Abstract
Introduction
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.
Objectives
To describe the relationship between type of skin lesions and NFC pattern in MCTD patients.
Methods
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)).
Results
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.
Conclusions
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.
Digital dry gangrene as a primary manifestation of systemic lupus erythematosus
