Sarcoidosis Vasc Dif
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Sarcoidosis Vasc Dif · Mar 2013
ReviewSurgical lung biopsy for the diagnosis of interstitial lung disease: a review of the literature and recommendations for optimizing safety and efficacy.
Making an accurate diagnosis of a specific type of interstitial lung disease (ILD) requires a structured and comprehensive approach that includes a complete patient history, careful physical examination, appropriate laboratory testing, and thoracic imaging. If invasive procedures are required, bronchoscopy with bronchoalveolar lavage (BAL) and/or endoscopic lung biopsy (ELB) can frequently establish a confident diagnosis. However, surgical lung biopsy (SLB) may be required to make a confident diagnosis. ⋯ The overall 30-day mortality for open lung biopsy (OLB) was 4.3% versus 2.1% for video-assisted thorascopic surgery (VATS) biopsy, and non-lethal complications appeared to occur more frequently with OLB (18.1%) vs. VATS (9.6%) procedures. In addition to presenting the results of our comprehensive literature review on SLB for the diagnosis of ILD, we suggest an approach that minimizes risks to patients and optimizes the diagnostic utility of SLB when SLB must be performed to obtain a confident ILD diagnosis.
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Sarcoidosis Vasc Dif · Jun 2006
ReviewPast achievements and future directions of sarcoidosis research: a NHLBI perspective.
This history of research on sarcoidosis is largely from the perspective of the National Heart, Lung, and Blood Institute of the National Insititutes of Health which has had an interest in this disease since the inception of the Lung Program in 1969. ⋯ NHLBI has continued active support of research in sarcoidosis, both basic and clinical, such as the A Case Control Etiologic Study of Sarcoidosis (ACCESS) program, 1995-2003, whose conclusions are continuing to be published. A workshop on "Future Directions in Sarcoidosis Research" provided new research ideas to explore basic immunity mechanisms in human sarcoidosis tissue and search for latent microbial agents in tissue. The organization of sarcoidosis patient support groups has heightened awareness of the need for research on multiple organs affected by the disease in addition to the respiratory tract. In response, a trans-NIH sarcoidosis working group has been formed to assess this need and to better coordinate NIH research efforts.
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Sarcoidosis Vasc Dif · Dec 2005
Review Comparative StudyLung transplantation for rare pulmonary diseases.
Emphysema, idiopathic pulmonary fibrosis and cystic fibrosis are the major indications for lung transplantation. This article will present an overview of lung transplantation in the context of rare pulmonary diseases, in particular pulmonary sarcoidosis, lymphangioleiomyomatosis (LAM), and pulmonary Langerhans Cell Histiocytosis X (LCH). It will present criteria used in deciding when a patient should be referred and will discuss medical management in the context of lung transplantation.
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Sarcoidosis Vasc Dif · Dec 2005
Review Comparative StudyClinical and molecular insights into lymphangioleiomyomatosis.
Lymphangioleiomyomatosis (LAM) is a rare disease of women that is characterized by a proliferation of abnormal smooth muscle-like cells (LAM cells), which leads to cystic lung lesions, lymphatic abnormalities, and abdominal tumors (e.g., angiomyolipomas). LAM occurs sporadically or in association with tuberous sclerosis complex, an autosomal dominant syndrome characterized by hamartoma-like tumor growths. The tumor suppressor genes TSC1 and TSC2 have been implicated in the etiology of LAM, as mutations and loss of heterozygosity (LOH) in TSC2 have been detected in LAM cells. ⋯ In the proper clinical setting, LAM may be diagnosed by a thoraco-abdominal CT scan. Tissue biopsy with special stains (HMB-45) should be reserved for cases with atypical presentations. There is no effective treatment for LAM, but on-going therapeutic trials with rapamycin appear promising.
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Sarcoidosis Vasc Dif · Dec 2005
ReviewIdiopathic pulmonary hypertension: what did we learn from genes?
Pulmonary arterial hypertension (PAH) is an uncommon disorder. PAH can be idiopathic, associated with other conditions or clustered in families. Indeed, at least 6% of individuals diagnosed with so-called "primary" pulmonary hypertension have a family history of the disorder. ⋯ Analysis of other genes encoding TGF-beta receptor proteins, led to the demonstration that PAH in association with hereditary hemorrhagic telangiectasia, an autosomal dominant vascular dysplasia, can involve other TGF-beta receptor subtypes. These observations support the hypothesis that mutations in the TGF-beta superfamily may be a trigger for pulmonary vascular remodeling. Nevertheless, PAH pathobiology remains unclear and genomic approaches may identify additional molecular determinants for this disorder.