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Immunoglobulin G4-related coronary periarteritis: a systematic literature review with a case series.Clinical Rheumatology Aug 2022We aimed to assess the clinical and radiological characteristics of immunoglobulin G4-related coronary periarteritis through a systematic literature review and from our... (Review)
Review
We aimed to assess the clinical and radiological characteristics of immunoglobulin G4-related coronary periarteritis through a systematic literature review and from our case series. In the systematic literature review, we assessed English language manuscripts on immunoglobulin G4-related coronary periarteritis cases. Additionally, we identified patients with immunoglobulin G4-related coronary periarteritis at St. Luke's International Hospital in Tokyo, Japan, from 2014 to 2020. We summarized patients' demographics, immunoglobulin-G and -G4 titers, site and morphological features of the coronary lesion, and other organ involvements. We identified 38 cases from the literature and four patients from our institute. Coronary lesions were detected using coronary computed tomography in 40 (95.2%) patients. Mass-like or diffuse wall-thickening lesion was the most frequently observed type in 33 (78.6%) patients. No trends at the site of the coronary arteries were identified. Overall, 32 (76.1%) patients had multiple-organ involvement, of which the most common lesion was peri-aortitis in 21 (50.0%) patients. Ten (23.8%) patients with an isolated coronary lesion had significantly lower immunoglobulin-G4 titers than those with other organ involvements (immunoglobulin-G4: 261 [161.0, 564.0] vs. 1355.0 [320.8, 2480.0] mg/dL, p = 0.033). The wall-thickening lesions responded well to immunosuppressive treatments. Mass-like or diffuse wall-thickening on coronary computed tomography is a characteristic radiographic finding of immunoglobulin G4-related coronary periarteritis, which can occur in any branch. Immunoglobulin G4-related coronary periarteritis showed similar characteristics to other organ lesions, including its relatively low serum immunoglobulin-G4 level in patients with a single-organ disease and its high responsiveness to glucocorticoids.
Topics: Aortitis; Arteritis; Coronary Vessels; Heart; Humans; Immunoglobulin G
PubMed: 35445950
DOI: 10.1007/s10067-022-06179-y -
Measurement Properties of the Polymyalgia Rheumatica Activity Score: A Systematic Literature Review.The Journal of Rheumatology Jun 2022To perform a COnsensus-based Standards for the selection of health Measurement INstruments (COSMIN)-based systematic literature review of measurement properties of the...
OBJECTIVE
To perform a COnsensus-based Standards for the selection of health Measurement INstruments (COSMIN)-based systematic literature review of measurement properties of the Polymyalgia Rheumatica Activity Score (PMR-AS).
METHODS
PubMed, EMBASE, and CINAHL were broadly searched. English full-text articles, with (quantitative) data on ≥ 5 patients with PMR using the PMR-AS were selected. Seven hypotheses for construct validity and 3 for responsiveness, concerning associations with erythrocyte sedimentation rate, physical function, quality of life, clinical disease states, ultrasound, and treatment response, were formulated. We assessed the structural validity, internal consistency, reliability, and measurement error, or the hypotheses on construct validity or responsiveness of the PMR-AS based on COSMIN criteria.
RESULTS
Out of the identified 26 articles that used the PMR-AS, we were able to use 12 articles. Structural validity, internal consistency, construct validity, and responsiveness were assessed in 1, 2, 8, and 3 articles, respectively. Insufficient evidence was found to confirm structural validity and internal consistency. No data were found on reliability or measurement error. Although 60% and 67% of hypotheses tested for construct validity and responsiveness, respectively, were confirmed, there was insufficient evidence to meet criteria for good measurement properties.
CONCLUSION
While there is some promising evidence for construct validity and responsiveness of the PMR-AS, it is lacking for other properties and, overall, falls short of criteria for good measurement properties. Therefore, further research is needed to assess its role in clinical research and care.
Topics: Blood Sedimentation; Giant Cell Arteritis; Humans; Polymyalgia Rheumatica; Psychometrics; Quality of Life; Reproducibility of Results
PubMed: 35232811
DOI: 10.3899/jrheum.211292 -
Reumatismo Feb 2022Since of the last publication of last recommendations on primary large-vessel vasculitis (LVV) endorsed by the Italian Society of Rheumatology (SIR) in 2012, new...
