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Purpose Although computed tomography (CT) has been used previously to assess disease severity in lymphangioleiomyomatosis (LAM), the associations between the extent of pulmonary cysts on CT and six-minute walk test (6MWT), matrix metalloproteinases (MMPs) and vascular endothelial growth factor (VEGF-D) are not well established. We performed a cross-sectional study to quantify the extent of pulmonary cysts in CT and to establish their correlations with pulmonary function tests (PFTs), 6MWT results, including a composite index (desaturation-distance ratio, DDR), and levels of VEGF-D and MMPs in LAM. Methods Twenty-three LAM patients underwent CT scanning to automatically quantify the extent of pulmonary cysts and performed PFTs and 6MWT. Serum levels of MMP-2, MMP-9, and VEGF-D were also measured. Results The severity of pulmonary cystic involvement was mild (the extent of cysts was 6.8 %) and correlated best with FEV.sub.1/FVC (r = -0.84), residual volume (r = 0.66), DL.sub.CO (r = -0.82), the DDR index (r = 0.77), and desaturation during the 6MWT (r = -0.81). There was a weak correlation with VEGF-D (r = 0.45), but no association was found with MMP-2 and MMP-9. Conclusions The severity of pulmonary cystic involvement was mild in this sample of LAM patients and correlated best with airway obstruction, air trapping, reduced DL.sub.CO, the DDR index, and desaturation during the 6MWT. Serum VEGF-D cannot be completely defined as a valuable marker of disease severity and there may be a mechanism independent of MMPs to explain the formation of pulmonary cysts. Electronic supplementary material The online version of this article (doi:10.1007/s00408-014-9641-2) contains supplementary material, which is available to authorized users., Author(s): Bruno Guedes Baldi[sup.1] , Mariana Sponholz Araujo[sup.1] , Carolina Salim Gonçalves Freitas[sup.1] , Gustavo Borges da Silva Teles[sup.2] , Ronaldo Adib Kairalla[sup.1] , Olivia Meira Dias[sup.1] , Daniel Antunes [...]

Purpose Although computed tomography (CT) has been used previously to assess disease severity in lymphangioleiomyomatosis (LAM), the associations between the extent of pulmonary cysts on CT and six-minute walk test (6MWT), matrix metalloproteinases (MMPs) and vascular endothelial growth factor (VEGF-D) are not well established. We performed a cross-sectional study to quantify the extent of pulmonary cysts in CT and to establish their correlations with pulmonary function tests (PFTs), 6MWT results, including a composite index (desaturation-distance ratio, DDR), and levels of VEGF-D and MMPs in LAM. Methods Twenty-three LAM patients underwent CT scanning to automatically quantify the extent of pulmonary cysts and performed PFTs and 6MWT. Serum levels of MMP-2, MMP-9, and VEGF-D were also measured. Results The severity of pulmonary cystic involvement was mild (the extent of cysts was 6.8%) and correlated best with [FEV.sub.1]/FVC (r = -0.84), residual volume (r = 0.66), [DL.sub.CO] (r = -0.82), the DDR index (r = 0.77), and desaturation during the 6MWT (r = -0.81). There was a weak correlation with VEGF-D (r = 0.45), but no association was found with MMP-2 and MMP-9. Conclusions The severity of pulmonary cystic involvement was mild in this sample of LAM patients and correlated best with airway obstruction, air trapping, reduced [DL.sub.CO], the DDR index, and desaturation during the 6MWT. Serum VEGF-D cannot be completely defined as a valuable marker of disease severity and there may be a mechanism independent of MMPs to explain the formation of pulmonary cysts. Keywords Computed tomography * Exercise * Lymphangioleiomyomatosis * Matrix metalloproteinase * Pulmonary function tests * Vascular endothelial growth factor, Introduction Lymphangioleiomyomatosis (LAM) is characterized by atypical muscle cell proliferation, which can lead to vascular and airway obstruction and formation of cysts. The extent of pulmonary involvement in LAM varies [...]

Heart disease (HD) can stress the alveolar blood-gas barrier, resulting in parenchymal inflammation and remodeling. Patients with HD may therefore display any of the symptoms commonly attributed to primary pulmonary disease, although tissue documentation of corresponding changes through surgical lung biopsy (SLB) is rarely done. Intent on exploring the basis of HD-related alveolar-capillary barrier dysfunction, a retrospective analysis of SLB histopathology was conducted in patients with clinically diagnosed HD, diffuse pulmonary infiltrates, and no evidence of primary pulmonary disease. Patients eligible for the study had a clinical diagnosis of heart disease, acute or chronic, and presented with diffuse infiltrates on chest X-ray. All qualified subjects (N = 23) who underwent diagnostic SLB between January 1982 and December 2005 were subsequently examined. Specific biopsy parameters investigated included demonstrable edema, siderophage influx, hemorrhage, venous and lymphatic ectasia, vascular sclerosis, capillary congestion, and fibroblast proliferation. Based on observed alveolar-capillary barrier (ACB) alterations, three main morphologic groups emerged: one group (6 patients) with alveolar edema; a second group (11 patients) characterized by pulmonary congestion; and a final group (6 patients) showing microscopic foci of acute ACB lung injury. Alveolar-capillary stress due to acute high-pressure or volume overload often manifests as diffuse pulmonary infiltrates with variable but generally predictable histopathology. In patients with biopsy-proven alveolar edema, pulmonary congestion, or acute microscopic lung injury, the clinician must be alert for the possibility of primary heart disease, particularly if the patient is elderly or when a history of myocardial, valvular, or coronary vascular disease exists. Keywords Heart disease * Pulmonary histological patterns * Pulmonary edema * Pulmonary congestion * Surgical lung biopsy * Microfoci of acute lung injury, Introduction Cardiac dysfunction typically involves one of the following mechanisms: blood loss, irregular heartbeat, obstructed flow, regurgitating flow, contractile dysfunction (systolic failure), or inadequate filling (diastolic failure) (1) . Any [...]

Abstract Epithelial remodeling probably contributes to parenchymal deterioration in usual interstitial pneumonia/idiopathic pulmonary fibrosis (UIP/IPF), but understanding its mechanisms is still a challenge. The aim of our study was to [...]

Abstract. Integrin-immunoglobulin family ligand (CAMs) interactions between lung parenchymal cells (fibroblasts and epithelial cells) and integrin-extracellular matrix component interactions may be involved in the pathogenesis of idiopathic interstitial pneumonia (IIP). [...]

Abstract. Recently, several reports suggest differences in the vascularization of the various histopathologic patterns of parenchymal remodeling seen in usual interstitial pneumonia (UIP). In this study, we sought to validate [...]