Lymphangioleiomyomatosis (LAM)

Lymphangioleiomyomatosis, usually called LAM, is a rare, long-term and progressive condition. LAM almost always occurs in females.

• Rare – happens in around 3 – 8 females per million people
• Long-term – a condition you’ll always have
• Progressive – a condition that gets worse over time

LAM mainly affects your lungs, but it can also affect your kidneys and lymphatic system. The lymphatic system is a network of tubes (vessels) and glands (nodes) in your body that help fight infection and remove excess fluid.

LAM is caused by changes in the tuberous sclerosis complex (TSC) genes. This causes the growth of abnormal cells, called LAM cells.

LAM cells grow throughout your lungs, causing holes or cysts, blocking your airways and preventing your lungs from getting oxygen to the rest of your body. 

There are two ways you can get LAM:

• on its own – this is called sporadic LAM. The cause of sporadic LAM is currently unclear. Sporadic LAM is not inherited, and it's not passed on to children.
• with a rare inherited condition called tuberous sclerosis. Tuberous sclerosis is passed down from a parent through family genes. Many people who have tuberous sclerosis also get LAM. But most people with LAM do not have tuberous sclerosis.

It’s thought the female hormone oestrogen encourages LAM cells to grow. But it’s not a direct cause.

Some people with LAM have no or very mild symptoms.

Symptoms include:

• breathlessness, which may get worse the longer you have the condition and at the same time as your period
• feeling very tired
• chest pain
• coughing
• wheezing
• stomach bloating
• coughing. Sometimes you may cough up blood if your lungs are affected.

Complications of LAM

LAM affects everyone differently. Complications of LAM include:

• a collapsed lung (pneumothorax)
• fluid around your lungs (pleural effusion)
• angiomyolipomas (kidney tumours). They’re usually harmless, but they can bleed and cause pain.
• low oxygen levels, especially when exercising. Low oxygen levels that do not get better are usually only a problem for people with advanced LAM.

If you have a collapsed lung, fluid around your lung or large, growing kidney tumours, you might need additional hospital treatment.

Speak to your healthcare professional about the signs of complications. They can tell you what you should do if you notice them.

It may take some time to get a diagnosis because LAM is rare.

LAM is usually suspected when lung cysts are seen on a CT scan or in females with tuberous sclerosis.

There are other causes of lung cysts, so you may also have blood tests or a lung biopsy.

You should also have an ultrasound or CT scan of the stomach.

In the UK, the National Centre for LAM is at Nottingham University Hospital. If LAM is suspected, it’s likely that you’ll need to go there to be diagnosed and treated.

LAM progresses at different rates. Not everyone will need treatment.

There’s currently no cure. But you may be given rapamycin (sirolimus) if you have an active case of LAM. 

Rapamycin is an immunosuppressant medicine, which means it helps to calm your body’s immune system. If taken continuously, this medicine can slow down how quickly your condition progresses.

If you’re breathless, you may be prescribed inhalers and offered pulmonary rehabilitation.

You may also be assessed for oxygen therapy if your blood oxygen levels are low. If you need oxygen therapy, you may need to have it during exercise. Other people may need it continuously.

A lung transplant may be an option for people with very severe LAM.

If you have LAM, you should:

• eat a regular healthy diet
• keep active
• stay up-to-date with your regular vaccinations, including the flu, COVID-19 and pneumonia vaccines.

If you smoke, take a look at our information on stopping smoking to help you quit.

It’s thought oestrogen products can make LAM worse. This includes contraceptive pills and hormone replacement therapy (HRT).

If you have LAM and are pregnant, or are considering having a baby, talk to your specialist team. There’s a possibility that pregnancy can make LAM worse.

Living with a long-term condition that gets worse over time like LAM can be difficult. Speak to your health care professional if you feel like you’re struggling emotionally. Support is there if you need it.

We also have information on looking after your mental health.

Useful resources

• National centre for LAM – the centre for LAM diagnosis in the UK, based in Nottingham. The NHS recommend keeping some information about LAM with you in case of an emergency.
• LAM Action – a charity which supports you and your family if you have LAM, and your health care professionals.