OBJECTIVE
Since of the last publication of last recommendations on primary large-vessel vasculitis (LVV) endorsed by the Italian Society of Rheumatology (SIR) in 2012, new evidence emerged regarding the diagnosis and the treatment with conventional and biologic immunosuppressive drugs. The associated potential change of clinical care supported the need to update the original recommendations.
METHODS
Using the grading of recommendations assessment, development and evaluation (GRADE)-ADOLOPMENT framework, a systematic literature review was performed to update the evidence supporting the European Alliance of Associations for Rheumatology (EULAR) guidelines on LVV as reference. A multidisciplinary panel of 12 expert clinicians, a trained nurse, and a patients' representative discussed the recommendation in cooperation with an Evidence Review Team. Sixty-one stakeholders were consulted to externally review and rate the recommendations.
RESULTS
Twelve recommendations were formulated. A suspected diagnosis of LVV should be confirmed by imaging or histology. In active GCA or TAK, the prompt commencement of high dose of oral glucocorticoids (40-60 mg prednisone-equivalent per day) is strongly recommended to induce clinical remission. In selected patients with GCA (e.g., refractory or relapsing disease or patients at risk of glucocorticoid related adverse effects) the use of an adjunctive therapy (tocilizumab or methotrexate) is recommended. In all patients diagnosed with TAK, adjunctive therapies, such as conventional synthetic or biological immunosuppressants, should be given in combination with glucocorticoids.
CONCLUSIONS
The new set of SIR recommendations was formulated in order to provide a guidance on both diagnosis and treatment of patients suspected of or with a definite diagnosis of LVV.
Topics: Giant Cell Arteritis; Humans; Italy; Methotrexate; Rheumatology; Takayasu Arteritis
PubMed: 35130681
DOI: 10.4081/reumatismo.2021.1470 -
Frontiers in Immunology 2021Takayasu's arteritis (TA) is a rare, chronic granulomatous large-vessel vasculitis that can lead to ocular ischemia. Ocular outcomes after therapeutic management in TA...
BACKGROUND
Takayasu's arteritis (TA) is a rare, chronic granulomatous large-vessel vasculitis that can lead to ocular ischemia. Ocular outcomes after therapeutic management in TA remain largely unknown. We herein conduct a case-based systematic review to address the current treatment options in this particular cohort.
METHODS
PubMed, Medline, and EMBASE databases were searched pertaining to ocular outcomes after systemic treatment in TA. Studies reporting ocular examinations before and after treatment in TA patients with ocular ischemia were included. Clinical characteristics, therapies, ocular outcomes, and complications were recorded.
RESULTS
A 29-year-old woman with newly diagnosed TA showed dramatic regression of Takayasu's retinopathy (TR) following balloon angioplasty. Optical coherence tomography angiography (OCTA) was used as a novel strategy for subsequent follow-up. A total of 117 eyes of 66 patients with a median age of 27 years were included for systematic review. TR was the most common ocular manifestation. Oral steroids were prescribed in nearly all patients (n = 65), followed by the use of methotrexate and antiplatelet therapy. Of the patients, 65.8% and 34.2% underwent open surgery and endovascular procedure, respectively. The median follow-up period was 12 weeks (interquartile range 8-33.5). Surgical therapy showed better ocular improvement (including visual and imaging responses) in both acute and chronic vision loss, along with fewer complications than medical therapy alone. In the surgical group, the visual prognosis was significantly better in patients with initial visual acuity better than 20/200 (p = 0.03) and those who underwent surgery before stage III TR (p = 0.01). Ocular outcomes were equivalent in the two surgical approaches.
