BACKGROUND:Patients with polymyalgia rheumatica (PMR) may have subclinical large vessel vasculitis. We compared the incidence of aortic complications in PMR and giant cell arteritis (GCA) to the general population. METHODS:A retrospective cohort study was performed of patients with PMR and GCA identified by ≥2 ICD-9/ICD-10-CM diagnostic codes and concurrent corticosteroid treatment in the US-based TriNetX database (2000-2024). Matched general population controls were identified (1:3 ratio). The primary outcome, aortic complications, was a composite of aortic aneurysm and dissection/rupture. Adjusted hazard ratios (aHR) were calculated using Cox proportional cause-specific hazard models with PMR as the referent category. FINDINGS:Of 57,336 patients, 17,327 had PMR, 4,734 had GCA, and 35,275 were matched controls. Median follow-up time was 3.74 years (interquartile range, 1.8-6.4). The incidence rate of any aortic complication per 1,000 person-years was highest for GCA (11.69), followed by PMR (6.78) and the general population (5.09). Compared to patients with PMR, patients with GCA had a higher risk of any aortic complication (aHR 1.87, 95 % confidence interval (CI) 1.58-2.21); the general population risk was similar (aHR 0.95, 95 % CI 0.84-1.06). In a sensitivity analysis, patients with PMR who later developed GCA had a risk similar to those initially diagnosed with GCA (aHR 0.85, 95 % CI 0.60-1.19). INTERPRETATION:Patients with PMR had a similar risk of large vessel complications compared to the general population and a lower risk compared to those with GCA. These results do not support screening for aortic inflammation among patients with PMR who lack features of GCA.

OBJECTIVES:To examine the clinicopathologic features of patients with polymyalgia rheumatica (PMR) who had thoracic aorta repair surgery. Findings were compared with those of a cohort of patients with giant cell arteritis (GCA) requiring thoracic aorta repair. METHODS:All patients evaluated at Mayo Clinic in Rochester, MN, with Current Procedural Terminology (CPT) codes for thoracic aorta repair surgery between 2000-2021 were identified. All patients were screened for prior PMR diagnosis. Patients with PMR and no signs of GCA were categorized as clinically isolated PMR. The medical records of all patients were manually reviewed, and pathologists re-examined all the aortic tissues. RESULTS:Of the 4621 patients with at least one CPT code for thoracic aorta repair surgery, 43 patients were diagnosed with clinically isolated PMR before the surgery. Detailed histopathological examination of the aortic tissues revealed active inflammation in 30/43 (70%) patients after a median (IQR) of 10.0 (4.7-13.3) years from the PMR diagnosis. When compared with aortic tissue from patients with a prior diagnosis of GCA, the aorta of patients with PMR had more severe inflammation (Grade 3: 15/30 [50%] vs 5/34 [15%], P = 0.002). Patients with PMR and thoracic aorta repair may experience a 40% increased risk of mortality compared with the general population, but this did not reach statistical significance (standardized mortality ratio: 1.40; 95% CI: 0.91-2.07). CONCLUSIONS:Some patients with PMR have subclinical aortic inflammation that is detectable many years after initial diagnosis and may contribute to the development of aortic aneurysm.

Clinical Images: Sarcoidosis with concomitant aortitis and coronary arteritisA 49-year-old Black female patient with HIV diagnosed and on therapy for 19 years and a 12-year history of pulmonary sarcoidosis was evaluated for chest pain radiating to the left arm.She was on prednisone 10 mg daily for several years but stopped two years before her presentation.Erythrocyte sedimentation rate and troponin T were elevated: 41 mm/hour (reference: <20 mm/hour) and 52 ng/L (reference <10 ng/L), respectively.HIV RNA was undetectable, CD4 T-cells 887/mcL, antinuclear antibodies, antineutrophil cytoplasmic antibodies, and serum IgG4 were within reference ranges.

To the Editor: We read with interest the article by Majerovich et al describing the duration of glucocorticoid (GC) therapy and temporal artery biopsy (TAB) positivity in giant cell arteritis (GCA).1 Their results are noteworthy, indicating GC therapy does not appear to significantly affect TAB positivity to at least 6 weeks1; findings that the authors noted are also supported by our institution’s experience.2 It is our group’s opinion that GCA should be confirmed by biopsy and/or imaging (temporal artery or large-vessel), and patients with clinical diagnosis should be restricted to those with characteristic features for whom appropriate investigations were negative or nondiagnostic and alternative etiologies were excluded. Current GCA management consensus guidelines by the American College of Rheumatology (ACR) conditionally recommend obtaining a TAB within 2 weeks of starting oral GCs, compared to waiting longer than 2 weeks.3 We agree with Majerovich and colleagues1 that TAB, particularly in patients with cranial symptoms, can still be diagnostic beyond 2 weeks, especially if other objective methods of disease confirmation have not yet been attempted, or were done and were inconclusive. Temporal artery … Address correspondence to Dr. M.J. Koster, Associate Professor of Medicine, Division of Rheumatology, Mayo Clinic, 200 1st Street SW, Rochester, MN 55905, USA. Email: koster.matthew{at}mayo.edu.

