The absence of evidence suggesting that systemic lupus erythematosus is a zoonosis of dogs.
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Biomedical subjects
Publications and source records attributed to R Manthorpe.
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A new experimental design was developed to study the value of clinical parameters of disease activity in patients with rheumatoid arthritis. Ten patients with classical rheumatoid arthritis were examined by five senior doctors in a department of medical rheumatology. In spite of an attempt to make the clinical examination as uniform as possible the inter-observer variation among the doctors was greater than the variation among the patients, for the following parameters: joint pain at rest, joint tenderness and joint swelling. An acceptable inter-observer variation in relation to patient variation was found for 1) a combined registration of joint pain at rest or on movement, 2) duration of morning stiffness, 3) grip strength, 4) subjective well-being as indicated on a visual analogue scale, 5) fingertip--palm distance, and 6) maximum flexion-extension in elbows, wrists and knees. The variation from morning to afternoon and from day to day was negligible. It is concluded that registration of elaborated articular scores is useless in the daily routine in rheumatological departments when different doctors examine the patients.
The transcapillary escape rate of albumin was measured in 27 consecutive patients with inflammatory rheumatic diseases before and after 1 and 7 days of prednisone treatment in doses of 45 mg/day. The transcapillary escape rate decreased from 7.33%/h (range 5.11-9.55) before prednisone treatment to 3.11%/h (0.04-6.18) (p less than 0.05) after 1 day of treatment and 5.80%/h (4.36-7.24) after 7 days of treatment. It is concluded that prednisone inhibits vascular permeability in patients with inflammatory rheumatic diseases.
Sera from 70 patients with Sjogren's syndrome (SS), of whom 40 had primary and 30 secondary SS, were tested for various auto-antibodies of the IgG, IgA and IgM classes. 20% had liver-cell-membrane antibody (LMA), 90% had anti-nuclear antibodies (ANA), 40% salivary-gland antibodies (SGA), 13% anti-mitochondrial antibodies (AMA), 33% smooth-muscle cell antibodies (SMA), 9% skeletal-muscle antibodies (SKA) and only 1% had parietal-cell antibodies (PCA). In addition, 53% had IgM rheumatoid factor and 6 patients with anti-DNA antibodies all had SLE. 64% had increased serum IgG, 24% IgA and 14% IgM. A significantly higher incidence of ANA was found within all three Ig classes in patients with secondary SS. AMA and IgM-rheumatoid factor were also found to be elevated in this group. On the other hand, SGA occurred most frequently in patients with primary SS. LMA was mainly of the IgG class and IgG AMA and SMA were more often present in these patients than in those with a negative LMA reaction. The results of the investigation suggest inter alia that inflammatory liver disease, although not indicated by either the case history, physical examination or biochemical values, is probably present in patients with SS.
The medical history was obtained and a physical examination carried out--in addition to an immunological investigation of the blood and skin--on 11 dogs in the ownership of patients with systemic lupus erythematosus (SLE). The results were compared with those obtained from a control group and it was found that they do not support the hypothesis that SLE is a zoonosis.
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Existing treatment for Sjögren's syndrome is unsatisfactory, and uncontrolled observations have suggested that bromhexine may be effective. Twenty-nine patients with Sjögren's syndrome were therefore assigned to two randomised double-blind crossover trials with bromhexine and placebo, each comprising two two-week periods. In the first trial bromhexine 24 mg/day was given by mouth; in the second the dose was increased to 48 mg/day. After each treatment period the Schirmer test response, break-up time, Bijsterveld score, and the time taken for the patient to eat a dry biscuit were recorded, as well as the patient's estimate of moistness in the eyes and mouth. In the second (higher-dose) trial values on the Schirmer test were significantly higher after bromhexine than after placebo and the break-up time was also increased after bromhexine, which suggested that the drug has a dose-dependent effect on lacrimal gland secretion in Sjögren's syndrome. It had no effect on salivary gland function. Bromhexine is therefore valuable in the treatment of Sjögren's syndrome.
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Anti-DNA antibody determination in serum is increasingly used because it supports the diagnosis of systemic lupus erythematosus (SLE) with high selectivity. The present work evaluates several of the technical variables of the Farr radioimmunoassay for anti-DNA antibody determination and describes a recommendable procedure, emphasizes the most important sources of error and gives the range of normal blood donors and a group of hospital patients without SLE.
A 73-year-old man who had suffered for many years from psoriasis was treated with systemic psoralen and longwave ultraviolet light. After 1 year he developed a preleukaemic condition characterized by a refractory anaemia, a slight thrombocytopenia and a normo- to hypercellular bone marrow with an excess of myeloblasts. Karyotyping revealed an abnormal chromosome (12p-) and agar cultures of bone marrow showed moderate growth with a high cluster/colony ratio. He died 1 year later of renal failure precipitated by an acute pancreatitis. Owing to the possible causal relationship we would like to advise increased attention to the haematopoietic system of patients treated with psoralen.
