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Extrinsic allergic alveolitis Hypersensitvity pneumonitis ปอดอักเสบภูมิไวเกิน. ศ . น . พ . อรรถ นานา คณะแพทยศาสตร์ศิริราชพยาบาล มหาวิทยาลัยมหิดล. HP: Introduction. immunologically induced lung disease diffuse inflammation of lung parenchyma & airways in previously sensitized patients
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Extrinsic allergic alveolitisHypersensitvity pneumonitisปอดอักเสบภูมิไวเกิน ศ.น.พ. อรรถ นานา คณะแพทยศาสตร์ศิริราชพยาบาล มหาวิทยาลัยมหิดล
HP: 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
HP: 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
HP: Selected etiological agents Disease Farmer’s lung ปอดชาวไร่ Bagassosis โรคชานอ้อย Bird-breeder’s lung Bird-fancier’s lung Pigeon-breeder’s lung Mushroom-worker’s lung คนเพาะเห็ด Humidifier/air conditioner lung ปอดเครื่องทำความชื้น ปอดอักเสบการระบาย อากาศ Source หญ้าแห้งที่ขึ้นรา ชานอ้อยที่ขึ้นรา มูลนก, ขน (พิราบ,นกแก้ว) ปุ๋ยที่ขึ้นรา,หญ้าแห้ง เครื่องทำความชื้น ท่อเครื่องปรับอากาศ Antigen Micropolyspora faeni Thermoactinomyces sacchari Avian proteins Thermoactinomyces vulgaris Micropolyspora faeni Thermoactinomyces vulgaris
HP: Epidemiology • Varies • 0.5-5% of farmers (Farmer’s lung disease) • 8-30% of members of pigeon breeding clubs (pigeon breeder’s disease) • Prevalence Farmer’s lung UK France Finland U.S.A. 420-3000 4370 1400-1700 540 cases/100,000 / “ “ “ persons at “ “ “ risk “ “ “
Bagassosis : A Report of 8 Cases จดหมายเหตุทางแพทย์ 2517:57;468-74. 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
HP : Pathogenesis • immune - complex mediated process • precipitating Ab against specific Ag • 50% of asymptomatic persons exposed also have Ab
HP: 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
HP : 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
HP : 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
HP : Diagnostic criteria 3. Compatible CXR or HRCT 4. Lymphocytosis in BAL 5. Compatible histologic changes 6. Positive “natural challenge” or by controlled inhalational challenge
HP : Diagnostic criteria Minor criteria 1. Basilar crackles 2. Decreased diffusion capacity 3. Arterial hypoxemia, at rest or with exercise
HP : Diagnostic criteria Four major criteria Two minor criteria Other diseases have been excluded Adapted from Schuyler + Cormier Chest 1997; 111: 534-6.
HP : Diagnosis • often unrecognized & misdiagnosed • respiratory symptoms with Hx. of • environmental • occupational exposure • respiratory symptoms with episodic radiographic infiltrates “Recurrent pneumonia”
HP : 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)
HP : Radiographic findings • Subacute HP • fine linear shadows, small nodules = reticulonodular appearance • Chronic HP • volume loss • reticulonodular infiltrates • honeycombing • predominantly upper & mid lung zones
HP : 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)
HP : BAL fluid • intense lymphocytosis • predominantly CD 8+ T-suppressor cells ~ timing of the last antigen exposure, stage of disease
HP: key features Immunopathology alveolitis, immune complex granulomas, bronchiolitis lymphocytic infiltration, fibrosis,air space destruction Prognosis good good good Acute Subacute Chronic Time frame 4-48 hr weeks to 4 M. 4 M. to years 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
HP : Differential diagnosis • Acute stage • pneumonia • acute tracheobronchitis • organic dust toxic syndrome • BOOP
HP : Differential diagnosis • Subacute stage • recurrent pneumonia • granulomatous lung diseases • pneumoconiosis • Wegener’s granulomatosis
HP : Differential diagnosis • Chronic stage • IPF • bronchiectasis • COPD with pulmonary fibrosis • MAC
HP: 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
HP: key features Immunopathology alveolitis, immune complex granulomas, bronchiolitis lymphocytic infiltration, fibrosis,air space destruction Prognosis good good good Acute Subacute Chronic Time frame 4-48 hr weeks to 4 M. 4 M. to years 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
Subacute HP, a 60-year-old dairy farmer had a 8-year history of intermittent dyspnea. CXR shows bilateral reticulonodular interstitial infiltration.
Chronic HP, Pigeon breeder’s disease. Bilateral reticulonodular densities are present.
HRCT of a patient with Chronic HP demonstrating centrilobular nodules not associated with bronchovascular bundles.
Chronic HP, honeycombing in right upper lung & traction bronchiectasis
Acute HP, mononuclear infiltration & noncaseating granulomas.
Chronic HP, mostly lymphocytic cellular infiltrate with epithelioid cells & numerous and clearly defined granuloma. (Wright-Giemsa)
Chronic HP shows interstitial inflammation associated with fibrosis.