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Bronchiolitis Obliterans Bronchiolitis Obliterans Organizing PneumoniaOrganizing Pneumonia
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HistoryHistory
68 y female admitted to H6 68 y female admitted to H6
X smoker 4y 40 packX smoker 4y 40 pack
Unresolving respiratory symptoms Unresolving respiratory symptoms
since Jan/04 since Jan/04 Cough , SOB, Fever Cough , SOB, Fever
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HistoryHistory SOBE on minimal exertion SOBE on minimal exertion
Cough with minimal sputumCough with minimal sputum
Fever low grade & occasional night sweatingFever low grade & occasional night sweating
SR: wt loss 10 lb , bilateral lower costal painSR: wt loss 10 lb , bilateral lower costal pain
No orthopnea , PND ,wheezeNo orthopnea , PND ,wheeze
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HistoryHistory NO GI , Renal , CTD symptomsNO GI , Renal , CTD symptoms
PMH: HTN & HypothyroidismPMH: HTN & Hypothyroidism
Rx: HCTZ , L ThyroxineRx: HCTZ , L Thyroxine
PSH& FH –vePSH& FH –ve
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HistoryHistory Office job ,Office job ,
No travel & No petsNo travel & No pets
Had received multiple Abx without significant Had received multiple Abx without significant improvementimprovement
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ExaminationsExaminations
Afebrile RR 18 Sat95%Afebrile RR 18 Sat95% BP 130/70 HR 90BP 130/70 HR 90 No clubbing , LN , Skin rash No clubbing , LN , Skin rash Chest : tender lower ribsChest : tender lower ribs
minimal crackles & wheeze bilateral minimal crackles & wheeze bilateral CVS : S1+S2+0CVS : S1+S2+0 Abd & LL Abd & LL NN
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InvestigationInvestigation
WBC 12 Poly 10.8 Lymph0.7WBC 12 Poly 10.8 Lymph0.7
Hb 99 MCV N Coagulation NHb 99 MCV N Coagulation N
BUN , Creat , Lytes & LFT BUN , Creat , Lytes & LFT N N
UA & microscopy NUA & microscopy N
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InvestigationInvestigation
ESR 99 ESR 99
ABG PH 7.46 PAO2 66 Sat 93%ABG PH 7.46 PAO2 66 Sat 93% PCO2 38 HCO3 26.8PCO2 38 HCO3 26.8
CXR & CT ChestCXR & CT Chest
PFTPFT
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InvestigationInvestigation
BAL BAL -ve cultures & cytology -ve cultures & cytology
ANA , Anti DNA , RF & ANCA -ve ANA , Anti DNA , RF & ANCA -ve
Bone Scan single non specific uptake focus ?Bone Scan single non specific uptake focus ?fracturefracture
Open Lung Bx RML & RLLOpen Lung Bx RML & RLL
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Open Lung Bx Open Lung Bx BOOP BOOP
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BOOPBOOP
Multiple etiologies Multiple etiologies
Extensive proliferation of granulation tissue in Extensive proliferation of granulation tissue in the small airways the small airways
Inflammation of the surrounding alveoliInflammation of the surrounding alveoli
Incidence 6 /100,000 hospital admissionIncidence 6 /100,000 hospital admission
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BOOPBOOP Equal male : female 5Equal male : female 5thth-6-6thth decades decades
Smoking is not a risk factorSmoking is not a risk factor
Mimicker of CAPMimicker of CAP
Symptoms , Signs , Radiological & Laboratory Symptoms , Signs , Radiological & Laboratory findings are not specificfindings are not specific
Good response to steroidsGood response to steroids
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EtiologiesEtiologies IdiopathicIdiopathic
Post Infectious Post Infectious Atypical ,Viral ,PCP, MalariaAtypical ,Viral ,PCP, Malaria
Drug Drug Abx, Chemo , Gold ,Amiodarone Abx, Chemo , Gold ,Amiodarone
CTD CTD SLE , Rheumatoid ,PM , Sjogren SLE , Rheumatoid ,PM , Sjogren
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EtiologiesEtiologies
Organ transplantation Organ transplantation BMT ,Renal , LungBMT ,Renal , Lung
