"Usual" interstitial pneumonia with autoimmune features: a prospective study on a cohort of idiopathic pulmonary fibrosis patients.

OBJECTIVES: 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). METHODS: 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. RESULTS: 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). CONCLUSIONS: 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.

Disease progression across the spectrum of idiopathic pulmonary fibrosis: A multicentre study.

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.

Comorbidities of IPF: How do they impact on prognosis.

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.

Histogram-based analysis in progressive pulmonary fibrosis: relationships between pulmonary functional tests and HRCT indexes.

OBJECTIVES: To investigate relationships between histogram-based high-resolution CT (HRCT) indexes and pulmonary function tests (PFTs) in interstitial lung diseases. METHODS: Forty-nine patients having baseline and 1-year HRCT examinations and PFTs were investigated. Histogram-based HRCT indexes were calculated; strength of associations with PFTs was investigated using Pearson correlation. Patients were divided into progressive and non-progressive groups. HRCT indexes were compared between the two groups using the U-test; within each group, baseline and follow-up Wilcoxon analysis was performed. Receiver operating characteristic analysis was used for predicting disease progression. RESULTS: At baseline, moderate correlations were observed considering kurtosis and diffusion capacity of the lungs for carbon monoxide (DLCO) (r = 0.54) and skewness and DLCO (r = 0.559), whereas weak but significant correlations were observed between forced vital capacity and kurtosis (r = 0.368, p = 0.009) and forced vital capacity and skewness (r = 0.391, p = 0.005). Negative correlations were reported between HAA% and PFTs (from r = -0.418 up to r = -0.507). At follow-up correlations between quantitative indexes and PFTs were also moderate, except for high attenuation area (HAA)% -700 and DLCO (r = -0.397). In progressive subgroup, moderate and strong correlations were found between DLCO and HRCT indexes (r = 0.595 kurtosis, r = 0.672 skewness, r=-0. 598 HAA% -600 and r = -0.626 HAA% -700). At follow-up, we observed significant differences between the two groups for kurtosis (p = 0.029), HAA% -600 (p = 0.04) and HAA% -700 (p = 0.02). To predict progression, ROC analysis reported sensitivity of 90.9% and specificity of 51.9% using a threshold value of δ kurtosis <0.03. CONCLUSION: At one year, moderate correlations suggest that progression could be assessed through HRCT quantification. ADVANCES IN KNOWLEDGE: This study promotes histogram-based HRCT indexes in the assessment of progressive pulmonary fibrosis.

A New Method for the Assessment of Myalgia in Interstitial Lung Disease: Association with Positivity for Myositis-Specific and Myositis-Associated Antibodies.

In this study, it was found that myositis-specific and myositis-associated antibodies (MSAs and MAAs) improved the recognition of idiopathic inflammatory myopathies (IIMs) in interstitial lung disease (ILD) patients. The objective of this study is to propose a clinical method to evaluate myalgia in respiratory settings as a possible tool for the recognition of MSA/MAA positivity in ILD patients. We prospectively enrolled 167 ILD patients with suspected myositis, of which 63 had myalgia evoked at specific points (M+ILD+). We also enrolled in a 174 patients with only myalgia (M+ILD-) in a rheumatological setting. The patients were assessed jointly by rheumatologists and pulmonologists and were tested for autoantibodies. M+ILD+ patients were positive for at least one MAA/MSA in 68.3% of cases, as were M-ILD+ patients in 48.1% of cases and M+ILD- patients in 17.2% of cases (p = 0.01 and <0.0001, respectively). A diagnosis of IIM was made in 39.7% of M+ILD+ patients and in 23.1% of the M-ILD+ group (p = 0.02). Myalgia was significantly associated with positivity for MSA/MAAs in ILD patients (p = 0.01, X2: 6.47). In conclusion, myalgia in ILD patients with suspected myositis is associated with MSA/MAA positivity, and could support a diagnosis of IIM. A significant proportion of M+ILD- patients also had MSA/MAA positivity, a phenomenon warranting further study to evaluate its clinical meaning.

Immediate Effects of Sforzesco® Bracing on Respiratory Function in Adolescents with Idiopathic Scoliosis.

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 < 0.05) compared to both predicted and baseline values of respiratory variables. In addition, a significant correlation (p < 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.

Quantitative Evaluation of Fibrosis in IPF Patients: Meaning of Diffuse Pulmonary Ossification.

