Sarcoidosis – causes, symptoms, diagnosis, complications. How is sarcoidosis treated?

Proven content

Sarcoidosis is a disease that attacks the body’s immune system. The hallmark of sarcoidosis is inflammatory nodules, called granulomas, that occur on various organs and are not absorbed. Read about sarcoidosis and what its symptoms may be.

Sarcoidosis – what is it?

Sarcoidosis is an inflammatory disease characterized by the formation of granulomas – small clusters of inflammatory cells – in one or more organs of the body. When the immune system becomes over-dynamic and too many granulomas form, they can disrupt the structure and function of the organ. Uncontrolled chronic inflammation can lead to fibrosis, which is permanent scarring of organ tissue.

The disorder affects the lungs approximately 90% of the time, but can affect almost any organ in the body. Despite the increasing advances in research, sarcoidosis remains difficult to diagnose with limited treatment options and no known cure.

The specificity and severity of the disease vary from person to person. In some cases, the disease goes away on its own. In others, the disease may not progress clinically, but individuals will continue to suffer from certain symptoms that threaten their quality of life. The rest of people – up to a third who are diagnosed with the disease – will require long-term treatment.

Sarcoidosis – causes

No one knows exactly what causes sarcoidosis, although understanding the mechanisms behind the development of sarcoidosis is increasingly advanced. Experts now believe that one or more exposures in people with a specific genetic makeup could cause cells in the body to react and start recruiting inflammatory cells into the organs involved; that is, something triggers an immune response.

Some research suggests that bacteria, viruses, or chemicals can cause the disease. Such triggers, while usually harmless in most people, can irritate the immune systems of people at risk of developing sarcoidosis.

There are theories that the immune response may be overactive or in some cases inadequate, resulting in ongoing inflammation, the formation of granulomas and in some cases scarring or fibrosis. There is increasing evidence that this immune response to sarcoidosis may also involve an “autoimmune” response, with some response to “self” proteins. However, at present it is NOT considered that sarcoidosis is mostly an autoimmune disease such as rheumatoid arthritis (RA) or systemic lupus erythematosus.

1. Articular cartilage preparations in the treatment of RA

While the role of autoimmune mechanisms is still not well understood, some studies have found increased rates of anti-human protein antibodies in some people with sarcoidosis compared to others with and without sarcoidosis. In addition, cells in some people with sarcoidosis show an immune response to some of their own proteins similar to autoimmune diseases.

The exact role of this “autoimmunity” in sarcoidosis is unclear, although it is being actively studied. It’s unclear if the reaction to your own proteins is actually the main culprit behind granulomatous inflammation – it may be the casual observer embroiled in a larger reaction.

The presence of this “autoimmune” process may support the use of some therapies. Right now, while sarcoidosis is not thought to be the same as diseases such as RA or lupus, research shows that some immune responses and genetic factors are similar between these diseases.

Read: Experts: RA patients go to a rheumatologist too late

Sarcoidosis – symptoms

The signs and symptoms of sarcoidosis vary widely depending on the organs affected. While many people with sarcoidosis have very little or no symptoms of the disease, others suffer debilitating effects that can interfere with daily functioning. It is important to discuss all symptoms with your doctor so that he can make an appropriate diagnosis and develop the best treatment plan. When patients are first diagnosed they often have the classic set of symptoms described as Lofgren’s syndrome:

1. fever,
2. enlarged lymph nodes,
3. swollen and painful joints, arthritis,
4. erythema nodosum, raised red and tender lumps that form on the skin, usually on the front of the legs. The nearby joints are often swollen and painful.

Often the presence of erythema nodosum is a good sign of a type of sarcoidosis that also resolves on its own after several months or years, often without treatment.

Because sarcoidosis affects the lungs so often, other common symptoms of sarcoidosis include shortness of breath, wheezing, and chronic coughing. Some people will also experience chest pain, and others will not have any symptoms, even with the current inflammation.

Since sarcoidosis can affect every organ in the body, many different symptoms can be observed, including:

1. general fatigue,
2. unexplained weight loss
3. night sweats
4. feeling sick
5. irregular heartbeat
6. swollen legs
7. headaches,
8. sight problems,
9. weakness or numbness in an arm, leg or part of the face
10. discoloration of the nose, cheeks, mouth and ears,
11. scaly skin rash
12. arthralgia,
13. muscle swelling and soreness
14. arthritis,
15. burning, itchy, watery or sore eyes
16. red eyes
17. sensitivity to light,
18. blurred image.

The editorial board recommends: Is bad weather really responsible for joint pain?

