Arkachaisri Thaschawee, Fertig Noreen, Pino Sally, Medsger Thomas A
Division of Rheumatology, Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania 15213, USA.
J Rheumatol. 2008 Dec;35(12):2439-44. doi: 10.3899/jrheum.080098. Epub 2008 Nov 1.
To determine the frequency of selected serum autoantibodies and their clinical associations in patients with childhood-onset (ChO) or adult-onset (AO) linear scleroderma (LiScl) evaluated at a single institution.
Seventy-two patients (ChO = 40, AO = 32), including 12 with en coup de sabre, were studied. All ChO patients had disease onset before age 16 years. Clinical features (extent of cutaneous disease, activity, and joint contractures) were recorded. Antinuclear antibodies (ANA) were identified by indirect immunofluorescence (HEp-2 cells), and anti-single-stranded DNA (anti-ssDNA), antihistone (AHA), and antichromatin (AChA) autoantibodies were detected by ELISA.
There were no significant differences between groups in regard to gender, proportion with LiScl/E, or clinical features except joint contractures (ChO > AO; p = 0.04). There were no differences in the frequency of ANA or other autoantibodies between the groups except for AHA (ChO > AO). AHA was more frequently found with anti-ssDNA (p < 0.0001). LiScl patients with positive anti-ssDNA and/or AHA had more extensive cutaneous involvement and more often had joint contractures (p < 0.05). Anti-ssDNA was present more frequently in AO than in ChO patients with active lesions (p = 0.04). ANA and AChA were not associated with any clinical features. Both AHA and anti-ssDNA levels showed good correlation with disease severity.
Over two-thirds of LiScl patients had ANA. Patients with ChO were similar to those with AO with regard to the frequency of selected serum autoantibodies. Anti-ssDNA and AHA were frequently found together and both were associated with more extensive skin disease with joint contractures. LiScl disease severity correlated with the serum levels of both these antibodies.
确定在单一机构评估的儿童期起病（ChO）或成人期起病（AO）的线状硬皮病（LiScl）患者中选定血清自身抗体的频率及其临床关联。
研究了72例患者（ChO = 40例，AO = 32例），其中包括12例剑伤样硬皮病患者。所有ChO患者在16岁之前发病。记录临床特征（皮肤疾病范围、活动度和关节挛缩）。通过间接免疫荧光法（HEp-2细胞）鉴定抗核抗体（ANA），并通过酶联免疫吸附测定法检测抗单链DNA（抗ssDNA）、抗组蛋白（AHA）和抗染色质（AChA）自身抗体。
除关节挛缩外（ChO > AO；p = 0.04），两组在性别、LiScl/E比例或临床特征方面无显著差异。除AHA外（ChO > AO），两组之间ANA或其他自身抗体的频率无差异。AHA与抗ssDNA同时出现的频率更高（p < 0.0001）。抗ssDNA和/或AHA阳性的LiScl患者皮肤受累范围更广，关节挛缩更常见（p < 0.05）。在有活动性病变的患者中，AO患者抗ssDNA的出现频率高于ChO患者（p = 0.04）。ANA和AChA与任何临床特征均无关联。AHA和抗ssDNA水平均与疾病严重程度呈良好相关性。
超过三分之二的LiScl患者有ANA。ChO患者与AO患者在选定血清自身抗体的频率方面相似。抗ssDNA和AHA经常同时出现，且两者均与更广泛的皮肤疾病伴有关节挛缩相关。LiScl疾病严重程度与这两种抗体的血清水平相关。
