26 Aug Interstitial Lung Disease and Idiopathic Pulmonary Fibrosis
Interstitial lung abnormalities: erecting fences in the path towards advanced pulmonary fibrosis
Hunninghake GM. Interstitial lung abnormalities: erecting fences in the path towards advanced pulmonary fibrosis. Thorax Published Online First: 05 February 2019. doi: 10.1136/thoraxjnl-2018-212446
Interstitial lung abnormalities, when present in members of undiagnosed family members recruited on the basis of familial interstitial pneumonia, or in undiagnosed research participants, have been associated with a syndrome that includes distinct sets of imaging abnormalities, restrictive physiological and exercise impairments, and an increased prevalence of histopathological findings, and genetic predictors, that have been noted in patients with idiopathic pulmonary fibrosis.
Recent longitudinal studies have demonstrated that qualitative and quantitative assessments of interstitial abnormalities are associated with accelerated lung function decline, an increased rate of clinical diagnoses of interstitial lung disease and an increased rate of mortality.
In this perspective, in addition to reviewing the prior information, four major efforts that could help the field of early pulmonary fibrosis detection move forward are discussed. These efforts include:
- Developing standards for characterising and reporting imaging findings from patients with existing CTs
- Developing consensus statements on when undiagnosed and asymptomatic imaging abnormalities should be considered a disease
- Identifying populations for which screening efforts might be beneficial
- Considering approaches to developing effective secondary prevention trials
Treatment of Interstitial Lung Disease Associated Cough
Birring, Surinder S.Adams, Todd M. et al. Treatment of Interstitial Lung Disease Associated Cough. Chest, Volume 154, Issue 4, 904-917. doi.org/10.1016/j.chest.2018.06.038
Chronic cough in interstitial lung disease (ILD) causes significant impairment in quality of life. Effective treatment approaches are needed for cough associated with ILD. This systematic review asked: Is there evidence of clinically relevant treatment effects for therapies for cough in ILD? Studies of adults aged > 18 years with a chronic cough ≥ 8 weeks’ duration were included and assessed for relevance and quality. The summary of recommendations and suggestions are as follows:
For patients with ILD who present with a troublesome cough, we suggest that patients be assessed for progression of their underlying ILD, or complications from immunosuppressive treatment (eg, drug side effect, pulmonary infection) and also be considered for further investigation/treatment trials for their cough according to guidelines for acute, subacute and chronic cough. (Ungraded Consensus-Based Statement)
For patients with IPF, chronic cough and a negative workup for acid gastroesophageal reflux, we suggest that proton pump inhibitor therapy should not be prescribed. (Ungraded Consensus-Based Statement)
For patients with pulmonary sarcoidosis, we suggest that inhaled corticosteroids should not be routinely prescribed to treat the chronic cough. (Grade 2C).
For patients with ILD and refractory chronic cough, we suggest trials of therapies recommended for patients with unexplained chronic cough according to the CHEST guidelines, with treatments such as gabapentin and multimodality speech pathology therapy, or entering into clinical trials if available. (Ungraded Consensus-Based Statement)
For patients with chronic cough due to ILD, when alternative treatments have failed and the cough is adversely affecting their quality of life, we suggest that opiates be recommended for symptom control in a palliative care setting with reassessment of the benefits and risks at 1 week and then monthly before continuing. (Ungraded Consensus-Based Statement)