Objective: Isolated tricuspid valve surgery is uncommon and associated with high perioperative morbidity and mortality. We aimed to study the overall outcomes of patients who underwent minimally invasive right thoracotomy tricuspid valve surgery (Mini-TVS), consisting of either tricuspid valve repair (TVre) or replacement (TVR). Methods: We performed a retrospective analysis of all Mini-TVS procedures (20172022), through which we identified fi ed isolated tricuspid valve surgeries. We examined in-hospital outcomes, survival analysis over a 4-year period, and competing risk analysis for reoperative surgery. Results: Among a total of 51 patients, the average age was 60 +/- 16 years, and 67% % (n = 34) were female. Severe tricuspid regurgitation was present in all cases. Infective endocarditis was noted in 7.8% % (n = 4), and 24% % (n = 12) had preexisting pacemakers. Mini-TVS included TVre in 18 patients (35%) % ) and TVR in 33 patients (65%). % ). The in-hospital and 30-day mortality rates were 4% % (n = 2) and 6% % (n = 3), respectively. At 4 years, the overall TVS survival was 76% % (confidence fi dence interval, 62-93%), % ), with no significant fi cant difference between TVre and TVR (91% % vs 69%, % , P = .16). At follow-up, 3 patients required repeat surgery for recurrent regurgitation after 2.6, 3.3, and 11 months, with a reoperation rate of 7.3% % (confidence fi dence interval, 2.4-22%) % ) at 2 years. Factors associated with worse overall survival included nonelective surgery, right ventricular dysfunction, serum creatinine > 2 g/dL, and concomitant left-sided valve disease. Conclusions: A nonsternotomy minimally invasive approach is a feasible option for high-risk patients. Midterm outcomes were similar in repair or replacement. Patients with right ventricular dysfunction and left-sided disease had worse outcomes. (JTCVS Open 2024;17:98-110)

Giant cell arteritis (GCA) and Takayasu arteritis (TAK) are large-vessel vasculitides affecting the aorta and its branches. Arterial damage from these diseases may result in ischemic complications, aneurysms, and dissections. Despite their similarities, the management of GCA and TAK differs. Glucocorticoids are used frequently but relapses are common, and glucocorticoid toxicity contributes to significant morbidity. Conventional immunosuppressive therapies can be beneficial inTAK, though their role in the management of GCA remains unclear. Tumor necrosis factor inhibitors improve remission rates and appear to limit vascular damage in TAK; these agents are not beneficial in GCA. Tocilizumab is the first biologic glucocorticoidsparing agent approved for use in GCA and also appears to be effective in TAK. A better understanding of the pathogenesis of both conditions and the availability of targeted therapies hold much promise for future management.

Background Screening for large artery manifestations in patients with Giant Cell Arteritis (GCA) has been adopted in clinical practice. However, the effect of recent use of imaging studies on incidence and mortality of large artery manifestations remains unclear. Objectives In this study, we aimed to investigate incidence trends and outcomes of large artery manifestations in a population-based cohort of patients with GCA over a span of seven decades. Methods The study cohort included patients with incident GCA between 1950-2016 in a defined geographical area. Incident large artery manifestations were defined as aortic aneurysm, aortic dissection, stenosis in the aorta or any of its main branches diagnosed within 1 year prior to the diagnosis of GCA or anytime afterwards. Patients were followed till December 31st, 2020, death, or migration.Cumulative incidence of large artery manifestations adjusted for the competing risk of death was estimated. Cox proportional hazards models adjusted for age and sex were used to assess the association of clinical characteristics with large artery manifestations. Results The study included 289 patients with GCA: 222 (77%) females; temporal artery biopsy was positive in 235 patients (81%); mean follow up period was 10.4 ± 7 years; mean age at diagnosis was 76.4 ± 8.2 years. Incident large artery manifestations developed in 104 patients throughout the follow up period, the majority (76%) were found incidentally.Cumulative incidence rates of large artery manifestations at 5 years were 7.3% (95% CI 2.4-22.1%), 15.9% (95% CI 10.6-23.8%) and 27.2% (95% CI 20.2-36.6) for patients diagnosed in 1950-1974, 1975-1999 and 2000-2016, respectively. Moreover, cumulative incidence of large vessel manifestations at 15 years was 14.8% (95% CI 7.0-31.6%), 30.2% (95% CI 23.1-39.4%) and 48.8% (95% CI 38.8-61.3%) for patients diagnosed in 1950-1974, 1975-1999 and 2000-2016, respectively (Figure 1).Patients with GCA diagnosed in 2000-2016 and 1975-1999 had more than 3.5-fold and 2-fold increase in incidence of large artery manifestations (HR:3.49 95%CI 1.67-7.3 and HR: 1.98 95% CI 0.97–4.07, respectively) compared to 1950-1974. However, there was no significant increase in aortic aneurysm or dissection (Table 1).Mortality risk has decreased significantly in patients with large artery manifestations diagnosed in the latest cohort 2000-2016 (HR 0.38 95% CI 0.17–0.81) following non-significant improvements in 1975-1999 (HR 0.61 95% CI 0.29-1.30) compared to 1950-1974 (reference).Clinical predictors for large artery manifestations included weight loss, fatigue, arm claudication, ever smoker and bruit on physical examination. Cranial symptoms were negatively associated with large artery manifestations. Conclusion Incidence of large artery manifestations (mainly large artery stenosis) has increased over time likely from increased use of imaging studies. However, the incidence of aortic aneurysm/dissection has been stable over the last 7 decades.Mortality improvement in the recent years may be due to earlier detection of large artery involvement. REFERENCES: NIL. Acknowledgements: NIL. Disclosure of Interests Mohanad Elfishawi: None declared, Mahmut Kaymakci: None declared, Sara Achenbach: None declared, Cynthia S. Crowson: None declared, Tanaz Kermani: None declared, Weyand Cornelia: None declared, Matthew Koster: None declared, Kenneth J Warrington Consultant of: Chemocentryx, Sanofi, Grant/research support from: Eli Lilly, Kiniksa, GSK.Figure 1Cumulative incidence for time to any large artery manifestation (large artery stenosis, aortic aneurysm, aortic dissection) in patients with incident GCA 1950-2016 adjusted for the competing risk of death.Table 1Cox proportional hazards model showing Age- and Sex- adjusted risk of large artery manifestation among patients with incident GCA 1950-2016Event TypeGCA YearTotal (Events)HR (95% CI)Large artery stenosis1950-197441 (3)Reference1975-1999128 (21)2.63 (0.78-8.88)2000-2016118 (39)7.66 (2.30-25.5)Aortic Aneurysm/Dissection1950-197441 (7)Reference1975-1999127 (34)1.65 (0.73-3.75)2000-2016119 (16)1.29 (0.51-3.25)Any Large Vessel manifestation1950-197441 (9)Reference1975-1999126 (47)1.98 (0.97-4.07)2000-2016118 (48)3.49 (1.67-7.30)

