4.1. Scleroderma and scleroderma-like
The presence of megacapillaries (giant capillaries) and a decreased capillary density are the hallmarks of the scleroderma capillary pattern, and have been detected by nailfold capillaroscopy. In a large recent study, 166 patients with RP, 65 cases with UCTD, 47 patients with SLE, 26 patients with dermato/polymyositis, 14 with rheumatoid arthritis, seven cases with primary Sjogren's syndrome and 102 patients with systemic sclerosis were investigated (Nagy and Czirjak, 2004).
Of the 16 patients with diffuse cutaneous SSc and the 86 limited cutaneous SSc cases, 87.5% and 61.6% showed the scleroderma capillary pattern, respectively. Nine of the 65 (13.8%) cases with UCTD and 24 of the 186 (12.9%) cases with RP also exhibited the same pattern. Four of the 47 (8.5%) with SLE and seven of the 26 (26.9%) with DM, and no patients with rheumatoid arthritis or Sjogren's syndrome, exhibited the scleroderma capillary pattern. The conclusion is that the scleroderma capillary pattern is often present in systemic sclerosis and DM. Furthermore, patients with RP and UCTD may also occasionally exhibit this pattern. Therefore, capillaroscopy seems to be an useful tool for the early selection of those patients who are potential candidates for developing scleroderma spectrum disorders.
In particular, a well-defined pattern has been reported in patients affected by DM (Klyscz et al., 1996). This pattern, often associated with classical aspects of the scleroderma pattern, includes the presence of two or more of the following findings in at least two nailfolds: enlargement of capillary loops, loss of capillaries, disorganization of the normal distribution of capillaries, 'budding'
('bushy') capillaries, twisted enlarged capillaries and capillary haemorrhages (microbleeding). Typical SLE pattern includes morphological alterations of capillary loops, venular visibility and studying of blood with variability of capillary loop length (Candela et al., 1998).
A recent interesting study on scleroderma patients, investigated the relationship between some specific serum autoantibodies and the expression of the nailfolf video capillaroscopy (NVC) patterns in relation to the different subsets of skin involvement (limited and diffuse scleroderma) of the disease (Cutolo et al., 2004). The Scl70 positivity was found lower in patients showing the 'early' than in those with the 'active' and the 'late' NVC patterns, whereas no significant correlation was found between the Scl70 presence and both RP and SSc duration. The anticentromeric (ACA) positivity was found higher in patients showing the 'early' NVC pattern as well as in patients with longer disease duration. Therefore, the presence of the Scl70 antibodies seems related to a more rapid progression of the sclerodermic microangiopathy. On the contrary, the presence of ACA seems to be related to a slower progression of the SS micro-vascular damage. The sclerodermic peripheral mi-croangiopathy is similar as in patients with limited systemic sclerosis, as in those affected by diffuse systemic sclerosis.
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