Bronchiolitis Obliterans Organizing Pneumonia

History  68 y female admitted to H6  X smoker 4y 40 pack  Unresolving respiratory symptoms since Jan/04  Cough, SOB, Fever since Jan/04  Cough, SOB, Fever

History  SOBE on minimal exertion  Cough with minimal sputum  Fever low grade & occasional night sweating  SR: wt loss 10 lb, bilateral lower costal pain  No orthopnea, PND,wheeze

History  NO GI, Renal, CTD symptoms  PMH: HTN & Hypothyroidism  Rx: HCTZ, L Thyroxine  PSH& FH –ve

History  Office job,  No travel & No pets  Had received multiple Abx without significant improvement

Examinations  Afebrile RR 18 Sat95%  BP 130/70 HR 90  No clubbing, LN, Skin rash  Chest : tender lower ribs minimal crackles & wheeze bilateral minimal crackles & wheeze bilateral  CVS : S1+S2+0  Abd & LL  N

Investigation  WBC 12 Poly 10.8 Lymph0.7  Hb 99 MCV N Coagulation N  BUN, Creat, Lytes & LFT  N  UA & microscopy N

Investigation  ESR 99  ABG PH 7.46 PAO2 66 Sat 93% PCO2 38 HCO PCO2 38 HCO  CXR & CT Chest  PFT

Investigation  BAL  -ve cultures & cytology  ANA, Anti DNA, RF & ANCA -ve  Bone Scan single non specific uptake focus ?fracture  Open Lung Bx RML & RLL

Open Lung Bx  BOOP

BOOP  Multiple etiologies  Extensive proliferation of granulation tissue in the small airways  Inflammation of the surrounding alveoli  Incidence 6 /100,000 hospital admission

BOOP  Equal male : female 5 th -6 th decades  Smoking is not a risk factor  Mimicker of CAP  Symptoms, Signs, Radiological & Laboratory findings are not specific  Good response to steroids

Etiologies  Idiopathic  Post Infectious Atypical,Viral,PCP, Malaria  Drug Abx, Chemo, Gold,Amiodarone  CTD SLE, Rheumatoid,PM, Sjogren

Etiologies  Organ transplantation BMT,Renal, Lung  Radiotherapy  Autoimmune diseases PBS, IBD,Thyroditis  Environmental textile printing dye

?Steroid Response  Higher vasculrization Higher levels of VEGF vascular endothelial growth factor & its receptors in BOOP > UIP J Pathology Feb 2002 J Pathology Feb 2002  Higher Apoptotic Activity Higher apoptotic activity index in BOOP > UIP Similar levels of apoptosis regulating proteins Lung 1999 Lung 1999

Relapse Predictors  Retrospective study  GERM “O”P Registry  1100 cases by 1999  Looking for relapse characteristics & possible predictors & possible predictors  Inclusion criteria 1) Bx diagnosis 1) Bx diagnosis 2)Compatible clinical & radiological picture 2)Compatible clinical & radiological picture 3)Absence of etiology 3)Absence of etiology 4) Treatment with steroid 4) Treatment with steroid Am Jr Respir Crit Care Med vol Am Jr Respir Crit Care Med vol

Study Population  1993  / 92 cases were included  65% Female 35% Male  Mean Age 61y  71% Non smoker

Study Population  Symptoms duration prior to Dx 13weeks  Dx was made by surgical Bx 69% Transbronchial Bx 31% Transbronchial Bx 31%  Follow up 35 months

Relapse Predictors  42% had no relapse NR  31% experience single relapse  27% experience >1 relapse MR  Time of relapse 6 months from initial episode  Highest probability of relapse in the first year

Relapse Predictors  68% were still on prednisone at time of relapse  Mean dose at relapse time 12 mg  Statistical significant predictors NR Vs MR Delay between symptoms & diagnosis Delay between symptoms & diagnosis Elevated GGT, Alk Phos & ALT Elevated GGT, Alk Phos & ALT  NO difference in Age, Gender, Smoking, PFT or BAL or steroid dose in Age, Gender, Smoking, PFT or BAL or steroid dose

Outcomes  No significance difference NR Vs MR clinical, radiological & PFT at last follow up clinical, radiological & PFT at last follow up  5 y survival 95% 2 Mortality  PE & rupture AAA 2 Mortality  PE & rupture AAA  Steroid Side effects were similar NR Vs MR

Will Low Dose Steroid Do the Job  12/28 MR treated with <20 mg prednisone  16/28 MR >20  Similar relapse number & clinical course  Slower radiological improvement in Low dose  More steroid side effects in High dose