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Purpose: This study investigates the accuracy of either computerized tomography (CT) or magnetic resonance imaging (MRI) for the evaluation of various orbital diseases., Methods: We collected 126 CT scans and 65 MRI scans from 144 subjects and asked two radiologists to interpret the images without clinical information. Images included 14 with a clinical diagnosis of orbital infection, 144 with orbital inflammation, and 33 with orbital neoplasm. The inflammatory diseases included thyroid eye disease (TED, n = 69), non-specific orbital inflammation (NSOI, n = 44), IgG4-related disease (IgG4-RD, n = 15), sarcoidosis (Sarcoid, n = 9), granulomatosis with polyangiitis (GPA, n = 5), and Erdheim-Chester disease (ECD, n = 2)., Results: The balanced accuracy (BA) for the two radiologists ranged from 0.87 to 0.90 for cellulitis, 0.81 to 0.86 for inflammation, and 0.82 to 0.85 for neoplasm. Radiologists were excellent at recognizing GPA (BA = 0.98 to 0.99) and very good for TED (BA = 0.80 to 0.86). They also did well identifying IgG4-RD (BA = 0.75 to 0.77), but slightly less well for NSOI (BA = 0.69 to 0.75) and poorly for Sarcoid (BA = 0.48 to 0.50)., Conclusions: CT or MRI scanning contributes to the evaluation of patients with orbital disease, but accuracy does varies based depending on the diagnosis. We could not evaluate issues such as determination of disease activity, variability based on the unit used for imaging or the skills beyond those of our two specialized neuroradiologists. Future studies should directly compare the two imaging modalities and assess the utility of imaging to determine disease activity., Competing Interests: At the time that this study was conceived and data collected, James T. Rosenbaum was a consultant to Janssen, Abbvie, Roche, UpToDate, Novartis, Gilead, UCB, Santen, Horizon, Corvus, Celldex and Eyevensys, and had received research funding from Alcon Research Institute and Pfizer. He is now employed by Corvus Pharmaceuticals and is no longer receiving research support or serving as a consultant. Min Joung Lee is a consultant of Thyroscope. Bobby S. Korn is a consultant to Amgen, Viridian, Immunovant, and has received Royalties from Elsevier. Don O. Kikkawa is a consultant to Amgen (Horizon), Immunovant, Lassen, Thyroscope, and has received Royalties from Elsevier. The following authors have no financial disclosures: Rohan Verma, Bronwyn E. Hamilton, David Pettersson, Dongseok Choi, and Eun Soo Kim.” This does not alter our adherence to PLOS ONE policies on sharing data and materials., (Copyright: © 2024 Lee et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.)

Objective: This study aimed to examine the characteristics of nasal and imaging findings of sinonasal lesions in granulomatosis with polyangiitis (GPA) patients and how these lesions change over time in both the active and remission phases of the disease., Methods: We retrospectively reviewed GPA patients with sinonasal lesions who were followed up at our department between January 2005 and December 2020. The following data were collected: age, sex, symptoms at initial presentation, anti-neutrophil cytoplasmic antibody (ANCA) type, and histopathological, nasal (initial and follow-up), and imaging (initial and follow-up) findings., Results: This study included 17 patients with GPA aged 30 to 79 years. Computed tomography (CT) of the sinuses showed mucosal thickening in 16 patients, bone thickening in 12, bone destruction in 4, and an orbital invasion mass in 3 at the time of diagnosis. After initiating treatment, mucosal thickening of the sinuses improved in 3 of 16 patients and remained unchanged in 13. Bone thickening at the time of diagnosis remained unchanged in 10 of 12 patients and worsened in 2; 1 patient displayed newly developed bone thickening. Destructive nasal findings on CT were positive for proteinase 3-ANCA., Conclusions: Our study revealed that mucosal thickening, bone thickening, bone destruction, and orbital invasion mass were major CT findings in patients with GPA. Intranasal findings such as granulations, crusting, and necrosis were seen in the active phase; moreover, saddle nose, loss of turbinate, and nasal septal perforation were subsequently seen in the course of the disease. Sinonasal findings of GPA vary depending on the disease stage and period., Competing Interests: Declaration of competing interest The authors declare that they have no competing interests., (Copyright © 2024. Published by Elsevier B.V.)

