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Restrictive lung diseases

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Flashcards

Restrictive lung diseases

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Questions

USMLE® Step 1 style questions USMLE

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A 52-year-old man presents to the clinic with a dry cough and shortness of breath. He reports worsening symptoms for months and states he is otherwise healthy and does not take any medications daily. He usually drinks two beers everyday after he finishes his work as a sandblaster for the city government. Vitals are within normal limits. His physical examination is unremarkable. He undergoes spirometry which demonstrates an increased FEV1/FVC ratio. Which of the following radiographic features is most likely to be observed based on this patient’s clinical presentation?  

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A-a gradient

restrictive lung disease p. 694

Adverse effects/events

restrictive lung disease p. 694

Amiodarone p. 327

restrictive lung disease p. 694

Anthracosis p. 696

restrictive disease p. 694

Black lung disease p. 696

Busulfan toxicity p. 445

restrictive lung disease p. 694

Forced expiratory volume (FEV)

restrictive lung disease p. 694

Goodpasture syndrome p. 48, 614

restrictive lung disease p. 694

Granulomatosis with polyangiitis (Wegener) p. 616

restrictive lung disease and p. 694

Guillain-Barré syndrome

restrictive lung disease p. 694

Myasthenia gravis p. 480

restrictive lung disease p. 694

Neonatal respiratory distress syndrome (NRDS) p. 679

restrictive lung disease p. 694

Noncaseating granulomas

restrictive lung disease p. 695

Poliomyelitis p. 545

restrictive lung disease p. 694

Pulmonary fibrosis

restrictive lung disease p. 695

Sarcoidosis p. 695

restrictive lung disease p. 695

Wegener granulomatosis p. NaN, 322

restrictive lung disease p. 694

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Transcript

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Content Reviewers

Charles Davis, MD

Restrictive lung diseases are just as they sound, restrictive.

There are two types of restrictive lung diseases, interstitial and extra-pulmonary.

For the interstitial type, it refers to the lung tissue itself being damaged.

Imagine a lung being hard and stiff like tough rubber, that lung tissue won’t easily allow air to enter during inhalation, thereby reducing the lung volume.

In the extra-pulmonary type, the structures around the lung are damaged and that prevents chest expansion.

Think about how hard it would be to take a breath when you have someone sitting on your chest.

During inhalation, both the diaphragm and intercostal muscles located in between your ribs contract to pull the ribs up and out and expand the chest cavity. This creates a vacuum which pulls the lungs open.

The air reaches the alveoli and this is where the majority of gas exchange occurs in the lungs.

Between the alveoli, there’s connective tissue made up of proteins like elastin fibers, which give the lungs their rubber-band like properties, and collagen, which gives the lungs their firmness and their overall shape.

During exhalation, both the diaphragm and the intercostal muscles relax to allow the chest wall to fall and return the chest cavity back to normal.

At the same time, the elastin and collagen fibers in the interstitium allow the lung to spring back and push the air back out.

There are a number of ways to measure the volume of air as it is inhaled and exhaled from the lungs.

For example, total lung capacity is the total amount of air that the lungs can hold.

Tidal volume is the volume of air inhaled during normal inhalation and the functional residual capacity is the total amount of air left in the lungs after a normal exhalation.

There’s also the forced vital capacity, or FVC, which is the maximum amount of air exhaled after a full inhalation, and the forced expiratory volume in one second, or FEV1, which is the amount of air forcibly breathed out in one second after a maximum inhalation.

The normal ratio of FEV1 to FVC is 0.8, meaning that 80% of the air should be able to get forced out in the first second.

Summary

Restrictive lung diseases are a group of lung conditions that make it difficult for the lungs to expand fully, leading to a decrease in the amount of air that can be inhaled. This results in a decrease in lung function, leading to difficulty breathing and reduced oxygenation of the body's tissues.

Common causes of restrictive lung diseases include interstitial lung diseases such as sarcoidosis, idiopathic pulmonary fibrosis, and asbestosis, as well as neuromuscular conditions such as muscular dystrophy and scleroderma. Diagnosis is made based on spirometry and chest imaging, and treatment involves bronchodilators and corticosteroids to reduce inflammation and improve lung function, as well as oxygen therapy to increase oxygen levels in the body. In some cases, lung transplantation may be necessary.

Sources

  1. "Robbins Basic Pathology" Elsevier (2017)
  2. "Harrison's Principles of Internal Medicine, Twentieth Edition (Vol.1 & Vol.2)" McGraw-Hill Education / Medical (2018)
  3. "Pathophysiology of Disease: An Introduction to Clinical Medicine 8E" McGraw-Hill Education / Medical (2018)
  4. "CURRENT Medical Diagnosis and Treatment 2020" McGraw-Hill Education / Medical (2019)
  5. "Fishman's Pulmonary Diseases and Disorders, 2-Volume Set, 5th edition" McGraw-Hill Education / Medical (2015)
  6. "Diffuse Lung Disorders" Springer Science & Business Media (2012)
  7. "Dyspnea and Decreased Variability of Breathing in Patients with Restrictive Lung Disease" American Journal of Respiratory and Critical Care Medicine (2002)
  8. "Clinical Advances in the Diagnosis and Therapy of the Interstitial Lung Diseases" American Journal of Respiratory and Critical Care Medicine (2005)
  9. "Idiopathic non‐specific interstitial pneumonia" Respirology (2015)