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A 57-year-old woman with a diagnosis of antisynthetase syndrome (ASSD) underwent a nailfold videocapillaroscopy (NVC) showing a scleroderma pattern. Alterations in capillary morphology have been reported in adults with inflammatory myositis (IM) but only recently have the differences in NVC findings between these two diseases been established. ASSD is currently classified as a subset of IM, for which reason only a few studies in literature evaluate its specific hallmarks, showing nonspecific features of NVC in patients with polymyositis and dermatomyositis (DM) and antisynthetase antibodies. To our knowledge, this is the first description of ASSD capillaroscopy features, and the first report of NVC in ASSD with evidence of scleroderma pattern. Further studies are needed to define clearly frequency, typical features, and possible correlation with clinical and serological data of NVC changes in ASSD, differences between microangiopathy in ASSD and systemic sclerosis or DM.

Patients initially diagnosed as having primary Raynaud’s phenomenon (PRP) may shift to secondary (SRP) during the follow-up. Nailfold videocapillaroscopy (NVC) is a tool that allows to distinguish between PRP and SRP through the identification of the “early” scleroderma-pattern of microangiopathy. The aim of this study was to evaluate the transition from PRP to SRP in an Italian cohort of patients during their follow-up. 129 patients with PRP were identified and followed-up for 2721 months. The diagnosis of PRP was achieved as suggested by LeRoy. The NVC diagnosis of scleroderma-pattern was based on the presence of specific “early” capillary abnormalities (i.e. giant capillaries, microhaemorrhages, and/or slight reduction of capillary density). Based on the identification of the “early” scleroderma-pattern by NVC, 14% of patients changed from PRP to SRP during the follow-up. Interestingly, 4.6% of these patients showed at baseline a fully normal NVC pattern (transition from normal to scleroderma NVC pattern in 3427 months), and 10% showed slight and not-specific nailfold capillary abnormalities (i.e. dystrophic capillaries and/or enlarged capillaries) at baseline (transition to scleroderma NVC pattern in 2515 months). Following a careful NVC analysis, we showed the progression from PRP to SRP in 14% of the analyzed patients. We suggest the capillaroscopic analysis twice a year in presence of PRP, in order to early detect the transition to SRP in patients showing at the beginning a normal pattern or not-specific nailfold capillary abnormalities, as assessed by NVC.

Background: Systemic sclerosis (SSc) is an autoimmune disease characterized by fibrosis of the skin and visceral organs. The microangiopathy is early detectable in the course of the disease by nailfold videocapillaroscopy (NVC), a non-invasive technique with a high diagnostic value. Objective: Aim of our study was to evaluate the feasibility of a quantitative score and its correlation with the digital skin ulcers, which frequently complicate SSc microangiopathy. Methods: We retrospectively analysed the NVC of 65 SSc patients, performed by 200x videocapillaroscopy connected to image analyse software (Videocap; DS MediGroup, Milan, Italy). The analysis of NVC images included: total number of capillaries in the distal row (N), maximum diameter (D) and number of giant capillaries (M), M/N ratio and percentage of M, presence/absence of micro-haemorrhages and tortuosity. Results: 21/65 SSc patients experienced digital ulcers within three months after the NVC examination. The N, D, M/N, and percentage of M significantly correlated with the appearance of ischemic ulcers. A multiple regression analysis showed a statistically significant correlation for N, M/N and D, while sensitivity and specificity of these parameters were unsatisfactory. A capillaroscopic score, according to the formula D·M/N2, showed a high specificity and sensibility (93.2% and 85.7% respectively; area under ROC curve: 0.918) to predict the appearance of digital ulcers. Conclusions: This capillaroscopic score may represent a feasible and simple tool in SSc patients’ assessment. The routinely use of this parameter might permit to recognize and to preventively treat SSc patients at high risk to develop digital ulcers.

Osteopoikilosis (OPK) is a rare autosomal dominant bone disorder characterized by numerous hyperostotic areas that tend to localize in periarticular osseous regions. It is usually asymptomatic and is often diagnosed incidentally during X-rays. OPK may be an isolated finding or associated with other pathologies, e.g. skin manifestations, rheumatic and/or skeletal disorders. We report a literature review and, for the first time, the coexistence of OPK with seronegative spondyloarthritis and Raynaud’s phenomenon in a 48-year old female. To the best of our knowledge, this is the first case of OPK studied by videocapillaroscopy, demonstrating the absence of specific microvascular abnormalities of nailfold capillaries.

Microvascular involvements represent one of the first step in many autoimmune diseases such as scleroderma (SSc) or antiphospholipid syndrome. Early in the disease, the peripheral microangiopathy may be well recognised and studied by nailfold videocapillaroscopy (NVC), a noninvasive and safe technique, that is reported to have both diagnostic and prognostic value, especially in the presence of Raynaud’s phenomenon (RP). The classification of defined major NVC patterns in SSc might be useful in assessing the appearance and progression of the sclerodermic microangiopathy. In addition, the NVC changes might represent a morphological reproduction of the evolution of the SSc. The early appearance of giant capillaries and haemorrhages (Early pattern) is of great relevance for the early diagnosis of the SSc. Therefore, these alterations are more evident in the active phase of the disease (Active pattern). Converserly, the NVC observation of loss of capillaries, vascular architectural disorganisation and the presence of ramified/ bushy capillaries (Late pattern) represents the clearest aspect of advanced SSc microvascular damages. Interesting, correlations between the NVC and clinical aspects of SSc, as well as the effects of therapeutical intervenctions have been observed.

Objectives: Systemic sclerosis (SSc) is characterized by altered microvascular structure and function. Nailfold videocapillaroscopy (NVC) is the tool to evaluate capillary morphological structure and laser-Doppler Blood flowmetry (LDF) can be used to estimate cutaneous blood flow of microvessels. The aim of this study was to investigate possible relationships between capillary morphology and blood flow in SSc. Methods: 27 SSc patients and 12 healthy subjects were enrolled. SSc microvascular involvement, as evaluated by NVC, was classified in three different patterns (“Early”, “Active”, “Late”). LDF analysis was performed at the II, III, IV, V hand fingers in both hands and both at cutaneous temperature and at 36°C. Statistical evaluation was carried out by non-parametric procedures. Results: Blood flow was found significantly lower in SSc patients when compared with healthy subjects (p