류마티스 관절염과 동반된 비정형 베게너 육아종증 1예
Atypical Wegener’s Granulomatosis Accompanied with Rheumatoid Arthritis: A Case Report
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베게너 육아종증(Wegener’s granulomatosis, WG)은 항호중구세포질항체(antineutrophil cytoplasmic antibodies, ANCA) 중 세포질(cytoplasmic) ANCA (c- ANCA)와 높은 연관성을 보이는 것으로 알려져 있다. 류마티스 관절염(rheumatoid arthritis, RA) 환자에서 WG가 발생하는 경우는 매우 드물며 해외에서는 몇 차례 보고된 적이 있으나 국내에서는 아직 보고된 사례가 없다. 본 저자들은 RA 환자에서 발생한 WG에서 핵주위(perinuclear) ANCA (p-ANCA) 양성이면서 항myeloperoxidase (MPO) 항체가 음성으로 관찰되어 보고하는 바이다. 62세 여자환자가 다발성 관절통을 주소로 내원하였다. 혈액검사에서 류마티스 인자와 항cyclic citrullinated peptide 항체는 고역가 양성이었다. 적혈구 침강속도와 C반응단백질은 증가되어 있었다. 2010년 미국 류마티스 학회와 유럽 류마티스 학회에서 공동으로 정한 RA의 분류범주를 적용하면 총 10점으로 RA가 분명한 환자였다. Autoimmune target 검사에서 반점형(speckled pattern), 골격형(skeleton pattern)이 각각 1:160의 역가로 관찰되었고 ANCA 검사에서 p-ANCA가 1:2,560의 역가로 관찰되었다. 항proteinase3 항체와 항MPO 항체는 음성이었다. 입원 후 시행한 흉부 X선 검사와 흉부 컴퓨터 단층촬영 검사에서 다발성 결절이 발견되었고 류마티스 결절이나 혈관염이 의심되어 개방폐생검을 실시하였다. 괴사성 육아종성 혈관염이 발견되어 WG로 진단하였다. RA를 가진 환자에서 발생한 WG의 특이한 자가항체 양상(c-ANCA 음성, p-ANCA 양성, 항MPO 항체 음성)을 증례로 보고하는 바이며, 이를 통해 향후 WG의 진단에 도움이 되고자 한다.
Abstract
Wegener’s granulomatosis (WG) is highly correlated with cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA). Patients with rheumatoid arthritis (RA) rarely contract WG. Although several concurrent cases have been reported overseas, there are no known cases in Korea. Here we report a unique case of RA with atypical WG testing positive for perinuclear ANCA (p-ANCA) and negative for anti-myeloperoxidase (MPO) antibodies. The 62-yr-old female patient presented with multiple joint pain and showed typical blood test results for RA, i.e., an elevated erythrocyte sedimentation rate and C-reactive protein concentration, and positive for rheumatoid factor and anti-cyclic citrullinated peptide antibodies. RA was clear based on a total score of 10 when applying the classification criteria developed by the American College of Rheumatology/European League Against Rheumatism (2010). In an autoimmune target test, speckled and skeleton patterns were observed. In an ANCA test, p-ANCA was observed (titer, 1:2,560), and tests for anti-proteinase 3 (PR3) and anti-MPO antibodies were negative. After admission, multiple nodules were detected on a chest X-ray and a computed tomography scan. We suspected that she had rheumatic nodules or vasculitis and performed an open lung biopsy. We detected necrotic granulomatous vasculitis, classified as WG, thus leading to WG diagnosis. In conclusion, WG was diagnosed in an RA patient who was negative for c-ANCA (negative PR3) and positive for p-ANCA (negative anti-MPO), and this peculiar finding is likely to improve diagnosis in cases of RA with atypical WG.
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