Idiopathic interstitial pulmonary fibrosis: an unspeakable pain for respiratory physicians!

Idiopathic interstitial pulmonary fibrosis, or IPF, is a chronic progressive fibrotic disease involving the interstitium and parenchyma of the lungs, which pathologically manifests itself as alternating zones of interstitial inflammation, fibrosis, honeycomb lungs, and areas of normal lung tissue, with the most prominent being the focal fibroblastic hyperplasia seen. Pathologically, it manifested as a common type of interstitial pneumonia, i.e., UIP, and its most striking feature at low magnification was the variable severity and uneven distribution of the lesions. The main imaging manifestations are subpleural reticular shadows at the base and periphery of both lungs, often bilateral and asymmetric, mixed with decreased lung volume, and honeycomb lung changes in the advanced stage. Chest CT, especially high-resolution CT, i.e. HRCT, mainly shows: focal ground-glass shadow; reticular shadow; honeycomb lung, deformation of lung structure and volume reduction, irregular interfacial interface, pleural thickening, thickening of bronchial vascular bundles, enlarged mediastinal lymph nodes, etc. However, its typical manifestations are bilateral lung bases and peripheral subpleura. However, its typical manifestations are reticular shadows distributed bilaterally at the base of the lungs and under the surrounding pleura, honeycomb lungs, pulling bronchiectasis, deformation of lung structure, no or few frosted glass shadows, and lack of small nodules. In general, the diagnosis of IPF can be made directly on HRCT with a typical UIPIPF presentation, whereas if the presentation is atypical, surgical lung biopsy is required to confirm the diagnosis. Bronchoscopy and percutaneous lung aspiration biopsy are not useful for the diagnosis of IPF, but can be used as the basis for differential diagnosis. Zhang Meichun, Department of Respiratory Medicine, Guangzhou First People’s Hospital, Guangzhou, China To date, the etiology of IPF has not been clearly defined, and the mechanism of its fibrosis is also poorly understood, coupled with the fact that many secondary non-interstitial fibrosis can have similar manifestations, which leads to the diagnosis of IPF is quite difficult, and the identification is complicated; coupled with the fact that there is a single means of treatment, with limited choices of medications and the efficacy of the drugs is not yet certain, and the evaluation index of the efficacy of the therapy is still limited, and most of them are only evaluated based on CT imaging changes. The evaluation index of the efficacy is still limited, most of them are only evaluated according to the CT imaging changes, and no effective and certain evaluation factor has been found yet. So far, the prognosis of IPF is still unsatisfactory, and the survival rate after diagnosis is usually around 3 years, and at most not more than 5 years. It can be said that IPF is a benign non-invasive lesion, but it is not possible to obtain radical treatment and reversal, which is called “modern cancer that can be treated but not cured”, all of which have been plagued by respiratory physicians, so that respiratory physicians are helpless, at a loss, full of inexpressible acid and pain. Happily, recent studies have shown that pirfenidone can significantly improve patients’ symptoms and quality of life, and significantly prolong patients’ survival, although it still cannot reduce their mortality. I believe that one day mankind will be able to finally defeat IPF!