Sarcoidosis: What is sarcoidosis and what are its causes/symptoms? Pulmonary and extrapulmonary forms

Sarcoidosis: What is sarcoidosis and what are its causes/symptoms? Pulmonary and extrapulmonary forms
Photo source: Getty images

Sarcoidosis is the name for a rare disease affecting various organ systems. Why does this rare disease occur and what symptoms characterize it?

Characteristics

Sarcoidosis is an autoimmune disease affecting various organs and parts of the body. It is manifested by the formation of small inflammatory nodules. Most often the lung area is affected, but the disease can attack any organ.

The etiology of sarcoidosis, its symptoms, treatment options and much interesting information can be found in the following article.

What is sarcoidosis?

A disease in which granulomas (small nodules of inflammatory cells) form in organs. The most commonly affected organs are the lungs and the respiratory system.

This multisystem disease is rare. It occurs mainly between the ages of 30 and 40. It affects the female sex more often.

Sarcoidosis is also known as Besnier-Boeck disease. Sarcoidosis is divided into acute and chronic forms according to the duration and intensity of onset of symptoms.

There is the formation of clumps and inflammatory foci of cells of the body's own immune system. As a result of this autoimmune disorder, the body reacts by destroying its own cells.

In sarcoidosis, there is a misreaction of the body's own immune system and, as a result, damage to the tissues of organs in the body.

The immune cells attack the organs' own tissues. They form clusters, called foci, in the affected tissues. This is a granulomatous form of the disease.

In addition to the lungs, sarcoidosis often affects the lymph nodes, liver, heart, pancreas, kidneys, skin, bones and eye area. In some cases, the salivary glands, nervous system and other areas of the body may be affected.

Common manifestations of sarcoidosis are increased body temperature, fever, enlarged lymph nodes and weight loss. Due to frequent involvement of the lungs, there is increased shortness of breath and the development of a dry cough.

Sarcoidosis cannot be completely cured, but its clinical symptoms can be significantly reduced.

Causes

Sarcoidosis is a group of autoimmune diseases in which the immune system attacks its own cells.

The exact cause of the disease is unknown.

The exact cause of sarcoidosis has not yet been identified because it is an autoimmune disease.

The disease occurs mainly in younger to middle-aged people, women and smokers. The disease may be more severe in black and Native American populations. However, it is more common in Europeans.

Risk factors for sarcoidosis are mainly the presence of a family history of the disease, more severe forms of bacterial and viral infections(Lyme disease, tuberculosis, etc.). Inhalation of heavy metals such as silicon or silicon is also a risk factor.

However, no microorganism or environmental factor has yet been definitively confirmed or refuted.

Sarcoidosis - formation of granulomas
Sarcoidosis. Formation of granulomas: inflammatory nodules in the respiratory system. Source: Getty Images

Symptoms

The manifestations of sarcoidosis are varied due to the pathophysiology of the disease.

In general, however, one can expect enlarged lymph nodes (anywhere on the body), weight loss, increased fatigue and fever.

The most common symptom is lung involvement leading to dry cough, chest pain and shortness of breath. In some cases, coughing up of blood occurs.

When the skin is affected, a specific pinkish-brown rash, erythema and redness appear.

Sarcoidosis can affect the eye area (uveitis, visual disturbances, photophobia, redness, soreness and others).

Attack on the heart is mainly manifested by chest pain, arrhythmias (irregular heart rhythm), palpitations to cardiomyopathy or impaired cardiac function.

Common accompanying symptoms are increased potassium levels (hypercalcaemia) in the blood and, conversely, anaemia.

Sarcoidosis may initially be asymptomatic. As part of the enlargement of the lymph nodes, respiratory problems such as dyspnoea, difficulty breathing, chest pain and dry cough also begin. An increase in blood calcium levels is typical.

If sarcoidosis is in acute form, it has clinical signs of an infectious disease. Fever, sore throat, muscle and joint pain, swollen ankles and redness or a specific bumpy rash on the skin appear.

In the chronic form of the disease, increased fatigue, weight loss in conjunction with inappetence are especially present in the long term.

Whole-body lymph node enlargement, joint pain, visual impairment, chest pain, and the development of respiratory or cardiac disorders are common.

Possible manifestations and symptoms of sarcoidosis:

  • Enlarged lymph nodes
  • Breathing difficulties
  • Chest pain
  • Dry cough
  • Red specific rash
  • Joint pain and swelling
  • Increased fatigue
  • Weight loss
  • Irregular heartbeat
  • Eye pain and impaired vision

Diagnostics

Diagnosis of sarcoidosis is quite challenging. Currently, there is no specific test or diagnostic method for this disease.

The diagnosis is usually established only after several examinations of the affected organs and internal systems. Its exact course depends on the clinical symptoms of the patient.

Diagnosis includes blood tests designed primarily to exclude anaemia and elevated serum calcium levels. Sarcoidosis is an inflammatory disease. Therefore, in the acute phase, the erythrocyte sedimentation rate and CRP are elevated in the blood sample.

Immunological tests, respiratory examinations, bronchoscopy, liver and kidney tests or electrocardiography are performed individually according to the physician's indication and the patient's clinical symptoms.

Instrumental imaging methods such as X-ray, MRI, CT, PET or ultrasound are indicated. Tissue biopsy of the affected organ may also be performed.

Sarcoidosis is most often diagnosed by chest X-ray, which shows enlarged lymph nodes and lung involvement. However, CT (computed tomography) is the most sensitive for sarcoidosis.

Biopsy with histological examination is indicated especially when there is diagnostic ambiguity, such as unclear skin processes. During the biopsy examination, a special thin needle is inserted into the affected area. A small tissue sample is taken and then laboratory diagnosis is made.

Sarcoidosis can be confirmed by a special blood test called SACE. This is an enzyme whose concentration in the serum is very often used as an indicator of sarcoidosis activity.

Prognosis of sarcoidosis

A large proportion of patients have mild clinical symptoms. More severe cases respond well to established treatment and usually resolve within a few years.

Quality of life is not significantly reduced in most cases of sarcoidosis and patients are able to live a full life. However, patients are at risk of a return of clinical symptoms, i.e. recurrence of the disease. Therefore, regular follow-ups with a doctor are necessary.

The mortality rate for sarcoidosis is relatively low - approximately 5%.

April - Month and World Sarcoidosis Awareness Day
13 April - World Sarcoidosis Awareness Day. Source: Getty Images

How it is treated: Sarcoidosis

Treatment of sarcoidosis: drugs and sometimes surgery

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