What is nonspecific interstitial pneumonia? IIP differ in that their etiology is not yet established, but they have a number of distinctive features from each other: the course of the disease, the morphological signs, the outcome of the disease.


The history of the disease group begins in 1935, when the first 4 patients with rapidly progressive respiratory failure were described. Their death came in six months. At autopsy, pulmonary fibrosis was found( consolidation of connective tissue with the formation of multiple scars).Then such a disease was called acute interstitial diffuse pulmonary fibrosis.

Further, in 1964, the pathology was renamed fibrosing alveolitis, since such a diagnosis more accurately characterizes the course of the disease: fibrosis and inflammation. And in the CIS countries the name fibrosing idiopathic alveolitis is used.

Interstitial desquamative pneumonia was first described in 1965, and in 1968 interstitial pneumonia groups were identified according to morphological features:

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  • Obliterating bronchiolitis;
  • Interstitial pneumonia;
  • Desquamative interstitial pneumonia
  • Lymphoid interstitial pneumonia;
  • Giant cell interstitial pneumonia.

Subsequently, the classification was several times changed, and the final version was approved in 2001.

So, the ISP is divided into 7 groups:

  1. fibrosing alveolitis;
  2. cryptogenic organizing;
  3. nonspecific interstitial;
  4. acute interstitial;
  5. desquamative interstitial;
  6. respiratory bronchiolitis;
  7. lymphoid interstitial.

It is difficult to establish the exact distribution of IIPS in the world, since often a correct diagnosis is not made.

Common signs of

The first sign is impaired lung ventilation associated with a decrease in lung capacity. As an exception, respiratory bronchiolitis appears - there is an increase in pulmonary volume. When analyzing the blood, hypocapnia is identified with respiratory alkalosis and hypoxemia. The patient has shortness of breath, often cough, difficulty breathing, weight loss.

General Characteristics of Nonspecific Interstitial Pneumonia( NPI)

Until recently, NIP has been treated with idiopathic pulmonary fibrosis, but has been isolated as a separate disease group because it has a relatively favorable prognosis and characteristic morphological features.

Diagnosis of a NID is complicated by the fact that histological examination of the affected lungs results in a picture similar to a number of other diseases. Therefore diagnose by an exception method.

Found that the disease is more likely to affect people in the age group from 40 to 50 years, more often women.

Morphology

As a rule, changes affect the interstitium of the alveoli. There are changes in microvessels, and fibrinoid necrosis is possible. Cases of the formation of destructively productive vasculitis have been noted. Affected areas of the lung are often located in a mosaic pattern, that is, areas of damaged areas alternate with healthy areas.

The results of microscopic examination depend on the stage of the disease:

  • for the early stages is characterized by the presence of inflamed areas with interstitial macrophages and lymphocytes, swelling of the stroma is noticeable;
  • in the advanced stages of fibrous tissue is a coarse-fibrous connective tissue.

Reasons for the development of the

. The spread and frequency of the disease is difficult to establish. But it is assumed that the NPI takes second place in frequency among the ISP.

Most patients have had a NID in the presence of existing systemic diseases associated with connective tissue. But in most cases the cause remains unclear. Smoking is not related to the emergence of this pathology.

Often the occurrence of a NID is associated with autoimmune diseases, for example, with scleroderma or rheumatoid arthritis.

Symptoms of the disease

Symptoms develop gradually. It often takes a long time to develop all the signs. Because of this, diagnosis of the disease takes up to three years.

  • First of all, dry cough and shortness of breath appear. Their intensity increases for a long time.
  • In 50% of cases, the patient's weight was reduced by approximately 6 kg.
  • The temperature rises in rare cases. And the nail phalanges do not change more than in 35% of patients.
  • Pulmonary ventilation is impaired slightly, diffusion pulmonary capacity is reduced, with the appearance of hypoxemia is typical.
  • When examining the radiological method in the lower pulmonary department, infiltrative bilateral changes are noticeable.
  • In studies using QD, subpleurally located symmetrical regions of the type of "frosted glass" are found. In about 30% of patients, this symptom is the only criterion for diagnosing a NID.
  • Approximately 50% of patients have seen reticular changes.
  • Sections of compacted pulmonary tissue and a symptom of the "cellular lung" are rarely noted.

Diagnosis

As mentioned earlier, diagnosing the disease is extremely difficult. This is due to the absence of specific symptoms and with a long time of their manifestation.

It is necessary to pay attention to the fact that with NIP fibrosis and inflammation appear immediately. As a rule, a specific morphological type of the disease is not established, since the treatment of IPA is conducted at a single rate.

However, in the treatment, an accurate diagnosis can be established, since the NID in most cases is easily treatable. But the structure of the lungs is not fully recoverable.

Pay attention to the rate of progression of respiratory failure: with a NID, it is small.

When X-ray examinations are trying to detect reticular changes, which are localized, as a rule, in the basal departments. Characteristic features are the presence of traction bronchiectasis, foci of consolidation and a smaller pulmonary volume.

Histological examination reveals signs of fibrosis and inflammation. The fibrosis itself is uniform.

Often, the diagnosis is made by excluding other pathologies. Therefore, in a number of cases, other studies are also being carried out:

  • CT of lungs gives a detailed picture of them and nearby structures;
  • The use of high-resolution CT scans improves the quality of the image obtained and facilitates the diagnosis;
  • Special tests are performed to evaluate respiratory function( spirometry, bodipletismography);
  • Biopsy material obtained from the lungs is subject to microscopic examination. Often only this method can accurately diagnose a NIP.

Treatment of a NID

The treatment regimen is selected individually in each case. As a rule, corticosteroids are used, it is possible to use cytostatics. In time, the diagnosed disease is quite easy to treat, but complete recovery of the lung tissue is not.

In case of pulmonary insufficiency, special preparations are used to supply oxygen to the body.

In the most severe cases, an effective treatment can only be a lung transplant.

Forecast

After the treatment, relapses are often observed. Without a lung transplant, complete tissue repair does not occur. Approximately the lethality within 10 years is less than 20%.