Five factor score of more than 1 is associated with relapse during the first 2 year‐follow up in patients with eosinophilic granulomatosis with polyangiitis

Choi

Clinical Laboratory Analysis

et al. 2019

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Background: So far, there has been no tool to estimate activity at diagnosis and predict all-cause mortality in patients with ANCA-associated vasculitis (AAV). Hence, we determined the initial predictors of them in patients with AAV. Methods:We retrospectively reviewed the medical records of 182 patients with AAV. Severe AAV was defined as Birmingham Vasculitis Activity Score (BVAS) ≥ 16.The cutoffs were extrapolated by the receiver operator characteristic (ROC) curve.The odds ratio (OR) and the relative risk (RR) were assessed using the multivariable logistic regression analysis and the chi-square test, respectively. Results:In the comparison analysis, patients with severe AAV exhibited the higher neutrophil and platelet counts, creatinine, erythrocyte sedimentation rate and C-reactive protein, and the lower lymphocyte count, hemoglobin, and serum albumin than those without. In the multivariable logistic regression analysis, creatinine ≥ 0.9 mg/dL (OR 2.264), lymphocyte count ≤ 1430.0/mm 3 (OR 1.856), and hemoglobin ≤ 10.8 g/ dL (OR 2.085) were associated with severe AAV. We developed a new equation of a multivariable index for AAV (MVIA) = 0.6 × (Lymphocyte count ≤ 1430.0/mm 3 ) + 0. 7 × (Hemoglobin ≤ 10.8 g/dL) + 0.8 × (Creatinine ≥ 0.9 mg/dL). The optimal cutoff of MVIA for severe AAV was set as 1.35. Severe AAV was identified more frequently in patients with MVIA at diagnosis ≥1.35 than those without (RR 4.432). Patients with MVIA at diagnosis ≥1.35 exhibited the lower cumulative patient survival rate than those without. Conclusion:Multivariable index for AAV could assess the cross-sectional activity and predict all-cause mortality in patients with AAV.

Section: Discussionsupporting

confidence: 89%

Multivariable index for assessing the activity and predicting all‐cause mortality in antineutrophil cytoplasmic antibody‐associated vasculitis

Choi

Clinical Laboratory Analysis

et al. 2019

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“…The mean FFS was 0.9, and a FFS of 1.0 was calculated as a cut-off to predict relapse of EGPA by a receiver operator characteristic curve. Finally, we demonstrated that relapse was more frequent in patients with FFS ≥1 than those with FFS <1 (68.8% vs. 7.1%, relative risk 28.6) 34…”

Section: Predictive Factors For Prognosis In Korean Patients With Egpamentioning

confidence: 74%

“…Proteinuria (>1 g/day) was significantly more often observed in both MPO-ANCA-positive and PR3-ANCA-positive patients than in ANCA-negative patients (27.3% vs. 6.3% and 66.7% vs. 6.3%, respectively). In addition, MPO-ANCA-positive patients with EGPA showed a higher relapse rate than ANCA-negative patients with EGPA (54.6% vs. 25.0%) 34. Taking the results of the two previous studies together, in Korean patients with EGPA, ANCA positivity may be associated with skin and renal involvement for EGPA at diagnosis and with the occurrence of relapse during follow-up.…”

Section: Comparison Between Anca-positive Versus Anca-negative Egpamentioning

confidence: 76%

Eosinophilic Granulomatosis with Polyangiitis: Experiences in Korean Patients

2019

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Eosinophilic granulomatosis with polyangiitis (EGPA) is one form of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Identical to what has been called Churg-Strauss syndrome, EGPA exhibits both allergic and vasculitis features. EGPA was first described as a syndrome consisting of asthma, fever, eosinophilia, and organ involvement including heart failure, neuropathy, and kidney damage, by Churg and Strauss in 1951. On the basis of the 2012 Chapel Hill Consensus Conferences Nomenclature of Vasculitis, EGPA comprises three typical allergic components, including asthma, peripheral eosinophilia, and eosinophil-rich granuloma of the respiratory tracts. EGPA has three clinical and histological stages. The first is an allergic stage composed of asthma and sinusitis, and the second is an eosinophilic stage characterised by peripheral hypereosinophilia and intra-organ infiltration of eosinophils. The last is a vasculitic stage, including necrotising inflammation of small vessels and end-organ damage. In this review, we describe the classification criteria for EGPA and recommendations for the evaluation and management of EGPA with conventional and newly suggested drugs for EGPA. Also, we discuss a variety of clinical aspects such as predictive values for prognosis and associations with other Th2-mediated diseases and hepatitis B virus.

“…Also, older age, diagnosis at earlier stage, less cutaneous involvement, more respiratory involvement, high C-reactive protein (CRP), and more generalized symptoms were observed in good responders. Meanwhile, in another study, Kim, et al42 investigated initial variables associated with relapse during follow-up in Korean patients with EGPA. They first obtained the optimal cut-off of FFS to predict relapse of EGPA as 1.…”

Section: Predictors At Diagnosis Of Prognosismentioning

confidence: 99%

Antineutrophil Cytoplasmic Antibody-Associated Vasculitis in Korea: A Narrative Review

2019

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Antineutrophil cytoplasmic antibody-associated vasculitis (AAV) is a group of systemic necrotising vasculitides, which often involve small vessels, and which lead to few or no immune deposits in affected organs. According to clinical manifestations and pathological features, AAV is classified into three variants: microscopic polyangiitis, granulomatosis with polyangiitis (GPA), and eosinophilic GPA. The American College of Rheumatology 1990 criteria contributed to the classification of AAV, although currently the algorithm suggested by the European Medicines Agency in 2007 and the Chapel Hill Consensus Conference Nomenclature of Vasculitides proposed in 2012 have encouraged physicians to classify AAV patients properly. So far, there have been noticeable advancements in studies on the pathophysiology of AAV and the classification criteria for AAV in Western countries. However, studies analysing clinical features of Korean patients with AAV have only been conducted and reported since 2000. One year-, 5 year-, and 10 year-cumulative patient survival rates are reported as 96.1, 94.8, and 92.8%. Furthermore, initial vasculitis activity, prognostic factor score, age and specific organ-involvement have been found to be associated with either all-cause mortality or poor disease course. The rate of serious infection is 28.6%, and 1 year-, 5 year- and 10 year-cumulative hospitalised infection free survival rates range from 85.1% to 72.7%. The overall standardised incidence ratio of cancer in AAV patients was deemed 1.43 compared to the general Korean population.