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Objectives: 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., Conclusion: 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., (© The Author(s) 2023. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Objective: To evaluate the incidence and outcomes of large artery (LA) involvement among patients with giant cell arteritis (GCA) and to compare LA involvement to non-GCA patients., Methods: The study included Olmsted County, Minnesota, USA residents with incident GCA between 1950 and 2016 with follow-up through 31 December 2020, death or migration. A population-based age-matched/sex-matched comparator cohort without GCA was assembled. LA involvement included aortic aneurysm, dissection, stenosis in the aorta or its main branches diagnosed within 1 year prior to GCA or anytime afterwards. Cumulative incidence of LA involvement was estimated; Cox models were used., Results: The GCA cohort included 289 patients (77% females, 81% temporal artery biopsy positive), 106 with LA involvement.Reported cumulative incidences of LA involvement in GCA at 15 years were 14.8%, 30.2% and 49.2% for 1950-1974, 1975-1999 and 2000-2016, respectively (HR 3.48, 95% CI 1.67 to 7.27 for 2000-2016 vs 1950-1974).GCA patients had higher risk for LA involvement compared with non-GCA (HR 3.22, 95% CI 1.83 to 5.68 adjusted for age, sex, comorbidities). Thoracic aortic aneurysms were increased in GCA versus non GCA (HR 13.46, 95% CI 1.78 to 101.98) but not abdominal (HR 1.08, 95% CI 0.33 to 3.55).All-cause mortality in GCA patients improved over time (HR 0.62, 95% CI 0.41 to 0.93 in 2000-2016 vs 1950-1974) but remained significantly elevated in those with LA involvement (HR 1.89, 95% CI 1.39 to 2.56)., Conclusions: LA involvement in GCA has increased over time. Patients with GCA have higher incidences of LA involvement compared with non-GCA including thoracic but not abdominal aneurysms. Mortality is increased in patients with GCA and LA involvement highlighting the need for continued surveillance., Competing Interests: Competing interests: The authors MME, MSK, SA, CC, TAK, CW and MK have no financial disclosures to declare. Disclosures for KJW include Eli Lilly, Kiniksa, Sanofi, BMS and Amgen., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Objectives: To investigate the clinicopathologic features of patients with giant cell arteritis (GCA) who had thoracic aorta aneurysm or dissection surgery., Methods: Patients who had thoracic aorta surgery between January 1, 2000, and December 31, 2021, at the Mayo Clinic, Rochester, Minnesota, were identified with current procedural terminology (CPT) codes. The identified patients were screened for a prior diagnosis of GCA with diagnostic codes and electronic text search. The available medical records of all the patients of interest were manually reviewed. Thoracic aorta tissues obtained during surgery were re-evaluated in detail by pathologists. The clinicopathologic features of these patients were analyzed. Overall observed survival was compared with lifetable rates from the United States population., Results: Of the 4621 patients with a CPT code for thoracic aorta surgery, 49 had a previous diagnosis of GCA. Histopathologic evaluation of the aortic tissue revealed active aortitis in most patients with GCA (40/49, 82%) after a median (IQR) of 6.0 (2.6-10.3) years from GCA diagnosis. All patients were considered in clinical remission at the time of aortic surgery. The overall mortality compared to age and sex-matched general population was significantly increased with a standardized mortality ratio of 1.55 (95% CI, 1.05-2.19)., Conclusion: Histopathologic evaluation of the thoracic aorta obtained during surgery revealed active aortitis in most patients with GCA despite being considered in clinical remission several years after GCA diagnosis. Chronic, smoldering aortic inflammation likely contributes to the development of aortic aneurysm and dissection in GCA., Competing Interests: Declaration of Competing Interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: Kenneth J Warrington reports a relationship with Eli Lilly that includes: funding grants. Kenneth J Warrington reports a relationship with GlaxoSmithKline Inc. that includes: funding grants. Kenneth J Warrington reports a relationship with Kiniksa Pharmaceuticals Ltd. that includes: funding grants. Kenneth J Warrington reports a relationship with Sanofi that includes: consulting or advisory., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Objective: To examine whether a change in the presentation of incident gout happened over the last 20 years and to determine the risk of subsequent gout flares after an initial gout attack., Methods: All incident cases of gout were identified among residents of Olmsted County, Minnesota, diagnosed in 1989-1992 and 2009-2010 according to the earliest date fulfilling the 1977 American Rheumatism Association preliminary criteria, or the New York or Rome criteria for gout. Patients in both cohorts were then followed for up to 5 years. Cumulative incidence and person-year methods were used to compare flare rates, and conditional frailty models were used to examine predictors., Results: A total of 429 patients with incident gout (158 patients in 1989-1992 and 271 patients in 2009-2010) were identified and followed for a mean of 4.2 years. The majority of patients were male (73%) and the mean age (SD) at gout onset was 59.7 (17.3) years. Classic podagra decreased significantly from 74% to 59% (p < 0.001). Cumulative incidence of first flare was similar in both cohorts (62% vs 60% by 5 yrs in 1989-1992 and 2009-2010, respectively; p = 0.70), but overall flare rate was marginally higher in 2009-2010 compared to 1989-1992 (rate ratio: 1.24). Hyperuricemia (HR 1.59) and kidney disease (HR 1.34) were significant predictors of future flares., Conclusion: Gout flares were common in both time periods. Hyperuricemia and kidney disease were predictors of future flares in patients with gout. Podagra as a presentation of gout has become relatively less frequent in recent years.

