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Background: Granulomatous and Lymphocytic Interstitial Lung Diseases (GLILD) is a severe non-infectious complication of Common Variable Immunodeficiency (CVID), often associated with extrapulmonary involvement. Due to a poorly understood pathogenesis, GLILD diagnosis and management criteria still lack consensus. Accordingly, it is a relevant cause of long-term loss of respiratory function and is closely associated with a markedly reduced survival. The aim of this study was to describe clinical, immunological, laboratory and functional features of GLILD, whose combination in a predictive model might allow a timely diagnosis. Methods: In a multicenter retrospective cross-sectional study we enrolled 73 CVID patients with radiologic features of interstitial lung disease (ILD) associated to CVID (CVID-ILD) and 125 CVID patients without ILD (controls). Of the 73 CVID-ILD patients, 47 received a definite GLILD diagnosis while 26 received a clinical-radiologic diagnosis of CVID related ILD defined as uILD. Results: In GLILD group we found a higher prevalence of splenomegaly (84.8 vs. 39.2%), autoimmune cytopenia (59.6 vs. 6.4%) and bronchiectasis (72.3 vs. 28%), and lower IgA and IgG serum levels at CVID diagnosis. GLILD patients presented lower percentage of switched-memory B cells and marginal zone B cells, and a marked increase in the percentage of circulating CD21lo B cells (14.2 vs. 2.9%). GLILD patients also showed lower total lung capacity (TLC 87.5 vs. 5.0%) and gas transfer (DLCO 61.5 vs. 5.0%) percent of predicted. By univariate logistic regression analysis, we found IgG and IgA levels at CVID diagnosis, presence of splenomegaly and autoimmune cytopenia, CD21lo B cells percentage, TLC and DCLO percent of predicted to be associated to GLILD. The joint analysis of four variables (CD21lo B cells percentage, autoimmune cytopenia, splenomegaly and DLCO percent of predicted), together in a multiple logistic regression model, yielded an area under the ROC curve (AUC) of 0.98 (95% CI: 0.95-1.0). The AUC was only slightly modified when pooling together GLILD and uILD patients (0.92, 95% CI: 0.87-0.97). Conclusions: we propose the combination of two clinical parameters (splenomegaly and autoimmune cytopenia), one lung function index (DLCO%) and one immunologic variable (CD21lo%) as a promising tool for early identification of CVID patients with interstitial lung disease, limiting the use of aggressive diagnostic procedures.

The oral cavity constitutes a complex anatomical area that can be affected by many developmental, inflammatory, and tumoural diseases. MultiSlice Computed Tomography (MSCT) and Magnetic Resonance Imaging (MRI) currently represent the essential and complementary imaging techniques for detecting oral cavity abnormalities. Advanced MRI with diffusion-weighted imaging (DWI) and dynamic contrast-enhanced perfusion-weighted imaging (DCE-PWI) has recently increased the ability to characterise oral lesions and distinguish disease recurrences from post therapy changes. The analysis of the oral cavity area via imaging techniques is also complicated both by mutual close appositions of different mucosal surfaces and metal artifacts from dental materials. Nevertheless, an exact identification of oral lesions is made possible thanks to dynamic manoeuvres and specific stratagems applicable on MSCT and MRI acquisitions. This study summarises the currently available imaging techniques for oral diseases, with particular attention to the role of DWI, DCE-PWI, and dynamic manoeuvres. We also propose MSCT and MRI acquisition protocols for an accurate study of the oral cavity area.

Purpose(1) To investigate correlations between different types of FAI and the ratio of acetabular volume (AV) to femoral head volume (FV) on MR arthrography. (2) To assess 2D/3D measurements in identifying different types of FAI by means of cut-off values of AV/FV ratio (AFR).Materials and methodsAlpha angle, cranial acetabular version, acetabular depth, lateral center edge angle, AV, and FV of 52 hip MR arthrography were measured. ANOVA test correlated different types of FAI with AFR. ROC curves classified FAI by cut-off values of AFR. Accuracy of 2D/3D measurements was calculated.ResultsANOVA test showed a significant difference of AFR (p value < 0.001) among the three types of FAI. The mean values of AFR were 0.64, 0.74, and 0.89 in cam, mixed, and pincer types, respectively. Cut-off values of AFR were 0.70 to distinguish cam types from mixed and pincer types, and 0.79 to distinguish pincer types from cam and mixed types. Cut-off values identified 100%, 73.9%, and 55.6% of pincer, cam, and mixed types. 2D and 3D classifications of FAI showed accuracy of 40.4% and 73.0%.Conclusions3D measurements were clearly more accurate than 2D measurements. Distinct cut-off values of AFR discriminated cam types from pincer types and identified pincer types in all cases. Cam and mixed types were not accurately recognized.