ANCA-associated vasculitides (AAV) comprise granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis. All forms may involve different organ systems, yet kidney and lung involvement are common and fatal in many cases. Here, we aimed to determine the predictive value of pulmonary disease manifestation and individual CT findings in AAV patients. Available CT scans and clinical information on mortality, renal outcomes, occurrence of relapses and damage scores were analysed retrospectively from a tertiary rheumatology center in Germany. We included a total of 94 AAV patients (49 with GPA, 41 with MPA). Forty-four patients had lung involvement with available CT scans, 70.5% of which with GPA and 72.7% with renal involvement. Nodule formation and cavities were more frequent among GPA patients, whereas ground-glass opacities (GGO), ILD and pleural effusion were observed predominantly in MPA patients. Over a median follow-up of 37 months, GPA patients had a slightly higher overall mortality, whereas end-stage kidney failure rates were significantly increased in MPA patients. Relapse frequencies were comparable between both entities. The presence of GGO and pleural effusion were associated with higher relapse rates, whereas nodules were negatively correlated with relapses. Notably, RTX-treated patients had less infections as compared to individuals under different therapies. Our data demonstrate the outstanding importance of characteristic CT patterns in AAV diagnosis assessment. Especially certain CT patterns including GGO and pleura effusion may help to identify patients who are at higher risk for relapsing disease., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Abstract: A 72-year-old man with fever and weakness in both lower limbs underwent thoracolumbar MRI and 18 F-FDG PET/CT. The PET/CT scan revealed diffused FDG uptake along the spinal dura mater from T7 to S2 level like a "bottle brush." Pathologic examination after biopsy of spinal canal lesions manifested granulomatous inflammation. The blood test showed cytoplasmic antineutrophil cytoplasmic antibody (ANCA) and myeloperoxidase-ANCAs were positive, whereas the perinuclear ANCA was negative. Eventually, he was diagnosed with granulomatosis with polyangiitis., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Small artery vasculitis of the CNS is a rare and serious condition characterized by the inflammation of blood vessels within the brain and spinal cord. There are two groups of small artery vasculitis determined by the presence or absence of immunoglobulin complex deposition in the vessel wall. The former includes anti-glomerular basement membrane disease, cryoglobulinemic vasculitis, and IgA vasculitis. Absence of immune complex deposition is associated with anti-neutrophil cytoplasmic antibody (ANCA) and includes microscopic polyangiitis, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, and primary angiitis of the CNS. These conditions present a diagnostic challenge in which imaging plays a crucial role., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: The pulmonary involvement in patients with microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) is well known at disease onset but data during follow-up (after the induction regimen and when the first relapse occurs) are limited. Our goal was to analyze chest high-resolution computed tomography (HRCT) findings of (ANCA)-associated vasculitis patients., Method: All consecutive unselected AAV patients over eighteen with positive ANCA status and with HRCT chest performed at the diagnosis were retrospectively enrolled between 2004 and 2019 at the Toulouse University Hospital (France). Two experienced pulmonologists and one expert respiratory radiologist reviewed independently HRCT chest scans., Results: A total of 157 AAV patients were included in the study. Two-thirds of AAV patients had pulmonary involvement at diagnosis. Diffuse alveolar hemorrhage (DAH) was observed in 31.2 % of cases, nodules and masses in 18.5 %, bronchial airway involvement in 13.4 %, and interstitial involvement in 12.7 %. Following the induction regimen, chest HRCT scans over a two-year period demonstrated significant improvement in DAH and nodular manifestations, whereas bronchial airway involvement exhibited variability and half of cases of interstitial lung disease (ILD) had progressive course. Outcomes and survival rates are better for nodular and bronchial involvement. DAH was the most frequent cause of deaths. Progressive fibrotic changes in ILD over time could impact prognosis despite AAV remission., Conclusion: Employing a pattern-based approach with HRCT chest scans to assess lung involvement could be valuable in predicting treatment response, relapse, mortality, and could improved the management of AAV patients., Competing Interests: Declaration of Competing Interest TV has received consulting fees from Boeringer Ingelheim. SF has received consulting fees for Abionyx, Pharma, CSL-Vifor, Sanofi-Genzyme, Novartis SA, Alexion, Baxter. GPr, GPu and SC have nothing to disclose., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: Granulomatosis with polyangiitis (GPA) is characterized by necrotizing granulomatous vasculitis involving small-sized vessels in the upper airways, lower airways, and kidneys. Renal pathology is usually characterized by focal segmental necrotizing glomerulonephritis, which often leads to rapidly progressive renal failure. This type of renal involvement is usually unapparent on radiography. The presence of a renal mass in a patient with GPA, although extremely rare, is recognizable. Herein, we report a rare case of GPA presenting as a solitary renal mass and present a review of the literature., Case Presentation: A 75-year-old woman presented with a solitary right kidney mass measuring 4 × 3.5 cm detected by enhanced computed tomography. There was no history of sinusitis, rhinitis, cough, or pneumonia suggestive of systemic GPA. Nephrectomy was performed based on the suspicion of renal cell carcinoma or malignant lymphoma. Three months later, she was admitted because her serum creatinine levels increased from 54.81 μmol/L to 405.76 μmol/L accompanied by a high C-reactive protein level of 159 mg/L. Anti-neutrophil cytoplasmic antibodies against myeloperoxidase and anti-proteinase 3 were negative. Histological examinations of the solitary renal mass revealed necrotizing granulomatous arteritis in the cortex and medullary vasa recta, surrounded by interstitial fibrosis, and focal segmental necrotizing glomerulonephritis in the localized lesion; however, signs of vasculitis were not observed in areas other than the solitary mass. Therefore, the patient was diagnosed with granulomatosis with polyangiitis (GPA). Despite treatment with prednisolone (30 mg/day), the patient developed oliguria with an elevation of her serum creatinine level to 583.44 μmol/L, which required hemodialysis within one month after the initiation of steroid therapy. The patient could successfully discontinue hemodialysis 21 months later, following a decrease in her serum creatinine level to 251.06 μmol/L., Conclusions: GPA should be considered as one of the differential diagnoses of a solitary renal mass. Furthermore, patients with solitary renal masses associated with GPA may exhibit a favorable response to steroid or immunosuppressive treatment., (© 2023. The Author(s).)

