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52. Reply

Author

Grayson, Peter C., primary, Ponte, Cristina, additional, Robson, Joanna C., additional, Suppiah, Ravi, additional, Judge, Andrew, additional, Hutchings, Andrew, additional, Craven, Anthea, additional, Khalid, Sara, additional, Luqmani, Raashid A., additional, Watts, Richard A., additional, and Merkel, Peter A., additional

Published
2022
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53. Ruxolitinib is more effective than other JAK inhibitors to treat VEXAS syndrome: a retrospective multicenter study

Author

Heiblig, Maël, primary, Ferrada, Marcela A., additional, Koster, Matthew T., additional, Barba, Thomas, additional, Gerfaud-Valentin, Mathieu, additional, Mékinian, Arsène, additional, Coelho, Henrique, additional, Fossard, Gaelle, additional, Barraco, Fiorenza, additional, Galicier, Lionel, additional, Bienvenu, Boris, additional, Hirsch, Pierre, additional, Vial, Guillaume, additional, Boutin, Anne Blandine, additional, Galland, Joris, additional, Le Guenno, Guillaume, additional, Bigot, Adrien, additional, Warrington, Kenneth J., additional, Kermani, Tanaz A., additional, Grayson, Peter C., additional, Patel, Bhavisha A., additional, Beck, David B., additional, Jamilloux, Yvan, additional, Fenaux, Pierre, additional, and Sujobert, Pierre, additional

Published
2022
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54. Genomic ascertainment for UBA1 variants and VEXAS syndrome: a population-based study

Author

Beck, David B., primary, Bodian, Dale L., additional, Shah, Vandan, additional, Mirshahi, Uyenlinh L., additional, Kim, Jung, additional, Ding, Yi, additional, Strande, Natasha T., additional, Cantor, Anna, additional, Haley, Jeremy S., additional, Cook, Adam, additional, Hill, Wesley, additional, Grayson, Peter C., additional, Ferrada, Marcela A., additional, Kastner, Daniel L., additional, Carey, David J., additional, and Stewart, Douglas R., additional

Published
2022
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57. Reply.

Author

Ponte, Cristina, Grayson, Peter C., Gribbons, Katherine Bates, Robson, Joanna C., Suppiah, Ravi, Judge, Andrew, Watts, Richard A., Luqmani, Raashid A., and Merkel, Peter A.

Subjects
*NURSES' associations, *GIANT cell arteritis diagnosis, *AGE distribution, *RHEUMATOLOGY, *GIANT cell arteritis, *TAKAYASU arteritis, *PHENOTYPES
Published
2023
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58. Longitudinal Characterization of Vascular Inflammation and Disease Activity in Takayasu Arteritis and Giant Cell Arteritis: A Single‐Center Prospective Study.

Author

Alessi, Hugh D., Quinn, Kaitlin A., Ahlman, Mark A., Novakovich, Elaine, Saboury, Babak, Luo, Yiming, and Grayson, Peter C.

Subjects
VASCULAR diseases, POSITRON emission tomography, DISEASE duration, DISEASE progression, MANN Whitney U Test, TAKAYASU arteritis, GIANT cell arteritis
Abstract

Objective: To examine and compare disease activity over time in giant cell arteritis (GCA) and Takayasu arteritis (TAK) using multimodal assessment combining clinical, laboratory, and imaging‐based testing. Methods: Patients with GCA or TAK were enrolled into a single‐center prospective, observational cohort at any point in the disease course. Patients underwent standardized assessment, including 18F‐fluorodeoxyglucose positron emission tomography (FDG‐PET) at enrollment and follow‐up visits. Each FDG‐PET finding was subjectively interpreted as active or inactive vasculitis. Global arterial FDG uptake was quantified by the PET Vascular Activity Score (PETVAS). Patients were stratified by disease duration at enrollment (0–2 years; 2–5 years; >5 years). Fisher exact and Mann‐Whitney U tests, Spearman's correlation, and linear regression were used for statistical analyses. Results: A total of 126 patients with large vessel vasculitis (GCA = 50; TAK = 76) were evaluated across 319 visits. Clinical disease activity was present in 33% of patients in the second to fifth year of disease and in 24% of patients evaluated >5 years after diagnosis. Active vasculitis by PET was observed in 66% of patients in years 2 to 5 after diagnosis and in 50% of patients enrolled >5 years into disease. PETVASs were consistently higher in GCA than TAK in the early and later phases of disease and significantly decreased over time in GCA but not TAK. Correlations between clinical, laboratory, and imaging findings were complex and varied with disease duration. Conclusion: Disease activity in GCA and TAK is common throughout the disease course. Patterns of vascular PET activity at diagnosis and later in disease differ between GCA and TAK. [ABSTRACT FROM AUTHOR]

