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Sarcoidosis: options for the course of a strange disease

Sarcoidosis was first described in the 19th century by D. Hutchinson, a practicing dermatologist surgeon in London. It was the cutaneous form of this disease that attracted the attention of doctors. Later, doctors began to note that not only the skin is affected by this previously unknown disease, but also the internal organs.

Sarcoidosis is a special disease:

  • The reasons for its development are still unknown today (there are only a number of theories and assumptions about multifactorism).
  • For a long time, it can swell in a latent form and show up at an advanced stage.
  • There are frequent cases of spontaneous recovery from sarcoidosis.
  • Symptoms depend on the localization process.
  • This disease is characterized by a large percentage of diagnostic and therapeutic medical errors due to the variety of clinical manifestations and the lack of clear treatment algorithms.  

Sarcoidosis is characterized by the presence of the following symptoms:  

  • The formation of granulomas in organs and tissues for unknown reasons.
  • The lungs, lymph nodes, eyes, skin, and liver are most often affected.
  • The disease is associated with a person’s ethnicity.  

Thus, the disease is considered to be multiorgan, therefore it can occur in the practice of a doctor of any profile.  

Symptoms of the disease

The clinical picture of this pathology depends on a number of factors:  

  • Ethnic background;
  • Localization of sarcoid lesions;
  • Duration of illness;
  • Prevalence process;
  • The activity of the formation of granulomas.          

Symptoms that occur regardless of the localization process (non-specific):  

  • Increased body temperature;
  • Fatigue;
  • Weight loss  

Since 90% of patients have pulmonary forms of sarcoidosis, symptoms of lung lesions are added to non-specific complaints:  

  • Dyspnea;
  • Chest pain;
  • Cough.

Lung sarcoidosis  

For a long time, sarcoidosis was considered a “problem” by TB specialists. Currently, the majority of cases with the disease lie on the shoulders of pulmonologists, as most often sarcoidosis affects the lung and hilar lymph nodes. The cause of death in this case is respiratory failure.  

Characteristic of the disease is the formation of granulomas, consisting of epithelioid cells, giant multinucleated cells (Pirogov-Langhans, cells of foreign bodies). Unlike tuberculous granulomas, there is no caseous necrosis zone in sarcoid granulomas.  

Another characteristic feature of granulomatous inflammation in sarcoidosis is progressive sclerosis (proliferation of connective tissue at the site of inflammation). The process captures lung tissue, bronchi, hilar lymph nodes. As the connective tissue grows, respiratory failure forms, which underlies the clinical manifestation of pulmonary sarcoidosis.  

Eye sarcoidosis

The defeat of the sarcoid granulomas of the organ of vision is more common in Japanese and African Americans. It may be asymptomatic. The most common lesion of the choroid is sarcoid uveitis.  

Skin sarcoidosis

An interesting fact is that erythema nodosum, as a manifestation of skin sarcoidosis, is more common in Europeans, while in dark-skinned people and in Japan it is never found.

Although skin sarcoidosis is found in only 10% of cases, it is with this form of the disease that the doctor is most often referred to, since this type of disease is visible to the naked eye.

The disease is characterized by the appearance on the skin of small or large nodules, as well as spots or plaques that do not cause pain, but spoil the aesthetic appearance, often located on the face, open parts of the body.  

Liver sarcoidosis

The liver is involved in the process in about 60% of cases. It is characterized by the formation of numerous sarcoid granulomas in the liver tissue, which may not clinically manifest. Rarely, liver sarcoidosis is accompanied by portal hypertension or bile stasis. They detect the disease by changes in the analyzes: liver sarcoidosis is characterized by a change in liver function tests, an increase in alkaline phosphatase activity.

Liver biopsy reveals the presence of granulomatous inflammation with no caseous necrosis.  

Heart sarcoidosis

Surprisingly, sarcoidosis with myocardial damage is more common in Japan. The cause of death in sarcoidosis in this country is more often lesions of the heart muscle.

Clinically sarcoidosis localized in   heart, characterized by the presence of arrhythmias, it can simulate myocardial infarction or myocarditis, sudden cardiac death can also develop.  

