[3 cases of obliterating bronchiolitis during treatment of rheumatoid polyarthritis with D-penicillamine]. / Trois observations de bronchiolite oblitérante lors du traitement de la polyarthrite rhumatoïde par la D-pénicillamine.

The authors report three cases of bronchiolitis obliterans occurring during treatment of rheumatoid arthritis with D-penicillamine after 4, 6 and 8 months of treatment and when the rheumatoid arthritis was in a state of remission. None of these patients (2 sero-positive and 1 sero-negative) present any past history of pulmonary or bronchial disease. The daily dosage of D-penicillamine never exceeded 600 mg. High dose corticosteroid therapy was unable to improve the clinical signs or the respiratory function tests, which were severely disturbed. After 15 months to 3 years, the respiratory signs deteriorated in 2 of the 3 patients. The onset of dyspnea and cough, in the absence of a seasonal infection, in a subject treated with D-penicillamine should raise the possibility of this very serious complication.

[How to evaluate the activity of Paget's disease in clinical practice and which patients should be treated?]. / Comment évaluer, en pratique, l'activite de la maladie de Paget et quels malades traiter?

In patients with Paget's disease, the alkaline phosphatase level (AP) is a measure of the phosphatase activity throughout the skeleton, i.e. the activity of normal bone tissue as well as that of Pagetic bone, in proportion to their respective volumes. This test therefore greatly under-estimates the activity of Pagetic bone in localized forms of the disease. To accurately evaluate the activity of Pagetic bone, the value of AP needs to be expressed in terms of the percentage of bone tissue affected by the disease. Two tables enable us to assess this percentage approximately. It is therefore possible to evaluate the activity of Pagetic bone on the basis of the tables presented by the authors. We can see that localized forms of Paget's disease may have an alkaline phosphatase activity of 2 000 or 3 000 I.U., while the AP levels in the blood are less than 200. So, in the presence of localized forms of the disease, we are unable to say that the disease is barely active on the basis of very low values for AP. It is important to treat localizations which have a "functional" risk for the future, especially when there localizations are highly active. The histopathological and scintigraphic data are compatible with areas of ery high activity. A similar rationale should be applied to the figures obtained for urinary hydroxyproline and the results of 45Ca kinetic studies.

[Treatment of Paget's disease with 1-ethane-1-hydroxy-1-diphosphonate]. / Le traitement de la maladie de Paget par l'éthane-1, hydroxy-1, 1-diphosphonate (EHDP).

The authors treated 11 patients suffering from severe and/or quickly developing Paget's disease (3 of whom had leontiasis ossea), using etidronate (E.H.D.P.) at doses of 7 to 15 mg.kg/day for a period of 6 months. The patients were followed up for 18 months, on average. The following were the conclusions of the investigation: E.H.D.P. is an effective treatment of Paget's disease: the decrease in hydroxyprolinuria was greater and more durable than that of the alcalin phosphatases; a variation between these two values even seems to appear under treatment. However, although some patients responded very well to the treatment, others were less responsive; at the dosages used, E.H.D.P. reduced the scanning hyperfixation of the active pagetic areas in 6 patients: it likewise more or less markedly reduced hyperosteoclastosis, with no apparent increase in the osteoid volume; two patients suffering from upper maxillary disease regressed clinically (dentures had become too large); headache and hypacusia were not modified; the response to the treatment seems to depend on three factors: the activity of the patient, the dosage used, and an individual factor which may be linked to the severity of the osteoidosis.