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Left renal vein variants are not commonly observed in the general population. Usually, the renal vein runs in front of the aorta before entering the inferior vena cava, while the most common variants include the presence of a circumaortic or retroaortic renal vein. However, when present, left venal rein variants are important to recognize due to their potential clinical and surgical relevance. In this regard, CE-CT is an instrument with high sensitivity and specificity in detecting vascular anomalies and can certainly help diagnose. In this article, we present a unique case of a left venal rein compressed between the left iliac artery and vertebral bodies associated with the presence of a superior mesenteric artery Syndrome, another rare entity that occurs when the duodenum is compressed between the aorta and the superior mesenteric artery.

Wernicke encephalopathy (WE) is a rare but severe neurological syndrome characterized, in its classic form, by the acute onset of ocular disturbances, ataxia, and cognitive impairment. It is caused by a deficiency of thiamine (vitamin B1) and mainly affects chronic alcoholics, although it can also affect patients with pathologies that lead to malnutrition. We present a case of a 58-year-old woman, who presented with significant weight loss over the past 6 months and who came to the emergency department for episodes of repetitive vomiting and a sleepy state. The patient underwent blood chemistry tests and a brain CT scan, which revealed symmetrical and bilateral hypodensity of the medial portion of the thalamus, tectal plate, and periaqueductal gray matte, suggestive of WE. She was subsequently referred to the Department of Neurology and underwent a brain MRI, which confirmed the clinical suspicion. She also had an abdominal CT scan and ileo-colonoscopy and was diagnosed with Crohn's disease. Immediately after the clinical diagnosis of WE, a replacement therapy based on intravenous thiamine at high doses was promptly set up, and the patient improved from a clinical point of view.Wernicke encephalopathy can be difficult to diagnose when it occurs in non-alcoholic patients; WE associated with IBD is a rare condition, and it can present with atypical and more subtle symptoms. Radiologists and physicians must be aware of this condition and imaging findings for rapid diagnosis and treatment.

Extramedullary hematopoiesis represents a clinical compensatory condition characterized by the growth of hematopoietic tissue outside the bone marrow. It can mainly occur in patient with myeloproliferative disorders where alteration or neoplastic invasion of the bone marrow causes ineffective production of blood cells with the recruitment of progenitrix blood cells in non-hematopoietic organs, including kidneys. Renal extramedullary hematopoiesis is a rare condition manifesting as parenchymal or perirenal soft tissue masses with different patterns mimicking neoplasms, infectious or vascular diseases. We describe a unique case of a patient affected by primary myelofibrosis underwent ultrasound and magnetic resonance examinations showing bilateral perirenal alterations to be related to hemopoietic tissue. We also focused on the pathophysiology of this condition with imaging correlation. The case we present emphasises the importance of recognising the main radiological features of renal extramedullary hematopoiesis. MR examination should become part of the diagnostic pathway of the patient with primary myelofibrosis.

Abstract Purpose The purpose of this systematic review and meta-analysis was to evaluate the safety, technical, and clinical effectiveness of percutaneous Transarterial Embolization (TAE) in treating spontaneous retroperitoneal hematomas as well as assess treatment outcomes in patients who underwent target or empirical embolization. Materials and methods Through the PubMed, Embase, and Google Scholar databases, an extensive search was performed in the fields of spontaneous retroperitoneal hematomas treated with transcatheter arterial embolization. We collected pooled data on 141 patients from 6 separate articles selected according to the inclusion and exclusion criteria. Results Technical success rate was 100% in all six studies, for both targeted and empirical embolization. The clinical success rate varied from 56.3 to 89.5%. The total number of complications related to the embolization procedure was 10 events out of 116 procedures analyzed. Empirical or empirical embolization was performed in three studies, where the source of active bleeding was not evident during DSA. A meta-analysis compared the rebleeding rates between targeted and empirical embolization groups. The odds ratio from pooled data from the three assessed studies (72 patients) showed no significant difference in rebleeding rates after empirical TAE compared with targeted TAE. Conclusions TAE is a safe, effective, and potentially life-saving procedure for the treatment of life-threatening spontaneous retroperitoneal hematomas. Empirical and targeted TAE procedures demonstrate a relatively low risk of complications, compared to the high technical and relatively high clinical success rates.

Atherosclerotic disease of the carotid arteries is a crucial risk factor in predicting the likelihood of future stroke events. In addition, emerging studies suggest that carotid stenosis may also be an indicator of plaque load on coronary arteries and thus have a correlation with the risk of acute cardiovascular events. Furthermore, although in symptomatic patients the degree of stenosis is the main morphological parameter studied, recent evidence suggests, especially in asymptomatic patients, that plaque vulnerability should also be evaluated as an emerging and significant imaging parameter. The reference diagnostic methods for the evaluation of carotid stenosis are currently ultrasonography, magnetic resonance imaging (MRI), and computed tomography angiography (CTA). In addition, other more invasive methods such as 123I-metaiodobenzylguanidine (MIBG) scintigraphy and PET-CT, as well as digital subtraction angiography, can be used. Each method has advantages and disadvantages, and there is often some confusion in their use. For example, the usefulness of MRI is often underestimated. In addition, implementations for each method have been developed over the years and are already enabling a significant increase in diagnostic accuracy. The purpose of our study is to make an in-depth analysis of all the methods in use and in particular their role in the diagnostic procedure of carotid stenosis, also discussing new technologies.

Ultrasound (US) is the primary tool for evaluating patients with thyroid nodules, and the risk of malignancy assessed is based on US features. These features help determine which patients require fine-needle aspiration (FNA) biopsy. Classification systems for US features have been developed to facilitate efficient interpretation, reporting, and communication of thyroid US findings. These systems have been validated by numerous studies and are reviewed in this article. Additionally, this overview provides a comprehensive description of the clinical and laboratory evaluation of patients with thyroid nodules, various imaging modalities, grayscale US features, color Doppler US, contrast-enhanced US (CEUS), US elastography, FNA biopsy assessment, and the recent introduction of molecular testing. The potential of artificial intelligence in thyroid US is also discussed.

Background: type 1 neurofibromatosis (NF1) is the most common neurocutaneous disorder, and it is an inherited condition that causes a tumour predisposition. Central nervous system (CNS) manifestations are a significant cause of morbidity and mortality in NF1. We provide a pictorial review of neuroradiological features of NF1, with emphasis on magnetic resonance imaging (MRI), and we assess the frequency of those features on a cohort of NF1 patients. Methods: we retrospectively evaluated all patients with a diagnosis of NF1 who underwent MRI of the spine and brain in our centre over a period of almost 5 years. A total of 74 patients were enrolled, 28 males and 46 females, with a mean age of 21 ± 12.67 years. The frequency of CNS manifestations encountered in our cohort of NF1 patients was assessed and compared with the data found in other studies published in the literature. Results: many of our findings were in line with the literature, and possible interpretations for those that turned out to be different were suggested in the discussion. Conclusion: imaging plays a central role in the diagnosis and management of NF1, and the knowledge of CNS manifestations could be critical for its early detection and identification, such as for treatment planning and prognostic implications.