Published
2023
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59. 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for eosinophilic granulomatosis with polyangiitis

Author

Grayson, Peter, Ponte, Cristina, Suppiah, Ravi, Robson, Joanna C., Craven, Anthea, Judge, Andrew, Khalid, Sara, Luqmani, Raashid, Watts, Richard, Merkel, Peter, Hutchings, Andrew, Grayson, Peter C., Luqmani, Raashid A., Merkel, Peter A., Gatenby, Paul, Watts, Richard A., Hill, Catherine, Ranganathan, Dwarakanathan, Kronbichler, Andreas, Blockmans, Daniel, Barra, Lillian, Carette, Simon, Pagnoux, Christian, Dhindsa, Navjot, Fifi-Mah, Aurore, Khalidi, Nader, Liang, Patrick, Milman, Nataliya, Pineau, Christian, Tian, Xinping, Wang, Guochun, Wang, Tian, Zhao, Ming hui, Tesar, Vladimir, Baslund, Bo, Hammam, Nevin, Shahin, Amira, Pirila, Laura, Putaala, Jukka, Hellmich, Bernhard, Henes, Jörg, Lamprecht, Peter, Neumann, Thomas, Schmidt, Wolfgang, Sunderkoetter, Cord, Szekanecz, Zoltan, Danda, Debashish, Das, Siddharth, Gupta, Rajiva, Rajasekhar, Liza, Dobashi, Hiroaki, Sharma, Aman, Wagh, Shrikant, Clarkson, Michael, Kawakami, Tamihiro, Kobayashi, Shigeto, Kono, Hajime, Makino, Hirofumi, Matsui, Kazuo, Muso, Eri, Suzuki, Kazuo, Ikeda, Kei, Takeuchi, Tsutomu, Tsukamoto, Tatsuo, Uchida, Shunya, Wada, Takashi, Yamada, Hidehiro, Yamagata, Kunihiro, Yumura, Wako, Lai, Kan Sow, Flores-Suarez, Luis Felipe, Hinojosa, Andrea, Rutgers, Bram, Tak, Paul Peter, Grainger, Rebecca, Quincey, Vicki, Stamp, Lisa, Besada, Emilio, Diamantopoulos, Andreas, Sznajd, Jan, Azevedo, Elsa, Geraldes, Ruth, Rodrigues, Miguel, Santos, Ernestina, Song, Yeong Wook, Moiseev, Sergey, Hocˇevar, Alojzija, Arimura, Yoshihiro, Cid, Maria Cinta, Moreno, Xavier Solanich, Atukorala, Inoshi, Berglin, Ewa, Molloy, Eamonn, Salvarani, Carlo, Schiavon, Franco, Tombetti, Enrico, Vaglio, Augusto, Amano, Koichi, Fujimoto, Shouichi, Harigai, Masayoshi, Hirano, Fumio, Hirahashi, Junichi, and Honma, Sakae

Abstract

Objective: To develop and validate revised classification criteria for eosinophilic granulomatosis with polyangiitis (EGPA). Methods: Patients with vasculitis or comparator diseases were recruited into an international cohort. The study proceeded in 5 phases: 1) identification of candidate criteria items using consensus methodology, 2) prospective collection of candidate items present at the time of diagnosis, 3) data-driven reduction of the number of candidate items, 4) expert panel review of cases to define the reference diagnosis, and 5) derivation of a points-based risk score for disease classification in a development set using least absolute shrinkage and selection operator logistic regression, with subsequent validation of performance characteristics in an independent set of cases and comparators. Results: The development set for EGPA consisted of 107 cases of EGPA and 450 comparators. The validation set consisted of an additional 119 cases of EGPA and 437 comparators. From 91 candidate items, regression analysis identified 11 items for EPGA, 7 of which were retained. The final criteria and their weights were as follows: maximum eosinophil count ≥1 × 109/liter (+5), obstructive airway disease (+3), nasal polyps (+3), cytoplasmic antineutrophil cytoplasmic antibody (ANCA) or anti–proteinase 3 ANCA positivity (−3), extravascular eosinophilic predominant inflammation (+2), mononeuritis multiplex/motor neuropathy not due to radiculopathy (+1), and hematuria (−1). After excluding mimics of vasculitis, a patient with a diagnosis of small- or medium-vessel vasculitis could be classified as having EGPA if the cumulative score was ≥6 points. When these criteria were tested in the validation data set, the sensitivity was 85% (95% confidence interval [95% CI] 77–91%) and the specificity was 99% (95% CI 98–100%). Conclusion: The 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for EGPA demonstrate strong performance characteristics and are validated for use in research.

Published
2022

61. Association of 18F‐Fluorodeoxyglucose–Positron Emission Tomography Activity With Angiographic Progression of Disease in Large Vessel Vasculitis.

