Sarcoidosis: what is it, types, symptoms, treatment, is it curable?

Contents

What is sarcoidosis?

Sarcoidosis is a disease characterized by the appearance of non-infectious inflammatory granulomas in several organs, such as lungs, skin, ganglia, among others.

The appearance of spots and rashes on the skin is a situation that irritates many women, who promptly go to the dermatologist for help. It turns out that sometimes the problem is not necessarily aesthetic. This is the case of sarcoidosis, a disease characterized by granulomas in several organs – including the skin.

Granulomas are accumulations of inflammatory cells that seek to eliminate possible infectious agents that, in the case of sarcoidosis, do not exist. In addition to the appearance of these granulomas, the disease also has peculiar signs such as an enlarged liver and changes in calcium metabolism.

Also called Besnier-Boeck-Schaumman disease, sarcoidosis is an inflammatory disease little known by medicine. It affects more women than men and its consequences and symptoms vary widely in severity from case to case. The organs most commonly affected are the lung (90% of cases), lymph nodes, skin, liver and eyes.

In the United States, the disease is more common in black women, and the complications of the disease are also more severe for this group. However, sarcoidosis ends up being quite common in Scandinavian countries like Sweden and Finland, where the black population is a minority. Therefore, it is not clear whether ethnicity influences the onset of the disease.

It is estimated that 10 in 100,000 people in Brazil are affected by sarcoidosis.

What are the causes?

It is not well known why granulomas are formed, but how they are formed is not a very big mystery.

It is believed that, due to a kind of breakdown in the immune system, certain parts of the body send inflammatory responses that promote the accumulation of macrophages – conjunctive cells that “eat” foreign elements such as microorganisms, are part of the defense mechanism against infections – in certain parts of the body. This accumulation is what makes up granulomas.

In general, the body produces these granulomas to isolate microorganisms and foreign bodies that may be doing harm to cells. The strange thing is that, in the case of sarcoidosis, there is no foreign element inside the granulomas.

While some experts believe that it is triggered by exogenous antigens, that is, substances and microorganisms outside the patient’s body, there are also those who believe that the problem is in self-antigens: something that the body itself produces is interpreted as a threat.

The fact that nothing is found to be isolated by granulomas leads many to believe that it is an autoimmune disease. In the end, there is no consensus on the origin of sarcoidosis.

Types

Sarcoidosis is classified according to the affected organs. Because it is a systemic disease, it can affect several tissues and organs, and having a type of sarcoidosis does not mean being free from others, as several organs can be affected at the same time. Understand:

Cutaneous sarcoidosis

When it affects the skin, it is called cutaneous sarcoidosis. The most common manifestations are granulomas on the skin, characterized by reddish and purplish rashes, which can be hot and painful when touching.

Pulmonary sarcoidosis

In most cases, the main affected organ is the lung, and granulomas are not visible without the help of medical tests. It is characterized by respiratory symptoms such as coughing and wheezing when breathing.

Cardiac sarcoidosis

The heart, unfortunately, is not free from disease. Just like in the lung, granulomas can only be seen through exams and the main symptoms are chest pain, shortness of breath and feeling faint.

Ocular sarcoidosis

In ocular sarcoidosis, the main affected are the eyes. They have redness, blurred vision and pain.

Groups of risk

Although sarcoidosis can affect anyone, there are some groups that are more likely to suffer from the problem. Are they:

  • People between 20 and 40 years old: Sarcoidosis appears most commonly between 20 and 40 years old, although it can reach other ages as well. However, it is extremely rare during childhood;
  • Women: For some reason, sarcoidosis affects more women than men;
  • Afrodescendants: Especially in the United States, people of African descent are most affected by the disease;
  • Scandinavians: Across Europe, Scandinavians seem to be the most affected by sarcoidosis;
  • People with a family history: Although there is nothing to prove a possible inheritance in sarcoidosis, people with a family history are more likely to develop the disease.

Is sarcoidosis transmissible?

