In the list of diseases that cannot be contracted from humans or animals and that occur for reasons not fully understood, Beck's disease is not the last. Sarcoidosis is its modern name. It is diagnosed quite rarely, in 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 to make it easier for doctors in any country to navigate the definition of an insidious disease, to jointly search for new methods of treatment and quickly find the right solution when a patient needs help.
Sarcoidosis - what is it?
Beck's sarcoidosis occurs when groups of cells capable of phagocytosis suddenly begin to divide and transform in various human organs. 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 condition.he alth. Granulomas can appear in any organ, including the heart, eyes, kidneys, liver, but are most often localized in the lungs. Deaths from sarcoidosis are rare and are recorded only in very debilitated patients with low immunity who have not been treated. In about 10% of patients, granulomas resolve on their own without the use of medications. Most people with sarcoidosis require specific treatment and consultation with a pulmonologist, cardiologist, ophthalmologist, neurologist, dermatologist, rheumatologist.
Discovery history
Beck's (Beck's) sarcoidosis was first described by British 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 physician Besnier described the course of the disease in a patient who had similar granulomas in the nose. In addition, this patient had grayish-blue swelling of the ears and fingers. Independently of Besnier, the Norwegian physician Caesar Böck performed a histological examination of these granulomas and gave them the name "benign skin sarcoidosis". He also noticed that purple nodules can appear on the mucous membranes and in the lungs, and the Swedish doctor Schaumann tried to systematize data on the various manifestations of sarcoidosis. As a result, the disease was called "Besnier-Boeck-Schaumann disease". This term can still be found in some medical documents.
International classification
In the ICD-10 system Beck's sarcoidosisclassified as a third class disease. This means that a violation of immunity is involved in its etiology. According to the international catalog, this disease is assigned the code D 86. Sarcoidosis of a specific organ affected by granulomas has the following numbering:
- in lungs - D86.0;
- in lymph nodes - D86.1;
- simultaneously in the lungs and in the lymph nodes - D86.2;
- on the skin - D86.3;
- unspecified pathogenesis - D86.9.
If other diseases are diagnosed in 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 by the nature of the flow
Beck's 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 extremities.
3. Subacute. There is a wave-like increase in temperature, the general condition is moderate.
There is also a refractory form (cannot be treated).
Classification by severity
The described disease is differentiated into three degrees of severity:
First. Patients have enlarged thoracic lymph nodes(bronchopulmonary, tracheobronchial, paratracheal, bifurcation).
Second. Beck's sarcoidosis grade 2 is characterized by the fact that inflammatory interstitial foci are found in the lungs.
Third. Fibrosis (pneumosclerosis) of the lung tissues appears, while the intrathoracic nodes do not increase, but emphysema forms. Together with foci of fibrosis, they form extensive confluent conglomerates. Patients complain of chest pains, poor appetite, severe fatigue, lethargy, dry cough, shortness of breath, joint pain.
There is a classification in which there are five stages of sarcoidosis:
- Zero. The disease has begun, but the X-ray of the lungs does not show anything.
- First. Intrasternal lymph nodes begin to increase.
- Second. Lymph nodes are enlarged, granulomas begin to appear in the lung tissue.
- Third. Changes occur in the lung tissue.
- Fourth. Pulmonary fibrosis.
Etiology
Beck's sarcoidosis ICD 10th revision refers to diseases associated with impaired immunity, since as a result of multiple studies, the role of HLA (human leukocyte antigens) in the appearance of granulomas has been revealed. So, loci have been found that protect against sarcoidosis or, conversely, provoke it, causing 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 that's allknow exactly about the etiology of the disease. There is no definitive answer to the question of what affects the development of the disease. Scientists suggest that risk factors may be:
- infectious or fungal infections;
- plant pollen;
- harmful chemical gases and vapors;
- poor nutrition;
- bad environment.
