PubMed · 13324939
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J H HEJTMANCIK, M A MAGID. Bilateral periureteritis plastica.. https://pubmed.ncbi.nlm.nih.gov/13324939/
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BACKGROUND AND PURPOSE: Patient related co-factors may increase the risk of radiation morbidity. Connective tissue diseases (CTD) are among the co-morbidities that are relatively well studied and have been shown to be of potential clinical relevance for radiotherapy. The aim of this systematic review is to quantify the contribution of CTD to the risk of radiation related side effects. PATIENTS AND METHODS: Relevant publications reporting the outcome of radiotherapy in patients with CTD were retrieved according to a standardized query and evaluated for their methodology and quality of reporting, using defined quality criteria. A quantitative estimate of the relative risk of developing side effects in patients with CTD was derived from the observed proportions of responders in patients with or without CTD. Risk estimates were synthesized across studies. RESULTS: Eight studies which include data of 9-209 patients who had CTD and were treated with radiotherapy were identified and reviewed. Three of these studies included data suited for further quantitative analysis. The test of the uni-directional hypothesis that patients with CTD had an increased risk of late effects after radiotherapy reached statistical significance, the one-tailed P-value being 0.03. The pooled relative risk was 2.0 with 95% confidence interval (0.99, 4.1). CONCLUSIONS: CTD is associated with an increased risk of late radiation induced normal tissue reaction. The literature published to date provides some support for this assertion although most of the reports are plagued by methodological weaknesses, thus calling for a large coordinated study.
The number of publications in the field of pulmonary hypertension has increased exponentially over the past 10 years. As a consequence our understanding of the condition has advanced enormously. Pulmonary hypertension is now recognised as an uncommon and treatable disease, rather than a rare lethal disease. Progress in the field of connective tissue disease associated pulmonary hypertension has been less consistent. This paper covers the most influential published data on connective tissue associated pulmonary hypertension, and adds some insights from data available on file with pharmaceutical companies and from our database at the Royal Free Hospital. The following conclusions may be drawn. First, the prevalence of pulmonary hypertension associated with connective tissue diseases is lower than generally accepted. Second, the natural history and prognosis, at least in the setting of systemic sclerosis, is worse than seen in patients with idiopathic pulmonary hypertension. Finally, widespread and timely availability of disease modifying therapies substantially improves the prognosis for patients with systemic sclerosis associated pulmonary hypertension, in the treatment population and consequentially in the population as a whole. Identifying patients with very early disease and analysing the drivers for disease progression, is the key to further progress. Early diagnosis is most readily achieved in patients with connective tissue disease associated pulmonary hypertension. This represents an opportunity for those with an interest in academic rheumatology.