Cystic lung disease is not uncommon in men with tuberous sclerosis complex.

Objective: To assess the frequency of cystic lung disease suggestive of pulmonary lymphangiomyomatosis in men with tuberous sclerosis complex.

Patients And Methods: Retrospective review of CT scans of the chest and abdomen on 29 men with tuberous sclerosis complex encountered during a 13-year period, 1998 to 2010.

Results: Cystic lung disease (defined as ≥4 cysts) was seen in 11 of 29 men (38%) with tuberous sclerosis complex.

Spectrum of Disorders Associated with Elevated Serum IgG4 Levels Encountered in Clinical Practice.

IgG4-related disease (IgG4-RD) is a recently described systemic fibroinflammatory disease associated with elevated circulating levels of IgG4 and manifests a wide spectrum of clinical presentations. Although serum IgG4 level has been described to be the most sensitive and specific laboratory test for the diagnosis of IgG4-RD, it is recognized that an elevated serum IgG4 level can be encountered in other diseases. In this study, we sought to identify the frequency of IgG4-RD and other disease associations in patients with elevated serum IgG4 levels seen in clinical practice.

Histopathologic Overlap between Fibrosing Mediastinitis and IgG4-Related Disease.

Fibrosing mediastinitis (FM) and IgG4-related disease (IgG4-RD) are two fibroinflammatory disorders with potentially overlapping clinical and radiological features. In this paper, we looked for histopathologic features of IgG4-RD and enumerated infiltrating IgG4-positive plasma cells within mediastinal tissue biopsies from FM patients. We identified 15 consecutive FM surgical mediastinal tissue biopsies between 1985 and 2006.

Apocynin attenuates lipopolysaccharide-induced lung injury in an isolated and perfused rat lung model.

Apocynin (Apo) suppresses the generation of reactive oxygen species that are implicated in lipopolysaccharide (LPS)-induced lung injury (LPSLI). We thus hypothesized that Apo may attenuate LPSLI. In addition, we explored the cellular and molecular mechanisms of Apo treatment in LPSLI.

A multidimensional index and staging system for idiopathic pulmonary fibrosis.

Background: Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic lung disease with an overall poor prognosis. A simple-to-use staging system for IPF may improve prognostication, help guide management, and facilitate research.

Objective: To develop a multidimensional prognostic staging system for IPF by using commonly measured clinical and physiologic variables.

Increased IgG4-Positive Plasma Cells in Granulomatosis with Polyangiitis: A Diagnostic Pitfall of IgG4-Related Disease.

Granulomatosis with polyangiitis (Wegener's) (GPA) may mimic IgG4-related disease (IgG4-RD) on histologic examination of some biopsies, especially those from head and neck sites. IgG4 immunostain is often performed in this context for differential diagnosis with IgG4-RD. However, the prevalence of IgG4+ cells in GPA has not been explored.

Relative versus absolute change in forced vital capacity in idiopathic pulmonary fibrosis.

Background: Decline in forced vital capacity (FVC) over time reliably predicts mortality in patients with idiopathic pulmonary fibrosis. The use of this measure in clinical practice is recommended by current evidence-based guidelines. It is unknown if the method of calculating decline in FVC (relative vs.

Risk factors for the development of acute lung injury in patients with infectious pneumonia.

Introduction: Although pneumonia has been identified as the single most common risk factor for acute lung injury (ALI), we have a limited knowledge as to why ALI develops in some patients with pneumonia and not in others. The objective of this study was to determine frequency, risk factors, and outcome of ALI in patients with infectious pneumonia.

Methods: A retrospective cohort study of adult patients with microbiologically positive pneumonia, hospitalized at two Mayo Clinic Rochester hospitals between January 1, 2005, and December 31, 2007.

Smoking-related interstitial lung diseases.

Cigarette smoke, a toxic collection of thousands of chemicals generated from combustion of tobacco, is recognized as the primary causative agent of certain diffuse interstitial and bronchiolar lung diseases. Most patients afflicted with these disorders are cigarette smokers, and smoking cessation has been shown to be capable of inducing disease remission and should occupy a pivotal role in the management of all smokers with these diffuse lung diseases. The role of pharmacotherapy with corticosteroids or other immunomodulating agents is not well established but may be considered in patients with progressive forms of smoking-related interstitial lung diseases.

N-acetylcysteine attenuates ventilator-induced lung injury in an isolated and perfused rat lung model.

N-acetylcysteine (NAC) suppresses the generation of reactive oxygen species (ROS) that are implicated in ventilator-induced lung injury (VILI). We thus hypothesised that NAC attenuates VILI. VILI was induced by mechanical ventilation with a tidal volume (Vt) of 15mlkg(-1) in isolated and perfused rat lung.

Are respiratory complications common causes of death in inflammatory myopathies? An autopsy study.

Background And Objective: Polymyositis (PM) and dermatomyositis (DM) are idiopathic inflammatory myopathies that are associated with a variety of clinical manifestations including pulmonary complications. The objective of the present study was to determine the causes of deaths in this complex patient population.