RadiotherapyRadiotherapy
Autoimmune diseases Autoimmune diseases PBS , IBD ,ThyroditisPBS , IBD ,Thyroditis
Environmental Environmental textile printing dyetextile printing dye
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?Steroid Response?Steroid Response
Higher vasculrizationHigher vasculrization
Higher levels of VEGF vascular endothelial Higher levels of VEGF vascular endothelial growth factor & its receptors in BOOP > UIPgrowth factor & its receptors in BOOP > UIP
J Pathology Feb 2002J Pathology Feb 2002
Higher Apoptotic ActivityHigher Apoptotic Activity
Higher apoptotic activity index in BOOP > UIP Higher apoptotic activity index in BOOP > UIP
Similar levels of apoptosis regulating proteins Similar levels of apoptosis regulating proteins
Lung 1999Lung 1999
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Relapse PredictorsRelapse Predictors Retrospective studyRetrospective study GERM “O”P Registry GERM “O”P Registry 1100 cases by 19991100 cases by 1999 Looking for relapse characteristics Looking for relapse characteristics
& possible predictors & possible predictors Inclusion criteriaInclusion criteria
1) Bx diagnosis1) Bx diagnosis
2)Compatible clinical & radiological picture2)Compatible clinical & radiological picture
3)Absence of etiology3)Absence of etiology
4) Treatment with steroid4) Treatment with steroid Am Jr Respir Crit Care Med vol 162 2000Am Jr Respir Crit Care Med vol 162 2000
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Study PopulationStudy Population
199319931997 48 / 92 cases were included1997 48 / 92 cases were included
65% Female 35% Male65% Female 35% Male
Mean Age 61y Mean Age 61y
71% Non smoker 71% Non smoker
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Study PopulationStudy Population
Symptoms duration prior to Dx 13weeksSymptoms duration prior to Dx 13weeks
Dx was made by surgical Bx 69%Dx was made by surgical Bx 69%
Transbronchial Bx 31%Transbronchial Bx 31%
Follow up 35 monthsFollow up 35 months
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Relapse PredictorsRelapse Predictors 42% had no relapse NR42% had no relapse NR
31% experience single relapse 31% experience single relapse
27% experience >1 relapse MR27% experience >1 relapse MR
Time of relapse 6 months from initial episodeTime of relapse 6 months from initial episode
Highest probability of relapse in the first yearHighest probability of relapse in the first year
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Relapse PredictorsRelapse Predictors 68% were still on prednisone at time of relapse 68% were still on prednisone at time of relapse
Mean dose at relapse time 12 mgMean dose at relapse time 12 mg
Statistical significant predictors NR Vs MRStatistical significant predictors NR Vs MR Delay between symptoms & diagnosisDelay between symptoms & diagnosis Elevated GGT , Alk Phos & ALT Elevated GGT , Alk Phos & ALT
NO differenceNO difference in Age, Gender , Smoking , PFT or BAL or steroid in Age, Gender , Smoking , PFT or BAL or steroid
dose dose
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OutcomesOutcomes No significance difference NR Vs MRNo significance difference NR Vs MR
clinical , radiological & PFT at last follow upclinical , radiological & PFT at last follow up
5 y survival 95%5 y survival 95%
2 Mortality 2 Mortality PE & rupture AAA PE & rupture AAA
Steroid Side effects were similar NR Vs MR Steroid Side effects were similar NR Vs MR
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Will Low Dose Steroid Do the JobWill Low Dose Steroid Do the Job
12/28 MR treated with <20 mg prednisone12/28 MR treated with <20 mg prednisone 16/28 MR >2016/28 MR >20
Similar relapse number & clinical course Similar relapse number & clinical course
Slower radiological improvement in Low doseSlower radiological improvement in Low dose
More steroid side effects in High doseMore steroid side effects in High dose