Cryptogenic Organising Pneumonia

OVERVIEW

Cryptogenic Organising Pneumonia (COP) is also known as bronchiolitis obliterans organising pneumonia (BOOP)

  • it is not the same as bronchiolitis obliterans (another life-threatening condition, characterised by non-reversible obstructive lung disease in which the bronchioles are compressed and narrowed by fibrosis and/or inflammation)
  • the rapidly progressive form has a very poor prognosis

CAUSES

  • idiopathic
  • can follow on from all types of pneumonia
  • drugs (many!)
  • connective tissue disorders
  • industrial toxicants
  • organ transplantation (especially bone marrow and lung)
  • malignancies
  • radiotherapy
Show an exhaustive list of causes…

Post-infection:

Bacterial infection:

  • Mycoplasma pneumoniae
  • Staphylococcus aureus
  • Streptococcus pneumoniae
  • Chlamydia pneumoniae
  • Pseudomonas aeruginosa
  • Legionella pneumophila
  • Nocardia asteroides
  • Coxiella burnetii
  • Serratia marcescens

Viral infection:

  • Herpes virus
  • Influenza virus
  • Parainfluenza virus
  • Human immunodeficiency virus

Drugs:

  • Antimicrobials: Amphotericin, Cephalosporins, sulfa drugs
  • Antiarrhythmics: Amiodarone, sotalol
  • Chemotherapy: Bleomycin, Busulphan
  • Immunosuppressants: Methotrexate, Tacrolimus
  • Antiepileptics: Carbamazepine, Phenytoin
  • Street drugs: Cocaine
  • Other: Gold salts, Interferon alpha, Ticlopidine

Connective tissue/immunologic disease:

  • lupus erythematosus
  • rheumatoid arthritis
  • Sjogren syndrome
  • polymyositis/dermatomycitis
  • Behcet disease
  • Polymylagia rheumatica
  • Ankylosing spondylitis
  • Sweet syndrome
  • Essential mixed cryoglobulinemia
  • Common variable immunodeficiency syndrome

Organ transplantation:

  • Lung, renal, bone marrow transplant

Radiotherapy

Environmental

  • Textile printing dye
  • House fire

Miscellaneous:

  • Inflammatory bowel disease
  • Cancer (solid and hematological)

CLINICAL FEATURES

  • usually presents after 2 weeks to 2 months of illness, initially flu-like
  • nonspecific systemic symptoms: e.g. fevers, chills, night sweats, fatigue, weight loss
  • respiratory symptoms: e.g. dyspnea, cough, respiratory distress

INVESTIGATIONS

Important cause of peripheral consolidation on HRCT

  • Bilateral or unilateral patchy alveolar airspace consolidation is revealed, often subpleural and peribronchial in location and mainly in the lower zones
  • Generally, the infiltrates gradually enlarge from their original size or new infiltrates appear
  • Cavities and nodules may also occur

Other

  • Definitive diagnosis is by lung biopsy
  • Negative microbiology
  • PFT: decreased DLCO, restrictive pattern

MANAGEMENT

Specific therapy

  • COP is usually steroid-responsive, but may relapse when steroids are stopped
  • e.g prednisone 0.75 mg/kg weaned over 6 months
  • non-responsive to antibiotics

Supportive care and monitoring


References and Links

LITFL

Journal articles

  • Al-Ghanem S, Al-Jahdali H, Bamefleh H, Khan AN. Bronchiolitis obliterans organizing pneumonia: pathogenesis, clinical features, imaging and therapy review. Ann Thorac Med. 2008 Apr;3(2):67-75. doi: 10.4103/1817-1737.39641. PubMed PMID: 19561910; PubMed Central PMCID: PMC2700454.
  • Cordier JF. Cryptogenic organising pneumonia. Eur Respir J. 2006 Aug;28(2):422-46. Review. PubMed PMID: 16880372. [Free Full Text]
  • Palmucci S, Roccasalva F, Puglisi S, Torrisi SE, Vindigni V, Mauro LA, Ettorre GC, Piccoli M, Vancheri C. Clinical and radiological features of idiopathic interstitial pneumonias (IIPs): a pictorial review. Insights Imaging. 2014 Jun;5(3):347-64. doi: 10.1007/s13244-014-0335-3. Epub 2014 May 22. PubMed PMID: 24844883; PubMed Central PMCID: PMC4035488.

FOAM and web resources


CCC 700 6

Critical Care

Compendium

Chris is an Intensivist and ECMO specialist at the Alfred ICU in Melbourne. He is also the Innovation Lead for the Australian Centre for Health Innovation at Alfred Health and Clinical Adjunct Associate Professor at Monash University. He is a co-founder of the Australia and New Zealand Clinician Educator Network (ANZCEN) and is the Lead for the ANZCEN Clinician Educator Incubator programme. He is on the Board of Directors for the Intensive Care Foundation and is a First Part Examiner for the College of Intensive Care Medicine. He is an internationally recognised Clinician Educator with a passion for helping clinicians learn and for improving the clinical performance of individuals and collectives.

After finishing his medical degree at the University of Auckland, he continued post-graduate training in New Zealand as well as Australia’s Northern Territory, Perth and Melbourne. He has completed fellowship training in both intensive care medicine and emergency medicine, as well as post-graduate training in biochemistry, clinical toxicology, clinical epidemiology, and health professional education.

He is actively involved in in using translational simulation to improve patient care and the design of processes and systems at Alfred Health. He coordinates the Alfred ICU’s education and simulation programmes and runs the unit’s education website, INTENSIVE.  He created the ‘Critically Ill Airway’ course and teaches on numerous courses around the world. He is one of the founders of the FOAM movement (Free Open-Access Medical education) and is co-creator of litfl.com, the RAGE podcast, the Resuscitology course, and the SMACC conference.

His one great achievement is being the father of two amazing children.

On Twitter, he is @precordialthump.

| INTENSIVE | RAGE | Resuscitology | SMACC

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