Diffuse interstitial lung disease(DIBL) is a general term for a group of diseases characterized by diffuse inflammatory infiltration and fibrosis of the small bronchi and alveoli.
Code according to the international classification of diseases ICD-10:
Etiology and risk factors. Inhalation of various substances.. Mineral dust (silicates, asbestos).. Organic dust.. Mercury vapor.. Aerosols. Taking drugs (bisulfan, bleomycin, cyclophosphamide, penicillamine, etc.). Radiation therapy. Recurrent bacterial or viral diseases lungs. Syndrome respiratory distress adults. Neoplasms.. Bronchoalveolar cancer.. Leukemia.. Lymphomas. Bronchoalveolar dysplasia (Wilson-Mikiti syndrome, interstitial mononuclear focal fibrosing pneumonia). Sarcoidosis. Diffuse connective tissue diseases.. Rheumatoid arthritis.. SLE.. Systemic scleroderma.. Sjogren's syndrome. Pulmonary vasculitis.. Wegener's granulomatosis.. Churg-Strauss syndrome.. Goodpasture's syndrome. Amyloidosis. Hemosiderosis of the lungs. Alveolar pulmonary proteinosis. Histiocytosis. Hereditary diseases.. Neurofibromatosis.. Niemann-Pick disease.. Gaucher disease. CRF. Liver diseases.. Chronic active hepatitis.. Primary biliary cirrhosis. Intestinal diseases.. Nonspecific ulcerative colitis.. Crohn's disease.. Whipple's disease. Graft versus host disease. Left ventricular heart failure. Idiopathic interstitial fibrosis, or cryptogenic fibrosing alveolitis (50% of cases of pulmonary fibrosis), is a chronic progressive hereditary disease with diffuse inflammatory infiltration of the alveoli and increased risk development of lung cancer.
Genetic aspects. Hamman-Rich syndrome (178500, Â). Laboratory: increased collagenase content in lower sections respiratory tract, increased concentration of g - globulins, hyperproduction of platelet b - growth factor. Pulmonary fibrocystic dysplasia (*135000, В) is clinically and laboratory identical to Hamman-Rich disease. Familial interstitial desquamative pneumonitis (pneumocyte proliferation disease type 2, 263000, r), early onset, death before three years. Cystic lung disease (219600, r) is characterized by recurrent respiratory tract infections and spontaneous neonatal pneumothorax.
Pathogenesis. Acute stage. Damage to capillaries and alveolar epithelial cells with interstitial and intraalveolar edema and subsequent formation of hyaline membranes. Both complete reversal and progression to acute interstitial pneumonia are possible. Chronic stage. The process progresses to extensive lung damage and collagen deposition (advanced fibrosis). Hypertrophy smooth muscle and deep breaks in the alveolar spaces lined with atypical (cuboidal) cells. Terminal stage. Lung tissue takes on a characteristic “honeycomb” appearance. Fibrous tissue completely replaces the alveolar and capillary network with the formation of expanded cavities.
Pathomorphology. Severe fibrosis of small bronchi and alveoli. Accumulation of fibroblasts, inflammatory cellular elements (mainly lymphocytes and plasma cells) and collagen fibers in the lumen of small bronchi and alveoli. The growth of terminal and respiratory bronchioles, as well as alveoli, with granulation tissue leads to the development of pulmonary fibrosis.
Pathomorphological classification. Simple interstitial fibrosis. Desquamative interstitial fibrosis. Lymphocytic interstitial fibrosis. Giant cell interstitial fibrosis. Bronchiolitis obliterans with pneumonia.
Clinical picture. Fever. Shortness of breath and dry cough. Weight loss, fatigue, general malaise. Objective research data.. Tachypnea.. Deformation of fingers in the form of “ drumsticks"(with a long course of the disease) .. Inspiratory dry crackling rales (usually in the basal parts of the lungs) .. In severe forms - signs of right ventricular failure.
Laboratory research. Leukocytosis. Moderate increase in ESR. Negative results of serological tests with Ag of mycoplasmas, Coxiella, Legionella, Rickettsia, fungi. Negative results of virological studies.
