Diffuse interstitial disease of the lungs (DIBL) - a general term for a group of diseases characterized by diffuse inflammatory infiltration and fibrosis of small bronchi and alveoli.
Code for the international classification of diseases of the ICD-10:
Etiology and risk factors. Inhalation of various substances .. Mineral dust (silicates, asbestos) .. Organic dust .. Couples mercury .. aerosols. Reception of drugs (bisulfan, bleomycin, cyclophosphamide, penicillamine, etc.). Radiation therapy. Recurrent bacterial or viral diseases lungs. Syndrome of respiratory distress adults. Neof formation .. bronchoalveolar cancer .. leukemia .. lymphoma. Bronchoalveolar dysplasia (Wilson-Mikiti syndrome, interstitial mononuclear focal fibrosis pneumonia). Sarcoidosis. Diffuse diseases connective tissue.. Rheumatoid arthritis.. SCM .. Systemic sclerodermia .. Syndrome Schogrena. Easy vasculitis .. Vegetter granulosis .. Syndrome of the Black Strauss syndrome. Hoodpascher syndrome. Amyloidosis. Hemosiderosis of lungs. Proteinosis of lungs alveolar. Gisticiocytosis. Hereditary diseases .. Neurofibromatosis .. Nimanne-Peak's disease .. Gosh's disease. CPN. Liver disease .. chronic active hepatitis .. primary biliary cirrhosis. Intestinal diseases .. Nonspecific ulcerative colitis .. Crohn's disease .. Wipple's disease. Reaction "Transplant against the host". Left-deceiving heart failure. Idiopathic interstitial fibrosis, or cryptogenic fibrosis alveolit \u200b\u200b(50% of cases of lung fibrosis), is a chronic progressive hereditary disease with diffuse inflammatory infiltration of alveoli and an increased risk of solicing the lung cancer.
Genetic aspects. Hamman Richa syndrome (178500, Â). Laboratory: an increase in the content of collagenase in the lower heights of the respiratory tract, an increase in the concentration of G - globulin, the hyperproduction of platelet b - growth factor. Dysplasia Easy fibrocistrious (* 135000, Â) Clinical - laboratory is identical to Hamman Rich's disease. Family interstitial desquamation pneumonitis (Pneumocyte proliferation disease 2 type, 263000, R), early start, death up to three years. A lightweight cystic disease (219600, R) is characterized by recurrent infections of the respiratory tract and spontaneous neonatal pneumothorax.
Pathogenesis. Acute stage. The defeat of the capillaries and cells of the alveolar epithelium with an interstitial and intrastallyolar edema and the subsequent formation of hyaline membranes. Possible both complete reverse development and progression to acute interstitial pneumonia. Chronic Stage. The process progresses to extensive damage to the lung and collagen deposit (common fibrosis). Hypertrophy of smooth muscles and deep ruptures of alveolar spaces lined by atypical (cubic) cells. Terminal stage. Light fabric acquires a characteristic appearance of "beesh cells". Fibrous fabric completely replaces the alveolar and capillary network with the formation of extended cavities.
Patomorphology. Pronounced fibrosis of small bronchi and alveoli. The accumulation of fibroblasts, inflammatory cell elements (mainly lymphocytes and plasma cells) and collagen fibers in the lumen of small bronchi and alveoli. The germination of terminal and respiratory bronchiols, as well as the alveoli granulation tissue leads to the development of fibrosis of lungs.
Patomorphological classification. Simple interstitial fibrosis. Deskvamative interstitial fibrosis. Lymphocytic interstitial fibrosis. Gigantaeer interstitial fibrosis. Reputing bronchiolitis with pneumonia.
Clinical picture. Fever. Dyspnea and dry cough. Reducing body weight, fatigue, general ailment. An objective study data .. Tahipneoe .. deformation of the fingers of the hands in the form of "drum sticks" (with a long-term course of the disease) .. Inspector dry crackles (usually in the basal departments of lungs) .. with heavy forms - signs of right-hand deficiencies.
Laboratory research. Leukocytosis. Moderate increase in SE. Negative results of serological tests with AG Mikopolsm, Koksiell, Legionell, Rickettsius, Mushrooms. Negative results of virological studies.
