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Diseases

Organizing pneumonia

OVERVIEW

  • Highlights & Basics
  • Images

DIAGNOSIS

  • Diagnostic Approach
  • Risk Factors
  • History & Exam
  • Tests
  • Differential Diagnosis
  • Criteria
  • Screening

TREATMENT

  • Tx Approach
  • Tx Options
  • Emerging Tx
  • Prevention

FOLLOW-UP

  • Overview
  • Complications

REFERENCES

  • Citations
  • Guidelines
  • Credits

PATIENT RESOURCES

  • Patient Instructions

Highlights & Basics

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Key Highlights
  • Organizing pneumonia (OP) is an inflammatory disorder involving both the peripheral bronchioles and alveoli simultaneously. It has distinctive radiographic findings, histologic features, and response to corticosteroids (unlike usual interstitial pneumonia).

  • OP may be caused by multiple insults such as medication, infection, rheumatologic disease, autoimmune disease, post transplantation, radiation, and environmental causes. In cryptogenic organizing pneumonia, a cause cannot be elicited after a careful history, examination and pertinent laboratory studies.

  • High-resolution chest computed tomography scan shows bilateral patchy triangular ground-glass opacities with air bronchograms usually located peripherally.

  • Most often, diagnosis is made using clinico-radiologic criteria and usually in the setting of a multidisciplinary team. However, lung biopsy may be required to establish the definitive diagnosis in patients with unusual findings.

  • Cryptogenic OP may require treatment, and prednisone is the most commonly used drug.

High-resolution chest CT showing bilateral ground-glass opacities and a posterior triangular-based i
High-resolution chest CT showing bilateral ground-glass opacities and a posterior triangular-based infiltrate with an air bronchogram
From the collection of Gary R. Epler, MD

Quick Reference

  • History & Exam

    • Key Factors

      • Other Factors

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      • Diagnostics Tests

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        • Treatment Options

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          Definition

          Epidemiology

          Etiology

          Pathophysiology

          content by BMJ Group
          Last updated

          Images

          • Medium-powered pathology slide showing circular and branching bronchioles filled with polypoid plugs

            Medium-powered pathology slide showing circular and branching bronchioles filled with polypoid plugs of granulation tissue and alveoli filled with organizing pneumonia

          • ​Algorithmic approach to organizing pneumonia. ∗ A formal MDD may not be required in all cases, espe

            ​Algorithmic approach to organizing pneumonia. ∗ A formal MDD may not be required in all cases, especially if the combination of clinical context and radiographic pattern is sufficiently convincing of the OP diagnosis. In such cases, a discussion between the physician and the radiologist is strongly encouraged. CRP = C-reactive protein; DAH = diffuse alveolar hemorrhage; Dx = diagnosis; ESR = erythrocyte sedimentation rate; GGO = ground-glass opacification; HRCT = high-resolution CT; MDD = multidisciplinary discussion; nl Pro-Cal C = normal procalcitonin; nl WBC = normal WBC; OP = organizing pneumonia

          • Chest x-ray showing bilateral patchy infiltrates

            Chest x-ray showing bilateral patchy infiltrates

          • High-resolution chest CT showing bilateral ground-glass opacities and a posterior triangular-based i

            High-resolution chest CT showing bilateral ground-glass opacities and a posterior triangular-based infiltrate with an air bronchogram

          Citations

            Key Articles

            • Epler GR, Colby TV, McLoud TC, et al. Bronchiolitis obliterans organizing pneumonia. N Engl J Med. 1985 Jan 17;312(3):152-8.[Abstract]

            • Epler GR. Bronchiolitis obliterans organizing pneumonia, 25 years: a variety of causes, but what are the treatment options? Expert Rev Respir Med. 2011 Jun;5(3):353-61.[Abstract]

            • Bradley B, Branley HM, Egan JJ, et al; British Thoracic Society Interstitial Lung Disease Guideline Group, British Thoracic Society Standards of Care Committee; Thoracic Society of Australia; New Zealand Thoracic Society; Irish Thoracic Society. Interstitial lung disease guideline. Thorax. 2008 Sep;63 Suppl 5:v1-58.[Abstract][Full Text]