Special studies. Lung biopsy (open or transthoracic) is the method of choice for differential diagnosis. FVD study - restrictive, obstructive or mixed type violations. Fiberoptic bronchoscopy allows differential diagnosis with neoplastic processes in the lungs. ECG - hypertrophy of the right heart during development pulmonary hypertension. X-ray of the chest organs (minimal changes against the background of pronounced clinical symptoms) .. Finely focal infiltration in the middle or lower lobes of the lungs .. On late stages- picture of a “cellular lung”. Bronchoalveolar lavage - the predominance of neutrophils in the lavage fluid.
TREATMENT. GK.. Prednisolone 60 mg/day for 1-3 months, then gradually reduce the dose to 20 mg/day for several weeks (in the future, the drug at the same dose can be given as maintenance therapy) to avoid acute adrenal insufficiency. The duration of treatment is at least 1 year. Cytostatics (cyclophosphamide, chlorambucil) - only if steroid therapy is ineffective. Bronchodilators (adrenergic agonists inhaled or orally, aminophylline) are advisable only at the stage of reversible bronchial obstruction. Oxygen replacement therapy is indicated when p a O 2 is less than 50-55 mm Hg. Treatment of the underlying disease.
Complications. Bronchiectasis. Pneumosclerosis. Arrhythmias. Acute disorder cerebral circulation. THEM.
Age characteristics. Children - development of interstitial mononuclear focal fibrosing pneumonia due to underdevelopment of the elastic elements of the lung. Long-term course, constant cough, stridor. Frequent formation of bronchiectasis. Elderly people over 70 years of age rarely get sick.
Reduction. DIBL - diffuse interstitial lung disease
ICD-10. J84 Other interstitial pulmonary diseases
APPLICATIONS
Hemosiderosis of the lungs — rare disease, characterized by episodic hemoptysis, pulmonary infiltration and secondary IDA; Children get sick more often younger age. Genetic aspects: hereditary hemosiderosis of the lungs (178550, В); hemosiderosis due to deficiency of g - A globulin (235500, r). Forecast: outcome in pulmonary fibrosis with the development of respiratory failure; The cause of death was massive pulmonary hemorrhage. Diagnostics: FVD study- disorders of the restrictive type, but the diffusion capacity of the lungs may falsely increase due to the interaction of carbon dioxide with hemosiderin deposits in the lung tissue; X-ray of the chest organs - transient pulmonary infiltrates; lung biopsy - identification of macrophages loaded with hemosiderin. Treatment: GK, iron replacement therapy for secondary IDA. Synonyms: anemia pneumohemorrhagic hypochromic remitting, brown idiopathic induration of the lungs, Celena syndrome, Celena-Gellerstedt syndrome. ICD-10. E83 Disorders of mineral metabolism.
Pulmonary histiocytosis- a group of diseases characterized by the proliferation of mononuclear phagocytes in the lungs (Letterer-Siwe disease; Hand-Schüller-Christian disease; eosinophilic granuloma [benign reticuloma, Taratyn's disease] - a disease characterized by the development in the bones or skin of a tumor-like infiltrate consisting of large histiocytes and eosinophils ). The predominant gender is male. Risk factor is smoking. Pathomorphology: progressive proliferation of mononuclear cells and infiltration of eosinophils into the lungs, followed by the development of fibrosis and honeycomb lung. Clinical picture: nonproductive cough, shortness of breath, pain in chest, spontaneous pneumothorax. Diagnostics: moderate hypoxemia; in alveolar washings there is a predominance of mononuclear phagocytes, the possible presence of Langerhans cells identified by monoclonal AT OCT - 6; chest x-ray- pulmonary dissemination with the formation of small cysts, localized mainly in the middle and upper parts of the lungs; FVD study- restrictive-obstructive ventilation disorders. Treatment: smoking cessation, GC (impermanent effect). Forecast: both spontaneous recovery and uncontrolled progression and death from respiratory or heart failure are possible. Note. Langerhans cells - Ag - representing and processing Ag dendritic cells epidermis and mucous membranes, contain specific granules; carry surface cell receptors for Ig (Fc) and complement (C3), participate in HRT reactions, and migrate to regional lymph nodes.