Author

Quinn, Kaitlin A., Ahlman, Mark A., Alessi, Hugh D., LaValley, Michael P., Neogi, Tuhina, Marko, Jamie, Novakovich, Elaine, and Grayson, Peter C.

Subjects
DISEASE progression, MAGNETIC resonance angiography, PATIENT aftercare, GIANT cell arteritis, TAKAYASU arteritis, COMPARATIVE studies, RADIOPHARMACEUTICALS, POSITRON emission tomography, DESCRIPTIVE statistics, DEOXY sugars, VASCULITIS, LONGITUDINAL method
Abstract

Objective: To assess whether vascular activity seen on 18F‐fluorodeoxyglucose–positron emission tomography (FDG‐PET) scan is associated with angiographic change in large vessel vasculitis (LVV). Methods: Patients with LVV were recruited into a prospective cohort. All patients underwent magnetic resonance angiography or computed tomography angiography and FDG‐PET imaging. Follow‐up imaging using the same imaging modalities was obtained ≥6 months later per a standardized imaging protocol. Arterial damage, defined as stenosis, occlusion, or aneurysm, and corresponding FDG uptake were evaluated in 17 arterial territories. On follow‐up, development of new lesions was recorded, and existing lesions were characterized as improved, worsened, or unchanged. Results: A total of 1,091 arterial territories from 70 patients with LVV (38 patients with Takayasu arteritis, 32 patients with giant cell arteritis) were evaluated. Over a median 1.6 years of follow‐up, new lesions developed only in 8 arterial territories in 5 patients with Takayasu arteritis. Arterial lesions improved in 16 territories and worsened in 6 territories. Most arterial territories that did not have vascular activity on FDG‐PET scan at baseline had no angiographic change over the follow‐up period (787 [99%] of 793). Few territories with baseline FDG‐PET activity had angiographic change over time (24 [8%] of 298), but of the territories that developed angiographic change, 80% had FDG‐PET activity at baseline. Within the same patient, an arterial territory with baseline FDG‐PET activity had significantly increased risk for angiographic change compared to a paired arterial territory without FDG‐PET activity (odds ratio 19.49 [95% confidence interval 2.44–156.02]; P < 0.01). Concomitant edema and wall thickening further increased risk for angiographic change. Conclusion: Development of angiographic change was infrequent in this cohort of patients with LVV. A lack of baseline FDG‐PET activity was strongly associated with stable angiographic disease. In cases of angiographic progression, change was preceded by the presence of FDG‐PET activity. [ABSTRACT FROM AUTHOR]

Published
2023
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63. Response to: Correspondence on ‘2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for granulomatosis with polyangiitis’ by Joanna C Robson et al and ‘2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for microscopic polyangiitis’ by Ravi Suppiah et al

Author

Grayson, Peter C, primary, Robson, Joanna C, additional, Suppiah, Ravi, additional, Ponte, Cristina, additional, Watts, Richard A, additional, Luqmani, Raashid A, additional, and Merkel, Peter A, additional

Published
2022
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64. 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Kawasaki Disease

Author

Gorelik, Mark, primary, Chung, Sharon A., additional, Ardalan, Kaveh, additional, Binstadt, Bryce A., additional, Friedman, Kevin, additional, Hayward, Kristen, additional, Imundo, Lisa F., additional, Lapidus, Sivia K., additional, Kim, Susan, additional, Son, Mary Beth, additional, Sule, Sangeeta, additional, Tremoulet, Adriana H., additional, Van Mater, Heather, additional, Yildirim‐Toruner, Cagri, additional, Langford, Carol A., additional, Maz, Mehrdad, additional, Abril, Andy, additional, Guyatt, Gordon, additional, Archer, Amy M., additional, Conn, Doyt L., additional, Full, Kathy A., additional, Grayson, Peter C., additional, Ibarra, Maria F., additional, Merkel, Peter A., additional, Rhee, Rennie L., additional, Seo, Philip, additional, Stone, John H., additional, Sundel, Robert P., additional, Vitobaldi, Omar I., additional, Warner, Ann, additional, Byram, Kevin, additional, Dua, Anisha B., additional, Husainat, Nedaa, additional, James, Karen E., additional, Kalot, Mohamad, additional, Lin, Yih Chang, additional, Springer, Jason M., additional, Turgunbaev, Marat, additional, Villa‐Forte, Alexandra, additional, Turner, Amy S., additional, and Mustafa, Reem A., additional

Published
2022
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65. VEXAS Syndrome: A Case Series From a Single‐Center Cohort of Italian Patients With Vasculitis