Defeat of the digestive system

Lesions of the sarcoidosis of the digestive system are rare, but in the literature there are data on the localization process in the salivary glands, occurring with a picture of inflammation in them – sialoadenitis.

There is also evidence of the existence of granulomatous gastritis and even ulcers. To confirm the diagnosis in such cases only microscopic examination of gastrobiopsy helps.  

Neurosarcoidosis

Foci of sarcoid inflammation may be located in the brain or its membranes. The process may have a diffuse character or simulate a tumor. Depending on the location, a clinical picture appears in the form of meningitis, encephalitis, meningoencephalitis.

Known cases of lesions of the pituitary, hypothalamus.  

Sarcoidosis in gynecology

Gynecologists also have to deal with sarcoidosis. Granulomas can be located in the genital organs, simulating various female diseases.  

The defeat of the female reproductive system of central origin

Violations of the female genital function of central genesis associated with the defeat of the granulomatous nature of the pituitary. This is one of the manifestations of neurosarcoidosis.

With such a localization of sarcoidosis, women go to a gynecologist with complaints about the appearance of milk from the mammary glands during the period not associated with lactation.

This phenomenon is called galactorrhea. This happens in connection with the production of an excess amount of the hormone responsible for lactation – prolactin. In women, hyperprolactinemia may be accompanied by a disorder of the menstrual cycle, infertility.

Galactorrhea can occur in men.

Also, with the defeat of the hypothalamus and pituitary by sarcoid granulomas, the production of other hormones – luteinizing, follicle-stimulating – may be disrupted. When they are deficient, there is a violation of follicle production. Before puberty in a girl, this is manifested by the absence of menstruation and the underdevelopment of secondary sexual characteristics.  

Uterine sarcoidosis

Sarcoid granulomas can be located in the uterus, diagnose the disease in this case when conducting examinations related to uterine bleeding. Histological examination revealed foci of granulomatous inflammation without necrosis.

Localization of foci may be different: in the neck   uterus, endometrium, myometrium, subserous layer, parametrii.  

More often detected in postmenopausal women. Uterine sarcoidosis can be combined with granulomatous lesions of other organs, and can occur as an independent disease.  

Breast sarcoidosis

The localization process in the mammary glands is clinically expressed in the appearance of dense nodules, which can be of different diameters. On palpation, they are painless. Often women with such formations are screened for the presence of a breast tumor – benign (fibroadenoma) or malignant (breast cancer).

Only a biopsy with subsequent histological examination allows a differential diagnosis between these diseases. Although there have been cases when breast sarcoidosis was detected simultaneously with a malignant tumor or fibromatous nodes.  

Pregnancy with sarcoidosis

Expressing the impact of the disease on the course of pregnancy and childbirth is clearly impossible. No such data. Some observations show that women with sarcoidosis are capable of bearing and giving birth to healthy children. There is evidence that some women had sarcoidosis in the early postpartum period, while others, on the contrary, gave birth to the onset of remission.  

However, a woman suffering from sarcoidosis and planning   pregnancy should take into account a number of factors that may adversely affect its health and the health of the child:

  • Pregnancy can cause exacerbation of the disease.
  • During pregnancy, it may be necessary to take medications that can adversely affect the fetus.
  • You need to be ready for delivery by cesarean section.

Sarcoidosis and the male reproductive system  

Long-term observations show that, if we consider the defeat of the male reproductive system, the descriptions of granulomatous inflammation in the testes and appendages are more common. This process is called testicular sarcoidosis.

At the same time, men groping in the scrotum are rounded, painless formations. The process can be both bilateral and one-sided. Sometimes testicular sarcoidosis can be combined with testicular cancer.  

The disease occurs with or without other manifestations of sarcoidosis. Testicular sarcoidosis does not have a negative effect on potency. Impotence in sarcoidosis can develop, but experts believe that the cause of it in this case is the defeat of the pituitary in neurosarcoidosis.

Thus, sarcoidosis is a multisystem disease, the clinical picture in which is less pronounced than pathological changes in the internal organs.

There is no uniform standard of treatment, since the causes of the disease are unknown (it is not clear what to treat). In addition, in some cases the disease passes on its own.

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