid vasculitis 12.5/million (8.5 to 17.7). These data suggest that the overall incidence of systemic vasculitis is greater than previously thought (10/million) with Wegener's granulomatosis and systemic rheumatoid vasculitis being the most common. Whether this represents a genuine increase in incidence or increased physician awareness is uncertain.
Churg-Strauss Syndrome↗
Vasculitis.
The systemic vasculitides are a group of rare inflammatory conditions resulting in inflammation and necrosis of blood vessel walls. They are somewhat commoner than previously believed with an annual incidence approaching 40 per million. Furthermore the annual incidence of rheumatoid vasculitis is 12.5 per million and Wegener's granulomatosis is 8.5 per million. The first useful classification system for systemic vasculitis was published in 1952, since then a number of different schemes have been published. The major changes have been the recognition of the importance of dominant blood vessel size, the distinction between primary and secondary vasculitis and the incorporation of pathogenetic markers such as ANCA (see Table 6). Until relatively recently there were no widely agreed diagnostic or classification criteria. In 1990 the ACR published criteria for the diagnosis of polyarteritis nodosa, Churg-Strauss syndrome, Wegener's granulomatosis, hypersensitivity vasculitis, Henoch-Schönlein purpura, giant cell arteritis and Takayasu's arteritis. The criteria were provided in both traditional and tree format. Sensitivity and specificity rates varied considerably: 71.0-95.3% for sensitivity and 78.7-99.7% for specificity. The criteria were not tested against the general population or against patients with other connective tissue diseases or rheumatic conditions. In 1993/94 the Chapel Hill Consensus Conference developed and published definitions for the nomenclature of systemic vasculitis based on clinical features. These have not met with universal acceptance. However, they are a useful addition, since their use should result in different centres studying more homogeneous populations of patients and facilitate comparison of data between different centres. Assessment of vasculitis comprises an activity score (BVAS), damage index and quality of life/health status (SF-36). These are recent developments which are still undergoing validation.
Humans↗
Isolated nail fold vasculitis in rheumatoid arthritis.
OBJECTIVES: To establish the clinical significance of isolated nail fold vasculitis in patients with rheumatoid arthritis (RA). METHODS: Patients attending hospital with established RA were assessed by a single observer using unaided vision. Isolated nail fold vasculitis (NFV) was diagnosed as small nail edge or nail fold lesions without any evidence of other extra-articular or systemic vasculitis. Patients were followed prospectively. RESULTS: Thirty patients with isolated NFV were identified and followed for a median interval of 22 months. Three patients died within three months of diagnosis and six developed complications possibly indicative of extra-articular disease: xerostomia (three), pericarditis (one), pleural effusion and empyaema (one), pulmonary fibrosis (one). No patient developed systemic vasculitis. CONCLUSION: Isolated NFV has a favourable prognosis compared with systemic vasculitis. There is a low risk of developing systemic or extra-articular disease.
Adult↗
A longitudinal study of anticardiolipin antibody in polymyalgia rheumatica and giant cell arteritis.
OBJECTIVE: To determine the incidence of elevated levels of anticardiolipin antibody (aCL) in patients with newly diagnosed polymyalgia rheumatica (PMR) and/or giant cell arteritis (GCA); and to determine the relationship between these antibodies at diagnosis and subsequent course of the disease over a period of 2 yrs. METHODS: Ninety-eight consecutive patients with PMR and/or GCA were examined for the presence of aCL, at presentation and every 6 mo for 2 yrs. Sixty-four patients had PMR alone, 22 had coexistent PMR and GCA, and 12 presented with pure GCA. Patients presenting with suspected clinical diagnosis, overt or covert, of GCA were subjected to temporal artery biopsy from the symptomatic side within 3 days of presentation. Appropriate serological, biochemical, and hematological investigations were undertaken at presentation and subsequently at times of periodic assessments. One hundred healthy age and sex matched elderly subjects were also screened for the presence of aCL as a control group. RESULTS: Elevated levels of aCL were detected in 20 patients at presentation. These included 9 patients with PMR/GCA and 11 patients with pure PMR. During followup, 10 patients with pure PMR at presentation developed GCA. These comprised 5 of the 11 patients with high aCL at presentation and 5 of the 53 patients with normal levels of aCL at presentation. This was statistically significant with relative risk (4.82, 95% CI, 1.72-13.51) of developing GCA in the presence of PMR and a high aCL at presentation. Furthermore, 3 of the 5 patients with pure GCA and high aCL at presentation progressed to severe vascular complications (stroke, 2; anterior ischemic optic neuritis, 1) compared to none of the other patients in the study. Elevated levels of antineutrophilic cytoplasmic antibody were also analyzed and detected in only 4 patients, 3 with pure PMR and one with biopsy proven GCA. CONCLUSION: This prospective study suggests that a significant number of patients with PMR and/or GCA with elevated levels of aCL at presentation have increased risk of developing GCA or other major vascular complications. It is possible that aCL may be an independent prognostic marker for future vascular complications in patients with PMR and/or GCA.
Aged↗
Classification and epidemiology of systemic vasculitis.
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Female↗
The arthropathy of the Muckle-Wells syndrome.
Muckle-Wells syndrome (MWS) is a rare condition characterized by urticaria, arthralgias, deafness and amyloid nephropathy. The arthropathy is poorly documented. We describe the arthropathy occurring in four cases of MWS and discuss the management. Each patient developed recurrent bouts of transient synovitis. One patient developed a persistent sterile pyoarthrosis.
Adult↗