Bronchiolitis Obliterans Organizing Pneumonia

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Bronchiolitis Obliterans Organizing Pneumonia
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History

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

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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

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History

• NO GI , Renal , CTD symptoms
• PMH HTN Hypothyroidism
• Rx HCTZ , L Thyroxine
• PSH FH ve

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History

• Office job ,
• No travel No pets
• Had received multiple Abx without significant
  improvement

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Examinations

• Afebrile RR 18 Sat95
• BP 130/70 HR 90
• No clubbing , LN , Skin rash
• Chest tender lower ribs
• minimal crackles wheeze
  bilateral
• CVS S1S20
• Abd LL ?N

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Investigation

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

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Investigation

• ESR 99
• ABG PH 7.46 PAO2 66 Sat 93
• PCO2 38 HCO3 26.8
• CXR CT Chest
• PFT

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Investigation

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

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Open Lung Bx ? BOOP
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BOOP

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

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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

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Etiologies

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

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Etiologies

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

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?Steroid Response

• Higher vasculrization
• Higher levels of VEGF vascular endothelial growth
  factor its receptors in BOOP gt UIP
• J
  Pathology Feb 2002
• Higher Apoptotic Activity
• Higher apoptotic activity index in BOOP gt UIP
• Similar levels of apoptosis regulating proteins
• 
  Lung 1999

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Relapse Predictors

• Retrospective study
• GERM OP 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

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Study Population

• 1993?1997 48 / 92 cases were included
• 65 Female 35 Male
• Mean Age 61y
• 71 Non smoker

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Study Population

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

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Relapse Predictors

• 42 had no relapse NR
• 31 experience single relapse
• 27 experience gt1 relapse MR
• Time of relapse 6 months from initial episode
• Highest probability of relapse in the first year

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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

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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

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Will Low Dose Steroid Do the Job

• 12/28 MR treated with lt20 mg prednisone
• 16/28 MR gt20
• Similar relapse number clinical course
• Slower radiological improvement in Low dose
• More steroid side effects in High dose