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Nonspecific interstitial pneumonia
Last reviewed: 05.07.2025
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[ What causes nonspecific interstitial pneumonia?
Non-specific interstitial pneumonia is probably a separate nosological entity. Its frequency and prevalence are unknown, but it is probably the second most common among all forms of idiopathic interstitial pneumonia (accounting for 14 to 36% of the described cases). Most cases are registered in patients with systemic connective tissue diseases, drug-induced IBLAR, or chronic hypersensitivity pneumonitis. In some cases, the etiology of the disease remains unknown.
Symptoms of non-specific interstitial pneumonia
The symptoms of nonspecific interstitial pneumonia are similar to those of idiopathic pulmonary fibrosis. Most patients are between 40 and 60 years of age. Cough and shortness of breath are present for many months or years.
Diagnosis of non-specific interstitial pneumonia
Chest X-rays show predominantly increased pulmonary markings in the lower sections. Bilateral infiltrates may also be present. HRCT shows bilateral ground-glass opacities, bilateral foci of consolidation, irregular linear structures, and bronchial dilation. Ground-glass opacities are the predominant finding in most cases and the only sign of the disease in about a third of cases.
The main feature of histological changes in nonspecific interstitial pneumonia is the development of homogeneous inflammation and fibrosis, which is the opposite of the heterogeneity of inflammation foci in usual interstitial pneumonia. The lesions are usually identical, but the process can be focal, with separate areas of intact lung. Cellularity is rare.
Treatment of non-specific interstitial pneumonia
Most patients have a good prognosis after treatment with glucocorticoids.
What is the prognosis for nonspecific interstitial pneumonia?
Non-specific interstitial pneumonia has a poor prognosis. Relapses may occur. In some patients, the disease progresses; in this case, their average life expectancy is 5 to 10 years after diagnosis. The approximate ten-year mortality rate is less than 15-20%.