Bronchiolitis Obliterans Bronchiolitis Obliterans Organizing PneumoniaOrganizing Pneumonia

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

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

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

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

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

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

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

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

Open Lung Bx Open Lung Bx BOOP BOOP

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

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

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

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

?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

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

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

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

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

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

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

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