Bronchiolitis obliterans organizing pneumonia


Bronchiolitis obliterans organizing pneumonia BOOP, also known as cryptogenic organizing pneumonia, is a form of non-infectious pneumonia; more specifically, BOOP is an inflammation of the bronchioles bronchiolitis1 and surrounding tissue in the lungs2 It is often a complication of an existing chronic inflammatory disease such as rheumatoid arthritis, dermatomyositis, or it can be a side effect of certain medications such as amiodarone BOOP was first described by Gary Epler in 19853

Some authors have recommended the use of an alternate name, cryptogenic organizing pneumonia COP,45 to reduce confusion with bronchiolitis obliterans, a distinct and unrelated disease6

The clinical features and radiological imaging resemble infectious pneumonia However, diagnosis is suspected after there is no response to multiple antibiotics, and blood and sputum cultures are negative for organisms

Contents

  • 1 Terminology
  • 2 Signs and symptoms
  • 3 Causes
  • 4 Diagnosis
    • 41 Imaging
  • 5 Complications
  • 6 Treatment
  • 7 References
  • 8 External links

Terminologyedit

"Organizing" refers to unresolved pneumonia in which the alveolar exudate persists and eventually undergoes fibrosis in which fibrous tissue forms in the alveoli The phase of resolution and/or remodeling following bacterial infections is commonly referred to as organizing pneumonia, both clinically and pathologically

Signs and symptomsedit

The classic presentation of COP is the development of nonspecific systemic eg, fevers, chills, night sweats, fatigue, weight loss and respiratory eg difficulty breathing, cough symptoms in association with filling of the lung alveoli that is visible on chest x-ray7 This presentation is usually so suggestive of an infection that the majority of patients with COP have been treated with at least one failed course of antibiotics by the time the true diagnosis is made7

Causesedit

  • Pulmonary infection by bacteria, viruses and parasites
  • Drugs: antineoplastic drugs, erlotinib
  • Toxic fumes
  • Ionizing radiations89
  • Inflammatory diseases
    • Systemic lupus
    • Rheumatoid arthritis RA-associated BOOP
    • Scleroderma
  • Bronchial obstruction
    • Proximal bronchial squamous cell carcinoma10

It was identified in 1985, although its symptoms had been noted before but not recognised as a separate lung disease The risk of BOOP is higher for people with inflammatory diseases like lupus, dermatomyositis, rheumatoid arthritis, and scleroderma11

Diagnosisedit

On clinical examination, crackles are common, and more rarely, patients may have clubbing <5% of cases Laboratory findings are nonspecific

Almost 75% of people have symptoms for less than two months before seeking medical attention A flu-like illness, with a cough, fever, a feeling of illness malaise, fatigue, and weight loss heralds the onset in about 40% of patients Doctors do not find any specific abnormalities on routine laboratory tests or on a physical examination, except for the frequent presence of crackling sounds called rales upon auscultation with a stethoscope by the care provider Pulmonary function tests usually show that the amount of air the lungs can hold is below normal The amount of oxygen in the blood is often low at rest and is even lower with exercise

Imagingedit

CT scan showing bronchiolitis obliterans organizing pneumonia biopsy-proven

The chest x-ray is distinctive with features that appear similar to an extensive pneumonia, with both lungs showing widespread white patches The white patches may seem to migrate from one area of the lung to another as the disease persists or progresses Computed tomography CT may be used to confirm the diagnosis Often the findings are typical enough to allow the doctor to make a diagnosis without ordering additional tests To confirm the diagnosis, a doctor may perform a lung biopsy using a bronchoscope Many times, a larger specimen is needed and must be removed surgically

Plain chest radiography shows normal lung volumes, with characteristic patchy unilateral or bilateral consolidation Small nodular opacities occur in up to 50% of patients and large nodules in 15% On high resolution computed tomography, airspace consolidation with air bronchograms is present in more than 90% of patients, often with a lower zone predominance A subpleural or peribronchiolar distribution is noted in up to 50% of patients Ground glass appearance or hazy opacities associated with the consolidation are detected in most patients

Pulmonary physiology is restrictive with a reduced diffusion capacity of the lung for carbon monoxide DLCO Airflow limitation is uncommon; gas exchange is usually abnormal and mild hypoxemia is common Bronchoscopy with bronchoalveolar lavage reveals up to 40% lymphocytes, along with more subtle increases in neutrophils and eosinophils In patients with typical clinical and radiographic features, a transbronchial biopsy that shows the pathologic pattern of organizing pneumonia and lacks features of an alternative diagnosis is adequate to make a tentative diagnosis and start therapy On surgical lung biopsy, the histopathologic pattern is organizing pneumonia with preserved lung architecture; this pattern is not exclusive to BOOP and must be interpreted in the clinical context

