Behaviour of von Willebrand factor antigen in follow-up of polymyalgia rheumatica/giant cell arteritis.
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Biomedical subjects
Publications and source records attributed to M L Ciompi.
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Forty-four patients with typical palindromic rheumatism were typed for HLA-A,B,C and DR antigens. The mean duration of follow-up of our patients was 4.0 yrs. (range 1-12 yrs.). During this time 27% of patients showed a seropositivity; 22% of patients developed polyarthritis, 1 patient revealed a Sjögren syndrome and 3 patients showed a spontaneous improvement. The HLA-B16 antigen was increased in patients compared with controls, but the difference was not statistically significant. A, C and other B antigens were found with normal frequencies. The HLA-DR antigens did not reveal any differences between patients' and controls' values, either 40% of patients who developed RA carried the HLA DR4 antigen.
Tigason (etretinate, RO-10-9359) is an oral aromatic retinoid acid which is effective in psoriasis and other dermatological syndromes. The present study reports the results of the use of this drug in 15 patients with a confirmed diagnosis of psoriatic arthritis, aged between 42 and 74 (average 60.2) years. Two patients dropped out after one month because they showed dryness of lips, chapping and intolerable itching, and another two due to intolerable pruritus and deterioration of their skin lesions. The 11 remaining patients, five males and six females, were treated with a daily dose of 50 mg etretinate, reduced to 25 mg after two weeks. Assessments which included grip strength, Ritchie joint index, pain, and dermatological assessment were performed at monthly intervals. Routine blood tests for toxic effects and erythrocyte sedimentation rate (ESR) were also performed, as well as CPR (C-protein reactive) as a test of disease activity. All patients showed an improvement in the psoriasis, joint swelling and pain. The elevated ESR observed in all patients studied also fell gradually during the course of etretinate treatment, as did as the intake of antiinflammatory agents. Elevated serum lipid levels (cholesterol and triglycerides) were found in three patients; further drug-specific side-effects such as dryness of lips and mouth, loss of hair, thinning of skin and scaling were frequently found in all patients, but they remained tolerable.
In order to verify whether measurement of plasma fibronectin (Fn) could represent a useful tool in acute-phase-response assessment, Fn was measured in 16 previously untreated patients (group A) affected by polymyalgia rheumatica giant cell arteritis (PMR-GCA), both before, during, and after 45 days of steroid therapy, and its course was compared with the behavior of some acute-phase reactants such as erythrocyte sedimentation rate (ESR), fibrinogen (Fng), and prealbumin (Preal). No difference was detected between the baseline Fn levels found in patients and those registered in a control group composed of 15 sex- and age-matched healthy subjects; no correlation was found with the other acute-phase parameters considered, and no significant variation of plasma Fn levels was registered as a result of the steroid therapy administered. On the contrary, all the other parameters revealed a good degree of correlation and tended progressively and homogeneously towards normalization as a result of the therapy administered. Plasma Fn was also measured in another group of 16 PMR-GCA patients (group B), all of whom had pathological retinal fluoroangiographic findings, and its levels were compared with those of the von Willebrand factor antigen (vWfAg), a biochemical index of vascular damage. While the levels of Fn continued to be the same as those detected in the control group, the values of vWfAg registered in group B proved to be significantly different from those found in another homogeneous control group of 25 healthy subjects. Finally, no correlation could be detected between Fn and vWfAg, and neither of them showed any significant correlation with the ESR.(ABSTRACT TRUNCATED AT 250 WORDS)
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The case of a 37 year old man with a longstanding HLA-B27 associated bilateral Achilles tendinitis without seronegative spondyloarthropathy is reported. This case suggests that heel enthesopathy may for a long time be the only clinical manifestation of the HLA-B27 associated disease process.
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A case of erosive sterile arthritis following meningococcal meningitis is described. High levels of immune complexes were detected in serum and synovial fluid. This is the first case in literature in which destruction of subchondral bone is documented. The erosion showed a progressive remineralization in the six months following clinical recovery.
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Lymphocyte responsiveness to various mitogens was studied in patients with palindromic rheumatism. The results show an enhanced lymphocyte reactivity to T-dependent mitogens during the first few days after an acute attack of the disease.
Two patients with coexistent Down's syndrome and gout are described. Although increased serum urate levels are frequently reported in Down's syndrome, only a few such patients have been described with concomitant gout. In our patients no significant alterations of the purine salvage pathway were found, and the turnover parameters of uric acid, determined by means of a 14C-labeled uric acid study, were consistent with the metabolic findings observed in normoexcretor gouty patients.
Fifty-seven patients took part in a controlled double-blind trial between tiopronin and D-penicillamine as basic treatment for rheumatoid arthritis. Thirty-nine (19 receiving tiopronin, 20 receiving D-penicillamine) completed the trial after 1 year. Both drugs resulted in a decrease of the erythrocyte sedimentation rate, Ritchie index, and Lee index and in a sparing effect on symptomatic antiinflammatory therapy. Improvement in these variables was statistically highly significant at any interval with tiopronin, but was sometimes less or not at all significant with D-penicillamine. Nevertheless, the difference in effects between the 2 drugs never reached statistical significance. Six patients receiving tiopronin and 6 receiving D-penicillamine were taken out of the experiment because of side effects.
A study of patients with rheumatoid arthritis permitted us to note the interest of tiopronine (sulfhydryl compound of the d-penicillamine type) in the basic treatment of rheumatoid arthritis. The dose of the drug was on average, 1 gram daily, which, in one series of patients, was administered at the start of treatment and in another series, with an increase of dosage of 250 mg every 20 days. The results, judged by the reduction of the Ritchie and Lee index, of the E.R.S. and the steroid and non-steroid anti-inflammatory requirements, are good (very good and good) in 47% of cases. These patients were in an advanced stage of the disease and there had been numerous therapeutic failures. Side effects similar to those observed during treatment with D-penicillamine were present in 38% of cases, and in 31% required stopping treatment. It is likely that the frequency of stopping treatment for side effects was definitely exaggerated. The place of this new drug in the basic treatment of rheumatoid arthritis requires further study by a double blind test with D-penicillamine, and this is at present in progress.
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