Management of repository corticotrophin injection therapy for pulmonary sarcoidosis: a Delphi study.

In patients treated with repository corticotrophin injection (RCI) for pulmonary sarcoidosis, effective management of adverse events may improve adherence. However, management of adverse events may be challenging due to limitations in real-world clinical experience with RCI and available published guidelines.We surveyed 12 physicians with a modified Delphi process using three questionnaires. Questionnaire 1 consisted of open-ended questions. Panellists' answers were developed into a series of statements for Questionnaires 2 and 3. In these, physicians rated their agreement with the statements using a Likert scale.Key consensus recommendations included a starting dose of 40 units twice a week for patients with less severe disease, continued at a maintenance dose for patients who responded, particularly those with chronic refractory sarcoidosis. Panellists reached consensus that concomitant steroids should be quickly tapered in patients receiving RCI, but that concomitant use of immunosuppressive medications should be continued. Panellists developed consensus recommendations for adverse event management, and reached consensus that RCI should be down-titrated or discontinued if other interventions for the adverse effects fail or if the adverse effect is severe.In the absence of clinical evidence, our Delphi consensus opinions may provide practical guidance to physicians on the management of RCI to treat pulmonary sarcoidosis.

Improving Lung Function in Severe Heterogenous Emphysema with the Spiration Valve System (EMPROVE). A Multicenter, Open-Label Randomized Controlled Clinical Trial.

Rationale: Less invasive, nonsurgical approaches are needed to treat severe emphysema.Objectives: To evaluate the effectiveness and safety of the Spiration Valve System (SVS) versus optimal medical management.Methods: In this multicenter, open-label, randomized, controlled trial, subjects aged 40 years or older with severe, heterogeneous emphysema were randomized 2:1 to SVS with medical management (treatment) or medical management alone (control).Measurements and Main Results: The primary efficacy outcome was the difference in mean FEV1 from baseline to 6 months. Secondary effectiveness outcomes included: difference in FEV1 responder rates, target lobe volume reduction, hyperinflation, health status, dyspnea, and exercise capacity. The primary safety outcome was the incidence of composite thoracic serious adverse events. All analyses were conducted by determining the 95% Bayesian credible intervals (BCIs) for the difference between treatment and control arms. Between October 2013 and May 2017, 172 participants (53.5% male; mean age, 67.4 yr) were randomized to treatment (n = 113) or control (n = 59). Mean FEV1 showed statistically significant improvements between the treatment and control groups-between-group difference at 6 and 12 months, respectively, of 0.101 L (95% BCI, 0.060-0.141) and 0.099 L (95% BCI, 0.048-0.151). At 6 months, the treatment group had statistically significant improvements in all secondary endpoints except 6-minute-walk distance. Composite thoracic serious adverse event incidence through 6 months was greater in the treatment group (31.0% vs. 11.9%), primarily due to a 12.4% incidence of serious pneumothorax.Conclusions: In patients with severe heterogeneous emphysema, the SVS shows significant improvement in multiple efficacy outcomes, with an acceptable safety profile.Clinical trial registered with www.clinicaltrials.gov (NCT01812447).

Pulmonary Artery Systolic Pressure Measured Intraoperatively by Right Heart Catheterization Is a Predictor of Kidney Transplant Recipient Survival.

BACKGROUND The effect of pulmonary artery systolic pressure (PASP) measured by Swan-Ganz right heart catheter (SG-RHC) on kidney transplant recipient survival has not been previously studied. The objective of this study was to assess the relationships between PASP measured via SG-RHC, done intraoperatively at the time of initiating anesthesia at the beginning of kidney transplant surgery, and patient survival. Multiple comorbidities, time on dialysis before the transplantation, and graft function were also analyzed in our study. MATERIAL AND METHODS This was a retrospective cohort study using data from all consecutive patients undergoing kidney transplant between January 1, 2005 and December 31, 2009 at Tampa General Hospital. Kidney transplant recipients were divided into 2 groups: Group 1 with PASP <35 mmHg and group 2 with PASP ≥35 mmHg. Patients and graft survival data, time on dialysis before transplant, and comorbidities were compared between the 2 groups. RESULTS Only 363 patients were found to have a documented PASP measurement at the time of anesthesia induction for the transplant surgery, and were included in the specific analysis of our study. Patients with PASP ≥35 mmHg showed a significant decrease in survival in comparison to patients having PASP values <35 mmHg (HR 1.88; 95% CI 1.012 to 3.47, P=0.04). There was a significant positive correlation between time on dialysis and PASP (rho 0.20; 95% CI 0.09 to 0.30, p<0.001), as well as a significant difference in median time on dialysis between PASP <35 vs. PASP ≥35 (22 vs. 29 months, p=0.004). There were no significant differences in graft failure between the 2 PASP groups (HR 0.34; 95% CI 0.12 to 1.01, P=0.05). CONCLUSIONS Patients with PASP ≥35 mmHg, measured intraoperatively by SG-RHC, showed significantly shorter survival in comparison to patients having PASP values <35 mmHg. This result suggests the need for a randomized controlled trial to address the importance of post-transplant pulmonary hypertension management in patient survival.

Should hut lung be called domestically acquired particulate lung disease or domestically acquired pneumoconiosis?

Indoor air pollution appears to be a major environmental and public health hazard for large numbers of the underdeveloped world's population. A detailed environmental history is important for making diagnosis in most individuals from foreign rural settings with nonspecific respiratory symptoms. In this report, we describe an illustrative case of domestically acquired particulate lung disease (DAPLD) or "hut lung" in a 65-year-old Sudanese male who immigrated to the United States in 1986. He presented with symptoms of chronic productive cough and dyspnea. Imaging and pathology from transbronchial cryo biopsy revealed anthracosis consistent with DAPLD. This case demonstrates persistence of physiologic, radiographic, and histopathologic abnormalities years after removal from exposure. To the best of our knowledge, this is the first case of DAPLD reported from Sudan.

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia diagnosed by tranbronchoscopic cryoprobe biopsy technique.

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) remains a poorly understood clinical entity. It is currently classified as a premalignant condition by the World Health Organization (WHO). Symptoms are similar to those associated with obstructive lung disease, including breathlessness and cough. The presentation is often initially ascribed to other diseases such as asthma or chronic obstructive pulmonary disease. Here, we present what we believe is the first described case of DIPNECH diagnosed by transbronchoscopic cryoprobe biopsy. The patient presented with chronic cough, dyspnoea, pulmonary function tests consistent with obstruction, and a computed tomography (CT) scan of chest with multiple nodules. The patient went on to have transbronchoscopic cryoprobe biopsies of the lung, which confirmed the diagnosis of DIPNECH.

Argon plasma coagulation in the management of uncovered tracheal stent fracture.

Endotracheal and endobronchial stenting, particularly with uncovered stents, can be complicated by stent fracture, granulation tissue formation, direct airway injury, and airway obstruction. While stent removal is possible, it can result in significant complications and long-term benefit is not guaranteed. Argon plasma coagulation can be employed to trim fractured stent fragments and remove granulation tissue simultaneously. In this manuscript, we report a case and describe our experience with using this technique.