Do not be afraid to approach if someone in your life has been diagnosed, as there is no evidence that sarcoidosis can be transmissible. In addition, no type of microorganism involved in the disease has ever been found. Just don’t let your guard down for other illnesses, see?

Symptoms

Fatigue , fever , swollen lymph nodes and weight loss are just the initial and nonspecific symptoms of sarcoidosis. Regardless of the affected organ, these are the first manifestations of the disease, which can be easily confused with any type of common infection of everyday life – that’s why they are called “non-specific symptoms”.

The thing takes shape when the symptoms related to the organs appear. However, sometimes the disease can be completely asymptomatic .

Some clearer symptoms are:

In the lung

  • Persistent dry cough;
  • Shortness of breathe;
  • Wheezing;
  • Chest pain.

In the skin

  • Elevated skin lesions that may be in the form of reddened nodules or plaques, usually hot and painful to touch;
  • Nodules under the skin, especially near scars and tattoos;
  • Lighter or darker stains;
  • Injuries to areas of the face such as nose, cheeks and ears.

In the eyes

  • Blurred vision;
  • Pain in the eyes;
  • Redness;
  • Photosensitivity (sensitivity to light).

In the heart

  • Chest pain;
  • Shortness of breathe;
  • Feeling faint (syncope);
  • Fatigue;
  • Arrhythmias;
  • Palpitations;
  • Swelling caused by fluid accumulation (edema).

Lupus pernio

When talking about sarcoidosis, it is impossible not to mention lupus pernium, one of the possible cutaneous manifestations in the disease.

Characterized by high-relief lesions with red coloring that pull to purple, this condition is related to worse prognosis and little probability of total resolution of the disease.

Lupus pernium is most often found in parts of the face such as the nose, ear, cheeks, lips and forehead.

How is sarcoidosis diagnosed?

As one of the first symptoms noticed of sarcoidosis are skin rashes, mainly by women, the biggest tendency is to look for a dermatologist who, although he can help in the diagnosis and treatment of the disease, is only one of the doctors who should be consulted.

Other specialized professionals who can help in this task are rheumatologist (treats autoimmune diseases ), pulmonologist (takes care of the lungs) and ophthalmologist (treats eye diseases).

As there is no specific exam for this diagnosis, the following tests must be performed:

Physical exam

As it is a disease that can be easily confused with other autoimmune diseases, the doctor should first look for signs of the disease. Thus, he must:

  • Examine skin lesions;
  • Check for swelling in the lymph nodes;
  • Listening to the heart and lungs, which may present different sounds;
  • Palpate the liver area to see an enlarged organ and local pain.

The results of this physical examination may increase or decrease the suspicion of sarcoidosis. When there are many signs compatible with the disease, the doctor should order a battery of extra tests.

Xrays

In order to check for the presence of granulomas in the internal organs such as the lung and heart, as well as swelling in lymph nodes that do not appear on the skin, the doctor may order an X-ray or a CT scan.

If there is a suspicion of involvement of the heart or nervous system, an MRI may also be requested, which facilitates the visualization of these organs.

Spirometry

Because granulomas in the lung affect breathing capacity, spirometry can help determine if there is something wrong with that organ.

Bronchoscopy

Bronchoscopy is an examination that inserts a microcamera into the bronchi in order to be able to effectively see the path to the lung. Not infrequently, some of the tissue is removed and sent for analysis to investigate the composition of the granulomas.

Biopsy

Biopsies are samples of affected tissue that go for laboratory analysis to check for the presence and composition of granulomas. These biopsies can be of the skin, in the case of cutaneous manifestations, of the lung and of the lymph nodes.

Bloodtests

Sarcoidosis is able to alter the result of several blood tests, especially those that measure kidney and liver function.

In the complete blood count , the values ​​indicate anemia (low number of red blood cells), leukopenia and lymphopenia (decrease in leukocytes and lymphocytes). In the urinary calcium test, which measures kidney function, there is an increase in calcium in the urine (hypercalcemia).

