Sarcoidosis Vasc Dif
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Sarcoidosis Vasc Dif · Oct 2011
Case ReportsSuccessful pregnancy complicated by persistent pneumothorax in a patient with lymphangioleiomyomatosis (LAM) on sirolimus.
We report a successful pregnancy in a patient with longstanding LAM on treatment with sirolimus. During temporary discontinuation fo sirolimus in early pregnancy, lung function declined but recovered after resumption of sirolimus. Pregnancy was complicated by a persistent pneumothorax which was treated surgically postnatally. The child has had a normal development despite exposure to low dose sirolimus intermittently during early embryonal and mid-fetal life.
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Sarcoidosis Vasc Dif · Oct 2011
Prognostic factors for idiopathic pulmonary fibrosis: clinical, physiologic, pathologic, and molecular aspects.
Previous studies identified clinical and physiologic factors of idiopathic pulmonary fibrosis (IPF) that are related to an increased risk of mortality. But there are few studies about histologic and molecular approach. ⋯ This study confirmed the amount of smoking, abrupt decrease in follow-up pulmonary function parameters, fibroblastic foci, and increased levels of CRP concentration at diagnosis were significantly associated with poor survival. Larger studies are required to confirm all prognostic factors including CRP.
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Fluor-18 fluorodeoxyglucose (18F-FDG) PET is able to demonstrate sarcoidosis activity. Ongoing pulmonary sarcoidosis activity can be reflected by a decline in pulmonary function tests (PFT). To assess whether diffuse metabolic activity of the lung parenchyma imaged by 18F-FDG PET predicts future pulmonary deterioration, 18F-FDG PET was compared with PFT. ⋯ In sarcoidosis, diffuse parenchymal disease imaged by 18F-FDG PET, predicts a future deterioration of DLCO when untreated. Treatment however, improves VC, FEV1 and DLCO significantly suggesting that 18F-FDG PET represents the pulmonary improvement that can be achieved. The absence of metabolic activity in the lung parenchyma justifies a wait-and-see policy.
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Sarcoidosis Vasc Dif · Jul 2011
Case ReportsRapid improvement of osseous sarcoidosis after the treatment of pulmonary aspergillosis by itraconazole.
Osseous sarcoidosis is relatively uncommon, and treatment with corticosteroids is not always effective. Moreover, patients with an advanced stage of pulmonary sarcoidosis are sometimes infected with aspergillus in the cavities of the pulmonary lesions, and long-term use of corticosteroids should be prohibited to prevent the development of fatal invasive pulmonary aspergillosis. Here, we described a unique case of osseous sarcoidosis with pulmonary aspergillosis, showing a rapid improvement of the osseous symptoms just after the administration of the antifungal agent, itraconazole. Itraconazole is likely to become a candidate among new therapeutic agents for osseous sarcoidosis.
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Sarcoidosis Vasc Dif · Jul 2010
C-reactive protein predicts response to infliximab in patients with chronic sarcoidosis.
This study assessed the value of C-reactive protein as a predictor of disease severity and response to infliximab therapy in patients with chronic pulmonary sarcoidosis. ⋯ In chronic sarcoidosis patients, elevated CRP appears to identify a subset with more severe disease who may respond better to treatment with infliximab.