Font J, Ramos-Casals M, Cervera R, Bosch X, Mirapeix E, García-Carrasco M, Morlà R M, Ingelmo M
Department of Medicine, Institut d'Investigacions Biomèdiques August Pi i Sunyer, Hospital Clínic, School of Medicine, University of Barcelona, Catalonia, Spain.
Br J Rheumatol. 1998 Dec;37(12):1287-91. doi: 10.1093/rheumatology/37.12.1287.
To evaluate the prevalence of cytoplasmic (c) and perinuclear (p) antineutrophil cytoplasmic antibodies (ANCA) in patients with primary Sjogren's syndrome (SS), and to correlate the presence of ANCA with extraglandular and immunological manifestations related to SS.
In a cross-sectional study, we included 82 consecutive patients (75 female and seven male; mean age 61 yr; range 33-87 yr) attending our unit. All patients fulfilled four or more of the diagnostic criteria for SS proposed by the European Community Study Group in 1993. Extraglandular manifestations such as arthralgia and/or arthritis, Raynaud's phenomenon, autoimmune thyroiditis, peripheral neuropathy, renal involvement and cutaneous vasculitis were also recorded. Serum samples were examined by indirect immunofluorescence (IIF) and by ELISA using as substrates myeloperoxidase (MPO) and proteinase 3 (PR3).
ANCA were detected in nine (11%) patients: seven had pANCA and two an atypical pattern. These two atypical ANCA became cANCA when paraformaldehyde fixation was applied. ELISA findings showed that two patients had antibodies against MPO, and no patient had antibodies to PR3. The most common extraglandular manifestations in the ANCA-positive patients were articular involvement in six (66%) patients, peripheral neuropathy in five (55%), Raynaud's phenomenon in four (44%) and cutaneous vasculitis in four (44%). Of the four patients with cutaneous vasculitis and ANCA, two had a mononuclear inflammatory vascular disease (MIVD) in the biopsy specimen. When compared with patients without ANCA, those with these antibodies had a higher prevalence of cutaneous vasculitis (44% vs 8%, P = 0.01), Raynaud's phenomenon (44% vs 8%, P = 0.01) and peripheral neuropathy (55% vs 7%, P < 0.001).
ANCA positivity can be found in patients with primary SS and its detection is associated with the presence of clinical manifestations attributable to vascular involvement (cutaneous vasculitis, peripheral neuropathy and Raynaud's phenomenon).
评估原发性干燥综合征（SS）患者中胞浆型（c）和核周型（p）抗中性粒细胞胞浆抗体（ANCA）的患病率，并将ANCA的存在与SS相关的腺外表现和免疫表现进行关联分析。
在一项横断面研究中，我们纳入了连续就诊于我院的82例患者（75例女性和7例男性；平均年龄61岁；范围33 - 87岁）。所有患者均符合1993年欧洲共同体研究小组提出的SS诊断标准中的四项或更多项。还记录了关节痛和/或关节炎、雷诺现象、自身免疫性甲状腺炎、周围神经病变、肾脏受累及皮肤血管炎等腺外表现。血清样本通过间接免疫荧光法（IIF）以及以髓过氧化物酶（MPO）和蛋白酶3（PR3）为底物的酶联免疫吸附测定（ELISA）进行检测。
在9例（11%）患者中检测到ANCA：7例为pANCA，2例为非典型模式。当应用多聚甲醛固定时，这2例非典型ANCA变为cANCA。ELISA结果显示，2例患者有抗MPO抗体，无患者有抗PR3抗体。ANCA阳性患者中最常见的腺外表现为6例（66%）有关节受累，5例（55%）有周围神经病变，4例（44%）有雷诺现象，4例（44%）有皮肤血管炎。在4例有皮肤血管炎和ANCA的患者中，2例活检标本显示为单核细胞炎性血管病（MIVD）。与无ANCA的患者相比，有这些抗体的患者皮肤血管炎（44%对8%，P = 0.01）、雷诺现象（44%对8%，P = 0.01）和周围神经病变（55%对7%，P < 0.001）的患病率更高。
原发性SS患者中可发现ANCA阳性，其检测与血管受累相关的临床表现（皮肤血管炎、周围神经病变和雷诺现象）的存在有关。
