The role for endobronchial biopsy in the era of endobronchial ultrasound guided transbronchial needle aspiration for the diagnosis of sarcoidosis: a single center experience.

BACKGROUND AND AIM: Endobronchial biopsy (EBBX) has been reported to increase diagnostic yield for pulmonary sarcoidosis. The purpose of this study is to investigate the diagnostic yield for EBBX following endobronchial ultrasound guided transbronchial needle aspiration (EBUS-TBNA). METHODS: We identified a cohort of patients in the University of Minnesota Sarcoidosis Registry who had EBBx and EBUS-TBNA as part of workup for abnormal chest imaging. Data regarding demographics, biopsy approach and technique were recorded. RESULTS: Our cohort included 37 patients (53.24±9.5, Male, 22±0.57; 3.8% were African American). In these patients who had EBBX, EBUS-TBNA was performed in 100% of patients and TBBX was performed in 2 patients (5%). EBBX was positive in 9 patients (24%) and EBUS-TBNA was positive in 34 patients (92%). TBBX was diagnostic in one of two patients. EBBX was the only diagnostic tissue in 3 of the 37 patients (8%).  Conclusion: The diagnostic yield of EBBX is lower than previously reported, with only 8% of EBBXs demonstrating granulomatous inflammation. However, instrumentation used for obtaining EBBX as well as the presence of visible lesions does influence the diagnostic yield. Studies with adequate power are needed before implementing changes in clinical practice.  When performed alongside EBUS-TBNA, EBBX did not significantly add to the diagnostic yield in sarcoidosis unless visible lesions were observed.

Pulmonary transplant complications: a radiologic review.

Lung transplantation has become the definitive treatment for end stage respiratory disease. Numbers and survival rates have increased over the past decade, with transplant recipients living longer and with greater comorbidities, resulting in greater complexity of care. Common and uncommon complications that occur in the immediate, early, intermediate, and late periods can have significant impact on the course of the transplant. Fortunately, advancements in surgery, medical care, and imaging as well as other diagnostics work to prevent, identify, and manage complications that would otherwise have a negative impact on survivability. This review will focus on contextualizing complications both categorically and chronologically, with highlights of specific imaging and clinical features in order to inform both radiologists and clinicians involved in post-transplant care.

A novel, evidence-based, comprehensive clinical decision support system improves outcomes for patients with traumatic rib fractures.

BACKGROUND: Traumatic rib fractures are associated with high morbidity and mortality. Clinical decision support systems (CDSS) have been shown to improve adherence to evidence-based (EB) practice and improve clinical outcomes. The objective of this study was to investigate if a rib fracture CDSS reduced hospital length of stay (LOS), 90-day and 1-year mortality, unplanned ICU transfer, and the need for mechanical ventilation. The independent association of two process measures, an admission EB order set and a pain-inspiratory-cough score early warning system, with LOS were investigated. METHODS: The CDSS was scaled across nine US trauma centers. Following multiple imputation, multivariable regression models were fit to evaluate the association of the CDSS on primary and secondary outcomes. As a sensitivity analysis, propensity score matching was also performed to confirm regression findings. RESULTS: Overall, 3,279 patients met inclusion criteria. Rates of EB practices increased following implementation. On risk-adjusted analysis, in-hospital LOS preintervention versus postintervention was unchanged (incidence rate ratio [IRR], 1.06; 95% confidence interval [CI], 0.97-1.15, p = 0.2) but unplanned transfer to the ICU was reduced (odds ratio, 0.28; 95% CI, 0.09-0.84, p = 0.024), as was 1-year mortality (hazard ratio, 0.6; 95% CI, 0.4-0.89, p = 0.01). Provider utilization of the admission order bundle was 45.3%. Utilization was associated with significantly reduced LOS (IRR, 0.87; 95% CI, 0.77-0.98; p = 0.019). The early warning system triggered on 34.4% of patients; however, was not associated with a significant reduction in hospital LOS (IRR, 0.76; 95% CI, 0.55-1.06; p = 0.1). CONCLUSION: A novel, user-centered, comprehensive CDSS improves adherence to EB practice and is associated with a significant reduction in unplanned ICU admissions and possibly mortality, but not hospital LOS. LEVEL OF EVIDENCE: Therapeutic/Care Management; Level III.

Granulomatous-Lymphocytic Interstitial Lung Disease Mimicking Sarcoidosis.

