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BRONCHIOLITIS OBLITERANS

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BRONCHIOLITIS OBLITERANS Dr. zlem zdemir Kumbasar * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * * Our BO(S) Cases due to HSCT 18 cases: 10 female ... – PowerPoint PPT presentation

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Title: BRONCHIOLITIS OBLITERANS


1
BRONCHIOLITIS OBLITERANS
  • Dr.Özlem Özdemir Kumbasar

2
Terms
  • Bronchiolitis obliterans
  • Obliterative bronchilitis
  • Constrictive bronchiolitis

3
  • BO is a chronic inflammatory and fibrotic
    process of the bronchioles.
  • As a result of this process, cicatricial luminal
    narrowing and severe obstructive airways disease
    develop.
  • BOnarrowing of the bronchiol lumen due to mural
    fibrosis of the bronchiol.

4
Causes
  • Allograft recipients (lung, HSCT)
  • Post-infectious (viruses, mycoplasma)
  • Connective tissue diseases (rheumatoid arthritis)
  • Inhalation injury (NO2, ammonia)
  • Ingested toxin (Sauropus androgynus)
  • Drugs (penicillamine, gold)
  • Miscellaneous diseases (infl.bowel dis.,
    microcarcinoid tumorlet, paraneoplastic
    pemphigus)
  • Idiopathic

5
CLINICAL PRESENTATION
  • Clinical onset is often insidious and
    nonspecific.
  • Dyspnea on exertion
  • Chronic productive cough
  • Fatigue

6
CLINICAL PRESENTATION
  • Sometimes BO may present acutely as an episode
    resembling asthmatic exacerbation
  • Cough
  • Wheeze
  • Low-grade fever

7
PHYSICAL EXAMINATION
  • In the early stages it is usually unremarkable,
    may show physical evidence of hyperinflation
  • Cracles
  • Wheezes
  • Squeak-squawk

8
RADIOLOGICAL FEATURESCHEST X-RAY
  • Normal
  • Nonspecific abnormalities
  • hyperinflation
  • peripheral attenuation of the vascular markings
  • nodular or reticulonodular opacities
  • Serial radiographs
  • progressive increase in lung volumes
  • bronchial wall thickening and bronchiectasis

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HRCT
  • Mosaic perfusion segmental or lobular areas of
    hypoattenuation that are associated with
    narrowing of the caliber of the pulmonary vessels
  • Air-trapping accentuated on expiratory views
    (80sensitive and 94specific for the diagnosis
    of BO)

12
HRCT
  • Bronchiectasis tends to be peripheral and
    cylindrical. It is often a late finding and a
    sequel of bronchiolitis
  • In a few cases HRCT may appear normal.

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Diagnosis
  • The diagnosis of BO requires histologic
    confirmation.
  • The sensitivity of TBB in the diagnosis of BO is
    low because of patchy nature of BO and small
    quantity of bronchiolar material that is
    obtained.
  • Surgical lung biopsy should be considered in the
    absence of the TBB diagnosis, especially if the
    case is atypical.

19
BO / BOS
  • Bronchiolitis obliterans syndrome (BOS) was
    proposed as a clinical description of BO and is
    defined by spirometric rather than histologic
    criteria in lung transplant recipients.
  • BOS is diagnosed when a patient has a decline of
    more than 20 in FEV1 compared with the mean of
    the two highest postop. FEV1 obtained at least 3
    weeks apart.

20
BOS Original classification
  • BOS 0 FEV1 80 or more of baseline
  • BOS 1 FEV1 66 to 80 of baseline
  • BOS 2 FEV1 51 to 65 of baseline
  • BOS 3 FEV1 50 or less of baseline

21
BOS Current classification
  • BOS 0 FEV1gt90 of baseline and FEF25-75gt75 of
    baseline
  • BOS 0-p FEV1 81 to 90 of baseline and/or
    FEF25-75lt75of baseline
  • BOS 1 FEV1 66 to 80 of baseline
  • BOS 2 FEV1 51 to 65 of baseline
  • BOS 3 FEV1 50 or less of baseline

22
BO(S) Associated with HSCT
  • Diagnosis is based primarily on spirometric
    measurements.
  • Infections and other causes of airflow
    obstruction must be excluded.
  • FEV1lt80
  • FEV1/FVClt70 OR

23
BO(S) Associated with HSCT
  • 3 or more of the followings
  • FEV1lt80
  • FEV1/FVClt80
  • MMFRlt50
  • RVgt120

24
BO(S) Associated with HSCT
  • Alternative criteria for defining HSCT
    associated BO
  • -lowest post HSCT FEV1/FVClt80
  • -more than 5 per year decline in the percent
    predicted FEV1

25
PFT
  • Airflow obstruction
  • Hyperinflation
  • Low DLCO
  • Low alveolar volume

26
Idiopathic Constrictive Bronchiolitis
  • This rare clincal entity occurs mostly in women.
  • Persistent cough, progressive dyspnea
  • Accelerated severe obstructive defect
  • Wheezes or cracles

27
Idiopathic Constrictive Bronchiolitis
  • Suspected case
  • Age less than 40 years
  • Modest or absent smoking history
  • Severity of obstruction out of proportion to
    tobacco use history
  • Rapid progression of symptoms
  • Radiographic findings and PFT results compatible
    with BO

28
Idiopathic Constrictive Bronchiolitis
  • Open lung biopsy ?
  • BAL-in patients who are poor surgical candidates.
  • Prolonged high dose corticosteroid therapy may be
    beneficial

29
BO
  • Mortality rate is high (gt50, 25-60,)
  • Complications
  • Respiratory tract infections
  • Respiratory failure
  • Pneumthorax

30
BO-Infection
  • Colonisation of the airways
  • Pseudomonas aerugionosa
  • Staphylococcus spp
  • Aspergillus
  • Infectious exacerbations are often an immediate
    cause of death in patients with BO

31
Treatment
  • Agressive treatment of infectious exacerbations
  • Supportive therapy-Oxygen
  • Bronchodilators ?
  • Inhaled steroids ?
  • Immunosuppressive therapy

32
Immunosuppressive therapy
  • Corticosteroids
  • Azathioprine
  • Cyclosporine
  • Tacrolimus
  • Mycophenolate mofetil
  • Methotrexate
  • Inhaled cyclosporine

33
Other treatment strategies
  • Extracorporeal photochemotherapy
  • Total lymphoid irradiation
  • Macrolides
  • Statin medications
  • Lung transplantation

34
Our BO(S) Cases due to HSCT
  • 18 cases 10 female, 8 male
  • Pretransplant diagnosis
  • CML9
  • AML7
  • MDS1
  • AA1

35
  • Chronic GVHD18
  • Acute GVHD12
  • Allogeneic PSC14
  • Allogeneic BMT4
  • Median age31 (16-46)
  • Presentation time of symptoms after tx6 months
    (3-15)

36
  • Symptoms dyspnea, cough
  • PExam. crackles, wheezes, squeak
  • Treatment imunosuppressive drugs,
    bronchodilators
  • Pnx 2 cases
  • Colonisation with P.aeruginosa 2 cases
  • Tuberculosis1 case
  • LTOT 7 cases
  • Mortality 6 cases. (Five of them because of
    respiratory failure)

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