Usual interstitial pneumonia (UIP) refers to a combination of radiologic and histologic findings, which include patchy interstitial fibrosis with fibroblastic foci and dense acellular collagen that causes architectural distortion due to scarring and honeycomb change with alternating areas of normal lungs. The UIP pattern is not a synonymous term with idiopathic pulmonary fibrosis (IPF). IPF is diagnosed when an etiologic workup has been performed, deemed to be unrevealing, with a radiologic or histologic UIP pattern. While the 2018 American Thoracic Society (ATS)/European Respiratory Society (ERS) guideline categories of UIP help eliminate the need for surgical lung biopsy (SLB) in two categories, i.e., \"definite UIP\" and \"probable UIP,\" when characterizing a patient in the other categories, clinicians should wary about prolonging SLB in patients to determine the fibrosis pattern. Changes in the treatment and overall prognosis of patients can occur due to SLB confirming a UIP pattern on histology. Here, we report the case of a patient with an indeterminate UIP pattern on high-resolution computed tomography (HRCT) with histopathologic diagnosis of UIP on SLB. With no underlying identifiable cause for the UIP pattern, the patient was diagnosed and managed as IPF, ultimately requiring lung transplantation. This case highlights the importance of pursuing surgical lung biopsy in patients with indeterminate UIP on HRCT scanning to facilitate prompt treatment and guide further management.

An 87-year-old man presented with dyspnea. Computed tomography revealed progressive subpleural consolidation in the apex, reticular shadows in the lower lobes, and bilateral ground glass opacifications. He died of respiratory failure on day 3. The post-mortem examination showed exudative stage diffuse alveolar damage and pulmonary edema. Intraalveolar collagenous fibrosis and subpleural elastosis were observed in the upper lobes, accompanied by interlobular septal and pleural thickening and lung architecture remodeling in the lower lobes. He was diagnosed with acute exacerbation of pleuroparenchymal fibroelastosis with lower lobe usual interstitial pneumonia, which can be fatal.

Idiopathic pulmonary fibrosis (IPF) is a severe progressive fibrotic disease of the lung. Its etiology is not yet completely clear. The affected population is exclusively older than 40 years with maximum incidence in the age categories of 60 to 70 years. Its worldwide prevalence varies from 2 to 29/100 000 people (in the Czech Republic 5-6/100 000). Annual incidence is constantly rising, mainly thanks to the ever-improving diagnostic possibilities. Untreated IPF disease causes rapid structural and functional devastation of the lungs with development of respiratory insufficiency and death of the patient within 2 to 3 years after diagnosis, prognosis with IPF is therefore fully (without any exaggeration) comparable to untreated bronchogenic carcinoma. In recent years, the prognosis and quality of life of patients have significantly improved thanks to available specific antifibrotic treatment, which can substantially slow down the disease progression and thus prolong survival. However, a necessary condition for the timely treatment initiation is a quick and accurate diagnosis. The following case report describes a protracted journey to the correct diagnosis in a patient with atypical radiological findings, so that the definitive diagnosis was established only as a result of a surgical lung biopsy.

Dendriform pulmonary ossification (DPO) is a rare condition characterized by metaplastic bone formation in the lung parenchyma. It has been reported to be often associated with primary lung diseases, such as usual interstitial pneumonia (UIP) or chronic aspiration of gastric acid; however, its clinical features and pathophysiology remain unclear, especially in idiopathic cases. We herein report five DPO cases, including three with an idiopathic origin. In all cases of idiopathic DPO, the pathological and radiological examinations showed localized pulmonary lesions suggesting inflammation or hemorrhaging.

Objectives: Interstitial lung disease (ILD) is an extra-articular manifestation in rheumatoid arthritis (RA), detected in 10.7% of patients, and causing a poor prognosis. Hence, biomarkers for ILD are urgently required in RA. Low molecular weight metabolites can be assessed by metabolomic analyses, and although these have been conducted in RA and in idiopathic pulmonary fibrosis, few have been carried out for ILD in the context of RA. Therefore, we analyzed serum metabolomic profiles of ILD in RA to identify novel biomarkers. Methods: Serum samples from 100 RA patients with ILD and 100 matched RA patients without chronic lung disease (CLD) were collected. These samples were subjected to metabolomic analyses using capillary electrophoresis time-of-flight mass spectrometry. Results: A total of 299 metabolites were detected in the metabolomic analysis. By univariate analysis, serum levels of decanoic acid and morpholine were lower in RA with ILD (false discovery rate Q = 1.87 × 10-11 and 7.09 × 10-6, respectively), and glycerol was higher (Q = 1.20 × 10-6), relative to RA without CLD. Serum levels of these metabolites in RA with usual interstitial pneumonia or RA with non-specific interstitial pneumonia were also altered. The partial least squares-discriminant analysis model generated from these three metabolites could successfully discriminate ILD in RA (area under the curve: 0.919, 95% confidence interval: 0.867-0.968, sensitivity 0.880, specificity 0.780). Conclusions: Serum levels of some metabolites were significantly different in RA with ILD compared with RA without CLD. It is concluded that metabolomic profiling will be useful for discovering candidate screening biomarkers for ILD in RA.