The term congenital lung malformation (CLM) is used to describe a wide range of pathological conditions with different imaging and clinical manifestations. These anomalies stem from abnormal embryological lung development, potentially occurring across various stages of prenatal life. Their natural history can be variable, presenting in a wide range of severity levels and encompassing asymptomatic individuals who remain so until adulthood, as well as those who experience respiratory distress in the neonatal period. Through the PubMed database, we performed an extensive review of the literature in the fields of congenital lung abnormalities, including their diagnostic approach and findings. From our RIS-PACS database, we have selected cases with a final diagnosis of congenital lung malformation. Different diagnostic approaches have been selected, including clinical cases studied using plain radiograph, CT scan, prenatal ultrasound, and MR images. The most encountered anomalies can be classified into three categories: bronchopulmonary anomalies (congenital pulmonary airway malformations (CPAMs), congenital lobar hyperinflation, bronchial atresia, and bronchogenic cysts), vascular anomalies (arteriovenous malformation), and combined lung and vascular anomalies (scimitar syndrome and bronchopulmonary sequestration). CLM causes significant morbidity and mortality; therefore, the recognition of these abnormalities is necessary for optimal prenatal counseling and early peri- and postnatal management. This pictorial review aims to report relevant imaging findings in order to offer some clues for differential diagnosis both for radiologists and pediatric consultants.

In this retrospective case series, we investigate the synergistic effect and the immunomodulatory potential of combination radiotherapy and immunotherapy on 11 patients affected by locally advanced or metastatic cutaneous squamous cell carcinoma (CSCC), treated at our institution between 2020 and 2023. The primary endpoints of this study are objective tumor response, assessed by Immunotherapy Response Evaluation Criteria in Solid Tumors (iRECIST), and time to treatment failure (disease progression). In all patients, surgery was deemed not amenable, due to its potential functional and aesthetic impact. Therefore, upon multidisciplinary agreement, radiotherapy and immunotherapy with cemiplimab were alternatively administered. After 6 months, an early objective tumor response was observed in 9/11 patients, with 17/20 cutaneous lesions (85%) presenting either a complete or partial response. Only 2/11 patients, with a total of 3/20 cutaneous lesions (15%), had stable disease. These benefits persisted at a longer follow-up (21.4 ± 9.7 months), with no patients presenting disease progression. Despite the retrospective nature of this study and small sample size, our experience highlights the ability of concomitant radiotherapy and cemiplimab to promote an early objective response in patients with advanced CSCC. Moreover, in our population, the clinical benefits were also related to a longer progression-free survival, without any safety alert reported.

Background/Objectives: Benign prostatic hyperplasia (BPH) has a significant impact on the quality of life of symptomatic patients. In patients manifesting lower urinary tract symptoms (LUTS), prostatic arterial embolization (PAE) has become a topic of interest in recent years. The purpose of this systematic review is to analyze and review techniques and clinical outcomes of patients who underwent endovascular treatment of BPH, with a special focus on the comparison of surgical and endovascular procedures. Methods: Through the major scientific databases, 1225 articles were selected from the initial research utilizing specific keywords and medical subject headings. Based on the inclusion and exclusion criteria established for selecting relevant studies for our purposes, the systematic analysis of the literature was conducted on a total of seven articles. Results: We collected data on 718 male patients (408 patients underwent PAE and 310 received TURP). The technical success rate varied from 86% to 100% for TAE and was 100% for TURP. During a 12-month follow-up period, both PAE and TURP were comparable on the reduction in IPSS and QoL questionnaire, while TURP showed significant improvements in Qmax and in the reduction in prostate volume. Length and cost of hospitalization were lower for PAE. Complication and adverse events rates were higher in the TURP group rather than in the PAE group (60.6% vs. 35.5%). Conclusions: Prostatic artery embolization represents an emerging minimally invasive procedure for BPH. According to previously released clinical studies, quality-of-life and urological symptom scores of the PAE group were comparable to those of the TURP group. Our research strengthens the evidence supporting the effectiveness and safety of PAE as a therapy for LUTS related to BPH.

Prune exopolyphosphatase 1 (PRUNE1) is a short-chain phosphatase that is part of the aspartic acid-histidine-histidine (DHH) family of proteins. PRUNE1 is highly expressed in the central nervous system and is crucially involved in neurodevelopment, cytoskeletal rearrangement, cell migration, and proliferation. Recently, biallelic PRUNE1 variants have been identified in patients with neurodevelopmental disorders, hypotonia, microcephaly, variable cerebral anomalies, and other features. PRUNE1 hypomorphic mutations mainly affect the DHH1 domain, leading to an impactful decrease in enzymatic activity with a loss-of-function mechanism. In this review, we explored both the clinical and radiological spectrum related to PRUNE1 pathogenic variants described to date. Specifically, we focused on neuroradiological findings that, together with clinical phenotypes and genetic data, allow us to best characterize affected children with diagnostic and potential prognostic implications.

The WWOX gene encodes a 414-amino-acid protein composed of two N-terminal WW domains and a C-terminal short-chain dehydrogenase/reductase (SDR) domain. WWOX protein is highly conserved among species and mainly expressed in the cerebellum, cerebral cortex, brain stem, thyroid, hypophysis, and reproductive organs. It plays a crucial role in the biology of the central nervous system, and it is involved in neuronal development, migration, and proliferation. Biallelic pathogenic variants in WWOX have been associated with an early infantile epileptic encephalopathy known as WOREE syndrome. Both missense and null variants have been described in affected patients, leading to a reduction in protein function and stability. The most severe WOREE phenotypes have been related to biallelic null/null variants, associated with the complete loss of function of the protein. All affected patients showed brain anomalies on magnetic resonance imaging (MRI), suggesting the pivotal role of WWOX protein in brain homeostasis and developmental processes. We provided a literature review, exploring both the clinical and radiological spectrum related to WWOX pathogenic variants, described to date. We focused on neuroradiological findings to better delineate the WOREE phenotype with diagnostic and prognostic implications.