Background: Although paranasal sinuses are one of the most representative organs affected by eosinophilic granulomatosis with polyangiitis (EGPA), they have not been studied sufficiently. The aim of this study was to compare computed tomography (CT) findings in paranasal sinuses of EGPA with those of other eosinophilic sinus diseases and elucidate the clinical relevance of their severity., Methods: CT findings of paranasal sinuses in EGPA patients prior to therapeutic intervention (n = 30) were evaluated using the Lund-Mackay staging (LMS) system and compared with those of three control diseases [(NSAID-exacerbated respiratory disease (N-ERD), aspirin-tolerant asthma, and eosinophilic chronic rhinosinusitis without asthma (ECRS)]. We divided EGPA patients into three groups based on their LMS scores and examined their association with disease manifestation., Results: Total scores of the LMS system in EGPA were significantly lower than those of N-ERD and ECRS without asthma. There was a large variation in total LMS scores in EGPA, suggesting considerable heterogeneity of their sinus lesions. Although EGPA with low LMS system scores showed only minor findings in maxillary and anterior ethmoid regions, those with high LMS system scores were characterized by high scores in the ostiomeatal complex. However, the frequencies of patients with a Five-Factor Score ≥2 and with cardiac involvement were significantly higher for EGPA with low LMS system scores., Conclusions: Although paranasal sinus lesions in EGPA were less severe than those of other eosinophilic sinus diseases, their milder CT findings may be associated with a higher frequency of extra-respiratory organ involvement., (Copyright © 2023 Japanese Society of Allergology. Published by Elsevier B.V. All rights reserved.)