Sarcoidosis and the lungs

The lungs are the most commonly affected organ in sarcoidosis. Ninety percent or more of people with sarcoidosis have their lungs affected, whether they have symptoms or not. Symptoms specific to sarcoidosis affecting the lungs include:

1. dry cough,
2. breathing problems, wheezing or painful breathing
3. chest pain, pressure, or discomfort
4. coughing up blood (which is rare), especially in the early stages of sarcoidosis.

Sarcoidosis that affects the lungs can cause many problems, usually only in the most severe cases. These problems include:

Pulmonary fibrosis: scarring that occurs in the lungs as a result of untreated or persistent inflammation / granuloma formation. As scar tissue builds up in the walls of the air sacs in the lungs, it can obstruct the supply of oxygen to the blood over time, leading to low oxygen levels in the blood and shortness of breath. Pulmonary fibrosis belongs to a larger group of lung diseases called ‘interstitial lung disease’.

Fibrocystic disease: advanced lung disease in which scar tissue is blocking the airways in one or both of the lungs

Pulmonary hypertension: a condition in which scar tissue narrows or blocks the arteries in the lungs, causing high blood pressure. High blood pressure, also known as hypertension, forces the heart to work harder to pump blood through the vessels. Over time, the heart muscle grows and weakens, making it difficult for it to deliver oxygen to the lungs and other parts of the body.

Bronchiectasis: a condition in which the walls of the bronchi or airways in the lungs thicken due to chronic inflammation / granuloma formation. Over time, the bronchi are unable to clear the mucus. The mucus then builds up, creating an environment in which bacteria can thrive. Over time, the airways become inflamed, stretched, and scarred, making it difficult for air to pass through the lungs.

aspergilloma: a clump of fungus that forms in healed lung scars and enlarged airways and can cause bleeding in the lungs.

See: During her period, she coughed up blood. A second uterus was growing in her lungs

Sarcoidosis and lymph nodes

Sarcoidosis affects the lymph nodes in up to 90 percent of people with this disease. Lymph nodes are glands throughout the body that produce and store white blood cells. When sarcoid inflammation attacks these glands, they become enlarged. Swollen lymph nodes can be uncomfortable but rarely cause health problems unless they constrict organs or blood vessels.

Most often, the lymph nodes in the chest are affected, but this can be difficult to spot without an x-ray. Some of the other places affected by enlarged lymph nodes (which look like swollen lumps) include:

1. neck,
2. under the chin,
3. smells,
4. groin.

Unlike the common cold or flu, swollen lymph nodes in these areas are usually not sensitive when associated with sarcoidosis.

Read: Tattoos dangerous to health! They harm the lymph nodes and can cause serious infections

Sarcoidosis and the eyes

Sarcoidosis affects the eyes in at least a quarter of people with the disease. In many cases, these problems resolve on their own within a year. Many eye problems can be treated with a variety of methods.

Eye symptoms may include:

1. burning, itching and / or pain
2. dry eyes
3. red eyes
4. vision problems such as seeing black spots or blurred vision
5. sensitivity to light,
6. small, pale yellow bumps on the eye.

Eye problems caused by sarcoidosis can include:

1. Uveitis (the most common eye problem), i.e. inflammation of the middle membrane – or uveitis – of the eye,
2. Dry eye syndrome (also called keratoconjunctivitis), which occurs when the tear ducts are blocked by inflammation.
3. Enlargement of the tear glands (also called enlargement of the tear glands), which occurs when the tear glands are swollen by inflammation and granulomas,
4. Glaucoma, cataracts and blindnesswhich are rare but serious problems that can occur if uveitis is not treated.

The editorial board recommends: A silent thief of sight. It develops insidiously and gives no symptoms for a long time

Sarcoidosis – skin

About a quarter of people with sarcoidosis will develop skin problems related to the disease. Often these skin problems can indicate the severity of a case of sarcoidosis.

Skin problems can include:

1. Erythema nodosum: which causes raised, red, and tender lumps to form on the skin, usually on the front of the legs. The nearby joints are often swollen and painful. Erythema nodosum usually resolves on its own within six to eight weeks, even without treatment. Often the presence of erythema nodosum is a good sign of a type of sarcoidosis that also resolves on its own after a few months or years.
2. Lupus pernio: which is a rare skin condition that causes hard, reddish-purple lumps to form on the cheeks, nose, mouth and / or ears. These nodules do not go away on their own and often come back after stopping treatment. They are usually associated with chronic sarcoidosis. In some cases, wounds become disfiguring and can damage cartilage and bone.
3. Other lumps on or under the skin, rashes, sores, peeling, and / or changes to old scars or tattoos that do not go away and are rarely sore or itchy. These skin problems are related to a type of sarcoidosis that lasts a long time.

See: Adult skin cells can be converted to cartilage

Sarcoidosis and the brain and nervous system

Sarcoidosis affects the brain and nervous system in up to 15 percent of cases. While it can affect almost any part of the nervous system, cranial nerves, which are important nerves at the base of the brain, are often involved.