Abstract Background Acute non-variceal gastrointestinal bleedings (GIBs) are pathological conditions associated with significant morbidity and mortality. Embolization without angiographic evidence of contrast media extravasation is proposed as an effective procedure in patients with clinical and/or laboratory signs of bleeding. The purpose of this systematic review is to define common clinical practice and clinical and technical outcomes of blind and preventive embolization for upper and lower gastrointestinal bleeding. Main body Through the PubMed, Embase and Google Scholar database, an extensive search was performed in the fields of empiric and preventive embolization for the treatment of upper and lower gastrointestinal bleedings (UGIB and LGIB). Inclusion criteria were: articles in English for which it has been possible to access the entire content; adults patients treated with empiric or blind transcatheter arterial embolization (TAE) for UGIB and/or LGIB. Only studies that analysed clinical and technical success rate of blind and empiric TAE for UGIB and/or LGIB were considered for our research. Exclusion criteria were: recurrent articles from the same authors, articles written in other languages, those in which the entire content could not be accessed and that articles were not consistent to the purposes of our research. We collected pooled data on 1019 patients from 32 separate articles selected according to the inclusion and exclusion criteria. 22 studies focused on UGIB (total 773 patients), one articles focused on LGIB (total 6 patients) and 9 studies enrolled patients with both UGIB and LGIB (total 240 patients). Technical success rate varied from 62% to 100%, with a mean value of 97.7%; clinical success rate varied from 51% to 100% with a mean value of 80%. The total number of complications was 57 events out of 1019 procedures analysed. Conclusion TAE is an effective procedure in the treatment of UGIB patients in which angiography does not demonstrate direct sign of ongoing bleeding. The attitude in the treatment of LGIBs must be more prudent in relation to poor vascular anastomoses and the high risk of intestinal ischemia. Blind and preventive procedures cumulatively present a relatively low risk of complications, compared to a relatively high technical and clinical success.

Background: We proposed the term “UIPAF” to define patients with Usual Interstitial Pneumonia (UIP) associated with only one domain of the classification called “Interstitial Pneumonia with Autoimmune Features” (IPAF). The objective of this study was to evaluate the clinical presentation and prognosis of UIPAF patients, compared with two cohorts, composed of IPAF and idiopathic pulmonary fibrosis (IPF) patients, respectively. Methods: The patients were enrolled as IPAF, UIPAF, or IPF based on clinical, serological, and radiological data and evaluated by a multidisciplinary team. Results: We enrolled 110 patients with IPF, 69 UIPAF, and 123 IPAF subjects. UIPAF patients were similar to IPAF regarding autoimmune features, except for the prevalence of Rheumatoid Factor in UIPAF and anti-SSA in IPAF. A similar proportion of the two cohorts progressed toward a specific autoimmune disease (SAD), with differences in the kind of SAD developed. The real-life management and prognosis of UIPAF patients proved to be almost identical to IPF. Conclusions: UIPAF shared with IPAF similar autoimmune features, suggesting the opportunity to be considered IPAF, excluding the morphological domain by the classification. However, the real-life management and prognosis of UIPAF are similar to IPF. These data suggest a possible modification in the therapeutic management of UIPAF.

Orbital and ocular adnexa lymphomas are rare neoplasms confined to the orbital region. The prognosis is generally favorable, with a high proportion of localized disease, indolent clinical course, prolonged disease-free intervals, and low lymphoma-related mortality rate. We report our experience on eleven patients with confirmed histological diagnosis of lymphoma stage IE-IIE, treated between 2010 and 2021 with radiotherapy alone or in association with chemotherapy or immunotherapy. Eight patients were treated with primary radiotherapy only, while three received previous systemic treatments. Six patients were treated with Proton beam therapy (PBT), and five with external beam radiotherapy (EBRT). The five-year local control rate was 98%; only one patient developed an out-of-field recurrence. We also conducted a comprehensive literature review using electronic databases (PubMed, EMBASE, and Cochrane Library). Articles were selected based on their pertinence to treatment of the ocular and adnexal lymphoma focusing on radiotherapy techniques (electron beam radiotherapy, photon beam radiotherapy, or proton beam radiotherapy), treatment total dose, fractionation schedule, early and late radio-induced toxicities, and patient’s clinical outcome. Radiotherapy is an effective treatment option for orbital lymphoma, especially as standard treatment in the early stage of orbital lymphoma, with excellent local control rate and low rates of toxicity.

Acute colonic diverticulitis (ACD) is the most common complication of diverticular disease and represents an abdominal emergency. It includes a variety of conditions, extending from localized diverticular inflammation to fecal peritonitis, hence the importance of an accurate diagnosis. Contrast-enhanced computed tomography (CE-CT) plays a pivotal role in the diagnosis due to its high sensitivity, specificity, accuracy, and interobserver agreement. In fact, CE-CT allows alternative diagnoses to be excluded, the inflamed diverticulum to be localized, and complications to be identified. Imaging findings have been reviewed, dividing them into bowel and extra-intestinal wall findings. Moreover, CE-CT allows staging of the disease; the most used classifications of ACD severity are Hinchey’s modified and WSES classifications. Differential diagnoses include colon carcinoma, epiploic appendagitis, ischemic colitis, appendicitis, infectious enterocolitis, and inflammatory bowel disease. We propose a structured reporting template to standardize the terminology and improve communication between specialists involved in patient care.

Histopathologically, uveal melanomas (UMs) can be classified as spindle cell, mixed cell and epithelioid cell type, with the latter having a more severe prognosis. The aim of our study was to assess the correlation between the apparent diffusion coefficient (ADC) and the histologic type of UMs in order to verify the role of diffusion-weighted magnetic resonance imaging (DWI) as a noninvasive prognostic marker. A total of 26 patients with UMs who had undergone MRI and subsequent primary enucleation were retrospectively selected. The ADC of the tumor was compared with the histologic type. The data were compared using both one-way analysis of variance (ANOVA) (assessing the three histologic types separately) and the independent t-test (dichotomizing histologic subtypes as epithelioid versus non-epithelioid). Histologic type was present as follows: the epithelioid cell was n = 4, and the spindle cell was n = 11, the mixed cell type was n = 11. The mean ADC was 1.06 ± 0.24 × 10−3 mm2/s in the epithelioid cells, 0.98 ± 0.19 × 10−3 mm2/s in the spindle cells and 0.96 ± 0.26 × 10−3 mm2/s in the mixed cell type. No significant difference in the mean ADC value of the histopathologic subtypes was found, either when assessing the three histologic types separately (p = 0.76) or after dichotomizing the histologic subtypes as epithelioid and non-epithelioid (p = 0.82). DWI-ADC is not accurate enough to distinguish histologic types of UMs.

Autosomal dominant polycystic renal disease (ADPKD) is the most frequent kidney inheritable disease, characterized by the presence of numerous bilateral renal cysts, causing a progressive increase in total kidney volume (TKV) and a progressive loss of renal function. Several methods can be used to measure TKV by using MRI, and they differ in complexity, accuracy and time consumption. This study was performed to assess the performance of the ellipsoid method and the semi-automatic segmentation method, both for TKV and SKV (single kidney volume) computation. In total, 40 patients were enrolled, and 78 polycystic kidneys analyzed. Two independent operators with different levels of experience evaluated renal volumetry using both methods. Mean error for ellipsoid method for SKV computation was −2.74 ± 11.79% and 3.25 ± 10.02% for the expert and the beginner operator, respectively (p = 0.0008). A Wilcoxon test showed a statistically significant difference between the two operators for both methods (SKV p = 0.0371 and 0.0034; TKV p = 0.0416 and 0.0171 for the expert and the beginner operator, respectively). No inter-operator significant difference was found for the semi-automatic method, in contrast to the ellipsoid method. Both with a Wilcoxon test and Bland–Altman plot, statistically significant differences were found when comparing SKV and TKV measurements obtained with the two methods for both operators, even if the differences are stronger for the beginner operator than for the expert one. The semi-automatic segmentation method showed more inter-observer reproducibility. The ellipsoid method, in contrast, appears to be affected by greater inter-observer variability, especially when performed by operators with limited experience.