To investigate the role of diffuse pulmonary ossification (DPO) in disease severity in a population of Idiopathic Pulmonary Fibrosis (IPF) patients. This retrospective study was carried out on 95 IPF patients-44 with DPO on high resolution computed tomography (HRCT) and 51 with no calcifications detected on HRCT. Pulmonary Function Tests (PFTs) acquired nearest to the HRCT were collected. Images were analyzed by two radiologists using a qualitative method, based on HRCT fibrosis visual score, and using a quantitative method, based on histogram-based analysis. The Spearman's rank correlation coefficient was used to measure the strength and direction of the linear relationship between HRCT fibrosis score and PFTs; in addition, Spearman's rank correlation coefficient was used to explore the relationships between HRCT fibrosis score and quantitative index and between quantitative indexes and PFTs. A weak correlation between HRCT fibrosis score and PFTs was proven (r =-0.014 and p = 0.9347 for FVC (Forced Vital Capacity), r = -0.379 and p = 0.0174 for DLCO (Carbon monoxide diffusing capacity)). We found a moderate negative correlation between HRCT fibrosis score and kurtosis (r = -0.448, p = 0.004272) and skewness (r = -0.463, p = 0.003019) and a weak positive correlation with High Attenuation Area (HAA)% (r = 0.362, p = 0.0235). Moreover, a moderate linear correlation between Quantitative Indexes and FVC (r = 0.577, p = 0.000051 for kurtosis and FVC, r = 0.598, p = 0.000023 for skewness and FVC, r = -0.519, p = 0.0000364 for HAA% and FVC) and between quantitative indexes and DLCO (r = 0.469, p = 0.001508 for kurtosis, and DLCO, r = 0.474, p = 0.001309 for skewness and DLCO, r = -0.412, p = 0.005996 for HAA% and DLCO) was revealed. To better investigate the influence of DPO in disease progression, a longitudinal evaluation should be performed.

Differences between Acute Exacerbations of Idiopathic Pulmonary Fibrosis and Other Interstitial Lung Diseases.

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.

Patient-reported outcomes and patient-reported outcome measures in interstitial lung disease: where to go from here?

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.

Morphological Patterns of Sarcoidosis and Clinical Outcome: Retrospective Analysis through a Multidisciplinary Approach.

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 = -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 = -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.

Complications in Idiopathic Pulmonary Fibrosis: Focus on Their Clinical and Radiological Features.

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-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.

HRCT Patterns of Drug-Induced Interstitial Lung Diseases: A Review.

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.

Cryptogenic Organizing Pneumonia: Evolution of Morphological Patterns Assessed by HRCT.

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' 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.

Cystic Interstitial Lung Diseases: A Pictorial Review and a Practical Guide for the Radiologist.

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.

Assessment of Lung Cancer Development in Idiopathic Pulmonary Fibrosis Patients Using Quantitative High-Resolution Computed Tomography: A Retrospective Analysis.

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.

Contribution of pulmonary function tests (PFTs) to the diagnosis and follow up of connective tissue diseases.

INTRODUCTION: Connective Tissue Diseases (CTDs) are systemic autoimmune conditions characterized by frequent lung involvement. This usually takes the form of Interstitial Lung Disease (ILD), but Obstructive Lung Disease (OLD) and Pulmonary Artery Hypertension (PAH) can also occur. Lung involvement is often severe, representing the first cause of death in CTD. The aim of this study is to highlight the role of Pulmonary Function Tests (PFTs) in the diagnosis and follow up of CTD patients. MAIN BODY: Rheumatoid Arthritis (RA) showed mainly an ILD with a Usual Interstitial Pneumonia (UIP) pattern in High-Resolution Chest Tomography (HRCT). PFTs are able to highlight a RA-ILD before its clinical onset and to drive follow up of patients with Forced Vital Capacity (FVC) and Carbon Monoxide Diffusing Capacity (DLCO). In the course of Scleroderma Spectrum Disorders (SSDs) and Idiopathic Inflammatory Myopathies (IIMs), DLCO appears to be more sensitive than FVC in highlighting an ILD, but it can be compromised by the presence of PAH. A restrictive respiratory pattern can be present in IIMs and Systemic Lupus Erythematosus due to the inflammatory involvement of respiratory muscles, the presence of fatigue or diaphragm distress. CONCLUSIONS: The lung should be carefully studied during CTDs. PFTs can represent an important prognostic tool for diagnosis and follow up of RA-ILD, but, on their own, lack sufficient specificity or sensitivity to describe lung involvement in SSDs and IIMs. Several composite indexes potentially able to describe the evolution of lung damage and response to treatment in SSDs are under investigation. Considering the potential severity of these conditions, an HRCT jointly with PFTs should be performed in all new diagnoses of SSDs and IIMs. Moreover, follow up PFTs should be interpreted in the light of the risk factor for respiratory disease related to each disease.

Interstitial Lung Disease in patients with Polymyalgia Rheumatica: A case series.

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.

Clinical, serological and radiological features of a prospective cohort of Interstitial Pneumonia with Autoimmune Features (IPAF) patients.

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.

Quantitative assessment of interstitial lung disease in Sjögren's syndrome.

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 (p<0.001). Moreover, SS-ILD patients with ILD >20% (by Goh score) had a QCT index statistically different from those with limited ILD extent (p<0.001). Finally, QCT indices showed a moderate-to-good correlation with the Goh and Taouli scores (from 0.44 to 0.65; p<0.001). CONCLUSIONS: QCT indices can identify patients with SS and ILD and discriminate those with lesser or greater lung disease.