            • Travis WD, Costabel U, Hansell DM, et al. An official American Thoracic Society/European Respiratory Society statement: Update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 2013 Sep 15;188(6):733-48.[Abstract][Full Text]

            • Lazor R, Vandevenne A, Pelletier A, et al. Cryptogenic organizing pneumonia: characteristics of relapses in a series of 48 patients. Am J Respir Crit Care Med. 2000 Aug;162(2 Pt 1):571-7.[Abstract][Full Text]

            Referenced Articles

            • 1. Epler GR, Colby TV. The spectrum of bronchiolitis obliterans. Chest. 1983 Feb;83(2):161-2.[Abstract][Full Text]

            • 2. Epler GR, Colby TV, McLoud TC, et al. Bronchiolitis obliterans organizing pneumonia. N Engl J Med. 1985 Jan 17;312(3):152-8.[Abstract]

            • 3. Epler GR. Bronchiolitis obliterans organizing pneumonia, 25 years: a variety of causes, but what are the treatment options? Expert Rev Respir Med. 2011 Jun;5(3):353-61.[Abstract]

            • 4. Bradley B, Branley HM, Egan JJ, et al; British Thoracic Society Interstitial Lung Disease Guideline Group, British Thoracic Society Standards of Care Committee; Thoracic Society of Australia; New Zealand Thoracic Society; Irish Thoracic Society. Interstitial lung disease guideline. Thorax. 2008 Sep;63 Suppl 5:v1-58.[Abstract][Full Text]

            • 5. Travis WD, Costabel U, Hansell DM, et al. An official American Thoracic Society/European Respiratory Society statement: Update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 2013 Sep 15;188(6):733-48.[Abstract][Full Text]

            • 6. Chang J, Han J, Kim DW, et al. Bronchiolitis obliterans organizing pneumonia: clinicopathologic review of a series of 45 Korean patients including rapidly progressive form. J Korean Med Sci. 2002 Apr;17(2):179-86.[Abstract][Full Text]

            • 7. Chakravorty I, Oldfield WL, Gómez CM. Rapidly progressive bronchiolitis obliterans organizing pneumonia presenting with pneumothorax, persistent air leak, acute respiratory distress syndrome and multi-organ dysfunction: a case report. J Med Case Reports. 2008 May 6;2:145.[Abstract][Full Text]

            • 8. Oymak FS, Demirbas HM, Mavili E, et al. Bronchiolitis obliterans organizing pneumonia: clinical and roentgenological features in 26 cases. Respiration. 2005 May-Jun;72(3):254-62.[Abstract]

            • 9. Maldonado F, Daniels CE, Hoffman EA, et al. Focal organizing pneumonia on surgical lung biopsy: causes, clinicoradiologic features, and outcomes. Chest. 2007 Nov;132(5):1579-83.[Abstract][Full Text]

            • 10. Almaslmani M, Derbala MF, Albozom I, et al. Bronchiolitis obliterans organizing pneumonia associated with Pneumocystis jiroveci infection in orthotopic liver transplantation. Transpl Infect Dis. 2008 Oct;10(5):339-42.[Abstract]

            • 11. Epler GR. Bronchiolitis obliterans organizing pneumonia (BOOP) induced by drugs and radiotherapy. In: Drug-induced and iatrogenic respiratory disease. London, UK: Hodder Arnold; 2010:268-79.

            • 12. Fenton ME, Kanthan R, Cockcroft DW. Nitrofurantoin-associated bronchiolitis obliterans organizing pneumonia: report of a case. Can Respir J. 2008 Sep;15(6):311-2.[Abstract][Full Text]

            • 13. Mori S, Cho I, Koga Y, et al. A simultaneous onset of organizing pneumonia and rheumatoid arthritis, along with a review of the literature. Mod Rheumatol. 2008;18(1):60-6.[Abstract]

            • 14. Sohn DI, Laborde HA, Bellotti M, et al. Juvenile rheumatoid arthritis and bronchiolitis obliterans organized pneumonia. Clin Rheumatol. 2007 Feb;26(2):247-50.[Abstract]

