Help the development of the site, sharing the article with friends!

Sarcoidosis, or Besnier-Boeck-Schaumann disease, is an autoimmune disease that affects practically the entire body, affecting primarily young people. The symptoms of sarcoidosis are often confused with tuberculosis or other respiratory disease. So far, no definite cause of sarcoidosis has been identified, although there are many hypotheses about this. Importantly, sarcoidosis is not an infectious disease, so you cannot catch it.

Sarcoidosis(Besnier-Boeck-Schaumann disease, Latinsarcoidosis ) isautoimmune disease (autoimmune disease). The immune system, used to destroying intruders that enter the body, suddenly becomes too active in some people.

In the internal organs, the formation ofgranulomas , i.e. small inflammatory lumps, which, unlike tuberculosis, are non-keratinizing granulomas. Granulomas are formed from inflammatory cells such as lymphocytes and macrophages.

Although sarcoidosis can affect any organ, it is most commonly found in the lungs and lymph nodes, primarily in the mediastinum. This is why sarcoidosis is included in theinterstitial lung disease . This means that it produces heterogeneous, diffuse changes in the lungs, which lead toimpaired gas exchange.

Sarcoidosis - who gets sick most often?

Sarcoidosis is considered a disease primarily of young people, mainly in thethird decade of life (20-30 years) . In women, the second peak incidence is between50. and 60 years of age .

Although there is no definite gender predominance, sarcoidosis is thought to be slightly more common inwomen .

It has also been observed that sarcoidosis is found quite often in people inScandinavian countries , and thatblack racegets sick about ten times more often than the rest of the population.

Sarcoidosis - symptoms

Sarcoidosis in its initial course often has no symptoms . However, as the disease progresses, the most common symptoms are lymphadenopathy, i.e. generalized enlargement of the lymph nodes.

Often in sarcoidosis the following appear:

  • weight loss
  • coughing
  • night sweats
  • feeling unwell
  • general weakness

These are non-specific symptoms, occurring even in about 30 percent of patients, so when they appear, we are rarely focused on a specific disease, what's more - such ailments are known from autopsy to practically all of us and occur in the event of long-term stress or exhaustion.

Symptoms that are more typical of sarcoidosis are associated primarily with the affected organ. Therefore, we can observe, among others:

  • shortness of breath, cough, retrosternal chest pain or discomfort
  • muscle and joint pain
  • liver enlargement and / or spleen enlargement (hepato- and / or splenomegaly)
  • enlarged lymph nodes that are mobile and painless
  • in case of cardiac involvement arrhythmias, symptoms of circulatory failure
  • dry eyes or visual disturbances
  • disorders of the central nervous system, especially paralysis of the cranial nerves, mainly of the facial nerves (so-called neurosarcoidosis)
  • skin lesions of various types and severity, e.g. nodular sarcoid, angiolupoid (vascular lesion, mainly in women, mainly located on the nose), sarcoid, nodular, disseminated and annular; lesions usually leave scars on the surface of the skin
  • bone symptoms, also known as Jüngling's syndrome
  • enlargement of the parotid gland on one or both sides
  • symptoms of hypercalcemia, i.e. elevated levels of calcium in the blood that often accompany sarcoidosis, such as polyuria, nephrolithiasis and biliary stones, muscle weakness, sleepiness, headaches, nausea, vomiting, loss of appetite, pancreatitis, disease gastric and duodenal ulcers, arrhythmias or hypertension

Sarcoidosis - how to make an accurate diagnosis?

Since sarcoidosis can affect any organ, many abnormalities can often be observed in various additional tests. Ultimately, however, the diagnosis of sarcoidosis is determined by the histopathological examination of a sample of the changed organs, most often from the lymph nodes, lung parenchyma, conjunctiva of the eye, liver, kidneys or skin.

