There are many pathologies that are associated with damage to the lung tissue and lead to respiratory failure. One of these diseases is histiocytosis X. Unlike diseases such as tuberculosis, pneumonia or lung cancer, this pathology is quite rare. Nevertheless, histiocytosis should be detected in a timely manner in order to select the correct treatment. This disease is more common among children.
The term "Histiocytosis X" combines several pathological conditions. Some diseases of this group are characterized not only by damage to the lung tissue, but also to other organs. Despite the fact that these pathologies are not related to oncological processes, their treatment includes cytostatic and radiation therapy.
Histiocytosis X - what is it?
Normally, every person has immune cells. They are represented by histiocytes, which are found in the tissues of the body. Under the influence of unknown factors, these cells begin to divide intensively, formingidentical clones. This leads to the formation of granulomas. They are the cause of the development of all the symptoms of pathology. All diseases included in the group of histiocytosis have a similar mechanism of development. However, each ailment has its own characteristics of pathogenesis.
According to statistics, histiocytosis develops in 5 out of 1 million people. The incidence of the disease in women and men is the same. The main reason for the formation of granulomas is the abuse of tobacco products. It is known that in 85% of cases, limited histiocytosis develops, in which predominantly lung tissue is affected. Symptoms of this form of pathology are similar to manifestations of other diseases of the respiratory system. Among them: chest pain, shortness of breath, sputum production and coughing.
It is almost impossible to detect pathology on the basis of clinical data, since the symptoms of the disease are nonspecific. To make a correct diagnosis, morphological confirmation is required - a biopsy of lung tissue. Treatment of histiocytosis X is based on hormonal and chemotherapy. If the pathological process is limited to lung damage, the prognosis may be favorable. However, this depends on the degree of tissue damage and adequate treatment.
Causes of pathology
What causes histiocytosis X? The causes of this disease have been studied for a long time. However, the exact factor causing this group of diseases has not yet been established. Possible causes include:
- Heredity.
- Smoking.
- Viruses.
In favor of the genetic theory of the occurrence of the pathological process is the fact that histiocytosis is diagnosed only in certain population groups, namely, in Caucasians. In addition, the likelihood of getting sick increases with aggravated heredity. It is also believed that the onset of the disease can be influenced by a chronic viral infection, which is involved in cell transformation. Specifically, herpes.
Smoking is the main trigger. Almost all patients diagnosed with histiocytosis abuse tobacco products. Despite the fact that the disease is more often detected in children, smoking is still considered the underlying cause. Toddlers who inhale cigarette smoke get sick more often than patients whose parents do not abuse tobacco products.
The mechanism of development of the disease
To study the pathogenesis of the disease, you need to know what a granuloma is. This pathological formation is a cluster of cells. In other words, a granuloma is a nodule in the normal tissue of an organ that interferes with functioning. Such formations can be represented by various types of cellular elements. The morphological substrate of the disease is histiocytic granuloma. What is this education and where does it come from?
Granulomas appear as a result of autoimmune aggression. This means that some kind of failure occurs in the body, and the protective system begins to fight the harmful factor. However, "breakdown" affects the immune system, andcells begin to divide rapidly. This aggravates the situation. Instead of eliminating the cause of the disease, histiocytes accumulate in granulomas and damage tissues.
Classification of the pathological condition
The most common type of proliferation of elements of the immune system is Langerhans cell histiocytosis. This term refers to a group of diseases that have a common pathogenesis. It includes three ailments that affect various organs. The classification is based not only on the distribution of granulomas, but also on the clinical course of the pathological process, as well as the prognosis.
The most formidable disease from the group of histiocytosis is Abt-Letterer-Siwe disease. It is mainly diagnosed in young children. Pathology is characterized by systemic damage to tissues rich in immune cells.
Hand-Schuller-Christian disease is almost the same as the previous disease. The difference is that the manifestation of clinical manifestations is observed later. The age of patients ranges from 7 to 35 years. The disease proceeds favorably.
The third pathology from the group of histiocytosis is eosinophilic granuloma. Compared to other diseases, this disease mainly affects the lungs. Damage to other tissues develops in 1/5 of patients. Signs of the disease usually appear by adolescence.
Features of pathology in babies
To a greater extent, the disease is attributed to the problems of pediatrics, since histiocytosis X in children develops more often. Averagethe age of the patients is 3 years. Hand-Schuller-Christian syndrome is characterized by a later onset and slow progression. In children with generalized forms of histiocytosis X, skin changes come to the fore. Babies from the first months of life suffer from eczema and seborrheic dermatitis. They are also characterized by damage to the mucous membranes - gum hypertrophy, chronic stomatitis.