Granulomatosis with polyangiitis (GPA) commonly presents with glomerulonephritis and inflammation of upper and lower respiratory tracts. It can also involve other organs including those of the urinary tract. The involvement of the urethra is very rarely reported. We present a case of GPA in a patient who had recurrent urinary tract infections and an acute bladder outlet obstruction due to a urethral thickening by GPA. In this report, we discuss urethral involvement with GPA. The incidence of such involvement, as with other urinary tract organs, might be underestimated. It can affect both sexes, with male predominance, and can occur at any age. It responds to standard GPA medical treatment but may require surgical intervention. Rheumatologists should be aware of this limited form of GPA as early recognition and treatment can decrease the risk of complications.

Introduction: Behçet's disease is an autoimmune disease with diverse clinical manifestations with vasculitis being the hallmark of the disease. The aim of this work is to study the genetic association between human leukocyte antigen (HLA) class-I molecules of Egyptians with Behçet's disease and the disease susceptibility and clinical patterns., Methods: Fifty-seven patients diagnosed with Behçet's disease according to the 1990 International Study Group (ISG) criteria for Behçet's disease coming from Egyptian origin up to the third grandfather were included in the study. Healthy controls were taken from HLA Class-I case control studies in Egyptian population yielding a pool of 221 healthy controls. HLA Class-I typing for patients was done using Reverse Sequence specific oligonucleotide probes (rSSO)., Results: Male patients represented 89% of the sample. Mean age of onset was 25.81 (± 6.7) years and mean disease duration was 9.47 (± 7.4) years. Behçet's disease was associated with HLA-A*24 and HLA-B*42 (p = 0.001) and highly associated with HLA-A*68 and B*15 and B*51 (p < 0.001). While HLA A*03 and B*52 were protective for Behçet's (p = 0.002 and 0.007). Interestingly, HLA-B*51 and HLA-A*68 (p = 0.005 and 0.023) were associated with the blinding eye disease. HLA-B*51 was protective from Neurological and vascular involvement (p = 0.005 and 0.032, respectively)., Conclusion: Behçet's disease is associated with HLA Class-I A*24, A*68 and B*15, B*42 and B*51 in Egyptian patients while A*03 and B*52 were found to be protective. Interestingly, HLA B*51 and A*68 could be considered as poor prognostic factor for eye involvement.