Special studies. The biopsy of the light (open or transomber) is a method of choice when conducting differential diagnostics. Research FVD - restrictive, obstructive or mixed type of disorders. Fibrobronchoscopy allows you to spend differential diagnosis with neoplastic processes in lungs. ECG - hypertrophy of the right heart departments in development light hypertension. X-ray of the chest organs (minimum changes against the background of severe clinical symptoms) .. fine-food infiltration in the middle or lower lungs .. In the later stages - the picture of the "Cellular Light". Bronchoalveolar lavage is the predominance of neutrophils in a boating liquid.
TREATMENT. GK .. prednisone in 60 mg / day for 1-3 months, then a gradual decrease in the dose to 20 mg / day for several weeks (in the future, the drug in the same dose can be given as supportive therapy) to avoid acute adrenal insufficiency. The duration of treatment is at least 1 year. Cytostatics (cycloofosphamide, chlorambucil) - only with the ineffectiveness of steroid therapy. Bronchodulators (adrenomimetics inhalation or inside, aminoophyllin) are appropriate only at the stage of reversible bronchial obstruction. Replacement oxygenotherapy is shown at P A O 2 less than 50-55 mm Hg. Treatment of the main disease.
Complications. Bronchiectase. Pneumosclerosis. Arrhythmia. Acute violation of cerebral circulation. THEM.
Age features. Children - the development of interstitial mononuclear focal focal fibrosis pneumonia due to the underdevelopment of elastic elements of the lung. Long flow, permanent cough, stridor .. Frequent formation of bronchiectasis. Elderly - people over 70 years old are extremely rare.
Reduction. DIBL - diffuse interstitial disease of the lungs
MKB-10. J84 Other Intersitial Email Diseases
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Hemosiderosis of Lögsky - a rare disease characterized by episodic hemopes, lighting infiltration and secondary waiting; More often sick children of younger. Genetic aspects: Inherited hemosiderosis of lungs (178550, Â); Gemosiderosis due to the insufficiency of G - A Globulin (235500, R). Forecast: Exodus in Light Fibrosis with the development of respiratory failure; The cause of death is massive light bleeding. Diagnostics: Investigation of FVD - disorders on restrictive type, but the diffusion capacity of the lungs can be falsely increased due to interaction carbon dioxide with hemosiderin sediments in lightweight fabric; Radiography of chest organs - transient light infiltrates; Light biopsy - detection of macrophages loaded with hemosiderin. Treatment: GK, replacement therapy with iron preparations during the secondary waiting. Synonyms: Anemia Pneumatic hemorrhagic hypochromic remittory, induction of lungs brown idiopathic, is designed syndrome, desiccated-gellershted by syndrome. MKB-10. E83 Mineral Violations.
Gistiocytosis Lekhsky - a group of diseases characterized by proliferation of mononuclear phagocytes in the lungs (Letterlee-Sivey's disease; Hinda-Shuller-Chrischene disease; Eosinophilic granuing [Reticulum benign, Taratynova disease] - disease characterized by developing in bones or skin of tumor-like infiltrates consisting of large histiocytes and eosinophils ). The prevailing floor is male. Risk factor - smoking. Patomorphology: Progressive proliferation of mononuclear cells and infiltration by eosinophils of lungs with the subsequent development of fibrosis and "cellular light". Clinical picture: Unproductive cough, shortness of breath, pain in chestspontaneous pneumothorax. Diagnostics: moderate hypoxemia; in alveolar flips - the predominance of mononuclear phagocytes, possibly the presence of Langherhance cells identified by monoclonal at OKT - 6; radiography of the chest organs - Easy dissemination with the formation of small cysts, localized mainly in the middle and upper parts of the lungs; research FVD - restrictively obstructive ventilation violations. Treatment: cessation of smoking, GK (non-permanent effect). Forecast: It is possible both spontaneous recovery, and uncontrolled progression and death from respiratory or heart failure. Note. Langerca - AG cells - representing and processing ag dendritic cells epidermis and mucous membranes contain specific granules; Surface cell receptors to Ig (Fc) and complement (C3) are carried (C3), they are involved in the RZT reactions, migrate to regional lymph nodes.