Common variable immunodeficiency (CVID) is one of the most common primary immunodeficiency disorders characterized by hypogammaglobulinemia and inadequate antibody response to immunizations. The impaired antibody response occurs due to the failure of B cells to differentiate into plasma cells resulting in low immunoglobulins levels and increased frequency of infections. Granulomatous and Lymphocytic Interstitial Lung Disease (GLILD) is a non-infectious complication of CVID that is seen in 10-30% of cases. GLILD is a multisystem inflammatory disease involving the lungs, lymph node, liver, spleen and gastrointestinal tract that mimics sarcoidosis. This report describes a series of cases who presented with dyspnea, recurrent respiratory infections or autoimmunity and on further evaluation revealed features suggestive of GLILD. There is very limited understanding of GLILD in terms of clinical presentation, the histo-pathological logical findings, and the diagnostic criteria by itself are limited. A diagnosis of GLILD is established in cases of CVID when there is evidence of lymphoproliferation, cytopenia, autoimmune processes and a lung biopsy demonstrating lymphocytic interstitial pneumonia, follicular bronchiolitis, lymphoid hyperplasia, and/or non-necrotizing granulomas. We review the treatment strategies, including replacement of immunoglobulin and agents targeting B and T lymphocytes. Systematic characterization of GLILD cases and long term follow up studies are sorely needed to understand the natural history of GLILD.

Impact of 2016 SCCT/STR guidelines for coronary artery calcium scoring of noncardiac chest CT scans on lung cancer screening CT reporting.

The 2016 SCCT/STR guideline for coronary artery calcification (CAC) scoring on non-cardiac chest CT (NCCT) scans explicitly calls for the reporting of CAC. Whether the publication of the 2016 SCCT/STR guideline has had any impact on CAC reporting in lung cancer screening (LCS) scans has not been investigated. Consecutive patients with a LCS scan were identified from the University of Minnesota LCS registry and evaluated for CAC reporting in 3 separate cohorts: 6 months before, 6 months after, and 1 year after the publication of the 2016 SCCT/STR guideline. Scans were evaluated for CAC and quantified using the Agatston method. CAC reporting, downstream testing and initiation of preventive therapy were assessed. Among 614 patients (50% male, mean age 64.1 ± 6.0 years), CAC was present in 460 (74.9%) with a median Agatston score of 62 (IQR 0, 230). Of these, 196 (31.9%) had a CAC score of 1-100, 125 (20.4%) had 101-300, and 118 (19.2%) had > 300. Overall, CAC was reported in 325 (70.7%) patients with CAC present. CAC reporting relative to publication of the 2016 SCCT/STR guideline was as follows: 6 months prior-74.1%, 6 months after-64.6%, and 1 year after-77.5%. In the 308 patients with a new diagnosis of sub-clinical CAD based on CAC presence, 6 (1.9%) patients were referred to cardiology, and 15 (4.9%) patients underwent testing for obstructive CAD. Only 6 (1.9%) and 9 (2.9%) patients were newly started on aspirin and statin respectively. CAC detected incidentally on lung cancer screening CT scans is prevalent, and rarely acted upon clinically. CAC reporting is fairly high, and publication of the 2016 SCCT/STR guideline for CAC scoring on NCCT scans did not have any significant impact on CAC reporting.

Clinical characteristics and organ system involvement in sarcoidosis: comparison of the University of Minnesota Cohort with other cohorts.

