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Thursday, January 22, 2009

BRONCHIOLITIS OBLITERANS AND ORGANIZING PNEUMONIA

BRONCHIOLITIS OBLITERANS AND ORGANIZING PNEUMONIA - David A. Pope, MD
BASICS
DESCRIPTION
• A primary or secondary process of the lungs characterized by granulation-like tissue involving the distal airways and alveoli
• A specific reaction of lung tissue to a variety of injuries
• It may occur as patchy infiltrates, or it may be nodular or secondary to another lung disease.
• The process may also appear to be a migrating one.
• It may have a gradual or sudden onset.
• Lungs show a pattern of multiple patchy pneumonia, which are seen on the chest radiograph as patchy alveolar or ground glass opacifications with or without interstitial infiltrates; there may be air bronchograms as well.
• Most cases will respond to corticosteroids, which may have to be given for a year or more
• Synonym(s): Intraluminal fibrosis of distal airways; Idiopathic bronchiolitis obliterans and organizing pneumonitis; Cryptogenic organizing pneumonia; Obliterative bronchiolitis
ALERT
Geriatric Considerations
More common than originally thought and may be sudden and very severe
Pediatric Considerations
Rare, but has been reported after viral pneumonia (adenovirus influenza):
- Characteristics include delayed recovery, persistent cough, crackles, or wheezing after pneumonia
- Laboratory findings generally not helpful
- Imaging shows: Ventilation-perfusion ratio matched defects; high-resolution CT, bronchiectasis, bronchogram, pruned tree appearance
- Diagnosis confirmed by biopsy
- Treatment includes steroids: 1 mg/kg q24h for 1 month, followed by weaning over several months
GENERAL PREVENTION
Except for prevention of relapse, none known
EPIDEMIOLOGY
• Incidence/prevalence in United States: Unknown
• Predominant age: Reported cases range age 0-70, mean age: 50s
Incidence
Unknown
Prevalence
Unknown
RISK FACTORS
• AIDS
• Immunocompromised patients, including transplant patients
• More likely in smokers
ETIOLOGY
Idiopathic. A complex response to a variety of injuries, such as toxic inhalation; post mycoplasma, viral and bacterial infection; aspiration; immunologic factors; drugs
ASSOCIATED CONDITIONS
• Drug-induced pneumonitis
- Paraquat poisoning
- Amiodarone toxicity
- Acebutolol toxicity
- Amphotericin B
- Bleomycin
- Caramazepine
- Cephalosporins
- Gold
- Minocycline
- Nitrofurantoin
- Phenyltoin
- Sulphamethoxypyridazine
- Sulphasalazine
- Ticloppidine
- Freebase cocaine pulmonary toxicity
- Overdose of L-tryptophan
• Infections
- Chronic infectious pneumonia
- Malaria
- Chlamydia
- Legionella
- Mycoplasma
- Pneumocystis
- Cryptococcus
• Immunocompromise: Bone marrow, lung, renal, transplantation
• Connective tissue diseases
- Rheumatic lung
- Sjogren syndrome
- Polymyositis
- Scleroderma
- Essential mixed cryoglobulinemia
• Miscellaneous
- Cystic fibrosis
- Bronchopulmonary dysplasia
- Renal failure
- CHF
- Adult respiratory distress syndrome
- Chronic eosinophilic pneumonia
- Hypersensitivity pneumonitis
- Histiocytosis X
- Sarcoidosis
- Pneumoconioses
- Radiation pneumonitis


