Complications of Pneumonia: Postinfective Bronchiolitis Obliterans
Paul C. Stillwell, MD, FAAP, and Deborah R. Liptzin, MD, MS, FAAP
Introduction/Etiology/Epidemiology
•Obliterative bronchiolitis, or bronchiolitis obliterans, is obliteration of the small brochiolar airways with fibrinoproliferative material.
•It occurs in a variety of settings (Box 65-1).
—In North America, it occurs infrequently after viral infection and more commonly after lung transplantation or hematopoietic stem cell transplantation.
—In indigenous populations, it is commonly seen after viral infections (postinfectious bronchiolitis obliterans).
Box 65-1. Underlying Systemic Disease or Trigger for Development of Obliterative Bronchiolitis
| Autoimmune disease | Posttransplant causes |
| Rheumatoid arthritis | Hematopoietic stem cell transplant |
| Sjogren syndrome | |
| Systemic lupus erythematosus | Lung transplant |
| Inhalational agents | Aspiration |
| Sulfur mustard | |
| Nitrogen oxides | Stevens-Johnson syndrome |
| Mold | |
| Avian antigens | |
| Others | |
| Infections | |
| Adenovirus | |
| Measles | |
| Mycoplasma | |
| Others |
•Signs of obliterative bronchiolitis are inspiratory crackles, barrel chest, hypoxemia, and tachypnea.
•Symptoms of obliterative bronchiolitis are cough, dyspnea, and wheezing.
•Signs and symptoms may be subtle. Dyspnea is often very prominent, even with minimal exertion.
Diagnostic Considerations
•The standard of reference for diagnosis is lung biopsy; however, disease can be patchy and missed at biopsy (particularly transbronchial biopsy).
•The pathologic process causes the airways to be narrowed or completely obliterated; airway fibrosis will be present.
•At pulmonary function testing, obstruction and air trapping will be found, typically with minimal postbronchodilator improvement (Figure 65-1).
•Chest radiographs show variable, nonspecific findings that range from patchy to diffuse air space opacity (Figure 65-2). Computed tomographic (CT) findings are more specific and include air trapping, mosaic perfusion, and vascular attenuation, with or without bronchiectasis (Figure 65-3).
—Mosaic perfusion appears as areas of decreased attenuation that are darker on CT images, due to decreased perfusion.
—Vascular attenuation appears as loss of blood vessel visibility in areas of decreased lung perfusion.
•Lung transplant recipients with a clinically significant change in pulmonary function test results (obstruction) without a lung biopsy have bronchiolitis obliterans syndrome.
Treatment
•Treatment is based on expert opinion and on adult trials for bronchiolitis obliterans syndrome.
•Monitor the patient for hypoxemia with exercise and at night, and treat as needed.
•Monitor the patient for pulmonary hypertension, and treat as needed.
•Consult with a pediatric pulmonologist with expertise in children’s interstitial and diffuse lung disease.
•Common medication regimens are listed in Table 65-1.
•Adult studies also suggest fluticasone, azithromycin, and montelukast.
•If the patient also has bronchiectasis, airway clearance and early antibiotics should be initiated for a cough.
Figure 65-3. Obliterative bronchiolitis in a patient after having Stevens-Johnson syndrome. Axial chest computed tomographic image demonstrates regions of mosaic perfusion (typical of obliterative bronchiolitis) scattered between areas of normal lung. The mosaic perfusion areas can be recognized by the well-defined margins and low attenuation due to decreased visualization of the normal vascular structures.
| Table 65-1. Treatment for Obliterative Bronchiolitis | ||
| Treatment | Dose | Interval |
| Methylprednisolone | IV 10–30 mg/kg for 3 d | Every month |
| IV immunoglobulin | IV 2 g/kg for 1 d | Every month |
| Azithromycin | 10 mg/kg by mouth (maximum, 500 mg) Alternate dosing: 18.0–35.9 kg: 250 mg >36.0 kg: 500 mg | 3 d a week |
IV, intravenous.
Prognosis
•Few outcome data exist in children.
•Some patients, especially those with postinfectious bronchiolitis obliterans, may have stable lung function for years.
