Antineutrophilic Cytoplasmic Antibodies 연관 혈관염 환자에서 발생한 양안의 삼출성 망막박리 1예

Ji Young Suh,Woo Jin Jeong 한국망막학회 2016 Journal of Retina Vol.1 No.1

Purpose: To report a rare case of bilateral exudative retinal detachment in an antineutrophilic cytoplasmic antibodies (ANCA)-associated vasculitis patient. Case summary: A 28-year-old woman presented with decreased vision and was found to have bilateral exudative retinal detachment. Fluorescein angiography showed multiple hyperfluorescent signals, and optical coherence tomography showed subretinal fluid. The patient had a history of chronic sclerosing glomerulonephritis and tested positive for ANCA. She was treated with a combination therapy of cyclophosphamide and a high dose of systemic methylprednisolone and responded rapidly. By week 4, vision had normalized and clinical signs had been resolved. Conclusions: We report a rare case of bilateral exudative retinal detachment in an ANCA-associated vasculitis patient that was treated successfully with a combination of cyclophosphamide and methylprednisolone. 목적: Antineutrophilic cytoplasmic antibodies (ANCA) 연관 혈관염 환자에서 양안의 삼출성 망막 박리 1예를 경험하였기에 이를 보고하고자 한다. 증례요약: 28세 여자 환자가 양안의 시력저하로 내원하였다. 안저 검사에서 양안의 삼출성 망막박리 소견을 보였으며, 형광안저혈관조영에서 초기에 다발성 점상 누출이 관찰되었고, 황반부 빛간섭단층촬영에서 망막하액 소견을 보였다. 환자는 만성 경화성 사구체신염으로 진단 받은 바 있었고 혈액 검사상 ANCA 양성이었다. 이를 바탕으로 ANCA-연관 혈관염과 동반된 삼출성 망막박리로 진단하였고, 싸이클로포스파마이드 및 고용량의 전신적 스테로이드 병합 치료를 시행하였다. 환자는 치료에 잘 반응하여 치료 한 달 후 안저검사상삼출성 망막박리가 완전히 흡수되었으며, 시력도 호전되었다. 결론: ANCA 연관 혈관염 환자에서 양안의 삼출성 망막 박리가 발생한 드문 예를 경험하고 이를 효과적으로 치료하였기에 이를 보고하는 바이다.

Clinical features of Korean elderly patients with antineutrophil cytoplasmic antibody-associated vasculitis

( Byung-woo Yoo ),( Jason Jungsik Song ),( Yong-beom Park ),( Sang-won Lee ) 대한내과학회 2021 The Korean Journal of Internal Medicine Vol.36 No.3

Background/Aims: We compared the clinical and laboratory data between elderly and non-elderly patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) at diagnosis; further, we investigated the predictors at diagnosis for all-cause mortality and end-stage renal disease (ESRD) occurrence during follow-up in Korean elderly patients with AAV. Methods: We reviewed the medical records of 191 AAV patients regarding clinical manifestations and laboratory results at diagnosis and during follow-up. The follow-up duration was defined as the period from diagnosis to death for deceased patients or to the time of dialysis for ESRD patients, or to the last visit. Elderly (n = 67) and non-elderly (n = 124) patients were grouped based on an age threshold of 65 years. Results: At diagnosis, elderly patients exhibited higher median Birmingham Vasculitis Activity Score (BVAS) and higher frequencies of ANCA positivity and pulmonary manifestations than non-elderly patients. Furthermore, elderly patients exhibited increased median white blood cell count, blood urea nitrogen (BUN), alkaline phosphatase, erythrocyte sedimentation rate, and C-reactive protein and decreased median hemoglobin. However, there were no significant differences in all-cause mortality and ESRD occurrence between elderly and non-elderly patients. Meanwhile, elderly patients exhibited lower cumulative patients’ and ESRD-free survival rates than non-elderly patients. In the multivariable Cox hazards model, BUN, creatinine and serum albumin at diagnosis were independent predictors for ESRD occurrence, whereas there were no independent predictors at diagnosis for all-cause mortality. Conclusions: Elderly AAV patients exhibited substantially higher rates of all-cause mortality and ESRD occurrence during follow-up compared than non-elderly AAV patients.

Comparison of the 2022 ACR/EULAR Classification Criteria for Antineutrophil Cytoplasmic Antibody-Associated Vasculitis with Previous Criteria

표정윤,Lucy Eunju Lee,박용범,이상원 연세대학교의과대학 2023 Yonsei medical journal Vol.64 No.1

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) comprises group of small vessel vasculitides, including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). In 2022, the American College of Rheumatology (ACR) and the European Alliance of Associations for Rheumatology (EULAR) jointly proposed new classification criteria for AAV (the 2022 ACR/EULAR criteria). In this review, we briefly summarize the 2022 ACR/EULAR criteria for GPA, MPA, and EGPA, and introduce our clinical experience with applying them to patients who were previously diagnosed with AAV based on three criteria: firstly, the classification criteria for GPA and EGPA proposed by the ACR in 1990; secondly, the algorithm for the classification of AAV and polyarteritis nodosa proposed by the European Medi cines Agency algorithm in 2007 (the 2007 EMA algorithm); and thirdly, the revised International Chapel Hill Consensus Confer ence nomenclature of vasculitides in 2012 (the 2012 CHCC definitions). We found that concordance rate was highest in patients with MPA (96.6%), followed by those with EGPA (86.3%) and GPA (73.8%). In addition, compared to previous criteria, we noted several issues of the undervalued or overvalued items in the 2022 ACR/EULAR criteria for classifying AAV and provided several suggestions. To increase the diagnostic accuracy and reduce the discordance rate among the new and previous criteria for AAV, we suggest that the previous criteria should be considered together with the 2022 ACR/EULAR criteria when applying the classifi cation criteria for AAV to patients suspected of AAV.

