Hystiocytosis X of the lungs. Causes, symptoms, treatment
Hystiocytosis X of the lung is a disease of unknown etiology, which is characterized by increased multiplication of histiocytes with the formation of granulomas.
Causes of histiocytosis X
The etiology of the disease is not fully understood. An important role is played by autoimmune mechanisms. Langerhans cells are regulatory cells of the macrophage system, which are antigen-presenting cells. With a change in their function, there is an accumulation of immune complexes and their deposition in the walls of the vessels. They activate the macrophage system. Histiocytes, or they are also called autoimmune pathological cells, multiply very actively, forming scar tissue. Especially the development of this process is characteristic of lungs and bones. Pulmonary granulomatosis X is a type of histiocytosis from Langerhans cells, with isolated organ damage( lungs, skin, bones, pituitary gland and nodes of the lymphatic system).It is characterized by the formation of peculiar granulomas and lesions of all organs and tissues.
There are two forms of histiocytosis X:
1. Acute form( Abta-Leggerer-Sieve disease).With this form of the disease, the following symptoms are observed:
- lungs increase in volume;
- appear cystic lesions up to one centimeter in diameter;
- on a microscopic examination shows a granuloma from histiocytes, eosinophils and plasma cells.
2. Primary chronic form( Hvda-Schuller-Krischen disease).This form is characterized by:
- many small nodules on the surface of the lungs;
- pleural overlap;
- swelling emphysematous, in the form of cysts;
- the lungs on the cut are in the form of honeycombs.
When irritation with mucous cigarette smoke occurs the formation of alveolar macrophages of cytokines and growth factors. Under their influence, there is an increase and multiplication of Langerhans cells.
Almost all patients have:
- unproductive cough,
- chest pain.
Approximately twenty percent of patients experience spontaneous pneumothorax. Approximately fifteen percent of the symptoms do not develop, and the disease is detected by radiography of the chest, when the patient is treated for a completely different reason.
Symptoms of histiocytosis X are usually scarce, and may be the cause of not necessarily this disease. The results of microscopic studies usually give a clear picture of the disease.
Acute disease or Abta-Letterter-Sieve disease , usually occurs in young children under three years of age and is accompanied by the following symptoms:
- dry, painful cough;
- shortness of breath.
This form is characterized by an accelerated progression of the pathological process with the appearance of signs of damage to the kidneys, bones, skin, the central nervous system, development of purulent otitis is not ruled out. If the treatment is not completed on time, a lethal outcome is possible.
Primary Chronic Form or Het-Schuller-Kristen Disease
This form of the disease usually affects young people up to 30 years of age. Patients turn to the clinic with complaints: shortness of breath, dry cough, general weakness. Some complain of acute pain in the chest, which indicates the development of spontaneous pneumothorax.
Due to granulomatous lesion of the bone system, the patient may have pain in the bones, mainly the pelvic bone, skull, ribs. The disease is accompanied by the destruction of the Turkish saddle due to damage to the hypothalamic-pituitary zone. There is a violation of the secretion of antidiuretic hormone with the advent of a clinic of diabetes insipidus, namely:
- pronounced dryness in the oral cavity;
- frequent profuse urination( urine density is low).
appearance of an acre of cyanosis, finger bones are thickened, become as "drumsticks", and nails as a "watch glasses." These symptoms are typical for patients who had long been suffering from this disease. Most can be seen xanthelasma( yellow spots) in the field of... the upper eyelids
if it affects the spinal column, you can see the curvature of his skull bones, spine, ribs, pelvis, painful percussion
In light percussion observed:
- clear lung sounds;
- box sound can be heard when razvTII emphysema;
- when a pneumothorax can hear the sound of tympanic
When histiocytosis X is often possible to observe weakening of vesicular breathing, less wheezing( dry), and crepitus
How to diagnose histiocytosis X pulmonary
The disease can be diagnosed on the basis of complaints of the patient, physical examination..?And radiography. With the help of an X-ray, you can easily see symmetrical infiltrates with cystic changes. The lesion is typical for the upper and middle divisions, while the lower parts of the lungs do not affect the disease. In the event that the radiography can not give complete information, then perform a bronchography and a biopsy. If the amount of CDIa cells in the bronchoalveolar fluid is more than 5 percent, then it says that the risk of the disease is quite high. During biopsy, the multiplication of Langerhans cells and the formation of a small number of clusters of eosinophils are observed.
On the roentgenogram, three stages of this disease can be seen:
- This stage is characteristic of the earliest stage. Two-sided small-focal darkening appear. The intramammary lymph nodes are not enlarged;
- For the second stage characterized by the development of interstitial fibrosis, fine meshed( fine mesh) pulmonary drawing;
- characterized by cystic bullous formation, the appearance of the painting "honeycombing" expressed fibro sclerotic manifestations. For the final verification of the diagnosis, an open lung biopsy is performed. A biopsy specimen reveals a granuloma, which consists of proliferating histiocytes.
main criteria diagnosed chronic form of histiocytosis X pulmonary consider:
- recurrent pneumothorax;
- pulmonary ventilation disorder;
- "honeycomb light";
- formation of granulomas.
Treatment of histiocytosis X of the lungs
The main component of successful treatment is the cessation of smoking. It is known that almost a third of patients, after they quit smoking, there was an improvement and they were on the mend. Glucocorticoid and cytotoxic agents are used in the treatment of the disease, although the effectiveness of these drugs has not been proven. Lung transplantation is effective in fighting the disease, but there is one downside - if the patient continues to smoke, the disease develops in the transplant. It is not excluded and the complete disappearance of symptoms in patients with the slightest manifestations of the disease. However, for many, the slow progression of the disease is characteristic, in this huge role is played by such factors as:
- duration of smoking;
- multiple organ dysfunction;
- symptoms of histiocytosis X are constant and indicate a progression of the disease.
- numerous cysts.
The cause of death is respiratory failure or cancer.