CONCLUSION
Clinicians should be familiar with ophthalmic manifestations of this potentially treatable complication in TA. Compared with medical therapy alone, surgical intervention might be a better choice for both acute and chronic vision loss. Surgery is best recommended before the onset of irreversible ischemia to the globe. A combined regimen (oral steroids, immunosuppressants, and antiplatelet drugs) might be effective for those with surgical contradictions or reluctance to an invasive procedure. Physicians should be aware of the importance of ocular examinations, including OCTA, during the diagnosis and follow-up in TA.
Topics: Adult; Eye; Eye Diseases; Female; Humans; Ischemia; Takayasu Arteritis; Vascular Surgical Procedures
PubMed: 35095866
DOI: 10.3389/fimmu.2021.791278 -
Cranio : the Journal of... Jan 2022The aim of this review was to highlight jaw-related complications in COVID-19 manifestations, their etiology, and prevention methods.
OBJECTIVE
The aim of this review was to highlight jaw-related complications in COVID-19 manifestations, their etiology, and prevention methods.
METHODS
A systematic review of literature was conducted. MEDLINE/PubMed, and Google Scholar were searched for the following keywords: "COVID-19" "Oral manifestations", "Musculoskeletal patients", "Mandible", "Jaw", "Osteonecrosis", "MRONJ", and "dry socket".
RESULTS
Only nine articles were included in this review. Jaw-related disorders associated with COVID-19 were dry socket, osteonecrosis, and orofacial pain related to temporomandibular joint disorders (TMD) and giant cell arteritis (GCA).
CONCLUSION
COVID-19 potentially predisposes to osteonecrosis due to thrombotic inflammatory phenomena caused by the disease itself or its therapeutic modalities. All jaw osteonecrosis cases reported so far in relation to COVID-19 affected the upper jaw. Orofacial pain in COVID-19 patients was related to TMD and GCA. Clinical evidence-based studies are required to investigate the actual prevalence and possible correlation between COVID-19 and jaw-related disorders.
PubMed: 35083956
DOI: 10.1080/08869634.2022.2031438 -
Autoimmunity Reviews Mar 2022Vasculitis are severe systemic autoimmune diseases which may involve different organs and systems. Conversely, muscles do not represent an organ commonly involved by... (Review)
Review
Vasculitis are severe systemic autoimmune diseases which may involve different organs and systems. Conversely, muscles do not represent an organ commonly involved by systemic vasculitis and myositis is not include among any classification or diagnostic criterion of vasculitis. In this regard, we aimed to review the literature in order to report all the available evidence concerning the inflammatory involvement of muscle in patients affected by systemic vasculitis. We collected a total of 108 papers, for a sum of 395 patients affected by muscle vasculitis. Most of them suffered from medium and small vessels vasculitis (mainly polyarteritis nodosa and ANCA-associated vasculitis) or from vasculitis secondary to rheumatoid arthritis. Conversely, muscle involvement in case of large vessel vasculitis occurred seldom, while only few papers reported such occurrence in Kawasaki or Behçet's disease. Histological findings may differ, but the most common ones displayed a necrotizing vasculitis of perimysium vessels, while granulomatous vasculitis was assessed only in case of ANCA-associated vasculitis patients. Creatine kinase were usually within normal range, seldom elevated, while imaging findings were generally undistinguishable from the ones found in idiopathic inflammatory myopathies: magnetic resonance imaging displays signal hyperintensity in T2 and STIR scans, while few data exist for positron emission tomography. The presentation of the disease may be fearsome and severe, sometimes life-threatening, but an overall good response to conventional immunosuppressants and/or glucocorticoids has been reported.
Topics: Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Arteritis; Behcet Syndrome; Humans; Muscles; Polyarteritis Nodosa
PubMed: 34971804
DOI: 10.1016/j.autrev.2021.103029 -
Autoimmunity Reviews Feb 2022Polymyalgia rheumatica (PMR) is an inflammatory rheumatic disease that is common in elderly people. Its classification in the spectrum of autoinflammatory and autoimmune... (Review)
Review
BACKGROUND AND AIM
Polymyalgia rheumatica (PMR) is an inflammatory rheumatic disease that is common in elderly people. Its classification in the spectrum of autoinflammatory and autoimmune diseases is difficult because of its only partially understood immune-mediated mechanisms. The literature concerning the innate and adaptive immune system activation in PMR was systematically reviewed highlighting the relative weight of autoinflammation and autoimmunity in its pathogenesis and disease progression.