Dermatological manifestations of the disease are difficult to treat and often recur. Skin changes begin with the trunk and scalp. The rash can be varied, more often - it is polymorphic and strip-like. Papular elements are often covered with a crust, which then turns into an ulcerative surface.
Unlike adults, lung damage in children occurs later. In malignant histiocytosis (Abt-Letterer-Siwe disease), it causes rapidly developing respiratory failure and cardiovascular complications. Also, a feature of the disease is the defeat of bone tissue and the violation of its formation.
Symptoms and clinical forms of pathology
Abt-Letterer-Siwe disease refers to childhood diseases and almost never occurs in the adult population. This form is the most malignant, as it often leads to death within 1 year or less. Histiocytosis X in adults implies two types of pathology. These are eosinophilic granuloma and Hand-Schuller-Christian syndrome. In adults, these diseases appear at a young age.
Eosinophilic granuloma is characterized by the fact that Langerhans cells (cells related to histocytes) are concentrated in one of the target organs. Most often it is the lungs, bones, spleen or liver. A synonym for eosinophilic granuloma is Taratynov's disease. Damage to the lung tissue is manifested by a constant dry cough and shortness of breath. Periodically noted intoxication, which is expressed slightly. According to the clinical picture, the disease resembles chronic pulmonary tuberculosis. When bones are affected, patients complain of pain in the limbs when moving, sometimes swelling is noted.
Hand-Schuller-Christian pathology is characterized by systemic damage to target organs. It is similar to Abt-Letterer-Siwe disease, but the symptoms develop more slowly and not simultaneously. The first signs of the disease are nonspecific. These include general weakness, decreased appetite and moderate symptoms of intoxication. Then, on thin areas of the skin (groin folds, armpits), areas of peeling appear. The progression of dermatological symptoms leads to the spread of the rash and the addition of infection.
Patients suffer from chronic gingivitis and stomatitis. Due to the violation of the bone structure and mucous membranes, destruction of the teeth occurs. The terminal stage of the disease is characterized by complex symptoms, which include exophthalmos (bulging eyes), pathological fractures, and diabetes insipidus. Damage to the lungs leads to constant shortness of breath due to the formation of bullae and atelectasis. Complications of the disease is spontaneous pneumothorax (accumulationair in the chest) and neurological disorders.
Diagnostic criteria for disease
When symptoms of a respiratory system disease appear, X-rays and computed tomography of the chest are performed. The same studies are done for bone imaging. At the same time, small areas of compaction (granulomas), bullae and atelectasis are found in the lungs. In the bones, cystic formations and tissue destruction are visualized. Histological or cytological examination is required to confirm the diagnosis. For this purpose, bronchoscopy with biopsy is performed.
Performing differential diagnosis
Diseases with which histiocytosis from Langerhans cells is differentiated include: pulmonary tuberculosis, cancer, pneumosclerosis, COPD. If other tissues are involved in the pathological process, examinations such as skin scraping for infections and fungi, bone biopsy, abdominal ultrasound are performed. This helps to distinguish histiocytosis from diseases of the digestive tract, oncological and dermatological ailments. However, instrumental research methods without morphological verification do not allow establishing a diagnosis.
Methods of treating the disease
Corticosteroids are used to treat each of the forms of this disease. The list of drugs to be taken can only be approved by a doctor. In case of lung damage, a pulmonologist is engaged in treatment. It includes hormone therapy and the use of cytostatics. Bone destruction requires surgical treatment. Some patients are indicated for radiationtherapy, as well as lung or bone marrow transplantation.
Groups of drugs for the treatment of disease
The main treatment for histiocytosis is drug therapy. The following groups of medicines are distinguished:
- Cytostatic agents. These include chemotherapy drugs used to inhibit the growth of abnormal histiocytes. Examples are Azathioprine, Methotrexate, and Vinblastine.
- Systemic and topical corticosteroids. The list of drugs includes the drugs "Prednisolone" and "Hydrocortisone".
- Folic acid antagonists. The representative of this pharmacological group is the drug "Leucovorin".
- Immunomodulators. The drug "Alpha-interferon".
Hormonotherapy should be carried out continuously even with minimal disease activity. If the patient has signs of exacerbation, cytostatics and immunomodulating agents are prescribed.
Prevention measures and prognosis
The main way to prevent histiocytosis is the complete rejection of tobacco products. Even passive smoking can provoke an exacerbation and worsen the prognosis for life. If you change your lifestyle and follow the doctor's instructions, you can achieve a stable remission with a limited form of histiocytosis. In some cases, the eosinophilic granuloma completely regresses. Systemic histiocytosis is more difficult to treat. The five-year survival rate for Hand-Schuller-Christian pathology is 75%. If untreated, the disease progresses and takes an acuteshape. Continued smoking can lead to malignant transformation of histiocytes.
