Epidemiology

• Endemic Regions
  • Asia
  • Middle East
  • Eastern Europe
  • Central and South America

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Etiology

• Klebsiella rhinoscleromatis
  • Member of Klebsiella Genus (see Klebsiella)
  • Lactose-Fermenting Gram-Negative Rod

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Pathogenesis

• Chronic Inflammatory and Fibrosing Condition of Upper (and Sometimes Lower) Airways: may extend to and destroy adjacent bones and cartilage

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Pathologic Pattern

• Loosely Organized Granulomas: with plasma cell, lymphocyte, and foamy macrophage infiltration

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Diagnosis

• PFT’s: abnormal flow-volume loop
• Bronchoscopy
  • Subglottic or lower tracheal stenosis
  • Bronchial stenosis
• CXR/Chest CT Pattern
  • Normal CXR (no evidence of parenchymal lesions): usually (except possibly for post-obstructive signs)
• Nasal Mucosal Biopsy: short Gram-negative rod (difficult to find in tissue sections)

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Clinical

• Subglottic Tracheal Obstruction (see Obstructive Lung Disease)
• Atelectasis (see Atelectasis)

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Treatment

• Antibiotic Therapy: required for months-years
• Laser Bronchoscopy: may be required for some lesions

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References

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