Restrictive Lung Disease

Updated Practice Guideline: IPF and Progressive Pulmonary Fibrosis

By

Colby Stong

Publish Date June 3, 2022

A recent update of IPF clinical practice guideline addresses the progression of pulmonary fibrosis in patients with interstitial lung diseases other than IPF.

ATS Guideline: Outpatient Management of Pediatric Post-Prematurity Respiratory Disease

By

Brandon May

Publish Date April 15, 2022

How should clinicians handle the outpatient management of infants, children, and adolescents with post-prematurity respiratory disease? A summary of updated recommendations from the American Thoracic Society offers guidance.

Pulmonologists vs Rheumatologists: Treating Rheumatoid Arthritis-Associated Interstitial Lung Disease

By

Linda Peckel

Publish Date November 19, 2021

Rheumatologists and pulmonologists provide differing perspectives on risk factors, disease assessment, and treatment strategies for patients with rheumatoid arthritis-associated interstitial lung disease.

Pirfenidone Benefits Patients With Arthritis-Associated Interstitial Lung Disease

By

Bradley van Paridon

Publish Date October 5, 2022

A phase 2 trial of pirfenidone for patients with RA-ILD yielded findings on the drug’s efficacy, safety, and tolerability, despite early termination of the trial.

Orkambi Indication Expanded to Include More Children With Cystic Fibrosis

By

Diana Ernst, RPh

Publish Date September 9, 2022

The safety profile of Orkambi was found to be similar in patients aged 1 to less than 2 years compared with those 2 years of age and older.

Risk Stratification in Cystic Fibrosis Patients Listed for Lung Transplantation

By

Ron Goldberg

Publish Date August 12, 2022

A newly developed cystic fibrosis frailty index calculates the risk for poor outcomes both in patients post lung transplant and among those awaiting transplant.

Early Cystic Fibrosis Airway Disease Markers and PD-1

By

Sheila Jacobs

Publish Date August 10, 2022

Do links exist between macrophage programmed cell death protein-1 (PD-1) and markers of airway disease in very young children with cystic fibrosis?

Predicting Pediatric CF Events with Patient-Reported Outcomes and Lung Measures

By

Sheila Jacobs

Publish Date August 2, 2022

Can acute respiratory events in children with cystic fibrosis be predicted using PRO tools that measure patient- and parent-reported respiratory symptoms?

High-Resolution CT Scoring Promising for RA-Associated Interstitial Lung Disease

By

Inga Back, MPH

Publish Date July 12, 2022

Researchers reviewed 2 studies that evaluated high-resolution computed tomography images (HCRT) and their ability to predict important clinical outcomes for rheumatoid arthritis-associated interstitial lung disease (RA-ILD).

Azithromycin and Structural Lung Disease in Newborns With Cystic Fibrosis

By

Ron Goldberg

Publish Date July 7, 2022

Will azithromycin, given 3 times weekly to infants with cystic fibrosis from diagnosis until age 36 months, reduce damage from structural lung disease?

Prognosis After Acute Exacerbation in Rheumatoid Arthritis-ILD vs IPF

By

Sheila Jacobs

Publish Date July 5, 2022

How frequent are acute exacerbations in rheumatoid arthritis-related ILD and how does the prognosis for these compare with AEs in idiopathic pulmonary fibrosis?

Efficacy/Safety of Idiopathic Pulmonary Fibrosis Treatment With BI 1015550

By

Sheila Jacobs

Publish Date June 28, 2022

A phase 2 trial explored the efficacy and safety of BI 1015550 — an oral preferential inhibitor of the PDE4B subtype — in patients with IPF.

Risk Score Developed for RA-Linked Interstitial Lung Disease

Publish Date June 24, 2022

A score has been developed to stratify the risk for subclinical rheumatoid arthritis-associated interstitial lung disease (RA-ILD).

Pulmonary Ultrasonography Detects Interstitial Lung Disease in Rheumatoid Arthritis

By

Emily Pond

Publish Date June 17, 2022

Researchers investigated the potential role in ultrasonography in the detection of interstitial lung disease (ILD) in patients with rheumatoid arthritis (RA).