•Other patients may have progressive disease despite therapy.
•Once fibrosis has set in, reversal of the disease may be challenging.
•There may be a role for antifibrotic therapy in this population.
When to Refer
•Establishing the correct diagnosis
•Treatment induction and maintenance therapy
•Disease follow-up and management of exacerbations
Resource for Families
•What Is Interstitial Lung Disease in Children? (American Thoracic Society) www.thoracic.org/patients/patient-resources/resources/interstitial-lung-disease-in-children.pdf
Clinical Pearls
•Consider a diagnosis of obliterative bronchiolitis in patients who have new shortness of breath, exercise intolerance, and/or crackles after a hematopoietic stem cell transplant or lung transplant.
•A diagnosis of obliterative bronchiolitis should also be considered in patients with a history of severe pneumonia, particularly with adenovirus, Stevens-Johnson syndrome, or measles, and poorly reversible airway obstruction (“atypical asthma”).
CHAPTER 45: UPPER RESPIRATORY INFECTIONS
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CHAPTER 46: LARYNGITIS
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CHAPTER 47: EPIGLOTTITIS
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CHAPTER 48: CROUP
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CHAPTER 50: PERTUSSIS
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CHAPTER 51: BACTERIAL TRACHEITIS
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CHAPTER 52: BRONCHITIS
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CHAPTER 53: BRONCHIOLITIS
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CHAPTER 54: BACTERIAL PNEUMONIA
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CHAPTER 55: VIRAL PNEUMONIA
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CHAPTER 56: MYCOPLASMA PNEUMONIA
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•Jain S, Williams DJ, Arnold SR, et al; CDC EPIC Study Team. Community-acquired pneumonia requiring hospitalization among U.S. children. N Engl J Med. 2015; 372(9):835–845
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CHAPTER 57: CHLAMYDIAL PNEUMONIA
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CHAPTER 58: TUBERCULOSIS
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CHAPTER 59: NONTUBERCULOUS MYCOBACTERIAL PULMONARY DISEASE
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CHAPTER 60: FUNGAL PNEUMONIA
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CHAPTER 61: HISTOPLASMOSIS AND OTHER ENDEMIC FUNGAL PNEUMONIAS
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•Lease ED, Alexander BD. Fungal diagnostics in pneumonia. Semin Respir Crit Care Med. 2011;32(6):663–672
•Thompson GR III, Cadena J, Patterson TF. Overview of antifungal agents. Clin Chest Med. 2009;30:203–215
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CHAPTER 62: COMPLICATIONS OF PNEUMONIA: PLEURAL EFFUSIONS
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•Kontouli K, Hatziagorou E, Kyrvasilis F, Roilides E, Emporiadou M, Tsanakas J. Long-term outcome of parapneumonic effusions in children: Lung function and exercise tolerance. Pediatr Pulmonol. 2015;50(6):615–620
CHAPTER 63: COMPLICATIONS OF PNEUMONIA: EMPYEMA
•Corcoran JP, Wrightson JM, Belcher E, DeCamp MM, Feller-Kopman D, Rahman NM. Pleural infection: past, present, and future directions. Lancet Respir Med. 2015;3(7):563–577
•Hendaus MA, Janahi IA. Parapneumonic effusion in children: an up-to-date review. Clin Pediatr (Phila). 2016;55(1):10–18
•Walker W, Wheeler R, Legg J. Update on the causes, investigation and management of empyema in childhood. Arch Dis Child. 2011;96(5):482–488
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•Kontouli K, Hatziagorou E, Kyrvasilis F, Roilides E, Emporiadou M, Tsanakas J. Long-term outcome of parapneumonic effusions in children: Lung function and exercise tolerance. Pediatr Pulmonol. 2015;50(6):615–620
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CHAPTER 64: COMPLICATIONS OF PNEUMONIA: PULMONARY ABSCESS
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CHAPTER 65: COMPLICATIONS OF PNEUMONIA: POSTINFECTIVE BRONCHIOLITIS OBLITERANS
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•Moonnumakal SP, Fan LL. Bronchiolitis obliterans in children. Curr Opin Pediatr. 2008;20(3):272–278
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