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The classification interstitial pneumonia with autoimmune features (IPAF) includes patients with interstitial lung disease (ILD) associated with autoimmune characteristics insufficient to reach classification criteria for a specific autoimmune disease (SAD). These criteria are divided into three domains: clinical, serological and morphological. The latter domain does not include the usual interstitial pneumonia (UIP) pattern, which is deemed not to be significantly associated with SAD. Therefore, the enrolment of these patients is more difficult, requiring at least one item from both of the other domains. The objective of this study is to evaluate the rate of progression towards SAD of a cohort of UIP patients satisfying only one IPAF domain (we called this group "UIPAF") compared with classic idiopathic pulmonary fibrosis (IPF).We prospectively enrolled IPF patients with radiologic and/or histologic UIP pattern, followed jointly by rheumatologists and pulmonologists from January 2017 to January 2021, with a minimum follow-up of 12 months.We enrolled 190 IPF patients, 38 (20%) of whom were classified as UIPAF. IPF and UIPAF patients were similar for general characteristics, severity and prognosis, at presentation and at annual check-up. However, 28.9% of UIPAF patients progressed towards SAD, compared with 2% of IPF patients (χ2=30.4, p≤0.0001).The association between a single clinical or serological domain of IPAF and UIP pattern is predictive for the development of a SAD if compared with isolated UIP. ILD can be the first manifestation of SAD, even with a UIP pattern, therefore, the morphological domain of IPAF criteria could be removed.

Interstitial lung diseases (ILDs) comprise a wide group of pulmonary parenchymal disorders. These patients may experience acute respiratory deteriorations of their respiratory condition, termed “acute exacerbation” (AE). The incidence of AE-ILD seems to be lower than idiopathic pulmonary fibrosis (IPF), but prognosis and prognostic factors are largely unrecognized. We retrospectively analyzed a cohort of 158 consecutive adult patients hospitalized for AE-ILD in two Italian university hospitals from 2009 to 2016. Patients included in the analysis were divided into two groups: non-IPF (62%) and IPF (38%). Among ILDs included in the non-IPF group, the most frequent diagnoses were non-specific interstitial pneumonia (NSIP) (42%) and connective tissue disease (CTD)-ILD (20%). Mortality during hospitalization was significantly different between the two groups: 19% in the non-IPF group and 43% in the IPF group. AEs of ILDs are difficult-to-predict events and are burdened by relevant mortality. Increased inflammatory markers, such as neutrophilia on the differential blood cell count (HR 1.02 (CI 1.01–1.04)), the presence of pulmonary hypertension (HR 1.85 (CI 1.17–2.92)), and the diagnosis of IPF (HR 2.31 (CI 1.55–3.46)), resulted in negative prognostic factors in our analysis. Otherwise, lymphocytosis on the differential count seemed to act as a protective prognostic factor (OR 0.938 (CI 0.884–0.995)). Further prospective, large-scale, real-world data are needed to support and confirm the impact of our findings.

Introduction: Severe morning stiffness with painful involvement of the girdles are often referred by patients with Interstitial Lung Disease (ILD), but the association between ILD and Polymyalgia Rheumatica (PMR) is rarely reported. The purpose of the work is to describe a series of patients classified as having PMR with ILD. Material and methods: We retrospectively enrolled patients with a diagnosis of PMR referred to our center during the previous year for respiratory symptoms. Data concerning clinical and serological manifestations suggesting Connective Tissue Disease (CTD), High-Resolution Chest Tomography (HRCT), and Pulmonary Function Tests (PFTs) were systematically collected in order to verify the diagnosis. Results: Fifteen out of seventeen PMR patients had ILD. Ten patients had a confirmed diagnosis of PMR, while in five patients a CTD was discovered. Seven patients showed a severe restrictive pattern at PFTs requiring oxygen supplementation (five with PMR and two with CTD). In thirteen patients pulmonary symptoms started before or together with muscular symptoms. Regarding HRCT patterns, patients showed a Nonspecific Interstitial Pneumonia in nine cases, Usual Interstitial Pneumonia (UIP) and possible UIP in two and three cases, and a single case of Organizing Pneumonia and Combined Pulmonary Fibrosis and Emphysema Syndrome. Conclusions: Lung involvement should be evaluated in PMR patients, especially if asthenia is poorly responsive to low doses of steroids. In these cases, the diagnosis should be re-evaluated in depth, looking for a seronegative Rheumatoid Arthritis, a clinically amyopathic myositis or Interstitial Pneumonia with Autoimmune features. Keywords: Interstitial lung disease, Polymyalgia rheumatica, Connective tissue disease, Antisynthetase syndrome, Myositis, Interstitial pneumonia with autoimmune features