Among the diffuse parenchymal diseases of the lungs (DPZL), a number of pathological processes are distinguished, which are not associated with infectious factors and for a number of signs can resemble the painting of the Ords, i.e. For them, it is characteristic:
Acute start;
P a O 2 / FIO 2 ≥200 mm Hg. (≤300 mm Hg);
Double-sided light infiltrates on the front x-ray shift;
Swimming pressure eastern artery 18 mm Hg or less or lack clinical signs left-handed hypertension.
Despite such similarity of these diseases with the ORDS (some experts use the term "simulators" of the ORDS), they are based on another morphological picture, and the most important, in these diseases, additional anti-inflammatory and immunosuppressive therapy is needed, which has a huge impact on the forecast. The true frequency of these diseases among patients is not known. Most DPZl- "simulators" ORDS is rarely found in clinical practiceBut all together they significantly affect the number of reasons for one. DPZL diagnosis is very complex, often requires differentiation with pneumonia. Despite the general similarity of the clinical picture, diseases from the DPZL group have certain features that help to put the correct diagnosis. Of great importance in the diagnosis are CT lungs, bronchoalveolar lavage (ball) with cytological research Washwater, as well as determining some biological markers. The respiratory support tactics of DPZl is practically no different from the ORDS used. Timely immunosuppressive therapy for DPZL often allows you to save the life of the patient, so the most important condition The success of this therapy is its early appointment.
Hammen Richa syndrome.
J84.8. Other refined interstitial light diseases.
Acute interstitial pneumonia (OIP) is included in the group of idiopathic interstitial pneumonium - clinical and pathological forms of diffuse parenchymal diseases of light, characterized by many similar features (unknown nature, close clinical and radiological signs), not allowing to consider each of the forms of interstitial pneumonia with a separate nosological unit. Interstitial pneumonia, however, have a sufficient number of differences: first of all, morphology, as well as different approaches to therapy and the forecast (Table 4-17).
Table 4-17. Histological I. clinical classification idiopathic interstitial pneumonia (ATS / ERS, 2002)
Histological picture | Clinical diagnosis |
Normal interstitial pneumonia | Idiopathic light fibrosis (synonym - cryptogenic fibrosis alveolo) |
Alveolar macrophageal pneumonia | Deskvamative interstitial pneumonia |
Respiratory bronchiolitis | Respiratory bronchiolite with an interstitial disease of the lungs |
Organizing pneumonia | Cryptogenic organic pneumonia |
Diffuse alveolar damage | Acute interstitial pneumonia |
Nonspecific interstitial pneumonia |
|
Lymphocytic interstitial pneumonia |
The morphological basis of the OIP is a diffuse alveolar damage: in the early phase - interstitial and intrastallyolar edema, hemorrhage, clusters of fibrin in alveoli, the formation of hyaline membranes and interstitial inflammation; In late - discontinuation of Alveol, the proliferation of alveolocyte type II, fibrosis of parenchyma.
Etiology is unknown. Among the potential causal factors, the impact of infectious agents or toxins, genetic predisposition, as well as a combination of these factors.
Editor
IIP (idiopathic interstitial pneumonia) is a separate group of inflammatory pathologies of lungs, which differ from each other by the type of the pathological process of non-infectious nature, the flow and forecast. The etiology of the disease is not fully established.
The international classifier implies the code on the ICD-10 - J 18.9. The course of the disease is usually long-term and severe, consequences are possible due to and sclerosis of pulmonary fabric in the form of pulmonary heart failure.
Almost in all cases, the quality of the patient's life is significantly reduced, disability, disability, fatal outcome is possible.
In 2001, pulmonologists adopted an international ATS / ERS Agreement, regularly revised, according to which pathology is classified in this way:
The term "idiopathic" means that the exact cause of pathology failed to establish. There are groups of provoking factors that can contribute to the development of IIP:
The pathological process can be accelerated by microorganisms. If we talk about whether it is possible to infect interstitial pneumonia, initiated by bacteria and viruses, pathogenic flora penetrates into the lungs in the following ways:
New types of IIP:
Each type of disease has distinctive features:
Important! Regardless of the form of the disease, interstitial pneumonia is a dangerous pathology that needs immediate treatment.
The symptoms of all types of IIP or erased, or are not specific, so the diagnostic process is quite difficult.
With bronchipolitis, in order to eliminate obstruction, inhalation and non-digitalia brightness are prescribed.
Additionally, a leaf is assigned - special exerciseswhich contribute to the improvement of pulmonary ventilation, important in the aspect of preventing respiratory failure.