To investigate the clinical impact of multiple courses of irradiation on pediatric patients with progressive diffuse intrinsic pontine glioma (DIPG), we conducted a retrospective case series on three children treated at our institution from 2018 to 2022. All children were candidates to receive systemic therapy with vinorelbine and nimotuzumab. Radiotherapy was administered to a total dose of 54 Gy. At any disease progression, our local tumor board evaluated the possibility of offering a new course of radiotherapy. To determine feasibility and assess toxicity rates, all children underwent clinical and hematological evaluation both during and after the treatment. To assess efficacy, all children performed contrast-enhanced MRI almost quarterly after the end of the treatment. In all children, following any treatment course, neurological improvement (>80%) was associated with a radiological response (41.7–46%). The longest overall survival (24 months) was observed in the child who underwent three courses of radiotherapy, without experiencing significant side effects. Even though it goes beyond the understanding of conventional radiobiology, first and second reirradiation in pediatric patients with progressive DIPG may represent a feasible and safe approach, capable of increasing overall survival and disease-free survival in selected patients and improving their quality of life.

Abstract Connective tissue diseases (CTDs) include a spectrum of disorders that affect the connective tissue of the human body; they include autoimmune disorders characterized by immune-mediated chronic inflammation and the development of fibrosis. Lung involvement can be misdiagnosed, since pulmonary alterations preceded osteo-articular manifestations only in 20% of cases and they have no clear clinical findings in the early phases. All pulmonary structures may be interested: pulmonary interstitium, airways, pleura and respiratory muscles. Among these autoimmune disorders, rheumatoid arthritis (RA) is characterized by usual interstitial pneumonia (UIP), pulmonary nodules and airway disease with air-trapping, whereas non-specific interstitial pneumonia (NSIP), pulmonary hypertension and esophageal dilatation are frequently revealed in systemic sclerosis (SSc). NSIP and organizing pneumonia (OP) may be found in patients having polymyositis (PM) and dermatomyositis (DM); in some cases, perilobular consolidations and reverse halo-sign areas may be observed. Systemic lupus erythematosus (SLE) is characterized by serositis, acute lupus pneumonitis and alveolar hemorrhage. In the Sjögren syndrome (SS), the most frequent pattern encountered on HRCT images is represented by NSIP; UIP and lymphocytic interstitial pneumonia (LIP) are reported with a lower frequency. Finally, fibrotic NSIP may be the interstitial disease observed in patients having mixed connective tissue diseases (MCTD). This pictorial review therefore aims to provide clinical features and imaging findings associated with autoimmune CTDs, in order to help radiologists, pneumologists and rheumatologists in their diagnoses and management.

(1) Background: Spontaneous retroperitoneal hematomas are a relatively common occurrence in hospitalized patients with COVID-19 related pneumonia, and endovascular treatment of trans-arterial embolization (TAE) may be a life-saving procedure after failure of medical and supportive therapy. The aim of our study was to evaluate spontaneous retroperitoneal hematomas in the COVID-19 era, focusing on their imaging features at CTA and DSA and on the safety, as well as technical and clinical success, of TAE, comparing patients affected by COVID-19 and non-COVID-19 patients. (2) Materials and Methods: We retrospectively enrolled 24 patients with spontaneous retroperitoneal hematoma who underwent TAE; of these, 10 were hospitalized for COVID-19-related pneumonia, while the other 14 were without COVID-19 infection. We evaluated the demographic data, hemoglobin values before and after the procedure, preprocedural aPTT, preprocedural INR, diagnostic and interventional imaging findings, procedural outcome (technical success) and survival periprocedural (clinical success), and major and minor complications. (3) Results: The mean age of the study population was 72.7 ± 11.2 years. CTA revealed signs of active bleeding in 20 patients (83%). DSA showed signs of active bleeding in 20 patients (83%). In four patients (17%), blind embolization was performed. The overall technical success rate was 100%. Clinical success was achieved in 17 patients (71%), while seven patients (29%) rebled within 96 h, and all of them were retreated. No major periprocedural complication was reported. The comparison between the two groups did not show statistically significant differences for gender, mean age, mean pre- and postprocedural hemoglobin, aPTT and INR, mean hematoma volume (cm3), or mean delay between CT and DSA. Active bleeding at CTA was detected in 90% of COVID-19 patients and 79% of non-COVID-19 patients (p = 0.61). At DSA, active bleeding was assessed in eight out of 10 (80%) patients in the COVID-19 group and 12 out of 14 (86%) patients in the non-COVID-19 group (p = 1). Technical success was obtained in 100% of patients in both groups. Clinical success rates were 70% for COVID-19 group and 71% for the non-COVID-19 group. We found no statistical significance between the clinical success rates of retroperitoneal spontaneous hematoma embolization in patients with or without SARS-CoV-2 infection. (4) Conclusions: We suggest that, similar to what has been reported in other studies in non-COVID-19 patients, TAE should be considered an important safe, effective, and potentially life-saving option for the management and the treatment of patients affected by COVID-19 who present with spontaneous retroperitoneal hematoma and who could not benefit from conservative treatment.

In recent decades, several pieces of evidence have drawn greater attention to the topic of innate immunity, in particular, interferon (IFN) and Interleukin 6 in the pathogenesis of idiopathic inflammatory myopathies (IIM). Both of these molecules transduce their signal through a receptor coupled with Janus kinases (JAK)/signal transducer and activator of transcription proteins (STAT). In this review, we discuss the role of the JAK/STAT pathway in IIM, evaluate a possible therapeutic role for JAK inhibitors in this group of diseases, focusing on those with the strongest IFN signature (dermatomyositis and antisynthetase syndrome).

Introduction: Pulmonary artery intimal sarcoma (PAIS) is a rare malignant neoplasm with imaging features that can mimic pulmonary embolism (PE). It must be recognized early because a radical resection may be useful to prolong survival. Case description: A clinical case of a 57-year-old Caucasian male affected by PAIS is presented, which describes the computed tomography (CT) findings found in PAIS and the elements of overlap and differentiation with PE. The main common element is represented by the endoluminal filling defect of the pulmonary arterial vessels in contrast-enhanced CT examinations; a characteristic polypoid morphology or polylobulated contours are typical findings of PAIS. Other specific elements of the neoplasm such as wall eclipse sign, extension beyond the arterial wall, and metastasis are also explained. Conclusions: The overlap of the clinical–radiological findings and the epidemiological difference between PAIS and PE cause a diagnostic delay. By knowing the differential elements, the radiologist can detect the neoplasm early to accelerate diagnosis and suggest optimal management.