Patient-reported outcome measures (PROMs), tools to assess patient self-report of health status, are now increasingly used in research, care and policymaking. While there are two well-developed disease-specific PROMs for interstitial lung diseases (ILD) and idiopathic pulmonary fibrosis (IPF), many unmet and urgent needs remain. In December 2019, 64 international ILD experts convened in Erice, Italy to deliberate on many topics, including PROMs in ILD. This review summarises the history of PROMs in ILD, shortcomings of the existing tools, challenges of development, validation and implementation of their use in clinical trials, and the discussion held during the meeting. Development of disease-specific PROMs for ILD including IPF with robust methodology and validation in concordance with guidance from regulatory authorities have increased user confidence in PROMs. Minimal clinically important difference for bidirectional changes may need to be developed. Cross-cultural validation and linguistic adaptations are necessary in addition to robust psychometric properties for effective PROM use in multinational clinical trials. PROM burden of use should be reduced through appropriate use of digital technologies and computerised adaptive testing. Active patient engagement in all stages from development, testing, choosing and implementation of PROMs can help improve probability of success and further growth.

The thoraco-lumbar bracing is an effective management of adolescent idiopathic scoliosis (AIS). Studies have shown that brace wearing reduces lung volume. Whether or not the Sforzesco brace, frequently used in Italy, affects lung volume has not been investigated. We studied the immediate effect of Sforzesco bracing on lung volumes in 11 AIS patients (10 F, 1 M, aged 13.6 ± 1.6 yrs) mean Cobb angle 26 ± 4.49 degrees. Lung function variables and the perceived respiratory effort were recorded twice, before and 5 min after bracing. The one-way analysis of variance repeated measures, and multiple comparison tests, showed that means of unbraced variables were not significantly different from the corresponding means of predicted values, whereas means under brace were significantly lower (p &lt, 0.05) compared to both predicted and baseline values of respiratory variables. In addition, a significant correlation (p &lt, 0.0001) was found between unbraced and braced values, and linear regression equations were calculated. A significant but clinically unimportant increase in perceived effort was observed under the brace. In conclusion, data indicate that lung function is not impaired in moderate AIS and that wearing the Sforzesco brace causes an immediate, predictable reduction of lung volumes. Data also suggest that the respiratory discomfort during brace wearing could not be due to respiratory function defects.

Idiopathic pulmonary fibrosis (IPF) is a fibrotic lung disease with uncertain origins and pathogenesis, it represents the most common interstitial lung disease (ILD), associated with a pathological pattern of usual interstitial pneumonitis (UIP). This disease has a poor prognosis, having the most lethal prognosis among ILDs. In fact, the progressive fibrosis related to IPF could lead to the development of complications, such as acute exacerbation, lung cancer, infections, pneumothorax and pulmonary hypertension. Pneumologists, radiologists and pathologists play a key role in the identification of IPF disease, and in the characterization of its complications&mdash, which unfortunately increase disease mortality and reduce overall survival. The early identification of these complications is very important, and requires an integrated approach among specialists, in order to plane the correct treatment. In some cases, the degree of severity of patients having IPF complications may require a personalized approach, based on palliative care services. Therefore, in this paper, we have focused on clinical and radiological features of the complications that occurred in our IPF patients, providing a comprehensive and accurate pictorial essay for clinicians, radiologists and surgeons involved in their management.

