Updated: Oct 6, 2009
Organizing pneumonia is characterized by the presence of granulation tissue in the distal air spaces. When organizing pneumonia is associated with granulation tissue in the bronchiolar lumen, the qualifying term bronchiolitis obliterans (BO) is added.
Cryptogenic organizing pneumonia (COP) is often confused with bronchiolitis obliterans organizing pneumonia (BOOP).2 COP is a clinicopathologic syndrome that rapidly resolves with the use of corticosteroids but that is also marked by frequent relapses when treatment is tapered or stopped.
Radiologically identical peripheral airspace consolidation occurs in patients with chronic eosinophilic pneumonia (CEP) and BOOP. CEP primarily involves the upper lobe; by contrast, in BOOP, consolidation is predominantly in the lower zones, although some patients have pathologic characteristics of CEP and BOOP.
A tissue biopsy specimen is needed for a precise diagnosis, but clinicoradiologic characteristics determined through biopsy-based studies may provide enough diagnostic information. This article discusses BOOP in the general context of organizing pneumonia; it combines data from BOOP and COP patient research. Organizing pneumonias that are of known cause are indistinguishable from those that are of unknown cause.3,4,5,6,7,8,9,10,11
Recent studies
In a retrospective study by Vasu et al of 33 patients with BOOP on surgical lung biopsy over a 10-year period, dyspnea was found to be the most common symptom, followed by dry cough and fever, and the main radiologic finding was bilateral patchy consolidation. Crackles was the most common physical finding. Although most patients had a favorable prognosis, 17% did not respond to therapy. Compared with patients with idiopathic BOOP, patients with secondary BOOP had more frequent fevers and were generally more symptomatic. In addition, pleural effusion was present in 60% of patients with secondary BOOP, whereas none of the patients with idiopathic BOOP had pleural effusion.2
Because bronchiolitis combined with interstitial pneumonitis has been equated with BOOP, according to Mark and Ruangchira-urai, the authors compared the findings in 31 patients with lung biopsies revealing both bronchiolar and interstitial pneumonitis with the clinical and pathologic findings in bronchiolitis obliterans, BOOP, nonspecific interstitial pneumonitis, usual interstitial pneumonitis, airway-centered interstitial fibrosis, and idiopathic bronchiolocentric interstitial pneumonia, along with findings in 10 cases of cystic fibrosis. The common finding was a combination of bronchiolitis and interstitial inflammation and fibrosis but little or no intra-alveolar organizing pneumonia. BOOP involved less area than the interstitial pneumonitis in each case. Of the 31 cases noted, 19 had follow-up, and all received corticosteroids, but the response was less than that seen with BOOP; however, disease generally did not progress in the patients given corticosteroids.4
About 50% of cases of bronchiolitis obliterans organizing pneumonia are idiopathic.12 The following conditions are associated with BOOP:
Demographics
Symptoms do not respond to broad-spectrum antibiotics. A significant number of patients have associated collagen disease (16%) and a history of inhalation exposure to toxins (17%). BOOP may be the first manifestation of non-Hodgkin lymphoma and collagen disease.16 In most patients, clinical examination of the thorax demonstrates fine, dry lung crepitations. Clubbing is unusual. The erythrocyte sedimentation rate not only is invariably higher but may be greatly increased. Pulmonary function tests characteristically show a restrictive pattern. The diffusing capacity is reduced, the resting alveolar arterial oxygen gradient is widened, and exercise-related hypoxemia is present. By contrast, chronic eosinophilic pneumonia (CEP) involves an obstructive pattern of lung physiology.17,18
Bronchoalveolar lavage reveals the following cytologic and immunocytologic characteristics in patients with BOOP19 :
Treatment
Most patients with bronchiolitis obliterans organizing pneumonia require open lung biopsy for diagnosis. However, some evidence suggests that combining the cytologic bronchoalveolar lavage and histologic transbronchial lung biopsy data obtained during a fiberoptic procedure appears to be an effective method for initially investigating cases of BOOP in which there are radiographic findings of patchy shadows. Percutaneous lung biopsy has been used in a few patients, but on the whole, it appears to be inadequate.