Severity and mortality of COVID-19 largely depends on the ability of the immune system to clear the virus. Among various comorbidities potentially impacting on this process, the weight and the consequences of an antibody deficiency have not yet been clarified. We used serum protein electrophoresis to screen for hypogammaglobulinemia in a cohort of consecutive adult patients with COVID-19 pneumonia, hospitalized in non-intensive care setting between December 2020 and January 2021. The disease severity, measured by a validated score and by the need for semi intensive (sICU) or intensive care unit (ICU) admission, and the 30-day mortality was compared between patients presenting hypogammaglobulinemia (HYPO) and without hypogammaglobulinemia (no-HYPO). Demographics, comorbidities, COVID-19 specific treatment during the hospital stay, disease duration, complications and laboratory parameters were also evaluated in both groups. We enrolled 374 patients, of which 39 represented the HYPO cohort (10.4%). In 10/39 the condition was previously neglected, while in the other 29/39 hematologic malignancies were common (61.5%); 2/39 were on regular immunoglobulin replacement therapy (IgRT). Patients belonging to the HYPO group more frequently developed a severe COVID-19 and more often required sICU/ICU admission than no-HYPO patients. IgRT were administered in 8/39 during hospitalization; none of them died or needed sICU/ICU. Among HYPO cohort, we observed a significantly higher prevalence of neoplastic affections, of active oncologic treatment and bronchiectasis, together with higher prevalence of viral and bacterial superinfections, mechanical ventilation, convalescent plasma and SARS-CoV-2 monoclonal antibodies administration during hospital stay, and longer disease duration. Multivariate logistic regression analysis and Cox proportional hazard regression confirmed the impact of hypogammaglobulinemia on the COVID-19 severity and the probability of sICU/ICU admission. The analysis of the mortality rate in the whole cohort showed no significant difference between HYPO and no-HYPO. Hypogammaglobulinemia, regardless of its cause, in COVID-19 patients hospitalized in a non-intensive care setting was associated to a more severe disease course and more frequent admission to s-ICU/ICU, particularly in absence of IgRT. Our findings emphasize the add-value of routine serum protein electrophoresis evaluation in patients admitted with COVID-19 to support clinicians in patient care and to consider IgRT initiation during hospitalization.

Background: Chest computed tomography (CT) is the gold standard for the evaluation of systemic sclerosis-related interstitial lung disease (SSc-ILD). Lung ultrasound (LUS) is a radiation-free tool that identifies the B-lines as a main feature of ILD. We aimed to investigate the role of LUS in the evaluation of the extent of SSc-ILD. Methods: Adult SSc patients underwent pulmonary function tests (PFTs), LUS and CT. The CT images were qualitatively, semi-quantitatively (the Wells score on five levels and the categorical Goh et al. staging) and quantitatively (histogram-based densitometry) analysed for ILD. LUS quantified B-lines in 21 intercostal spaces on both the anterior and posterior chest wall. Results: Out of the 77 SSc patients eligible for the study, 35 presented with ILD on CT (21 limited, 14 extensive). Total B-lines significantly differentiated ILD vs. no ILD (median 24 vs. 8, p < 0.001). Posterior and total B-lines significantly differentiated limited from absent ILD, while anterior B-lines distinguished extensive from limited ILD. Total B-lines correlated with the Wells score (r = 0.446, p < 0.001) and MLA (r = −0.571, p < 0.001); similar results were confirmed when anterior and posterior B-lines were analysed separately. Conclusions: LUS is a useful tool to identify SSc-ILD and to correlate with different evaluations of ILD extent and severity.

Sarcoidosis and Granulomatous and Lymphocytic Interstitial Lung Diseases (GLILD) are two rare entities primarily characterised by the development of Interstitial Lung Disease (ILD) in the context of systemic immune dysregulation. These two conditions partially share the immunological background and pathologic findings, with granuloma as the main common feature. In this narrative review, we performed a careful comparison between sarcoidosis and GLILD, with an overview of their main similarities and differences, starting from a clinical perspective and ending with a deeper look at the immunopathogenesis and possible target therapies. Sarcoidosis occurs in immunocompetent individuals, whereas GLILD occurs in patients affected by common variable immunodeficiency (CVID). Moreover, peculiar extrapulmonary manifestations and radiological and histological features may help distinguish the two diseases. Despite that, common pathogenetic pathways have been suggested and both these disorders can cause progressive impairment of lung function and variable systemic granulomatous and non-granulomatous complications, leading to significant morbidity, reduced quality of life, and survival. Due to the rarity of these conditions and the extreme clinical variability, there are still many open questions concerning their pathogenesis, natural history, and optimal management. However, if studied in parallel, these two entities might benefit from each other, leading to a better understanding of their pathogenesis and to more tailored treatment approaches.