Author

Muratore, Francesco, primary, Marvisi, Chiara, additional, Castrignanò, Paola, additional, Nicoli, Davide, additional, Farnetti, Enrico, additional, Bonanno, Orsola, additional, Longo, Rosina, additional, Zaldini, Piera, additional, Galli, Elena, additional, Balanda, Nicholas, additional, Beck, David B., additional, Grayson, Peter C., additional, Pipitone, Nicolò, additional, Boiardi, Luigi, additional, and Salvarani, Carlo, additional

Published
2022
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66. 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis with Polyangiitis

Author

Grayson, Peter C, primary, Ponte, Cristina, additional, Suppiah, Ravi, additional, Robson, Joanna C, additional, Craven, Anthea, additional, Judge, Andrew, additional, Khalid, Sara, additional, Hutchings, Andrew, additional, Luqmani, Raashid A, additional, Watts, Richard A, additional, and Merkel, Peter A, additional

Published
2022
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67. 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for microscopic polyangiitis

Author

Suppiah, Ravi, primary, Robson, Joanna C, additional, Grayson, Peter C, additional, Ponte, Cristina, additional, Craven, Anthea, additional, Khalid, Sara, additional, Judge, Andrew, additional, Hutchings, Andrew, additional, Merkel, Peter A, additional, Luqmani, Raashid A, additional, and Watts, Richard A, additional

Published
2022
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68. 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for granulomatosis with polyangiitis

Author

Robson, Joanna C, primary, Grayson, Peter C, additional, Ponte, Cristina, additional, Suppiah, Ravi, additional, Craven, Anthea, additional, Judge, Andrew, additional, Khalid, Sara, additional, Hutchings, Andrew, additional, Watts, Richard A, additional, Merkel, Peter A, additional, and Luqmani, Raashid A, additional

Published
2022
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69. A prospective observational cohort study and systematic review of 40 patients with mouth and genital ulcers with inflamed cartilage (MAGIC) syndrome

Author

Luo, Yiming, primary, Bolek, Ertugrul Cargi, additional, Quinn, Kaitlin A., additional, Wells, Kristina, additional, Rose, Emily, additional, Sikora, Keith, additional, Goodspeed, Wendy, additional, Rominger, Emily, additional, Kilic, Levent, additional, Karadag, Omer, additional, Clark, Cindy, additional, Livinski, Alicia A., additional, Grayson, Peter C., additional, and Ferrada, Marcela A., additional

Published
2022
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71. Use of 18F-fluorodeoxyglucose positron emission tomography to standardize clinical trial recruitment in Takayasu’s arteritis

Author

Quinn, Kaitlin A, primary, Alessi, Hugh D, additional, Ponte, Cristina, additional, Rose, Emily, additional, Ahlman, Mark A, additional, Redmond, Christopher, additional, Luo, Yiming, additional, Bolek, Ertugrul Cagri, additional, Langford, Carol A, additional, Merkel, Peter A, additional, and Grayson, Peter C, additional

Published
2022
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72. Large-vessel vasculitis

Author

Pugh, Dan, primary, Karabayas, Maira, additional, Basu, Neil, additional, Cid, Maria C., additional, Goel, Ruchika, additional, Goodyear, Carl S., additional, Grayson, Peter C., additional, McAdoo, Stephen P., additional, Mason, Justin C., additional, Owen, Catherine, additional, Weyand, Cornelia M., additional, Youngstein, Taryn, additional, and Dhaun, Neeraj, additional

Published
2022
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73. Longitudinal Characterization of Vascular Inflammation and Disease Activity in Takayasu Arteritis and Giant Cell Arteritis: A Single‐CenterProspective Study

Author

Alessi, Hugh D., Quinn, Kaitlin A., Ahlman, Mark A., Novakovich, Elaine, Saboury, Babak, Luo, Yiming, and Grayson, Peter C.

Abstract

To examine and compare disease activity over time in giant cell arteritis (GCA) and Takayasu arteritis (TAK) using multimodal assessment combining clinical, laboratory, and imaging‐based testing. Patients with GCA or TAK were enrolled into a single‐center prospective, observational cohort at any point in the disease course. Patients underwent standardized assessment, including 18F‐fluorodeoxyglucose positron emission tomography (FDG‐PET) at enrollment and follow‐up visits. Each FDG‐PET finding was subjectively interpreted as active or inactive vasculitis. Global arterial FDG uptake was quantified by the PET Vascular Activity Score (PETVAS). Patients were stratified by disease duration at enrollment (0–2 years; 2–5 years; >5 years). Fisher exact and Mann‐Whitney U tests, Spearman's correlation, and linear regression were used for statistical analyses. A total of 126 patients with large vessel vasculitis (GCA = 50; TAK = 76) were evaluated across 319 visits. Clinical disease activity was present in 33% of patients in the second to fifth year of disease and in 24% of patients evaluated >5 years after diagnosis. Active vasculitis by PET was observed in 66% of patients in years 2 to 5 after diagnosis and in 50% of patients enrolled >5 years into disease. PETVASs were consistently higher in GCA than TAK in the early and later phases of disease and significantly decreased over time in GCA but not TAK. Correlations between clinical, laboratory, and imaging findings were complex and varied with disease duration. Disease activity in GCA and TAK is common throughout the disease course. Patterns of vascular PET activity at diagnosis and later in disease differ between GCA and TAK.