The aim of this work was to verify the correlations between different pulmonary morphological patterns and functional outcomes in sarcoidosis patients, using a validated score for the comparison between the high-resolution computed tomography (HRCT) of patients belonging to different imaging patterns. From the electronic database of the reference center for interstitial lung diseases of our University Hospital, we retrospectively selected 55 patients with a diagnosis of sarcoidosis according to the American Thoracic Society (ATS) criteria, we evaluated the initial HRCT examination and pulmonary function tests collected at baseline and after a year. Patients were divided into typical (48% of patients) and atypical (52%) HRCT patterns, and a computer tomography activity score (CTAS) was associated with each HRCT appearance detected, clinical history, impact of therapy, and extra-thoracic locations were also considered. We found that worsening of diffusing capacity for carbon monoxide (DLCO) is related to the CTAS (r = &minus, 0.20, p = 0.01), and there was an inverse correlation between the variation of forced vital capacity (FVC) and the value of the CTAS (r = &minus, 0.30, p = 0.23) in the subgroup of patients with atypical patterns. CTAS were higher in patients with extra-pulmonary localizations (p = 0.05) and the subgroup of patients with extra-thoracic locations and atypical manifestations had a greater worsening in terms of variation of FVC (p = 0.03) and DLCO% (p = 0.04). No difference between treated and untreated patients was found.

Interstitial Lung Diseases (ILDs) represent a heterogeneous group of pathologies, which may be related to different causes. A low percentage of these lung diseases may be secondary to the administration of drugs or substances. Through the PubMed database, an extensive search was performed in the fields of drug toxicity and interstitial lung disease. We have evaluated the different classes of drugs associated with pulmonary toxicity. Several different high resolution computed tomography (HRCT) patterns related to pulmonary drug toxicity have been reported in literature, and the most frequent ILDs patterns reported include Nonspecific Interstitial Pneumonia (NSIP), Usual Interstitial Pneumonia (UIP), Hypersensitivity Pneumonitis (HP), Organizing Pneumonia (OP), Acute Respiratory Distress Syndrome (ARDS), and Diffuse Alveolar Damage (DAD). Finally, from the electronic database of our Institute we have selected and commented on some cases of drug-induced lung diseases related to the administration of common drugs. As the imaging patterns are rarely specific, an accurate evaluation of the clinical history is required and a multidisciplinary approach—involving pneumologists, cardiologists, radiologists, pathologists, and rheumatologists—is recommended.

To evaluate the radiological findings in patients with cryptogenic organizing pneumonia (COP) before steroid treatment and their behavior after therapy, we retrospectively evaluated a total of 22 patients with a diagnosis of COP made by bronchoalveolar lavage (BAL), biopsy or clinical/radiological features, and the patients were followed between 2014 and 2018 at the hospital, the demographic data, symptoms, radiologic findings, diagnostic methods and treatment plans of patients were collected from patients&rsquo, hospital records. At least two CT scans of 22 patients (16 female and six men) were evaluated, the first one before starting steroid therapy and the others after therapy. At baseline CT scans, the most common radiological finding was the presence of consolidations (18/22 patients, 81.8%), ground-glass opacities were also very common (15/25, 68.1%). The other findings were as follows: nodules and masses (5/22, 22.7%), atoll sign (4/22, 18.1%), perilobular pattern (3/22, 13.6%) and parenchymal bands (3/22, 13.6%). Two patients had a significant relapse after reducing/interrupting therapy, while three had a complete resolution and are not currently under therapy (maintenance of clinical remission with no oral corticosteroid (OCS)). In High-resolution computed tomography (HRCT) scans after therapy, consolidations were still observable in seven patients (five in new areas of the lung-migratory infiltrates), while most of them disappeared, leaving a residual area of ground glass opacity in two patients. One patient had a residual of the perilobular pattern, with the disappearing of the other findings (consolidations and ground-glass opacities). Two patients developed a fibrosing pattern despite the therapy (9.5%). Cryptogenic organizing pneumonia tends to respond to oral corticosteroid treatment, but some patients may have a null or partial response. We highlight the behavior of this disease after proper therapy.

