ANCA-Associated Vasculitis: Core Curriculum 2020

Geetha, Duvuru; Jefferson, J. Ashley

doi:10.1053/j.ajkd.2019.04.031

Cited by 311 publications

(315 citation statements)

References 0 publications

Supporting

Mentioning

274

Contrasting

Unclassified

Order By: Relevance

“…Figure 1 summarizes the pathogenic processes elicited by ANCAs. While MPO and PR3 are intracellular antigens, they can be accessed by antigen-presenting cells under a specific condition called priming [141]. Neutrophils primed with inflammatory stimuli (cytokines, lipopolysaccharides, complement fragments, etc.)…”

Section: Pathogenic Roles Of Ancamentioning

confidence: 99%

Autoantibody Biomarkers in Rheumatic Diseases

Kang

Lee

2020

IJMS

View full text Add to dashboard Cite

Autoantibodies encountered in patients with systemic rheumatic diseases bear clinical significance as a biomarker to help or predict diagnosis, clinical phenotypes, prognosis, and treatment decision-making. Furthermore, evidence has accumulated regarding the active involvement of disease-specific or disease-associated autoantibodies in the pathogenic process beyond simple association with the disease, and such knowledge has become essential for us to better understand the clinical value of autoantibodies as a biomarker. This review will focus on the current update on the autoantibodies of four rheumatic diseases (rheumatoid arthritis, myositis, systemic sclerosis, and anti-neutrophil cytoplasmic antibody associated vasculitis) where there has been a tremendous progress in our understanding on their biological effects and clinical use.

show abstract

Section: Pathogenic Roles Of Ancamentioning

confidence: 99%

Autoantibody Biomarkers in Rheumatic Diseases

Kang

Lee

2020

IJMS

View full text Add to dashboard Cite

show abstract

“…When strong clinical suspicion for GPA exists, early diagnosis and initiation of disease arresting immunosuppressive therapy are suggested as progression can occur quickly and can be potentially irreversible. Serologies can be misleading in a minority of patients and treatment modalities vary depending on the underlying extent and severity of the disease [30]. Furthure research aimed at better understanding the pathogenesis of GPA and the development of new novel more effective agent with less side effect is warranteed.…”

Section: Resultsmentioning

confidence: 99%

Granulomatosis with polyangiitis (GPA) in a 76-year old woman presenting with pulmonary nodule and accelerating acute kidney injury

Darwich¹,

Schnell²,

Cossey³

2020

J Clini Nephrol

View full text Add to dashboard Cite

Granulomatosis with polyangiitis (GPA), a form of ANCA-associated vasculitis (AAV), is a rare disease with an often-occult presentation. It is more common in 4 th and 5 th decades of life but can be seen in all ages. This case report details a 76-year-old female presenting with abdominal pain, generalized weakness, and malaise, who was found to have pulmonary nodules on chest imaging. Biopsy of the lung nodule showed organizing pneumonia. Initially, antibiotics were used to treat the patient. However, she developed acute renal failure a few days after presentation and found to have positive serum C-ANCA as well as elevated ANCA-PR3 serologies. A subsequent kidney biopsy demonstrated pauci-immune necrotizing and crescentic glomerulonephritis that was consistent with GPA and the patient was started immediately on combination immunosuppressive therapy, plasmapheresis, and hemodialysis. GPA's clinical and radiological presentation can mimic other common conditions such as pneumonia, malignancy, bacterial sinusitis, pulmonary tuberculosis, sarcoidosis, and urinary tract infection. Because of this, a high level of suspicion is required for early diagnosis and treatment to alter the high mortality rate in this disease entity. All forms of ANCA-associated vasculitis (AAV) should be in the differential diagnosis for all patients presenting with multiorgan system involvement particularly in individuals with pulmonary and renal manifesations.

show abstract

“…Although the difference in study design could have affected the discordant result, this discrepancy could be also accounted for by the fact that PR3-ANCA is detected more frequently than is MPO-ANCA in AAV patients in the Western world, and is associated with higher disease relapse [24][25][26]. In contrast, MPO-ANCA-positive AAV is more common than is PR3-ANCA positive AAV in Asia, and its presence is associated with higher disease activity in Korean patients [24,27]. Therefore, in-creased inflammatory burdens, rather than disease subtypes or ANCA types, may be an important factor influencing lipid profiles in patients with AAV.…”

Section: Discussionmentioning

confidence: 99%

Lipid Profiles in Anti-neutrophil Cytoplasmic Antibody-associated Vasculitis: A Cross-sectional Analysis

2020

View full text Add to dashboard Cite

Objective. Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is an autoimmune disease (AID) characterised by necrotising intravascular inflammation. Growing evidence suggests that immune system triggers altered lipid metabolism in AIDs. We investigated whether changes in lipid profile correlate with severity of disease in AAV. Methods. Seven lipid profiles were evaluated utilizing frozen serum samples from 67 patients registered in the Severance Hospital ANCA-associated VasculitidEs cohort by a chemistry autoanalyzer. The Birmingham Vasculitis Activity Score (BVAS) version 3 was used to measure patient's assessment of global disease activity. The relationship between the BVAS with continuous variables was calculated by Pearson's correlation analysis. Results. Thirty-five (52.2%), 19 (28.4%), and 13 (19.4%) patients were diagnosed with microscopic polyangiitis, granulomatosis with polyangiitis, and eosinophilic granulomatosis with polyangiitis, respectively. Patients' mean age was 60.0 years, and 22 (32.8%) were male. Among the lipid profiles investigated, total cholesterol, high-density lipoprotein, and low-density lipoprotein, and apolipoprotein A1 and B were significantly associated with BVAS; apolipoprotein A1 showed the highest correlation with BVAS (r=−0.521, p＜0.001), remaining consistent even in patients with new-onset disease (r=−0.430, p=0.012). Apolipoprotein A1 had the highest association with the renal manifestation score among the clinical scores comprising BVAS (r=−0.457, p＜0.001). Conclusion. Decreased lipid levels, especially apolipoprotein A1, are relevant to increased AAV disease activity, and differ according to organ involvement. Measuring lipid profiles could have clinical implications regarding the assessment of global disease activity and organ involvement patterns.

show abstract

ANCA-Associated Vasculitis: Core Curriculum 2020

Cited by 311 publications

References 0 publications

Autoantibody Biomarkers in Rheumatic Diseases

Autoantibody Biomarkers in Rheumatic Diseases

Granulomatosis with polyangiitis (GPA) in a 76-year old woman presenting with pulmonary nodule and accelerating acute kidney injury

Lipid Profiles in Anti-neutrophil Cytoplasmic Antibody-associated Vasculitis: A Cross-sectional Analysis

Contact Info

Product

Resources

About