Published
2023
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75. Clinical Efficacy of JAK Inhibitors in Patients with Vexas Syndrome: A Multicenter Retrospective Study

Author

Heiblig, Maël, primary, Ferrada, Marcela A, additional, Gerfaud-Valentin, Mathieu, additional, Barba, Thomas, additional, Mékinian, Arsène, additional, Koster, Matthew, additional, Coelho, Henrique, additional, Fossard, Gaelle, additional, Barraco, Fiorenza, additional, Galicier, Lionel, additional, Bienvenu, Boris, additional, Hirsch, Pierre, additional, Vial, Guillaume, additional, Boutin, Anne Blandine, additional, Le guenno, Guillaume, additional, Bigot, Adrien, additional, Warrington, Kenneth, additional, Kermani, Tanaz, additional, Jamilloux, Yvan, additional, Grayson, Peter C, additional, Patel, Bhavisha A., additional, Beck, David B., additional, Fenaux, Pierre, additional, and Sujobert, Pierre, additional

Published
2021
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79. 2022 American College of Rheumatology/EULAR Classification Criteria for Giant Cell Arteritis.

Author

Ponte, Cristina, Grayson, Peter C., Robson, Joanna C., Suppiah, Ravi, Gribbons, Katherine Bates, Judge, Andrew, Craven, Anthea, Khalid, Sara, Hutchings, Andrew, Watts, Richard A., Merkel, Peter A., Luqmani, Raashid A., Gatenby, Paul, Hill, Catherine, Ranganathan, Dwarakanathan, Kronbichler, Andreas, Blockmans, Daniel, Barra, Lillian, Carette, Simon, and Pagnoux, Christian

Subjects
*TEMPORAL arteries, *C-reactive protein, *BIOPSY, *CONFIDENCE intervals, *RESEARCH methodology, *GIANT cell arteritis, *SEVERITY of illness index, *BLOOD sedimentation, RESEARCH evaluation
Abstract

Objective: To develop and validate updated classification criteria for giant cell arteritis (GCA). Methods: Patients with vasculitis or comparator diseases were recruited into an international cohort. The study proceeded in 6 phases: 1) identification of candidate items, 2) prospective collection of candidate items present at the time of diagnosis, 3) expert panel review of cases, 4) data‐driven reduction of candidate items, 5) derivation of a points‐based risk classification score in a development data set, and 6) validation in an independent data set. Results: The development data set consisted of 518 cases of GCA and 536 comparators. The validation data set consisted of 238 cases of GCA and 213 comparators. Age ≥50 years at diagnosis was an absolute requirement for classification. The final criteria items and weights were as follows: positive temporal artery biopsy or temporal artery halo sign on ultrasound (+5); erythrocyte sedimentation rate ≥50 mm/hour or C‐reactive protein ≥10 mg/liter (+3); sudden visual loss (+3); morning stiffness in shoulders or neck, jaw or tongue claudication, new temporal headache, scalp tenderness, temporal artery abnormality on vascular examination, bilateral axillary involvement on imaging, and fluorodeoxyglucose–positron emission tomography activity throughout the aorta (+2 each). A patient could be classified as having GCA with a cumulative score of ≥6 points. When these criteria were tested in the validation data set, the model area under the curve was 0.91 (95% confidence interval [95% CI] 0.88–0.94) with a sensitivity of 87.0% (95% CI 82.0–91.0%) and specificity of 94.8% (95% CI 91.0–97.4%). Conclusion: The 2022 American College of Rheumatology/EULAR GCA classification criteria are now validated for use in clinical research. [ABSTRACT FROM AUTHOR]

Published
2022
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80. 2022 American College of Rheumatology/EULAR Classification Criteria for Takayasu Arteritis.

Author

Grayson, Peter C., Ponte, Cristina, Suppiah, Ravi, Robson, Joanna C., Gribbons, Katherine Bates, Judge, Andrew, Craven, Anthea, Khalid, Sara, Hutchings, Andrew, Danda, Debashish, Luqmani, Raashid A., Watts, Richard A., Merkel, Peter A., Gatenby, Paul, Hill, Catherine, Ranganathan, Dwarakanathan, Kronbichler, Andreas, Blockmans, Daniel, Barra, Lillian, and Carette, Simon