Purpose The purpose of this study was to investigate histogram-based quantitative high-resolution computed tomography (HRCT) indexes in the assessment of lung cancer (LC) development in idiopathic pulmonary fibrosis (IPF) patients. Materials and methods From IPF databases of 2 national respiratory centers, we retrospectively studied patients with and without LC development-respectively, divided into Group A (n=16) and Group B (n=33). The extent of fibrotic disease was quantified on baseline and follow-up HRCT examinations using kurtosis, skewness, percentage of high attenuation area (HAA%), and percentage of fibrotic area (FA%). These indexes were compared between the 2 groups using the Mann-Whitney U test. In the prediction of LC development, receiver operating characteristic analysis was performed to assess threshold values of HRCT indexes. Results At baseline, no difference was reported among groups for kurtosis, skewness, HAA%, and FA%, with P-values of 0.0881, 0.0606, 0.0578, and 0.0606, respectively. On follow-up, significant differences were reported, with P-values of 0.0174 for kurtosis, 0.0313 for skewness, 0.0297 for HAA%, and 0.0407 for FA%.On baseline HRCT, in the prediction of LC development, receiver operating characteristic analysis reported sensibility and specificity of 87.5% and 45.45% for kurtosis, 68.75% and 63.64% for skewness, 81.25% and 54.55% for FA%, and 75% and 60.61% for HAA%. Conclusions LC development is associated with progression of fibrosis; at baseline, FA% and HAA% reported more convenient sensitivity/specificity ratios in the prediction of LC development.

Background and objective In clinical practice, a working diagnosis of IPF may be performed to provide effective antifibrotic treatment to patients who cannot undergo SLB. In this study, we compared the disease course across IPF diagnostic categories in a real-life clinical setting to clarify the appropriateness of a working diagnosis of IPF and treatment initiation in these patients. Methods Longitudinal data from IPF patients receiving antifibrotic treatment (pirfenidone or nintedanib) were retrospectively collected at three tertiary centres in Italy. Univariate and multivariate analyses were performed to compare time to death and to a composite endpoint of disease progression between two diagnostic subgroups, that is, patients with UIP on HRCT and/or SLB, and patients with possible UIP and no histological confirmation. Results A total of 249 IPF patients were included in the analysis. Among patients with a possible UIP pattern on HRCT, 41 (55%) were prescribed antifibrotic treatment (either nintedanib or pirfenidone) despite absence of histological confirmation. This group demonstrated similar mortality and disease progression as compared to patients with a definite diagnosis of IPF as per diagnostic guidelines (log-rank test P = 0.771 and P = 0.139, respectively). Such findings were confirmed on multivariate analysis (HR: 1.19, 95% CI: 0.49-2.89, P = 0.7 for death; HR: 1.42, 95% CI: 0.83-2.44, P = 0.201 for disease progression). Conclusion In patients receiving antifibrotics following a working diagnosis of IPF, disease progression rates were similar to patients with a confident diagnosis of IPF according to consensus guidelines, supporting the rationale for treatment initiation in these patients by expert multidisciplinary teams.

Background: Interstitial lung disease (ILD) is a frequent manifestation of Sjögren's syndrome (SS), an autoimmune disease of salivary and lacrimal glands, and affects approximately 20% of patients. No clinical or serological features appear to be useful to predict its presence, severity or progression, and chest high-resolution computed tomography (CT) remains the gold standard for diagnosis. Semiquantitative CT (SQCT) based on visual assessment (Goh and Taouli scoring) can estimate ILD extent, although it is burdened by relevant intra- and interobserver variability. Quantitative chest CT (QCT) is a promising alternative modality to assess ILD severity. Aim: To determine whether QCT assessment can identify extensive or limited lung disease in patients with SS and ILD. Methods: This multi-center, cross-sectional and retrospective study enrolled patients with SS and a chest CT scan. SQCT assessment was carried out in a blinded and centralized manner to calculate both Goh and Taouli scores. An operator-independent analysis of all CT scans with the open-source software platform Horos was used to evaluate the QCT indices. Patients were classified according to the extent of ILD and differences in QCT index distribution were investigated with non-parametric tests. Results: From a total of 102 consecutive patients with SS, the prevalence of ILD was 35.3% (36/102). There was a statistically significant difference in QCT index distribution between the SS with ILD and SS without ILD groups (p20% (by Goh score) had a QCT index statistically different from those with limited ILD extent (p

A cyst is a round circumscribed area of low attenuation, surrounded by epithelial or fibrous wall. Cysts can frequently occur on chest computed tomography (CT) and high-resolution computed tomography (HRCT); multiple parenchymal cysts of the lungs are the most typical feature of cystic lung interstitial diseases, characterizing a wide spectrum of diseases—ranging from isolated lung disorders up to diffuse pulmonary diseases. The aim of this review is to analyze scientific literature about cystic lung interstitial diseases and to provide a practical guide for radiologists, focusing on the main morphological features of pulmonary cysts: size, shape, borders, wall, location, and distribution. These features are shown on free-hand drawings and related to HRCT images, in order to help radiologists pursue the correct differential diagnosis between similar conditions.

