In the list of diseases that cannot be infected from humans or animals and which arise for reasons not fully understood, Beck's disease is not the last. Sarcoidosis is its modern name. It is rarely diagnosed, no more than 150 out of 100,000 people, but it affects people on all continents, and therefore it has been assigned an international code in the ICD-10 classification system. This is necessary so that it is easier for doctors in any country to navigate with the definition of an insidious disease, by joint efforts to look for new methods of treatment and quickly find the right solution when the patient needs help.
What is sarcoidosis?
Beck sarcoidosis occurs when groups of cells capable of phagocytosis suddenly begin to divide and transform in various organs of a person. As a result of this process, nodules (granulomas) are formed, which may not manifest themselves in any way or cause a significant deterioration in the state of health. Granulomas can appear in any organ, including the heart, eyes, kidneys, liver, but are most often localized in the lungs. Fatal cases with sarcoidosis are rare and are recorded only in very weakened patients with low immunity who have not been treated. In approximately 10% of patients, granulomas resolve on their own without the use of medication. Most people with sarcoidosis require specific treatment and consultation with a pulmonologist, cardiologist, ophthalmologist, neurologist, dermatologist, rheumatologist.
Discovery story
The first described sarcoidosis Beck (Beck), a British surgeon-dermatologist D. Hutchinson. In 1877, he observed two patients: a 53-year-old man and a 64-year-old woman who had purple granulomas on the skin of their legs and arms. After 12 years, the French doctor Benier described the course of the disease in a patient who had similar granulomas in the nose. In addition, grayish-blue tumors of the ears and fingers were observed in this patient. Regardless of Bénier, the Norwegian doctor Caesar Böck performed a histological examination of such granulomas and gave them the name "benign sarcoidosis of the skin." He also noted that purple nodules are able to appear on the mucous membranes and in the lungs, and the Swedish doctor Schaumann tried to systematize data on various manifestations of sarcoidosis. As a result, the disease was called "Benje-Böck-Schaumann disease." This term can now be found in some medical documents.
International classification
In the ICD-10 system, Beck sarcoidosis is classified as a disease of the third class. This means that immunity is involved in its etiology. According to the international catalog, this ailment is assigned the code D 86. Sarcoidosis of a specific organ affected by granulomas has the following numbering:
- in the lungs - D86.0;
- in the lymph nodes - D86.1;
- simultaneously in the lungs and lymph nodes - D86.2;
- on the skin - D86.3;
- unspecified pathogenesis - D86.9.
If other diseases are diagnosed with sarcoidosis, the numbering is as follows:
- accompanied by iridocyclitis or anterior uveitis - D86.8 + H22.1 *;
- with cranial nerve palsy - D86.8 + G53.2 *;
- with arthropathy - D86.8 + M14.8 *;
- with myocarditis - D86.8 + I41.8 *;
- with myositis - D86.8 + M63.3 *.
Classification according to the nature of the course
Beck sarcoidosis can occur in three forms:
1. Chronic. Patients have a general deterioration in well-being, unreasonable weakness, decreased ability to work.
2. Acute. This form is characterized by a sharp jump in temperature, an increase in lymph nodes, swelling of the joints of the limbs.
3. Subacute. There is a wave-like increase in temperature, the general condition is moderate.
A refractory form is also isolated (cannot be treated).
Severity classification
The described disease is differentiated by three degrees of severity:
First one. Patients have enlarged thoracic lymph nodes (bronchopulmonary, tracheobronchial, paratracheal, bifurcation).
The second one. Beck sarcoidosis of the 2nd degree is characterized by the fact that inflammatory interstitial foci are found in the lungs.
The third. Fibrosis (pneumosclerosis) of the lung tissue appears, while the intrathoracic nodes do not increase, but emphysema is formed. They, together with foci of fibrosis, form extensive confluent conglomerates. Patients complain of chest pain, poor appetite, severe fatigue, lethargy, dry cough, shortness of breath, joint pain.
There is a classification in which five stages of sarcoidosis are distinguished:
- Zero The disease has begun, but an X-ray of the lungs shows nothing.
- First one. Intrathoracic lymph nodes begin to increase.
- The second one. Lymph nodes are enlarged, granulomas begin to appear in the lung tissue.
- The third. Changes occur in lung tissue.
- Fourth. Fibrosis of the lungs.
Etiology
Beck's sarcoidosis of the ICD of the 10th revision refers to diseases associated with impaired immunity, as a result of multiple studies revealed the role of HLA (human leukocyte antigens) in the appearance of granulomas. So, loci were found that protect against sarcoidosis or, conversely, provoke it, causing manifestation of damage to the brain, eyes and other organs.
It is unequivocally established that sarcoidosis is not contagious. The fact that this disease occurs among members of the same family does not exclude its hereditary transmission.