Is sarcoidosis curable?

Unfortunately, there is no cure for sarcoidosis. However, it is believed that it is a self-limited disease, that is, it goes away on its own, since in a large part of the cases, it lasts between 12 and 36 months.

Cases where the disease lasts longer than that, such as decades, are rare. In fact, many people have a small sarcoidosis “crisis” that resolves on its own and has never even been diagnosed with the disease.

What is the treatment for sarcoidosis?

Although a large number of patients do not need treatment because of mild symptoms, others may end up needing treatment for many years. These, however, are more rare cases.

In general, the patient must take steps to maintain good general health in order to avoid complications over time. Some of these measures are:

  • Eat properly with a good amount of fruits and vegetables;
  • Do medical check-ups frequently;
  • Drink plenty of water every day;
  • Sleep adequately for 6 to 8 hours a night;
  • Practice physical exercises and maintain weight;
  • Stop smoking in order to avoid pulmonary complications.

Organ transplantation

In rare cases, when the patient has suffered very serious damage to organs such as the heart, lung and liver, some doctors may opt for an organ transplant in order to guarantee a better quality of life.

Sarcoidosis medications

When needed, sarcoidosis drugs are focused on reducing symptoms and reducing tissue inflammation. Therefore, anti-inflammatory drugs are used , especially corticosteroids, which mimic the action of inflammation-regulating hormones.

Other drugs used are immunosuppressants and inhibitors of tumor necrosis factor α , a protein responsible for the death of tumor cells and very active in autoimmune diseases.

Drug treatment usually lasts from 1 to 2 years, depending on the duration of the disease. However, there are cases where the patient needs to take medication for decades, as the disease does not resolve.

Certain medications are administered orally, while others, especially in the case of cutaneous sarcoidosis, are in the form of a topical cream or ointment.

Some examples of sarcoidosis medications are:

Corticosteroid anti-inflammatory drugs

  • Prednisone ;
  • Methylprednisolone .

Immunosuppressants

  • Methotrexate ;
  • Azathioprine ;
  • Chlorambucil ;
  • Cyclophosphamide ;
  • Cyclosporine .

Tumor necrosis factor α inhibitors

  • Adalimumab ;
  • Certolizumab pegol ;
  • Golimunab ;
  • Infliximab ;
  • Etanercept .

Complications

We know that sarcoidosis can be a very mild disease that, in fact, does not bother at all. However, in some people, the symptoms are quite severe and serious sequelae are possible. Are they:

Pulmonary fibrosis

Granulomas that form in the lung can leave scars that develop into fibrosis. This process “hardens” the lung and impairs breathing.

It is estimated that 30% of patients with sarcoidosis have lung function affected by organ damage.

Ophthalmic conditions

When it affects the eyes, inflammation can damage eye tissues and cause blindness, cataracts and glaucoma .

Kidney stones and kidney failure

By affecting the way the body metabolizes calcium, sarcoidosis can lead to the formation of kidney stones, which can cause injury, which culminates in chronic kidney failure.

Heart problems

Cardiac injuries can lead to abnormal heart rhythms, which impair circulation.

Nervous system

Very rarely, granulomas form in the nervous system – both in the brain and in the spinal cord. These inflammation in the nerves can lead to paralysis and difficulty in controlling certain parts of the body.

Death

In sarcoidosis, mortality varies from 1 to 10% of cases and can occur due to complications such as respiratory, renal failure and heart problems. However, the disease rarely progresses to that stage.

How to prevent sarcoidosis?

Since it is not a communicable disease, measures against microorganisms will not help prevent sarcoidosis. Trying to prevent chronic diseases does not seem to help sarcoidosis either.

In fact, there are no effective measures to prevent the disease. However, maintaining a healthy lifestyle will certainly help you stay well in the event of a diagnosis.


Although it is often perceived only as an aesthetic discomfort, sarcoidosis is a disease that affects several organs. With mild symptoms, people are often unaware of its danger.

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