BOOP may resolve spontaneously; however, patients usually require treatment with steroids. Most patients recover with treatment, and symptoms resolve within days or weeks. Radiographic findings reportedly demonstrate improvement in 50-86% of patients; however, in a minority of patients, the disease may persist. Approximately 30% of patients experience relapse upon withdrawal of treatment. Patients with BOOP respond favorably to treatment with steroids.20
Wegener Granulomatosis, Thoracic
Chronic interstitial pneumonia (organizing interstitial pneumonia, chronic diffuse sclerosing alveolitis)
Acute interstitial pneumonia (AIP)
Focal organizing pneumonia
Interstitial lung disease
Hypogammaglobulinemia
Pulmonary metastasis and primary adenocarcinoma
Pulmonary tuberculosis
Community-acquired pneumonia
Chronic eosinophilic pneumonia
Usual interstitial pneumonia
Bronchoalveolar carcinoma
Techniques and findings
Plain radiographic findings in patients with bronchiolitis obliterans organizing pneumonia include the following (see Images above and Images 1-7 in Multimedia Section)21 :
Accuracy
Imaging pearls
Techniques and findings
CT-scan and high-resolution CT-scan findings include the following (see Image above and Image 8 in Multimedia Section)13 :
In the early stages, clinical and chest radiographic findings of acute AIP and BOOP may be similar; however, HRCT findings of acute interstitial pneumonia (AIP) and BOOP may differ. Traction bronchiectasis, interlobular septal thickening, and intralobular septal thickening are significantly more prevalent in patients with AIP than in patients with BOOP, whereas parenchymal nodules and peripheral distribution are more prevalent in BOOP. Areas with ground-glass attenuation, airspace consolidation, and architectural distortion are common in AIP and BOOP.
Accuracy
Plain radiographic and CT findings are nonspecific in BOOP and may be seen in a variety of pulmonary infectious or inflammatory processes and neoplastic diseases. However, CT scanning is more sensitive than chest radiography in assessing disease pattern and distribution. CT scanning is also superior in determining the biopsy site; therefore, high-resolution CT (HRCT) is usually performed before lung biopsy.
Imaging pearls
Techniques and findings
An early report of the value of gadolinium-enhanced magnetic resonance imaging (MRI) in the evaluation of disease activity in chronic infiltrative lung diseases showed promising results. A cohort of 25 patients included patients with sarcoidosis, BOOP, usual interstitial pneumonia (UIP), radiation pneumonitis, desquamative interstitial pneumonia, rheumatoid lung, vasculitis, alveolar proteinosis, bronchoalveolar carcinoma, and/or CEP.33
One or more studies—bronchoalveolar lavage, gallium-67 citrate radionuclide scanning, serum angiotensin-converting enzyme assay, and open lung biopsy—were employed to assess disease activity. T1-weighted breath-hold magnetic resonance images were obtained before and after the intravenous administration of gadolinium-based contrast agent. Fourteen out of the 17 patients with active disease were found to have enhancing lesions.33
Bronchoalveolar carcinoma may mimic BOOP. The white lung sign is not commonly found in pulmonary consolidations that have been assessed with heavily T2-weighted sequences. However, although the sign is usually negative in patients with BOOP, 1 study found the sign to be positive in 5 out of 5 patients with bronchoalveolar carcinoma. Thus, MRI has a potential role in the differential diagnosis of BOOP.34
Gadolinium-based contrast agents (gadopentetate dimeglumine [Magnevist], gadobenate dimeglumine [MultiHance], gadodiamide [Omniscan], gadoversetamide [OptiMARK], gadoteridol [ProHance]) have been linked to the development of nephrogenic systemic fibrosis (NSF) or nephrogenic fibrosing dermopathy (NFD). For more information, see the eMedicine topic Nephrogenic Fibrosing Dermopathy. The disease has occurred in patients with moderate to end-stage renal disease after being given a gadolinium-based contrast agent to enhance MRI or magnetic resonance angiography scans.
NSF/NFD is a debilitating and sometimes fatal disease. Characteristics include red or dark patches on the skin; burning, itching, swelling, hardening, and tightening of the skin; yellow spots on the whites of the eyes; joint stiffness with trouble moving or straightening the arms, hands, legs, or feet; pain deep in the hip bones or ribs; and muscle weakness. For more information, see the FDA Public Health Advisory or Medscape.