BACKGROUND: Short telomeres are recognized as risk factor for idiopathic pulmonary fibrosis (IPF). We aimed to assess the role of telomere length (TL) in fibrotic-Interstitial Lung Diseases (f-ILDs) associated with a usual interstitial pneumonia (UIP) pattern as well as in IPF acute exacerbation (IPF-AE).
OBJECTIVE: TL was measured from peripheral white blood cells using a multiplex quantitative polymerase chain reaction in consecutive patients with f-ILDs, all presenting UIP pattern in the high-resolution chest-computed-tomography and compared to age-matched healthy controls.
RESULTS: Seventy-nine individuals were included (mean age 69.77 ± 0.72 years); 24 stable IPF, 18 IPF-AE, 10 combined pulmonary fibrosis and emphysema, 7 Rheumatoid arthritis-UIP-ILDs and 20 controls. TL in all patients was significantly shorter compared to controls [mean T/S ratio (SE) 0.77 (±0.05) vs 2.26 (±0.36), p < 0.001] as well as separately in each one of f-ILD subgroups. IPF-AE patients presented significantly shorter TL compared to stable IPF (p = 0.029). Patients with IPF and shorter than the median TL (0-0.72) showed reduced overall survival (p = 0.004). T/S < 0.72 was associated with increased risk for IPF-AE (OR = 30.787, 95% CI: 2.153, 440.183, p = 0.012) independent of age, gender, smoking and lung function impairment. A protective effect of TL was observed, as it was inversely associated with risk of death both in UIP-f-ILDs (HR = 0.174, 95%CI: 0.036, 0.846, p = 0.030) and IPF patients (HR = 0.096, 95%CI: 0.011, 0.849, p = 0.035).
CONCLUSIONS: Shorter TL characterizes different UIP f-ILDs. Although no difference was observed in TL among diverse UIP subgroups, IPF-AE presented shorter TL compared to stable IPF. Reduced overall survival and higher hazard ratio of death are associated with shorter TL in IPF.

We herein report a case of asymmetrical interstitial lung disease (ILD) that remained almost completely asymmetrical over time on chest computed tomography (CT). An open lung biopsy from the right lung showed severe pleural adhesion, obstruction of the pulmonary artery, and dilated systemic arteries in addition to the usual interstitial pneumonia pattern. Three-dimensional CT angiography showed partial defects of pulmonary arteries on the affected side. After excluding other known causes of ILD and gastroesophageal reflux, we suspected that decreased pulmonary artery perfusion in the present case may have been responsible for the observed asymmetrical unilateral fibrosis.

A 78-year-old man who had worked in the building industry visited our hospital because of groundglass opacity with smoothly thickened, intralobular interstitial lines and interlobular septal lines on chest high-resolution computed tomography (HRCT). HRCT image also showed a focal area of reticulation and pleural thickening. Lung specimens obtained by surgical lung biopsy showed accumulations of intra-alveolar periodic acid-Schiffpositive materials, usual interstitial pneumonia (UIP)-like subpleural lung fibrosis and asbestos bodies (1 body/cm2 in high-power field, ×400). Serum granulocyte-macrophage colony stimulating factor autoantibody was positive. The patient was diagnosed as having autoimmune pulmonary alveolar proteinosis (PAP) and needed differential diagnosis from secondary PAP caused from pulmonary asbestosis and UIP. Careful observation of the manifestations of pulmonary asbestosis and the progression of fibrosis using HRCT will be necessary in this patient.

Diffuse parenchymal lung diseases (DPLD), also known as interstitial lung diseases (ILD), are a group of lung disorders affecting alveolar epithelium, pulmonary capillary endothelium, and surrounding lung tissue. Over time due to injury, the tissue around the air sacs becomes fibrotic leading to poor oxygen exchange, eventually resulting in the patient experiencing shortness of breath. This case describes a 69-year old male who presented in 2017 with a complex clinical picture involving both cardiac and pulmonary systems. Drug toxicity was initially thought to be the cause of the patients interstitial lung process; however, ultimately, a diagnosis of UIP was made.

Idiopathic pulmonary fibrosis (IPF) is the most common of the idiopathic interstitial pneumonias. The high-resolution tomography (HRCT) is highly recommended among patients with suspected IPF. Usual interstitial pneumonia (UIP) is the histopathological and radiological pattern of IPF. IPF is diagnosed if the appropriate combination of HRCT patterns and histopathological patterns are present.
METHODS: The authors present a case of a 49-years-old woman who was hospitalised due to the detection of a focal lesion in the left lung in an X-ray examination. A CT scan of the chest with contrast revealed a solid infiltration of the same area. The histopathology from a transbronchial lung biopsy from this abnormality showed microscopic honeycomb, fibroblast fibrosis, macrophage clusters loaded with hemosiderin and widened bronchioles - an image suggesting Usual Interstitial Pneumonia (UIP). Following that, a HRCT showed considerable differences in the lung image compared to the previous CT examination - no polycyclic lesion in the left lung was detected and the fibrous changes decreased. The patient had substantial formal features of UIP from the histopathological examination from TBLB. Therefore, there was a clinicalradiological discrepancy in the histopathology report. After a multidisciplinary consultation by renowned experts in pulmonology, radiology and histopathology, regardless of the patient\'s diagnostic path, the primary cause of the disease could not be determined. The analysis of this case strongly suggests that the histopathological examination alone is not sufficient in the diagnosis of IPF.