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.

Detection of myocardial inflammation by 18F-FDG-PET/CT in patients with systemic sclerosis without cardiac symptoms: a pilot study.

OBJECTIVES: Primary cardiac manifestation is a common complication of systemic sclerosis (SSc) with poor prognosis. The aim of the current study was to detect potential myocardial inflammation present in asymptomatic SSc patients by 18F-FDG-PET/CT and to investigate its relationship with early signs of myocardial dysfunction as detected by 2D speckle tracking echocardiography (2DSTE). METHODS: Sixteen consecutive patients with SSc and 9 control patients without clinical evidence of cardiac involvement were enrolled in the study. On 18F-FDG-PET acquired images blood-pool normalised SUV ratio and heterogenity index (HI: standard deviation of SUV divided with mean SUV) were calculated. Within 24 hours all SSc patients underwent 2DSTE strain analysis. RESULTS: Eight of 16 SSc patients were found to be visually PET-positive and showed significantly higher myocardial 18F-FDG SUV ratio (1.78±0.74 vs. 0.98±0.03; p<0.05) and heterogenity index (0.13±0.02 vs. 0.05±0.02; p<0.001) as compared to the control group. FDG-PET/CT derived values did not differ significantly between visually PET-negative (8/16) and control patients (SUV ratio: 0.98±0.05 vs. 0.98±0.03; HI: 0.05±0.01 vs. 0.05±0.02). Global left ventricular longitudinal strain values did not differ significantly between PET-positive and negative patients (17.18±3.49% vs. 17.59±3.65%). CONCLUSIONS: Myocardial inflammation, as a potential sign of early cardiac involvement can be detected by 18-FDG-PET/CT in a considerable percentage of systemic sclerosis patients presenting without cardiac symptoms.

18F-FDG-PET/CT in the evaluation and differential diagnosis of active large-vessel vasculitis. A prospective study. / [18F-FDG-PET/CT-vel szerzett tapasztalataink az aktív nagyérvasculitisek diagnosztikájában és differenciáldiagnózisában. Prospektív vizsgálat.]

Introduction: Large-vessel vasculitis has non-specific clinical symptoms, which can delay the diagnosis. Early recognition and treatment of the disease can help to avoid late complications. 18 F-FDG-PET can detect the inflammation of the vessel wall in the early stage of the disease with high sensitivity. CT is used to localize vasculitis. Aim: To examine the performance of 18F-FDG-PET/CT in patients with suspected large-vessel vasculitis, during relapse and remission, focusing on disease activity and extent. Method: 43 patients were evaluated. They were classified according to the clinical questions: steroid-naive suspected vasculitis, suspected vasculitis on steroid treatment, patients with relapse and in remission. We examined 10 cancer patients in control. We carried out visual and quantitative analysis of the 18F-FDG uptake of vessel walls. During quantitative evaluation, we determined standardised uptake values (SUVmax) of vessel wall segments compared to liver. Results: We found active disease in 5 patients examined for primary diagnosis, moreover, in 5 patients with relapse. The disease involved 3 or more vessel segments in fifty percent of the active cases. In the visually active group, the SUVmax was significantly lower in patients on steroid treatment than in steroid-naive cases (1.17 ± 0.11 vs. 1.43 ± 0.29; p = 0.005). We confirmed remission in 2 cases after therapy. In the inactive group, we found other types of inflammatory disorders in 8 cases. Conclusion: 18F-FDG-PET/CT is an effective diagnostic tool for large-vessel vasculitis, and can be used to determine the activity and extent of the disease. Steroid treatment influences the 18F-FDG-uptake of vessel wall. Orv Hetil. 2020; 161(20): 829-838.