Scleroma

Scleroma (rhinoscleroma, scleroma of the respiratory tract, sclerotic disease) is a chronic infectious disease caused by the Frish-Volkovich bacillus (Klebsiella pneumoniae rhinoscleromatis), characterized by the formation of granulomas in the walls of the upper respiratory tract (mainly nose), which subsequently undergo fibrosis and cicatricial wrinkling, leading to to stenosis of individual parts of the respiratory tract.

ICD-10 code

J31.0. Rhinitis granulomatous chronic.

Epidemiology of scleroma

The disease is spread all over the world in the form of large, medium and small foci. Endemic to sclera is considered Central and Eastern Europe, including Western Ukraine and Belarus, Italy, Central and South America. Africa, South-East Asia, Egypt, India, the Far East. The area, endemic to the sclera, has certain characteristics. First of all), these are lowland areas of wood with sparse forests and swamps, where the majority of the population is engaged in agriculture. Scleroma is more common in women. There were cases of scleroma in some isolated villages. The members of one family are often affected, where 2-3 people are ill. The disease is associated with a low socioeconomic status, and in the developed countries, for example the USA, it is very rare. The situation may change due to population migration.

To date, no precise mechanisms and conditions for human infection have been established. Most researchers believe that the transmission of infection from the patient occurs through contact and through public objects. It was noted that in the bacteriological study of the material from the affected organs, members of the same family with scleroma suffer from Klebsiella pneumoniae rhinoideromatis with the same characteristics.

[1], [2], [3], [4], [5]

Causes of scleroma

At present, the infectious nature of the disease is beyond doubt. This is confirmed by the natural focal spread of the disease and by the contact route of transmission of the infection. The causative agent of the scleroma is the gram-negative daddy of Frish-Volkovich (Klebsiella pneumoniae rhinoscieromatis), first described in 1882 by Frisch. Klebsiella pneumoniae rhinoscleromatis is detected in all patients, especially during the active period of infiltrate formation and granuloma, mucosal dystrophy.

[6], [7]

Pathogenesis of scleroma

Klebsiella pneumoniae rhinoscleromatis is classified as encapsulated microorganisms. The presence of the capsule protects the bacilli and inhibits the phagocytosis process by macrophages, which leads to the formation of specific large Mikulic cells that are distinguished by a peculiar foamy structure of the protoplasm. At the beginning of the disease, local disorders in the respiratory tract are not observed. In the second, active period, changes develop in different parts of the respiratory tract, which can occur in the form of dystrophic or productive phenomena with the formation of an infiltrate, granulomas in various parts of the respiratory tract. Epithelium covering scleral infiltration, as a rule, is not damaged. Infiltrates can have endophytic growth, spreading to the skin of the external nose, causing it to deform, or exophytic, leading to a disruption in the function of respiration (in the nasal cavity, nasopharynx, larynx and trachea).

The final stage of transformation of the scleral infiltrate is the formation of the scar, which sharply narrows the lumen of the respiratory cavity cavities to a limited extent or at a considerable extent, leading to stenosis and a sharp impairment of the functional state. In the stage of scarring, connective tissue elements prevail, the scleroma rod and Mikulich cells are not detected.

Scleroma is distinguished by the transition of the granuloma immediately to the cicatrical stage, the absence of destruction and decay of the infiltrate. When sclera is never affected by bone tissue.

Symptoms of scleroma

At the beginning of the disease, patients complain of weakness, fatigue, headache, loss of appetite, sometimes thirst, the phenomenon of arterial and muscular hypotension. Local changes in respiratory organs are not observed.

Attention is drawn to the decrease in the tactile and pain sensitivity of the mucous membrane of the respiratory tract. Such symptoms can be observed for a long time and do not have a specific character. However, given the permanence, stability of these manifestations, one can suspect scleroma and refer the patient to a specific bacteriological examination. During this period, Klebsiella pneumoniae rhinoscleromatis can be found in the material from any part of the respiratory tract, more often from the mucous membrane of the nasal cavity.

