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Interstitial Lung Disease Made Simple

Nirav Shah, MD and Neda Frayha, MD
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22:50

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There are over 140 different types of interstitial lung disease, all with their own acronym. This adds up to a complex alphabet soup that can be hard to keep straight in our daily practice. In this segment, pulmonologist and medical educator Dr. Nirav Shah provides us with a broad framework for understanding ILD and how to approach the workup and early supportive treatment.

Pearls:

  • Interstitial lung diseases are a large and diverse group of ailments that can be divided into four main groups: smoking-related, steroid-responsive, connective tissue-related and exposure-related.

  • Diagnosis includes pulmonary function tests and high resolution CT.

  • Treatment is dependent on the type. Do not start steroids until you know what you’re dealing with because it may cause further complications and muddy the diagnostic window.

 

  • Interstitial Lung Disease (aka: diffuse parenchymal lung disease): diverse and large group of lung diseases (more than 140 different ones) that can be thought of as follows:

    • 1. Smoking-related

    • 2. Steroid-responsive

    • 3. Connective tissue-related

    • 4. Exposure-related

  • Clinical presentation concerning for ILD

    • Shortness of breath or dyspnea: may initially be with exertion and then progress to be even at rest.

    • Cough

    • Headache given chronic hypoxia

    • Back tightness and back pain

    • Crackles on lung exam (velcro-like or dry crackles)

    • Non-acute, insidious process

  • Smoking-related ILD:

    • Types:

      • Desquamative interstitial pneumonia (DIP)

      • Respiratory bronchiolitis interstitial lung disease (RBILD)

      • Pulmonary Langerhans cell histiocytosis (PLCH)

      • Idiopathic pulmonary fibrosis (IPF): whole upcoming episode on this diseases

    • Thought of as a continuum of diseases in all people who smoke but not all progress to interstitial lung disease

    • Counseling: STOP SMOKING. Definitive treatment for PLCH is smoking cessation.

  • Steroid-responsive ILD:

    • Types:

      • Sarcoidosis

        • Thought to be genetic predisposition plus environmental trigger but exact cause is unknown

        • 6-9 months of early prednisone taper results in symptoms improvement and resolution of abnormalities found on imaging

      • Organizing pneumonia - if no cause is found it is called cryptogenic organizing pneumonia

        • Lung heals itself from the outside in, so you get peripheral sparing on CT scan

        • Pearl: Often these patients have presented with fever, cough and consolidation on imaging. They are treated with antibiotics and their symptoms don’t resolve. They then have a biopsy or further imaging for diagnosis and treatment with steroids.

      • Eosinophilic pneumonia

        • Steroids dampen the eosinophilic response

      • Hypersensitivity pneumonia

        • Needs to remove the trigger and treat with steroids

  • Connective tissue-related:

    • Types:

      • Rheumatoid arthritis: often times presents with RA symptoms first or the lung disease is caught first while on hindsight they had typical RA symptoms all along

      • Lupus

      • Scleroderma

      • Polymyositis

      • Dermatomyositis

    • Treatment: if you treat the underlying connective tissue disease you also treat the lung disease; hence, serologies to identify the underlying disease is really important.

      • Often times steroid-sparing agents like methotrexate

  • Exposure-related:

    • Types:

      • Asbestos: huge latency between exposure and disease onset

      • Amiodarone

      • Coal

      • Silica

      • Beryllium

    • Treatment: remove the exposure and protect against future exposure

  • Focus of history:

    • Characterize dyspnea severity (flight of stairs, walk to mailbox), duration and compensation

    • Characterize cough

      • Sputum color

      • Chronic

      • Worse at night, humid or dry settings, laying flat, taking a shower

  • Physical exam:

    • General breathing pattern at rest and with conversation

    • Ambulatory oxygenation

    • Thorough skin exam

    • Lung and cardiac exam

  • Work-up:

    • Full set of pulmonary function tests looking for restrictive lung pattern →

      • Decreased forced expiratory volume in one second (FEV1), forced vital capacity (FVC) and decrease in total lung capacity (TLC). Normal or even elevated ratio of FEV1 to FVC.

      • May also see decrease in diffusion lung capacity (DLCO)

    • Often will order inspiratory and expiratory mouth pressures because it tells whether or not they are having any weakness associated with the ILD

    • High resolution CT scan

    • May also do inspiratory/expiratory or prone/supine films to see how much air trapping there is

  • Treatment:

    • If hypoxic, give them oxygen for at rest or with exertion

    • Don’t start steroids unless you know you have a steroid-responsive interstitial lung disease

    • Referral to pulmonology

    • Health maintenance: flu and pneumonia vaccine

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Good Samaritans Full episode audio for MD edition 206:06 min - 97 MB - M4AHippo PC RAP August 2018 Written Summary 511 KB - PDF

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