Perhaps this is all that is absolutely known about the etiology of the disease. There is no final answer to the question of what affects the development of the disease. Scientists suggest that risk factors may include:
- infectious or fungal infections;
- plant pollen;
- harmful chemical gases and vapors;
- poor nutrition;
- bad ecology.
Epidemiology
If the causes of Beck's sarcoidosis have not yet been precisely established, then the epidemiology of the disease is well known. So, it is proved that this ailment affects people of any age, including young children, but more often it is observed in patients aged 20-40 years, and women are more susceptible to it. With regard to race, there is also a slight difference. People from the Middle East and Japan are extremely rarely affected by sarcoidosis, and in India, 150 out of 100,000 people are diagnosed with it. In the northern part of Europe, 40 out of 100,000 get sick, in the southern part the indicators are slightly higher. In Australia, Beck's disease is detected in 92 people per 100,000, in the United States among African Americans, the figure is 40-64 cases, and among people with fair skin, only 10-14 people out of 100,000 people get sick.
Surprisingly, smokers are less likely to have sarcoidosis than those who do not have this bad habit.
Symptomatology
Sarcoidosis in the initial stages most often proceeds without symptoms. Usually people do not even suspect that they have this ailment. The signs are most pronounced already in grade 3 disease, when the so-called pulmonary-mediastinal form of Beck sarcoidosis occurs. In this case, most patients have the following symptoms:
- loss of appetite;
- apathy, lethargy;
- fatigue (noted from the moment of awakening);
- temperature;
- muscle pain
- body weight loss;
- cough that is not treated with antitussive drugs.
Such complaints are often diagnosed with a cold or ARI, but as sarcoidosis progresses, the cough becomes lingering, hemoptysis appears, and granulomas become visible on the skin. In the future, without treatment, eyes, liver, heart, and other organs may be affected. Symptoms in each form have characteristic features. So, with sarcoidosis of the type D86.8 + H22.1 *, vision deteriorates, the eyelids become inflamed, and lacrimation appears. With type D86.8 + I41.8 * there are signs of heart failure, shortness of breath, arrhythmia. With type D86.3, erythema nodosum appears on the skin. They may resemble a rash. The face, forearms, legs are affected.
Diagnostics
Beck sarcoidosis has symptoms similar to manifestations of other diseases. To differentiate it correctly, the patient needs examination and consultation of many narrow-profile doctors and a number of tests to exclude:
- tuberculosis;
- berylliosis (appears when in contact with beryllium);
- rheumatism;
- lymphoma (malignant neoplasms in the lymph nodes);
- allergic reactions to anything;
- fungal infections.
The patient is tested:
- blood (general and biochemical);
- urine (total);
- ECG;
- bronchoscopy;
- research of washing bronchial waters;
- tuberculosis tests;
- X-ray (it can be carried out together with CT of the respiratory system organs), multislice CT scan gives especially good results, and MRI is prescribed to detect granulomatous changes in the heart;
- taroscopy (used in especially difficult cases).
Ultrasound with Beck sarcoidosis is performed transesophageal, giving excellent results when examining intrathoracic lymph nodes. At the same time, a biopsy is performed.
Another type of examination is scanning with gallium. This metal tends to accumulate in the foci of inflammation. 2 days after intravenous administration, the patient’s substances are scanned. The disadvantage of this method is that gallium is able to accumulate in any inflammatory foci, regardless of whether they are caused by sarcoidosis or another disease.
Beck sarcoidosis treatment
The goal of therapy for this disease is to preserve the functions of all affected organs. If a patient has scars in the lungs, it is no longer possible to get rid of them.
When the diagnosis of sarcoidosis is confirmed as a result of all tests, the doctor prescribes glucocorticosteroids. The main drug is Prednisolone. The course of treatment is long, up to 8 months. In this case, side effects may be observed:
- swelling
- weight gain;
- stomach pains;
- mood swings;
- hypertension;
- acne.
When taking the drug, a positive effect is observed very quickly, but after stopping therapy, the symptoms of the disease may return.
Pentoxifylline, Methotrexate, and Chloroquine are prescribed in the complex.
Given the fact that granulomas can disappear on their own, patients for whom sarcoidosis does not cause any discomfort or pain are not prescribed treatment, but they regularly monitor their health status.
Forecast
If Beck’s sarcoidosis is diagnosed by CT, lung X-ray, and biopsy tests, there can be no error, but there is no need to despair. This disease with proper treatment does not reduce the standard of living, does not affect the ability to work, and women with such an ailment give birth to healthy children without problems.
Complications arise only in that part of patients who did not receive treatment in a timely manner. They may have:
- respiratory failure;
- significant visual impairment, up to blindness;
- aggravation of diseases of internal organs.
Prevention of sarcoidosis is not developed due to the uncertainty of its etiology. Doctors give only general recommendations:
- observe the correct regimen of the day;
- eat rationally;
- do not abuse alcohol;
- Avoid contact with harmful chemicals, especially those with high volatility, as well as gas and dust.