Background The term Interstitial Pneumonia with Autoimmune Features (IPAF) describes patients with Interstitial Lung Diseases (ILDs) and clinical or serological features of autoimmune diseases insufficient to reach a specific classification of a Connective Tissue Disease (CTD). Currently, retrospective studies on IPAF patients have proven to be heterogeneous in general characteristics, outcomes and High-Resolution Computed Tomography (HRCT) pattern. This study aims to describe for the first time the clinical, serological and radiological features of a prospective cohort of IPAF patients. This cohort is then compared to a group of patients with Idiopathic Pulmonary Fibrosis (IPF). Material and methods From 626 consecutive ILD patients evaluated, 45 IPAF and a comparison cohort of 143 IPF patients were enrolled. All patients underwent clinical assessment with rheumatologic and respiratory evaluation, HRCT, Pulmonary Function Tests and Nailfold Videocapillaroscopy. Results The IPAF patients had a predominance of female gender (62.12%) with a median age of 66 years. The most common findings were: Nonspecific Interstitial Pneumonia (NSIP, 68.89%), Antinuclear Antibody positivity (17.77%) and Raynaud Phenomenon (31.11%). In comparison with IPF, IPAF patients showed younger age, better performances in Pulmonary Function Tests, less necessity of O2 support and predominance of female sex and NSIP pattern. Discussion This is the first report of a prospective cohort of IPAF patients. IPAF patients seem to have a less severe lung disease than IPF. IPAF criteria probably need to be revisited and validated, but their capacity to recruit patients with incomplete forms or early onset of CTD could be useful for further research.

Background: correlation between pulmonary function tests (PFTs) and CT-histogram derived indexes is poorly known in Idiopathic Pulmonary Fibrosis (IPF). Objective: To investigate correlation between PFTs (FVC and DLCO) and CT-histogram derived indexes in a cohort of patients (pts) with IPF. Methods: 42 IPF pts having at least two HRCT (baseline and follow-up) and two PFTs available collected no more than 1 month from HRCT evaluation were analyzed. The extent of fibrotic disease was quantified on HRCT examinations calculating Kurtosis, High Attenuation Area (HAA) and Fibrotic Area (FA) indexes. Pearson’s correlation coefficient was performed to assess the strength of association between pulmonary function tests and HRCT indexes. Results: Considering baseline HRCT and PFTs, Pearson correlation analysis revealed moderate correlations between Kurtosis and FVC (r=0.58 with p=0.0001), Kurtosis and DLCO (r=0.52 with p=0.0005), HAA and FVC (r=-0.56 with p=0.0001), HAA and DLCO (r=-0.48 with p=0.0017), FA and FVC (r=-0.56 with p=0.0002), and FA and DLCO (r=-0.49 with p=0.0013). On follow-up, analyzing HRCT and PFTs, Kurtosis and FVC showed a positive strong correlation (r=0.62 with p Conclusions: HRCT quantification indexes showed a moderate or good strength of association with pulmonary function tests; therefore, they may be useful for radiologists in the monitoring of IPF disease.

Introduction: The term Interstitial Pneumonia with Autoimmune Features (IPAF) has been proposed to classify patients with Interstitial Lung Disease (ILD) associated with autoimmune signs not classifiable for a specific Connective Tissue Disease (CTD). Currently, no prospective IPAF cohort are reported. Aims and Objectives: To describe a prospective cohort of IPAF patients. Methods: The study was conducted according to the declaration of Helsinki. A total of 170 consecutive ILD patients with at least one IPAF criteria were considered in the last year (Fischer A, Eur Respir J 2015; 46 (4): 976-987). From these, 58 patients were able to satisfy classification criteria for CTDs, while 33 patients were classified as IPAF. Results: IPAF patients (69.7% females) referred a history of smoke in 51.5% of cases and reported a restrictive pattern in Pulmonary Function Tests in 66.7% of cases. A clinical domain was present in 72.7% of patients, whereas serological and morphological domains respectively in 72.7% and 87.9% of cases. The most frequent item from each domain were arthritis (42.4%), Antinuclear Antibodies (ANA) (30.3%) and Nonspecific Interstitial Pneumonia (75.8%) as HRCT pattern. No cases of ulcers, telangiectasia and mechanic’s hand were reported. Only ANA, Rheumatoid Factor, anti-SSA, anti DsDNA and Anti Citrullinated Protein Antibodies were found as serological domain. Conclusions: This study suggests that most of IPAF patients have a clinically relevant ILD without serological and clinical items highly specific for CTDs. When these items were present, patients were generally able to satisfy specific CTD criteria. Probably, some of IPAF patients can have an early form of CTDs.

