Emma C. Ferguson

Skin Gene Expression Correlates of Severity of Interstitial Lung Disease in Systemic Sclerosis

OBJECTIVE We undertook this hypothesis-generating study to identify skin transcripts correlating with severity of interstitial lung disease (ILD) in systemic sclerosis (SSc). METHODS Skin biopsy samples from 59 patients enrolled in the Genetics versus Environment in Scleroderma Outcome Study (GENISOS) cohort or an open-label imatinib study (baseline visit) were examined by global gene expression analysis using Illumina HT-12 arrays. Skin transcripts correlating with concomitantly obtained forced vital capacity (FVC) values and the modified Rodnan skin thickness score (MRSS) were identified by quantitative trait analysis. Also, immunofluorescence staining for selected transcripts was performed in affected skin and lung tissue. Plasma levels of CCL2, soluble SELP, and soluble P-selectin glycoprotein ligand 1 (sPSGL-1) were examined in all patients enrolled in the GENISOS cohort (n = 266). RESULTS Eighty-two skin transcripts correlated significantly with FVC. This gene list distinguished patients with more severe ILD (FVC <70% predicted) in unsupervised hierarchical clustering analysis (P < 0.001). These genes included SELP, CCL2, and matrix metalloproteinase 3, which are involved in extravasation and adhesion of inflammatory cells. Among the FVC correlates, 8 genes (CCL2, HAPLN3, GPR4, ADCYAP1, WARS, CDC25B, PLP1, and STXBP6) also correlated with the MRSS. Immunofluorescence staining revealed that SELP and CCL2 were also overexpressed in affected skin and lung tissue from SSc patients compared to those from controls. Plasma levels of CCL2 and sPSGL-1 correlated with concomitantly obtained FVC values (r = -0.22, P = 0.001 and r = 0.17, P = 0.015, respectively). This relationship was independent of potential confounders (age, sex, ethnicity, smoking status, anti-topoisomerase I positivity, treatment with immunosuppressive agents, MRSS, disease type, and disease duration). CONCLUSION A limited number of skin transcripts including genes involved in extravasation and adhesion of inflammatory cells correlate with severity of ILD.

Lung CT: Part 2, The Interstitial Pneumonias???Clinical, Histologic, and CT Manifestations

OBJECTIVE The interstitial pneumonias are a group of heterogeneous nonneoplastic lung diseases that may be idiopathic or associated with an underlying abnormality. Although they share some features in common, they also exhibit diverse pulmonary manifestations. Imaging plays an essential role in characterizing this group of disorders and can often suggest the diagnosis, though the final interpretation requires a coordinated effort involving the radiologist, pathologist, and clinician. The purpose of this article is to review the imaging features of the interstitial pneumonias according to their histologic patterns and to provide a brief overview of their clinical presentations. CONCLUSION This article reviews the interstitial pneumonias according to their histologic subtypes, including both idiopathic and secondary forms. On completion, the reader should have an improved understanding of the classification of the interstitial pneumonias, associated causes, characteristic imaging features, histologic descriptions, clinical manifestations, and prognoses.

Resident Education in the Radiological Sciences: What Now?

OBJECTIVE With the dizzying changes in the rapidly evolving profession of radiology, the structure of resident education in the associated sciences of imaging, physics, radiobiology, and radiation effects must be reevaluated continually. What roles do these basic radiologic sciences play in bolstering the neophyte radiologist on a career of patient care? How should we define the spectrum of material that should be learned? How should that spectrum be taught? Who decides these things? With the impending changes in the radiology board certification process, questions have been raised as to how these changes will affect education in a residency program. Should the basic science curriculum be enhanced or scaled back? With the emphasis on practical applied physics, what is considered old school and what is new school material? CONCLUSION This article describes one approach adopted by a large residency program to address these issues.

Abnormalities in the Cardiophrenic Space

Various types of lesions may occur in the cardiophrenic space, which is located in the most inferior aspect of the anterior mediastinum and is bordered by the heart, diaphragm, and chest wall. Abnormalities detected at radiography and CT often are due to prominent pericardial fat pads, fat-containing tumors such as lipoma, pericardial cysts, enlarged lymph nodes, diaphragmatic hernias, or anterior mediastinal masses, such as thymoma or lymphoma. Although chest radiography can hint at the correct diagnosis, such as a hernia if gas is visible within intestinal loops or a pericardial cyst if an ovoid structure is sharply demarcated and homogeneous, the imaging characteristics of these lesions often can overlap. Both benign and malignant lesions can have a similar appearance. Thus CT is valuable in the diagnosis and differentiation of cardiophrenic space lesions. This article illustrates and reviews the imaging findings and differential diagnoses of lesions that may occur in the cardiophrenic space.

Perforation of the Intrathoracic Esophagus: Common and Unusual Causes

Perforation of the intrathoracic esophagus is a life-threatening condition, in which the mediastinum may become rapidly contaminated along with other nearby vital structures, leading to shock and ultimately death. A rapid and accurate diagnosis is crucial, especially because signs and symptoms are often nonspecific and may mimic other disorders. The most common cause is iatrogenic because of instrumentation of the esophagus. The esophagus also may perforate spontaneously, from trauma arising internal or external to the esophagus, and from operative injury and tumors, both intrinsic and extrinsic. Complications such as fistula, abscess, and pneumonia can result. Plain chest films usually suggest the diagnosis. Contrast esophagography is the standard of diagnosis, often and usually aided by CT scanning. The goal of the diagnostic radiologist is to recommend the appropriate imaging study and effectively diagnose this condition so that treatment may be started within 24 hours of the inciting event, after which time mortality rate significantly increases. The information provided in this article will improve the diagnostic radiologists ability to provide an early accurate diagnosis of intrathoracic esophageal perforation, which in turn leads to early, often-life-saving treatment.

CT Imaging Manifestations of Cryptogenic Organizing Pneumonia

Cryptogenic organizing pneumonia (COP) (known until 2002 as bronchiolitis obliterans organizing pneumonia [BOOP]) has diverse imaging manifestations on CT, some of which are characteristic and quite specific for cryptogenic organizing pneumonia, whereas others are less distinctive and may mimic other diseases. The CT imaging findings include multiple peripheral opacities, focal pulmonary involvement, small or large nodules or masses, peribronchovascular opacities, ground-glass or patchy alveolar opacities, perilobular and reticular opacities, the crazy-paving pattern, and crescentic and ring shapes. Recognizing the various CT imaging characteristics of organizing pneumonia is valuable when trying to differentiate it from other benign or even malignant diseases that it may simulate. This article illustrates and reviews the numerous CT imaging findings and patterns that may be seen in cryptogenic organizing pneumonia.

CT Imaging of Thoracic Scleroderma

Because thoracic involvement is common in patients with scleroderma, CT and high-resolution CT (HRCT) of the chest routinely are performed to examine these patients. In the lungs alone, there are numerous imaging manifestations of scleroderma, such as ground-glass opacities, consolidation, reticulation, nodules, bronchiectasis, and honeycombing. Recognizing and understanding the pulmonary findings of scleroderma on CT is important, because these findings may indicate the level of disease involvement and dictate whether or not treatment is indicated. Scleroderma is a multisystemic disease, so imaging manifestations may extend beyond the lungs into the chest to involve the pleura, pulmonary arteries, lymph nodes, esophagus, and musculoskeletal system. Inspection of these other organ systems should become part of the radiologists routine search pattern.