Clinical features and outcomes of elderly patients with antineutrophil cytoplasmic antibody-positive vasculitis: a single-center retrospective study

김효진,한미연,송상헌,성은영 대한신장학회 2022 Kidney Research and Clinical Practice Vol.41 No.2

BackgroundWe aimed to investigate the clinical characteristics and outcomes of patients aged ≥65 years with antineutrophil cytoplasmic autoantibody (ANCA)-positive ANCA-associated vasculitis (AAV) in Korea.MethodsSeventy patients diagnosed with ANCA-positive AAV from 2006 to 2019 at a single center were analyzed and categorized into younger (aged <65 years) or elderly (aged ≥65 years) groups. Initial induction treatments were investigated according to age group. All-cause mortality and kidney outcomes were evaluated.ResultsAfter categorization by age, 34 (48.6%) and 36 patients (51.4%) were in the younger and elderly groups, respectively. In the elderly group, more patients were treated with oral cyclophosphamide (CYC) (30.6%) than with intravenous CYC (19.4%). During a median follow-up of 14.6 months (range, 3.0-53.1 months), 13 patients died (elderly group: 11 patients, 84.6%). In the elderly group, older age (hazard ratio [HR], 1.44; 95% confidence interval [CI], 1.09-1.90; p = 0.01), lower hemoglobin (HR, 0.21; 95% CI, 0.08-0.60; p = 0.003), and higher serum creatinine level (HR 14.17; 95% CI, 1.29-155.84; p = 0.03) were significant risk factors for all-cause mortality after adjustment. Oral CYC + steroid treatment was associated with decreased all-cause mortality compared to untreated induction immunosuppressants (HR, 0.01; 95% CI, 0.0003-0.47; p = 0.02). Kidney failure or renal recovery outcomes were not significantly different between the younger and elderly groups.ConclusionPatients aged ≥65 years had higher mortality rates than younger patients, and mortality was associated with older age, lower hemoglobin, higher serum creatinine level, and nontreatment compared to oral CYC + steroids.

폐결핵과 동시에 발병한 ANCA연관혈관염

석정임,박성훈,김경찬 대한신경과학회 2012 대한신경과학회지 Vol.30 No.1

Antineutrophil cytoplasmic antibodies (ANCA) are closely linked to primary systemic vasculitis, and ANCA detection has became an important diagnostic hallmark of ANCA-associated vasculitis (AAV). However, it has been reported that tuberculosis is associated with positivity for ANCA and it is difficult to differentiate clinically between tuberculosis and AAV. We report a patient with the concomitant appearance of AAV and pulmonary tuberculosis. Positivity for ANCA should be carefully interpreted as indicative of AAV, especially in countries with a high prevalence of tuberculosis.

The Efficacy of Mycophenolate Mofetil in Remission Maintenance Therapy for Microscopic Polyangiitis and Granulomatosis with Polyangiitis

표정윤,이은주,안성수,송정식,박용범,이상원 연세대학교의과대학 2021 Yonsei medical journal Vol.62 No.6

Purpose: The present study compared the efficacy of mycophenolate mofetil (MMF) with that of azathioprine (AZA) in Koreanpatients with microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA). Materials and Methods: The medical records of 69 patients with MPA and GPA who received cyclophosphamide and subsequentlyreceived AZA or MMF for remission maintenance therapy were reviewed. All-cause mortality, relapse, end-stage renal disease(ESRD), cerebrovascular accident, and cardiovascular disease were evaluated as poor outcomes. Having a lower BirminghamVasculitis Activity Score (BVAS) was defined as the lowest tertile of BVAS (BVAS ≤11 in this study). Results: In comparative analysis of the occurrence of poor outcomes among patients taking AZA only, MMF only, and MMF afterAZA, patients taking MMF only exhibited a significantly lower cumulative ESRD-free survival rate than patients taking AZA only(p=0.028). In terms of ESRD occurrence between the groups based on BVAS at diagnosis, among patients with MPA and GPA withhigher BVAS at diagnosis, patients taking MMF only exhibited a significantly lower cumulative ESRD-free survival rate than thosetaking AZA only (p=0.047). Among patients with MPA and GPA with the lowest tertile of BVAS at diagnosis, cumulative ESRD-freesurvival rates did not differ. Conclusion: With regard to ESRD occurrence, the efficacy of MMF in remission maintenance therapy was less effective than AZAin patients with MPA and GPA. However, among patients with lower BVAS, there was no difference in the occurrence of poor outcomesbetween patients taking MMF and those taking AZA.

A Case of Wegener's Granulomatosis with Central Nervous System Involvement Mimicking Lung Cancer with Brain Metastasis

박주희,이영호,지종대,송관규,이순욱,유서희,류자영,김해림,강근희,강성희,김선화,최성재 대한류마티스학회 2013 대한류마티스학회지 Vol.20 No.3

Wegener's granulomatosis (WG) classically consists of necrotizing granulomatous inflammation of the upper and/or lower respiratory tract, necrotizing glomerulonephritis, and an autoimmune necrotizing systemic vasculitis affecting predominantly small vessels. We report a case of WG with central nervous system (CNS) involvement. WG is being diagnosed through pulmonary nodule biopsy. A small nodular lesion in the left posterior basal ganglia of brain being highly suspicious for granulomatosis was detected by MRI. After IV pulse cyclophosphamide and oral corticosteroid treatment for over 4 months, clinical manifestations and CNS lesions in brain MRI is improved. WG might have multiple granulomatous lesions which could be misdiagnosed due to malignancy. CNS involvement in WG is rare but careful evaluation is necessary when there are suspicious symptoms or lesions in CNS.