METHODS
A literature search on PubMed Central and Embase scientific databases was performed by two independent reviewers. To be eligible, the studies needed to fully satisfy our initial PICO framework: a primary diagnosis of PMR as a population, the search for immune/inflammatory cells, cytokines and autoantibodies as an intervention, a control group consisting in healthy controls, patients with other inflammatory rheumatic diseases or PMR patients in remission after treatment and as outcomes the results of the investigations in the analyzed tissue samples. The most relevant data of the included papers were extracted by using a standardized template.
RESULTS
Of the 933 screened abstracts, 52 papers were included in the systematic review and categorized depending on their primary research objectives. The hyper-activity of neutrophils and monocytes, expressing toll-like receptor 7 in active disease, an impaired phagocytosis and endothelial dysfunction, as well as an increased count of innate T cells in patients with remission emerged among the major derangements of the innate immune response in PMR. Among the cytokines profile, interleukin-6 plays a key role but other pro-inflammatory mediators and angiogenesis markers such as chemokines, B-cell activating factor, vascular endothelial growth factor and angiopoietins seem to be involved in refractory or glucocorticoid-resistant PMR. The aberrant adaptive immune response was documented by tissue and serum findings of polarized T cells towards T helper 1 and 17 phenotypes, an increased expression of immunosenescent surface markers and a downregulated immunoregulatory response. The altered distribution of peripheral B cells, detected during active disease, suggested their peripheral migration towards unidentified sites. The interaction between innate and adaptive immune response was documented by a synovial infiltrate of macrophages and T cells. Despite multiple autoantibodies have been detected in PMR patients, none proved to correlate with disease activity seeming to be reactive to the marked inflammation or antigenic determinants provided by environmental triggers or tissue/cell damage.
CONCLUSIONS
The complex network between innate and adaptive immune system in PMR is supported by findings at molecular and cellular levels. By considering both the ends of the pathophysiological spectrum of immune-mediated rheumatic diseases, PMR may be regarded as an inflammatory immune-mediated disease with mixed mechanisms in a background of genetic and epigenetic factors together with immunological and endocrine senescence.
Topics: Autoimmunity; Giant Cell Arteritis; Humans; Neutrophils; Polymyalgia Rheumatica
PubMed: 34798314
DOI: 10.1016/j.autrev.2021.102995 -
Clinical Rheumatology Jan 2022Corticosteroid-sparing disease-modifying anti-rheumatic drugs are an area of active exploration in large vessel vasculitis (LVV), i.e., Takayasu arteritis (TAK) and... (Review)
Review
Corticosteroid-sparing disease-modifying anti-rheumatic drugs are an area of active exploration in large vessel vasculitis (LVV), i.e., Takayasu arteritis (TAK) and Giant Cell Arteritis (GCA). The role of Janus kinase (JAK) inhibitors has been recently identified in different inflammatory rheumatic diseases. We conducted a systematic review of the use of JAK inhibitors in LVV across MEDLINE, Scopus, Web of Science, EMBASE, PubMed Central, Cochrane database of controlled trials, clinicaltrials.gov, and major recent international conferences. We identified four cohort studies and ten case reports. The JAK inhibitors used in these studies were tofacitinib, baricitinib, and ruxolitinib. A cohort study in TAK compared 27 patients treated with tofacitinib with 26 others treated with methotrexate, with better clinical outcomes with tofacitinib but similar angiographic stabilization, relapses, corticosteroid-sparing effect, and adverse events in both groups. Most of the other studies favored clinical responses with JAK inhibitors in LVV but with a paucity of data on other outcomes. Most of the included studies were of moderate quality. Evidence from pre-clinical models of LVV as well as limited in vivo data in patients with TAK appears to suggest that JAK inhibition reduces adventitial fibrosis, intimal proliferation, and inflammatory T lymphocyte infiltration in the media as well as reduces resident memory T cells in the vascular wall (which are otherwise resistant to corticosteroids). Ongoing clinical trials of tofacitinib, baricitinib, and upadacitinib in LVV shall help to further clarify the potential promise of JAK inhibitors for LVV (PROSPERO registration number CRD42021273359). KEY POINTS : •Tofacitinib appeared to associate with better clinical outcomes than methotrexate in TAK. •JAKinibs reduce adventitial fibrosis, intimal proliferation, and inflammatory vascular infiltrate in pre-clinical models of LVV. •Tofacitinib downregulates resident memory vascular T lymphocytes in pre-clinical models of LVV.