May-Thurner syndrome (MTS) belongs to a group of uncommon vascular syndromes. It consists in left common iliac vein (LCIV) compression between the right common iliac artery (RCIA) anteriorly and the lumbar spine posteriorly. A compression of LCIV by the left common iliac artery (LCIA) or by both iliac arteries were described. We present a rare case of “double MTS” which consist in double stenosis of LCIV by both RCIA and LCIA. Double MTS can cause acute or chronic DVT; this latter could be clinical manifest or well compensated.A 58-year-old woman with chronic mild pelvic pain underwent Doppler Ultrasound (US) of the pelvis and lower extremity vessels which showed thrombosis of both LCIV and ipsilateral common femoral vein caused by the extrinsic compression by both common iliac arteries against the spine. CT angiography confirmed the US data and ruled out other causes of compression. CT scan also showed the development of a natural venous femoro-femoral bypass which allowed to counteract the venous stasis and compensate venous drainage.Therefore, we decide for a long-term prophylaxis with anticoagulant drugs and doppler US follow-up at 6 months.In conclusion, doppler US is a non-invasive, low-cost, repeatable and sensitive method which allows to diagnose MTS and associated DVT. It may be considered the first level exam which allows to easily detect pelvic vascular compression syndrome.

Tourette syndrome (TS) is characterized by multiple motor and vocal tics, and high-comorbidity rates with other neuropsychiatric disorders. Obsessive compulsive disorder (OCD), attention deficit hyperactivity disorder (ADHD), autism spectrum disorders (ASDs), major depressive disorder (MDD), and anxiety disorders (AXDs) are among the most prevalent TS comorbidities. To date, studies on TS brain structure and function have been limited in size with efforts mostly fragmented. This leads to low-statistical power, discordant results due to differences in approaches, and hinders the ability to stratify patients according to clinical parameters and investigate comorbidity patterns. Here, we present the scientific premise, perspectives, and key goals that have motivated the establishment of the Enhancing Neuroimaging Genetics through Meta-Analysis for TS (ENIGMA-TS) working group. The ENIGMA-TS working group is an international collaborative effort bringing together a large network of investigators who aim to understand brain structure and function in TS and dissect the underlying neurobiology that leads to observed comorbidity patterns and clinical heterogeneity. Previously collected TS neuroimaging data will be analyzed jointly and integrated with TS genomic data, as well as equivalently large and already existing studies of highly comorbid OCD, ADHD, ASD, MDD, and AXD. Our work highlights the power of collaborative efforts and transdiagnostic approaches, and points to the existence of different TS subtypes. ENIGMA-TS will offer large-scale, high-powered studies that will lead to important insights toward understanding brain structure and function and genetic effects in TS and related disorders, and the identification of biomarkers that could help inform improved clinical practice.

At the time of diagnosis, the vast majority of prostate carcinoma patients have a clinically localized form of the disease, with most of them presenting with low- or intermediate-risk prostate cancer. In this setting, various curative-intent alternatives are available, including surgery, external beam radiotherapy and brachytherapy. Randomized clinical trials have demonstrated that moderate hypofractionated radiotherapy can be considered as a valid alternative strategy for localized prostate cancer. High-dose-rate brachytherapy can be administered according to different schedules. Proton beam radiotherapy represents a promising strategy, but further studies are needed to make it more affordable and accessible. At the moment, new technologies such as MRI-guided radiotherapy remain in early stages, but their potential abilities are very promising.

Perinuclear Anti Neutrophil Cytoplasmic Antibody (p-ANCA) is a serological marker of Microscopic Polyangiitis (MPA), a vasculitis associated with lung involvement potentially mimicking Idiopathic Pulmonary Fibrosis (IPF). In this study, we evaluated the role of p-ANCA in predicting clinical evolution and prognosis in a cohort of IPF patients. In this observational, retrospective, case–control study, we compared 18 patients with an IPF diagnosis and p-ANCA positivity with 36 patients with seronegative IPF, matched for age and sex. IPF patients with and without p-ANCA showed similar lung function decline during the follow-up, but IPF p-ANCA+ showed better survival. Half of IPF p-ANCA+ patients were classified as MPA for the development of renal involvement (55%) or skin signs (45%). The progression towards MPA was associated with high levels of Rheumatoid Factor (RF) at baseline. In conclusion, p-ANCA, mainly when associated with RF, could predict the evolution of Usual Interstitial Pneumonia (UIP) towards a definite vasculitis in patients, with a better prognosis compared with IPF. In this view, ANCA testing should be included in the diagnostic workup of UIP patients.

Background: Anterior nutcracker syndrome is defined as the compression of the left renal vein (LRV) between the superior mesenteric artery (SMA) and the aorta, whereas posterior nutcracker syndrome refers to the compression of the retroaortic LRV between the aorta and the vertebral column—the presence of the circumaortic left renal vein may predispose to “combined nutcracker syndrome”. May–Thurner syndrome consists of obstruction of the left common iliac vein caused by the crossing right common iliac artery. We report a unique case of combined nutcracker syndrome associated with May–Thurner syndrome. Case presentation: A 39-year-old Caucasian female came to our radiology unit for triple-negative breast cancer computed tomography (CT) staging. She complained of pain in hermid-back and low-back regions and intermittent abdominal pain in the left flank region. Multidetector computed tomography (MDCT) incidentally revealed a circumaortic left renal vein draining to the inferior vena cava, with bulbous dilatation of both the antero-superior and posterior-inferior branches, which was associated with pathological serpiginous dilation of the left ovarian vein with varicose pelvic veins. Axial CT imaging of the pelvis also showed compression of the left common iliac vein by the overlying right common iliac artery consistent with May–Thurner syndrome without signs of venous thrombosis. Conclusion: Contrast-enhanced CT is the best imaging modality for suspected vascular compression syndromes. CT findings showed a combination of anterior and posterior nutcracker syndrome in the left circumaortic renal vein, associated with May–Thurner syndrome, which has not previously been described in the literature.