In addition, the following tests are used in the diagnosis of sarcoidosis:

  • lab tests that can show

- slight anemia - leukopenia - hypercalcemia - hypercalciuria - increased angiotensin converting enzyme activity in the blood - hyperergammaglobulinemia

  • imaging tests:

X-ray of the cagethoracic - based on the X-ray image, sarcoidosis can be classified in five stages, more precisely:

- stage 0 - the X-ray image of the chest is normal - stage I - enlargement of the lymph nodes of the cavities and / or mediastinum, no visible changes in the lung parenchyma - stage II - small nodular spreading in the lung parenchyma accompanying enlarged nodes lymphatic - stage III - small nodular spread in the parenchyma, no enlargement of the lymph nodes - stage IV - lung parenchyma fibrosis and emphysema changes ("honeycomb" image)

computed tomography of the chest:

- small nodular spread in the lung parenchyma - enlarged lymph nodes of the hilals and mediastinum

magnetic resonance imaging - most often helpful in assessing the involvement of other organs, mainly the central nervous system and the heart, ultrasound of the abdominal cavity to assess the liver, spleen and urinary system

  • EKG to assess possible arrhythmias
  • ophthalmological examination to be performed on each patient
  • examination of the cerebrospinal fluid - in the case of central nervous system involvement, it will reveal lymphocytosis and an increase in protein levels in the vast majority of patients
  • tuberculin test performed in order to exclude the current infection with mycobacterium tuberculosis, however, in the case of sarcoidosis, the immune system is weakened, therefore this test may give false negative results

Sarcoidosis can be diagnosed when there is a typical clinical and radiological picture in more than two organs, which is confirmed by biopsy results. When biopsy is not possible, the diagnosis of stage I and II sarcoidosis can only be made on the basis of the clinical and radiological picture.

How do I differentiate sarcoidosis?

Due to the fact that sarcoidosis can occur in every organ of our body, a number of diseases that may have similar symptoms should be ruled out.

In the case of lymphadenopathy of the hilals and mediastinum, it is first of all necessary to exclude neoplastic disease, both malignant lymphomas and metastases from other organs. Lung lesions in imaging tests may lead to the suspicion of other interstitial diseases or, for example, neoplastic spread.

Granulomas detected in the histopathological examination may also occur in such diseases as:

  • tuberculosis
  • tinea
  • granulomatosis with polyangiitis
  • Crohn's disease

and many other, slightly rarer diseases.

Difficulty may also be in the differentiation of skin lesions that appear in sarcoidosis, and which mayresemble changes in lupus, allergic diseases or tuberculosis.

Sarcoidosis - treatment

The basis in the treatment of sarcoidosis are glucocorticosteroids, and treatment with them should lastat least a year .

Due to the high percentage of spontaneous remissions, patients with stage I and II sarcoidosis are usuallynot treated . In these cases, it is only recommended to have the disease checked every few months.

In the form of pulmonary sarcoidosis, the use of inhaled glucocorticosteroids may also be considered.

The indication for treatment is disease stage III and IV and II, if progression of changes in the lung parenchyma or increasing respiratory disorders are observed, as well as the involvement of the heart, central nervous system, eyes and the presence of hypercalcemia by sarcoidosis.

In addition to glucocorticosteroids, other immunosuppressive drugs are also used, such as methotrexate, azathioprine, leflunomide, mycophenolate mofetil or anti-TNF α antibodies.

As a last resort, in case of advanced disease, it is necessary to considerlung transplantation .

Sarcoidosis - clinical course

As previously mentioned, sarcoidosis may be asymptomatic for a long time. Some people have an acute onset with fever, joint pain, inability to walk, erythema nodosum, and bilateral cavity lymphadenopathy. This form is then calledLöfgren's syndromeand despite the high intensity of symptoms at the beginning and their sudden onset, it is self-limiting and gives a good prognosis.

Sarcoidosis can also present asHeerfordt's syndrome , that is, a combination of anterior uveitis, parotid glands, facial nerve paralysis and fever.

In more than80%patients with stage I disease have remission up to two years after diagnosis.

Patients diagnosed with stage II disease come to remission in60%of cases, and in stage III patients in10-20%.

Sarcoidosis is generally associated with a good prognosis, and the mortality amounting toseveral percentis most often associated with respiratory and circulatory failure or a consequence of damage to the central nervous system.

  • BCG - tuberculosis vaccine
  • Plague - causes, symptoms, treatment

Help the development of the site, sharing the article with friends!

Category:

Immune system