Kadiru Rana Abdul, Hegde Spandana P, Shenoy Manjunath M
Department of Dermatology, Yenepoya Medical College and University, Mangalore, Karnataka, India.
Indian Dermatol Online J. 2019 Jul-Aug;10(4):413-417. doi: 10.4103/idoj.IDOJ_398_18.
Connective tissue disorders (CTD) occur in 3-5% of the population. The advent of antibodies to extractable nuclear antigens (ENA) has become a reliable predictor to establish the diagnosis of CTD, subclassify patients into prognostic groups, and monitor disease activity.
The aim of this study was to (a) study the frequency of cutaneous manifestations, systemic manifestations, and anti-ENA antibodies in CTD; (b) determine the association between systemic manifestations and ENAs; and (c) determine the association between cutaneous and systemic manifestations of CTD.
An observational cross- sectional study was conducted on 50 patients diagnosed to have CTD. The clinical profile and antibodies to ENA (ANA Profile) reports were retrieved and studied.
The major dermatological manifestations were skin tightness (36%), salt and pepper pigmentation (30%), Raynaud's phenomenon (28%), and malar rash (28%). The common antibodies seen were anti SS-A (36%), anti-UI-ribonucleoprotein (U1-RNP) (34%), anti-dsDNA (32%), and anti-Sm (24%). Patients with anti-Sm and anti-dsDNA antibodies had increased frequency of renal manifestations. A strong association with significant values was seen between neurological manifestations and anti-Sm antibody, and cardiovascular manifestations and anti-RNP antibody. An association between gastrointestinal manifestations and malar rash as well as neurological manifestations and photosensitivity was also seen.
ENA panel predicts systemic involvement, thus helping in the multidisciplinary management. Cutaneous manifestations of CTD can be an early predictor in giving a clue to impending systemic manifestations.
结缔组织病(CTD)在3%至5%的人群中出现。可提取核抗原(ENA)抗体的出现已成为建立CTD诊断、将患者分为预后组以及监测疾病活动的可靠预测指标。
本研究的目的是(a)研究CTD中皮肤表现、全身表现和抗ENA抗体的频率;(b)确定全身表现与ENA之间的关联;以及(c)确定CTD皮肤和全身表现之间的关联。
对50例诊断为CTD的患者进行了一项观察性横断面研究。检索并研究了临床资料和ENA抗体(ANA谱)报告。
主要的皮肤表现为皮肤紧绷(36%)、椒盐样色素沉着(30%)、雷诺现象(28%)和蝶形红斑(28%)。常见的抗体有抗SS - A(36%)、抗U1 -核糖核蛋白(U1 - RNP)(34%)、抗双链DNA(32%)和抗Sm(24%)。抗Sm和抗双链DNA抗体的患者肾脏表现的频率增加。神经表现与抗Sm抗体、心血管表现与抗RNP抗体之间存在显著的强关联。还观察到胃肠道表现与蝶形红斑以及神经表现与光敏性之间的关联。
ENA检测可预测全身受累情况,从而有助于多学科管理。CTD的皮肤表现可能是即将出现全身表现的早期预测指标。