Histologically, cryptogenic organizing pneumonia is characterized by the presence of polypoid plugs of loose organizing connective tissue Masson bodies within alveolar ducts, alveoli, and bronchioles

Complicationsedit

Rare cases of BOOP have induced with lobar cicatricial atelectasis12

Treatmentedit

Most patients recover with corticosteroid therapy13 A standardized approach to dosing starting at 075 mg/kg and weaning over 24 weeks has been shown to reduce total corticosteroid exposure without affecting outcome

About two thirds of patients recover with corticosteroid therapy: the usual corticosteroid administered is prednisolone in Europe and prednisone in the USA; these differ by only one functional group and have the same clinical effect The corticosteroid is initially administered in high dosage, typically 50 mg per day tapering down to zero over a six-month to one-year periodcitation needed If the corticosteroid treatment is halted too quickly the disease may return Other medicationsvague must be taken to counteract side effects of the steroid

Referencesedit

  1. ^ "bronchiolitis obliterans with organizing pneumonia" at Dorland's Medical Dictionary
  2. ^ White, Eric J Stern, Charles S 1999 Chest radiology companion Philadelphia: Lippincott Williams & Wilkins p 76 ISBN 978-0-397-51732-9 
  3. ^ Epler GR June 2011 "Bronchiolitis obliterans organizing pneumonia, 25 years: a variety of causes, but what are the treatment options" Expert Rev Respir Med 5 3: 353–61 PMID 21702658 doi:101586/ers1119 
  4. ^ Alasaly K, Muller N, Ostrow DN, Champion P, FitzGerald JM July 1995 "Cryptogenic organizing pneumonia A report of 25 cases and a review of the literature" Medicine Baltimore 74 4: 201–11 PMID 7623655 doi:101097/00005792-199507000-00004 
  5. ^ Geddes DM August 1991 "BOOP and COP" Thorax 46 8: 545–7 PMC 463266  PMID 1926020 doi:101136/thx468545 
  6. ^ Cotran, Ramzi S; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L; Abbas, Abul K 2005 Robbins and Cotran pathologic basis of disease St Louis, Mo: Elsevier Saunders p 731 ISBN 0-7216-0187-1 
  7. ^ a b "Pulmonary Question 27: Diagnose cryptogenic organizing pneumonia" MKSAP 5 For Students Online American College of Physicians Retrieved 23 November 2012 
  8. ^ Nogi, S; Nakayama, H; Tajima, Y; Okubo, M; Mikami, R; Sugahara, S; Akata, S; Tokuuye, K 2014 "Cryptogenic organizing pneumonia associated with radiation: A report of two cases" Oncology Letters 7 2: 321–324 PMC 3881924  PMID 24396439 doi:103892/ol20131716 
  9. ^ Oie, Y; Saito, Y; Kato, M; Ito, F; Hattori, H; Toyama, H; Kobayashi, H; Katada, K 2013 "Relationship between radiation pneumonitis and organizing pneumonia after radiotherapy for breast cancer" Radiation Oncology 8: 56 PMC 3605133  PMID 23497657 doi:101186/1748-717X-8-56 
  10. ^ Radzikowska, E; Nowicka, U; Wiatr, E; Jakubowska, L; Langfort, R; Chabowski, M; Roszkowski, K 2007 "Organising pneumonia and lung cancer - case report and review of the literature" Pneumonologia i Alergologia Polska 75 4: 394–7 PMID 18080991 
  11. ^ Al-Ghanem Sara; Al-Jahdali Hamdan; Bamefleh Hanaa; Khan Ali Nawaz Apr–Jun 2008 "Bronchiolitis obliterans organizing pneumonia: Pathogenesis, clinical features, imaging and therapy review" Ann Thorac Med 3 2: 67–75 PMC 2700454  PMID 19561910 doi:104103/1817-173739641 
  12. ^ Yoshida, K; Nakajima, M; Niki, Y; Matsushima, T 2001 "Atelectasis of the right lower lobe in association with bronchiolitis obliterans organizing pneumonia" Nihon Kokyuki Gakkai zasshi = the journal of the Japanese Respiratory Society 39 4: 260–5 PMID 11481825 
  13. ^ Oymak FS, Demirbaş HM, Mavili E, et al 2005 "Bronchiolitis obliterans organizing pneumonia Clinical and roentgenological features in 26 cases" Respiration 72 3: 254–62 PMID 15942294 doi:101159/000085366 

External linksedit

  • Support & Information for COP & BOOP
  • "Idiopathic Interstitial Pneumonias" Merck Manual Professional May 2008 


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