Diagnosis of the disease at the initial stage can be of decisive importance with regard to the effectiveness of treatment, clinical observation and a positive prognosis.

In the second, active period, changes are observed in different parts of the respiratory tract, in the form of a dystrophic or productive form. It is possible to identify the atrophy of various parts of the nasal mucous membrane, pharynx, larynx, the formation of viscous mucus and dry crusts. With productive form, the formation of infiltrate, granuloma in various parts of the respiratory tract is noted. The size of the affected areas varies from limited small rashes to continuous tumor-like formations without destruction of the mucous membrane, without the formation of atresia and synechiae at the points of contact of the infiltrates of the opposite sections of the mucous membrane. Infiltrates can have zondophytic growth and spread to the skin of the external nose, causing it to deform, or exophytic, leading to a disruption in the function of respiration (in the nasal cavity, nasopharynx, larynx and trachea).

In addition to the violation of breathing, reflex, defensive, resonator dysfunctions develop, the sense of smell is greatly reduced. Difficulty in breathing (stenosis of the larynx), hoarseness, decreased protective function.

Infiltrates of the nasal cavity are often observed in the anterior part of the anterior end of the inferior nasal cavity and in the opposite sections of the nasal septum. In the middle section of the nasal cavity they are rare. More often infiltrates are located in the area of the khuans with the transition to the soft palate and a small tongue, the upper sections of the arms of the palatine tonsils, leading to their deformation. When scarring infiltrates formed incomplete atresia of the nasopharynx.

It is characteristic that in one patient infiltrates and scar changes can simultaneously be in different parts of the respiratory tract. Sometimes after the scarring of the granuloma, it is possible to observe the formation of an infiltrate at a neighboring site of the mucosa. In the larynx, infiltrates are more often localized in the lining department, causing a violation of respiratory, protective and voice-forming functions.

It should be noted that in a number of patients with the presence of scleral infiltrates, sites with signs of mucosal dystrophy (mixed form) are detected.

The clinical picture of scleroma in the active stage (obvious signs of the disease) depends on the form of the process. At the phenomena of atrophy patients complain of dryness in the nose, viscous, thick discharge, crust formation, decrease or loss of smell. Sometimes a large number of crusts in the nasal cavity is accompanied by the appearance of a sweetish-sugary smell, which is felt by others, but differs from that in the lake. With an objective examination of the patient, parts of the atrophic mucosa, the cortex are visible.

In the case of the formation of a scleral granuloma, the mucosa has dense, differently sized infiltrates of yellowish or grayish-pink color, covered with intact epithelium. With the formation of cicatricial changes, patients complain of a violation of the functions of the nose and larynx. The sclerotic process in the larynx can also lead to stenosis and require urgent tracheotomy.

Classification

The sclerotic process proceeds slowly, for years and decades, and passes several periods of its development: initial (hidden), active, regressive. The initial stage is characterized by nonspecific symptoms of rhinitis. Distinctive features of the active period of infiltration or atrophy. Scar formation indicates a regressive stage.

The scleroma affects mainly the airways, however the process can proceed in isolation and any organ or totally, affecting the nose, pharynx, larynx, trachea and bronchi in any forms of manifestation that are also used in the classification.

The main forms of the process are: dystrophic, productive and mixed.

[8], [9], [10], [11]

Screening

In the case of chronic rhinitis, especially in areas endemic to the sclera, it is necessary to remember the possible damage to the nasal mucosa of Klebsiella pneumoniae rhinoscleromatis and use additional specific methods of investigation.

Diagnosis of scleroma

Diagnosis of the disease is based on an analysis of the patient's history and complaints. It is necessary to pay attention: to the place of residence, assessing the natural-focal nature of scleroma development: for the presence of patients among family members. It is important to estimate the age of the patient, since the disease is often detected in 15-20 years. In children, the sclerotic process is more often localized in the larynx and can lead to its stenosis.