Subjects
*BLOOD pressure, *CONFIDENCE intervals, *RHEUMATOLOGY, *AGE distribution, *ANGINA pectoris, *TAKAYASU arteritis, *DIAGNOSTIC imaging, *SEX distribution, *DESCRIPTIVE statistics, *SENSITIVITY & specificity (Statistics), *VASCULITIS, *INTERMITTENT claudication, *PULSE (Heart beat)
Abstract

Objective: To develop and validate new classification criteria for Takayasu arteritis (TAK). Methods: Patients with vasculitis or comparator diseases were recruited into an international cohort. The study proceeded in 6 phases: 1) identification of candidate criteria items, 2) collection of candidate items present at diagnosis, 3) expert panel review of cases, 4) data‐driven reduction of candidate items, 5) derivation of a points‐based classification score in a development data set, and 6) validation in an independent data set. Results: The development data set consisted of 316 cases of TAK and 323 comparators. The validation data set consisted of an additional 146 cases of TAK and 127 comparators. Age ≤60 years at diagnosis and imaging evidence of large‐vessel vasculitis were absolute requirements to classify a patient as having TAK. The final criteria items and weights were as follows: female sex (+1), angina (+2), limb claudication (+2), arterial bruit (+2), reduced upper extremity pulse (+2), reduced pulse or tenderness of a carotid artery (+2), blood pressure difference between arms of ≥20 mm Hg (+1), number of affected arterial territories (+1 to +3), paired artery involvement (+1), and abdominal aorta plus renal or mesenteric involvement (+3). A patient could be classified as having TAK with a cumulative score of ≥5 points. When these criteria were tested in the validation data set, the model area under the curve was 0.97 (95% confidence interval [95% CI] 0.94–0.99) with a sensitivity of 93.8% (95% CI 88.6–97.1%) and specificity of 99.2% (95% CI 96.7–100.0%). Conclusion: The 2022 American College of Rheumatology/EULAR classification criteria for TAK are now validated for use in research. [ABSTRACT FROM AUTHOR]

Published
2022
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82. Somatic Mutations in UBA1 Define a Distinct Subset of Relapsing Polychondritis Patients With VEXAS

Author

Ferrada, Marcela A., primary, Sikora, Keith A., additional, Luo, Yiming, additional, Wells, Kristina V., additional, Patel, Bhavisha, additional, Groarke, Emma M., additional, Ospina Cardona, Daniela, additional, Rominger, Emily, additional, Hoffmann, Patrycja, additional, Le, Mimi T., additional, Deng, Zuoming, additional, Quinn, Kaitlin A., additional, Rose, Emily, additional, Tsai, Wanxia L., additional, Wigerblad, Gustaf, additional, Goodspeed, Wendy, additional, Jones, Anne, additional, Wilson, Lorena, additional, Schnappauf, Oskar, additional, Laird, Ryan S., additional, Kim, Jeff, additional, Allen, Clint, additional, Sirajuddin, Arlene, additional, Chen, Marcus, additional, Gadina, Massimo, additional, Calvo, Katherine R., additional, Kaplan, Mariana J., additional, Colbert, Robert A., additional, Aksentijevich, Ivona, additional, Young, Neal S., additional, Savic, Sinisa, additional, Kastner, Daniel L., additional, Ombrello, Amanda K., additional, Beck, David B., additional, and Grayson, Peter C., additional

Published
2021
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83. Dynamic Changes in the Nasal Microbiome Associated With Disease Activity in Patients With Granulomatosis With Polyangiitis

Author

Rhee, Rennie L., primary, Lu, Jiarui, additional, Bittinger, Kyle, additional, Lee, Jung‐Jin, additional, Mattei, Lisa M., additional, Sreih, Antoine G., additional, Chou, Sherry, additional, Miner, Jonathan J., additional, Cohen, Noam A., additional, Kelly, Brendan J., additional, Lee, Hongzhe, additional, Grayson, Peter C., additional, Collman, Ronald G., additional, and Merkel, Peter A., additional

Published
2021
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84. 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Giant Cell Arteritis and Takayasu Arteritis

Author

Maz, Mehrdad, primary, Chung, Sharon A., additional, Abril, Andy, additional, Langford, Carol A., additional, Gorelik, Mark, additional, Guyatt, Gordon, additional, Archer, Amy M., additional, Conn, Doyt L., additional, Full, Kathy A., additional, Grayson, Peter C., additional, Ibarra, Maria F., additional, Imundo, Lisa F., additional, Kim, Susan, additional, Merkel, Peter A., additional, Rhee, Rennie L., additional, Seo, Philip, additional, Stone, John H., additional, Sule, Sangeeta, additional, Sundel, Robert P., additional, Vitobaldi, Omar I., additional, Warner, Ann, additional, Byram, Kevin, additional, Dua, Anisha B., additional, Husainat, Nedaa, additional, James, Karen E., additional, Kalot, Mohamad A., additional, Lin, Yih Chang, additional, Springer, Jason M., additional, Turgunbaev, Marat, additional, Villa‐Forte, Alexandra, additional, Turner, Amy S., additional, and Mustafa, Reem A., additional