Background: the predictive role of CT-histogram indexes in assessing survival was poorly studied in IPF. Objective: To investigate the accuracy of CT-histogram derived indexes in the assessment of survival in IPF patients (pts). Methods: 42 IPF pts with a follow-up time of 3 years were retrospectively collected; HRCT and pulmonary function tests (FVC and DLCO) performed at diagnosis were analyzed; the extension of fibrosis was quantified on HRCT using Kurtosis, Skewness, Mean Lung Density (MLD), High Attenuation Areas (HAA%) and Fibrotic Areas (FA%). Univariate Cox-regression analysis assessed Hazard ratios for each variable. A Multivariate model including Skewness, FVC, DLCO and age tested their prognostic value in assessing survival. Through ROC analysis, threshold values demonstrating the best sensitivity and specificity in predicting mortality were identified and used as cut-off points to graph Kaplan-Meier curves specific for the CT-indexes. Results: Kurtosis, Skewness, MLD, HAA% and FA% were good predictors of mortality (HR 0.44, 0.74, 1.01, 1.12, 1.06; p=0.03, p=0.01, p=0.02, p=0.02 and p=0.017 respectively). Skewness, which demonstrated the lowest AIC value (55.52), was considered the best analyzed CT-index in predicting mortality. Significant survival differences considering proposed cut-off points were also demonstrated according to Kurtosis (p=0.02), Skewness (p=0.005), MLD (p=0.003), HAA% (p=0.009) and FA% (p=0.02) – obtained from quantitative HRCT analysis at the diagnosis time. Conclusions: CT-histogram derived indexes may provide an accurate estimation of survival in a population of IPF patients since the time of diagnosis.

The term interstitial pneumonia with autoimmune features (IPAF) has been proposed to define patients with interstitial lung disease (ILD) associated with autoimmune signs not classifiable for connective tissue diseases (CTDs). This new definition overcomes previous nomenclatures and provides a uniform structure for prospective studies through specific classification criteria.This work evaluates the characteristics of IPAF patients reported in the literature, to highlight potential limits through a comparative analysis and to suggest better performing classification criteria.Four retrospective studies on the IPAF population have been considered. The study subjects differed in age, sex, smoking habit, ILD pattern and outcomes. Another important difference lies in the diverse items considered in the classification criteria. The retrospective design of the studies and the absence from some of them of a rheumatologist clearly involved in the diagnosis may have influenced the data, but current IPAF criteria seem to include a rather heterogeneous population. To overcome these discrepancies, this review suggests a limitation in the use of single items and the exclusion of extremely specific CTD criteria. This should avoid the definition of IPAF for those diseases at different stages or at early onset. The investigation of a functional or morphological cut-off of pulmonary involvement would be useful.

Idiopathic Pulmonary Fibrosis (IPF) is a severe parenchymal lung disease characterized by an intense deposition of collagen in the interstitial spaces. The introduction of anti-fibrotic drugs increased patients' life expectancy highlighting the role of comorbidities in patients' management and prognosis. IPF is frequently associated with other diseases mainly because of its onset during middle age and sometimes because of the presence of common pathogenic pathways such as in the case of lung cancer. Comorbidities may differently influence prognosis of IPF patients. However, except for major impacting ones as LC, PH and cardiovascular diseases, data exploring their impact on prognosis are still few and sometimes conflicting highlighting the need of new large and targeted studies. In this review we discuss the current knowledge on the most common comorbidities associated with IPF (cardiovascular diseases, pulmonary hypertension, lung cancer, emphysema, gastro-oesophageal reflux and depression), focusing on their prognostic role.