Airway Inflammatory Disorders

Airway Inflammatory Disorders include a wide spectrum of conditions including:

  • Kartagener syndrome
  • Difficult asthma (severe persistent asthma)
  • Vocal cord dysfunction (uncontrolled closing of the vocal cords when you breath in)
  • Spasmodic croup
  • Reflexive vasomotor disease
  • Autonomic disorders
  • and many more

Chronic diseases such as GERD and chronic sinusitis can be a precursor but are not the primary cause.
“Blausen 0620 Lungs NormalvsInflamedAirway” by BruceBlaus.

The primary causes of Airway Inflammatory Disorders include:

  • Airway mucosal inflammation
  • Airway inflammatory debris
  • Upper and lower airway anatomy
  • Upper and lower airway lymphatic tissue


Orphan Disease Evolution of Airway Inflammatory Disorders and Diagnosis

  • The circular diagnosis results in a disgruntled sickly patient and a frustrated primary care provider (See Figures 1, 2, and 3)
    1. Poor recognition of one condition or the overtreatment of one condition results in the “diagnosis” cascade
    2. Late into the condition adverse drug effects and/or systemic deconditioning results in the frustrated physician response of “it’s all in your head”

 chart 1

 Figure 1.

figure 1

 Figure 2.

figure 2

 Figure 3.


Current Treatments for Airway Inflammatory Disorders

  • Many anecdotal treatments (not based on hard facts) and very few studied in a comprehensive, peer reviewed manner

TPIRC’s Novel Diagnostic & Treatment Approaches

  • Define disease spectrums (avoid lumped diagnoses)
  • Define involvement in specific systems (dysmotility)
  • Define scoring systems or criteria for diagnosis
  • Approach treatment patterns to similar conditions (tourette’s = chronic cough)
  • Multidisciplinary approach to research involving appropriate specialists 

TPIRC’s Clinical Care Goals

      • Many airway inflammatory disorders have clinical sites of care (ie difficult asthma, etc.)
      • Coordination of care between multiple specialists and a specific airway condition will be the purpose of TPIRC

    • For patients with Kartagener’s disease, sinus and lung involvement is severe. A combined clinic setting where sinus experts (ENT) and lung experts can comprehensively manage patients provides a unique aspect to orphan disease care in Southern California

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