Objectives: Interstitial lung disease (ILD) has been described as a possible pulmonary involvement in antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitides (AAV), mainly granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). Aim of this cross-sectional Italian national study was to describe demographic, clinical and serological profile of ILD related to MPA and GPA and investigate possible correlations between radiologic patterns of ILD and vasculitis features., Methods: We enrolled 95 consecutive patients with AAV-ILD, 56 affected by MPA (58.9%) and 39 by GPA (41.1%)., Results: NSIP was the most frequently detected ILD pattern, observed in c-ANCA patients in 60.9% of cases, followed by UIP pattern mainly observed in p-ANCA patients (47.7%, p=0.03). ILD represented the first clinical manifestation, preceding vasculitis diagnosis in 22.1% of cases and, globally, ILD was already detectable at AAV diagnosis in 66.3% of patients. The diagnosis of ILD preceded that of AAV in 85.7% of p-ANCA positive-patients, while only one patient with c-ANCA developed ILD before AAV (p= 0.039). Multivariate analysis confirmed the correlation of UIP pattern with p-ANCA-positivity and a diagnosis of ILD before AAV, also when adjusted for age and sex., Conclusions: Our study confirms that UIP is a frequent pattern of lung disease in AAVILD patients. Our results also suggest that ILD can represent an early complication of AAV but also occur in the course of the disease, suggesting the need of a careful evaluation by both pulmonologist and rheumatologist to achieve an early diagnosis. Further prospective studies are needed to define ILD prevalence and evolution in AAV patients.

Objectives: Patients affected by eosinophilic granulomatosis with polyangiitis (EGPA) display an increased risk of atherothrombotic events compared with the general population. An increased frequency of subclinical markers of atherosclerosis has been observed in other ANCA-associated vasculitis, but no specific study focused on EGPA. We therefore evaluated subclinical atherosclerosis in EGPA patients and in a control population., Methods: Forty EGPA patients and 80 controls matched by age, sex and traditional cardiovascular risk factors underwent sonographic assessment of common carotid artery (CCA) intima-media thickness (IMT). The presence of plaques of the CCA was also investigated. The correlation between CCA-IMT and clinical and laboratory features was also assessed., Results: Median CCA-IMT was significantly higher in EGPA patients compared with controls (P = 0.002). Also, the proportion of subjects with increased CCA-IMT and with presence of plaques was significantly higher among EGPA patients (P < 0.001 for both). Moreover, within the EGPA cohort, CCA-IMT tended to increase with disease duration (P = 0.034) and corticosteroid cumulative dose (P = 0.004). No significant associations were found between CCA-IMT, ANCA status, other clinical features and therapeutic regimens. Notably, the prevalence of traditional cardiovascular risk factors was comparable in patients with vs without an increased CCA-IMT., Conclusion: Ultrasound markers of subclinical atherosclerosis are increased in EGPA patients as compared with controls, independently of traditional cardiovascular risk factors., (© The Author(s) 2022. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Granulomatosis with polyangiitis (GPA) is a multisystemic autoimmune small vessel vasculitis predominantly affecting the respiratory and renal systems. Other systems such as the central nervous system, orbital, cardiac and gastrointestinal systems may also be involved to a lesser degree. Although there are no imaging features that are pathognomonic for GPA, there are known radiological patterns suggestive of the disease and imaging plays an important role in diagnosis, assessment and monitoring of disease activity. This is more evident when combined with clinical features, biochemical values and histopathology results. This pictorial review aims to present both common and uncommon radiological features of GPA., (© 2022 The Authors. Journal of Medical Imaging and Radiation Oncology published by John Wiley & Sons Australia, Ltd on behalf of Royal Australian and New Zealand College of Radiologists.)

Competing Interests: The authors declare that they have no conflict of interest.

