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Hypersensitivity Pneumonitis
Wael Batobara
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Case 1 82 y Female smoker (30 pack) Seen in OPD because of
6/12 SOBE , Cough No Orthopnea , PND ,LL swelling No CP , Wheeze , Fever ,Sputum , Wt loss No similar episode in the past Able to walk 3 blocks & climb 20 stairs
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History PMH : HTN , IHD ,Hypothyroidism
Medication: Thyroxine , Altace , Ranitidine Ventolin PSH : Partial gastrectomy for bleeding GU Works as Cook Travel to Arizona & North ON No pets No Contact with similar case
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Examination Afebrile BP 120/70 RR14 Sat93% RA
No Clubbing or lymphadenopathy Chest : N BS No wheeze Fine crackles Bilaterally basally CVS : N JVP S1+S2 +0 No Signs of Pulmonary HTN Abd & LL : NAD
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Investigation CBC & Coagulation N BUN , Creat & Lytes N LFT & UA N
Cardiac Enzyme & EKG & 2DE N CXR & CT
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Investigation ABG PH 6.42 PCO2 36 PO2 68 Hco3 24 Sat 93%
PFT : Isolated decrease in DLCO 60% 6MWT : 100 m HR 115 Sat 85% Open Lung Bx Non caseating granuloma & organizing pneumonia Sent to Mayo Clinic Hypersensitivity Pneumonitis
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Course Patient was symptomatic persistently
H/O exposure To Wheat & Flour 24 lbs /week Persistent decrease DLCO & walking hypoxemia Prednisolone 40 mg was initiated Will be seen in OPD in follow up
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Case 2 52 y Female Xsmoker (15pack) Seen In OPD because of
6 months H/O SOB ,Cough Cough non productive SOBE NO orthopnea ,PND No fever , night sweating , chest pain , wheeze No Wt Loss , anorexia
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History NO CTD symptoms No leg pain or swelling
PMH Hypothyroidism & Migraine No H/O CAP ,TB contact NO IHD risk factor Rx Thyroxine Lives in Portage la Pairie Works as animal rescue aids 25 y
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Examination Afebrile BP 120/70 HR 80 RR 16 Sat 93% RA
No clubbing ,lymphadenopathy JVP N Chest Bilateral fine crackles No wheeze CVS ,Abd & LL NAD No CTD signs
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Investigations CBC & Coagulation N BUN , Creat , Lytes N LFT N UA N
CXR , CT Chest PFT
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Patient Course Seen By Allergy / Immunology
+ve precipitant to avian protein Hypersensitivity pneumonitis Bird Fancier lung Advised to avoid exposure Steroids has not been started Given the mild symptoms & N exercise test
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Hypersensitivity Pneumonitis
Syndrome of varying presentations & natural history Immunologic reaction to an inhaled antigen mainly organic Genetic predisposition 44 Pt with HP Vs 50 Asymptomatic Vs 99 control MHC 2 & TNF alpha levels difference Am J Respir Crit Care Med 2001 Jun
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Epidemiology Data from Farmers lung & Bird fancier lung
Great variation because of geographical , seasonal & local practice Bird fancier prevalence / Farmer % Life guard in swimming pools Contaminated forced air system
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Smoking Effect Less symptoms & immune response 102 pigeon breeder
1/23 smokers +ve IgG Vs 39/65 Non smokers Clin Allergy 1985 Sep 92 dairy farmers Micropolyspora faeni antibody Non smoker 27% Vs 7% smoker Am Rev Respir Dis 1989 Sep
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Smoking Effect 12 smoker Vs 31 non smokers farmers
No difference in age ,gender ,working environment & exposure duration Acute FLD in non smoker 58% Vs 8% Chronic insidious in Smokers 92% Vs 42% 10 y survival 91% Non Sm Vs 70% Sm Persistent symptoms & radiological findings & higher reccurent episodes in Smokers Intern Med 1995 Oct
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Etiologies through droppings ,feathers
Farming ,Dairy or Cattle workers Different from Organic Dust Toxic Syndrome more common than HP occur with single day exposure & non immunologic reaction Bird ,Poultry & Animal handling through droppings ,feathers processed products or serum
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Etiologies Grain and flour processing and loading
being colonized with organisms & easily aerosolized Contaminated Ventilation & Water Source Forced air systems , heated water reservoir Spa Hot tubs, swimming pools water damaged carpet MAC as a cause in immunocompetent Construction & Lumber milling Plastic , Painting & electronic industry
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Classification Acute 6-12 hours of heavy exposure Misdiagnosed as CAP
Fever ,Chills ,Cough, SOB , CP & Malaise Tachypnea & fine crackles
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Classification Acute Symptoms will resolve with removal of
offending agent Complete radiological resolution takes weeks Elevated ESR & Immunoglobulin +ve specific Precipitating IgG BAL Lymphocytosis PFT restrictive pattern
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Classification Acute Radiologically HRCT > CXR
