Sarcoidosis, symptoms and treatment

March 27, 2018 20:37 | Symptoms And Treatment
Sarcoidosis is not a hematological disease in the exact sense of the word. Most often occurs in puberty, although isolated cases have been described in infants. Sarcoidosis is associated with the hematological clinic because in 80% of cases it affects the lymphatic apparatus, which requires differentiating it from other lymphadenopathies, which at this age are very common.

Symptoms of sarcoidosis .The disease is long, has a benign character and a diverse clinical picture, depending on the localization of the process. Until recently, this syndrome was considered as a skin disease. Now it is known that the disease can develop in any organ, even if it appears macroscopically intact. With the current state of science, the syndrome is considered as a common disease of RES.From the histological point of view, sarcoidosis is characterized by the emergence of a specific granulation, which resembles tuberculosis. There are tuberculosis, which includes epithelioid cells and often giant cells such as Langhans'a. Unlike tuberculosis, there is no decay and cheesy degeneration.

The most easily recognizable manifestations of this syndrome are skin changes. They manifest themselves in the form of sarcoidosis benigna multiplex or as granulomatous changes in the subcutaneous tissue, sometimes in the mucous membranes. This type of sarcoidosis, however, is very rare, and in most cases changes occur in the internal organs, most often in the lymph nodes. First of all, cervical, ulnar, inguinal nodes are affected, and, more rarely, mediastinal nodes.

The pathological process can affect both the lungs. In the pulmonary phase of the disease, there are 3 periods: defeat of mediastinal nodes, myeliation and fibrotic changes. Depending on the prevalence of the process in the lungs, respiratory and circulatory functions are impaired. Pulmonary changes occur generally slowly, and they have a plastic character, do not reveal a tendency to decay and cheesy regeneration. Sometimes the process is accidentally detected during X-ray examination, and in far-reaching cases there are widespread hylus shadows going symmetrically.

Radiological picture is very heterogeneous and deciphering it is not always easy. Occasionally, generalized tubercles with specific granulations are found throughout the body. They can even be found in the skeleton, forming the so-called ostitis tuberculosa multiplex cystica. In the X-ray picture there is then sharply delimited, spherical osteoporosis of bone tissue;sometimes the process takes the form of a generalized seeding of bones, concentrating mainly on the bones of the hands and feet. Granulomatous tubercles can also be found in the myocardium, in arterial walls, in the nervous system and other organs. Sometimes we get a picture of a generalized billion tuberculosis. Due to different generalizations, diverse clinical patterns can arise. It should be emphasized, however, that benign course, slow development and frequent absence of temperature reaction and weight loss lead to the fact that parents and the surrounding sick child do not notice the development of the disease.

The frequent symptoms of sarcoidosis include Heefordt's syndrome, consisting of subacute inflammation of the parotid gland, the choroid of the eye and sometimes in the defeat of the cranial nerves. In other cases, it comes down to severe eye disorders and can sometimes lead to atrophy of the eyeball. In most cases, the pathological process affects the liver and spleen. When histological examination( biopsy) very rarely find specific changes in the bone marrow.

The picture of peripheral blood is uncharacteristic. In most cases, anemia is not found. The number of white blood cells is normal or slightly increased. Even frequent hematological studies do not reveal any changes;sometimes there is a slight monocytosis and eosinophilia.

The etiology of sarcoidosis is unknown. The similarity of histological and clinical patterns and the transition of sarcoidosis to tuberculosis suggests that the etiology of both diseases is identical. Some authors argue that sarcoidosis is a modification of tuberculosis, differing from the latter by a special reaction of a sick organism to tuberculosis infection.

Diagnosis of .The histological examination of the removed lymph node helps to diagnose this disease. The study establishes the presence of delimited tubercles consisting of epithelial cells. Unlike tuberculosis, there is neither decay nor curdling degeneration. There may, however, be separate necrotic foci. Sometimes there is a lymphocyte infiltrate, in a fresh change can detect a large number of eosinophilic granulocytes. Always there are giant cells Langhans'a however the number of them is small. Cytological examination of the site does not make any significant difference. It, however, helps differentiate this disease from other diseases.

In some cases, bronchoscopy should be performed, which can give material for pathohistological examination, if, of course, a pathological process in the bronchi is detected. The spleen often increases slightly. Only in exceptional cases it comes to splenomegaly and then a picture of the disease is joined by a number of circulatory disorders in the form of anemia, granulocytopenia, thrombopenia, pancytopenia. In the case of enlarged spleen and inaccessibility of the lymph nodes, as well as the absence of other data confirming sarcoidosis, the diagnosis can sometimes be established based on tissue biopsy taken from the area of ​​the anterolateral muscle. These studies are conducted in cases where a biopsy of the spleen has not given definitive results or when there are contraindications for puncture of this organ.

In differential diagnosis, tuberculosis of nodes or granulomas should be excluded first. Long-term follow-up helps differentiate these diseases. Certain stages of the disease may resemble other syndromes occurring in children with lymph node involvement - some leukemias or less frequent diseases - cat scratch disease, tularemia, brucellosis and leprosy not found in our geographical latitudes.

Treatment of is anti-tuberculosis. You can use cytostatic agents( derivatives of nitrogen mustard gas), as well as hormonal treatment( ACTH, cortisone, prednisone).

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