Interventions to Improve Symptoms, Quality of Life in Fibrotic ILD: Do They Work? by Brett Ley, MD Patients with fibrotic interstitial lung diseases (e.g. idiopathic pulmonary fibrosis) have a poor overall prognosis, and there are no therapies proven to halt disease progression or extend life. Further, many of these patients have debilitating symptoms, limited functional [... 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]
Pulmonary Vasculitis Update / Review Part 2: Treatment and Prognosis (Go to Part 1: Clinical Features and Diagnosis) by Brett Ley, MD Treatment of the ANCA-associated pulmonary vasculitides consists of systemic corticosteroids and cytotoxic therapies given in two phases, induction and maintenance, where the aggressiveness of the regimen is based on disease severity. The standard [... read more]
Pulmonary Vasculitis Update / Review Part 1: Clinical Features & Diagnosis (Go to Part 2: Treatment and Prognosis) by Brett Ley, MD Vasculitides are disorders of inflammation and necrosis of the blood vessel wall. Pulmonary vasculitides can present in multiple ways: with alveolar hemorrhage, infiltrates, nodules, cavities, or airway disease. They are categorized by the [... read more]
(image: Wikipedia) As we reported a few months ago, the PANTHER-IPF trial was stopped early for safety, when it became clear that the combination of prednisone and azathioprine was hurting people with idiopathic pulmonary fibrosis (IPF). Ganesh Raghu (U-Washington), Kevin Anstrom (Duke), Talmadge King (UCSF) et al report the final results in the May 24 New [... read more]
Using BAL Cellular Analysis in Interstitial Lung Disease: 2012 ATS Guideline The role of bronchoalveolar lavage (BAL) in diagnosing and managing patients with interstitial lung disease (ILD) has always been uncertain and controversial. An American Thoracic Society (ATS) expert panel including Keith Meyer, Ganesh Raghu, Brent Wood et al reviewed 35 years of published literature and [... 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]
GERD Treatment Improves Survival in Idiopathic Pulmonary Fibrosis? GERD is extremely common in people with idiopathic pulmonary fibrosis, and chronic microaspiration has long been suspected of playing a role in the pathogenesis of IPF. In a retrospective review, Joyce Lee, Jay Ryu, Harold Collard et al collected records on 84 patients with idiopathic pulmonary fibrosis [... 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]
Alpha-1 Antitrypsin Deficiency: 2012 Review & Update More 2012 Topic Updates This alpha-1 antitrypsin review is periodically updated and reposted as new research findings are published. Alpha-1 antitrypsin deficiency is underrecognized and by implication undertreated, according to James Stoller and Loutfi Aboussouan of the Cleveland Clinic in their excellent concise clinical review in the Blue [... read more]
Hypersensitivity Pneumonitis 2012 Review (More PulmCCM Topic Updates) Hypersensitivity pneumonitis (external intrinsic alveolitis) is an “orphan disease,” which means it’s uncommon and lacks any likely way to effectively “monetize” the disease with drugs or device therapy, resulting in its being largely ignored from a research funding standpoint. If you think you don’t know much about [... read more]
In the 1950s, half of coal miners who worked 25 years contracted black lung, or coal workers pneumoconiosis. A 1969 federal law mandated dust controls in coal mines, and the incidence of black lung declined dramatically. But that was temporary. A new report by the Center for Public Integrity and National Public Radio shows that [... read more]
Pulmonary alveolar proteinosis occurs as an idiopathic disease, or can be secondary to other conditions (HIV, silica exposure, infections, blood cancers). In the idiopathic form, autoantibodies to GM-CSF are thought to be responsible. Rituximab (a chimeric mouse-human monoclonal antibody binding CD-20) depletes human B-cells, reducing antibody production. Autoantibodies are particularly reduced after rituximab administration, leading [... read more]
Please also see the newer post describing the final results of PANTHER-IPF, published in NEJM 2012. PANTHER-IPF, a landmark clinical trial testing commonly used therapies for idiopathic pulmonary fibrosis, was stopped in October 2011 for apparent harm caused to patients by prednisone and azathioprine. This news was released back then, but maybe you missed it, as [... read more]
Although forced vital capacity has long been used to assess the status and progression of idiopathic pulmonary fibrosis (IPF), it’s been largely on a “gestalt” basis, because there hasn’t been sufficient data to properly determine FVC’s test performance characteristics. FVC has a natural 2.5% intra-patient variation in healthy people on sequential measurements; this intra-patient variability [... read more]
Air Travel for Patients with Lung Disease (BTS Recommendations) Some interesting facts, figures, and recommendations on air travel for those with chronic lung disease, thanks to Ahmedzai et al who produced this 32 page document for the British Thoracic Society: At 8,000 feet in a commercial aircraft, you’re breathing 15% O2, and normal people’s SpO2 [... read more]
Noble et al report results of two Phase III placebo-controlled trials in N. America and Europe, with 779 patients total. In one trial, pirfenidone reduced FVC decline by ~4% at 72 weeks; in the other, it did not; pooling all the data, pirfenidone reduced FVC decline by 2.5% compared to placebo. There were abundant mild [... read more]
At least 7 papers have reported an increase in risk of death after lung transplantation, mostly in the first year. For this reason, obesity is a soft contraindication to lung transplantation at many centers. David Lederer et al sought to parse out how much of that signal might be due to primary graft dysfunction, 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]
Talc pleurodesis is 90-95% effective at prevention of recurrent primary spontaneous pneumothorax. However, there have been reports of ARDS occurring after talc pleurodesis, mostly in patients with malignant pleural effusions, raising concerns over its use. Bridevaux et al report results of talc pleurodesis on 418 patients with primary spontaneous pneumothorax (PSP) at nine centers in Europe and [... read more]