Diffuse Interstitial Lung Dz Flashcards
UIP Histology & HRCT Findings
Histology: Fibroblasts, peripheral patchy fibrosis, honeycombing, +/- inflammation
HRCT: Subpleural/basal predominance, reticular changes, traction bronchiectasis, +/- honeycombing
NSIP Histology & HRCT Findings
Histology: Homogenous, chronic interstitial inflammation/fibrosis
HRCT: Homogenous, GGOs, reticular, +/- subpleural sparing
OP Histology & HRCT Findings
Histology: Patchy, granulation tissue plugs
HRCT: Patchy consolidation / GGOs, Atoll sign (reverse halo sign)
RBILD / DIP Histology & HRCT Findings
Histology: Pigmented (smoker’s) Macrophages, bronchiolocentric or diffuse
HRCT: Vague nodules, patchy to diffuse GGOs
DAD Histology & HRCT Findings
Histology: hyaline membranes, granulation tissue, organizing fibroproliferative
HRCT: Diffuse consolidation / GGOs
LIP Histology & HRCT Findings
Histology: Lymphoid, plasma cells
HRCT Findigns: Patchy GOs, nodules, cysts
PPFE Histology & HRCT Findings
Histology: Pleural / subpleural fibroelastosis, septal elastosis
HRCT: Upper / subpleural fibrosis, pneumomediastinum, pneumothorax
Five smoking-related IIPs
- RB-ILD
- DIP
- PLCH (Pulm Langerhan’s Cell Histiocytosis)
- AEP (Acute Eosinophilic Pneumonia)
- CPFE (Combined Pulm Fibrosis & Emphysema)
Two anti-fibrotic therapies for IPF
Nintedanib (Ofev)
Pirfenidone (Esbriet)
Age rage for IPF
50-80 years
IIPs which are steroid responsive
- RBILD
- DIP
- COP (recurs)
- AIP
Most connective tissue diseases cause ____ (form of IIP), except Sjogren’s, which causes ______.
- NSIP
2. LIP
Antisynthetase Syndrome antibody
Anti-Jo-1
Score for IPF Mortality
GAP Score
- Gender
- Age
- PFTs (FVC, DLCO)
Typical UIP HRCT Pattern
- Subpleural/basal predominance, heterogenous
- Reticular abnormalities
- Honeycombing +/- traction bronchiectasis
- Absence of GGOs, extensive consolidation, mosaic attenuation, nodules, cysts
Typical UIP Histology Pattern
- Subpleural or paraseptal predominance
- Patchy involvement
- Dense fibrosis / architectual distortion +/- honeycombing
- Fibroblast foci
- Absence of other features (granulomas)
Age for eligibility of clinical Dx of IPF
60yo
Acute IIPs
- OP / COP
- DAD / AIP
PFT abnormality in CPFE (Combined Pulmonary Fibrosis & Emphysema)
- Low DLCO
- Preserved spirometry & lung volumes
Term for when IIP is first manifestation of CTDz
IPAF (Interstitial Pneumonia w/ AI Features)
Types of granulomas in Sarcoidosis
Noncaseating, epitheloid cell granulomas w/o other cause
Epidemiology of Sarcoidosis
- Gender?
- Race?
- Nationality
- Women
- African Americans
- Scandinavians
Screen for extrapulmonay sarcoid with what testing?
EKG definitely. Consider TTE.
Staging of Sarcoid 0-4
0 = no lung involvement 1 = bilateral hilar lymphadenopathy w/o parenchymal involvement 2 = bilateral hilar lymphadenopathy w/ parenchymal involvement 3 = parenchymal involvement w/o hilar lymphadenopathy 4 = progressive fibrosis +/- cavitary lesion or cysts
Mortality from sarcoid due to what 3 things?
- Progressive pulmonary fibrosis
- Aspergilloma bleed
- Cardiac involvement
GLILD (Granulomatous Lymphocytic ILD) in what characteristic patient population
Severe immunosuppression, usually CVID
GLILD (Granulomatous Lymphocytic ILD) histologic features?
non-necrotizing granulomas, LIP< follicular bronchiolitis
GLILD (Granulomatous Lymphocytic ILD) treatment?
steroids, immunosuppression, IVIG
Treatment for cough in Sarcoidosis?
ICS
Indications for treatment in sarcoidosis?
- Pulmonary stage II-III w/ moderate-severe PFT changes
- Extra-pulmonary disease (cardiac, neurologic, occular not responding to topical therapy, hypercalcemia)
Treatment for sarcoidosis?
Prednisone, with 6-12month taper
2nd line is immunosuppressives, usually methotrexate