Duodenal varices are ectopic varices that can cause severe and life-threatening gastrointestinal bleeding. Diagnosis and treatment of ectopic varices is challenging, because endoscopy is often unproductive in detecting and treating ectopic varices. Interventional radiology appears as an alternative in this setting, thanks to its important role in treating the bleeding caused by ectopic varices and in preventing rebleedings. We present an interesting case of bleeding ectopic varices in a male 62-years-old cirrhotic patient (Child-Pugh B8). The patient presented with hemorrhagic shock caused by massive melena. CT angiography showed intraluminal blood and identified the source of bleeding as an ectopic varicose vein draining into the superior mesenteric vein. Interventional radiology approach was the only applicable one to reach and effectively treat the source and cause of bleeding by TIPS placement and embolization of the collateral feeding at the same session

Among Uterine Artery Embolization (UAE) complications, vaginal discharge is considered very frequent, especially for submucosal fibroids. Until now, it was reported as clear odorless viscous material. To our knowledge, we describe the first report of intrauterine microsphere migration after UAE. A 45-year-old-woman was admitted to our hospital complaining metromenorrhagia, menstrual cramping, pelvic pain and dyspareunia. After a preprocedural Magnetic Resonance Imaging (MRI) study, she underwent a superselective transradial UAE using 500-700 µm and 700-900 µm microspheres with a good morphological results. At 2-month follow-up, she complained viscous vaginal discharge with “strange pink stiff sphere of about 2 mm in diameter”. A post-procedural-MRI showed fibroid migration towards the uterine cavity, a frequent occurrence especially for submucosal fibroids. The microspheres transvascular migration outside the vessel wall was already reported in a study performed in sheep. Our hypothesis is that the phenomenon of transvascular migration along with the fibroid migration towards the uterine cavity after UAE, may have led the migration of the microspheres directly into the vaginal cavity causing chronic vaginal discharge with pink stiff sphere. Vaginal discharge mixed with “sphere of particles” should be included among the UAE late complication especially for intramural and/or submucosal fibroids with distance to the endometrium less than 2.4 mm at pre-procedural MRI.

Abstract Therapy of uveal melanoma aims to preserve the eye and its function and to avoid metastatic dissemination. The treatment choice is difficult and must keep into account several factors; the therapeutic strategy of uveal melanoma should therefore be personalized, sometimes requiring to combine different treatment techniques. Nowadays globe-sparing radiotherapy techniques are often preferred to enucleation. Plaque brachytherapy, the most commonly used eye-preserving therapy, is suitable for small- and medium-sized uveal melanomas. Proton beam radiotherapy is indicated for tumours with noticeable size, challenging shape and location, but is more expensive and less available than brachytherapy. Enucleation is currently restricted to advanced tumours, uveal melanomas with orbital or optic nerve involvement, blind and painful eyes because of treatment-related complications (neovascular glaucoma, chronic inflammatory processes). The effect of proton beam therapy on neoplastic tissue is related to direct cytotoxic action of the radiations, impairment of neoplastic vascular supply and immunologic response. Complications after radiotherapy are frequent and numerous and mainly related to tumour thickness, radiation dose and distance between the tumour and optic nerve. The purpose of this pictorial review is to provide the radiologists with awareness about diagnostic methods and therapeutic options of uveal melanoma. In the present second section, we discuss the therapeutic management of uveal melanoma, describing the main ocular-conserving radiotherapic techniques. We subsequently present an overview of the effects of radiations on neoplastic tissue. Lastly, we review ocular complications following radiotherapy that should be evaluated by radiologists during follow-up MRI examinations.

Abstract Uveal melanoma is a malignant neoplasm that derives from pigmented melanocytes of the uvea and involves, in order of decreasing prevalence, the choroid, ciliary body and iris. Its prognosis is related to histopathologic and genetic features, tumor size and location, extraocular extension. The diagnosis is fundamentally based on clinical evaluation (ophthalmoscopy, biomicroscopy) and ultrasonography. MRI is useful in case of untransparent lens or subretinal effusion. Moreover, MRI has a significant role to confirm the diagnosis, in the evaluation of the local extent of the disease with implications for treatment planning, and in the follow-up after radiotherapy treatment. Uveal melanoma can show different morphologic features (lentiform, dome or mushroom shape) and often determines retinal detachment. MR appearance of uveal melanoma mainly depends on the melanin content. Uveal melanoma typically displays high signal intensity on T1-weighted images and low signal intensity on T2-weighted images. Nevertheless, imaging appearance may be variable based on the degree of pigmentation and the presence of areas of necrosis or cavitation. Differential diagnosis includes other uveal lesions. The radiologists and in particular MRI play a significant role in the clinical management of uveal melanoma. The purpose of this pictorial review is to provide the radiologists with awareness about diagnostic methods and therapeutic options of uveal melanoma. In the present first section we summarize the MR anatomy of the eye and describe ophthalmological and radiological imaging techniques to diagnose uveal melanomas, with emphasis on the role of MR imaging. Additionally, we review MR imaging appearance of uveal melanomas.

Madelung's disease or multiple symmetric lipomatosis (MSL) is a rare benign disease characterized by abnormal, multiple and symmetric fat depositions in the subcutaneous layer, involving head, neck, back, trunk and also upper and lower limbs.MSL may be related to alcohol abuse or metabolic disorders; it may be both silent or clinically manifest.We describe a case of a 48-yo man with β-thalassemia admitted to medicine department for neck swelling without fever or respiratory symptoms. Patient denied a history of alcoholism and laboratory exam excluded metabolic disorders.Doppler ultrasound, contrast Enhanced-CT and Magnetic Resonance Imaging exams of the neck showed a symmetric, non-encapsulated fat deposition causing extrinsic compression of the right jugular vein without thrombosis. Once excluded the possibility of malignancy, patient's history, clinical, and radiological findings suggest the diagnosis of non–alcohol-related MSL disease.Knowing MSL imaging findings and its degree is crucial to guide towards the right management.Our patient did not require surgical treatment and an US follow-up is needed in order to detect any possible evolution.

Haemobilia is defined as bleeding from the biliary system due to abnormal communication between a blood vessel and the bile ducts. Melena or hematemesis, abdominal pain and jaundice represent the pathognomonic triad for haemobilia, but clinical presentation and aetiology of this entity are extremely variable. We report a case of a 50-year-old man with melena and anaemia and a clinical history of multivalvular endocarditis in which an extremely rare presence of 2 uncommon causes of haemobilia was found, such as a mycotic pseudoaneurysm and a giant hepatic haemangioma, both treated by transarterial embolization. In the management of haemobilia, TAE has been proven to be the treatment of choice because it combines a diagnostic angiography with therapeutic intervention in a minimally invasive, safe and effective way. Physician and radiologist should keep in mind also the uncommon aetiologies of haemobilia, knowing that the source of bleeding could be more than just one.

Abstract Background Posterior nutcracker syndrome is defined by the compression of the left renal vein between the abdominal aorta and a lumbar vertebral body. It can be clinically manifest with intermittent hematuria, gonadal or spermatic reflux resulting in varicocele. Ultrasound is the first-line imaging which require more accurate study with contrast-enhanced computed tomography. Management can be conservative in younger patients with mild hematuria due to the high spontaneous remission rate and invasive with open surgical and endovascular interventions. We describe a very rare case with compression of the left renal vein due to an osteophyte of the spine. Case presentation A 62-year-old Caucasic male came to our radiology department for chronic hepatitis B virus (HBV)-related liver disease follow-up and mild scrotal pain. The ultrasound examination revealed a compression of the left retro-aortic renal vein in the aorto-vertebral space caused by an osteophyte. Duplex Doppler ultrasound revealed flow congestion in the left renal vein and renal failure; power Doppler ultrasound showed left varicocele. Conclusions Doppler ultrasound is the first-line imaging and allows the detection of all the typical signs of posterior nutcracker: left renal vein stenosis, flow congestion and renal failure. Nutcracker syndrome should be suspected in older patients with left varicocele associated with hematuria. Failure to diagnose and treat these patients could have serious consequences for their health.

