Introduction

Cryptogenic organizing pneumonia (COP) is a diffuse interstitial lung disease that affects the distal bronchioles, respiratory bronchioles, alveolar ducts and alveolar walls. It affects around 2 people per 100,000. The aetiology is unknown.

Epidemiology

  • Incidence: 2.00 cases per 100,000 person-years
  • Peak incidence: 50-60 years
  • Sex ratio: 1:1
<1 1-5 6+ 16+ 30+ 40+ 50+ 60+ 70+ 80+

Clinical features

Males and females are equally affected and tend to present in the 5th or 6th decade of life and is not associated with smoking. Patients typically present with a cough, shortness of breath, fever and malaise. Symptoms can be present for weeks or months. There is often a history of non-response to antibiotics. Haemoptysis is rare. Clinical examination is often normal but inspiratory crackles can be heard. Wheeze and clubbing are rare.

Investigations

Bloods show a leukocytosis and an elevated ESR and CRP. Imaging typically shows bilateral patchy or diffuse consolidative or ground glass opacities. Lung function tests are most commonly restrictive but can be obstructive or normal. The transfer factor is reduced.

Management

Treatment is watch and wait if mild or high dose oral steroids if severe.