Complications associated with labial salivary gland biopsy in the investigation of connective tissue disorders.
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Biomedical subjects
Publications and source records attributed to P Maddison.
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Rheumatological disease is common but is often overlooked or inadequately assessed by doctors. This may reflect training in the discipline. The results of a survey of all British medical schools by the Arthritis and Rheumatism Council and the British Society for Rheumatology show that clinical rheumatology teaching forms a small part of most courses (median 4, range 0-8 weeks), usually as a second-year specialty attachment, and that specific assessment of basic clinical skills in rheumatology is often not undertaken. The results of the survey support the idea that the current teaching of rheumatology might help to marginalise the subject rather than to promote it.
Rheumatologists from 4 countries, representing 8 rheumatology centers, tested 3 systemic lupus erythematosus (SLE) disease activity indices: the SLE Disease Activity Index (SLEDAI) from Toronto; the Systemic Lupus Activity Measure (SLAM) from Boston and the British Isles Lupus Assessment Group (BILAG) for their reproducibility and validity in the assessment of real patients. Seven patients representing a spectrum of disease manifestations and activity were each examined by 4 of 7 observers from all centers except Toronto, using a Youden square design. Each observer completed all 3 indices and a category rating scale for disease activity on each of the 4 patients seen. All 3 indices detected differences among patients. There was no detectable observer effect among the 7 observers with each of the 3 indices. There was a detectable order effect with the SLAM. The 3 indices are comparable and reproducible for evaluating disease activity in SLE.
The recent description of a lupus-like disease in normal mice after injections of human mAb that bind DNA and carry the common Id 16/6 Id has excited much attention. In an effort to reproduce this model we have performed two experiments using eight human mAb three of which bear the 16/6 Id. Despite using an injection protocol very similar to that of the original authors and waiting for up to 1 yr we were unable to detect any autoantibodies or any evidence of renal disease. We suspect that our failure to reproduce the model may be due to differences in either or both the batches of CFA used, or in the animal house environments. It supports the view that a mosaic of effects is required to induce clinical expression of an autoimmune disease.
OBJECTIVE: To investigate the prevalence of Raynaud's phenomenon in the populations of five general practices. DESIGN: Two populations studied. A questionnaire was given to all new patients attending five general practices over four weeks, and the same questionnaire was sent by post to a random sample of adults from two of the practices. SETTING: General practices in inner London, Merseyside, and Cheshire. SUBJECTS: 1532 Patients who completed questionnaires (1119 who attended the surgeries (response rate unknown) and 413 respondents to the postal survey (response rate 69%)). MAIN OUTCOME MEASURES: Response to questionnaire on symptoms of Raynaud's phenomenon: patients were regarded as having the disease if they had episodes of blanching of the fingers that were precipitated by cold and accompanied by sensory symptoms (pins and needles or numbness). Subsequent interview and clinical appraisal of patients with the disease according to their responses to the questionnaire. RESULTS: The prevalence of Raynaud's phenomenon was 11% (26/231) and 19% (34/182) respectively in men and women who completed the postal questionnaire and 16% (56/357) and 21% (157/762) respectively in those who completed the questionnaire when attending their general practice. Thus the overall rates were slightly higher in women, but there was no effect of age even after adjustment of the rates for practice and method of survey. CONCLUSION: The prevalence of Raynaud's phenomenon is high compared with the low number of patients who seek treatment for the disease.
In a series of 163 patients with systemic lupus erythematosus (SLE) and/or systemic sclerosis and/or mixed connective tissue disease (MCTD), the presence of antibodies to a 68 kDa U1RNP associated polypeptide was more predictive for SLE (88%) than for MCTD (38-68%) using 3 different sets of proposed criteria for the latter condition. In all but one case studied serially the immunoblotting profile remained constant. In SLE recognition of the 68 kDa polypeptide identified a subset with increased vasoreactivity with Raynaud's phenomenon (96%, p less than 0.001) and swollen fingers (68%, p less than 0.001) whereas recognition of a 47 kDa La (SSB) polypeptide was associated with photosensitivity (72%, p less than 0.02), less renal involvement (p less than 0.05) and an older age of disease onset (46.6 years +/- 18.3 SD, p less than 0.002).
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Five centres in Great Britain and the Republic of Ireland have collaborated to produce a computerized index of clinical disease activity in systemic lupus erythematosus, based on the principle of the physician's intention to treat. The index assesses separately eight organ-based systems. The index has proved quick and easy to use despite a comprehensive database and compares favourably with two other indices of disease activity. It has great potential for use in multicentre studies of disease outcome and new therapies in systemic lupus erythematosus.
Autoantibody profiles were examined in the sera of untreated patients with tuberculosis, and those with klebsiella septicaemia, and klebsiella and E. coli urinary tract infections. Rheumatoid factors of the IgM, IgA and IgG isotypes, antinuclear antibodies and antibodies to poly(ADP-ribose) were all frequently detected (generally 15-40%). In contrast, antibodies to the extractable nuclear antigens and to the organ specific antigens were unusual (generally less than 10%). In comparison, in a group of lupus patients IgM rheumatoid factor, anti-nuclear antibodies, antibodies to poly(ADP-ribose) and antibodies to the extractable nuclear antigens were more frequently found, but IgA and IgG rheumatoid factors and antibodies to the organ-specific antigens were present in much the same frequency.
Clinical and serological evidence of connective tissue disease was found in a high proportion of 132 family members of 30 patients with systemic sclerosis. In 20 probands with the milder CREST form of the disease, 10 had HLA-DR5 and 12 had null alleles at the C4 loci. None of 11 probands with more severe systemic sclerosis had HLA-DR5; all 11 had null alleles at the C4 loci. All but two of the probands had either HLA-DR5 or a C4 null allele, and this was also the case for the majority of the relatives with autoantibodies. Genetic markers of the major histocompatibility complex, including HLA-DR5 and C4 null alleles, appear to be closely associated with markers of disease in these probands and their families.
One hundred consecutive admissions to an acute geriatric unit were examined for clinical and radiographic evidence of osteoarthritis (OA) and articular chondrocalcinosis (ACC). Thirty-four patients had ACC. This was age related, the prevalence rising from 15% in patients aged 65-74 years to 44% in patients over 84 years. The commonly involved joints were the knee (25%), public symphysis (15%), and wrist (9%). No other aetiological factors predisposing to ACC were found. Of the 25 patients with ACC in the knee 7 had no symptoms or signs and no radiographic evidence of OA at that site. However, the combination of ACC and radiographic OA was characterised by an increase in clinical joint disease. Features of inflammation (joint swelling and joint line tenderness) involving the knee, wrist, and elbow were particularly common in ACC. It is concluded that ACC is common in the elderly and is associated with an increased incidence of joint disease.
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A man with long standing and severe ankylosing spondylitis, treated with radiotherapy, developed a primary adenocarcinoma of the lung in an area of apical fibrosis. The significance of this is discussed and the literature reviewed.
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Seventy-two patients were studied whose sera contained antibodies to a cytoplasmic antigen, RO. The majority had clinical features typical of systemic lupus erythematosus and there appeared to be an overlap with Sjögren's syndrome. Of interest is that certain patients with lupus respond predominantly to cytoplasmic antigens and in some cases were ANA negative. Demonstration of this system is important in enlarging the serologic spectrum characteristic of SLE patients, and also may be an important marker for systemic disease in patients with predominantly cutaneous involvement.