Sujal R. Desai

CARD15/NOD2 polymorphisms are associated with severe pulmonary sarcoidosis

Sarcoidosis and Crohn’s disease are heterogeneous systemic diseases characterised by granulomatous inflammation. Caspase recruitment domain (CARD)15 is a major susceptibility gene for Crohn’s disease, and specifically for ileal and fibrostenotic subtypes. The C-C chemokine receptor (CCR)5 gene has been associated with both parenchymal pulmonary sarcoidosis and perianal Crohn’s disease. This study explored associations between CARD15 polymorphisms, CCR5 haplotype and distinct pulmonary sarcoidosis subtypes. 185 Caucasian sarcoidosis patients were genotyped for CARD15 and CCR5 polymorphisms. The genetic data were compared with 347 healthy controls and were examined for associations with serial pulmonary function tests and chest radiographs. CARD15 genotypes did not differ between the unselected sarcoidosis cohort and controls. However, patients carrying the functional 2104T (702W) polymorphism were more likely to have radiographic stage IV disease at 4-yr follow-up. All patients possessing both CARD15 2104T and CCR5 HHC haplotype had stage IV disease at presentation. Carriage of 2104T was associated with worse forced expiratory volume in 1 s, whereas carriage of the CARD15 1761G (587R) polymorphism was associated with better lung function. For the first time, an association between two CARD15 polymorphisms and specific sarcoidosis phenotypes has been demonstrated, as well as an additive effect of possessing CARD15 2104T and CCR5 HHC haplotype.

Increased epithelial permeability in pulmonary fibrosis in relation to disease progression

Epithelial injury contributes to pathogenesis in idiopathic pulmonary fibrosis (IPF) but its role in the interstitial lung disease (ILD) of systemic sclerosis (SSc) is uncertain. We quantified the prognostic significance of inhaled technetium-99m (99mTc)-labelled diethylene triamine pentacetate (DTPA) pulmonary clearance, a marker of the extent of epithelial injury, in both diseases. Baseline 99mTc-DTPA pulmonary clearance was evaluated retrospectively in patients with SSc-ILD (n = 168) and IPF (n = 97) against mortality and disease progression. In SSc-ILD, the rapidity of total clearance (hazard ratio (HR) 1.02, 95% CI 1.01–1.03; p = 0.001) and the presence of abnormally rapid clearance (HR 2.10; 95% CI 1.25–3.53; p = 0.005) predicted a shorter time to forced vital capcity (FVC) decline, independent of disease severity. These associations were robust in both mild and severe disease. By contrast, in IPF, delayed clearance of the slow component, an expected consequence of honeycomb change, was an independent predictor of a shorter time to FVC decline (HR 1.01, 95% CI 1.00–1.02; p<0.01). Epithelial injury should be incorporated in pathogenetic models in SSc-ILD. By contrast, outcome is not linked to the overall extent of epithelial injury in IPF, apart from abnormalities ascribable to honeycombing, suggesting that core pathogenetic events may be more spatially focal in that disease.

Reduced-intensity conditioned allogeneic haematopoietic transplantation in an adult with Langerhans’ cell histiocytosis and thrombocytopenia with absent radii

Reduced-intensity conditioned allogeneic haematopoietic transplantation in an adult with Langerhans’ cell histiocytosis and thrombocytopenia with absent radii

Fibrosing Mediastinitis and Occlusion of Pulmonary Veins After Radiofrequency Ablation

Pulmonary vein stenosis is a known complication of radiofrequency ablation; its incidence ranges from 8% to 50%. However, complete occlusion of unilateral pulmonary veins is uncommon. We report a case with radiofrequency ablation that was complicated by complete occlusion of pulmonary veins along with fibrosing mediastinitis, which we believe has not been previously reported.

Pulmonary Imaging: Contributions to Key Clinical Questions

Pulmonary Imaging: Contributions to Key Clinical Questions is one of the few textbooks in which 11 of the 15 chapters are written by a team consisting of a radiologist and a clinician, in an interactive format. The main aim of the book is to describe the value of various imaging modalities in certain clinical scenarios, not only to clinicians but also to the other members of the clinical team (physician assistants, respiratory therapists, and nurses). In the last few decades there have been major advances in the way we image the chest. The change has been so rapid that even radiologists sometimes find it difficult to keep up with newer modalities. In such an atmosphere this book is a welcome aid to all the members of the clinical team, and will enable them to catch up with the advances and learn the way these newer modalities work, when to use them, and their strengths and weaknesses. The editors have taken care to address the utility in different clinical settings, which fulfills their primary goal. The book is divided into 3 sections. The first section is an introduction by a chest physician. It encourages the reader to pay closer attention to the evidence behind the use and utility of various modalities. The author explains using the example of highresolution computed tomography (CT), a widely used modality. The second section is further divided into 3 chapters, which ease the reader into the world of pulmonary imaging. Chapter 2 deals with basic principles of thoracic imaging and introduces the modalities in use today, and describes their clinically utility on a broad scale. Plain radiography, CT (including multidetector CT), magnetic resonance imaging, nuclear medicine, and other modalities are addressed. The author simplifies understanding of these modalities for non-radiologists and provides insight into their basic techniques and working principles. Chapter 3 describes pulmonary anatomy by imaging in a more comprehensive and easily understandable language. The appearance of different structures is shown with side-by-side images from various modalities, which I think will enhance readers’ understanding. Post-processing images that enhance anatomic points are also used, rendering images close to real-life pictures. The last chapter in this section deals with basic radiologic patterns of lung disease as seen inday-to-dayclinical radiologypractice.Detailed differential diagnosis for each of the described radiologic signs is provided, which will simplify learning the basic radiological interpretation of pulmonary diseases via plain radiography and CT. The final section addresses the commonly encounteredclinical radiologysettings.Each chapter gives in-depth analysis of one specific clinical situation from the point of view of the evidence that guides the utility of imaging modalities. A common format is used, with situation-specific modifiers. This unique format consists of the clinician author posing common radiologic questions that arise in a particular clinical scenario, and responses by the radiologist author. The responses are based on sound evidence and add strength to the overall practical applicability of this book, which provides “onestop shopping” for information for clinicians and radiologists alike. The more I read through this book, the more I am convinced that it would be of value both to the clinical team and radiologists. The chapter on lung cancer has an excellent discussion of the status of and current thinking on lung cancer screening via low-dose CT, the work-up of a lung nodule, and the imaging modalities for staging. The chapter on diffuse lung diseases has depth that will exceed most readers’ expectations. Modalities available, utility and accuracy of each modality, timing of surgical lung biopsy, interobserver variability, the accepted standards, quantification, prognostic information, and the role of monitoring disease progression are all addressed. The chapter on connective-tissue disorders elegantly combines clinical and radiologic analysis. The only difficulty I had in reading this book was with the small size of the images, which obscured detail in some of them. Overall this is a well-written textbook, useful for beginners and veterans in medicine and radiology alike.