Sarcoidosis

Sarcoidosis is a condition in which the immune system forms tiny clusters of inflammatory cells, called granulomas, in one or more organs. These small areas of inflammation are what produce its effects, wherever in the body they happen to settle. Why this happens is not fully understood. It is thought to follow an exaggerated immune response, possibly to an environmental trigger, in someone genetically predisposed, but no single cause has been identified. It is not contagious, does not spread from person to person, and there is no clear evidence that it is inherited.

Sarcoidosis can occur at any age, though it most often appears in younger and middle-aged adults. It affects both men and women, a little more often in women, and occurs across all ethnic backgrounds, though it is more common in people of African or African-Caribbean heritage and in those of Scandinavian descent. One of the more reassuring things to know at the outset is that for many people it settles on its own, often within a few years and without any treatment.

Sarcoidosis is very variable, and its symptoms depend on which organs are involved and how active the inflammation is. Some people have no symptoms at all and are found to have sarcoidosis after a routine chest X-ray; others feel generally unwell while it is active. Common general symptoms include:

• Tiredness, which can be considerable and is often the most wearing part
• A mild fever, night sweats, or a general sense of being run down
• Aches, a reduced appetite, or some unintended weight loss

Beyond these, the symptoms come from wherever the inflammation settles, which the next section describes.

The lungs and lymph glands are involved most often, in about 9 in 10 people. This can cause a persistent dry cough, breathlessness or a feeling of chest tightness, although many people have changes on a chest scan with few or no symptoms. In a smaller number, longer-standing inflammation can lead to scarring of the lung tissue; our guide to interstitial lung disease explains that side of things and how it is monitored.

The skin can be affected, with rashes, small raised lumps, or changes in skin colour.

The eyes are a part of the body sarcoidosis can involve, and this deserves attention rather than reassurance alone. The most common eye problem is uveitis, inflammation inside the eye, which can threaten sight if it is not treated. A red, painful or light-sensitive eye, or new blurring of vision, should be seen the same day by an eye specialist. Our guide to uveitis explains the eye inflammation linked to rheumatic conditions and why it is taken so seriously.

The joints, muscles and bones can be involved too. When sarcoidosis affects the joints, there may be pain and swelling, often in the ankles and frequently on both sides. In one well-recognised form, swollen ankles occur together with tender red lumps on the shins and other features, and this pattern often settles over time. In some people, a more persistent arthritis affecting other joints can occur. Muscle and bone involvement is less common. Much of the joint involvement is very manageable once it is recognised and placed in the wider picture.

The nervous system is involved less often, in about 1 in 20 people. With specialist care, this is usually treatable.

The heart is involved in about 1 in 50 people with sarcoidosis. Heart involvement is often silent, causing no symptoms and being found only when a scan is done, and it is looked into when symptoms such as palpitations or breathlessness, or a finding on examination, give a reason to. Where the heart is affected, it is looked after together with a cardiologist, alongside the treatment of the sarcoidosis itself. Our guide to inflammation and your heart covers how inflammatory conditions can affect the heart more generally.

Diagnosis brings together the pattern of symptoms, an examination, blood tests, imaging such as a chest X-ray or CT scan, and sometimes a small sample of tissue (a biopsy), often in coordination with the other specialties involved in your care. Recognising the different threads as parts of one condition is important, as it links the picture together and guides treatment.

Where the joints are involved, ultrasound can help assess inflammation accurately. Dr Borukhson uses point-of-care ultrasound during the consultation, so the joints can often be examined in the same visit. You can read more on the ultrasound clinic page.

Many people need no treatment at all, because the inflammation settles by itself; in around 6 in 10 people it gets better without it. When treatment is needed, the aim is to calm the inflammation and protect the organs involved.

Steroid tablets are the usual first treatment where one is required, and they often work well. Where steroids alone are not enough, or cannot be reduced without the inflammation returning, a steroid-sparing medicine that calms the immune system, such as methotrexate, may be added so the steroid dose can be kept as low as possible. Where a single joint is troublesome, an ultrasound-guided injection may help. Any treatment is considered alongside the care for sarcoidosis in other organs, so the plans work together rather than against each other, and it is always agreed with you.

