CONCLUSIONS: Even if periaortitis secondary to EVAR is a very rare complication, it is important for the surgeon to know this possible rare complication and its characteristics, in order to immediately recognize it and treat it adequately to avoid complications.

Many aspects of IgG4-related diseases were initially described during the late 19th and early 20th century. A variety of clinical presentations caused by this common pathology have been named after the researchers who first described the disorders, such as Mikulicz, Küttner, Riedel or Ormond. However, the initial description of retroperitoneal fibrosis dates back to even 50 years earlier, when in 1846, the Prussian private practitioner Raphael Jakob Kosch described a hitherto unknown constellation of symptoms and pathological findings in a famous patient. This celebrity was the mathematician and astronomer Friedrich Wilhelm Bessel, a close friend of Alexander von Humboldt and Carl Friedrich Gauss.
UNASSIGNED: Zahlreiche Aspekte von IgG4-assoziierten Erkrankungen wurden erstmalig während des späten 19. und frühen 20. Jahrhunderts beschrieben. Verschiedene klinische Manifestationen, verursacht durch gemeinsam zugrundliegende Pathomechanismen, sind nach den Wissenschaftlern benannt worden, die diese Veränderungen als Erste beschrieben, z. B. Mikulicz, Küttner, Riedel oder Ormond. Jedoch datiert die Erstbeschreibung einer Retroperitonealfibrose noch 50 Jahre weiter zurück: Im Jahr 1846 beschrieb der preußische Landarzt Raphael Jakob Kosch eine bis dahin unbekannte Befund- und Symptomenkonstellation bei einem berühmten Patienten. Dies war der Mathematiker und Astronom Friedrich Wilhelm Bessel, ein enger Freund von Alexander von Humboldt und Carl Friedrich Gauss.

OBJECTIVE: ANCA-associated vasculitis (AAV) is currently categorized under the small vessel vasculitides. There is limited knowledge about large vessel involvement in AAV (L-AAV), mainly described in case reports and small series. L-AAV can involve temporal arteries (TA-AAV), aorta (A-AAV), and periaortic soft tissue (PA-AAV). We sought to characterize the features of patients with L-AAV.
METHODS: Patients older than 18 years at diagnosis of TA-AAV, A-AAV and PA-AAV seen at the Mayo Clinic, Rochester between 1 January 2000 and 31 December 2021 were identified through a proprietary medical text search algorithm. Patients were included if diagnosed with L-AAV, fulfilled 2022 ACR/EULAR classification criteria for GPA, MPA or EGPA, had positive ANCA test results, and had more than one outpatient or inpatient visit.
RESULTS: The study cohort consists of 36 patients with L-AAV. Of those, 23 had p-ANCA and/or MPO-ANCA, and 13 had c-ANCA and/or PR3-ANCA. Mean (s.d.) age at AAV diagnosis was 63.4 (12.79) years; 20 (56%) were male. Seventeen patients had TA-AAV, 10 had A-AAV and 9 had PA-AAV. Most patients (n = 25, 69%) were diagnosed with large vessel vasculitis and AAV within a 1-year timespan. Twenty-five (69%) patients had histopathological confirmation of AAV diagnosis in a location other than temporal artery, aorta or periaortic soft tissue. Glucocorticoids (36/36), rituximab (19/36) and methotrexate (18/36) were the most frequent treatments.
CONCLUSIONS: This is the largest single-centre cohort of patients with L-AAV to date. AAV can involve large arteries, albeit infrequent. AAV-targeted therapy should be considered in patients with L-AAV.

Inflammatory abdominal aortic aneurysms (iAAA) are a form of noninfectious aortitis in patients with abdominal aortic aneurysms (AAA). Ultrasound could help to detect iAAA early. This retrospective observational study assessed the potential of using ultrasound to detect iAAA in a case series of iAAA patients, and the diagnostic value of ultrasound to detect iAAA in consecutive patients in a follow-up for AAA, referred to as a feasibility study. In both studies, diagnosis of iAAA was based on a cuff surrounding the aneurysm using CT (golden standard). The case series included 13 patients (age 64 (61; 72) years; 100% male). The feasibility study included 157 patients (age 75 (67; 80) years; 84% male). In the case series, all iAAA patients showed a cuff surrounding the aortic wall on ultrasound. In the feasibility study with AAA patients, ultrasound yielded no cuff in 147 (93.6%; CT negative in all cases), a typic cuff in 8 (5.1%; CT positive in all cases), and an inconclusive cuff in 2 (1.3%; CT negative in both cases) patients. Sensitivity and specificity were 100% and 98.7%, respectively. This study indicates that iAAA can be identified with ultrasound, and safely ruled out. In positive ultrasound cases, additional CT imaging might still be warranted.