Epidemiology
If the exact causes of Beck's sarcoidosis have not yet been established, the epidemiology of the disease is well known. So, it has been proven that this disease affects people of any age, including small children, but more often it is observed in patients aged 20-40 years, and women are more susceptible to it. There is also some difference in terms of race. Sarcoidosis is extremely rare in the Middle East and Japan, and in India it is diagnosed in 150 people out of 100,000. In the northern part of Europe, 40 people out of 100,000 get sick, in the southern part the rates are slightly higher. In Australia, Beck's disease is detected in 92 people per 100,000, in the United States among African Americans the rate is 40-64 cases, and among people with fair skin, only 10-14 people out of 100,000 get sick.
Surprisingly, smokers get sarcoidosis less often than non-smokers.
Symptomatics
Sarcoidosis in its early stages is usually asymptomatic. Usually people do not even suspect that they have this disease. Most clearly, the signs are observed already with a disease of the 3rd degree, when it comes like thiscalled pulmonary-mediastinal form of Beck's sarcoidosis. However, most patients present with the following symptoms:
- loss of appetite;
- apathy, lethargy;
- fatigue (noted from the very moment of awakening);
- temperature;
- muscle pain;
- body weight loss;
- cough that is not treated with antitussives.
According to such complaints, the diagnosis of "cold" or "ARI" is often made, but as sarcoidosis progresses, the cough becomes prolonged, hemoptysis appears, and granulomas become visible on the skin. In the future, without treatment, the eyes, liver, heart, and other organs can be affected. Symptoms for each form has characteristic features. So, with sarcoidosis type D86.8 + H22.1vision deteriorates, eyelids become inflamed, lacrimation appears. With type D86.8 + I41, 8signs of heart failure, shortness of breath, arrhythmia appear. With type D86.3, erythema nodosum appears on the skin. They may look like a rash. The face, forearms, shins are affected.
Diagnosis
Beck's sarcoidosis has symptoms similar to those of other diseases. In order to differentiate it correctly, the patient needs an examination and consultation of many narrow-profile doctors and a series of tests to rule out:
- tuberculosis;
- beryllium (appears upon contact with beryllium);
- rheumatism;
- lymphoma (malignant neoplasms in the lymph nodes);
- allergic reactions to anything;
- fungal infections.
The patient is being tested:
- blood (general and biochemical);
- urine (general);
- ECG;
- bronchoscopy;
- study of bronchial lavage;
- TB tests;
- x-ray (it can be performed in conjunction with CT of the respiratory system), multislice CT gives especially good results, and MRI is prescribed to detect granulomatous changes in the heart;
- taroscopy (used in particularly difficult cases).
Ultrasound for Beck's sarcoidosis is performed by transesophageal, which gives excellent results when examining intrathoracic lymph nodes. At the same time, a biopsy is performed.
Another type of examination is gallium scanning. This metal tends to accumulate in the foci of inflammation. 2 days after intravenous administration of the substance, the patient is scanned. The disadvantage of the method is that gallium can 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. Once a patient has scars in their lungs, they cannot be removed.
When all tests confirm the diagnosis of sarcoidosis, the doctor prescribes glucocorticosteroids. The main drug is Prednisolone. The course of treatment is long, up to 8 months. This may cause side effects:
- edema;
- weight gain;
- stomach pain;
- mood swings;
- hypertension;
- acne.
When taking the drug, a positive effect is observed very quickly, but after stopping therapy, the signs of the disease may return.
Pentoxifylline, Methotrexate, Chloroquine are prescribed in the complex.
Given the fact that granulomas can disappear on their own, patients who do not cause any discomfort or pain from sarcoidosis are not prescribed treatment, but are regularly monitored for their state of he alth.
Forecast
If Beck's sarcoidosis is diagnosed based on CT scans, lung x-rays, biopsy tests, there can be no mistake, 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 this disease give birth to he althy children without any problems.
Complications occur only in that part of patients who were not treated in a timely manner. They may experience:
- respiratory failure;
- significant visual impairment, up to blindness;
- exacerbation of diseases of internal organs.
Prevention of sarcoidosis has not been developed due to the ambiguity of its etiology. Doctors give only general recommendations:
- observe the correct daily routine;
- eat rationally;
- do not abuse alcohol;
- avoid contact with harmful chemicals, especially those with high volatility, as well as gas anddust.
