Central Role of CT in Management of Pulmonary Fibrosis.

With the approval of antifibrotic medications to treat patients with idiopathic pulmonary fibrosis and progressive pulmonary fibrosis, radiologists have an integral role in diagnosing these entities and guiding treatment decisions. CT features of early pulmonary fibrosis include irregular thickening of interlobular septa, pleura, and intralobular linear structures, with subsequent progression to reticular abnormality, traction bronchiectasis or bronchiolectasis, and honeycombing. CT patterns of fibrotic lung disease can often be reliably classified on the basis of the CT features and distribution of the condition. Accurate identification of usual interstitial pneumonia (UIP) or probable UIP patterns by radiologists can obviate the need for a tissue sample-based diagnosis. Other entities that can appear as a UIP pattern must be excluded in multidisciplinary discussion before a diagnosis of idiopathic pulmonary fibrosis is made. Although the imaging findings of nonspecific interstitial pneumonia and fibrotic hypersensitivity pneumonitis can overlap with those of a radiologic UIP pattern, these entities can often be distinguished by paying careful attention to the radiologic signs. Diagnostic challenges may include misdiagnosis of fibrotic lung disease due to pitfalls such as airspace enlargement with fibrosis, paraseptal emphysema, recurrent aspiration, and postinfectious fibrosis. The radiologist also plays an important role in identifying complications of pulmonary fibrosis-pulmonary hypertension, acute exacerbation, infection, and lung cancer in particular. In cases in which there is uncertainty regarding the clinical and radiologic diagnoses, surgical biopsy is recommended, and a multidisciplinary discussion among clinicians, radiologists, and pathologists can be used to address diagnosis and management strategies. This review is intended to help radiologists diagnose and manage pulmonary fibrosis more accurately, ultimately aiding in the clinical management of affected patients. ©RSNA, 2024 Supplemental material is available for this article.

Spontaneous pneumothorax in diffuse cystic lung diseases.

PURPOSE OF REVIEW: Diffuse cystic lung diseases (DCLDs) are a heterogeneous group of disorders with varying pathophysiologic mechanisms that are characterized by the presence of air-filled lung cysts. These cysts are prone to rupture, leading to the development of recurrent spontaneous pneumothoraces. In this article, we review the epidemiology, clinical features, and management DCLD-associated spontaneous pneumothorax, with a focus on lymphangioleiomyomatosis, Birt-Hogg-Dubé syndrome, and pulmonary Langerhans cell histiocytosis. RECENT FINDINGS: DCLDs are responsible for approximately 10% of apparent primary spontaneous pneumothoraces. Computed tomography screening for DCLDs (Birt-Hogg-Dubé syndrome, lymphangioleiomyomatosis, and pulmonary Langerhans cell histiocytosis) following the first spontaneous pneumothorax has recently been shown to be cost-effective and can help facilitate early diagnosis of the underlying disorders. Patients with DCLD-associated spontaneous pneumothorax have a very high rate of recurrence, and thus pleurodesis should be considered following the first episode of spontaneous pneumothorax in these patients, rather than waiting for a recurrent episode. Prior pleurodesis is not a contraindication to future lung transplant. SUMMARY: Although DCLDs are uncommon, spontaneous pneumothorax is often the sentinel event that provides an opportunity for diagnosis. By understanding the burden and implications of pneumothoraces in DCLDs, clinicians can facilitate early diagnosis and appropriate management of the underlying disorders.

Privacy-preserving screen capture: towards closing the loop for health IT usability.

As information technology permeates healthcare (particularly provider-facing systems), maximizing system effectiveness requires the ability to document and analyze tricky or troublesome usage scenarios. However, real-world health IT systems are typically replete with privacy-sensitive data regarding patients, diagnoses, clinicians, and EMR user interface details; instrumentation for screen capture (capturing and recording the scenario depicted on the screen) needs to respect these privacy constraints. Furthermore, real-world health IT systems are typically composed of modules from many sources, mission-critical and often closed-source; any instrumentation for screen capture can rely neither on access to structured output nor access to software internals. In this paper, we present a tool to help solve this problem: a system that combines keyboard video mouse (KVM) capture with automatic text redaction (and interactively selectable unredaction) to produce precise technical content that can enrich stakeholder communications and improve end-user influence on system evolution. KVM-based capture makes our system both application-independent and OS-independent because it eliminates software-interface dependencies on capture targets. Using a corpus of EMR screenshots, we present empirical measurements of redaction effectiveness and processing latency to demonstrate system performances. We discuss how these techniques can translate into instrumentation systems that improve real-world health IT deployments.

Inhibition of antiapoptotic BCL-2 proteins with ABT-263 induces fibroblast apoptosis, reversing persistent pulmonary fibrosis.