Background: In patients with tracheobronchial involvement, the differential diagnosis between granulomatosis with polyangiitis (GPA) and relapsing polychondritis (RP) can be challenging. The aim of this study was to describe the characteristics of airway abnormalities on chest computed tomography (CT) in patients with GPA or RP and to determine whether specific imaging criteria could be used to differentiate them., Methods: GPA and RP patients with tracheobronchial involvement referred to a national referral center from 2008 to 2020 were evaluated. Their chest CT images were reviewed by two radiologists who were blinded to the final diagnosis in order to analyze the characteristics of airway involvement. The association between imaging features and a diagnosis of GPA rather than RP was analyzed using a generalized linear regression model., Results: Chest CTs from 26 GPA and 19 RP patients were analyzed. Involvement of the subglottic trachea (odds ratio for GPA=28.56 [95% CI: 3.17; 847.63]; P=0.001) and extensive airway involvement (odds ratio for GPA=0.02 [95% CI: 0.00; 0.43]; P=0.008) were the two independent CT features that differentiated GPA from RP in multivariate analysis. Tracheal thickening sparing the posterior membrane was significantly associated to RP (odds ratio for GPA=0.09 [95% CI: 0.02; 0.39]; P=0.003) but only in the univariate analysis and suffered from only moderate interobserver agreement (kappa=0.55). Tracheal calcifications were also associated with RP only in the univariate analysis (odds ratio for GPA=0.21 [95% CI: 0.05; 0.78]; P=0.045)., Conclusion: The presence of subglottic involvement and diffuse airway involvement are the two most relevant criteria in differentiating between GPA and RP on chest CT. Although generally considered to be a highly suggestive sign of RP, posterior tracheal membrane sparing is a nonspecific and an overly subjective sign., (© 2022. The Author(s).)

Methotrexate-associated lymphoproliferative disorders (MTX-LPD) frequently involve the extranodal organs throughout the body. Among the extranodal occurrences of MTX-LPD, pulmonary involvement is most frequent. In contrast, there are only a few reports of MTX-LPD in the nasal cavity or paranasal sinuses. Moreover, there are no previous reports of MTX-LPD mimicking granulomatosis with polyangiitis (GPA) in imaging examinations. We describe a case of a 53-year-old woman with MTX-LPD mimicking GPA in the nasal cavity and lungs. She complained of left nasal obstruction and discharge, general fatigue, and continual fever for 2 months. The patient had been diagnosed with rheumatoid arthritis and received methotrexate (MTX) for over 10 years. Contrast-enhanced computed tomography revealed unenhanced masses in the nasal cavity and multiple masses with cavitary changes in the bilateral lungs, suggesting GPA. However, histological examination of the nasal lesion and a history of MTX treatment indicated a diffuse large B-cell lymphoma type MTX-LPD. Two weeks after MTX withdrawal, prominent improvements in both lesions were observed. Complete regression of the nasal lesion was observed 3 months after discontinuation of MTX. Thus, MTX-LPD may mimic GPA in imaging examinations.

Eosinophilic granulomatosis with polyangiitis (EGPA) is often associated with peripheral neuropathy, but reports of central nervous system involvement are quite rare. We herein report a patient with EGPA first identified as having hypereosinophilia who later developed asthma, eosinophilic otitis media, sinusitis, and hemorrhagic colitis. She subsequently developed hemiparesis. Head magnetic resonance imaging revealed multiple cerebral infarctions with subcortical and subarachnoid hemorrhaging colocalized at the bilateral border zone areas. She was diagnosed with EGPA-induced stroke and successfully treated with oral prednisolone. Inflammation in the small cerebral arteries in EGPA may induce bilateral border zone infarction with colocalizing subcortical and subarachnoid hemorrhaging.