Editor
IIP (idiopathic interstitial pneumonia) is a separate group of inflammatory lung pathologies, which differ from each other in the type of non-infectious pathological process, course and prognosis. The etiology of the disease has not been fully established.
The international classifier implies the ICD-10 code – J 18.9. The course of the disease is usually long-term and severe; there may be consequences due to sclerosis of the lung tissue in the form of pulmonary-heart failure.
In almost all cases, the patient’s quality of life is significantly reduced, loss of ability to work, disability, and death are possible.
In 2001, pulmonologists adopted the ATS/ERS international agreement, which is regularly revised, according to which pathology is classified as follows:
The term “idiopathic” means that the exact cause of the pathology could not be established. 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 become infected with interstitial pneumonia initiated by bacteria and viruses, pathogenic flora penetrates 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 dangerous pathology, which needs immediate treatment.
The symptoms of all types of IIP are either erased or not specific, so the diagnostic process is quite difficult.
For bronchiolitis, inhaled and non-inhaled bronchodilators are prescribed to eliminate obstruction.
Additionally, exercise therapy is prescribed - special exercises that help improve pulmonary ventilation, which are important in preventing respiratory failure.
After six months of such therapy, its effectiveness is assessed. If the results are positive, it is recommended to follow this treatment regimen for a year.
To protect the patient from the addition of a secondary one, antibiotics are prescribed for prophylactic purposes. In some cases, vaccination against influenza and pneumococcal infection is carried out.
Non-traditional treatments (herbs) can be used during the remission stage. Doctors do not recommend self-medication and folk remedies with IIP, as the reaction may become unpredictable. The following give good results medicinal herbs, which have an expectorant and anti-inflammatory effect:
With a strong dry cough, which is accompanied by a sore throat, warm milk with natural honey helps.
The prognosis of the disease is entirely related to the type of pathology and the presence of complications:
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Idiopathic interstitial pneumonia is an interstitial lung disease of unknown etiology that shares similar clinical features. They are classified into 6 histological subtypes and they are characterized by varying degrees inflammatory response and fibrosis and are accompanied by shortness of breath and typical radiographic changes. The diagnosis is made by analyzing the history, physical examination, radiological studies, pulmonary function tests and lung biopsy.
There are 6 histological subtypes of idiopathic interstitial pneumonia (IIP), listed in descending order of frequency: usual interstitial pneumonia (UIP), known clinically as idiopathic pulmonary fibrosis; nonspecific interstitial pneumonia; bronchiolitis obliterans with organizing pneumonia; respiratory bronchiolitis associated with interstitial lung disease RBANZL; desquamative interstitial pneumonia and acute interstitial pneumonia. Lymphoid interstitial pneumonia, although still sometimes considered a subtype of idiopathic interstitial pneumonia, is now thought to be part of the lymphoproliferative disorders rather than primary IBLAP. These subtypes of idiopathic interstitial pneumonia are characterized by varying degrees of interstitial inflammation and fibrosis and all lead to the development of dyspnea; diffuse changes on chest x-ray, usually as an enhanced pulmonary pattern, and are characterized by inflammation and/or fibrosis on histological examination. The above classification is due to the different clinical features of individual subtypes of idiopathic interstitial pneumonia and their different response to treatment.
J84 Other interstitial pulmonary diseases
Known causes of IPD must be excluded. In all cases, chest x-ray, pulmonary function tests and high-resolution CT (HRCT) are performed. The latter makes it possible to differentiate lesions of hollow spaces from those of interstitial tissues, provide a more accurate assessment of the extent and localization of the lesion and are more likely to detect the underlying or concomitant disease(eg, occult mediastinal lymphadenopathy, malignancies, and emphysema). HRCT is best performed with the patient in the prone position to reduce atelectasis of the lower lung.
A lung biopsy is usually required to confirm the diagnosis, unless the diagnosis is made by HRCT. Bronchoscopic transbronchial biopsy can rule out IBLAP by establishing the diagnosis of another disease, but does not provide sufficient tissue to diagnose IBLAB. As a result, diagnosis may require biopsy of a large number of sites during open or video-assisted thoracoscopic surgery.
Bronchoalveolar lavage helps narrow the differential diagnosis in some patients and provides information about disease progression and response to treatment. However, the benefit of this procedure is the initial clinical examination and further observation in most cases of this disease was not established.
