In 2011 PulmCCM reported the findings of the phase 2 TOMORROW study, showing Boehringer Ingelheim’s tyrosine kinase inhibitor code-named BIBF 1120 reduced lung function decline and prevented exacerbations in people with idiopathic pulmonary fibrosis (IPF). At the 2014 American Thoracic Society annual meeting and in the New England Journal of Medicine, investigators announced their latest [... read more]
For idiopathic pulmonary fibrosis treatment pirfenidone, the third time wasn’t the charm; the fourth was. After reducing decline in forced vital capacity over one year in a Japanese trial of 275 IPF patients, pirfenidone went one-and-one in the multinational CAPACITY trials (n=779), meeting the primary outcome of FVC preservation in one but not the other. [... read more]
Riociguat (Adempas) Gets FDA Indications for Pulmonary Hypertension That was fast. The US Food and Drug Administration (FDA) approved riociguat (brand name Adempas, by Bayer) for the treatment of pulmonary arterial hypertension (PAH) and the treatment of chronic thromboembolic pulmonary hypertension (CTEPH), on October 8 2013. Adempas is the first of a class of drugs for pulmonary hypertension [... read more]
More Children Under 12 to Be Considered for Adult Lung Transplants The Organ Procurement and Transplantation Network (OPTN), which is responsible for the life-and-death policies for allocation of lung transplants and other organs, announced it will allow children needing lung transplants to file expedited appeals to be listed for adult lungs. Children under 12 with [... read more]
Bactrim/Septra for Idiopathic Pulmonary Fibrosis? by Brett Ley, MD Idiopathic pulmonary fibrosis (IPF) is a fatal fibrotic lung disease that lacks proven, effective treatments. Many novel medications have been trialed and painfully failed. So I commend Ludmila Shulgina and colleagues for trying an old medication, trimethoprim-sulfamethoxazole (TMP-SMX), with some preliminary evidence for potential benefit. What [... read more]
(image: Wikipedia) Access the online GAP Score Calculator for IPF Although idiopathic pulmonary fibrosis (IPF) has a poor prognosis — a median survival of only 3 years — there is wide heterogeneity among individual patients. Some have a precipitous decline and die within months of diagnosis; others live for a decade or longer, with little [... read more]
New Trachea Grown, Transplanted In Two Men Paolo Macchiarini, Philipp Jungebluth et al report in Lancet their successful bioprosthetic trachea creation and transplantation in a 36-year old man in Sweden after a distal tracheal resection for recurrent primary tracheal cancer. The same group transplanted a bioengineered trachea into a 30-year old Baltimore man, who is [... read more]
Idiopathic Pulmonary Fibrosis (IPF) 2012 Review (More PulmCCM Topic Updates) This document is updated periodically as new research findings in IPF are published. What Causes IPF? (Etiology/Pathogenesis of IPF) Since it’s still called “idiopathic,” you probably figured out already that the cause(s) of IPF remain unknown. Researchers use a paradigm for studying idiopathic pulmonary fibrosis called [... read more]
“So [gasp, gasp] That’s Where I Left Them!” 1:00 PM Tuesday 6/12/12: An NBC television affiliate is reporting that what appear to be a pair of lungs were found on a sidewalk in South Los Angeles at 8:30 pm on Sunday. At this point, no one knows if the lungs are human — or if [... read more]
From the excellent review by Lonny Yarmus and David Feller-Kopman in the April 2012 Chest, along with the British Thoracic Society and ACCP’s consensus statements: Mechanisms of Pneumothorax in the Critically Ill Air can accumulate in the pleural space in three ways: Rupture of the visceral pleura allowing air to travel from alveoli to the [... read more]
Lungs from brain-dead or deceased donors are considered unusable 85% of the time, worsening waiting-list times and death rates. At a single Toronto center, Cypel et al perfused damaged donor lungs ex-vivo (EVLP; here’s how) for 4 hours, and if their numbers were good (PaO2/FiO2 > 349; less than 15% worsening in compliance, pulmonary vascular resistance and [... read more]
Duke’s Momen Wahidi and other luminaries bring you a consensus statement on use of peri-procedure medications during bronchoscopy. I’m assuming you’ve done a few already, so here are some highlights (with slight liberties in paraphrasing): Use topical anesthesia as well as moderate sedation in all patients, unless there are contraindications or you practice at a secret CIA prison. [... read more]
LaPar et al prospectively followed 39 people undergoing lung transplantation, collecting circulating fibrocytes at periodic intervals. Numbers of circulating fibrocytes (analyzed by flow cytometry) were higher in those who developed bronchiolitis obliterans syndrome, and correlated with clinical stage of BOS. Ann Thorac Surg 2011;92:470-477.
It’s long been known in the pediatric cystic fibrosis population that exacerbations accelerate decline of lung function. de Boer et al show that exacerbations are just as bad or worse in adults. Following 446 adults with CF in Ontario for 3 years, those with >2 exacerbations per year had a hazard ratio of 4.05 for [... read more]
Bronchiolitis Obliterans Syndrome: The Final Frontier. No, it’s not the new Star Trek movie featuring a breathless William Shatner. It’s Todd JL, Palmer SM’s review on BOS, the lung transplantation scourge, in CHEST 2011;140:502-508.
Hartwig et al report a series of 157 people receiving fundoplication within 1 year after lung transplant, for reflux diagnosed by abnormal esophageal pH probe. They compared these to 65 with reflux who did not undergo fundoplication. The surgical patients had about 10% higher FEV1s, both at peak and at one year. This was not [... read more]
Lung transplantation. Sixteen articles, 217 pages. Clinics in Chest Med 2011;32:199-416.
Invasive aspergillosis is often lethal in lung transplant patients, and early diagnosis and treatment improve survival. Luong M et al showed that testing bronchoalveolar lavage fluid with new rapid-result Aspergillus PCR and galactomannan tests bring both sensitivity and specificity into the 93-100% range. 150 BAL samples from lung transplant patients at Pitt were tested (there were only [... read more]
In an observational study of 210 patients undergoing resection for lung cancer, those with a peak oxygen uptake (VO2 max) of <10 mL/kg/min were 4 times more likely to have cardiac or pulmonary complications than those with a VO2 > 17. ERJ 2011;37:1189-1198.
Using PCR testing on BAL fluid, Denning et al (in Manchester UK) found Aspergillus DNA in 4 of 11 (36%) healthy volunteers; 21 of 22 patients with culture/microscopy-proven invasive aspergillosis; and 70-80% of patients with allergic bronchopulmonary aspergillosis or chronic pulmonary aspergillosis. Culture was positive in none of the ABPA patients, and only 17% of the CPA [... read more]