Abstract Vascular compression syndromes are rare alterations that have in common the compression of an arterial and/or venous vessel by contiguous structures and can be congenital or acquired. The best known are the Thoracic Outlet Syndrome, Nutcracker Syndrome, May–Thurner Syndrome, and Dunbar Syndrome. The incidence of these pathologies is certainly underestimated due to the non-specific clinical signs and their frequent asymptomaticity. Being a first-level method, Ultrasound plays a very important role in identifying these alterations, almost always allowing a complete diagnostic classification. If in expert hands, this method can significantly contribute to the reduction of false negatives, especially in the asymptomatic population, where the finding of the aforementioned pathologies often happens randomly following routine checks. In this review, we briefly discuss the best known vascular changes, the corresponding ultrasound anatomy, and typical ultrasound patterns.

Fibromyalgia (FM) is a common rheumatologic disorder characterised by widespread muscular pain. Myalgia is also a common clinical feature in Connective Tissue Disease (CTD), and FM should be studied for the concomitant presence of a CTD. The aim of this study is to evaluate the prevalence of Myositis-Specific and Myositis-Associated Antibodies (MSA/MAA) in a cohort of FM patients. We enrolled 233 consecutive FM patients (defined according to the 2016 criteria) that did not report clinical signs of autoimmune disorders and followed them for at least one year. The patients were tested for MSA/MAA with immunoblotting. FM patients were seropositive for Antinuclear Antibodies (ANA) in 24% of cases, for MSA in 9%, and for MAA in 6%. A specific diagnosis of CTD was made in 12 patients (5.2%), namely, 5 cases of primary Sjögren’s Syndrome and 7 of Idiopathic Inflammatory Myopathy. Seropositive patients showed clinical features similar to those who were seronegative at baseline. A CTD diagnosis was associated with ANA positivity (p = 0.03, X2 4.9), the presence of a speckled pattern (p = 0.02, X2 5.3), positivity for MAA (p = 0.004, X2 8.1), and MSA (p = 0.003, X2 9.2). In conclusion, a non-negligible proportion of FM patients may be seropositive for MSA/MAA, and that seropositivity might suggest a diagnosis of CTD.

While surgery is considered the main treatment for early-stage rectal cancer, locally advanced rectal cancer needs to be handled with a multidisciplinary approach. Based on literature data suggesting promising advantages of total neoadjuvant therapy (TNT), we performed a retrospective, single-arm, single-center study on 45 patients affected by histologically and radiologically proven locally advanced rectal cancer, with the aim of analyzing the feasibility and short-term efficacy of an integrated intensified treatment in the setting of TNT. Each analyzed patient performed three cycles of FOLFOX4 or De Gramont induction chemotherapy (iCT), followed by concurrent chemoradiotherapy (CRT) with long course radiotherapy (LCRT) plus concomitant boost and continuous 5-FU infusion, followed by three cycles of FOLFOX4 or De Gramont consolidation chemotherapy (conCT) and then surgery with total mesorectal excision. At a median follow-up of 30 months, this strategy has shown to be feasible and effective in terms of pathological complete response (pCR) and short-term disease-free survival (DFS).

Among the multiple clinical manifestations of systemic lupus erythematosus, spontaneous bleedings are rare but clinically important events. They could be potentially fatal, if not promptly treated.The appropriate diagnosis, followed by the timely treatment of these rare clinical presentations, is essential to prevent their lethal consequences.The purpose of this paper is to describe the diagnostic features and the endovascular treatment of 2 cases of spontaneous bleeding—respectively occurred in a 42-year-old woman with abdominal pain and melena, and in a 33-year-old woman with an extensive and painful hematoma in the left axillary region.The timely endovascular treatment—performed by a minimally invasive approach of super-selective percutaneous embolization—has allowed an immediate clinical improvement, avoiding major surgery.

Abstract Several imaging findings of thoracic diseases have been referred—on chest radiographs or CT scans—to signs, symbols, or naturalistic images. Most of these imaging findings include the air bronchogram sign, the air crescent sign, the arcade-like sign, the atoll sign, the cheerios sign, the crazy paving appearance, the comet-tail sign, the darkus bronchus sign, the doughnut sign, the pattern of eggshell calcifications, the feeding vessel sign, the finger-in-gloove sign, the galaxy sign, the ginkgo leaf sign, the Golden-S sign, the halo sign, the headcheese sign, the honeycombing appearance, the interface sign, the knuckle sign, the monod sign, the mosaic attenuation, the Oreo-cookie sign, the polo-mint sign, the presence of popcorn calcifications, the positive bronchus sign, the railway track appearance, the scimitar sign, the signet ring sign, the snowstorm sign, the sunburst sign, the tree-in-bud distribution, and the tram truck line appearance. These associations are very helpful for radiologists and non-radiologists and increase learning and assimilation of concepts. Therefore, the aim of this pictorial review is to highlight the main thoracic imaging findings that may be associated with signs, symbols, or naturalistic images: an “iconographic” glossary of terms used for thoracic imaging is reproduced—placing side by side radiological features and naturalistic figures, symbols, and schematic drawings.

Abstract Due to the growing use of cross-sectional imaging in emergency departments, acute gynaecologic disorders are increasingly diagnosed on urgent multidetector computed tomography (CT) studies, often requested under alternative presumptive diagnoses in reproductive-age women. If clinical conditions and state-of-the-art scanner availability permit, magnetic resonance imaging (MRI) is superior to CT due to its more in-depth characterisationof abnormal or inconclusive gynaecological findings, owing to excellent soft-tissue contrast, intrinsic multiplanar capabilities and lack of ionising radiation. This pictorial review aims to provide radiologists with a thorough familiarity with gynaecologic emergencies by illustrating their CT and MRI appearances, in order to provide a timely and correct imaging diagnosis. Specifically, this second instalment reviews with examples and emphasis on differential diagnosis the main non-pregnancy-related uterine emergencies (including endometrial polyps, degenerated leiomyomas and uterine inversion) and the spectrum of pelvic inflammatory disease.

Abstract Acute gynaecologic disorders are commonly encountered in daily clinical practice of emergency departments (ED) and predominantly occur in reproductive-age women. Since clinical presentation may be nonspecific and physical findings are often inconclusive, imaging is required for a timely and accurate diagnosis. Although ultrasound is the ideal non-invasive first-line technique, nowadays multidetector computed tomography (CT) is extensively used in the ED, particularly when a non-gynaecologic disorder is suspected and differential diagnosis from gastrointestinal and urologic diseases is needed. As a result, CT often provides the first diagnosis of female genital emergencies. If clinical conditions and scanner availability permit, magnetic resonance imaging (MRI) is superior to CT for further characterisation of gynaecologic abnormalities, due to the excellent soft-tissue contrast, intrinsic multiplanar capabilities and lack of ionising radiation. The purpose of this pictorial review is to provide radiologists with a thorough familiarity with gynaecologic emergencies by illustrating their cross-sectional imaging appearances. The present first section will review the CT and MRI findings of corpus luteum and haemorrhagic ovarian cysts, gynaecologic haemoperitoneum (from either ruptured corpus luteum or ectopic pregnancy) and adnexal torsion, with an emphasis on differential diagnosis. Additionally, comprehensive and time-efficient MRI acquisition protocols are provided.

