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Idiopathic interstitial pneumonia
Last reviewed: 23.04.2024
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Idiopathic interstitial pneumonia is an interstitial lung disease of unknown etiology that combines similar clinical features. They are classified into 6 histological subtypes and they are characterized by varying degrees of inflammatory response and fibrosis and are accompanied by shortness of breath and typical changes in radiography. The diagnosis is established by analyzing the history, physical examination, the results of radiation studies, lung function tests and lung biopsy.
Six histological subtypes of idiopathic interstitial pneumonia (IPI) were identified, listed in order of decreasing frequency: usual interstitial pneumonia (IPI), known clinically as idiopathic pulmonary fibrosis; nonspecific interstitial pneumonia; obliterating bronchiolitis with organizing pneumonia; respiratory bronchiolitis associated with interstitial lung disease RRBNZL; desquamative interstitial pneumonia and acute interstitial pneumonia. Lymphoid interstitial pneumonia, although still sometimes seen as a subtype of idiopathic interstitial pneumonia, is now considered to be part of lymphoproliferative diseases, rather than primary IBLARB. 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 the roentgenogram of chest organs, usually as an enhancement of the pulmonary pattern, and are characterized by inflammation and / or fibrosis in histological examination. The above classification is due to various clinical features of individual subtypes of idiopathic interstitial pneumonia and their different response to treatment.
[Diagnosis of idiopathic interstitial pneumonia
The well-known causes of IDD should be excluded. In all cases, chest X-ray, lung function and high-resolution CT scan (CTWR) are performed. The latter allows differentiating lesions of hollow spaces from such interstitial tissues, providing a more accurate estimate of the prevalence and localization of the lesion, and more likely to detect underlying or concomitant disease (eg, hidden mediastinal lymphadenopathy, malignant tumors, and emphysema). HRCT is best performed in the patient's position lying on the abdomen to reduce the atelectasis of the lower parts of the lung.
To confirm the diagnosis, a lung biopsy is usually required, except for cases when the diagnosis is established according to HRCT data. A bronchoscopic transbronchial biopsy can rule out an IBLARB, diagnosing another disease, but does not allow sufficient tissue to be diagnosed for IBLARB. As a result, a biopsy of a large number of sites may be required to make a diagnosis when performing an open or videotouracoscopic surgical intervention.
Bronchoalveolar lavage helps to narrow the amount of differential diagnostic search in some patients and provide information on the progression of the disease and its response to ongoing treatment. However, the benefit of this procedure in the initial clinical examination and further observation in most cases of this disease has not been established.
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Treatment of idiopathic interstitial pneumonia
Treatment of idiopathic interstitial pneumonia depends on the subtype, but usually includes glucocorticoids and / or cytotoxic agents, but is often ineffective. Idiopathic interstitial pneumonia has a different prognosis. It depends on the subtype and varies from very favorable to almost always fatal.