Patients with progressive fibrosing interstitial lung diseases (PF-ILDs) carry a poor prognosis and have limited therapeutic options. A hallmark feature is fibroblast resistance to apoptosis, leading to their persistence, accumulation, and excessive deposition of extracellular matrix. A complex balance of the B cell lymphoma 2 (BCL-2) protein family controlling the intrinsic pathway of apoptosis and fibroblast reliance on antiapoptotic proteins has been hypothesized to contribute to this resistant phenotype. Examination of lung tissue from patients with PF-ILD (idiopathic pulmonary fibrosis and silicosis) and mice with PF-ILD (repetitive bleomycin and silicosis) showed increased expression of antiapoptotic BCL-2 family members in α-smooth muscle actin-positive fibroblasts, suggesting that fibroblasts from fibrotic lungs may exhibit increased susceptibility to inhibition of antiapoptotic BCL-2 family members BCL-2, BCL-XL, and BCL-W with the BH3 mimetic ABT-263. We used 2 murine models of PF-ILD to test the efficacy of ABT-263 in reversing established persistent pulmonary fibrosis. Treatment with ABT-263 induced fibroblast apoptosis, decreased fibroblast numbers, and reduced lung collagen levels, radiographic disease, and histologically evident fibrosis. Our studies provide insight into how fibroblasts gain resistance to apoptosis and become sensitive to the therapeutic inhibition of antiapoptotic proteins. By targeting profibrotic fibroblasts, ABT-263 offers a promising therapeutic option for PF-ILDs.

The use of transbronchial cryobiopsy for diffuse parenchymal lung disease in critically ill patients with acute hypoxemic respiratory failure-A case series.

OBJECTIVES: Accurate diagnosis and management of undifferentiated diffuse parenchymal lung disease (DPLD) in critically ill patients is challenging. Transbronchial forceps biopsies have limited utility and surgical lung biopsies can be detrimental for critically ill patients. Transbronchial cryobiopsy (TBC) has shown increased diagnostic yield compared to conventional forceps biopsy in DPLD. However, TBC has not been studied in intensive care unit (ICU) patients. In this case series, we describe our experience with TBC for diagnosis of DPLD in ICU patients with acute hypoxemic respiratory failure. METHODS: This case series includes critically ill patients who underwent TBC at two different tertiary care hospitals. Procedures were performed by the same interventional pulmonologist using the two therapeutic bronchoscopes with a 2.8-mm working channel, and a 1.9- or 2.4-mm cryoprobe. RESULTS: We performed TBC in 17 patients of which 12 (70.1%) were performed at bedside in ICU without fluoroscopic guidance. Pathological diagnosis was made in 15 (88%) patients which resulted in changes in management in most of these patients. Six patients (35.3%) developed pneumothorax post-procedure with 5 (29.4%) requiring a chest tube. Moderate bleeding was noted in one (6%) patient and no severe or fatal bleeding occurred. Our 30-day ICU mortality was 47% (n = 8); however, no deaths were directly attributable to the procedure. CONCLUSIONS: TBC is a feasible technique with an acceptable complication rate and a fairly high histopathological yield in ICU patients with DPLD and acute hypoxemic respiratory failure. Appropriate diagnosis can be crucial in making management decisions for these patients.

Safety of performing transbronchial lung cryobiopsy on hospitalized patients with interstitial lung disease.

INTRODUCTION: Transbronchial lung cryobiopsy (TBLC) has become a popular option for tissue diagnosis of interstitial lung disease (ILD), however reports vary regarding the safety of this procedure. Herein, we evaluate the safety of transbronchial cryobiopsy in hospitalized patients, comparing adverse events to outpatient procedures. METHODS AND MEASUREMENTS: This is a single center, retrospective chart review of all TBLC performed for suspected ILD between November 2013 and March 2017. Biopsies were performed by a board certified interventional pulmonologist or interventional pulmonology fellow using a two-scope technique. RESULTS: One hundred fifty-nine cryobiopsies were performed for the diagnosis of ILD. Rates of adverse events are as follows: pneumothorax 11%, persistent air leak 1.3%, moderate-severe bleeding 3.8%, ICU transfer within 48 h 3.1%, and all cause 30-day mortality 1.9%. No deaths were attributed to the procedure. Comparing adverse events between hospitalized patients and outpatients, rates of pneumothorax were 24% vs 9.9%, persistent air leak 5.9% vs 0.7%, ICU transfer 12% vs 2.1%, and 30-day mortality 5.9% vs 1.4%. However, no differences were statistically significant. CONCLUSION: Practitioners should recognize that while cryobiopsies are a high-yield, safe, and cost-effective alternative to surgical lung biopsy, not all procedures carry the same risk profiles. Hospitalized patients may have a greater propensity for pneumothorax, persistent air leak, transfer to the ICU, and 30-day mortality.

Effects of resveratrol treatment on bone and cartilage in obese diabetic mice.

BACKGROUND: Resveratrol is a polyphenolic phytoalexin that has been shown to exhibit osteoprotective and chondroprotective properties. We examine the effects of resveratrol treatment on bone and cartilage tissue of obese, diabetic ob/ob mice. METHODS: Eight-week-old ob/ob and lean control mice were given trans-resveratrol at an oral dose of 25 mg/kg for 3 weeks. Histomorphometric and cross-sectional-geometric variables were analyzed. RESULTS: Ob/ob mice in our study exhibit significantly reduced femoral length, resistance to loading, and tibial growth plate total area and calcified area than lean controls (P < 0.05). Resveratrol treatment significantly increased cortical area in both ob/ob and control mice, but did not improve cross-sectional indicators of resistance to bending. Resveratrol treatment also reduced tibial length and calcified growth plate cartilage area in comparison to untreated mice (P < 0.05). CONCLUSION: Resveratrol treatment of ob/ob mice had mixed effects on bone histomorphometry at the femoral midshaft. Treatment increased cortical area but decreased bone length.