Competing Interests: None

Hypertrophic pachymeningitis (HP) presents with thickening of the dura mater in the cerebrum and spine, and its symptoms vary depending on the affected location. The association of HP with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis has been recognized, and most cases are complicated by granulomatosis with polyangiitis. We report the case of a 47-year-old man who presented with HP upon relapse of eosinophilic granulomatosis with polyangiitis (EGPA), with literature review. He presented with disturbance of consciousness, and magnetic resonance imaging (MRI) revealed thickening of the dura mater around the left parietal lobe. Although myeloperoxidase (MPO)-ANCA was positive on EGPA diagnosis, the elevation of MPO-ANCA was not documented at the onset of HP. Brain perfusion scintigraphy showed an increase in blood flow in the left parietal lobe and temporal lobe, and electroencephalogram (EEG) revealed slow waves in the left parietal lobe. He was treated with a high dose of corticosteroid and rituximab, and the slow waves on EEG and brain perfusion were normalized. Although the most frequent symptom of HP is headache, disturbance of consciousness can be the manifestation of HP, and inflammation of HP could affect the cerebral parenchyma, which can be documented as abnormal EEG and perfusion scintigraphy. Literature review revealed that most of the HP in EGPA developed when EGPA relapsed, and was observed in patients with MPO-ANCA positivity. HP develops without evidence of other clinical features of EGPA; therefore, adequate imaging, including contrast-enhanced MRI, is necessary. Rituximab may be effective for treating HP complicated with EGPA.

Abstract: A 57-year-old woman was referred for progressive dyspnea. CT showed a tracheal mass, suspicious of tracheal neoplasm. The lesion was partially resected, and nonspecific granulation tissue was observed on histology. Her symptoms and CT findings worsened. FDG PET/CT showed increased FDG accumulation in the nasal septum and left eustachian tube in addition to the tracheobronchial lesions. Although the patient was ANCA (antineutrophil cytoplasmic antibodies) negative, a differential diagnosis of granulomatosis with polyangiitis was established and confirmed pathologically. FDG PET/CT was useful for diagnosis of ANCA-negative granulomatosis with polyangiitis, in which tracheobronchial and cartilage lesions were prominent., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Granulomatosis with polyangiitis (GPA) is an antineutrophil cytoplasmic antibody-associated vasculitis. It is an uncommon multisystem disease involving predominantly small vessels and is characterized by granulomatous inflammation, pauci-immune necrotizing glomerulonephritis, and vasculitis. GPA can involve virtually any organ. Clinical manifestations are heterogeneous and can be classified as granulomatous (eg, ear, nose, and throat disease; lung nodules or masses; retro-orbital tumors; pachymeningitis) or vasculitic (eg, glomerulonephritis, alveolar hemorrhage, mononeuritis multiplex, scleritis). The diagnosis of GPA relies on a combination of clinical findings, imaging study results, laboratory test results, serologic markers, and histopathologic results. Radiology has a crucial role in the diagnosis and follow-up of patients with GPA. CT and MRI are the primary imaging modalities used to evaluate GPA manifestations, allowing the differentiation of GPA from other diseases that could simulate GPA. The authors review the main clinical, histopathologic, and imaging features of GPA to address the differential diagnosis in the affected organs and provide a panoramic picture of the protean manifestations of this infrequent disease. The heterogeneous manifestations of GPA pose a significant challenge in the diagnosis of this rare condition. By recognizing the common and unusual imaging findings, radiologists play an important role in the diagnosis and follow-up of patients with GPA and aid clinicians in the differentiation of disease activity versus disease-induced damage, which ultimately affects therapeutic decisions. Online supplemental material is available for this article. © RSNA, 2021.

