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Three patients were hospitalised during treatment for congestive heart failure. Four patients showed improvement of their functional class and three patients in exercise capacity after 12 months of therapy.
Macitentan was well tolerated in five out of six cases with severe pulmonary sarcoidosis and PH. Functional capacity improved in four cases. Prospective controlled trials are warranted before therapeutic recommendations can be made.
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Macitentan was well tolerated in five out of six cases with severe pulmonary sarcoidosis and PH. Functional capacity improved in four cases. Prospective controlled trials are warranted before therapeutic recommendations can be made. (Sarcoidosis Vasc Diffuse Lung Dis 2020; 37 (1) 74-78).
Sarcoidosis is a multisystem and granulomatous disease associated with impaired functional capacity as a result of pulmonary and cardiac involvement. Factors adversely effecting functional capacity in patients with sarcoidosis have not been systematically assessed including myocardial strain imaging on echocardiography which enable to diagnose subclinical cardiac dysfunction. We aimed to evaluate the effect of left and right ventricular global longitudinal strain (GLS) on submaximal exercise capacity in patients with sarcoidosis who do not have clinically manifest cardiac involvement.
Extracardiac biopsy proven 56 patients with sarcoidosis and 26 controls were included consecutively. Submaximal exercise capacity of the subjects was assessed with six-minute walk test (6 MWT). Pulmonary function tests and standard transthoracic and two-dimensional speckle tracking echocardiography were performed to the all subjects. Linear regression analysis was performed to find independent predictors of 6 MWT.
Fifty-si 63-73).
Sarcoidosis typically presents with peribronchovascular and perilymphatic nodules on high-resolution computed tomography (HRCT); a miliary pattern is reported but not well described.
We describe four patients with miliary sarcoidosis and results of a systematic review of all previously reported cases from 1985 onwards.
We identified only 27 cases of "miliary" sarcoidosis in the HRCT era. These patients were older (85.2% older than 40 years), had more co-morbidities (72.7%) and were symptomatic compared to "typical" sarcoidosis. Respiratory symptoms were present in 61.9% at diagnosis. Hypercalcemia was seen in 28.5%. On review of HRCT images, only 34.6% (9/26) had a "true miliary" pattern without fissural nodules. STA-4783 mouse In our series, prominent perivascular granulomas were seen on histopathology in all. 44.4% (12/27) had tuberculosis preceding or concurrent to miliary sarcoidosis. Of the eight true associations, tuberculosis preceded sarcoidosis by 52 (median, IQR 36) weeks in six and occurred concurrently in ary" sarcoidosis may follow treatment for tuberculosis; concurrent cases possibly indicate the difficulty in differentiating both or a "tuberculo-sarcoid" presentation. (Sarcoidosis Vasc Diffuse Lung Dis 2020; 37 (1) 53-65).
Sarcoidosis is a rare, chronic systemic disease. Earlier data (2006-2010) suggest that the incidence of pulmonary sarcoidosis in Silesian voivodeship increased, however there is no current data on other clinical forms of the disease.
The aim of presented study was an analysis of the actual epidemiological situation of sarcoidosis with simultaneous estimation of treatment cost financed from public funds.
Epidemiological descriptive study concerned registered cases of sarcoidosis diagnosed in adult inhabitants of the Silesian voivodeship in years 2011-2015. Secondary epidemiological data on the main diagnosis and co-morbidity were obtained from the National Health Fund (NFZ) database in Katowice. Territorial and temporal variability of standardized incidence rates were analysed with simultaneous estimation of treatment costs reimbursed from the state budget.
Pulmonary sarcoidosis was the most frequently registered clinical form of such disease in the Silesian voivodeship (65% of total cases). The highest number of cases was diagnosed in the age 35-54 years, frequently in men than in women. Significantly decrease of the standardized incidence of sarcoidosis noticed between 2011 and 2015 is related with observed lower number of total cases of pulmonary disease. Observed territorial variability of the sarcoidosis incidence requires future, well-planned studies. The annual average direct cost of sarcoidosis treatment is high and exceed 538 EUR per patient.
It was confirmed that sarcoidosis in the Silesian Voivodeship is a rare disease, however reimbursed direct costs of treatment remains very high.
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It was confirmed that sarcoidosis in the Silesian Voivodeship is a rare disease, however reimbursed direct costs of treatment remains very high. (Sarcoidosis Vasc Diffuse Lung Dis 2020; 37 (1) 43-52).
To describe the clinical and serological patients characteristics with Microscopic Polyangiitis (MPA) and Interstitial lung disease (ILD).
Of all the patients with AAV diagnosed between 2007-2017 at the Hospital Clinico Universidad de Chile, those with MPA and ILD were selected and studied retrospectively.
All patients were Hispanic; median age at diagnosis 65 years (32-84). 59% were female. All were positive for p-ANCA, 16 patients for MPO. Most common manifestations were constitutional symptoms, weight loss and fever. CT-Scans patterns were Usual Interstitial Pneumonia (UIP) in 10 patients, Nonspecific Interstitial Pneumonia (NSIP) in 6 and fibrosis not UIP or NSIP pattern in 1. In 6 cases, ILD was diagnosed 0.5-14 years before MPA and concomitantly in 11.
Although infrequent, Microscopic Polyangiitis should be suspected in patients with ILD particularly if extra-pulmonary manifestations that rise the possibility of a systemic illness are present, regardless of the time elapsed between the latter and the diagnosis of this type of lung involvement.
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Although infrequent, Microscopic Polyangiitis should be suspected in patients with ILD particularly if extra-pulmonary manifestations that rise the possibility of a systemic illness are present, regardless of the time elapsed between the latter and the diagnosis of this type of lung involvement. (Sarcoidosis Vasc Diffuse Lung Dis 2020; 37 (1) 37-42).
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