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.

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 patient, a 26-year-old White male with a 4-year history of granulomatosis with polyangiitis (GPA), was evaluated for redness and a painless bulge in the right eye. Initial diagnosis of GPA included unilateral right-sided episcleritis, upper airway involvement, and pulmonary infiltrates. Nasal mucosa biopsy confirmed necrotizing granulomatous inflammation and laboratory assessment demonstrated positive antineutrophil cytoplasmic antibodies directed against proteinase 3. He was initially treated with glucocorticoids (GCs) and methotrexate (switched to azathioprine later because of side effects). At the time of presentation to ophthalmology, evaluation noted scleral inflammation in the superior quadrant associated with scleral thinning and protrusion of globe contents, consistent with scleral melt (arrow: globe protrusion). He did not have other features suggestive of active systemic vasculitis, and he had been off immunosuppressive therapy. The patient was started on remission induction therapy with rituximab and GCs but eventually required placement of multiple scleral patch grafts and amniotic membrane transplant surgeries. Scleritis and episcleritis are the most common ocular manifestations in GPA (1). Scleral involvement is a vision-threatening and severe manifestation of GPA (2, 3). Although remission of ocular involvement can be achieved in the majority of patients with scleritis with prompt recognition and treatment initiation, recalcitrant disease can result in scleral ischemia causing compromised scleral integrity requiring surgical intervention (1). Longitudinal follow-up for patients with GPA and ocular involvement is crucial, because this can be the only sign of GPA activity and can result in major comorbidity. Management of ocular involvement in GPA should be closely coordinated between rheumatology and ophthalmology. Disclosure form Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.

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OBJECTIVES:Clinically isolated aortitis (CIA) refers to inflammation of the aorta without signs of systemic vasculitis or infection. Population-based data on the epidemiology of CIA in North America is lacking. We aimed to investigate the epidemiology of pathologically confirmed CIA.METHODS:Residents of Olmsted County, Minnesota were screened for thoracic aortic aneurysm procedures with current procedural terminology codes between January 1, 2000, and December 31, 2021, using the resources of the Rochester Epidemiology Project. The medical records of all patients were manually reviewed. CIA was defined as histopathologically confirmed active aortitis diagnosed by evaluation of aortic tissue obtained during thoracic aortic aneurysm surgery in the absence of any infection, rheumatic disease, or systemic vasculitis. Incidence rates were age and sex adjusted to the 2020 United States total population.RESULTS:Eight incident cases of CIA were diagnosed during the study period; 6 (75%) of them were female. Median (IQR) age at diagnosis of CIA was 78.3 (70.2-78.9) years; all were diagnosed following ascending aortic aneurysm repair. The overall age and sex adjusted annual incidence rate of CIA was 8.9 (95% CI, 2.7-15.1) per 1,000,000 individuals over age 50 years. The median (IQR) duration of follow-up was 8.7 (1.2-12.0) years. The overall mortality compared to the age and sex matched general population did not differ (standardised mortality ratio: 1.58; 95% CI, 0.51-3.68).CONCLUSIONS:This is the first population-based epidemiologic study of pathologically confirmed CIA in North America. CIA predominantly affects women in their eighth decade and is quite rare.