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Interstitial Pulmonary Fibrosis (Signs and Symptoms (SOB, Dry…
Interstitial Pulmonary Fibrosis
Pathophysiology
Repeated damage to alveolar epithelial
Normal wound healing should occur
Intact epithelium should be reestablished
Levels of interleukins 1 and 8 and tumor necrosis factor-alpha are increased
Interstitial inflammatory response: WBC (neutrophils & lymphocytes) infiltration
Cytokines cause increased vascular permeability
Changes in alveolar capillary units
Aberrant activity of alveolar cells
Increase in alveolar epithelial cells
Activation of mesenchymal cells
Differentiate to myofibroblasts making collagen
Collagen deposition
Formation of fibroblastic foci
Honeycombing- widespread fibrosis of small cystic spaces with irregularly thickened walls composed of fibrous tissue
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Alveolar epithelial cells release fibrogenic cytokines and growth factors
Cytokines and growth factors transform fibroblasts into myofibroblasts
Recruitment of additional inflammatory mediators
Direct cellular damage
Cytokines and growth factors are
key factors in fibrogenesis
Myofibroblasts create extracellular matrix
Myofibroblasts don’t undergo apoptosis
Too much extracellular matrix produced
Pathological scar formation
Fibroblasts become apoptosis-resistant and contribute to progressive fibrosis
Destruction and death of the normal lung architecture
Decreased lung/alveolar compliance
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Hypoxemia
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Exposure to environment pollutant (moldy hay) + Hx of smoking/ secondary smoke exposure
Exposure to thermophilic actinomycetes/ fungi
Triggers an immunological response (IgG response)
Additional a cytotoxic CD8+ lymphocyte response
Lymphocyte mediation also triggered (Th1 mediation)
Evidence of lymphocyte stimulation is considered as diagnostic proof of the disease
Farmer's Lung
Subacute
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Chronic
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Acute
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Pulmonary Fibrosis
Medical Management
Diagnosis
High-resolution CT
Fine bibasilar inspiratory crackles
Pulmonary hypertension at rest
Chest X-Ray
Lab tests
Antinuclear antibodies, rhematoid factors
Elevated CRP & ESR
CBC: Polycythemia
ABG: hypoxemia
Pulmonary function tests: restrictive ventilatory defect, reduced diffusion capacity
Blood test
Bronchoscopy
Medications
Corticosteroids
Immunosuppresants
Supplemental O2
Antifibrotics
Tyrosine kinase inbibitors
Surgery
Lung transplant
Signs and Symptoms
SOB
Dry nonproductive cough
Chest tightness
Reduced diffusing capacity
Weight loss
Fever
Fatigue
Pain: arthralgia, myalgia
Dyspnea
Clubbing of finger tips/ toes
Risk factors
50-70 years of age
Occupational/ environmental toxins
GERD
Hx of Smoking
Chemotherapy
Radiation therapy
Viruses