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: Review & Update More PulmCCM Topic Updates Alpha-1 antitrypsin deficiency is underrecognized and by implication undertreated. But many people with alpha-1 antitrypsin deficiency are asymptomatic and will never develop significant emphysema, and the benefits of antiprotease replacement therapy are still unknown. Should you routinely test your patients with chronic obstructive pulmonary disease [… 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]
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]
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]
Is bronchoalveolar lavage useful in the diagnosis and management of interstitial lung disease? Meyer and Raghu believe so (although their manuscript title suggests they’re not 100% sure). They explain why in the October ERJ. They argue that if interpreted in the right clinical context, certain bronchoalveolar lavage findings can help cinch the diagnosis of ILD: Lymphocytes >= 25%: [… read more]
This month’s Current Opinion in Pulmonary Medicine has a section each on sarcoidosis, interstitial lung disease, and pulmonary vascular disease, mainly DVT/PE. Curr Opin Pulm Med 2011;17:297-402.
Lung fibrosis is partially mediated by tyrosine kinase pathways. BIBF 1120 is a tyrosine kinase inhibitor that targets vascular endothelial growth factor receptor (VEGFR), fibroblast growth factor receptor (FGFR) and platelet-derived growth factor receptor (PDGFR) — all receptors known to be involved in lung fibrosis. Supported by Boehringer Ingelheim, Richeldi et al randomized 432 patients with [… read more]
Walker et al give us a nice picture and high-yield tutorial on the reversed halo sign, in this case from cryptogenic organizing pneumonia: J Thorac Imaging 2011;26:w80. (FREE)
September’s Current Opinion in Pulmonary Medicine has 4-5 review articles each on sarcoidosis (how to handle calcium problems; cardiac sarcoid); interstitial lung disease (biologics for connective-tissue disease related ILD; LAM therapies; stem cell therapy for pulmonary fibrosis), and pulmonary vascular disease. Curr Opin Pulm Med 2011;17.
Unimpressed by the unimpressive results of the CAPACITY trials testing pirfenidone for idiopathic pulmonary fibrosis, the FDA bucked its advisory panel (which had recommended approval) and insisted on another randomized trial in order to reconsider the drug. In July 2011 InterMune started enrollment for ASCEND, which plans to provide results on pirfenidone vs. placebo in [… read more]
King et al report a series of 80 otherwise healthy young vets who failed a 2-mile run fitness test due to dyspnea at Fort Campbell, KY. Many had been exposed to sulfur fires or other inhalational exposures. Among 49 who underwent open lung biopsy, 38 had constrictive bronchiolitis, an extremely rare condition in otherwise healthy [… read more]
A retrospective review of 521 patients in one Virginia center from 2000-2009, by Nathan SD et al. CHEST 2011;140:221-229
Pulmonary alveolar proteinosis. Borie R et al, Eur Resp Rev 2011;20:98-107.
Follow-up and nonpharmacological management of the idiopathic pulmonary fibrosis patient. Egan JJ, Eur Resp Rev 2011;20:114-117.