Title: Extrinsic allergic alveolitis Hypersensitvity pneumonitis ???????????????????
1Extrinsic allergic alveolitisHypersensitvity
pneumonitis???????????????????
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2HP Introduction
- immunologically induced lung disease
- diffuse inflammation of lung parenchyma airways
in previously sensitized patients - sensitized to repeated inhalation of dusts
containing organic low molecular weight
chemical antigens
3HP Introduction
- dusts derived from
- dairy grain products
- animal dander proteins
- wood bark
- water reservoir vaporizers
- not atopic disease
- not associated with increase IgE or eosinophils
4HP Selected etiological agents
Disease Farmers lung ????????? Bagassosis ???????
??? Bird-breeders lung Bird-fanciers
lung Pigeon-breeders lung Mushroom-workers
lung ?????????? Humidifier/air conditioner lung ??
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Source ????????????????? ???????????????? ?????,
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Antigen Micropolyspora faeni Thermoactinomyces
sacchari Avian proteins Thermoactinomyces
vulgaris Micropolyspora faeni Thermoactinomyces
vulgaris
5HP Epidemiology
- Varies
- 0.5-5 of farmers (Farmers lung disease)
- 8-30 of members of pigeon breeding clubs
(pigeon breeders disease) - Prevalence Farmers lung
UK France Finland U.S.A.
420-3000 4370 1400-1700 540
cases/100,000 /
persons at
risk
6Bagassosis A Report of 8 Cases
?????????????????? 251757468-74.
- Pee Kamtorn, M.D.
- Poonkasem Charoenpan, M.D.
- Yosvi Sukumalchantra, M.D. ,
F.A.C.P. , F.R.C.P. (C) ,F.A.C.C. - Vijitr Boonpucknavig, M.D.
- Kalyanakit Kitiyakara, M.B. , B.S. ,
M.R.C.S. , L.R.C.P. - Chirotchana Suchato, M.D.
- Chaivej Nuchprayoon, M.D.
- Bagassosis is classified as an extrinsic
allergic - pneumonia. It is considered to be the
result of - allergic reaction to moldy sugar cane
inhalation(1) .
Case Report All of the patients worked at
a paper produc- tion factory in Karnchanaburi
province and were
7HP Pathogenesis
- immune - complex mediated process
- precipitating Ab against specific Ag
- 50 of asymptomatic persons exposed also have Ab
8HP Pathogenesis
- cell - mediated immunity more important
- response
- increase PMN in alveoli small airways
- influx of mononuclear cells
- formation of granulomas
- cytokine from T- lymphocytes macrophages
9HP Histologic findings
- diffuse interstitial infiltrate lymphocytes,
macrophages, mast cells, plasma cells - scattered noncaseating granulomas
- cellular inflammation of bronchioles,
bronchiolar obstruction - absent generalized vasculitis, necrotizing
granulomata - duration or stage of disease, adequacy of
biopsy sample
10HP Diagnostic criteria
- Major criteria
- 1. Symptoms c/w HP , appear or worsens within
hours after Ag exposure - 2. Confirmation of exposure to the offending
agent by - - Hx
- -investigation of the environment
- -serum precipitin test
- -BAL Ab
11HP Diagnostic criteria
- 3. Compatible CXR or HRCT
- 4. Lymphocytosis in BAL
- 5. Compatible histologic changes
- 6. Positive natural challenge or by
controlled inhalational challenge
12HP Diagnostic criteria
- Minor criteria
- 1. Basilar crackles
- 2. Decreased diffusion capacity
- 3. Arterial hypoxemia, at rest or with exercise
13HP Diagnostic criteria
- Four major criteria
- Two minor criteria
- Other diseases have been excluded
- Adapted from Schuyler Cormier Chest 1997 111
534-6.
14HP Diagnosis
- often unrecognized misdiagnosed
- respiratory symptoms with Hx. of
- environmental
- occupational exposure
- respiratory symptoms with episodic radiographic
infiltrates Recurrent pneumonia
15HP Radiographic findings
- vary to the stage of disease
- acute HP
- bilateral micronodular (1-4 mm.) infiltrates
- patchy ground-glass opacities
- decreased attenuation (air trapping from
bronchiolitis) and mosaic pattern
(expiratory view)
16HP Radiographic findings
- Subacute HP
- fine linear shadows, small nodules
reticulonodular appearance - Chronic HP
- volume loss
- reticulonodular infiltrates
- honeycombing
- predominantly upper mid lung zones
17HP Pulmonary function tests
- restrictive changes
- (superimposed obstruction in chronic HP)
- decreased diffusing capacity
- ABG increased alveolar-arterial oxygen
gradient - frank hypoxemia (severe cases)
- oxygen desat. with exercise (clue in
suspected case)
18HP BAL fluid
- intense lymphocytosis
- predominantly CD 8 T-suppressor cells
- timing of the last antigen exposure, stage of
disease
19HP key features
Immunopathology alveolitis, immune
complex granulomas, bronchiolitis lymphocytic
infiltration, fibrosis,air space destruction
Prognosis good good good
Time frame 4-48 hr weeks to 4 M. 4 M. to years
Acute Subacute Chronic
Clinical features fever, chills, cough hypoxemia,
aches dyspnea , cough, episodic flares dyspnea,
cough, fatigue, weight loss
HRCT ground glass infiltrates micronodules, air
trapping fibrosis, honey combing, emphysema
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22HP Differential diagnosis
- Acute stage
- pneumonia
- acute tracheobronchitis
- organic dust toxic syndrome
- BOOP
23HP Differential diagnosis
- Subacute stage
- recurrent pneumonia
- granulomatous lung diseases
- pneumoconiosis
- Wegeners granulomatosis
24HP Differential diagnosis
- Chronic stage
- IPF
- bronchiectasis
- COPD with pulmonary fibrosis
- MAC
25HP Management
- early diagnosis
- avoidance of further exposure
- protective devices - personal respirators
- relocation to a new job
- reducing microorganism contamination in the
environment - altering handling storage
- wetting compost
- using antibiotics to decrease fungal growth
- preventive maintenance on all A/C equipment
26HP key features
Immunopathology alveolitis, immune
complex granulomas, bronchiolitis lymphocytic
infiltration, fibrosis,air space destruction
Prognosis good good good
Time frame 4-48 hr weeks to 4 M. 4 M. to years
Acute Subacute Chronic
Clinical features fever, chills, cough hypoxemia,
aches dyspnea , cough, episodic flares dyspnea,
cough, fatigue, weight loss
HRCT ground glass infiltrates micronodules, air
trapping fibrosis, honey combing, emphysema
27Subacute HP, a 60-year-old dairy farmer had a
8-year history of intermittent dyspnea. CXR shows
bilateral reticulonodular interstitial
infiltration.
28Chronic pigeon breeders disease. This patient
has extensive pulmonary fibrosis cor pulmonale.
29Chronic HP, Pigeon breeders disease. Bilateral
reticulonodular densities are present.
30Acute HP, ground glass opacification
31HRCT of a patient with Chronic HP demonstrating
centrilobular nodules not associated with
bronchovascular bundles.
32Chronic HP, centrilobular nodules.
33Chronic HP, honeycombing in right upper lung
traction bronchiectasis
34Acute HP, mononuclear infiltration noncaseating
granulomas.
35Chronic HP, mostly lymphocytic cellular
infiltrate with epithelioid cells numerous and
clearly defined granuloma. (Wright-Giemsa)
36Giant cells are characteristic feature of HP.
37Chronic HP shows interstitial inflammation
associated with fibrosis.