Medical treatment of chronic inflammatory rheumatic diseases in adults.
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Biomedical subjects
Publications and source records attributed to M F Kahn.
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Thirty-one unrelated patients, 15-52 years old, were typed by microlymphocytotoxicity for 27 alleles of the HLA system. In addition, 12 families including 1 or more patient were also analysed. This criteria for diagnosis were those of Sohar et all. (Am. Intern. Med., 1967, 43, 227-253). All patients were of Israelite-Sephardin origin except two (Armenian and French); they were from North-Africa (Tunisia, Morocco and Algeria) and Israël. The results were compared to the antigen frequencies of 3 reference normal populations. The frequencies of the studied alleles do not differ from those of controls, except for HL-A28 and B14 slightly increased when compared to the normal frequencies. The study of 7 families with at least two sibs suffering from FMF shows a random distribution of the genotypes : 2 HLA identical, 6 different and 10 haploidentical diseased sibs. This distribution differs significantly (p less than 0.01) from that expected in the case of a recessive inheritance. These data do not support the hypothesis of a linkage between genes controlling FMF and HLA genes.
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The authors present 4 patients who had suffered for several years from isolated systemic scleroderma and in whom, after 3, 10, 19 and 22 years, clinical, biological and histological signs of DLE appeared. There was no current further progression of the scleroderma in two cases, at the time of onset of DLE. In the oter two cases, it had disappeared. All four patients had a serum cryoglobulin. In all cases the kidneys were involved histologically : extra membranous glomerulitis in one, segmental glomerulitis in a second, and diffuse progressive glomerulitis in the last two. The authors analyse the biological changes which accompanied the clinical transformation. One of the patients had anti-ribonucleoprotein antibodies. She died from severe renal involvement which would be against the good prognosis usually associated with the presence of this type of antibody.
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Three methods of determination of anti-DNA antibodies were compared. The indirect immunofluorescence method after exposure to animal DNA (IF) and Farr's radio-immunological method with bacterial DNA labelled with C14, gave highly similar results, although there were some cases in which Farr's method gave a negative and the IF method a positive result. In part, this discordance appears to be accounted for by the variable affinity of the anti-DNA antibodies for DNA. The counter-immuno-electrophoresis method (CIEP) on the other hand, gave very different results: positive reactions in a large proportion of normal sera and a considerable proportion of false-positive and false-negative results in disseminated lupus erythematosus. It is concluded that until the CIEP method is made more reliable by technical refinement, determination of anti-DNA antibodies may be carried out by either the IF or the radio-immunological method, but a double-check, by application of the two methods at the same time, is highly advisable.
A study was made of the mean blood uric acid level in a group of patients with myeloma, assessed before treatment and in cases where renal insufficiency had been formally excluded. This mean uric acid level of the blood was 63.3 +/- 15 mg. per liter in a groupe of 16 men, and 59.5 +/- 16.5 mg. per liter in a group of 18 women. Comparison of these findings with those obtained in a number of control groups suggests that in myeloma without renal failure, mild hyperuricemia exists. No clear mention of this fact is found in previous publications. The moderate extent of this hyperuricemia is probably due to the fairly slow growth of the myelomatous tumor mass. It is suggested that accurate determinations of the variations of the uricemia and uraturia might yield exact data concerning the sensitivity of the myelomatous cells to the cytolytic agents used to combat them.
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In all branches of medicine, a large percentage of patients do not take the drugs which are prescribed for them. This percentage is situated at about 40%. The authors study this phenomenon in connection with data in the literature drawn from studies with objective controls. Rheumatology does not escape from this problem, whether one considers inflammatory rheumatism or degenerative rheumatism. The authors provide data from a personal series observed in arthrosic patients with an anti-inflammatory drug under trial. The various implications of this phenomenon are briefly discussed.
The possibility of healing of a corticosteroid-induced ulcer depsite the continuation of the treatment, is now an established fact. It is thus reasonable to continue corticosteroids in those cases where interupption severely compromises the prognosis. The chances of healing of the ulcer would appear to be great, especially when corticosteroids are being used in low dosage, but the exact frequency of healing remains unknown, just as the frequency of ulcers due to corticosteroid therapy has not been accurately established.
Deep cutaneous lupus is a clinical form of lupus disease for which a very old description has recently been brought up to date. Two new cases are reported here and a review of the literature makes it possible to analyse the clinical, biochemical, histological, and immunological features of this panniculitis. Different from the Weber-Christian syndrome, it is characterized by repeated eruptions of nodules and/or subcutaneous plaques, and histologically by vasculitis, lymphocyte infiltration, and sometimes te presence of immunoglobulins on the basal dermal membrane and around the vessels.
The authors have observed 3 cases of pure scleroderma and one case of scleroderma in which tendinous or subcutaneous nodular structures were present. To the author's knowledge, such nodules have never been described previously in cases of scleroderma. Their site and their histology identifies them as "rheumatoid" nodules and at the same time provides other valuable information. Scleroderma is thus, together with rheumatoid polyarthritis and lupus erythematosus, one of the possible causes of nodule formation, and the authors put forward the hypothesis that these nodules might represent one of the first lesions of the Thibierge and Weissenbach syndrome.
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