bronchiolitis obliterans organizing pneumonia n.
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  1. Bronchiolitis Obliterans Organizing Pneumonia

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

  3. 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

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

  5. History • Office job , • No travel & No pets • Had received multiple Abx without significant improvement

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

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

  8. Investigation • ESR 99 • ABG PH 7.46 PAO2 66 Sat 93% PCO2 38 HCO3 26.8 • CXR & CT Chest • PFT

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

  10. Open Lung Bx  BOOP

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

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

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

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

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

  16. Relapse Predictors • Retrospective study • GERM “O”P Registry 1100 cases by 1999 • Looking for relapse characteristics & possible predictors • Inclusion criteria 1) Bx diagnosis 2)Compatible clinical & radiological picture 3)Absence of etiology 4) Treatment with steroid Am Jr Respir Crit Care Med vol 162 2000

  17. Study Population • 19931997 48 / 92 cases were included • 65% Female 35% Male • Mean Age 61y • 71% Non smoker

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

  19. 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

  20. 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 Elevated GGT , Alk Phos & ALT • NO difference in Age, Gender , Smoking , PFT or BAL or steroid dose

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

  22. 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