Naifold capillaroscopy in mixed connective tissue disease patients.

INTRODUCTION: Mixed connective tissue disease (MCTD) is a rare systemic disease characterized by overlapping features of systemic lupus erythematosus (SLE), systemic sclerosis (SSc), dermato-/polymyositis (DM/PM), and rheumatoid arthritis (RA). Naifold capillaroscopy (NFC) is a non-invasive test for evaluating the capillaries of the nail shaft used in the diagnosis of rheumatic diseases. OBJECTIVES: To determine whether there are characteristic abnormalities in NFC in MCTD patients, and whether the type of NFC lesions correlates with organ involvement in these patients. METHODS: Clinical picture and NFC patterns were analyzed in 43 patients with MCTD. Capillaroscopic images were divided into scleroderma-like pattern (SD-like pattern) according to the Cutolo classification, non-specific lesions, and normal images. Relationships between the clinical aspects considered in the MCTD classification criteria and the changes in the capillaroscopic images were evaluated. RESULTS: SD-like pattern was present in 20 MCTD patients (46.51%) with a predominance of the "early" pattern. Giant, branched, dilated capillaries and reduced capillary density were found more frequently in MCTD patients compared to the control group (p-values 0.0005, 0.005, 0.02, < 0.0001 respectively). There were associations found between the presence of a reduced number of vessels, avascular areas, and SD-like pattern with the presence of sclerodactyly in MCTD patients (p = 0.002, p = 0.006, p = 0.02, respectively), alongside an association between the presence of branched vessels and the subpapillary plexus with pulmonary arterial hypertension (PAH) (p = 0.04 and p = 0.005, respectively). CONCLUSIONS: MCTD patients are significantly more likely to have abnormalities upon NFC. It is worthwhile to perform capillaroscopic examination in MCTD patients. Key Points • Scleroderma-like pattern was found in more than half of the MCTD patients. • Reduced capillary density was found to be a significant predictor of the diagnosis of MCTD. • There were relationships between the presence of reduced capillary density, avascular areas, and SD-like with the presence of sclerodactyly in the MCTD patients. • There was an association between the presence of branched vessels and the visibility of the subpapillary plexus and pulmonary arterial hypertension (PAH).

Update on Current Imaging of Systemic Lupus Erythematous in Adults and Juveniles.

Systemic lupus erythematosus (SLE) is an autoimmune disease involving multiple organs and organ systems. Musculoskeletal (MSK) involvement is one of the most frequent and the earliest locations of disease. This disease affects joints and periarticular soft tissues, tendon sheaths and tendons, bones, and muscles. Multimodality imaging, including radiography, ultrasound (US), and magnetic resonance imaging (MRI), plays a significant role in the initial evaluation and treatment follow up of MSK manifestations of the SLE. In this paper, we illustrate MSK imaging features in three clinical forms of SLE, including nondeforming nonerosive arthritis, deforming nonerosive arthropathy, and erosive arthropathy, as well as the other complications and features of SLE within the MSK system in adults and juveniles. Advances in imaging are included. Conventional radiography primarily shows late skeletal lesions, whereas the US and MRI are valuable in the diagnosis of the early inflammatory changes of the soft tissues and bone marrow, as well as late skeletal manifestations. In nondeforming nonerosive arthritis, US and MRI show effusions, synovial and/or tenosynovial hypertrophy, and vascularity, whereas radiographs are normal. Deforming arthritis clinically resembles that observed in rheumatoid arthritis, but it is reversible, and US and MRI show features of inflammation of periarticular soft tissues (capsule, ligaments, and tendons) without the pannus and destruction classically observed in RA. Erosions are rarely seen, and this form of disease is called rhupus syndrome.

Relationship between type of skin lesions and nailfold capillaroscopy pattern in mixed connective tissue disease.

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.

Microvascular damage - a marker of specific organ involvement in mixed connective tissue disease?

Mixed connective tissue disease (MCTD) is a complex entity, which incorporates features of systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), systemic sclerosis (SSc) and polymyositis/dermatomyositis (PM/DM). Nailfold videocapillaroscopy (NVC) is a simple, safe and non-invasive technique of capillary vessel assessment, allowing for qualitative and quantitative assessment of microcirculation. NVC plays a pivotal role in the diagnostic algorithm of connective tissue diseases, especially in systemic sclerosis (SSc). Numerous studies have shown a correlation between organ involvement and disease progression in SSc. In the current literature, there are limited data on relationship between NVC and organ involvement in MCTD patients. In the present article the relevant literature describing NVC examination in patients with MCTD and comparisons with some clinical situations are discussed.