All pathological LE-cell tests and anti-DNA antibody determinations were registered in a prospective study over a period of 9 months at a department of internal medicine with rheumatology as a subspecialty. The results were compared with the clinical diagnosis of the patients, and correlated to SLE disease activity, as evaluated clinically and by blood analysis of five acute-phase reactants. Of 159 positive LE-cell tests, 43 were from patients with SLE, giving a diagnostic value of 27%. Of 37 positive anti-DNA antibody values, 30 were from patients having SLE, giving a diagnostic value of 81%. All of the 7 false-positive anti-DNA antibody determinations were only slightly above the normal range (22--26%: normal range less than or equal to 21%). No correlation was found between the activity of the SLE and the degree of the positive LE-cell test. Pathological levels of anti-DNA antibodies were always found when the patient had active SLE--clinically and by elevation of acute phase reactants. On the other hand, it was impossible to evaluate the disease activity from the level of the anti-DNA antibody test. It is concluded that the anti-DNA antibody determination should be preferred to the LE-cell test in the evaluation of patients with connective tissue diseases.
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A case of T-cell lymphoma occurred in a man, aged 66, whose symptoms started as itching and the appearance of 1-2 cm indurated plaques with central pustules on the skin of the trunk and face. A few months later generalized lymphomas and splenomegaly were present. Investigations of the lymphocytic subpopulations in situ in fresh frozen sections of lymphomas and skin showed only T cells. Samples of blood and bone marrow disclosed lymphocytosis and Sézary cells. Both T and B lymphocytes were definitely increased in the blood. The value of tissue studies in analyzing lymphocytic subpopulations is emphasized as a differential diagnostic aid in classifying lymphomas. A relationship between the T-cell neoplasia and the T and B lymphocytosis to mycosis fungoides and the Sézary syndrome is discussed.
A 41-year-old man developed intense itching without visible cutaneous changes, epigastric pressure pain, and a slight intolerance to alcohol. He was found to have persistent blood eosinophilia. The eosinophil granulocytes were of abnormal appearance in the light microscope: larger than normal, the nuclei were multilobulated (4-6 lobes), the cytoplasm contained atypical, large granules, ample glycogen, and up to 12 vacuoles. In the electron microscope too the eosinophil granules were entirely atypical, having an electron-dense matrix, often with a light central inclusion body which was inhomogeneous, having longitudinally oriented structures with a periodicity of about 10 nm. These findings are quite contrary to normal eosinophil granules. Enzymic studies of cytoplasmic enzymes from the granulocytes revealed a greatly reduced content of eosinophil cationic proteins, whereas 5 (7) other enzymes were present in a normal or slightly reduced quantity. The phagocytic capacity of the eosinophils against latex particles was normal. The patient developed generalized lymphomas, histologically very malignant, of the convoluted, acid phosphatase positive cell type (T-cell lymphoma). Sub-population studies of lymphocytes from a lymph node revealed 58% TE cells, while the remainder were B cells. At death, 3-1/2 years after the onset of symptoms, severe endomyocardial fibrosis was found. The thymus could not be identified. It is concluded that lymphomas should be described on the bais of clinical, histological, and histochemical criteria as well as studies of lymphocyte sub-populations and that the highly unusual eosinophil granulocytes still deserve particular attention. The endocardial fibrosis is assumed to have been due to substances liberated from the eosinophil cells.
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Male rabbits were injured by a single mechanical dilatation injury of aorta and then injected with prednisone 2 mg/kg or saline for 14 days or subjected to starvation. The biosynthesis of the sulfated glycosaminoglycans as evaluated by the uptake of 35S-sulfate and the content of the glycosaminoglycans were measured on the intima-media layer of the descending thoracic aorta. The results indicate that prednisone may inhibit the biosynthesis of heparan and/or dermatan sulfate while starvation increases the biosynthesis of all the sulfated glycosaminoglycans. No alterations were observed in the total amount of glycosaminoglycans in aorta following glucocorticoid injection or starvation. The metabolism of aortic glycosaminoglycans during repair is less sensitive to the action of prednisone than in undamaged aorta. This contrasts with the effect of prednisone on the metabolism of aortic collagen.
Thirteen patients, aged 27 to 70 years, with definite or classical rheumatoid arthritis were treated with penicillamine for six months. Skin biopsies and blood samples were compared with a clinical evaluation before and after therapy. The analyses of the skin included determinations of total collagen, thermal reaction of collagen, salt soluble collagen, in vitro uptake of 14C-proline and synthesis of 14C-hydroxyproline, as well as determinations of nucleic acids and proteoglycans. Serum concentrations of acute phase reactants, immunoglobulins, complement C3 and C4, rheumatoid factor, and iron, copper, and zinc were also determined. A positive correlation was found between clinical improvement and a fall in the number of granulocytes, a decrease in the concentration of acute phase reactants and serum copper, and an increase in salt soluble collagen of the skin. The total skin collagen decreased during treatment with penicillamine. The changes in skin collagen may reflect a generalized effect of penicillamine on collagen. These alterations may be part of an anti-inflammatory action of penicillamine.