Special attention should be paid to the patient's general complaints (weakness, fatigue, headache) under the circumstances indicated above (endemic focus, young age, presence of scleroma in a community or family.

With an obvious manifestation of scleroma in the respiratory tract, complaints are determined by the form of the disease (dryness, cortex, difficulty breathing, hoarseness, etc.).

[12], [13], [14], [15], [16]

Physical examination

If a sclera is suspected, a thorough examination of all parts of the respiratory tract should be carried out by generally available methods used in otorhinolaryngology and, if possible, by modern endoscopic methods (fibroendoscopy of the nasal cavity and nasopharynx, pharynx, larynx, trachea and bronchi). Mandatory determination of the functional state of the respiratory tract.

Laboratory research

It is necessary to investigate the microflora from different parts of the respiratory tract.

In doubtful cases, in the absence of growth of Klebsiella pneumoniae rhinoscleromatis, specific serological reactions can be used. Also conducts a histological examination of the biopsy material.

Instrumental research

The diagnosis can be facilitated by the use of endoscopic and X-ray methods of examination, in particular CT.

Differential diagnosis of scleroma

Differential diagnosis of scleroma is carried out with granulomatic processes in tuberculosis, syphilis, Wegener's granulomatosis. From these diseases, scleroma is distinguished by the absence of destruction and decomposition of the infiltrate, as well as the transformation of the granuloma directly into the scar tissue. When sclera is never affected by bone tissue. Klebsiella pneumoniae rhinoscleromatis is found on the surface of the mucous membrane and under the epithelial layer and thicker than the granulomas together with the specific large cells of Mikulich and the free-laying hyaline corpuscles of Roussel. Epithelium covering scleral infiltration, as a rule, is not damaged.

[17], [18], [19], [20], [21], [22], [23],

Indications for consultation of other specialists

In case of deformation of the external nose due to the spread of scleral infiltrates on the skin of the wings of the nose, consultation of the dermatologist is shown, with the involvement of the tear ducts, consultation of the oculist is necessary, in the initial stage of the disease in general manifestations (weakness, fatigue, headaches, etc.) therapist.

Objectives of scleroma treatment

The goals of the treatment are elimination of the pathogen, reduction of inflammation, prevention of breathing disorders, removal of infiltrates and scarring. Currently, these activities can lead to recovery at any stage of the disease.

Indications for hospitalization

Indications for hospitalization include the need for a comprehensive treatment of scleroma, including surgical, as well as a pronounced impairment of the respiratory function that requires bougieering, and in some cases tracheotomy or laryngophyssura application.

Non-drug treatment

Vouching (crushing) of infiltrates, anti-inflammatory R-therapy with doses from 800 to 1500.

Medicamentous treatment of scleroma

Streptomycin is prescribed in a dose of 0.5 g 2 times a day for a course of treatment lasting 20 days (the maximum total dose is 40 g).

Surgical treatment of scleroma

Surgical excision of infiltrates and scars.

Further management

Patients with scleroma need regular follow-up and, if necessary, repeated courses of drug therapy. It may be necessary to replace medicines and eliminate new infiltrative lesions by means of bougie, crushing, R-therapy, etc.

The terms of incapacity for work depend on the degree of impairment of the function of breathing and the methods of elimination taken, approximately 15-40 days.

It is necessary to pay attention to employment and examination of incapacity for work.

The patient is recommended to follow the rules of personal hygiene.

Prophylaxis of scleroma

Preventative measures should be aimed at preventing the possibility of transmission of infection from a sick person. This involves improving the living conditions, improving well-being, adhering to the rules of general and individual hygiene, changing the natural conditions in the lesion focus. About visible in this direction, activities in some areas in recent years have significantly reduced the incidence of scleroma.

Forecast

With a timely begun treatment, the prognosis is favorable. In the literature, in most cases, restoration of organon functions, complete elimination of the scleroma rod according to bacteriological studies, reduction or absence of specific serological reactions are described.

[24], [25]