BRONCHIOLITIS OBLITERANS

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BRONCHIOLITIS OBLITERANS

• Dr.Özlem Özdemir Kumbasar

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Terms

• Bronchiolitis obliterans
• Obliterative bronchilitis
• Constrictive bronchiolitis

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• 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.

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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

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CLINICAL PRESENTATION

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

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CLINICAL PRESENTATION

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

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PHYSICAL EXAMINATION

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

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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)

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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.

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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.

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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

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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

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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

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BO(S) Associated with HSCT

• 3 or more of the followings
• FEV1lt80
• FEV1/FVClt80
• MMFRlt50
• RVgt120

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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

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PFT

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

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Idiopathic Constrictive Bronchiolitis

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

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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

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Idiopathic Constrictive Bronchiolitis

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

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BO

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

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BO-Infection

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

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Treatment

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

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Immunosuppressive therapy

• Corticosteroids
• Azathioprine
• Cyclosporine
• Tacrolimus
• Mycophenolate mofetil
• Methotrexate
• Inhaled cyclosporine

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Other treatment strategies

• Extracorporeal photochemotherapy
• Total lymphoid irradiation
• Macrolides
• Statin medications
• Lung transplantation

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Our BO(S) Cases due to HSCT

• 18 cases 10 female, 8 male
• Pretransplant diagnosis
• CML9
• AML7
• MDS1
• AA1

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

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• 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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