In addition to today’s resource, please review ACR Appropriateness Criteria® Diffuse Lung Disease,
Abstract
Diffuse lung disease, frequently referred to as interstitial lung disease, encompasses numerous disorders affecting the lung parenchyma. The potential etiologies of diffuse lung disease are broad with several hundred established clinical syndromes and pathologies currently identified. Imaging plays a critical role in diagnosis and follow-up of many of these diseases, although multidisciplinary discussion is the current standard for diagnosis of several DLDs. This document aims to establish guidelines for evaluation of diffuse lung diseases for 1) initial imaging of suspected diffuse lung disease, 2) initial imaging of suspected acute exacerbation or acute deterioration in cases of confirmed diffuse lung disease, and 3) clinically indicated routine follow-up of confirmed diffuse lung disease without acute deterioration.
Today, I review, link to, and excerpt from CoreIM‘s “ILD and High-Risk Medication Prescribing: Gray Matters Segment“.*
*ILD and High-Risk Medication Prescribing: Gray Matters Segment
Posted: February 5, 2025
By: Dr. Alison Trainor, Dr. Kelly Graham, Dr. Rob Hallowell, Jason Krastein and Dr. Jason Freed
Graphic: Dr. Jesse Powell
Audio: Jerome Reyes
Peer Review: Dr. Armand M. Gottlieb, Dr. Robert Koichiro Arao
All that follows is from the above resource.
All that follows is from the above resource.
Time Stamps
- 01:30 Deep Dive 1: Evaluating Incidental Findings
- 06:45 Deep Dive 2: Categorizing ILD
- 12:42 Deep Dive 3: Treatment for ILD
- 21:19 Deep Dive 4: Addressing Barriers to Care
- 28:10 Deep Dive 5: Ethical Considerations in ILD Treatment
CME-MOC
- Get CME-MOC credit with ACP!
Show Notes
Deep Dive 1: Evaluating Incidental Findings
Why do incidental CT findings matter?
- Subtle interstitial changes on a CT scan may indicate early fibrosis, atelectasis, aspiration, pulmonary edema, or infection. (Though not exhaustive list)
- What is ILD protocol CT?
- High-resolution CT (HRCT) with inspiratory, expiratory, supine and prone images
- Differentiate fibrosis from mimics
- Involves:
- Number of Acquisitions:
- Supine Position: Inspiratory (volumetric) and expiratory (sequential or volumetric) acquisitions.
- Prone Position (Optional): Only inspiratory scans, which can be sequential or volumetric.
- Inspiratory scans should be obtained at full inspiration.
If fibrosis is suspected:
- Refer to pulmonology within 6 months
- Order [complete] pulmonary function tests (PFTs) to expedite evaluation.*
- PFTs provide data on disease progression and urgency of intervention.
*And includes a DLCO: See Diffusing Capacity of the Lungs for Carbon Monoxide from StatPearls. Last Update: October 6, 2024.
Deep Dive 2: Categorizing ILD
What is a clinically relevant way to categorize the causes of ILD?
- Broad groups to aid in diagnosis and treatment. Below is a streamlined overview:
- Infectious ILDs
- Examples: Viral pneumonias, tuberculosis, fungal infections.
- Environmental or Medication-Induced ILDs
- Environmental exposures examples: tobacco/smoking, asbestos, silica, and other occupational exposures.
- Medication-Induced ILD: Common culprits include amiodarone and methotrexate.
- Autoimmune-Related ILDs
- Associated with systemic rheumatologic conditions, such as systemic sclerosis, rheumatoid arthritis, and polymyositis.
- Subtype: Often referred to as connective tissue disease-associated ILDs (CTD-ILDs).
- Granulomatous ILDs
- Examples: Sarcoidosis, Langerhans cell histiocytosis.
- Sarcoidosis:
- Characterized by non-caseating granulomas.
- Requires exclusion of other granulomatous diseases.
- Langerhans Cell Histiocytosis:
- Often linked to smoking.
- Presents with cystic and nodular changes on HRCT.
- Idiopathic ILDs
- Definition: Diagnosed after ruling out all other potential causes.
- Examples:
- Idiopathic Pulmonary Fibrosis (IPF):
- Most common form
- Often managed with antifibrotics.
- Nonspecific Interstitial Pneumonia (NSIP):
- A more inflammatory subtype.
- Cryptogenic Organizing Pneumonia (COP):
- Frequently responds to corticosteroids.