Severe acute respiratory syndrome coronavirus type 2 (SARS-CoV-2) has caused a global pandemic resulting in many deaths. As a result, vaccines to prevent the onset of coronavirus disease 2019 (COVID-19) have been developed and have demonstrated high efficacy in large-scale clinical trials. Adverse events that develop within a few days after vaccination are common, such as fever, malaise, body aches, and headaches, and have become widely known as transient reactions. However, as COVID-19 vaccines are administered worldwide, several studies have highlighted that long-term side effects associated with vaccines against SARS-CoV-2 may include serious adverse events. There has been an increase in reports of COVID-19 vaccinations being associated with the onset of autoimmune diseases, such as anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis. This is a report of ANCA-associated vasculitis with periaortitis following the second dose of COVID-19 mRNA vaccination, in which a 56-year-old man developed numbness and pain in his lower extremities three weeks after COVID-19 vaccination. Following the onset of sudden abdominal pain, a fluorodeoxyglucose-positron emission tomography scan revealed periaortic inflammation. Serum myeloperoxidase (MPO)-ANCA levels were significantly elevated, and renal biopsy revealed pauci-immune crescentic glomerulonephritis. Treatment with steroids and cyclophosphamide alleviated abdominal pain and numbness in the lower limbs, resulting in a decrease in MPO-ANCA titers. The side effects of COVID-19 vaccination are still unclear. This report has indicated that side effects associated with vaccines against COVID-19 may include ANCA-associated vasculitis. However, a causal relationship between COVID-19 vaccination and the development of ANCA-associated vasculitis has not yet been clearly demonstrated. COVID-19 vaccination will continue internationally, so it is necessary to accumulate similar case reports in the future.

Periaortitis is a rare vascular manifestation and is often associated with retroperitoneal fibrosis. Herein, we describe a case of periaortitis accompanied by retroperitoneal fibrosis in a patient who developed acute kidney insufficiency due to bilateral ureteral stenosis. Ultrasonography at presentation detected thickness of the outer layer of the bilateral common iliac artery and right internal and external iliac arteries, consistent with periaortitis. Moreover, follow-up ultrasound images revealed subsiding of the thickness of the arterial wall in response to treatment with corticosteroids. Because of its noninvasiveness and handiness, ultrasonography has become popular for the assessment of large vessels in clinical practice, particularly monitoring for affected lesions. Computed tomography, magnetic resonance imaging, and positron emission tomography are currently used for the diagnosis and monitoring of periaortitis, but in this case, ultrasonography was utilized in the diagnosis and monitoring of periaortitis as a supportive imaging modality, as the use of contrast agents was contraindicated because of renal insufficiency.

Chronic periaortitis (CP) is a rare disease characterised by the presence of a fibro-inflammatory tissue typically enveloping the abdominal aorta, the iliac arteries and, in some cases, the nearby structures, such as the ureters and the inferior vena cava. Imaging plays a key role in the diagnosis and follow-up: computed tomography and magnetic resonance imaging scans are used to define the extension of the pathological tissue, whereas fluorodeoxyglucose positron emission tomography is the gold standard to establish the degree of its metabolic activity. CP must be distinguished from secondary forms of periaortic infiltration, which include malignant, infectious, and drug-related aetiologies. This review focuses on the clinical aspects of CP and the differential diagnosis with secondary cases, and aims to provide the clinician with a guide through this challenging clinical approach.