After six months of such therapy, its effectiveness is evaluated. If the results are positive, this treatment regimen is recommended for a year.
To protect the patient from joining secondary, with preventive goal Antibiotics are prescribed. In some cases, flu vaccination and pneumococcal infection are carried out.
Unconventional treatments (herbs) can be applied at the remission. Doctors do not recommend self-treatment and folk remedies When IIP, since the reaction can become unpredictable. Good results give the following medicinal herbalExcraceing and anti-inflammatory action:
With a strong dry cough, which is accompanied by a throat, helps warm milk with natural honey.
The forecast of the disease is entirely associated with the type of pathology and the availability of complications:
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Ethiopathic interstitial pneumonia - interstitial diseases of the lungs of unknown etiology, which combine similar clinical features. They are classified in 6 histological subtypes and they are characterized various degrees Inflammatory reaction and fibrosis and are accompanied by shortness of breath and typical changes in radiography. The diagnosis is established in the analysis of the data of the anamnesis, physical examination, the results of radiation research, the study of the function of lungs and biopsy of the lung.
6 histological subtypes of idiopathic interstitial pneumonia (IIP) specified in descending order of frequency: the usual interstitial pneumonia (OIP), known clinically as idiopathic fibrosis of the lungs; nonspecific interstitial pneumonia; binding bronchiolitis with organic pneumonia; Respiratory bronchioratus associated with an interstitial disease of the lungs of RBBZL; Deskvamative interstitial pneumonia and acute interstitial pneumonia. Lymphoid interstitial pneumonia, although it is still sometimes considered as a subtype of idiopathic interstitial pneumonia, as is now considered to be part of lymphoproliferative diseases, and not primary iblarb. These subtypes of idiopathic interstitial pneumonia are characterized by varying degrees of interstitial inflammation and fibrosis and everyone leads to the development of shortness of breath; diffuse changes On the radiograph of the chest organs, usually in the form of amplification of the pulmonary pattern, and are characterized by inflammation and / or fibrosis in histological examination. The above classification is due to various clinical features Separate subtypes of idiopathic interstitial pneumonia and their different responses for treatment.
J84 Other interstitial pulmonary diseases
The well-known causes of ILD should be excluded. In all cases, radiography of the chest organs is performed, research of the function of the lungs and high-resolution CT (KTRT). The latter allows the differentiation of hollow spaces from such interstitial tissues, to ensure a more accurate estimate of the prevalence and localization of the lesion and is more likely to detect the main or accompanying disease (for example, hidden mediastinal lymphadenopathy, malignant tumors and emphysema). Katr is better to perform in the patient's position lying on the stomach to reduce the atelectasis of the lower lung departments.
To confirm the diagnosis, the lung biopsy is usually required, with the exception of situations where the diagnosis is set according to the KTRT. The bronchoscopic transbronchial biopsy can exclude iblarb by setting a diagnosis of another disease, but does not allow to obtain a sufficient number of fabric for the diagnosis of IBLRB. As a result, for the diagnosis, a biopsy of a large number of sections may be required when performing open or video solicoscopic surgical intervention.
Bronchoalveolar lavary helps narrow the amount of differential diagnostic search in some patients and provide information on the progression of the disease and respond to the treatment carried out. However, the benefits of this procedure in the initial clinical examination and further observation in most cases of this disease was not established.
One of the most serious diseases The lungs are pneumonia. It is caused by the most different pathogens and leads to a large number of deaths among the children's and adult population of our country. All these facts cause the need to understand issues related to this disease.
Pneumonia - acute inflammatory lung disease characterized by exudation of fluid in alveoli caused by various species microorganisms.
Due to the delivered pneumonia, it is divided:
The modern classification of the disease developed by the European respiratory society makes it possible to evaluate not only the pathogen of pneumonia, but also the severity of the patient's condition.
According to International Classification Diseases and deaths of 1992 (ICD-10) identify 8 types of pneumonia, depending on the causative agent of the disease caused:
Since at pneumonia, it is rarely able to identify the pathogen, most often assign the code J18 (pneumonia without refining the pathogen)
International classification of pneumonium allocates the following types of pneumonia:
Complete pneumonia - this is a disease of light infectious nature, which developed to hospitalization in medical organization Under the influence of various groups of microorganisms.