Methods: A computer-assisted search of medical and autopsy records identified a total of 39 patients with either PM or DM who underwent an autopsy at the Mayo Clinic (Rochester, MN, USA) over a 29-year period from 1 January 1981 to 31 December 2009.

Frequency of undiagnosed cystic lung disease in patients with sporadic renal angiomyolipomas.

Objective: The aim of this study was to assess the frequency of undiagnosed cystic lung lesions suggestive of pulmonary lymphangioleiomyomatosis (LAM) in patients who received a diagnosis of sporadic renal angiomyolipomas (AMLs).

Methods: We conducted a retrospective review of CT scans of the chest or abdomen for cystic lung lesions on 176 adult patients who received a diagnosis of sporadic renal AML during a 10-year period, 1997 to 2006, and comparison with chest CT scans of 176 control subjects without renal AML but matched for age, sex, and smoking history. Patients presenting with suspected or known pulmonary LAM and those with underlying tuberous sclerosis were excluded.

Gastroesophageal reflux therapy is associated with longer survival in patients with idiopathic pulmonary fibrosis.

Rationale: Gastroesophageal reflux (GER) is highly prevalent in patients with idiopathic pulmonary fibrosis (IPF). Chronic microaspiration secondary to GER may play a role in the pathogenesis and natural history of IPF.

Objectives: To investigate the relationship between GER-related variables and survival time in patients with IPF.

An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management.

This document is an international evidence-based guideline on the diagnosis and management of idiopathic pulmonary fibrosis, and is a collaborative effort of the American Thoracic Society, the European Respiratory Society, the Japanese Respiratory Society, and the Latin American Thoracic Association. It represents the current state of knowledge regarding idiopathic pulmonary fibrosis (IPF), and contains sections on definition and epidemiology, risk factors, diagnosis, natural history, staging and prognosis, treatment, and monitoring disease course. For the diagnosis and treatment sections, pragmatic GRADE evidence-based methodology was applied in a question-based format.

Improving the safety of thoracentesis.

Purpose Of Review: Thoracentesis is a common bedside procedure associated with iatrogenic complications including pneumothorax. Experienced clinicians using optimal procedural techniques within a supportive system can achieve improvements in safety. However, clinicians have been relatively slow to adopt these changes.

Pneumothorax and bullae in Marfan syndrome.

Background: Increased risk of spontaneous pneumothorax has been described in patients with Marfan syndrome and has been attributed, in part, to the presence of apical blebs and bullae.

Objectives: We assess the risk of pneumothorax and its relationship to the presence of apical blebs and bullae in patients with Marfan syndrome in the era of CT imaging.

Methods: A retrospective cohort study was performed of all patients 13 years or older with Marfan syndrome evaluated at the Mayo Clinic, Rochester, Minn.

Update on uncommon pleural effusions.

Although infections, malignancies and heart failure are responsible for the majority of pleural effusions, there are many other causes and several uncommon but distinctive types of pleural fluid. For this update we have chosen several uncommon forms of pleural effusions or disorders in which there have been recent advances in our understanding over the past several years. Chylothorax, pseudochylothorax and urinothorax are associated with characteristic clinical contexts and pleural fluid parameters but are likely underdiagnosed.

Lung involvement in hypereosinophilic syndromes.

Background: Hypereosinophilic syndromes (HES) are a heterogeneous group of conditions that are characterized by tissue-associated eosinophilic inflammation and peripheral eosinophilia. Although clinical and radiologic features associated with most forms of eosinophilic lung diseases are relatively well-described, there is little known regarding lung involvement in HES. The aims of the present study were to ascertain the frequency of pulmonary involvement in HES and define associated clinical and radiologic features.

Advances in the management of idiopathic pulmonary fibrosis.

Idiopathic pulmonary fibrosis (IPF) is a common form of interstitial lung disease and usually results in progressive respiratory insufficiency and death. Steady progress has been made in understanding the pathogenesis of IPF and multiple clinical trials are ongoing, but effective therapy remains elusive.

Iron deposition and increased alveolar septal capillary density in nonfibrotic lung tissue are associated with pulmonary hypertension in idiopathic pulmonary fibrosis.

Background: Early diagnosis of pulmonary hypertension (PH) in idiopathic pulmonary fibrosis (IPF) has potential prognostic and therapeutic implications but can be difficult due to the lack of specific clinical manifestations or accurate non-invasive tests. Histopathologic parameters correlating with PH in IPF are also not known. Remodeling of postcapillary pulmonary vessels has been reported in the nonfibrotic areas of explanted lungs from IPF patients.

The International LAM Registry: a component of an innovative web-based clinician, researcher, and patient-driven rare disease research platform.

Background: A relative inability to capture a sufficiently large patient population in any one geographic location has traditionally limited research into rare diseases.

Methods And Results: Clinicians interested in the rare disease lymphangioleiomyomatosis (LAM) have worked with the LAM Treatment Alliance, the MIT Media Lab, and Clozure Associates to cooperate in the design of a state-of-the-art data coordination platform that can be used for clinical trials and other research focused on the global LAM patient population. This platform is a component of a set of web-based resources, including a patient self-report data portal, aimed at accelerating research in rare diseases in a rigorous fashion.