Ghirardello A, Doria A, Vesco P, Vaccaro E, Bernardi C, Catani C, Fagiolo U, Gambari P F
Division of Rheumatology, University of Padova, Italy.
Rheumatol Int. 1996;16(4):145-50. doi: 10.1007/BF01419727.
Serum reactivities towards individual U1 snRNP proteins were determined by immunoblotting in 32 patients with mixed connective tissue disease (MCTD). Time persistence of immunoblot profiles and clinical significance of anti-(U1)RNP antibody specificities were also investigated. IgG anti-(U1)RNP antibodies were found in the sera of 29 out of 32 patients (90.6%): 21 (65.6%) reacted with the 70-kD protein, 25 (78.1%) with A, 23 (71.9%) with C and 20 (62.5%) with B/B' proteins. None were reactive with the Sm-D peptide. Seventy kilodalton antibody specificity was strongly associated with a higher antinuclear antibody titre (> 160) and slightly associated with disease activity; anti-B/B' specificity was associated with lymphadenopathy. Anti-A, -C and -B/B' antibodies were negatively associated with systemic lupus erythematosus (SLE) skin rashes. Two types of anti-(U1)RNP blotting patterns were selected: "full spectrum" (53.1% of cases) and a "partially/no reactive" one (46.9%). Such patterns were unchanged over time in 14 out of 16 cases prospectively examined (87.5%), while the pattern shifted from "full spectrum" to "partially/no reactive" in 2 cases (12.5%): in 1 after a prolonged clinical remission (> or = 4 years) and in the other following immunosuppressive therapy. The anti-(U1)RNP antibody immunoblot profile in MCTD patients consisted of various reactivities and remained unchanged over time in most cases. Antibody reactivity against the 70-kD protein represented the major U1 snRNP specificity. The various anti-(U1)RNP specific reactivities demonstrated poor clinical significance within MCTD. Thus, MCTD seems to be characterized by a longstanding serological heterogeneity whose reactivities do not apparently correspond to distinct features within the broad clinical spectrum of MCTD.
通过免疫印迹法测定了32例混合性结缔组织病(MCTD)患者血清对单个U1 snRNP蛋白的反应性。还研究了免疫印迹图谱的时间持续性以及抗(U1)RNP抗体特异性的临床意义。在32例患者中的29例(90.6%)血清中发现了IgG抗(U1)RNP抗体:21例(65.6%)与70-kD蛋白反应,25例(78.1%)与A蛋白反应,23例(71.9%)与C蛋白反应,20例(62.5%)与B/B'蛋白反应。无一例与Sm-D肽反应。70-kD抗体特异性与较高的抗核抗体滴度(>160)密切相关,与疾病活动度轻度相关;抗B/B'特异性与淋巴结病相关。抗A、抗C和抗B/B'抗体与系统性红斑狼疮(SLE)皮疹呈负相关。选择了两种抗(U1)RNP印迹模式:“全谱型”(53.1%的病例)和“部分/无反应型”(46.9%)。在16例前瞻性检查的病例中,有14例(87.5%)这种模式随时间未发生变化,而有2例(12.5%)模式从“全谱型”转变为“部分/无反应型”:1例在长期临床缓解(≥4年)后发生转变,另1例在免疫抑制治疗后发生转变。MCTD患者的抗(U1)RNP抗体免疫印迹图谱由多种反应性组成,且在大多数情况下随时间保持不变。针对70-kD蛋白的抗体反应性代表了主要的U1 snRNP特异性。在MCTD中,各种抗(U1)RNP特异性反应性的临床意义不大。因此,MCTD似乎具有长期的血清学异质性,其反应性显然与MCTD广泛临床谱中的不同特征不对应。