Objective: To assess in-hospital gout flares in patients with gout., Methods: Hospitalizations were evaluated for gout flares in a cohort of Olmsted County, Minnesota, residents with incident gout in 1989-1992 or 2009-2010., Results: There were 429 patients followed up to 5 years. Of these, 169 patients experienced 454 hospitalizations. Hospitalization rates increased without reaching statistical significance from 1989-1992 to 2009-2010 [rate ratio (RR) 1.19, 95% CI 0.98-1.45]. The gout flare rate increased significantly during hospitalization (RR 10.2, 95% CI 6.8-14.5). In-hospital gout flare increased the average hospital stay by 1.8 days (p < 0.001)., Conclusion: Hospitalization increased the risk of gout flares 10-fold. In-hospital gout flares were associated with longer hospitalization.

Objective: Demonstration of rheumatoid arthritis (RA) characteristics in a large cohort of Egyptian patients., Methods: Retrospective analysis of data of 3219 RA patients attending the Rheumatology outpatient clinic, Kasr Alainy Hospital, Cairo University; from January 1995 till December 2015., Results: Mean age at disease onset was 36.1 ±13.4 years; 2774 (84%) were females and mean disease duration was 12.9 ±7.9 years. Regarding number of joint affected at disease onset; polyarticular pattern was found in 77.1%, pattern of joint involvement; combined small and large joints involvement was in 83.2%, subcutaneous nodules in 14.2%, interstitial lung disease in 0.3%, secondary Sjogren's syndrome in 10.5%, hand bony erosions at diagnosis in 20.6%. Rheumatoid factor was positive in 52%. There was annual increase in the newly diagnosed cases (P = 0.017) reflecting increase in patients' awareness and improvement of medical service, also annual increase in: mean age of onset (P < 0.001) reflecting changes in health measures, also in cases with monoarticular or oligoarticular patterns at disease onset (P = 0.02, 0.01 respectively) reflecting earlier diagnosis of patients and in patients with small joint involvement (P = 0.001) with a significant decline in: polyarticular pattern (P = 0.001), combined small and large joint affection (P < 0.001), and number of cases with hand bony erosions (P = 0.01) denoting earlier diagnosis, tight disease control., Conclusion: We found a female predominance, younger age at disease onset, lower frequency of extra articular manifestations, more frequent polyarticular pattern at disease onset and less erosive disease, denoting changing referral patterns, earlier diagnosis, improved disease control in Egyptian RA patients over 2 decades., Abbreviations: SNs: Subcutaneous nodules; 2ry SS: 2ry Sjogren's syndrome; ILD: Interstitial lung disease; ACPA: Anti-cyclic citrullinated peptide antibodies; DMARDs: Disease modifying anti-rheumatic drugs.

Objective: To examine the incidence of gout over the last 20 years and to evaluate possible changes in associated comorbid conditions., Methods: The medical records were reviewed of all adults with a diagnosis of incident gout in Olmsted County, Minnesota, USA, during 2 time periods (January 1, 1989-December 31, 1992, and January 1, 2009-December 31, 2010). Incident cases had to fulfill at least 1 of 3 criteria: the American Rheumatism Association 1977 preliminary criteria for gout, the Rome criteria, or the New York criteria., Results: A total of 158 patients with new-onset gout were identified during 1989-1992 and 271 patients during 2009-2010, yielding age- and sex-adjusted incidence rates of 66.6/100,000 (95% CI 55.9-77.4) in 1989-1992 and 136.7/100,000 (95% CI 120.4-153.1) in 2009-2010. The incidence rate ratio was 2.62 (95% CI 1.80-3.83). At the time of their first gout flare, patients diagnosed with gout in 2009-2010 had higher prevalence of comorbid conditions compared with 1989-1992, including hypertension (69% vs 54%), diabetes mellitus (25% vs 6%), renal disease (28% vs 11%), hyperlipidemia (61% vs 21%), and morbid obesity (body mass index ≥ 35 kg/m 2 ; 29% vs 10%)., Conclusion: The incidence of gout has more than doubled over the recent 20 years. This increase together with the more frequent occurrence of comorbid conditions and cardiovascular risk factors represents a significant public health challenge.