Restrictive lung diseases

Restrictive lung diseases

Restrictive lung diseases

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Restrictive Lung Dx - The Suffocating Squeeze

Characterized by ↓ lung compliance & volume; patients can't get air IN. Key feature is a normal or ↑ FEV1/FVC ratio (≥ 0.8), with ↓ FVC & TLC.

  • Intrinsic (Pulmonary Parenchyma):
    • Idiopathic Pulmonary Fibrosis (IPF): "honeycombing" on CT.
    • Sarcoidosis, Pneumoconioses (asbestosis, silicosis).
  • Extrinsic (Extra-parenchymal):
    • Chest wall (kyphoscoliosis), obesity.
    • Pleural disease (effusions).
    • Neuromuscular weakness (e.g., Guillain-Barré).

CT and histology of idiopathic pulmonary fibrosis

⭐ Both FEV1 and FVC are reduced, but FVC is reduced more, leading to a preserved or increased FEV1/FVC ratio.

Idiopathic Fibrosis - Scarred for Life

  • Chronic, progressive, fibrosing interstitial pneumonia of unknown cause, typically affecting older adults (>60 years).
  • Clinical: Insidious onset dyspnea, dry cough, digital clubbing, and characteristic bibasilar "Velcro-like" crackles.
  • Pathology: Histological pattern is Usual Interstitial Pneumonia (UIP).
    • Patchy, peripheral, subpleural fibrosis.
    • Temporal heterogeneity: areas of established fibrosis (collagen) adjacent to early fibroblastic foci.
    • Late stage: Honeycomb lung (cystic fibrotic airspaces).

UIP Histopathology: Fibroblastic Foci & Honeycomb Lung

Exam Favorite: The hallmark of UIP is spatial and temporal heterogeneity, meaning scarred and normal lung tissue coexist, reflecting ongoing, asynchronous injury.

  • Prognosis: Poor (median survival 3-5 years).
  • Tx: Pirfenidone, Nintedanib (slow decline); Lung transplant definitive.

Pneumoconioses - Dust Bunnies' Revenge

Chronic fibrosing lung diseases from inhalation of mineral dusts, leading to inflammation and interstitial fibrosis. Primarily affects workers in specific industries.

  • Asbestosis:
    • Source: Insulation, shipbuilding, roofing.
    • Pathology: Affects lower lobes. Ferruginous bodies (asbestos fibers coated with iron/protein), calcified pleural plaques.
  • Silicosis & Coal Workers' (CWP):
    • Source: Mining, sandblasting (Silica); Coal mining (CWP).
    • Pathology: Affects upper lobes. Macrophages ingest particles, leading to fibrosis.
    • 📌 Mnemonic: Asbestos from the roof (insulation) hits the base (lower lobes); Silica/Coal from the earth hit the roof (upper lobes).
  • Berylliosis:
    • Source: Aerospace, electronics manufacturing.
    • Pathology: Non-caseating granulomas, mimicking sarcoidosis.

⭐ Silicosis is associated with "eggshell calcification" of hilar lymph nodes and impairs macrophage function, increasing susceptibility to Tuberculosis.

Granuloma Gangs - Sarcoid & Friends

  • Sarcoidosis: Multisystem disorder defined by non-caseating granulomas, common in African American females.

    • Path: Unknown antigen drives a CD4+ T-cell response.
    • Features: Dyspnea, cough, uveitis, erythema nodosum. Bilateral hilar lymphadenopathy on CXR.
    • Labs: ↑ Serum ACE, ↑ CD4+/CD8+ ratio (>2:1) in BAL fluid, hypercalcemia.
    • Histo: Tightly-formed granulomas; may show Schaumann or asteroid bodies. Chest X-ray: Bilateral hilar lymphadenopathy in sarcoidosis

    ⭐ Hypercalcemia is due to PTH-independent production of active Vitamin D by epithelioid macrophages in granulomas.

  • Hypersensitivity Pneumonitis (HP): Immune reaction to inhaled organic antigens (e.g., Farmer's Lung).

    • Histo: Poorly-formed non-caseating granulomas.
  • Berylliosis: Occupational lung disease (aerospace, electronics) that mimics sarcoidosis; history is crucial.

High‑Yield Points - ⚡ Biggest Takeaways

  • Characterized by ↓ lung volumes (especially TLC) and a normal or ↑ FEV1/FVC ratio.
  • Presents with progressive dyspnea due to stiff, non-compliant lungs from interstitial fibrosis.
  • Idiopathic Pulmonary Fibrosis (IPF) is the most common, showing honeycombing on imaging.
  • Sarcoidosis is marked by non-caseating granulomas and bilateral hilar lymphadenopathy.
  • Asbestosis classically affects the lower lobes and is linked to mesothelioma.
  • Hypersensitivity pneumonitis is a reaction to inhaled organic dusts.

Practice Questions: Restrictive lung diseases

Test your understanding with these related questions

A 61-year-old male presents to your office with fever and dyspnea on exertion. He has been suffering from chronic, non-productive cough for 1 year. You note late inspiratory crackles on auscultation. Pulmonary function tests reveal an FEV1/FVC ratio of 90% and an FVC that is 50% of the predicted value. Which of the following would you most likely see on a biopsy of this patient's lung?

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Flashcards: Restrictive lung diseases

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_____ is fibrosis of the lung interstitium with unknown cause

TAP TO REVEAL ANSWER

_____ is fibrosis of the lung interstitium with unknown cause

Idiopathic pulmonary fibrosis

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