Bronchiolitis obliterans organizing pneumonia
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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. History. SOBE on minimal exertion Cough with minimal sputum Fever low grade & occasional night sweating

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Bronchiolitis Obliterans Organizing Pneumonia

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Bronchiolitis obliterans organizing pneumonia

Bronchiolitis Obliterans Organizing Pneumonia


History

History

  • 68 y female admitted to H6

  • X smoker 4y 40 pack

  • Unresolving respiratory symptoms

    since Jan/04  Cough , SOB, Fever


History1

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


History2

History

  • NO GI , Renal , CTD symptoms

  • PMH: HTN & Hypothyroidism

  • Rx: HCTZ , L Thyroxine

  • PSH& FH –ve


History3

History

  • Office job ,

  • No travel & No pets

  • Had received multiple Abx without significant improvement


Examinations

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


Investigation

Investigation

  • WBC 12 Poly 10.8 Lymph0.7

  • Hb 99 MCV N Coagulation N

  • BUN , Creat , Lytes & LFT  N

  • UA & microscopy N


Investigation1

Investigation

  • ESR 99

  • ABG PH 7.46 PAO2 66 Sat 93%

    PCO2 38 HCO3 26.8

  • CXR & CT Chest

  • PFT


Investigation2

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

Open Lung Bx  BOOP


Bronchiolitis obliterans organizing pneumonia

BOOP

  • Multiple etiologies

  • Extensive proliferation of granulation tissue in the small airways

  • Inflammation of the surrounding alveoli

  • Incidence 6 /100,000 hospital admission


Bronchiolitis obliterans organizing pneumonia

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


Etiologies

Etiologies

  • Idiopathic

  • Post Infectious Atypical ,Viral ,PCP, Malaria

  • Drug Abx, Chemo , Gold ,Amiodarone

  • CTD SLE , Rheumatoid ,PM , Sjogren


Etiologies1

Etiologies

  • Organ transplantation BMT ,Renal , Lung

  • Radiotherapy

  • Autoimmune diseases PBS , IBD ,Thyroditis

  • Environmental textile printing dye


Steroid response

?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


Relapse predictors

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


Study population

Study Population

  • 19931997 48 / 92 cases were included

  • 65% Female 35% Male

  • Mean Age 61y

  • 71% Non smoker


Study population1

Study Population

  • Symptoms duration prior to Dx 13weeks

  • Dx was made by surgical Bx 69%

    Transbronchial Bx 31%

  • Follow up 35 months


Relapse predictors1

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 predictors2

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


Outcomes

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


Will low dose steroid do the job

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


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