Among diffuse parenchymal lung diseases (DPLDs), there are a number of pathological processes, which are not related to infectious factors and in a number of ways may resemble the picture of ARDS, i.e. They are characterized by:
Acute onset;
P a O 2 /FiO 2 ≥200 mm Hg. (≤300 mmHg);
Bilateral pulmonary infiltrates on the frontal x-ray;
Jamming pressure pulmonary artery 18 mmHg or less or none clinical signs left atrial hypertension.
Despite the similarity of these diseases with ARDS (some experts use the term “mimitics” of ARDS), they fundamentally have a different morphological picture, and, most importantly, these diseases require additional anti-inflammatory and immunosuppressive therapy, which has a huge impact on the prognosis. The true incidence of these diseases among ICU patients is unknown. Most DPLD “simulators” of ARDS are quite rare in clinical practice, but all together they significantly influence the number of causes of ARF. Diagnosis of DPLD is very difficult and often requires differentiation from pneumonia. Despite the general similarities clinical picture, diseases from the DPPD group also have certain features that help to diagnose correct diagnosis. Great value diagnostics include CT scan of the lungs, bronchoalveolar lavage (BAL) with cytological examination of washing water, as well as the determination of some biological markers. The tactics of respiratory support for DPHL are practically no different from those used for ARDS. Timely immunosuppressive therapy for DPLD often saves the lives of patients, therefore the most important condition The success of this therapy is its early administration.
Hamman-Rich syndrome.
J84.8. Other specified interstitial pulmonary diseases.
Acute interstitial pneumonia (AIP) is included in the group of idiopathic interstitial pneumonias - clinicopathological forms of diffuse parenchymal lung diseases, characterized by many similar features (unknown nature, similar clinical and radiological signs), which do not allow each of the forms of interstitial pneumonia to be considered a separate nosological unit. Interstitial pneumonia, however, has a sufficient number of differences: first of all, morphology, as well as different approaches to therapy and prognosis (Table 4-17).
Table 4-17. Histological and clinical classification idiopathic interstitial pneumonia (ATS/ERS, 2002)
Histological picture | Clinical diagnosis |
Usual interstitial pneumonia | Idiopathic pulmonary fibrosis (synonym - cryptogenic fibrosing alveolitis) |
Alveolar macrophage pneumonia | Desquamative interstitial pneumonia |
Respiratory bronchiolitis | Respiratory bronchiolitis with interstitial lung disease |
Organizing pneumonia | Cryptogenic organizing pneumonia |
Diffuse alveolar damage | Acute interstitial pneumonia |
Nonspecific interstitial pneumonia |
|
Lymphocytic interstitial pneumonia |
The morphological basis of AIP is diffuse alveolar damage: in the early phase - interstitial and intra-alveolar edema, hemorrhages, fibrin accumulation in the alveoli, the formation of hyaline membranes and interstitial inflammation; in the late stage - collapse of the alveoli, proliferation of type II alveolocytes, fibrosis of the parenchyma.
Etiology unknown. Among the potential causal factors diseases consider impact infectious agents or toxins, genetic predisposition, or a combination of these factors.
One of the most serious illnesses lungs is pneumonia. It is caused by a variety of pathogens and leads to a large number deaths among children and adults in our country. All these facts make it necessary to understand the issues related to this disease.
Pneumonia– spicy inflammatory disease lungs, characterized by exudation of fluid in the alveoli, caused by various types of microorganisms.
Based on the cause of pneumonia, it is divided into:
Modern classification of the disease developed by the European respiratory society, allows you to evaluate not only the causative agent of pneumonia, but also the severity of the patient’s condition.
According to the International Classification of Diseases and Deaths of 1992 (ICD-10), there are 8 types of pneumonia depending on the pathogen that caused the disease:
Since it is rarely possible to identify the causative agent in pneumonia, code J18 (Pneumonia without specifying the causative agent) is most often assigned.
The International Classification of Pneumonia distinguishes the following types pneumonia:
Community-acquired pneumonia is a lung disease of an infectious nature that developed before hospitalization in medical organization under the influence various groups microorganisms.