DIAGNOSIS
Think of the possibility
SIGNS AND SYMPTOMS
• Most patients present with a flu-like illness that lasts 4-10 weeks or longer. Most have been treated with antibiotics without success.
• Fever
• Dry cough
• Weight loss
• Dyspnea may be severe
• Bilateral crackles
• Fatigue
TESTS
• Leukocytosis with a normal differential
• Elevated erythrocyte sedimentation rate:
• Negative cultures
• Negative serology for mycoplasma, Coxiella, Legionella, psittacosis, and fungus
• Negative viral studies
- Pulmonary function shows a restrictive/obstructive pattern.
- Flow-volume loop shows terminal airway obstruction.
- Chest radiograph may show patchy alveolar opacities, often in the middle or upper lung area, a ground glass pattern that may have air bronchograms
- The involved area may seem to migrate.
- Ventilation-perfusion ratio scan: Matched patchy defects
Imaging
• Chest radiograph: Often appears more normal than the physical examination
• CT scans more accurately define the distribution and extent of the patchy alveolar opacities with areas of hyperlucency.
• Diagnosis is difficult by CT only
Diagnostic Procedures/Surgery
• Open lung biopsy
• Transbronchial biopsy
• It may be wise to use a trial of steroids as a diagnostic trial, although not all would agree.
• If a diagnostic trial is successful, be prepared to treat the patient for at least 1 year.
Pathological Findings
• Intraluminal fibrosis of distal airspaces is the major pathologic feature.
• Fibroblasts and plugs of inflammatory cells and loose connective tissue fill these distal airways.
• Inflammatory cells are mainly lymphocytes and plasma cells.
• Interstitial fibrosis is present.
• Plugs of edematous granulation tissue in the terminal and respiratory bronchioles and alveolar ducts do not cause permanent damage.
DIFFERENTIAL DIAGNOSIS
• Usual interstitial pneumonitis
• Noninfectious diseases
• Tuberculosis
• Sarcoidosis
• Histoplasmosis
• Berylliosis
• Goodpasture syndrome
• Neoplasm
• Polyarteritis nodosa
• Systemic lupus erythematosus
• Wegener granulomatosis
• Sjogren syndrome
• Chronic eosinophilic pneumonia
• Cryptogenic bronchiolitis
TREATMENT
Inpatient may be required
GENERAL MEASURES
• Monitor blood gases or pulse oximetry.
• Oxygen as necessary
Diet
No special diet
Activity
As tolerated
MEDICATION (DRUGS)
First Line
Prednisone
• For 1-3 months, 60 mg/d
• Then taper over a few weeks to 20 mg (this dose may later be given as alternate-day therapy). Increase length of taper for patients on long-term therapy to avoid precipitating Addisonian crisis.
• Treatment may be needed for 1 year or more.
• Contraindications: Refer to the manufacturer's literature.
• Precautions: Be aware of the patient's Mantoux status and history of peptic ulcer disease. Long-term steroid treatment is associated with significant adverse effects including Cushing syndrome, fluid retention, osteoporosis, hyperkalemia, and poor wound healing.
• Significant possible interactions: Refer to the manufacturer's literature.
Second Line
• Steroids other than prednisone may be used.
• 1 paper reported the use of erythromycin 600 mg a day for 3-4 months after initial control with prednisone.
• Prescribe antimicrobials, if the original infection is persistent. The proper choice depends on the pathogen.
FOLLOW-UP
PROGNOSIS
Complete recovery, but individual case management is mandatory
COMPLICATIONS
• Bronchiectasis
• Death, but with proper treatment, recovery is usually complete without permanent sequelae
PATIENT MONITORING
• Frequent visits, weekly at 1st
• Prednisone must be continued because of the chance of relapse
• Monitor the lung disease and the side effects of prednisone therapy (Mantoux, monthly CBC, funduscopic exam every 3-6 months, serial dual energy x-ray absorptiometry (DEXA) scans for osteoporosis)
REFERENCES
1. Cordier JF, Loire R, Brune J. Idiopathic bronchiolitis obliterans organizing pneumonia. Chest. 1989;96:999-1004.
2. Epler GR, Colby TV, et al. Bronchiolitis obliterans organizing pneumonia. N Engl J Med. 1985;312:152-158.
3. Hardy KA, Schidlow D, Zaeri N. Obliterative bronchiolitis in children. Chest. 1988;93:460-466.
4. Lynch DA. Imaging of small airways diseases. Clin Chest Med. 1993;14:623-634.
5. Mueller NL, Staples CA, Miller RR. Bronchiolitis obliterans organizing pneumonia: CT features in 14 patients. AJR. 1990;154:983-987.
6. St John RC, Dorinsky PM. Cryptogenic bronchiolitis. Clin Chest Med. 1993;14:667-675.
7. http://www.epler.com/boop1.html
8. http://www.emedicine.com/radio/byname/bronchiolitis-obliterans-organizing-pneumonia.html
MISCELLANEOUS
• Other notes: This disease behaves differently than bronchiolitis obliterans. Bronchiolitis obliterans and organizing pneumonia is a restrictive problem; bronchiolitis obliterans is an obstructive problem. Bronchiolitis obliterans causes permanent lung damage; bronchiolitis obliterans and organizing pneumonia is completely reversible.
• See also: Sjogren Syndrome

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