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Non-Neoplastic Pulm Diseases (Hypersensitivity Pneumonitis (Pathology…
Non-Neoplastic Pulm Diseases
Hypersensitivity Pneumonitis
Diffuse interstitial
granulomatous
lung disease
Immunologic rxn to
inhaled organic antigens (or chemicals)
over weeks/months. E.g. Farmer's lung, Bird fancier's lung
Complex syndrome, varying intensity, presentation, natural history
Hypersensitivity III & IV
IgG Ab's against offending agent in serum
Clinical
Symps hrs-mos after exposure
Usually no Hx allergic disease
Mild/subclinical mis-dx virus or asthma
Pathology
Acute
Neutrophil infiltrate in alveoli, resp bronchioles
Diffuse alveolar damage pattern (AIP/ARDS)
Temporally uniform, nonspecific, chronic interstitial pneumonia
Subacute
lymphocytic interstitial pneumonitis
Loose granulomas (not tight like sarcoid)
Organizing pneumonia-like pattern
Fibrosis
Chronic
Diagnostic triad
Temporally uniform + Peribronchial accentuation (similar changes across lung & hug around bronchioles)
Non-necrotizing poorly formed granulomas around bronchiole interstitium
Foci of organizing pneumonia pattern
(intraluminal fibrosis)
Important enough to earn images
Peribronchial inflammation
Loosely formed granulomas
(less obvious than sarcoid, macrophages)
Fibrosis filling lumens & alveoli (fibrotic/fibroblastic plug)
Multinucleated giant cell visible (granuloma formation shitty so might not be easy to see)
Treatment
Remove cause
Corticosteroids (after r/o infection)
Irreversible if dense fibrosis + honeycombing
Usual Interstitial Pneumonia (UIP) aka Idiopathic Pulmonary Fibrosis
Clinical
Insidious onset dyspnea
Chronic, progressive downhill course
Males >> Females
Age 50-70
Risks
Cigarettes (1.6-2.3x risk)
Metal, wood dust exposure (long-term)
PE
Tachypneic
Bibasilar late inspiratory crackles (fine "velcro" rales)
Clubing, pulm HTN, cyanosis (late disease)
Treatment
Steroids
Med survival 3 yr
Worse prog w/ eosinophilia, incr fibroblastic foci
Pathology
TGF-B1 Mutation: shortens telomeres, increases cell death
Caveolin-1: inhibitor of pulm. fibrosis -- TGF-B1 mutation inhibits C-1, leading to uninhibited fibrosis
Stages
Early: hyaline membranes (may mis-dx as DAD/AIP/ARDS)
Later: loss of alveolar capillaries b/o fibrosis in septa
End: honeycomb lung (esp superior parts of lower lobes, subpleural, peripheral), broad sheets fibrosis (nl areas b/w fibrotic areas), obliterated alveoli
Histology
Fibrosis
Patchy parenchymal involvement, subplerual/septal
Fibroblastic foci
Mild interstitial chronic inflamm
Honeycomb change
"Loose, watery, fibroblastic plugs"
Diagnosis
Biopsy: Wedge (> area) best
Radiology (HR
CT
)
Clinical correlation
Non-Specific interstitial Pneumonia (NSIP)
Clinical
46-57 y.o.
Women > Men
Cellular pattern -- 26-50 y.o.
Fibrosing pattern -- 30-71 y.o.
Hx Smoking
Symps
Exertional dyspnea
Cough
Fever
PE
Crackles
Clubbing
Labs
ANA
RF (+)
Radiology
Consolidation or hazy opacities ("ground-glass") -- esp diffuse, in lower lobes
Treatment
Corticosteroids!!! (improve/recover 75% pts)
Etiology
Idiopathic
Collagen vascular/connective tissue disease (SLE, RA, polymyositis, scleroderma, Sjogren's, etc.)
Drug rxn (nitrofurantoin, amiodarone)
Pathology
Temporally uniform inflamm/fibrosis
Diffuse lung involvement
Histology - CELLULAR NSIP
Mild/mod chronic inflamm
Diffuse involvement affected parenchyma
Preserved alveolar architecture
Histology - FIBROTIC NSIP
Mild/mod interstitial chronic inflamm
Diffuse involvement affected parenchyma
Mild/mod alveolar architecture loss
Variable degree interstitial fibrosis
Uniformly thickened/fibrotic alveolar walls
Desquamative Interstitial Pneumonia (DIP)
Clinical
Cigarette smoking (90%)
Subacute onset (wks-mos)
Insidious onset dyspnea + cough
Male >> Female (2:1)
Middle age (40s-50s)
PE
Crackles (but less prominent)
Clubbing
Labs
LFT
Restrictive pattern
Reduced DLCO
ABG: Hypoxemic
BAL
Incr. total cells recovered
Mostly macrophages
Imaging
CT: Ground glass opacity (diffuse OR patchy)
Treatment
Stop smoking
Corticosteroids (mod/sev symps, gas exchange abnormalities)
Lung transplant for end-stage disease
Prognosis better than other idiopathic interstitial pneumonias (esp UIP) -- small # progress, death rare
Pathology
Histology
Uniform involvement
Macrophage accumulation
Brown pigment-laden Macrophages
Respiratory Bronchiolitis
Clinical
Heavy smokers
Bronchocentric macrophage accumulation
Bronchiolar fibrosis + chronic inflamm
Light brown cytoplasmic pigment in macrophages
Includes: desquamative interstitial lung disease, respiratory bronchiolitis interstitial lung disease (RBILD), langerhan's cell granulomatosis (LCG)
Pathology
"Leopard spots" b/o anthracotic pigment aggregation in macrophages