Topics: Antirheumatic Agents; Cohort Studies; Giant Cell Arteritis; Humans; Janus Kinase Inhibitors; Memory T Cells; Takayasu Arteritis
PubMed: 34729652
DOI: 10.1007/s10067-021-05973-4 -
Granulocyte-colony stimulating factor-associated aortitis in cancer: A systematic literature review.Cancer Treatment and Research... 2021Aortitis following granulocyte-colony stimulating factor (G-CSF) administration has been reported in 0.3-0.47% of cases. To evaluate the characteristics of...
BACKGROUND
Aortitis following granulocyte-colony stimulating factor (G-CSF) administration has been reported in 0.3-0.47% of cases. To evaluate the characteristics of G-CSF-associated aortitis, we systematically reviewed the literature.
METHODS
We searched PubMed and found 49 cases of G-CSF-associated aortitis and cancer comorbidities and analyzed their characteristics and treatments.
RESULTS
Since 2004, cases of G-CSF-associated aortitis have been increasing, particularly in Asia (75.5%). The mean age was 60.1 years; 79.6% of patients were 50 years and older; and most patients were females (91.8%). All patients underwent chemotherapy (taxane, 51.0%). The most frequent symptom was fever, which occurred within 10 days (61.2%) of G-CSF administration, similar to that in febrile neutropenia. The period to remission was within 14 days in 44.9% of cases. Steroids were administered to 59.2% of patients; however, treatment efficacy was not significant. No patients died.
CONCLUSIONS
High levels of inflammatory cytokines might induce aortitis; however, further studies are warranted.
Topics: Adult; Aged; Aortitis; Female; Granulocyte Colony-Stimulating Factor; Humans; Male; Middle Aged; Neoplasms
PubMed: 34530312
DOI: 10.1016/j.ctarc.2021.100454 -
Rheumatology Advances in Practice 2021This systematic review and meta-analysis aimed to evaluate the diagnostic value of the halo sign in the assessment of GCA.
OBJECTIVES
This systematic review and meta-analysis aimed to evaluate the diagnostic value of the halo sign in the assessment of GCA.
METHODS
A systematic literature review was performed using MEDLINE, EMBASE and Cochrane central register databases up to August 2020. Studies informing on the sensitivity and specificity of the US halo sign for GCA (index test) were selected. Studies with a minimum of five participants were included. Study articles using clinical criteria, imaging such as PET-CT and/or temporal artery biopsy (TAB) as the reference standards were selected. Meta-analysis was conducted with a bivariate model.
RESULTS
The initial search yielded 4023 studies. Twenty-three studies (patients = 2711) met the inclusion criteria. Prospective (11 studies) and retrospective (12 studies) studies in academic and non-academic centres were included. Using clinical diagnosis as the standard (18 studies) yielded a pooled sensitivity of 67% (95% CI: 51, 80) and a specificity of 95% (95% CI: 89, 98%). This gave a positive and negative likelihood ratio for the diagnosis of GCA of 14.2 (95% CI: 5.7, 35.5) and 0.375 (95% CI: 0.22, 0.54), respectively. Using TAB as the standard (15 studies) yielded a pooled sensitivity of 63% (95% CI: 50, 75) and a specificity of 90% (95% CI: 81, 95).
CONCLUSION
The US halo sign is a sensitive and specific approach for GCA assessment and plays a pivotal role in diagnosis of GCA in routine clinical practice.
REGISTRATION
PROSPERO 2020 CRD42020202179.
PubMed: 34514295
DOI: 10.1093/rap/rkab059