Department of Connective Tissue Diseases, National Institute of Geriatrics, Rheumatology and Rehabilitation, Warsaw, Poland.
Department of Internal Medicine, Connective Tissue Diseases and Geriatrics, Medical University of Gdansk, Gdansk, Poland.
Clin Rheumatol. 2022 Jan;41(1):281-288. doi: 10.1007/s10067-021-05717-4. Epub 2021 Aug 9.
Mixed connective tissue disease (MCTD) is a rare disease with clinical picture consisted of multiple organ manifestations, including skin changes resembling systemic lupus erythematosus (SLE), systemic sclerosis (SSc), or dermatomyositis (DM). On the background of these manifestations are microvascular changes - alteration of endothelial function and impairment of endothelial progenitor cell. Nailfold capillaroscopy (NFC) is a simple, non-invasive technique for investigating microvascular involvement in rheumatic diseases.
To describe the relationship between type of skin lesions and NFC pattern in MCTD patients.
We analyzed the clinical picture and NFC patterns in 79 patients with MCTD. The NFC changes were classified into Normal, "Early," "Active," and "Late" scleroderma-like patterns (SD-like pattern) based on Cutolo classification. In all patients, subjective and physical examinations were carried out, specifically the occurrence of skin lesions in the course of MCTD was assessed (systemic sclerosis-like (Ssc-like), systemic lupus erythematosus-like (SLE-like), dermatomysitis-like (DM-like)).
Skin changes were present in 64 (81%) patients, involving 43 (54%) SLE-like, 48 (61%) SSc-like, and 4 (5.1%) DM-like. NFC changes were observed in a total of 55 (69.6 %) patients with predominance of the "Early" pattern - 41 (51.9 %) patients. According to skin change phenotypes, NFC changes were observed in 31 (72%) patients with SLE-like and in 32 (66.7%) patients with SSc-like skin phenotypes. The "early" pattern predominated in both group.
We did not find any correlation between NFC pattern and the type skin changes. Key Points • The study did not show a correlation between the presence and absence of skin lesions and NFC pattern. • Scleroderma-like patterns were found in over 60% of patients with mixed connective tissue disease. • The "early" pattern is dominant regardless of the occurrence or absence of skin lesions in patients with MCTD. • Skin lesions, regardless of their type (SLE or SSc), do not correlate with type of lesion found in the NFC examination.
混合性结缔组织病（MCTD）是一种罕见的疾病，其临床表现包括多种器官表现，包括类似于系统性红斑狼疮（SLE）、系统性硬皮病（SSc）或皮肌炎（DM）的皮肤改变。在这些表现的背景下是微血管变化——内皮功能改变和内皮祖细胞损伤。甲襞毛细血管显微镜检查（NFC）是一种简单、无创的技术，用于研究风湿性疾病的微血管受累。
描述 MCTD 患者皮肤损伤类型与 NFC 模式之间的关系。
我们分析了 79 例 MCTD 患者的临床资料和 NFC 模式。根据 Cutolo 分类，NFC 变化分为正常、“早期”、“活动”和“晚期”硬皮病样（SD 样）模式。在所有患者中，均进行了主观和体格检查，具体评估了 MCTD 过程中皮肤损伤的发生情况（类似于系统性硬皮病（Ssc-like）、系统性红斑狼疮样（SLE-like）、皮肌炎样（DM-like））。
皮肤改变见于 64 例（81%）患者，其中 43 例（54%）为 SLE 样，48 例（61%）为 SSc 样，4 例（5.1%）为 DM 样。NFC 变化共观察到 55 例（69.6%）患者，以“早期”模式为主，41 例（51.9%）患者。根据皮肤改变表型，SLE 样皮肤表型中有 31 例（72%）和 SSc 样皮肤表型中有 32 例（66.7%）观察到 NFC 改变。两组均以“早期”模式为主。
我们没有发现 NFC 模式与皮肤改变类型之间存在任何相关性。关键点• 研究未显示存在或不存在皮肤病变与 NFC 模式之间存在相关性。• 混合性结缔组织病患者中超过 60%存在硬皮病样模式。• 无论 MCTD 患者是否存在皮肤病变，“早期”模式均占主导地位。• 无论其类型（SLE 或 SSc）如何，皮肤病变均与 NFC 检查中发现的病变类型无关。