Objective: To define the pattern of cardiac involvement in eosinophilic granulomatosis and polyangiitis (EGPA) and propose an algorithm for heart disease screening., Methods: This was a retrospective study of EGPA patients attending a specialized vasculitis clinic (1989-2016). Clinical characteristics and cardiovascular evaluation (CE) results (serum troponin, ECG, echocardiography and cardiac magnetic resonance) were collected and compared according to symptoms and inflammatory cardiac disease (ICD)., Results: A total of 131 EGPA patients were included, of whom 96 (73%) had undergone CE. The median (interquartile range) age was 50 (38-58) years and 36% showed ANCA+. Asthma preceded diagnosis by a median of 97 (36-240) months. Among the 96 patients who underwent CE, 43% were symptomatic, with dyspnea (47%) and chest pain (29%) being the predominant symptoms. In asymptomatic patients, CE reported abnormalities in 45% of cases, with a subsequent earlier diagnosis (4 vs 11 months). Overall, 27 patients had EGPA-related ICD (EGPA-rICD) that was already present at diagnosis in 20 cases, preceded it in 2 cases and developed later in 5 cases. EGPA-rICD patients were younger (46 vs 50 years; P = 0.04), had more frequently abnormal ECG (30.8 vs 2.1%; P < 0.001), negative ANCA (85 vs 69%; NS), higher BVAS score (3 vs 1; P = 0.005), higher eosinophil count (5.60 vs 1.60 × 109/l; P = 0.029) and higher CRP (52 vs 15 mg/l; P = 0.017). Overall, 11% of cases with EGPA-rICD were asymptomatic., Conclusion: In our study, 45% of asymptomatic patients had an abnormal baseline cardiac evaluation, which allowed an earlier diagnosis of cardiac disease. We recommend prompt cardiac screening in all EGPA patients, instead of a symptoms-guided algorithm., (© Crown copyright 2021.)

Introduction: Granulomatosis with polyangiitis (GPA) is an autoimmune disease leading to necrotizing lesions in the affected tissues. Computed tomography (CT) of paranasal sinuses reveals multiple lesions in patients with GPA, for example, sinus opacification, bone / cartilage destruction, and neoosteogenesis., Objectives: We aimed to describe and compare CT lesions found in patients with GPA and those with chronic rhinosinusitis (CRS) and to propose a new radiological marker of GPA-nasal strands., Patients and Methods: This retrospective study (2014-2019) included 53 patients with GPA (22 men, 31 women) at a median (interquartile range) age of 45 (34-60) years. Computed tomography findings of mucosal lesions in paranasal sinuses, neoosteogenesis, bony and cartilaginous lesions, and nasal strands were analyzed. Nasal strands were described as intermucosal adhesions resembling bands. A total of 71 patients with CRS (reference group) were assessed for the presence of the same parameters., Results: Computed tomography scans showed mucosal lesions in the sinuses of 35 patients (66%) with GPA. Nasal septum perforation was observed in 19 patients (35.8%), neoosteogenesis in 17 (32.1%), and bone damage in 14 (26.4%). External nose deformity was present in 16 patients (30.2%). Nasal strands on CT were found in 36 patients with GPA (68%) and 32 patients with CRS (45%). The presence of 5 or more strands was more characteristic of GPA than CRS (P <0.001). A positive correlation was found between the number of strands greater than or equal to 5 and the presence of proteinase 3 antineutrophil cytoplasmic antibodies (P = 0.046)., Conclusions: Nasal strands, a parameter reflecting pathologic mucus and atrophic lesions (tissue loss), should have a place in CT evaluation of the nasal cavities in patients suspected of or diagnosed with GPA.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic vasculitis disease involving small-sized vessels. The literature has reported involvement of the central nervous system (CNS) in 5% cases, and isolated CNS involvement is extremely rare. Due to its rarity and scarcity of clinical data, standardized treatment of isolated CNS involvement in EGPA is unclear. Mepolizumab, an anti-interleukin-5 monoclonal antibody, was previously introduced to treat EGPA with longer remission period, more patients showing remission, and reduction in prednisolone dose of those who already taking prednisolone compared to placebo. We describe a case of isolated CNS involvement in EGPA, confirmed by brain biopsy and treated with mepolizumab, which was refractory to conventional immunotherapy., (Copyright © 2021 Elsevier B.V. All rights reserved.)