Published
2021
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85. 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Antineutrophil Cytoplasmic Antibody–Associated Vasculitis

Author

Chung, Sharon A., primary, Langford, Carol A., additional, Maz, Mehrdad, additional, Abril, Andy, additional, Gorelik, Mark, additional, Guyatt, Gordon, additional, Archer, Amy M., additional, Conn, Doyt L., additional, Full, Kathy A., additional, Grayson, Peter C., additional, Ibarra, Maria F., additional, Imundo, Lisa F., additional, Kim, Susan, additional, Merkel, Peter A., additional, Rhee, Rennie L., additional, Seo, Philip, additional, Stone, John H., additional, Sule, Sangeeta, additional, Sundel, Robert P., additional, Vitobaldi, Omar I., additional, Warner, Ann, additional, Byram, Kevin, additional, Dua, Anisha B., additional, Husainat, Nedaa, additional, James, Karen E., additional, Kalot, Mohamad A., additional, Lin, Yih Chang, additional, Springer, Jason M., additional, Turgunbaev, Marat, additional, Villa‐Forte, Alexandra, additional, Turner, Amy S., additional, and Mustafa, Reem A., additional

Published
2021
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86. 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Polyarteritis Nodosa

Author

Chung, Sharon A., primary, Gorelik, Mark, additional, Langford, Carol A., additional, Maz, Mehrdad, additional, Abril, Andy, additional, Guyatt, Gordon, additional, Archer, Amy M., additional, Conn, Doyt L., additional, Full, Kathy A., additional, Grayson, Peter C., additional, Ibarra, Maria F., additional, Imundo, Lisa F., additional, Kim, Susan, additional, Merkel, Peter A., additional, Rhee, Rennie L., additional, Seo, Philip, additional, Stone, John H., additional, Sule, Sangeeta, additional, Sundel, Robert P., additional, Vitobaldi, Omar I., additional, Warner, Ann, additional, Byram, Kevin, additional, Dua, Anisha B., additional, Husainat, Nedaa, additional, James, Karen E., additional, Kalot, Mohamad, additional, Lin, Yih Chang, additional, Springer, Jason M., additional, Turgunbaev, Marat, additional, Villa‐Forte, Alexandra, additional, Turner, Amy S., additional, and Mustafa, Reem A., additional

Published
2021
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89. Neutrophil-Related Gene Expression and Low-Density Granulocytes Associated With Disease Activity and Response to Treatment in Antineutrophil Cytoplasmic Antibody–Associated Vasculitis

Author

Grayson, Peter C., Carmona-Rivera, Carmelo, Xu, Lijing, Lim, Noha, Gao, Zhong, Asare, Adam L., Specks, Ulrich, Stone, John H., Seo, Philip, Spiera, Robert F., Langford, Carol A., Hoffman, Gary S., Kallenberg, Cees G. M., St.Clair, William E., Tchao, Nadia K., Ytterberg, Steven R., Phippard, Deborah J., Merkel, Peter A., Kaplan, Mariana J., and Monach, Paul A.

Published
2015
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90. Somatic mutations in rheumatological diseases: VEXAS syndrome and beyond.

Author

Sikora, Keith A, Wells, Kristina V, Bolek, Ertugrul Cagri, Jones, Adrianna I, and Grayson, Peter C

Subjects
GENETIC mutation, PROFESSIONS, INFLAMMATION, CLINICAL competence, RHEUMATISM, HEMATOPOIESIS, AUTOINFLAMMATORY diseases
Abstract

Discovery of the VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome demonstrates that somatic mutations in haematological precursor cells can cause adult-onset, complex inflammatory disease. Unlike germline mutations, somatic mutations occur throughout the lifespan, are restricted to specific tissue types, and may play a causal role in non-heritable rheumatological diseases, especially conditions that start in later life. Improvements in sequencing technology have enabled researchers and clinicians to detect somatic mutations in various tissue types, especially blood. Understanding the relationships between cell-specific acquired mutations and inflammation is likely to yield key insights into causal factors that underlie many rheumatological diseases. The objective of this review is to detail how somatic mutations are likely to be relevant to clinicians who care for patients with rheumatological diseases, with particular focus on the pathogenetic mechanisms of the VEXAS syndrome. [ABSTRACT FROM AUTHOR]

Published
2022
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92. VEXAS Syndrome

Author

Grayson, Peter C, primary, Patel, Bhavisha A, additional, and Young, Neal S, additional

Published
2021
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95. Discordance in patient and physician global assessment in relapsing polychondritis.