Deep Dive 3: Treatment for ILD
What are the primary treatment options?
- Antifibrotics: Pirfenidone and Nintedanib slow the decline in FVC but do not reverse fibrosis.
- Indicated for all patients with progressive fibrosis, irrespective of subtype.
- Side effects include GI disturbances (e.g., diarrhea) and hepatotoxicity (requires liver enzyme monitoring).
- Key takeaways INBUILD trial
- 1. Efficacy of Nintedanib:
- Primary Outcome: Reduced annual FVC decline by 107 mL/year compared to placebo in the overall population (-80.8 mL vs. -187.8 mL, p<0.001).
- For patients with a UIP-like fibrotic pattern: Decline reduced by 128.2 mL/year (-82.9 mL vs. -211.1 mL, p<0.001).
- Patients with other fibrotic patterns showed a reduction of 75.3 mL/year.
- Secondary Outcomes:
- No statistically significant improvement in quality of life using the K-BILD questionnaire.*
- Lower incidence of acute exacerbation or death: 7.8% in nintedanib vs. 9.7% in placebo.
- Safety Profile:
- Most common adverse event: Diarrhea (66.9% in nintedanib vs. 23.9% in placebo).
- Liver enzyme elevations were more common in the nintedanib group (13.0% vs. 1.8%).
- Discontinuation due to adverse events: 19.6% in the nintedanib group vs. 10.3% in placebo.
- Immunosuppressants:
- Ex: Mycophenolate and azathioprine
- Used in non-IPF ILDs.
- Avoid in IPF, as shown by increased mortality in the PANTHER trial.
- PANTHER trial was a randomized, double-blind, placebo-controlled trial comparing a combination of prednisone, azathioprine, and N-acetylcysteine (NAC) (triple therapy) versus placebo.
- Patients included had mild-to-moderate IPF with FVC ≥50% and DLCO ≥30% of predicted values.
- Outcomes:
- Increased Mortality:
- Patients in the triple therapy group experienced an 8-fold higher mortality rate compared to placebo (10% vs. 1%, p = 0.01).
- Deaths were primarily related to respiratory causes or treatment-related complications.
- Hospitalizations:
- Rates of all-cause hospitalization were significantly higher in the triple therapy group (30% vs. 9%, p < 0.001).
- Serious Adverse Events (SAEs):
- 31% of patients in the triple therapy group reported SAEs, compared to 10% in the placebo group (p = 0.001).
- These included respiratory complications, infections, and general adverse drug reactions.
- The PANTHER trial provided definitive evidence that immunosuppressants, particularly azathioprine and prednisone, should not be used in IPF due to their association with increased mortality, hospitalizations, and adverse events. Antifibrotic therapies remain the standard of care for this patient population.
- What role do steroids play?
- Managing acute ILD exacerbations or flares
- Rapidly progressive or exacerbating ILD, where they can help reduce inflammation and stabilize the patient’s condition.
Deep Dive 4: Addressing Barriers to Care
How do you identify and address barriers to care?
- Use Maslow’s hierarchy of needs to guide assessment:
- A psychological theory that explains human motivation in five levels, starting from basic survival needs to personal growth.
- You must meet lower-level needs before focusing on higher ones.
- Basic Needs: Essentials like food, water, safety, and shelter.
- Specifically: Transportation, food security, and health literacy.
- Social Needs: Relationships, love, and belonging.
- Self-Worth: Feeling respected and accomplished.
- Personal Growth: Achieving your full potential and pursuing your passions.
- Collaborate with PCPs and social workers to address social determinants of health.
Deep Dive 5: Ethical Considerations in ILD Treatment
How do you balance ethics in ILD care?
- Utilitarian ethics focus on maximizing outcomes vs.
- Egalitarian ethics emphasize equal care opportunities.
- Success in ILD management often hinges on equitable access to resources, rather than identical treatment outcomes for all patients.
What is the role of shared decision-making?
- Frame treatment as a contract between physician and patient, ensuring both parties collaborate for effective care.
Key Takeaways
- Evaluate incidental CT findings using ILD protocol CT with prone imaging, and follow up with PFTs.
- Categorize ILD broadly (e.g., infections, exposures, idiopathic) to guide therapy.
- Treat progressive fibrosis with antifibrotics; use immunosuppressants for non-IPF ILDs.
- Address social barriers to care using frameworks like Maslow’s hierarchy to improve patient adherence.
- Strive for equitable care opportunities through ethical and collaborative practices.
