Organising pneumonia: symptoms, diagnosis and treatment

Organising pneumonia is a pattern of lung inflammation in which organised swirls of inflammation form in the small airways (bronchioles) and tiny air sacs (alveoli). Asthma + Lung UK explains that the word cryptogenic means the cause is unknown, a term that can also be written as idiopathic. If a cause is known, the condition is called secondary organising pneumonia.

The charity classifies COP as a type of interstitial lung disease, a group of conditions that damage lung tissue, with over 200 types. COP was previously called bronchiolitis obliterans organising pneumonia (BOOP), but it is not the same as bronchiolitis obliterans, so the name was changed. You may see both organising and organizing spellings.

The key reassurance is that organising pneumonia is not an infection and is not contagious. It is the lungs’ own inflammatory response, which normally helps to fight infection and heal injury, that has become overactive in a particular area.

Asthma + Lung UK describes symptoms as similar to pneumonia and flu, but usually developing gradually over several months. Sometimes they begin after an infection such as flu, bronchitis or a chest infection, which is one reason the diagnosis can be missed at first.

• A dry cough.

• Feeling breathless.

• A sudden high temperature (38 degrees Celsius or above) or chills, with sweating or shivering.

• Unexplained weight loss.

• Muscle aches and pains.

It is not known what causes COP. Asthma + Lung UK lists factors that may raise the risk: being aged between 50 and 60, although it can happen at any age; having an inflammatory or autoimmune disease such as lupus, rheumatoid arthritis or scleroderma; taking certain medicines including some antibiotics, anti-inflammatory and chemotherapy drugs; and having had a lung transplant.

When a clear trigger such as a medicine or an autoimmune disease is identified, doctors may call it secondary organising pneumonia, and treatment may also involve dealing with that trigger. A specialist will decide which applies to you.

Your GP will ask about your symptoms and medical history and may refer you to a specialist. Asthma + Lung UK lists the tests that help to confirm the diagnosis: a chest X-ray or CT scan to show changes in the lungs, blood tests to check for infection or an inflammatory condition (important because COP treatments can make an infection worse), lung function tests, and sometimes a bronchoscopy, where a small flexible tube passes into the lungs to take samples of cells and fluid.

It can take time to reach a diagnosis. The charity explains that your GP will want to rule out ordinary pneumonia and other conditions that cause similar symptoms. Hearing that it may take some time is understandable, but the tests above are how the picture becomes clear.

Treatment depends on how unwell you are. Asthma + Lung UK says some people with mild symptoms do not need treatment and should monitor their symptoms, contacting a GP or specialist if they get worse. For those who do need treatment, the length of treatment differs for everyone.

Asthma + Lung UK says that symptoms can start to improve within a week of starting treatment and that most people make a full recovery after 6 to 12 months of treatment. COP generally has a very good outlook, but it can take several weeks or months to recover fully.

Some people get symptoms again when the steroid dose is reduced or stopped. If that happens, the charity says your medicine may be changed to another type of immunosuppressant. In very rare cases ongoing inflammation may cause permanent scarring of the lungs, known as pulmonary fibrosis, but most people with COP do not develop this.

Because treatment with steroids is usually long, your specialist team will also monitor for side effects. Always talk to your GP or specialist before stopping steroids you have been prescribed.

Tell your GP if you have any new or worsening symptoms. For people with an interstitial lung disease, Asthma + Lung UK advises calling 999 or going to A&E immediately if you suddenly feel much more breathless, are finding it very hard to breathe, are coughing up a lot of blood, have new or severe chest pain, or feel unusually drowsy or confused.

If you are on steroids or immunosuppressants and develop a fever or feel generally unwell, tell your team promptly, because a weakened immune system makes infections more likely.

Living with a lung condition can be hard at times. Asthma + Lung UK suggests talking to someone you trust if you are struggling, and offers support groups, online communities and a helpline. Pulmonary rehabilitation, if you are suitable, can help you manage breathlessness and build stamina.