Perifollicular Hypopigmentation in Systemic Sclerosis: Associations With Clinical Features and Internal Organ Involvement.

Perifollicular Hypopigmentation in Systemic Sclerosis: Associations With Clinical Features and Internal Organ Involvement.
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DOI:
10.3899/jrheum.210983
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发表时间:
2022-05
影响因子:
3.9
通讯作者:
McMahan, Zsuzsanna H.
McMahan, Zsuzsanna H.
中科院分区:
医学2区
文献类型:
--
作者:
Chung, Melody P.;Mecoli, Christopher A.;Perin, Jamie;Richardson, Carrie;McMahan, Zsuzsanna H.

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确定系统性硬化症 (SSc) 中的毛囊周围色素沉着是否与人口统计学、独特的临床特征和自身抗体谱相关。 SSc 患者前瞻性地使用标准化数据表入组,用于收集毛囊周围色素减退的解剖分布。评估了色素沉着不足和 SSc 特征之间的关联。在 179 名成年 SSc 患者中,36 名 (20%) SSc 患者出现毛囊周围色素沉着不足;这 36 名患者中,94% (n=34) 为女性,33% (n=12) 患有有限的皮肤 SSc。在单变量逻辑回归中,黑人种族(OR 15.63,95% CI 6.6–37.20,p<0.001),弥漫性 SSc(OR 4.62,95% CI 2.11–10.09,p<0.001),较高的最大 MRSS(OR 1.05,95% CI 1.02–1.08,p=0.003),肌病(OR 3.92,95% CI 1.80–8.57,p<0.001)、肺纤维化(OR 2.69,95% CI 1.20–6.02,p=0.016)、预测最低 FVC % 较低(OR 0.96,95% CI 0.94–0.99,p=0.001)和最低 DLCO 较低预测百分比(OR 0.97,95% CI 0.95-0.99,p=0.009)与色素沉着不足相关。抗着丝粒抗体与色素沉着不足呈负相关(OR 0.24,95% CI 0.07–0.86,p=0.028)。调整年龄、种族和病程后,弥漫性 SSc(OR 4.28,95% CI 1.46-12.53,p=0.008)与色素沉着不足显着相关。在部分 SSc 患者中观察到毛囊周围色素沉着不足,并且与弥漫性亚型相关。需要进行更大规模的前瞻性研究来确定毛囊周围色素沉着是否先于终末器官受累以及是否与内脏器官受累相关的特定模式。
To determine whether perifollicular hypopigmentation in systemic sclerosis (SSc) is associated with demographics, distinct clinical features, and autoantibody profiles. SSc patients were prospectively enrolled with a standardized data form used to collect anatomic distribution of perifollicular hypopigmentation. Associations between hypopigmentation and features of SSc were assessed. Of 179 adult SSc patients, 36 (20%) SSc patients had perifollicular hypopigmentation; 94% (n=34) of these 36 were female and 33% (n=12) had limited cutaneous SSc. In univariable logistic regression, black race (OR 15.63, 95% CI 6.6–37.20, p<0.001), diffuse SSc (OR 4.62, 95% CI 2.11–10.09, p<0.001), higher maximum MRSS (OR 1.05, 95% CI 1.02–1.08, p=0.003), myopathy (OR 3.92, 95% CI 1.80–8.57, p<0.001), pulmonary fibrosis (OR 2.69, 95% CI 1.20–6.02, p=0.016), lower minimum FVC % predicted (OR 0.96, 95% CI 0.94–0.99, p=0.001), and lower minimum DLCO % predicted (OR 0.97, 95% CI 0.95–0.99, p=0.009) were associated with hypopigmentation. Anti-centromere antibodies inversely associated with hypopigmentation (OR 0.24, 95% CI 0.07–0.86, p=0.028). After adjusting for age, race, and disease duration, diffuse SSc (OR 4.28, 95% CI 1.46–12.53, p=0.008) was significantly associated with hypopigmentation. Perifollicular hypopigmentation is observed in a subset of SSc patients and associates with diffuse subtype. Larger prospective studies determining whether perifollicular hypopigmentation precedes end-organ involvement and whether specific patterns associate with internal organ involvement are needed.
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