Ghirardello A, Zampieri S, Tarricone E, Iaccarino L, Bendo R, Briani C, Rondinone R, Sarzi-Puttini P, Todesco S, Doria A
University of Padova, Division of Rheumatology, Department of Clinical and Experimental Medicine, Italy.
Autoimmunity. 2006 May;39(3):217-21. doi: 10.1080/08916930600622645.
To evaluate the clinical usefulness of serum autoantibody profiling in patients with autoimmune myositis.
We retrospectively studied 74 consecutive patients: 68 had definite or probable myositis according to Bohan-Peter criteria, six suffered from antisynthetase syndrome with subclinical myopathy. Myositis specific antibodies (MSA) (anti-ARS, -SRP, -Mi-2) were determined by RNA immunoprecipitation or immunoblot, myositis associated antibodies (MAA) (anti-RoRNP, -U1RNP, -PM/Scl, -Ku) by immunoblot.
Forty-three patients (58%) were positive for MSA: anti-Jo-1 in 15/27 polymyositis (PM) (55%), 4/33 dermatomyositis (DM) (12%), 1/8 overlap (12%) and 2/6 antisynthetase syndrome (33%); anti-ARS non-Jo-1 in 1/27 PM (4%), 2/33 DM (6%) and 4/6 antisynthetase syndrome (67%); anti-Mi-2 in 1/27 PM (4%) and 11/33 DM (33%); anti-SRP in 3/27 PM (11%) and 1/33 DM (3%). One patient was anti-Jo-1/Mi-2 positive, one anti-Jo-1/SRP positive. Moreover, 27 patients (36%) were positive for MAA: anti-Ro/SSA in 8/27 PM (30%), 7/33 DM (21%), 1/8 overlap (12%), and 3/6 antisynthetase syndrome (50%); anti-U1RNP in 1/27 PM (3.7%), 1/33 DM (3%), and 2/8 overlap (25%); anti-PM/Scl in 2/8 overlap (25%), anti-Ku in 2/8 overlap (25%). Anti-Jo-1 was predominantly associated with PM, anti-Mi-2 was almost exclusively found in DM patients. Anti-ARS antibodies were closely associated with interstitial lung disease and polyarthritis; notably, anti-ARS non-Jo-1 was more frequent in patients without overt muscle alterations. Anti-Ro/SSA antibody was not associated with any disease subset, but significantly more frequent in antisynthetase syndrome.
Searching for MSA and MAA in patients with autoimmmune myositis is recommended because of its diagnostic and clinical value. Anti-ARS non-Jo-1 antibodies seem to preferentially target patients with pulmonary fibrosis without overt myopathy.
评估血清自身抗体谱分析在自身免疫性肌炎患者中的临床应用价值。
我们回顾性研究了74例连续患者：68例根据博汉 - 彼得标准诊断为确诊或可能的肌炎，6例患有抗合成酶综合征且伴有亚临床肌病。通过RNA免疫沉淀或免疫印迹法检测肌炎特异性抗体（MSA）（抗ARS、抗SRP、抗Mi - 2），通过免疫印迹法检测肌炎相关抗体（MAA）（抗RoRNP、抗U1RNP、抗PM/Scl、抗Ku）。
43例患者（58%）MSA呈阳性：15/27例多发性肌炎（PM）患者抗Jo - 1阳性（55%），4/33例皮肌炎（DM）患者阳性（12%），1/8例重叠综合征患者阳性（12%），2/6例抗合成酶综合征患者阳性（33%）；1/27例PM患者抗ARS非Jo - 1阳性（4%），2/33例DM患者阳性（6%），4/6例抗合成酶综合征患者阳性（67%）；1/27例PM患者抗Mi - 2阳性（4%），11/33例DM患者阳性（33%）；3/27例PM患者抗SRP阳性（11%），1/33例DM患者阳性（3%）。1例患者抗Jo - 1/Mi - 2阳性，1例抗Jo - 1/SRP阳性。此外，27例患者（36%）MAA呈阳性：8/27例PM患者抗Ro/SSA阳性（30%），7/33例DM患者阳性（21%），1/8例重叠综合征患者阳性（12%），3/6例抗合成酶综合征患者阳性（50%）；1/27例PM患者抗U1RNP阳性（3.7%），1/33例DM患者阳性（3%），2/8例重叠综合征患者阳性（25%）；2/8例重叠综合征患者抗PM/Scl阳性（25%），2/8例重叠综合征患者抗Ku阳性（25%）。抗Jo - 1主要与PM相关，抗Mi - 2几乎仅见于DM患者。抗ARS抗体与间质性肺病和多关节炎密切相关；值得注意的是，抗ARS非Jo - 1在无明显肌肉改变的患者中更常见。抗Ro/SSA抗体与任何疾病亚组均无关联，但在抗合成酶综合征中显著更常见。
鉴于其诊断和临床价值，建议对自身免疫性肌炎患者进行MSA和MAA检测。抗ARS非Jo - 1抗体似乎优先靶向无明显肌病的肺纤维化患者。
