Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
Rheumatology (Oxford). 2021 Aug 2;60(8):3845-3850. doi: 10.1093/rheumatology/keab124.
To evaluate the prevalence and meaning of antineutrophil cytoplasmic antibodies (ANCA) positivity in a cohort of IgG4-related disease (IgG4-RD).
We identified patients with ANCA determination from a retrospective cohort of 69 patients with IgG4-RD. ANCA were measured by indirect immunofluorescence microscopy (IIF) and/or proteinase 3 (PR3)-ANCA and MPO-ANCA by ELISA. IIF patterns were classified as perinuclear (P-ANCA), cytoplasmic (C-ANCA) and atypical (X-ANCA). We compared the ANCA-positive vs the ANCA-negative IgG4-RD group.
Out of 69 patients, 31 IgG4-RD patients had an ANCA determination. Four patients with concomitant systemic autoimmune diseases were excluded. We found positive ANCA by IIF in 14 (56%) of 25 patients tested. The most common IIF pattern was C-ANCA in eight (57.1%), followed by dual C-ANCA/X-ANCA in four (28.6%) and P-ANCA and dual C-ANCA/P-ANCA in one each (7.1%). Of the 20 patients with ANCA determination by both IIF and ELISA, four have positive ANCA by ELISA (three for MPO-ANCA and one for PR3-ANCA). Of the two patients with only ELISA determination, one was positive for MPO-ANCA. The prevalence of ANCA positivity by ELISA was 22.7% (5 out of 22 patients). ANCA was more frequent in the Mikulizc/systemic phenotype (42.9%) compared with other phenotypes (P = 0.04). ANCA-positive IgG4-RD patients had more frequently lymph node and kidney involvement, high IgG1 levels and erythrocyte sedimentation rate, and positive antinuclear antibodies.
ANCA are found in a significant number of patients with IgG4-RD and differed from the ANCA-negative group in terms of clinical and serological features.
评估 IgG4 相关疾病（IgG4-RD）患者抗中性粒细胞胞浆抗体（ANCA）阳性的患病率及意义。
我们从 IgG4-RD 的回顾性队列中确定了进行 ANCA 检测的患者。通过间接免疫荧光法（IIF）和/或蛋白酶 3（PR3）-ANCA 和髓过氧化物酶（MPO）-ANCA ELISA 检测 ANCA。IIF 模式分为核周型（P-ANCA）、细胞质型（C-ANCA）和非典型（X-ANCA）。我们比较了 ANCA 阳性和 ANCA 阴性 IgG4-RD 组。
在 69 例患者中，有 31 例 IgG4-RD 患者进行了 ANCA 检测。排除了同时患有系统性自身免疫性疾病的 4 例患者。我们发现，在 25 例接受检测的患者中，有 14 例（56%）通过 IIF 检测到 ANCA 阳性。最常见的 IIF 模式为 8 例（57.1%）的 C-ANCA，其次为 4 例（28.6%）的 C-ANCA/X-ANCA 双重模式和 1 例（7.1%）的 P-ANCA 和双重 C-ANCA/P-ANCA 模式。在通过 IIF 和 ELISA 均进行 ANCA 检测的 20 例患者中，有 4 例通过 ELISA 检测到 ANCA 阳性（3 例为 MPO-ANCA，1 例为 PR3-ANCA）。在仅进行 ELISA 检测的 2 例患者中，有 1 例 MPO-ANCA 阳性。通过 ELISA 检测到的 ANCA 阳性率为 22.7%（22 例患者中有 5 例）。Mikulizc/系统性表型的 ANCA 阳性率（42.9%）高于其他表型（P=0.04）。与 ANCA 阴性 IgG4-RD 患者相比，ANCA 阳性 IgG4-RD 患者更常伴有淋巴结和肾脏受累、高 IgG1 水平和红细胞沉降率以及抗核抗体阳性。
在相当数量的 IgG4-RD 患者中发现了 ANCA，且其临床和血清学特征与 ANCA 阴性组不同。