            • 15. Garg R, Soud Y, Lal R, et al. Myelodysplastic syndrome manifesting as Sweet's syndrome and bronchiolitis obliterative organizing pneumonia. Am J Med. 2006 Nov;119(11):e5-7.[Abstract]

            • 16. Burton CM, Iversen M, Carlsen J, et al. Interstitial inflammatory lesions of the pulmonary allograft: a retrospective analysis of 2697 transbronchial biopsies. Transplantation. 2008 Sep 27;86(6):811-9.[Abstract][Full Text]

            • 17. Yotsumoto S, Okada F, Yotsumoto S, et al. Bronchiolitis obliterans organizing pneumonia after bone marrow transplantation: association with human leukocyte antigens. J Comput Assist Tomogr. 2007 Jan-Feb;31(1):132-7.[Abstract]

            • 18. Yoshihara S, Yanik G, Cooke KR, et al. Bronchiolitis obliterans syndrome (BOS), bronchiolitis obliterans organizing pneumonia (BOOP), and other late-onset noninfectious pulmonary complications following allogeneic hematopoietic stem cell transplantation. Biol Blood Marrow Transplant. 2007 Jul;13(7):749-59.[Abstract][Full Text]

            • 19. Bissoli L, Di Francesco V, Valbusa F, et al. A case of bronchiolitis obliterans organizing pneumoniae (BOOP) after nine months post-operative irradiation for breast cancer. Age Ageing. 2008 Mar;37(2):235.[Abstract][Full Text]

            • 20. Ogo E, Komaki R, Fujimoto K, et al. A survey of radiation-induced bronchiolitis obliterans organizing pneumonia syndrome after breast-conserving therapy in Japan. Int J Radiat Oncol Biol Phys. 2008 May 1;71(1):123-31.[Abstract][Full Text]

            • 21. Katayama N, Sato S, Katsui K, et al. Analysis of factors associated with radiation-induced bronchiolitis obliterans organizing pneumonia syndrome after breast-conserving therapy. Int J Radiat Oncol Biol Phys. 2009 Mar 15;73(4):1049-54.[Abstract][Full Text]

            • 22. Epler GR, Kelly EM. Post-breast cancer radiotherapy bronchiolitis obliterans organizing pneumonia. Respir Care. 2020 May;65(5):686-92.[Abstract][Full Text]

            • 23. Alleman T, Darcey DJ. Case report: bronchiolitis obliterans organizing pneumonia in a spice process technician. J Occup Environ Med. 2002 Mar;44(3):215-6.[Abstract]

            • 24. Ragno G, Brunetti G, Scafa F, et al. Rhabdomyolysis in a worker exposed to paraffinic mineral oils: unusual association with bronchiolitis obliterans and organizing pneumonia (BOOP) [in Italian]. G Ital Med Lav Ergon. 2007 Jul-Sep;29(3 Suppl):437-8.[Abstract]

            • 25. Rose JJ, Krishnan-Sarin S, Exil VJ, et al. Cardiopulmonary impact of electronic cigarettes and vaping products: a scientific statement from the American Heart Association. Circulation. 2023 Aug 22;148(8):703-28.[Abstract][Full Text]

            • 26. Mokhtari M, Bach PB, Tietjen PA, et al. Bronchiolitis obliterans organizing pneumonia in cancer: a case series. Respir Med. 2002 Apr;96(4):280-6.[Abstract][Full Text]

            • 27. Martinez-Gallo M, Puy C, Ruiz-Hernandez R, et al. Severe and recurrent episodes of bronchiolitis obliterans organizing pneumonia associated with indolent CD4+ CD8+ T-cell leukemia. Eur Respir J. 2008 Jun;31(6):1368-72.[Abstract][Full Text]

            • 28. Miyagawa-Hayashino A, Wain JC, Mark EJ. Lung transplantation biopsy specimens with bronchiolitis obliterans or bronchiolitis obliterans organizing pneumonia due to aspiration. Arch Pathol Lab Med. 2005 Feb;129(2):223-6.[Abstract][Full Text]