BACKGROUND: Sarcoidosis is a systemic granulomatous disease of unknown etiology. Clinical cohort studies of different populations are important to understand the high variability in clinical presentation and disease course of sarcoidosis. The aim of the study is to evaluate clinical characteristics, including organ involvement, pulmonary function tests, and laboratory parameters, in a sarcoidosis cohort at the University of Minnesota. We compare the organ system involvement of this cohort with other available cohorts. METHODS: We conducted a retrospective data collection and analysis of 187 subjects with biopsy-proven sarcoidosis seen at a tertiary center. Organ system involvement was determined using the WASOG sarcoidosis organ assessment instrument. Clinical phenotype groups were classified using the Genomic Research in Alpha-1 Antitrypsin Deficiency and Sarcoidosis criteria. RESULTS: Mean subject age at diagnosis was 45.8 ± 12.4, with a higher proportion of males (55.1%), and a higher proportion of blacks (17.1%) compared to the racial distribution of Minnesota residents (5.95%). The majority (71.1%) of subjects required anti-inflammatory therapy for at least 1 month. Compared to the A Case Control Etiologic Study of Sarcoidosis cohort, there was a higher frequency of extra-thoracic lymph node (34.2% vs. 15.2%), eye (20.9% vs. 11.8%), liver (17.6% vs. 11.5%), spleen (20.9% vs. 6.7%), musculoskeletal (9.6% vs. 0.5%), and cardiac (10.7% vs. 2.3%) involvement in our cohort. A multisystem disease with at least five different organs involved was identified in 13.4% of subjects. A restrictive physiological pattern was observed in 21.6% of subjects, followed by an obstructive pattern in 17.3% and mixed obstructive and restrictive pattern in 2.2%. Almost half (49.2%) were Scadding stages II/III. Commonly employed disease activity markers, including soluble interleukin-2 receptor and angiotensin-converting enzyme, did not differ between treated and untreated groups. CONCLUSIONS: This cohort features a relatively high frequency of high-risk sarcoidosis phenotypes including cardiac and multiorgan disease. Commonly-utilized serum biomarkers do not identify subpopulations that require or do better with treatment. Findings from this study further highlight the high-variability nature of sarcoidosis and the need for a more reliable biomarker to predict and measure disease severity and outcomes for better clinical management of sarcoidosis patients.

Pulmonary vein occlusion and veno-occlusive disease in a bilateral lung transplant patient: A case report.

A pulmonary vein occlusion and biopsy proven pulmonary veno-occlusive disease (PVOD) and hemangiomatosis is found in a bilateral lung transplant patient. A 61-year-old male presents with dyspnea and chest pain with minimal exertion at routine follow up on post-transplant day of 50. Chest CT demonstrates new occlusion of bilateral superior pulmonary veins and diffuse pulmonary edema. Pulmonary vein occlusion is confirmed by trans-esophageal echocardiogram, and PVOD and hemangiomatosis is corroborated with lung biopsy. Normal pulmonary capillary wedge pressure (PCWP) and reduced DLCO are also consistent with PVOD. Vigilant evaluation of large pulmonary venous thrombus is as important as of arterial thrombus in a postsurgical transplant status. A dedicated protocol of pulmonary venous phase scan would be beneficial to identify subtle pulmonary venous abnormalities. Although PVOD/PCH is normally considered in patients with nonspecific PAH symptoms, lacking of direct manifestation of PAH should not dismiss the diagnosis of PVOD/PCH, particularly in lung transplant individuals with large pulmonary vein occlusion, progressive respiratory symptoms, DLCO abnormalities, and pulmonary congestion since it may represent a wide spectrum of occlusive vascular disease.

Acute exacerbation of interstitial lung disease after procedures.

Interstitial lung disease (ILD) is a category of diffuse parenchymal lung diseases characterized by inflammation and/or fibrosis. The best characterized ILD is idiopathic pulmonary fibrosis (IPF). Acute exacerbation of IPF is a dreaded occurrence with grim prognosis and suboptimal treatment options. There have been recent reports that acute exacerbation can occur in other ILDs (AE-ILD). Of note, some of these acute exacerbations follow lung procedures. This review summarizes the available information on AE-ILD and discusses the procedures reported to cause AE-ILD. We also discuss proposed mechanisms, risk factors, treatment and prognosis. This review should help to inform decision-making about risks versus benefits of procedures that are commonly recommended to diagnose ILD.

Crohn's disease-associated interstitial lung disease mimicking sarcoidosis: a case report and review of the literature.

Respiratory involvement in Crohn's disease (CD) is a rare manifestation known to involve the large and small airways, lung parenchyma, and pleura. The clinical presentation is nonspecific, and diagnostic tests can mimic other pulmonary diseases, posing a diagnostic challenge and delay in treatment. We report a case of a 60-year-old female with a history of CD and psoriatic arthritis who presented with dyspnea, fever, and cough with abnormal radiological findings. Diagnostic testing revealed an elevated CD4:CD8 ratio in the bronchoalveolar lavage fluid, and cryoprobe lung biopsy results showed non-necrotizing granulomatous inflammation. We describe here the second reported case of pulmonary involvement mimicking sarcoidosis in Crohn's disease and a review of the literature on the approaches to making a diagnosis of CD-associated interstitial lung disease.

