Singh Pathology Respiratory Pathology #3 Flashcards
What is the most common type of interstitial lung disease processes?
- Idiopathic pulmonary fibrosis
- This damages pulmonary tissue with waves of inflammatory injury leading to fibrosis
What does Usual Interstitial Pneumonia consist of and what is it?
- Pathological diagnosis of IPF
- Consists of normal areas, inflammation, fibroblast foci and peripheral honeycombing
What factors contribute to getting IPF?
- Environmental such as industrialized societies and smoking
- Genetics
- Increasing age
Clinical findings in Idiopathic pulmonary fibrosis?
- Dyspnea
- Crackles (velcro like)
- Restrictive PFT
- Basilar infiltrates progressing to honeycomb pattern
How can you differentiate emphysema honeycombing vs IPF?
thick areas between fibrosis on histology and grossly differentiate IPF from emphysema
Prognosis of IPF? Therapy?
- Progressive- most patients die 3-5 yrs
- Therapies include
- lung transplant
- Tyrosine kinase inhibitors
- TGF-B inhibitors
What is Non specific Interstitial Pneumonia? What makes up the histology?
- Idiopathic pneumonia
- Unique histology
- Uniform infiltrates and fibrosis
- NO heterogeneity
- NO fibroblast foci
- NO granulomata
Describe Cryptogenic Organizing Pneumonia.
- Superimposed on prior infection or inflammatory process
- Presents with a pneumonia like consolidation in 50-60s
Why is COP a diagnosis of exclusion?
- Not an active infection, drug or toxin induced or related to CTD
- If you give steroids it clears up!
- Good prognosis with full recovery
If a patient has RA, systemic sclerosis, or SLE and develops IPF, NSIP, or COP how do you diagnose these patients?
- Connective Tissue Disease ILD
- prognosis is based on the CTD the person has
What two diseases have a prominent granulomatous pattern?
- Sarcoidosis
- Hypersensitivity Pneumonia
What is sarcoidosis? How does it present?
- Non caseating granulomata in various organs
- mostly in lungs or hilar lymph nodes
- Presents incidentally on radiopgraph or with dyspnea
What is this?
Schaumann bodies associated with Sarcoidosis
What is this?
Asteroid body associated with sarcoidosis
Clinical presentation of patients with Sarcoidosis?
- <40 yr old
- 10x higher in African Americans
- Elevated ACE levels
- Likely immune related etiology possible genetic presdisposition