Zeitschrift für Rheumatologie
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Being progressive and also involving variations in the severity of symptoms, chronic rheumatic diseases present difficulties with respect to documentation. Since dramatic changes in the course of disease are rare, early detection of very slight changes is essential to successful antirheumatic therapy. Practical means of documentation are necessary if an overview of the various clinical findings and subjectively reported symptoms is to be maintained over a period of many years. ⋯ After several modifications, a modularly structured documentation system is now available, elements of which can be expanded according to the needs of the individual therapeutic study. The form can both constitute a part of the patient history and act as a link between different but parallel-running studies, thus making it unnecessary to record the same information twice for different purposes. At the same time, the data base can be used to answer scientific and epidemiological questions independent of the study in progress.
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The clinical history and autopsy findings are reported of a 23 year old woman who had symptoms of mixed connective tissue disease (MCTD) for 4 years, and who died suddenly from pulmonary hypertension due to proliferative endarteritis of the lung vessels. These arterial lesions resembled those in progressive systemic sclerosis. Children and adolescents with MCTD appear to have an increased risk for getting this complication. The fatal outcome of pulmonary hypertension in MCTD as shown in this and other cases seems to warrant treatment with immunosuppressive drugs.