Sarcoidosis can affect several organs at once, so care frequently involves more than one specialty, such as respiratory medicine, rheumatology, ophthalmology and, where the heart is involved, cardiology. Dr Borukhson practises within a world-renowned tertiary centre, with ready access to consultant colleagues across the other specialties that may be involved in caring for this condition. Where appropriate, she can involve those specialists directly, and bring particularly complex cases to a multidisciplinary team meeting (MDT) with minimal delay. This means that, when more than one area of expertise is needed, your care can be joined up and decisions reached promptly.

Much of what sarcoidosis brings is manageable, and many people carry on with their usual lives, particularly as the inflammation settles. A few steady habits help:

• Keep your appointments, as sarcoidosis can change over time and is best reviewed regularly
• Mention new symptoms in any part of the body, since the condition can move beyond where it started
• Keep your eye checks up to date, as some eye inflammation can be quiet early on
• If steroids are advised, take them as directed and never stop them suddenly

Fatigue is common and real. Be kind to yourself, prioritise sleep, and lean on those around you while things settle.

Most symptoms of sarcoidosis can be reviewed routinely, but some deserve prompter attention.

Seek same-day eye assessment at an eye casualty or urgent eye clinic for a newly red, painful or light-sensitive eye, or new blurring of vision, because uveitis can threaten sight and early treatment protects it.

Seek prompt medical advice, too, for new or worsening breathlessness, chest pain, palpitations, or a joint that becomes hot, very swollen or rapidly worsening, or for joint symptoms that come with a fever or feeling generally unwell. Arrange a routine review for a persistent cough, joint pain or swelling, or any new symptom you are unsure about. If you ever feel severely unwell, treat it as an emergency rather than waiting.

Sarcoidosis is often mild and settles on its own, but because it can involve several organs, it is worth having the whole picture assessed and placed in context rather than viewed one symptom at a time. A specialist review can clarify what is going on, arrange the right tests, bring in the other specialties where they are needed, and agree a plan that fits your wider care. If you have sarcoidosis and develop new symptoms, or unexplained symptoms that might fit the condition, a specialist assessment can help.

What is sarcoidosis, and can I catch it or pass it on?

Sarcoidosis happens when the immune system over-reacts and forms small lumps of inflammation, called granulomas, in one or more organs. Nobody fully knows why it starts, but it is thought to be an immune reaction rather than anything you have caught or done wrong. It is not passed from person to person, and it does not appear to be inherited in any clear way. Reassuringly, in many people it is mild and eases over time, often without any treatment.

Which parts of the body does sarcoidosis affect?

It varies a great deal from person to person. The lungs and the lymph glands in the chest are involved most often, in the great majority of people, so a cough or breathlessness is a common way for it to show itself. Sarcoidosis can also affect the skin, the eyes and the joints, and less commonly the nervous system and the heart. Some people have no symptoms and are found to have it after a routine chest X-ray. Because the picture depends on where the inflammation settles, assessment looks at the body as a whole rather than one symptom at a time.

Will I need treatment, and what does it involve?

Often not. For a majority of people, roughly six in ten, the inflammation quietens by itself and no medication is required, although it is still worth being reviewed from time to time. When something is needed, a course of steroid tablets is the usual starting point and tends to help. If steroids do not settle it fully, or the dose cannot be lowered without symptoms coming back, a further medicine that calms the immune system, such as methotrexate, can be added to spare the steroids. Whatever is used is chosen with your care for other organs in mind, so nothing pulls in opposite directions.

I have sarcoidosis. When should I worry about my eyes or my heart?

For the eyes, treat any sudden redness, pain, light sensitivity or new blurring as urgent: have it checked the same day at an eye service, because sarcoidosis can inflame the eye (uveitis) and, left untreated, that can damage sight. For the heart, involvement is uncommon and usually causes nothing you would notice, but new palpitations, breathlessness or blackouts deserve prompt review, and if the heart is affected it is looked after jointly with a cardiologist alongside your sarcoidosis treatment.

Why would I see a rheumatologist for sarcoidosis?

Sarcoidosis often involves more than one part of the body, and a rheumatologist is well placed to assess the joints and muscles, help piece the wider picture together, and work alongside the other specialties involved. Where the joints are affected, Dr Borukhson uses point-of-care ultrasound during the consultation, so inflammation can often be assessed in the same visit. Where care needs several specialists, she can involve them directly or bring complex cases to a multidisciplinary team meeting promptly.