2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Granulomatosis With Polyangiitis

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

doi:10.1002/art.41986

Cited by 87 publications

(54 citation statements)

References 17 publications

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“…RA and osteoarthritis (OA) patients who underwent knee joint replacement surgery were recruited from the First Affiliated Hospital of Shandong First Medical University. RA patients were diagnosed according to the revised American College of Rheumatology (ACR) criteria ( 21 ). Patients with trauma, tumors, diabetes and other autoimmune diseases were excluded from this study.…”

Section: Methodsmentioning

confidence: 99%

ATF6α contributes to rheumatoid arthritis by inducing inflammatory cytokine production and apoptosis resistance

Wang

Shi

et al. 2022

Front. Immunol.

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ObjectiveThe contribution of activating transcription factor 6α (ATF6α) in rheumatoid arthritis (RA) pathogenesis, especially on fibroblast-like synoviocytes (FLSs), has been suggested by its sensitivity to inflammatory stimulus. However, the exact role and therapeutic potential of ATF6α in RA remains to be fully elucidated.MethodsATF6α expression was determined in joint tissues and FLS, and gain-of-function and loss-of-function analyses were applied to evaluate the biological roles of ATF6α in RA FLSs. A murine collagen-induced arthritis (CIA) model, combining both gene deletion of ATF6α and treatment with the ATF6α inhibitor Ceapin-A7, was employed. Joint inflammation, tissue destruction, circulating levels of inflammatory cytokines were assessed in CIA mice. Transcriptome sequencing analysis (RNASeq), molecular biology, and biochemical approaches were performed to identify target genes of ATF6α.ResultsATF6α expression was significantly increased in synovium of RA patients and in synovium of mice subjected to CIA. ATF6α silencing or inhibition repressed RA FLSs viability and cytokine production but induced the apoptosis. CIA-model mice with ATF6α deficiency displayed decreased arthritic progression, leading to profound reductions in clinical and proinflammatory markers in the joints. Pharmacological treatment of mice with Ceapin-A7 reduced arthritis severity in CIA models. RNA-sequencing of wild-type and knockdown of ATF6α in RA FLSs revealed a transcriptional program that promotes inflammation and suppresses apoptosis, and subsequent experiments identified Baculoviral IAP Repeat Containing 3 (BIRC3) as the direct target for ATF6α.ConclusionThis study highlights the pathogenic role of ATF6α-BIRC3 axis in RA and identifies a novel pathway for new therapies against RA.

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Section: Methodsmentioning

confidence: 99%

ATF6α contributes to rheumatoid arthritis by inducing inflammatory cytokine production and apoptosis resistance

Wang

Shi

et al. 2022

Front. Immunol.

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“…There is an incidence estimated at 3 per 100,000 individuals with a slight female predominance and is most often diagnosed in the 4th through 6th decades of life, with childhood diagnosis being significantly less common [4]. Initial presentation can take many forms and include constitutional, cutaneous, pulmonary, ear, nose and throat, and renal symptoms, with the most common presenting symptoms including constitutional manifestations [5]. Children typically present with more severe manifestations involving renal, gastrointestinal, and pulmonary organ involvement [6].…”

Section: Discussionmentioning

confidence: 99%

Cannot Ventilate: An Unexpected Cause of Respiratory Failure in a Ten-Year-Old Child

Mauro¹,

Nguyen²,

Dow³

2022

Cureus

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Granulomatosis with polyangiitis (GPA) is a necrotizing vasculitis known to affect the respiratory and renal systems. There are a multitude of clinical manifestations, many of which are not specific to the disease, such as dysfunction of the nasal, sinus, auditory, tracheal, pulmonary, ocular, renal, cardiac, and nervous systems. As a multisystemic illness without a "classic" presentation and insidious progression, it is often a challenging diagnosis. We report and discuss a case of a 10-year-old female with no significant past medical history who presented to the emergency department with a 10-day course of worsening respiratory symptoms. As her respiratory and clinical status began to precipitously decline, the decision was made to intubate the patient, which was performed without issue. Unfortunately, attempts at oxygenating and ventilating the patient were met with extreme resistance and difficulty-an airway situation that could have been catastrophic if not for quick reaction maneuvers performed that would ultimately go on to remedy the issue at hand. We hope to raise awareness regarding the airway challenges posed by GPA and delve into its management as a means of improving recognition and preparing clinicians to treat this condition.

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“…We made diagnosis of GPA according to the latest ACR/EULAR criteria (at least 5 points) [6] : CANCA positivity (+5); pulmonary nodules and cavitation on chest imaging (+2). Treatment with corticosteroids and immunosuppressants was started.…”

Section: Case Presentationmentioning

confidence: 99%

“…Computer tomography (CT) is the most sensitive exam in detecting GPA pulmonary nodules, many of which are cavitated and have a random distribution [5] . The diagnostic criteria used for diagnosis are ACR/EULAR [6] . Therapy is based on the use of corticosteroids and immunosuppressants [7] .…”

Section: Introductionmentioning

confidence: 99%

Migrant cavitation as primary involvement in a particular case of granulomatosis with polyangiitis

Manti

Battaglia

Pelaia

et al. 2022

Radiology Case Reports

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2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Granulomatosis With Polyangiitis

Cited by 87 publications

References 17 publications

ATF6α contributes to rheumatoid arthritis by inducing inflammatory cytokine production and apoptosis resistance

ATF6α contributes to rheumatoid arthritis by inducing inflammatory cytokine production and apoptosis resistance

Cannot Ventilate: An Unexpected Cause of Respiratory Failure in a Ten-Year-Old Child

Migrant cavitation as primary involvement in a particular case of granulomatosis with polyangiitis

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