Updated: 10/23/2019

Other Restrictive Etiologies

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Collagen Vascular Disease
  • Systemic lupus erythematosus
    • pleuritis + pleural effusions
  • Rheumatoid arthritis (rheumatoid lung disease)  
    • pleuritis + pleural effusions
    • also see pulmonary fibrosis (restrictive pattern)
    • bilateral, diffuse appearance on chest radiograph
      • progress to honeycomb lung in severe disease
    • presentation
      • gradual onset dyspnea
      • end-inspiratory rales at lung base
      • lung biopsy shows patchy interstitial lymphoid infiltrate into walls of alveolar units
    • NOT associated with rheumatoid nodules in the lung
      • this finding is associated with Caplan's syndrome (see Pneumoconioses )
  • Systemic sclerosis (scleroderma)
    • may also present with CREST syndrome (a variant of scleroderma)
    • interstitial fibrosis due to deposition of collagen
      • triggered by increased TGF-beta secreting T-cells accumulating in lungs 
      • mainly involve capillaries and small arterioles
    • may lead to pulmonary hypertension and cor pulmonale
Iatrogenic Causes
  • Drug-associated 
    • anti-cancer agents
      • bleomycin/busulfan, methotrexate, nitrosourea, and cyclophosphamide
    • anti-arrhythmics
      • amiodarone
  • Radiation-induced
    • post-treatment pneumonitis (1-6 months following)
    • associated with fever, dyspnea, and pleural effusions
Occupational Causes
  • Silicosis 
    • associated with occupational exposures of sandblasting, mining, and stone fabrication
  • Silo filler's disease
    • hypersensitivity pneumonitis to nitrogen oxide gases released by plant matter
  • Byssinosis
    • hypersensitivity penumonitis to textile dusts (including cotton, hemp, and linen)
  • Farmer's lung 
    • hypersensitivity pneumonitis to Saccharopholyspora rectivirgula (thermophilic actinomyes)
      • found in moldy hay
      • type III hypersensitivity reaction with antigen-antibody complex depositing in lung
      • can become type IV hypersensitivity reaction with chronic exposure
Idiopathic Pulmonary Fibrosis
  • Introduction
    • most common group of idiopathic interstitial pneumonia
    • chronic alveolitis with no known cause
    • persistent inflammation results in fibrosis (due to fibroblast proliferation) and cyst formation that is most prominent in subpleural regions (lower lung predominantly) 
    • typically seen in men 40-70 years old
    • requires transplant at most advanced stages
  • Presentation 
    • dyspnea on exertion most common symptom
    • gradual onset of dry cough
      • subpleural cystic enlargement = "honeycomb" lung
    • diagnosis can be made by HRCT if underlying causes excluded
      • surgical lung biopsy shows usual interstitial pneumonia (UIP)
Pulmonary Alveolar Proteinosis
  • Idiopathic pathology where proteinaceous material fills the avleoli
    • can be caused by primary (idiopathic) or secondary (infectious/malignancies) causes
  • Pathophysiology
    • defect in clearance (macrophages) or production of surfactant 
      • macrophage defect and atypical infections sometimes seenin this pathology
 

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(M1.PL.15.141) A 61-year-old male visits his primary care physician because of dyspnea that has worsened over several months. His other medical problems include essential hypertension and rheumatoid arthritis, but he refuses to take medication for either. He denies using alcohol, tobacco, or other drugs. Physical examination reveals bilateral end-inspiratory rales at the lung bases. Chest radiograph is shown in Figure A. Which of the following is the most likely explanation for this patient’s symptoms? Tested Concept

QID: 100944
FIGURES:
1

Chronic bronchitis

4%

(2/55)

2

Tension pneumothorax

4%

(2/55)

3

Pulmonary embolism

0%

(0/55)

4

Pulmonary fibrosis

82%

(45/55)

5

Mitral stenosis

7%

(4/55)

M 2 D

Select Answer to see Preferred Response

(M1.PL.13.52) 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? Tested Concept

QID: 100855
1

Subpleural cystic enlargement

22%

(27/121)

2

Charcot-Leyden crystals

17%

(20/121)

3

Hyaline membranes

37%

(45/121)

4

Arteriovenous malformations

2%

(2/121)

5

Anti-GBM antibodies

18%

(22/121)

M 2 E

Select Answer to see Preferred Response

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