Most often, the disease is caused by opportunistic bacteria, which are normally natural inhabitants of the human body. Under the influences various factors they are considered pathogenic and cause the development of pneumonia.
Factors contributing to the development of pneumonia:
Main sources community-acquired pneumonia:
The main ways in which microorganisms enter are causing pneumonia V lung tissue is the ingestion of microorganisms with air or inhalation of a suspension containing pathogens.
Under normal conditions, the respiratory tract is sterile, and any microorganism that enters the lungs is destroyed using the lungs’ drainage system. If the functioning of this drainage system is disrupted, the pathogen is not destroyed and remains in the lungs, where it affects the lung tissue, causing the development of the disease and the manifestation of all clinical symptoms.
Very rarely, a route of infection is possible with wounds to the chest and infective endocarditis, liver abscesses
The disease always begins suddenly and manifests itself with various signs.
Pneumonia is characterized by the following clinical symptoms:
Features of clinical symptoms associated with damage to certain areas of the lung. With focal broncho-pneumonia, the disease begins slowly a week after initial signs ailments. The pathology covers both lungs and is characterized by the development acute failure breathing and general intoxication of the body.
For segmental lesions lung is characterized by development inflammatory process generally lung segment. The disease progresses generally favorably, without fever or cough, and the diagnosis can be made accidentally during an X-ray examination.
For lobar pneumonia clinical symptoms are vivid, high body temperature worsens the condition up to the development of delirium, and if the inflammation is located in the lower parts of the lungs, abdominal pain appears.
Interstitial pneumonia possible when viruses enter the lungs. It is quite rare and often affects children under 15 years of age. There is an acute and subacute course. The outcome of this type of pneumonia is pneumosclerosis.
There are features of the course of community-acquired pneumonia in persons who have reached retirement age. Due to age-related changes immunity and the addition of chronic diseases, the development of numerous complications and erased forms of the disease is possible.
Severe develops respiratory failure, it is possible to develop disturbances in the blood supply to the brain, accompanied by psychoses and neuroses.
Hospital-acquired pneumonia is an infectious disease respiratory tract, developing 2-3 days after hospitalization in the hospital, in the absence of symptoms of pneumonia before admission to the hospital.
Among all nosocomial infections ranks 1st in terms of the number of complications. Renders great influence for the cost therapeutic measures, increases the number of complications and deaths.
Divided by time of occurrence:
There are several types of infection due to their occurrence:
Ventilator-associated pneumonia- occurs in patients who long time are on artificial ventilation lungs. According to doctors, one day of a patient being on a ventilator increases the likelihood of contracting pneumonia by 3%.
Postoperative pneumonia is an infectious and inflammatory disease of the lungs that occurs 48 hours after surgery.
Causes of postoperative pneumonia:
Aspiration pneumonia
– infectious disease lungs, resulting from the entry of the contents of the stomach and oropharynx into the lower respiratory tract.
Hospital-acquired pneumonia requires serious treatment with the most modern medicines due to the resistance of pathogens to various antibacterial drugs.
Today there is full list clinical and paraclinical methods.
The diagnosis of pneumonia is made after the following studies:
The process of treating pneumonia can take place both in a medical institution and at home.
Indications for hospitalization of a patient in a hospital:
Main medicines Antibacterial drugs aimed at treating pneumonia are:
If there is no effect from taking the drug for several days, a change is necessary. antibacterial drug. To improve sputum discharge, mucolytics (ambrocol, bromhexine, ACC) are used.
During the recovery period, it is possible to carry out physiotherapeutic procedures (laser therapy, infrared radiation and chest massage)
With untimely treatment or its absence, the following complications may develop:
In 80% of cases, the disease is successfully treated and does not lead to serious adverse consequences. After 21 days, the patient’s well-being improves, and X-ray images show partial resorption of the infiltrative shadows.
In order to prevent the development of pneumococcal pneumonia, vaccination is carried out with an influenza vaccine containing antibodies against pneumococcus.
Pneumonia is dangerous and treacherous enemy for humans, especially if it occurs unnoticed and has few symptoms. Therefore, it is necessary to be attentive to your own health, get vaccinated, consult a doctor at the first signs of illness, and remember what serious complications pneumonia can cause.