            • 29. Mukhopadhyay S, Katzenstein AL. Pulmonary disease due to aspiration of food and other particulate matter: a clinicopathologic study of 59 cases diagnosed on biopsy or resection specimens. Am J Surg Pathol. 2007 May;31(5):752-9.[Abstract]

            • 30. Kilic D, Findikcioglu A, Kocer E, et al. Unusual manifestation of bronchiolitis obliterans organizing pneumonia. Act Chir Belg. 2008 Jul-Aug;108(4):468-70.[Abstract]

            • 31. Gudmundsson G, Sveinsson O, Isaksson HJ, et al. Epidemiology of organising pneumonia in Iceland. Thorax. 2006 Sep;61(9):805-8.[Abstract][Full Text]

            • 32. Raghu G, Meyer KC. Cryptogenic organising pneumonia: current understanding of an enigmatic lung disease. Eur Respir Rev. 2021 Sep 30;30(161).[Abstract]

            • 33. Epler GR. Bronchiolitis obliterans organizing pneumonia: definition and clinical features. Chest. 1992;102(1 suppl):2S-6S.[Abstract][Full Text]

            • 34. Cazzato S, Zompatori M, Baruzzi G, et al. Bronchiolitis obliterans-organizing pneumonia: an Italian experience. Respir Med. 2000 Jul;94(7):702-8.[Abstract][Full Text]

            • 35. Zhang Y, Li N, Li Q, et al. Analysis of the clinical characteristics of 176 patients with pathologically confirmed cryptogenic organizing pneumonia. Ann Transl Med. 2020 Jun;8(12):763.[Abstract][Full Text]

            • 36. Zhou Y, Wang L, Huang M, et al. A long-term retrospective study of patients with biopsy-proven cryptogenic organizing pneumonia. Chron Respir Dis. 2019 Jan-Dec;16:1479973119853829.[Abstract][Full Text]

            • 37. Lappi-Blanco E, Soini Y, Pääkkö P. Apoptotic activity is increased in the newly formed fibromyxoid connective tissue in bronchiolitis obliterans organizing pneumonia. Lung. 1999;177(6):367-76.[Abstract]

            • 38. Forlani S, Ratta L, Bulgheroni A, et al. Cytokine profile of broncho-alveolar lavage in BOOP and UIP. Sarcoidosis Vasc Diffuse Lung Dis. 2002 Mar;19(1):47-53.[Abstract]

            • 39. Majeski EI, Paintlia MK, Lopez AD, et al. Respiratory reovirus 1/L induction of intraluminal fibrosis, a model of bronchiolitis obliterans organizing pneumonia, is dependent on T lymphocytes. Am J Pathol. 2003 Oct;163(4):1467-79.[Abstract][Full Text]

            • 40. Cottin V, Cordier JF. Cryptogenic organizing pneumonia. Semin Respir Crit Care Med. 2012 Oct;33(5):462-75.[Abstract][Full Text]

            • 41. Ould Kadi F, Abdesslam T, Nemery B. Five-year follow-up of Algerian victims of the "Ardystil syndrome". Eur Respir J. 1999 Apr;13(4):940-1.[Abstract][Full Text]

            • 42. Baha A, Yildirim F, Kokturk N, et al. 18F-FDG uptake in focal organising pneumonia mimicking bronchial carcinoma. Clin Respir J. 2016 Nov;10(6):740-5.[Abstract]

            • 43. Vadász I, Husain-Syed F, Dorfmüller P, et al. Severe organising pneumonia following COVID-19. Thorax. 2021 Feb;76(2):201-4.[Abstract][Full Text]

            • 44. Tonon CR, Tanni SE, Rocha J, et al. Organizing pneumonia and COVID-19. Am J Med Sci. 2023 Dec;366(6):458-63.[Abstract][Full Text]

            • 45. Fata F, Rathore R, Schiff C, et al. Bronchiolitis obliterans organizing pneumonia as the first manifestation of polymyositis. South Med J. 1997 Feb;90(2):227-30.[Abstract]