BACKGROUND: Immunoglobulin G4-related disease is characterized by swelling of various organs throughout the body and nodules/hypertrophic lesions. However, its cause remains unknown. We report a case of immunoglobulin G4-related disease that was diagnosed based on the histopathological findings of prostate biopsy.
METHODS: A 72-year-old Japanese man had been treated by a nearby doctor for hypertension, but subsequently developed lower urinary tract symptoms and was prescribed an α1 blocker for 1 year. However, the patient was subsequently referred to our department because his symptoms did not improve. Prostate-specific antigen was 1.258 ng/ml; however, the nodule was palpable in the right lobe on digital rectal examination, and magnetic resonance imaging suggested Prostate Imaging and Reporting and Data System category 3. Therefore, transrectal prostate needle biopsy (12 locations) under ultrasound was performed. Histopathological examination revealed no malignant findings, although infiltration of lymphocytes and plasma cells, and partial fibrosis were observed. No remarkable findings of obstructive phlebitis were observed. Immunoglobulin G4-related disease was suspected, and immunoglobulin and immunoglobulin G4 immunostaining was performed. Immunoglobulin G4 positive plasma cells were observed in a wide range, immunoglobulin G4 positive cells were noted at > 10 per high-power field, and the immunoglobulin G4 positive/immunoglobulin G positive cell ratio was > 40%. Serum immunoglobulin G4 levels were high at 1600 mg/dl. Enhanced abdominal computed tomography findings suggested periaortitis. Additionally, multiple lymphadenopathies were observed around the abdominal aorta. The patient was accordingly diagnosed with immunoglobulin G4-related disease definite, diagnosis group (definite). We proposed steroid treatment for periaortic soft tissue lesions and lower urinary tract symptoms; however, the patient was refused treatment. A computed tomography scan 6 months after diagnosis revealed no changes in the soft tissue lesions around the aorta. Follow-up computed tomography examinations will be performed every 6 months.
CONCLUSIONS: If immunoglobulin G4-related disease is suspected and a highly invasive examination is required for histopathological diagnosis, this can be performed by a relatively minimally invasive prostate biopsy for patients with lower urinary tract symptoms. Further evidence is needed to choose an optimal candidate for prostate biopsy for lower urinary tract symptoms patients with suspicion of immunoglobulin G4-related disease. For patients with lower urinary tract symptoms with immunoglobulin G4-related disease or a history, performing a prostate biopsy may avoid unnecessary treatment. However, if steroid therapy is ineffective, surgical treatment should be considered.

Immunoglobulin G (IgG) 4‑related diseases are fibrosing inflammatory systemic diseases that can affect almost every organ system. Typical disease manifestations include autoimmune pancreatitis, lymphadenopathy, retroperitoneal fibrosis, inflammatory orbitopathy and involvement of the salivary and lacrimal glands. Organ involvements can occur either isolated or in combination with other disease manifestations. Before diagnosing IgG4-related diseases, malignancies and other inflammatory diseases have to be excluded. The diagnosis requires a combination of laboratory findings, histological and radiological results. Typically, IgG4-related diseases respond well to glucocorticosteroids. In cases of relapse or severe organ involvement a longer term immunosuppression is often required, whereas watch and wait can also be sufficient in milder cases.
UNASSIGNED: Ig(Immunglobulin)G4-assoziierte Erkrankungen sind fibrosierende entzündliche Systemerkrankungen, die nahezu jedes Organsystem betreffen können. Typische Krankheitsmanifestationen sind z. B. die Autoimmunpankreatitis, Lymphadenopathien, die Retroperitonealfibrose, die entzündliche Orbitopathie sowie Beteiligungen der Speichel- und Tränendrüsen. Diese können gemeinsam oder isoliert auftreten. Die Diagnose fordert den Ausschluss maligner oder anderer entzündlicher Erkrankungen und setzt sich aus Laborbefunden, Histologie und radiologischen Befunden zusammen. Typischerweise sprechen die IgG4-assoziierten Erkrankungen gut auf Glukokortikosteroide an, bei Rezidiven oder schwerer Organbeteiligung ist auch eine längerfristige Immunsuppression indiziert, während in leichteren Fällen auch „watch and wait“ ausreichend sein kann.

Patients with retroperitoneal fibrosis (RPF), a rare condition, present with nonspecific abdominal pain or flank pain that can be complicated by urologic obstruction and/or vascular compromise. Reporting rare entities that often elude prompt diagnosis will aid clinicians to consider the entity in their differential diagnosis and potentially lead to earlier recognition and appropriate management. Our case emphasizes the importance of not just the diagnosis and treatment of RPF but how to best monitor RPF patients to minimize complications from disease progression or treatment-related adverse events.