Imaging pearls
Imaging pearls
Imaging pearls
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bronchiolitis obliterans organizing pneumonia, BOOP, bronchiolitis obliterans, BO, cryptogenic organizing pneumonia, COP, usual interstitial pneumonia, UIP, chronic interstitial pneumonia, CEP, interstitial pneumonia (IP), hematopoietic stem cell transplantation, HSCT, Ardystil syndrome, nonspecific interstitial pneumonia with fibrosis, proliferative bronchiolitis, pulmonary disease, pneumonia, organizing pneumonia, idiopathic organizing pneumonia
Ali Nawaz Khan, MBBS, FRCS, FRCP, FRCR, Consultant Radiologist and Honorary Professor, North Manchester General Hospital Pennine Acute NHS Trust, UK
Ali Nawaz Khan, MBBS, FRCS, FRCP, FRCR is a member of the following medical societies: American Association for the Advancement of Science, American Institute of Ultrasound in Medicine, British Medical Association, British Society of Interventional Radiology, Royal College of Physicians, Royal College of Physicians and Surgeons of the United States, Royal College of Radiologists, and Royal College of Surgeons of England
Disclosure: Nothing to disclose.
Klaus L Irion, MD, PhD, Consulting Staff, The Cardiothoracic Centre Liverpool NHS Trust, The Royal Liverpool University Hospital, UK
Klaus L Irion, MD, PhD is a member of the following medical societies: American Roentgen Ray Society and Radiological Society of North America
Disclosure: Nothing to disclose.
Simon Hanley, MBBS, MRCP, FRCP, DM, MHS, Consulting Staff, Department of Internal Medicine, North Manchester General Hospital
Simon Hanley, MBBS is a member of the following medical societies: British Cardiac Society
Disclosure: Nothing to disclose.
Sumaira MacDonald, MBChB, PhD, MRCP, FRCR, Lecturer, Sheffield University Medical School; Endovascular Fellow, Sheffield Vascular Institute
Sumaira MacDonald, MBChB, PhD, MRCP, FRCR is a member of the following medical societies: British Medical Association, Royal College of Physicians, and Royal College of Radiologists
Disclosure: Nothing to disclose.
Muthusamy Chandramohan, MBBS, DMRD, FRCR, Consultant Radiologist, Bradford Teaching Hospitals, UK
Disclosure: Nothing to disclose.
Sarah Al Ghanem, MBBS, Consulting Staff, Department of Medical Imaging, King Fahad National Guard Hospital, Saudi Arabia
Disclosure: Nothing to disclose.
Jeffrey A Miller, MD, Associate Professor of Clinical Radiology, University of Medicine and Dentistry of New Jersey; Associate Chief of Service, Department of Radiology, Veterans Affairs of New Jersey Health Care System
Jeffrey A Miller, MD is a member of the following medical societies: North American Society for Cardiac Imaging, Society for Health Services Research in Radiology, and Society of Thoracic Radiology
Disclosure: Nothing to disclose.
Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand
Disclosure: Nothing to disclose.
John D Newell Jr, MD, Professor of Radiology, Co-Director of Thoracic Imaging, Department of Radiology, University of Colorado Health Sciences Center; Professor of Medicine, Medical Director of Lung Imaging Center, National Jewish Medical and Research Center
John D Newell Jr, MD is a member of the following medical societies: American College of Chest Physicians, American College of Radiology, American Roentgen Ray Society, American Thoracic Society, Association of University Radiologists, Radiological Society of North America, and Society of Thoracic Radiology
Disclosure: Siemens Medical Grant/research funds Consulting; Forevision Technologies Ownership interest Consulting; Vida Corporation Ownership interest Board membership; TeraRecon Grant/research funds Consulting; eMedicine Honoraria Consulting
Robert M Krasny, MD, Resolution Imaging Medical Corporation
Robert M Krasny, MD is a member of the following medical societies: American Roentgen Ray Society and Radiological Society of North America
Disclosure: Nothing to disclose.
Eugene C Lin, MD, Consulting Radiologist, Virginia Mason Medical Center; Clinical Assistant Professor of Radiology, University of Washington School of Medicine
Eugene C Lin, MD is a member of the following medical societies: American College of Nuclear Medicine, American College of Radiology, Radiological Society of North America, and Society of Nuclear Medicine
Disclosure: Nothing to disclose.
Related eMedicine topics
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Collagen-Vascular Disease Associated With Interstitial Lung Disease
Pediatrics, Bronchiolitis
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