Background: The SARS-CoV-2 infection is now a part of the everyday lives of immunocompromised patients, but the choice of treatment and the time of viral clearance can often be complex, exposing patients to possible complications. The role of the available antiviral and monoclonal therapies is a matter of debate, as are their effectiveness and potential related adverse effects. To date, in the literature, the amount of data on the use of combination therapies and on the multiple lines of anti-SARS-CoV-2 therapy available to the general population and especially to inborn error of immunity (IEI) patients is small. Methods: Here, we report a case series of five adult IEI patients managed as inpatients at three Italian IEI referral centers (Rome, Treviso, and Cagliari) treated with combination therapy or multiple therapeutic lines for SARS-CoV-2 infection, such as monoclonal antibodies (mAbs), antivirals, convalescent plasma (CP), mAbs plus antiviral, and CP combined with antiviral. Results: This study may support the use of combination therapy against SARS-CoV-2 in complicated IEI patients with predominant antibody deficiency and impaired vaccine response.

This case report describes an 80‐year‐old woman with systemic sclerosis (SSc), complicated by interstitial lung disease (ILD) and new‐onset pulmonary hypertension (PH), likely triggered by an atypical SARS‐CoV‐2 infection. Diagnosed with SSc in 2016 and previously stable ILD, she experienced clinical deterioration in 2023 with worsening respiratory failure and PH. Despite negative PCR and antigen tests, high anti‐SARS‐CoV‐2 IgG levels and CT findings were consistent with COVID‐19‐related organising pneumonia. Intravenous glucocorticoids led to partial symptom improvement, although ILD progression continued. The patient died in December 2023 from pneumococcal pneumonia. This case highlights the complex interaction between SSc and COVID‐19, where overlapping mechanisms of endothelial injury and fibrosis may exacerbate pre‐existing organ damage. It underscores the need for a multidisciplinary approach and timely interventions, including vaccination, early clinical assessment and appropriate immunosuppressive or antiviral treatments, to prevent severe infectious complications and halt disease progression in this high‐risk patient population. This report describes a woman with SSc and mild interstitial lung disease who developed COVID‐19 pneumonia, leading to rapid ILD progression and new onset pulmonary hypertension, ultimately resulting in fatal pneumococcal infection. The case underscores the critical role of multidisciplinary care and timely vaccination in managing high‐risk SSc patients during and after SARS‐CoV‐2 infection.

Metastases from endometrial carcinoma to the lungs are uncommon and cavitating metastases are even rarer. In some cases, lung cavitating metastases may resemble diffuse \"Cheerios in the lung.\" This case is a rare example of metastatic endometrioid adenocarcinoma, manifested with chronic dry cough, with \"Cheerios in the lung\" appearence. The metastases responded well to immunotherapy, resulting in the regression of nodules into areas of diffuse emphysematous-like changes.

Background COVID-19 is a pandemic disease affecting predominantly the respiratory apparatus with clinical manifestations ranging from asymptomatic to respiratory failure. Chest CT is a crucial tool in diagnosing and evaluating the severity of pulmonary involvement through dedicated scoring systems. Nonetheless, many questions regarding the relationship of radiologic and clinical features of the disease have emerged in multidisciplinary meetings. The aim of this retrospective study was to explore such relationship throughout an innovative and alternative approach. Materials and methods This study included 550 patients (range 25–98 years; 354 males, mean age 66.1; 196 females, mean age 70.9) hospitalized for COVID-19 with available radiological and clinical data between 1 March 2021 and 30 April 2022. Radiological data included CO-RADS, chest CT score, dominant pattern, and typical/atypical findings detected on CT examinations. Clinical data included clinical score and outcome. The relationship between such features was investigated through the development of the main four frequently asked questions summarizing the many issues arisen in multidisciplinary meetings, as follows 1) CO-RADS, chest CT score, clinical score, and outcomes; 2) the involvement of a specific lung lobe and outcomes; 3) dominant pattern/distribution and severity score for the same chest CT score; 4) additional factors and outcomes. Results 1) If CT was suggestive for COVID, a strong correlation between CT/clinical score and prognosis was found; 2) Middle lobe CT involvement was an unfavorable prognostic criterion; 3) If CT score < 50%, the pattern was not influential, whereas if CT score > 50%, crazy paving as dominant pattern leaded to a 15% increased death rate, stacked up against other patterns, thus almost doubling it; 4) Additional factors usually did not matter, but lymph-nodes and pleural effusion worsened prognosis. Conclusions This study outlined those radiological features of COVID-19 most relevant towards disease severity and outcome with an innovative approach.