Lung Cancer Screening With Low-Dose CT: Implementation Amid Changing Public Policy at One Health Care System.

Screening for lung cancer with low-dose CT has evolved rapidly in recent years since the National Lung Screening Trial (NLST) results. Subsequent professional and governmental organization guidelines have shaped policy and reimbursement for the service. Increasingly available guidance describes eligible patients and components necessary for a high-quality lung cancer screening program; however, practical instruction and implementation experience is not widely reported. We launched a lung cancer screening program in the face of reimbursement and guideline uncertainties at a large academic health center. We report our experience with implementation, including challenges and proposed solutions. Initially, we saw less referrals than expected for screening, and many patients referred for screening did not clearly meet eligibility guidelines. We educated primary care providers and implemented system tools to encourage referral of eligible patients. Moreover, in response to the Centers for Medicare & Medicaid Services (CMS) final coverage determination, we report our programmatic adaptation to meet these requirements. In addition to the components common to all quality programs, individual health delivery systems will face unique barriers related to patient population, available resources, and referral patterns.

IgG4-related lung disease: a case series of 6 patients and review of the literature.

IgG4 related disease has been recently proposed as a unifying term for a group of inflammatory conditions previously referred to by a plethora of other names. The common denominator for these entities is the histopathologic finding of lymphocytic infiltrates rich in IgG4 producing plasma cells, often accompanied by storiform fibrosis and obliterative phlebitis. Many medical conditions have been attributed to IgG4-related disease,but few reports of IgG4-related lung disease have been published, and it remains a rare condition about which little is known. In this report, we describe the clinical and pathologic features of six patients with IgG4-related disease of the lung. Patients were followed 1-5 years following their diagnosis. We describe unique features of IgG4-related lung disease, including one patient who presented with alveolar hemorrhage and a positive anti-neutrophil cytoplasmic antibody and two patients whose disease improved after treatment with mycophenylate mofetil. Two patients presented with pulmonary pseudotumor. We conclude that the clinical presentation of IgG4-related lung disease varies widely, and histopathology remains the key to diagnosis.

Significance of granulomatous inflammation in usual interstitial pneumonia.

Sarcoidosis is a systemic granulomatous disease of unclear etiology with characteristic pulmonary lesions. We describe 2 unique cases of sarcoidosis where after approximately 20 years of clinical quiescence, patients developed interstitial opacities on chest CT scan and an increase in shortness of breath. With lack of therapeutic response to a course of prednisone, both patients underwent a surgical lung biopsy that revealed a pattern consistent with Usual Interstitial Pneumonia (UIP) with honeycombing and fibroblastic foci. Postoperatively, the course of the disease was consistent with what would be expected in Idiopathic Pulmonary Fibrosis. Ultimately the disease progressed with one patient needed lung transplantation and the other requiring high-flow oxygen supplementation. In conclusion, we present two patients in whom a diagnosis of sarcoidosis preceded the diagnosis of UIP by 20 years or more. The subsequent course of disease in both patients was consistent with Idiopathic Pulmonary Fibrosis.

Combined contrast-enhanced computed tomography and 18-fluoro-2-deoxy-D-glucose-positron emission tomography in the diagnosis and staging of non-small cell lung cancer.

We present the current optimal uses and limitations of positron emission tomography/computed tomography (PET/CT) as it relates to the diagnosis and staging of non-small cell lung cancer (NSCLC). PET/CT demonstrates increased accuracy in the workup of solitary pulmonary nodules for malignancy compared with CT alone, and we discuss its benefits and limitations. We review pitfalls in measured standardized uptake values of lung lesions caused by respiratory artifacts, the lower sensitivity for detection of small lung nodules on non-breath-hold CT, and the benefits of obtaining an additional diagnostic CT for the maximum sensitivity of lung nodule detection. There are limitations of quantitatively comparing separate PET/CT examinations from different facilities with standardized uptake values. As for staging, we describe how PET/CT supplements clinical tumor-nodes-metastases (ie, TNM) staging, as well as mediastinoscopy, endobronchial ultrasound, and endoscopic ultrasound, which are the gold standard pathologic staging methods. We touch on the 7th edition TNM staging system based on the work by the International Association for the Study of Lung Cancer, an anatomically based staging method.