Evaluation of cardiopulmonary exercise test in the prediction of disease progression in systemic sclerosis.

OBJECTIVES: Cardiopulmonary exercise test (CPET) is a widely used examination to predict the prognosis of many chronic pulmonary diseases, and it has also been tested in systemic sclerosis (SSc) with a focus on the development of pulmonary hypertension. CPET is a highly informative non-invasive tool that provides a more complex information than conventional lung function tests to predict the course of cardiopulmonary diseases, as it provides a general overview of the aerobic metabolism, influenced by pulmonary, cardiovascular and peripheral muscle function. The purpose of this investigation was to assess if the progression and the development of poor overall disease outcome in SSc can be predicted by this method. METHODS: Twenty-nine SSc patients were investigated prospectively with standard follow-up plus CPET for a mean of 3.7 years to match the results of conventional evaluation modalities and CPET. A composite end-point of several serious outcomes reflecting SSc-related vascular and cardiopulmonary damage was set up, and the predictive value of and correlations between the CPET parameters and resting lung function and echocardiography variables were assessed. RESULTS: None of the clinical parameters, resting lung function or echocardiographic test results proved to be predictive of the development of the endpoint of poor prognosis in this cohort. In contrast, several CPET parameters were found to discriminate between SSc patients with or without adverse outcome. The detection of desaturation (at any CPET test) was associated with a higher risk of poor prognosis (OR:5.265). VO2 and VE/VCO2 at baseline correlated with the annual decrease in FVC, anaerobic threshold with the development of digital ulcers, and VE/VO2 with the increase in pulmonary arterial pressure. CONCLUSIONS: Several CPET parameters obtained at the beginning of follow-up are informative of the appearance of various adverse end-points. CPET is a feasible examination in the care of SSc patients and provides excess information to current standard follow-up examinations.

A Newly Developed Sublingual Tonometric Method for the Evaluation of Tissue Perfusion and Its Validation In Vitro and in Healthy Persons In Vivo and the Results of the Measurements in COPD Patients.

Introduction. Since its first publication in the medical literature, an extremely large number of references have demonstrated that the tonometric measurement of tissue perfusion is a reliable indicator of the actual condition of critically ill patients. Later a new method was developed by the introduction of sublingual tonometry for the determination of tissue perfusion. In comparison with gastric tonometry, the new method was simpler and could even be used in awake patients. Unfortunately, at present, because of severe failures of manufacturing, the device is withdrawn from commerce. Materials and Methods. In this study, we present a new method using a newly developed tool for the PslCO2 measurement in sublingual tonometry as well as the data for its validation in vitro and in vivo and the results of 25 volunteers and 54 COPD patients belonging to different GOLD groups at their hospitalization due to the acute exacerbation of the disease but already in a stable condition at the time of the examination. Results and Conclusion. The results of the performed examinations showed that the method is suitable for monitoring the actual condition of the patients by mucosal perfusion tonometry in the sublingual region.

[Primary pulmonary epitheloid haemangioendothelioma]. / Primer pulmonalis epitheloid haemangioendothelioma.

A 49-year-old female patient was admitted in July 2009 because of cough, weight loss and effort dyspnoe. Chest X-ray and CT showed multiple bilateral nodules which have been identified earlier and these nodules were unchanged. However, there was a new parenchymal lesion in the right upper lobe, and new right hilar and mediastinal lymphadenomegaly was also found. Sample was taken by bronchoscope and the pathological diagnosis was pulmonary epitheloid haemangioendothelioma. This rare endothelial tumor usually affects middle-aged patients with a female predominance and it presents with chest pain, effort dyspnoe, cough, sputum, or it may remain asymptomatic. Multiple bilateral nodules are usually detected by radiologic examination. The diagnosis of this tumor is often challenging and, because of its rarity, it does not have any standard therapeutic regimen. Treatment can be surgery, chemo-, radio-, hormone- or immunotherapy. In order to find the most effective anticancer treatment, authors performed in vitro studies. On the basis of the results, chemotherapy was initiated which resulted in a partial regression.