Abstract Background Fibrosing, non-idiopathic pulmonary fibrosis (non-IPF) interstitial lung diseases (fILDs) are a heterogeneous group of diseases characterized by a different amount of inflammation and fibrosis. Therapy is currently based on corticosteroids and/or immunomodulators. However, response to these therapies is highly variable, sometimes without meaningful improvement, especially in more fibrosing forms. Pirfenidone and nintedanib have recently demonstrated to reduce functional decline in patients with IPF. However, their antifibrotic mechanism makes these two drugs an interesting approach for treatment of fibrosing ILDs other than IPF. Objectives We here report our experience with antifibrotic drugs in fibrosing non-IPF ILDs patients having a progressive phenotype during immunosuppressive therapy. Methods Patients with a multidisciplinary team diagnosis of fibrosing non-IPF ILDs experiencing a progressive phenotype during treatment with corticosteroids and/or immunomodulators between October-2014 and January-2018 at our tertiary referral Center for ILDs were retrospectively analyzed. Antifibrotic therapy was administered after application with the respective health insurance company and after consent by the patient. Pulmonary-function-tests and follow-up visits were performed every 6 ± 1 months. Results Eleven patients were treated with antifibrotic drugs (8 males, mean age 62 ± 12.8 years, mean FVC% 62.8 ± 22.3, mean DLCO% 35.5 ± 10.7, median follow-up under antifibrotic treatment 11.1 months). Patients had a diagnosis of unclassifiable ILD in 6 cases, pleuroparenchymal fibroelastosis in 2 cases, idiopathic-NSIP in 1 case, asbestos-related ILD in 1 case and Hermansky-Pudlak syndrome in 1 case. Treatment before antifibrotics consisted of corticosteroids in all patients: 5 combined with Azathioprin, 1 with either methotrexate or cyclophosphamide (i.v.). Ten patients were treated with pirfenidone (2403 mg/die) and 1 with nintedanib (300 mg/die). Median FVC was 56, 56, 50%, at time points − 24, − 12, − 6 before initiation, 44% at time of initiation and 46.5% at 6 months after initiation of antifibrotic treatment. Antifibrotic treatment was generally well tolerated with a need of dose reduction in 2 cases (rash and nausea) and early termination in 3 cases. Conclusions Antifibrotic treatment may be a valuable treatment option in patients with progressive fibrosing non-IPF ILD if currently no other treatment options exist. However, prospective, randomized clinical trials are urgently needed to assess the real impact of antifibrotic therapy in these patients.

Background: Interstitial lung diseases (ILDs) are often associated with rheumatic diseases. Their early diagnosis and management are not only difficult, but also crucial, because they are associated with major morbidity and mortality and can be the first cause of death in autoimmune rheumatic diseases (ARDs).Objectives: By using methodologies, such as Nominal Group Technique (NGT) and Delphi Survey, the aims of this study were (1) to measure consensus between pulmonologists, radiologists, and rheumatologists experienced in the management of ARD-ILD; (2) to highlight the importance of a multidisciplinary approach; and (3) to provide clinicians with a practical tool aimed at improving the prompt recognition and follow-up of ILD associated with ARDs and of any possible rheumatic conditions underlying ILD.Results: During the NGT round, the Steering Committee defined 57 statements to be used in the Delphi survey. A total of 78 experts participated in the Delphi survey, namely 28 pulmonologists, 33 rheumatologists, and 17 radiologists. During this round, consensus on agreement was reached in 47 statements, while disagreement was not reached in any statements. A secondary questionnaire was drafted by the Steering Committee to obtain clearer indications on ILD-ARD “red-flags” and follow-up. Delphi Panelists took part also in the second-questionnaire survey. Answers from both surveys were used to draft two checklists of “red flags” sign or symptom suggestive of ILD and ARD, respectively, and two checklists on identification and monitoring of rheumatoid arthritis (RA) and systemic sclerosis (SSc) ILD.Limitations: This study is a consensus work, which cannot produce empiric data, and is limited to the Italian scenario.Conclusions: This work showed a high level of agreement, but also shows some divergent opinions between different experts. This underlines the importance of a multidisciplinary approach. Eventually, we believe the drafted checklists can help clinicians in the diagnosis and follow-up of ILD-ARD.

Polymyositis and dermatomyositis are autoimmune idiopathic systemic inflammatory diseases, characterized by various degrees of muscle inflammation and typical cutaneous lesions—the latter found in dermatomyositis. The underlying pathogenesis is characterized by a high level of uncertainty, and recent studies suggest diseases may have different immunopathological mechanisms. In polymyositis, components of the cellular immune system are involved, whereas in dermatomyositis, the pathogenesis is mainly mediated by the humoral immune response. The interstitial lung disease occurs in one-third of polymyositis and dermatomyositis patients associated with worse outcomes, showing an estimated excess mortality rate of around 40%. Lung involvement may also appear, such as a complication of muscle weakness, mainly represented by aspiration pneumonia or respiratory insufficiency. The clinical picture is characterized, in most cases, by progressive dyspnea and non-productive cough. In some cases, hemoptysis and chest pain are found. Onset can be acute, sub-acute, or chronic. Pulmonary involvement could be assessed by High Resolution Computed Tomography (HRCT), which may identify early manifestations of diseases. Moreover, Computed Tomography (CT) appearances can be highly variable depending on the positivity of myositis-specific autoantibodies. The most common pathological patterns include fibrotic and cellular nonspecific interstitial pneumonia or organizing pneumonia; major findings observed on HRCT images are represented by consolidations, ground-glass opacities, and reticulations. Other findings include honeycombing, subpleural bands, and traction bronchiectasis. In patients having Anti-ARS Abs, HRCT features may develop with consolidations, ground glass opacities (GGOs), and reticular opacities in the peripheral portions; nonspecific interstitial pneumonia or nonspecific interstitial pneumonia mixed with organizing pneumonia have been reported as the most frequently encountered patterns. In patients with anti-MDA5 Abs, mixed or unclassifiable patterns are frequently observed at imaging. HRCT is a sensitive method that allows one not only to identify disease, but also to monitor the effectiveness of treatment and detect disease progression and/or complications; however, radiological findings are not specific. Therefore, aim of this pictorial essay is to describe clinical and radiological features of interstitial lung diseases associated with polymyositis and dermatomyositis, emphasizing the concept that gold standard for diagnosis and classification–should be based on a multidisciplinary approach.

Abstract 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.

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