Most often, the disease is caused by conditionally pathogenic bacteria, which are normal in the natural inhabitants of the human body. Under the influences of various factors, they are put by pathogenic and cause the development of pneumonia.
Factors contributing to the development of pneumonia:
Basic sources of community-friendly pneumonia:
The main methods of microorganisms hitting the pneumonium into the pulmonary fabric is to swallow microorganisms with air or inhalation of the suspension-containing pathogens.
Under normal conditions, the respiratory tract is sterile, and any in the light microorganism is destroyed by drainage system lungs. In disruption of the work of this drainage system, the causative agent is not destroyed and remains in the lungs, where the pulmonary fabric is striking, causing the development of the disease and the manifestation of all clinical symptoms.
It is very rare the path of infection during breast injuries and infectious endocardits, liver abscesses
The disease always begins suddenly and manifests itself with different signs.
Pneumonia characterize the following clinical symptoms:
Features of clinical symptoms related to the defeat of certain sections of the lung. In focal broncho-pneumonia, the disease begins slowly a week after the initial signs of malaise. Pathology covers both lungs is characterized by the development of acute respiration and general intoxication of the body.
With segmental lesion Lung is characteristic of development inflammatory process In general, the lung segment. The disease proceeds mainly favorably, without lifting temperature and cough, and the diagnosis can be randomly random when the radiological examination is passed.
With truck pneumonia Clinical symptoms are bright, high body temperature gives deterioration of the state right up to the development of nonsense, and the loss of inflammation in the lower lungs appear abdominal pain.
Interstitial pneumonia It is possible if viruses get into lungs. It is quite rare, children are often sick up to 15 years. Allocate acute and subacute flow. The outcome of this type of pneumonia is pneumosclerosis.
There are flow features complete Pneumonia In persons who have reached retirement age. Due to age-related changes in immunity and accession chronic diseases It is possible to develop numerous complications and erased forms of the disease.
Severe respiratory failure develops It is possible to develop violations of blood supply to the brain, accompanied by psychosis and neurosis.
Hospital (hospital) pneumonia - this is an infectious disease respiratory tract, developing 2-3 days after hospitalization in the hospital, in the absence of symptoms of pneumonia before entering the hospital.
Among all nosocomial infections takes 1 place in terms of complications. Has a big impact on the cost medical events, increases the number of complications and deaths.
By time of occurrence, it is divided:
Due to the occurrence, several types of infection are distinguished:
Fan-associated pneumonia - occurs in patients who are for a long time artificial ventilation lungs. According to doctors, one day of finding a patient on the machine of artificial ventilation of the lungs increases the likelihood of infection with pneumonia by 3%.
Postoperative pneumonia - infectious lung disease, occurring 48 hours after surgery
Causes of postoperative pneumonia:
Aspiration pneumonia – infectious disease The lungs arising from the ingress of the contents of the stomach and the rotogling into the lower respiratory tract.
Hospital pneumonia require serious treatment with the most modern medicinal preparations Due to the stability of pathogens to various antibacterial drugs.
To date, there is a complete list of clinical and paraklinic methods.
The diagnosis of pneumonia is raised after the following studies:
The treatment of pneumonia can be held both in a medical institution and at home.
Indications for hospitalization of the patient in the hospital:
Basic drugsaimed at treatment of pneumonia are antibacterial drugs:
In the absence of an occurrence of the effect of drug intake for several days, a change is required antibacterial drug. Mulcolics are used to improve sputum removal (amboccol, bromgexin, ADC).
During the recovery period, physiotherapy procedures are possible (laser therapy, infrared radiation and chest massage)
In case of untimely treatment or its absence, the development of the following complications is possible:
In 80% of cases, the disease is successfully treated and does not lead to serious adverse consequences. After 21 days, the health of the patient is improved, a partial absorption of infiltrative shadows begins on the X-ray.
In order to prevent the development of pneumococcal pneumonia, vaccination is carried out by an anti-influenza vaccine containing antibodies against pneumococcus.
Pneumonia is a dangerous and cunning enemy for a person, especially if it flows unnoticed and low-spirited. Therefore, it is necessary to be attentive to your own health, leaving vaccination, at the first signs of the disease to contact the doctor and remember what serious complications may threaten pneumonia.