We report a case of a 32-year-old man with recurrent fever, cough and left lumbago for more than one month. Computed tomography (CT) and magnetic resonance imaging (MRI) found bilateral multiple pulmonary nodules and a tumor-like mass in the left kidney. Fluorine-18-fluorodeoxyglucose positron emission tomography/computed tomography ( 18 F-FDG PET/CT) revealed increased uptake in the right pharyngeal recess along with pulmonary and renal hypermetabolic lesions. The pathologic findings of pulmonary and renal specimens were suggestive of granulomatous inflammatory changes. Further laboratory examinations showed an elevated level of serum cytoplasmic antineutrophil cytoplasmic antibody (c-ANCA) and serum proteinase 3-ANCA (PR3-ANCA). Clinical symptoms were significantly improved, and the size of pulmonary and renal lesions reduced following the use of steroids and cyclophosphamide together. Therefore, a final diagnosis of granulomatosis with polyangiitis (GPA) was made.

Background: Recent evidence suggests that cardiac involvement in patients with granulomatosis with polyangiitis (GPA) occurs more frequently than previously reported. Multimodality cardiac imaging is gaining attention in the diagnosis, prognostication, and follow-up of such patients; however, the data remain scarce., Results: 2D-TTE was useful for initial screening; while both cardiac magnetic resonance imaging and 18 F-fluoro-2-deoxyglucose positron emission tomography/computed tomography ( 18 FDG-PET/CT) metabolic imaging with rubidium-82 PET perfusion imaging were useful for characterization of myocardial disease. 18 FDG-PET/CT was very useful for the follow-up of cardiac disease activity following treatment., Conclusion: 18 FDG-PET/CT is sensitive for the detection of cardiac involvement by GPA and is useful for the tissue characterization and follow-up of disease activity following treatment.

The prevalence of undiagnosed cardiac involvement in granulomatosis with polyangiitis (GPA) is unknown. In this prospective study we investigated the utility of cardiovascular magnetic resonance (CMR) to identify myocardial abnormalities in GPA and their correlation with disease phenotype. Twenty-six patients with GPA and no cardiovascular disease or diabetes mellitus underwent contrast-enhanced CMR, including late gadolinium-enhancement (LGE), T1-mapping for native T1 and extra-cellular volume (ECV) quantification for assessment of myocardial fibrosis, cine imaging and tissue tagging for assessment of left ventricular (LV) function. Twenty-five healthy volunteers (HV) with comparable age, sex, BMI and arterial blood pressure served as controls. Patients with GPA had similar cardiovascular risk profile to HV. A focal, non-ischaemic LGE pattern of fibrosis was detected in 24% of patients and no controls (p = 0.010). Patients with myocardial LGE were less frequently PR3 ANCA (7% vs 93%, p = 0.007), and had involvement of the lower respiratory tract and skin. LGE scar mass was higher in patients presenting with renal involvement. Native T1 and ECV were higher in patients with GPA than HV; ECV was higher in those with relapsing disease, and native T1 was inversely associated with PR3 ANCA (β = - 0.664, p = 0.001). Peak systolic strain was slightly reduced in GPA compared to controls; LV ejection function was inversely correlated with disease duration (β = - 0.454, p = 0.026). Patients with GPA have significant myocardial abnormalities on CMR. ANCA, systemic involvement and disease severity were associated with myocardial fibrosis. CMR could be a useful tool for risk stratification of myocardial involvement in GPA.

Granulomatosis with polyangiitis is a rare autoimmune condition which causes respiratory tract granulomas, small to medium vessel vasculitis and renal disease. Head and neck manifestations are some of the most common presentations of the condition, with a significant proportion of patients experiencing sinonasal disease alone. The recognition of suggestive imaging findings, in combination with clinical history and serology, aids the diagnosis and appropriate treatment. This pictorial review describes and illustrates the head and neck imaging features of granulomatosis with polyangiitis, highlighting the range of CT and MRI findings of upper aerodigestive tract, orbital and skull-base disease. Recognition of the radiological appearances is of importance, since clinical presentations may be non-specific and limited disease may have negative serology. Imaging features may overlap with other pathologies so important differential diagnoses will be considered, and these are particularly relevant in the context of treatment resistance.