Scleroderma-Related Interstitial Lung Disease Scleroderma (SSc) is an autoimmune disease characterized by vasculopathy and fibrosis with multi organ involvement. Pulmonary complications such as interstitial lung disease (ILD) and pulmonary hypertension contribute significantly to mortality and morbidity of the disease. Up to 90% patients with scleroderma can have interstitial changes on high resolution CT scan (HRCT) [… read more]
The various forms of interstitial lung disease (ILD) can be impossible to tell apart without a biopsy. There’s too much overlap between them in their appearance on imaging (high-resolution chest CT), and in clinical features. A surgical lung biopsy is the standard in such cases. For many patients and physicians, though, the risk and discomfort [… read more]
ATS Releases New Idiopathic Pulmonary Fibrosis Treatment Guidelines Recent Studies Bring New Recommendations by Lekshmi Santhosh, MD This month, the American Thoracic Society, European Respiratory Society, Japanese Respiratory Society and Latin American Thoracic Association released their updated guidelines on the treatment of idiopathic pulmonary fibrosis (IPF). They are free on the ATS website for all to peruse, but [… read more]
The U.S. FDA approved the first two drugs proven to slow progression of idiopathic pulmonary fibrosis (IPF): pirfenidone (Esbriet by Roche, which bought Intermune) and nintedanib (Ofev by Boehringer Ingelheim) on Wednesday October 15. Both drugs will offer new hope for patients, and new pain to the insurance companies and the government who pay for them. Pirfenidone [… read more]
The American College of Chest Physicians (unaffiliated with PulmCCM) published its new consensus guidelines in August 2014 for the drug treatment of pulmonary arterial hypertension (PAH). They’re free to view on the Chest website, and well worth a look. Remember that pulmonary arterial hypertension (PAH) is but one small subset (“Group 1”) of the much larger [… read more]
Using BAL Cellular Analysis in Interstitial Lung Disease The role of bronchoalveolar lavage (BAL) in diagnosing and managing patients with interstitial lung disease (ILD) has always been uncertain and controversial. BAL findings are usually nonspecific, suggesting rather than proving the existence of any particular noninfectious condition (including interstitial lung disease). That said, in patients with [… read more]
NAC for IPF: Fail (PANTHER-IPF Wrap-Up) Long ago, when pulmonologists were more ingenuous (in 2000), flawed clinical trials convinced the American Thoracic Society to recommend prednisone and either azathioprine or cyclophosphamide in its consensus guideline for some patients with idiopathic pulmonary fibrosis (IPF). By not including a true control arm, the also-flawed follow-up 2005 IFIGENIA trial reinforced this [… read more]
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]
Is Idiopathic Pulmonary Fibrosis Caused by a Virus? The cause(s) of idiopathic pulmonary fibrosis have vexed researchers for decades. Although risk factors (smoking, familial gene mutations) provide clues, the actual trigger for the proliferative, fibrotic cellular process resulting in usual interstitial pneumonitis (on biopsy specimens) and IPF (clinically) have remained frustratingly elusive. But an exciting [… read more]
A large phase III randomized trial testing Ambrisentan (Letairis) for early idiopathic pulmonary fibrosis was stopped early after an interim analysis of the data showed possible harm. The results were reported in Annals of Internal Medicine. Patients being treated with Letairis had more evidence of progression of idiopathic pulmonary fibrosis and were hospitalized more often, including [… read more]
EUS with EBUS Superior at Diagnosing Sarcoidosis Sarcoidosis affects tens of thousands of people in the U.S., but making the diagnosis can be tricky. A biopsy is almost always required, and since >90% of patients have involvement of the lungs and/or lymph nodes in the chest, bronchoscopy has been the standard procedure for obtaining tissue. [… read more]
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]
(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]
(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]
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]
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