interstitial , micronodular middle &lower zones Bx mononuclear infiltrate poorly formed non caseating granuloma peribronchial distribution
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Classification Subacute , Intermittent Gradual symptoms & signs
More Wt loss Improvement with removal of exposure Improvement takes longer than acute
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Classification Subacute , Intermittent DLCO decreased
Radiologically Upper & middle zone Ground glass & micronodules Focal fibrosis & emphysema Bx Granuloma more formed Bronchiolitis +/- organizing Pneumonia Interstitial Fibrosis
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Classification Chronic Progressive Worsening cough & SOB
Removal of exposure yield partial relief ? Clubbing as bad prognosis 82 Pt followed for 5 years 16/44 +ve clubbing worsening PFT 5/38 -ve clubbing worsening PFT Arch Intern Med 1990 Sep
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Classification Chronic Progressive PFT Restrictive +/- obstructive
Hypoxemia exertional or at rest Radiologically Volume loss Honeycombing , Emphysema Less Ground glass Bx difficult to differentiate from IPF
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Diagnosis Exposure +ve aero/microbiological cultures
History +ve aero/microbiological cultures +ve serum precipitins Compatible Clinical & Radiological Finding Symptoms appearing or worsening after exposure Reticular .nodular or ground glass Restrictive / obstructive pattern
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Diagnosis +ve Inhalation challenge test
Rexposure either in environment or in PFT lab BAL / Bx Lymphocytosis & Low CD4/CD8 ratio Granuloma / Mononuclear Infiltrate
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Diagnosis Serum Precipitins Suggestive not diagnostic
High false –ve & +ve Used to convince about hypersensitivity Smoking Effect ?!
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Diagnosis Inhalation Challenge Test 2 reactions:
Common 6-8 hours post challenge with fever ,malaise ,SOB neutrophilia & decreased FVC Less common immediate wheeze & decreased FEV1 followed 6 hours later by decreased FVC & DLCO
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Diagnosis Inhalation Challenge Test 17 with HP Vs 11 ILD Vs Control
Challenged with pigeon serum Increase in Temp & Post Challenge decrease FVC ,PAo2 & Sat All HP & 3 ILD +ve but none of control Increase in Temp by 0.5 C 100 PPV & 85 NPV Decrease in FVC by 16%81%PPV & 83% NPV No complication were observed Am J Respir Crit Care Med 1998 Sep
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Diagnosis Inhalation Challenge Test Methacholine challenge
37 dairy farmers & 11 controls 12 with FLD , 13 Asymptomatic with +Ab 12 Asymptomatic with –Ab +ve in all farmers No statistical difference in farmers subgroup in term of degree of responsiveness Am J Respir Crit Care Med Jun
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Diagnosis BAL Lymphocytosis non specific but useful in
narrowing differential Dx CD4/CD8 ? Prognostic 17 Pt study HP with fibrosis Vs no Fibrosis High CD8 acute presentation & less fibrosis High CD4 chronic & more fibrosis Chest 1993 Jul
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Diagnosis Lung Bx Retrospective study
105 TBB 55 FLD Vs Control {other ILD} Gold Standard Test for FLD was not open Bx History ,fine crackles ,Lymphocytic BAL & resolution with removal of exposure 2 pathologist reading as Probable FL ,Possible FL , non specific or alternative Dx Chest Dec
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Diagnosis Lung Bx Interobserver agreement was fair
Upto 48.5% of Bxnon specific LHR for Probable FLD was Possible FLD Lymphocytic Infiltrates 9.1 Granuloma Chest Dec
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Management Environmental Control Reduction of antigenic burden
wetting compost & Abx use Design & Maintenance of Facilities Keeping Humidity below 60% Heating ,Ventilating & AC water shouldn’t be recirculated Early water damage control Protective devices ?
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Management Environmental Control
Relocation ? If persistence of exposure will lead to disease progression 61 Pt with FLD 37 /61 continued farming 5 y follow up on PFT At 1 & 3 year reduction in DLCO,TLC more in continued exposure At 5 no statically significance difference Chest 1985 Jun
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Management Steroids Accelerates initial recovery
but doesn’t change long term outcome 36 Pt FLD Prospective Randomized DB 8 weeks either Prednisolone (20Pt) Vs Placebo (16Pt) At 1 month difference in DLCO At 5 years not statically significant difference in DLCO ,FEV1 At 5 years 5 of Prednisolone group had recurrence Vs 1 of Placebo Not statically significant Am Rev Respir Dis 1992 Jan
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Management Steroids Prospective Randomized 3 arms 93 Pt FLD
12 weeks Vs 4 Weeks Vs Placebo Follow up 18 months No difference between 12 & 4 weeks outcomes Eur J Respir Dis 1983 May
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Thank You
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