Comparison of survivors and nonsurvivors of diffuse alveolar hemorrhage: risk factors for in-hospital death

Background: The objective of this study was to identify the predictors of in-hospital mortality among patients with diffuse alveolar hemorrhage (DAH).Methods: We conducted a retrospective review of 89 patients hospitalized for DAH at our institution. 49 patients who died during hospitalization and 40 patients who survived were compared. We reviewed their clinical course, radiologic and pathologic findings, along with medical management. We then performed univariate and multivariate analyses to identify the risk factors associated with in-hospital mortality.Results: We identified 12 factors to be associated with mortality when comparing survivor versus non-survivor cohorts: smoking (67 versus 42%, p=0.02), malignancy (17 versus 49%, p=0.002), interstitial lung disease (0 versus 14%, p=0.01), liver failure (2 versus 28%, p=0.001), autoimmune diseases (40 versus 8%, p=0.0006), thrombocytopenia (7 versus 71%, p<0.0001), ICU admission (57 versus 85%, p=0.004), mean ICU stay (p=0.4), steroid use (90 versus 63%, p=0.003), plasma exchange (15 versus 0 %, p=0.005), mechanical ventilation (37 versus 75%, p=0.0007) and acute respiratory distress syndrome (22 versus 77%, p<0.0001). On multivariate analysis, thrombocytopenia (p<0.0001) and ARDS (p=0.0013) were associated with higher odds of mortality in DAH while steroid use (p=0.0004) was associated with a lower risk of in-hospital mortality in patients with DAH.Conclusions: In DAH, thrombocytopenia and ARDS were predictors of in-hospital mortality whereas the use of steroid was associated with a more favorable prognosis.

Emphysematous changes in hypersensitivity pneumonitis: A retrospective analysis of 12 patients

Introduction: Emphysema is most commonly associated with smoking but also occurs in hypersensitivity pneumonitis (HP). The aim of this study was to further explore this relationship. Methods: A retrospective, computer-assisted search was performed to identify patients with HP seen at Mayo Clinic in Rochester, Minnesota, from January 1997 through February 2014. Demographic, clinical, and imaging features were analyzed. Patients were excluded if they had a smoking history of 10 pack-years or more. Results: Twelve patients (9 males) with HP and computed tomographic evidence of emphysema were identified. Ten were never smokers and 2 were ex-smokers. The median age at diagnosis was 47 (range, 29–77) years; median symptom duration was 2.2 (range, 0.2–13.4) years. The most common presenting symptoms were dyspnea (83%) and cough (67%). On pulmonary function testing, 6 patients (50%) had a restrictive defect, 2 (17%) had airflow obstruction, and 4 (33%) had an isolated reduction in diffusing capacity of lung for carbon monoxide. The severity of emphysema ranged from mild to severe to focal bullae. All patients had chronic hypersensitivity pneumonitis (CHP). Centrilobular emphysema was most commonly seen with coexistent paraseptal emphysema in 5 patients.Emphysema was most frequent in the upper lung but could be seen in any lobe. Conclusion: Emphysema can occur in patients with CHP independently of smoking history and exposure to specific types of antigens. Emphysematous changes seem to progress at a slower pace compare to reticulations/fibrosis. Keywords: Emphysema, Fibrosis, Hypersensitivity pneumoniti

Challenges in the Diagnosis and Management of Fibrotic Hypersensitivity Pneumonitis: A Practical Review of Current Approaches

Recent advances in fibrotic hypersensitivity pneumonitis include improved diagnostic guidance, systematic assessments of immunosuppressive therapy, and the recent availability of antifibrotic therapy (nintedanib) for those with progressive disease. A standardized approach to diagnosis may lead to better inclusion criteria for future therapeutic protocols and delineation of disease or treatment response predictors for real-world management. This review will highlight current diagnostic and treatment challenges and remaining knowledge gaps or areas of uncertainty, with a practical overview of supporting evidence and its clinical implications. Exposure history, serologic testing for antigen sensitivity, bronchoalveolar lavage lymphocytosis, histopathology, and radiologic findings will be covered in the diagnosis section, with immunosuppression, antifibrotic therapy, lung transplantation, and disease prognosis in the treatment and management section

A 62-year-old man with dyspnea

We describe the case of a 62-year-old man who presented with shortness of breath that had progressed over several years. He had a history of a paralyzed right hemidiaphragm for at least the previous 10 years. He also reported weakness in his proximal legs and daytime sleepiness. On examination, he was found to have thoracoabdominal paradox when in supine position. Pulmonary function testing revealed severe restriction; arterial blood gas showed chronic respiratory acidosis. Electromyography showed chronic phrenic neuropathy bilaterally, with mild proximal myopathy. Serum aldolase level was mildly elevated, but serologic tests for connective tissue disorders were within reference range. After extensive clinical investigations, the patient was found to have severely reduced acid α-glucosidase. Genetic analysis confirmed the diagnosis of adult-onset Pompe disease. The patient started treatment with bilevel positive airway pressure titrated during polysomnography, and acid α-glucosidase enzyme replacement was recommended