Author

Rose, Emily, Ferrada, Marcela A, Quinn, Kaitlin A, Arnaud, Laurent, Goodspeed, Wendy, Kim, Jeff, Allen, Clint, Sirajuddin, Arlene, Chen, Marcus, and Grayson, Peter C

Subjects
SELF-evaluation, CARTILAGE diseases, PHYSICIANS' attitudes, HEALTH outcome assessment, HEALTH surveys, SEVERITY of illness index, PATIENTS' attitudes, QUESTIONNAIRES, DESCRIPTIVE statistics, PSYCHOLOGY of the sick, MEDICAL needs assessment, EVALUATION
Abstract

Objectives Relapsing polychondritis (RP) is a rare, heterogeneous, systemic inflammatory disease that targets cartilage. Patient-reported outcome measures may differ from physician assessment. This study compared patient global assessment (PtGA) and physician global assessment (PhGA) scores in a prospective cohort of patients with RP. Methods Adult patients with RP underwent a standardized comprehensive evaluation at ∼6 month intervals. At each visit, three physicians scored PhGA by consensus. The patient independently completed four patient-reported outcomes: PtGA, 36-item Short Form Health Survey (SF-36), Brief Illness Perception Questionnaire (BIPQ) and Multidimensional Fatigue Inventory (MFI). Patient–physician discordance was defined as a difference between PtGA and PhGA of ≥3 on a 0–10 scale. Results A total of 76 patients were evaluated over 154 visits. The median PhGA was 3 [interquartile range (IQR) 2–3] and the median PtGA was 5 (IQR 4–7). PtGA and PhGA were concordant in 66 visits (42.9%) and patients scored disease severity ≥3 points higher than physicians scored disease activity (positive discordance) in 84 visits (54.5%). Compared with visits with concordance, visits with positive discordance were associated with significantly worse scores on the MFI, BIPQ, SF-36 physical component score and SF-36 mental component score. Conclusion Patients with RP typically self-report high PtGA that does not align with PhGA. Discordance is likely driven by the high physical and psychological burden of illness experienced by patients. Multifaceted treatment approaches that address the burden of disease in RP from the patient perspective are needed. [ABSTRACT FROM AUTHOR]

Published
2022
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97. Identification of susceptibility loci for Takayasu arteritis through a large multi-ancestral genome-wide association study

Author

Ortiz-Fernández, Lourdes, primary, Saruhan-Direskeneli, Güher, additional, Alibaz-Oner, Fatma, additional, Kaymaz-Tahra, Sema, additional, Coit, Patrick, additional, Kong, Xiufang, additional, Kiprianos, Allan P., additional, Maughan, Robert T., additional, Aydin, Sibel Z., additional, Aksu, Kenan, additional, Keser, Gokhan, additional, Kamali, Sevil, additional, Inanc, Murat, additional, Springer, Jason, additional, Akar, Servet, additional, Onen, Fatos, additional, Akkoc, Nurullah, additional, Khalidi, Nader A., additional, Koening, Curry, additional, Karadag, Omer, additional, Kiraz, Sedat, additional, Forbess, Lindsy, additional, Langford, Carol A., additional, McAlear, Carol A., additional, Ozbalkan, Zeynep, additional, Yavuz, Sule, additional, Çetin, Gozde Yildirim, additional, Alpay-Kanitez, Nilufer, additional, Chung, Sharon, additional, Ates, Askin, additional, Karaaslan, Yasar, additional, McKinnon-Maksimowicz, Kathleen, additional, Monach, Paul A., additional, Ozer, Hüseyin T.E., additional, Seyahi, Emire, additional, Fresko, Izzet, additional, Cefle, Ayse, additional, Seo, Philip, additional, Warrington, Kenneth J., additional, Ozturk, Mehmet A., additional, Ytterberg, Steven R., additional, Cobankara, Veli, additional, Onat, Ahmet Mesut, additional, Duzgun, Nurşen, additional, Bıcakcıgil, Muge, additional, Yentür, Sibel P., additional, Lally, Lindsay, additional, Manfredi, Angelo A., additional, Baldissera, Elena, additional, Erken, Eren, additional, Yazici, Ayten, additional, Kısacık, Bünyamin, additional, Kaşifoğlu, Timuçin, additional, Dalkilic, Ediz, additional, Cuthbertson, David, additional, Pagnoux, Christian, additional, Sreih, Antoine, additional, Reales, Guillermo, additional, Wallace, Chris, additional, Wren, Jonathan D., additional, Cunninghame-Graham, Deborah S., additional, Vyse, Timothy J., additional, Sun, Ying, additional, Chen, Huiyong, additional, Grayson, Peter C., additional, Tombetti, Enrico, additional, Jiang, Lindi, additional, Mason, Justin C., additional, Merkel, Peter A., additional, Direskeneli, Haner, additional, and Sawalha, Amr H., additional

Published
2021
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