[Anatomo-clinical conference. Pitié-Salpêtrière Hospital. Case no. 3--1993. Febrile myalgia and hypereosinophilia in a 59-year-old woman].
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Biomedical subjects
Publications and source records attributed to T Papo.
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Interferon alpha (INF-alpha)--In systemic diseases, most indications for INF-alpha result from its effect on haematological or hepatological manifestations. The spectacular effect of INF-alpha in chronic myeloid leukemia has led to its use for the treatment of hypereosinophilia syndrome and systemic mastocytosis. Over the last 6 years, we have treated 7 patients with the hypereosinophilia syndrome who were resistant to corticotherapy and had markers of myeloproliferation. Although both hydroxyurea and INF-alpha can be effective alone, their combination led to a decrease in the eosinophilia count to 1,000/ml, a decrease which was long-lasting in most cases. INF-alpha is also used in histiocytosis X alone or in combination with retinoids or with etoposide and has been found effective in several observations. In carcinoid syndromes whether treated priorly or not with a 5-fluoro-uracil-streptozoticin combination, INF-alpha leads to an objective response in two-thirds of the patients. Several multicentric protocols are currently assessing the efficacity of INF-alpha in mixed cryoglobulinaemias. In most observations these cryoglobulinaemias are seen in patients with markers of hepatitis C (mainly HCV) and the early results are encouraging. Temporary improvement has been reported in discoid or subacute lupus in 8 out of 10 cases. Haemangiomas of the infant, when life-threatening and corticoresistant, may be a good indication for INF-alpha. Thus 20 newborns or infants (including 4 with Kasabach-Merrit syndrome) have been treated with good results in 18. Interferon gamma (INF-gamma).(ABSTRACT TRUNCATED AT 250 WORDS)
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We describe a case of mouth and genital ulcers with inflamed cartilage (nasal and ear chondritis) (MAGIC syndrome). Our case was complicated by repeated aortic prosthetic valve dehiscence and femoral false aneurysm. The condition was resolved by treatment with corticosteroids and azathioprine.
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A 40-yr-old woman known for a multinodular goiter had hyperthyroidism. Treatment with antithyroid drugs and iodine therapy was effective. One year later, she received interferon-alpha for treatment of essential cryoglobulinemia. At that time, the patient was euthyroid. Testing for antithyroglobulin, antimicrosome, anti-TSH receptor, and antithyroid hormone antibodies was negative. After a 1-yr course of interferon-alpha, goiter enlargement was noticed. Apparently elevated free T3 and T4 serum values were measured by RIA, contrasting with clinical euthyroidism and normal TSH values. High serum levels of antithyroid hormone antibodies were found in the patient's serum, using a radiolabeled hormone immunoprecipitation assay. Antithyroglobulin and antimicrosome antibodies titers were also elevated and paralleled antithyroid hormone antibodies. After cessation of interferon-alpha therapy, clinical status and TSH levels remained normal, while thyroid hormone values and antithyroid hormone antibody levels progressively normalized. To our knowledge, this is the first report of antithyroid hormone antibodies induced by interferon-alpha. Since thyroid dysfunction is described in 10-15% of treated patients, the fact that interferon-alpha can induce antithyroid hormone antibodies has important implications: 1) the prevalence or intensity of thyroid dysfunction could be overestimated; and 2) artifactually elevated free T3 and T4 serum values could lead to inappropriate therapy of thyroid disease or cessation of interferon treatment.
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Emphysematous pyelonephritis is a rare condition which in most cases occurs as a result of Escherichia coli infection of the urinary tract in patients known as being diabetic. The gas-filled abscesses carry a high mortality rate, especially when the antibiotic treatment is not combined with early surgery. We report a case where the failure of medical treatment led to curative nephrectomy. Unusually, diabetes was revealed by the pyelonephritis. Another particularity of this case was that the emphysematous pyelonephritis was associated with genitourinary tuberculosis. This association, of which no other example could be found in the literature, is discussed.
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Antigenic peptides are presented to T lymphocytes by major histocompatibility complex (MHC) molecules. The binding of peptides to MHC class II molecules has been demonstrated directly, and is found to correlate with the ability of specific class II alleles to restrict the T-cell response to specific peptides. By comparison, a direct demonstration of a physical association between antigenic peptides and MHC class I molecules has proved difficult. A recent report shows that it is possible, however, and the three-dimensional structure of a class I MHC molecule illustrates the site where such binding must occur. Here we describe a simple assay which measures the binding of radiolabelled MHC class I molecules to peptides bound to a solid phase support. We find that class I molecules bind specifically to peptides known to be antigenic for class I-restricted cytotoxic T lymphocytes. Peptides which are recognized by cytotoxic T lymphocytes bind not only to the restricting MHC class I molecule but also to other class I molecules. Our results suggest that quantitative differences in the peptide/MHC class I interaction may influence the-pattern of MHC restriction observed in vivo.
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Two main types of antineutrophil cytoplasmic antibodies (ANCA) have been recognized by indirect immunofluorescence. ANCA-D are defined by diffuse fluorescence and are directed against proteinase 3. They are highly specific for Wegener's granulomatosis, and their titre varies with disease activity. ANCA-P are responsible for perinuclear fluorescence and are principally found in other types of vasculitis and in idiopathic crescentic glomerulonephritis. In patients with suggestive clinical signs, ANCA are of considerable diagnostic value when specific histology is difficult to obtain. Therapeutic monitoring and prevention of Wegener's disease relapses could rely on regular measurements of ANCA titres. Moreover, studies on the pathogenetic role of ANCA may transform current physiopathological concepts and nosology of vasculitis.