            • 46. Tazelaar HD, Viggiano RW, Pickersgill J, et al. Interstitial lung disease in polymyositis and dermatomyositis: clinical features and prognosis as correlated with histologic findings. Am Rev Respir Dis. 1990 Mar;141(3):727-33.[Abstract]

            • 47. Costa AN, Carraro RM, Nascimento EC, et al. Acute Fibrinoid Organizing Pneumonia in Lung Transplant: The Most Feared Allograft Dysfunction. Transplantation. 2016 Mar;100(3):e11-2.[Abstract][Full Text]

            • 48. American College of Radiology. ACR appropriateness criteria​: chronic dyspnea-noncardiovascular origin. 2024 [internet publication].[Full Text]

            • 49. American College of Radiology. ACR appropriateness criteria: diffuse lung disease​. 2021 [internet publication].[Full Text]

            • 50. Smedemark SA, Aabenhus R, Llor C, et al. Biomarkers as point-of-care tests to guide prescription of antibiotics in people with acute respiratory infections in primary care. Cochrane Database Syst Rev. 2022 Oct 17;10(10):CD010130.[Abstract][Full Text]

            • 51. Nagata N, Wakamatsu K, Kumazoe H, et al. Clinical significance of intra-alveolar fibrin deposition in transbronchial lung biopsy in patients with organizing pneumonia. Lung. 2015 Apr;193(2):203-8.[Abstract]

            • 52. Johannson KA, Marcoux VS, Ronksley PE, et al. Diagnostic yield and complications of transbronchial lung cryobiopsy for interstitial lung disease. A systematic review and metaanalysis. Ann Am Thorac Soc. 2016 Oct;13(10):1828-38.[Abstract][Full Text]

            • 53. Cherian SV, Patel D, Machnicki S, et al. Algorithmic approach to the diagnosis of organizing pneumonia: a correlation of clinical, radiologic, and pathologic features. Chest. 2022 Jul;162(1):156-78.[Abstract][Full Text]

            • 54. Tertemiz KC, Alpaydın AÖ, Güler N, et al. Transbronchial lung cryobiopsy for the diagnosis of diffuse parenchymal lung disease: pitfalls and challenges, a single center experience. Turk J Med Sci. 2023 Feb;53(1):100-8.[Abstract][Full Text]

            • 55. Park IN, Jegal Y, Kim DS, et al. Clinical course and lung function change of idiopathic nonspecific interstitial pneumonia. Eur Respir J. 2009 Jan;33(1):68-76.[Abstract][Full Text]

            • 56. Choi SI, Jung WJ, Lee EJ. Korean guidelines for diagnosis and management of interstitial lung diseases: part 4. cryptogenic organizing pneumonia. Tuberc Respir Dis (Seoul). 2021 Jul;84(3):171-5.[Abstract][Full Text]

            • 57. Beasley MB, Franks TJ, Galvin JR, et al. Acute fibrinous and organizing pneumonia: a histological pattern of lung injury and possible variant of diffuse alveolar damage. Arch Pathol Lab Med. 2002 Sep;126(9):1064-70.[Abstract][Full Text]

            • 58. Lazor R, Vandevenne A, Pelletier A, et al. Cryptogenic organizing pneumonia: characteristics of relapses in a series of 48 patients. Am J Respir Crit Care Med. 2000 Aug;162(2 Pt 1):571-7.[Abstract][Full Text]

            • 59. Stover DE, Mangino D. Macrolides: a treatment alternative for bronchiolitis obliterans organizing pneumonia? Chest. 2005 Nov;128(5):3611-7.[Abstract][Full Text]

            • 60. Ichikawa Y, Ninomiya H, Katsuki M, et al. Low-dose/long-term erythromycin for treatment of bronchiolitis obliterans organizing pneumonia (BOOP). Kurume Med J. 1993;40(2):65-7.[Abstract][Full Text]

            • 61. Purcell IF, Bourke SJ, Marshall SM. Cyclophosphamide in severe steroid-resistant bronchiolitis obliterans organizing pneumonia. Respir Med. 1997 Mar;91(3):175-7.[Abstract][Full Text]

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