Brain and nervous system symptoms can include:

1. facial palsy (also called Bell’s palsy), which causes drooping and / or paralysis on one side of the face (the most common symptom of the nervous system)
2. headaches,
3. eye pain and redness
4. blurred or double vision
5. blindness,
6. weakness, numbness, tingling and / or pain in the face, arms and / or legs
7. paralysis of the arms and / or legs,
8. epileptic seizures,
9. behavior and mood changes, irritability, memory loss and hallucinations (which are rare).

Problems caused by sarcoidosis of the nervous system can include:

1. Inflammation and / or Nerve Damage: which causes many of the symptoms described above, depending on which nerves are affected. For example, facial paralysis results from damage to a nerve or nerves at the base of the brain.
2. Peripheral neuropathy: which is a condition that affects the nerves’ ability to transmit information between the brain and spinal cord and the rest of the body. May cause the weakness and numbness described previously
3. Tire masses of granulomas cerebral or less commonly in the brain: which cause the headaches, vision problems and muscle weakness previously described. These masses can also cause meningitis, hydrocephalus, neuroendocrine disorders and coma.
4. Meningitis: that is, inflammation of the meninges, the membranes surrounding the brain and spinal cord,
5. Hydrocephalus: This is an excess of cerebrospinal fluid in the skull. May occur with or after meningitis
6. Neuroendocrine Disorders: which affect the interaction of the nervous and endocrine systems (the endocrine system produces and secretes hormones). When sarcoidosis damages an area at the base of the brain called the hypothalamus, it can lead to neuroendocrine disorders such as hypopituitarism (hypopituitarism).
7. Coma: although this is rare.

See: Night sweats after vaccination against COVID-19. Is this a disturbing symptom?

Sarcoidosis — diagnosis

Full diagnosis of this disease is carried out in the hospital. First of all, lung imaging tests are performed. A chest X-ray is very important in classifying the disease. On its basis, 5 stages of sarcoidosis are distinguished, on which treatment depends. However, in order to be able to confirm the diagnosis, morphological tests are required, which allow the collection of tissue in which the presence of characteristic granulomas can be visualized under a microscope.

Such examination may be bronchoscopy, i.e. inserting an endoscope through the oral cavity into the respiratory system. This makes it possible to retrieve a lymph node. Depending on the course and duration of the disease, as well as which organs are affected by the granulomas, other additional tests may be necessary.

Sarcoidosis – treatment

Medical treatment can be used to control symptoms, prevent complications, and improve outcomes in people with persistent sarcoidosis. If you have sarcoidosis, your doctor will monitor you closely to see if your sarcoidosis is getting better or worse, and will adjust your treatment depending on how your body is doing. Many doctors choose treatment when the quality of life is serious or when they believe there is a risk of organ damage or death.

In many cases of sarcoidosis, treatment is not necessary and sarcoidosis may resolve without treatment. The disease may never reappear, or it may reappear later in life. However, many other patients require consistent treatment of the ongoing effects of sarcoidosis.

Sarcoidosis is often treated with a multidisciplinary team of health professionals. As the disease can affect many organ systems, you can work with health professionals who specialize in treating the lungs, heart, brain, kidneys, liver, eyes, and skin.

Sarcoidosis — leki na sarcoidzę

The types of drugs used to treat sarcoidosis are mainly corticosteroids and drugs that suppress the immune system.

Corticosteroids: corticosteroids reduce inflammation. Corticosteroids are the primary treatment for sarcoidosis. Treatment with corticosteroids relieves symptoms in most people within a few months. The most commonly used corticosteroids are prednisone and prednisolone. People with sarcoidosis may need to take corticosteroids for many months.

Because these drugs can cause side effects (including weight gain, insomnia, mood swings, acne, difficulty controlling blood sugar, and bone loss), your doctor will gradually reduce your medication dose once you start taking them. The goal is to stabilize symptoms without unnecessary exposure to more corticosteroids than necessary to control the disease.

Drugs that suppress the immune system: because sarcoidosis can be the result of an overreaction of the immune system, suppressing it can relieve symptoms and prevent further organ damage.

Antimalarial drugs: These drugs were originally used (and are still used today) to treat malaria. In treating sarcoidosis, these drugs will most likely be effective in people with skin symptoms or high blood calcium levels. Hydroxychloroquine (Plaquenil) and chloroquine (Aralen) are antimalarial drugs used to treat sarcoidosis. Both can cause stomach irritation and eye problems.

People with severe or advanced pulmonary sarcoidosis may need oxygen therapy. In a few cases, lung transplantation – replacing a diseased lung or lungs with a healthy donor lung – is used to treat severe lung sarcoidosis.