Current concepts and dilemmas in idiopathic interstitial pneumonias [version 1; referees: 4 approved]

Idiopathic interstitial pneumonias comprise approximately one-third of interstitial lung diseases (also called diffuse parenchymal infiltrative lung diseases). The classification of idiopathic interstitial pneumonias has undergone several revisions since the initial description of 40 years ago, and the most recent version was published in 2013. Although some aspects have been clarified, this group of heterogeneous disorders continues to be a source of confusion and misunderstanding in clinical applications. In this article, we explore several topical themes in the evaluation and management of patients with idiopathic interstitial pneumonias

Mycophenolate mofetil for scleroderma-related interstitial lung disease: A real world experience.

Interstitial lung disease (ILD) remains the number one cause of mortality in scleroderma (SSc). Our goal was to determine the effectiveness of mycophenolate mofetil (MMF) in treating SSc-ILD in a retrospective study.A retrospective, computer-assisted search was performed to identify patients with SSc-ILD treated with MMF from 1997 through 2014. We used a novel software tool, Computer-Aided Lung Informatics for Pathology Evaluation and Rating (CALIPER), to quantify parenchymal lung abnormalities on high-resolution computed tomography. Lung function was evaluated at baseline, 6, 12, and 24 months of MMF therapy.We identified 46 patients (28 females) with SSc-ILD (mean age at diagnosis 55 y) treated with MMF for at least 1 year (majority on 2 gm/day). Twenty-one patients (45.7%) stopped using MMF during the follow up period after the first 12 months, and they took MMF for a median of 2.12 years (range, 0.91-8.93 years). Only 4 discontinued MMF because of disease progression. Compared to baseline, the mean percentage change in forced vital capacity (95% CI) at 6, 12, and 24 months, respectively, was 1.01% (-2.38%-4.39%) (n = 26), 2.06% (-1.09%-5.22%) (n = 31), and -0.07% (-3.31%-3.17%) (n = 30), and the mean percentage change in ILD as measured by CALIPER (95% CI) was -5.40% (-18.62%-7.83%) (n = 18), -1.51% (-14.69%-11.68%) (n = 17), and -8.35% (-20.71%-4.02%) (n = 22).The mean right ventricular systolic pressure (RVSP) remained stable over the study period.MMF is well tolerated and slows the rate of decline in lung function in SSc-ILD patients, even at doses lower at 3 g/day

Hepatic Actinomycosis with Infiltration of the Diaphragm and Right Lung: A Case Report

Actinomycosis is an indolent, slowly progressive infection caused by anaerobic or microaerophilic bacteria of the genus Actinomyces. Actinomycosis has a myriad of clinical presentations, inducing both a suppurative and granulomatous inflammatory response. The infection spreads contiguously through anatomical barriers and frequently forms external sinuses. The most common clinical presentations are cervicofacial, thoracic, abdominal and, in females, genital. Classic features include purulent foci surrounded by dense fibrosis that, over time, cross natural boundaries into contiguous structures, with the formation of fistulas and sinus tracts in some cases. Hepatic actinomycosis presents as single or multiple abscesses or masses. Reported here is the unusual occurrence of actinomycosis of the liver involving the diaphragm and right lung. The present case illustrates the difficulties in diagnosing this rare and unrecognized disease

Improvement of constrictive bronchiolitis (bronchiolitis obliterans) after rituximab therapy in 2 patients with primary sjögren syndrome

Constrictive bronchiolitis is one of the manifestations of small-airway involvement in primary Sjögren syndrome (SS) and is associated with fixed airflow obstruction despite treatment with bronchodilators, macrolides, corticosteroids, and corticosteroid-sparing agents. Reports have shown a beneficial effect of rituximab on interstitial lung disease associated with SS, but the effect of rituximab on constrictive bronchiolitis is unknown. Herein, we present 2 cases of patients with constrictive bronchiolitis associated with SS who experienced symptomatic improvement and stabilization of pulmonary function testing (PFT) after rituximab therapy. Lung function declined in one of the patients when B cells reconstituted, with improved PFT results on re-administration of rituximab. Our case reports suggest that B cells may be involved in the pathogenesis of SS-associated constrictive bronchiolitis. Therapy targeting B cells may therefore be helpful in treating this debilitating and refractory condition. Further research is warranted