Scleroma is a chronic disease of an infectious nature, which is characterized by the development of inflammation in the wall of the respiratory tract with the formation of granulomas. The pathological process progresses slowly and gradually affects the entire respiratory system, making the patient bedridden.
The disease affects mainly adults and children in adolescence; neonatal scleroma is extremely rare.
Etiology and pathogenesis of scleroma
The causative agent is the Frisch-Volkovich stick. This microorganism is quite rare in the environment. The source of scleroma is a sick person, but experts have not yet been able to thoroughly study the pathways of infection and the mechanisms of transmission of the pathogen. Many researchers believe that the disease is characterized by contact transmission. This theory is supported by cases of the development of the disease in family members of the patient. But there were also recorded cases when people remained healthy after contact with a sick person.
The pathogen enters a person in an encapsulated form, which causes a long incubation period. The capsule protects the bacillus and leads to difficulty in the processes of phagocytosis, the appearance of Mikulich cells, which are characterized by a large size and altered protoplasm. After some time, an active period begins - the development of inflammatory changes and the formation of granulomas. The latter can develop in 2 ways:
- Exophytic. Overlap of the airway lumen occurs, which causes respiratory distress.
- Endophytic. It usually develops in the nasal cavity. It leads to deformation of the skin of the nose and the development of granulomas on it.
Over time, deformation of the granulomas occurs, which consists in their scarring. As a result, airway stenosis occurs at the site of inflammatory elements. A distinctive feature of the disease is scarring of granulomas without ulceration and decay, as well as the absence of changes in bone structures.
Scleroma classification
At the moment, there are several ways to classify the disease:
By the stage of development of the process:
- Initial.
- Active.
- Regressive or cicatricial.
By clinical manifestations and extent of the disease:
- Hidden scleroma. This is the most common form of the disease, which is characterized by swelling and redness of the nasopharyngeal mucosa, the appearance of viscous discharge from the nose. Subsequently, the thinning of the mucous membrane occurs under the influence of the inflammatory process and its replacement with connective tissue (a scar appears).
- Atrophic form. The disease is characterized by the formation of crusts on the mucous membrane associated with atrophic processes. When localized in the nose, a specific smell arises from it, a person loses his sense of smell. With atrophic phenomena in the airways, their strong narrowing occurs.
- Infiltrative form. This disease is characterized by the formation of nodules located in the nasopharynx, palatine arches, on the larynx. Unlike other forms of scleroma development, Frisch-Volkovich sticks will be determined in the inoculation.
- Cicatricial form. It differs in the replacement of granulomas with scars, shortness of breath, possible fusion of the nasopharyngeal lumen, the development of stenosis of the larynx, concentric narrowing of the trachea.
- Mixed form. The disease can have signs of the above forms at the same time, for example, the presence of infiltrates and scars.
- Atypical form. The disease is characterized by the appearance of a kind of diaphragm, which will contribute to the narrowing of the larynx. In this case, granulomas develop on the epiglottis.
At the place of development of the disease:
- Laryngeal scleroma.
- Scleroma of the nose.
- Bronchial scleroma.
- Pharyngeal scleroma.
- Tracheal scleroma.
- Scleroma of other organs. Cases of the development of the disease in other organs were recorded, for example, in the middle ear, conjunctiva of the eye.
Scleroma of the nose
It manifests itself as banal rhinitis against the background of increased dryness of the mucous membrane. Patients note a decrease in the sense of smell, the appearance of crusts with a sweet smell.
Laryngeal scleroma
If inflammation develops in the larynx, then the patient's voice becomes hoarse, with a history of stenosis-like breathing disorder.
Pharyngeal scleroma
The disease leads to discomfort in the throat and difficulty swallowing.
Tracheal scleroma
With tracheal scleroma, the patient complains of thick sputum, painful cough, shortness of breath.
Bronchial scleroma
The clinical picture is identical to the defeat of the larynx: infiltrates, narrowing of the lumen of the bronchi can be determined. Sometimes the changes look like granulation.
Scleroma stages
The disease can develop over several years and decades. Experts identify the following stages of development:
- Initial - the longest period (about 2-3 years from the moment of infection), which is almost asymptomatic due to the absence of local changes. The patient may be disturbed only by signs of nonspecific rhinitis, headaches, and malaise.
- Active - at this stage, the inflammatory process is activated, granulomas, infiltrates or atrophy develop.
- Cicatricial - this period is characterized by the formation of scars in place of granulomas.
Scleroma symptoms
The clinic of scleroma is directly related to the stage of its development. During the initial period, the patient may complain of the presence of general signs of the disease: headache, feeling of fatigue, fatigue, decreased appetite, hypotension is possible. At this stage, the doctor may be alerted by the persistent nature of the above symptoms for a long time, which should become a reason for bacteriological research.
Only in the second period do local changes occur, which are associated with the development of infiltrates of various sizes. A distinctive feature of scleroma is the absence of synechiae at the point of contact of the infiltrates of the opposite walls.
Scleroma of the upper respiratory tract at the last stage is characterized by shortness of breath due to the development of stenosis.
Diagnostics
Early diagnosis of the disease is complicated by the nonspecific nature of the manifestations. If there is a suspicion of a scleroma, then it is necessary to prescribe a bacteriological examination of the nose and pharynx, which allows you to determine the Frisch-Volkovich bacillus even at the initial stage.
In the active phase, the disease can be determined during a thorough examination of the parts of the respiratory tract using publicly available otorhinolaryngological methods or as part of a modern endoscopic examination (fibroendoscopy). It is possible to prescribe an endoscopic biopsy, which allows further histological examination of the tissue taken. Such a diagnosis will be indicative only in the initial and active phases, when the pathogen and Mikulich's cells can be detected.
Additionally, CT and X-ray of the pharynx and larynx may be prescribed, allowing to identify foci of calcification or stripes of ossification. If the development of a disease of a different localization is suspected, then the patient is referred to an ophthalmologist, dentist, dermatologist.
Scleroma treatment
During the therapy of the disease, etiotropic and anti-inflammatory treatment is widely used. This will eliminate the causes of the development of the pathological process. In severe cases, X-ray therapy is used, bougienage of infiltrates in order to expand the lumen of the bronchi. If conservative therapy turns out to be ineffective, then they resort to surgical intervention to remove granulomas, scars and normalize respiratory function.
If you find the main signs of the development of scleroma, then contact the center "Clinic K + 31" for help. Our specialists will offer the most effective therapy methods and will be able to alleviate the condition. You can make an appointment by phone or leave a request on the website.
