Systemic sclerosis (SSc) is a collagenosis with a substrate of chronic inflammation, which is determined by autoimmunity. The pathogenesis of this disease involves microvasculopathy (small vessel pathology) followed by excessive cutaneous and visceral fibrosis. Although acoustic and vestibular impairment is not classified as being a secondary pathology of SSc, several studies have identified cases of SSc that associate hearing loss and especially vertigo and tinnitus. This paper presents data from the medical literature that have identified vestibular and auditory symptoms among patients with SSc, associating the clinical case presentation of a patient suffering from SSc, which is associated with hearing loss. The need for additional studies on larger groups of patients is underlined, in order to clarify the impact of vasculopathy and fibrosis on the acoustic and vestibular analyzer in patients with SSc.

The role of nailfold capillaroscopy (NC) in common non-rheumatic conditions has not been systematically reported. The aim of this review is to outline NC features observed in frequent non-rheumatic conditions, providing a practical tool to support rheumatologists for the interpretation of capillaroscopic abnormalities in patients with no established connective tissue disease (CTD).
We undertook a systematic search in PubMed and Web of Science databases. Studies reporting adults or children with common non-rheumatic diseases or conditions in which quantitative and/or qualitative assessment of morphological nailbed capillary findings was obtained, were included. The presence of a control group composed by subjects not affected by the studied condition and direct comparison of findings between groups were needed.
We included 25 articles. Diabetes mellitus (11 studies), glaucoma (7 studies) and essential hypertension (3 studies) were the most represented diseases. Reduced capillary density, tortuosity, dilated capillaries, microhaemorrhages, ramified capillaries and avascular areas can be observed in diabetic patients. Association was reported between poor glycaemic control or longer duration of diabetes, or presence of microvascular complications as retinopathy and neuropathy, and more severe capillaroscopic abnormalities. Decreased capillary density, tortuosity, microhaemorrhages, dilated capillaries, avascular areas and ramifications might also be present in glaucoma, while in essential hypertension a reduced capillary density might be expected.
Abnormal capillaroscopic findings are not uncommon even in individuals with no CTD. Therefore, presence of comorbidities known to potentially affect the microvascular array should always be investigated in patients undergoing NC and the interpretation of findings might be weighted accordingly.

Nailfold capillaroscopy is an easy, non-invasive technique to assess microvascular involvement in rheumatic diseases. Multiple studies describe capillaroscopic changes in systemic lupus erythematosus (SLE), including a wide range of non-specific findings. On behalf of the European League Against Rheumatism (EULAR) study group on microcirculation in rheumatic diseases, a systematic review was done to obtain all original research studies (in English) in which SLE patients had capillaroscopy. Forty such studies are identified. This article firstly provides a résumé of the results of these studies according to capillaroscopic parameters (density, dimensions, morphology, haemorrhages), semi-quantitative assessment and qualitative assessment of capillaroscopy in SLE patients. Secondly, the correlations between capillaroscopic parameters in SLE patients and clinical and laboratory parameters (including auto-immune parameters) are outlined. The following capillaroscopic parameters are found to be significantly more prevalent in SLE patients compared to healthy controls: tortuous capillaries, abnormal morphology and haemorrhages. Hairpin-shaped capillaries are significantly less prevalent than in healthy persons. The semi-quantitatively determined nailfold capillaroscopic score (NFC score) in SLE patients is also higher than in healthy controls. Several correlations between clinical and laboratory parameters and capillaroscopic parameters are identified in the review. Disease activity is correlated with NFC score in seven studies, with abnormal morphology (i.e. \"meandering\") in one study and with haemorrhages in one study. Frequent attacks of Raynaud\'s phenomenon (RP) and gangrene are significantly correlated with dilated capillaries. In two studies a possible correlation between anti-SSA antibodies and lower density of capillaries is withheld. About other immune parameters conflicting results are found. In one study a significant negative correlation is found between 24-hour proteinuria and abnormal morphology (i.e. \"meandering\"). For the first time, an overview of the nailfold capillaroscopic changes that have been described in SLE and their correlations with clinical and laboratory findings is given. Further large-scale research on the identification of capillaroscopic changes in SLE and their correlations with standardised clinical and laboratory parameters, is ongoing at the EULAR study group on microcirculation in rheumatic diseases.

BACKGROUND: Bier spots are asymptomatic white anemic macules with irregular shape located on the upper limbs and less often on the lower limbs and trunkus. They are observed predominantly in youg males and may be idiopathic or associated with other conditions.
OBJECTIVE: We report two cases with Bier spots in which videodermatoscopy and nailfold capillaroscopy were performed.
RESULTS: Patient 1 was a 34-year-old otherwise healthy male who presented with white spots on his upper limbs for 10 years. Dermoscopy revealed marked narrowing of skin capillaries within the borders of white macules whereas capillaroscopy pattern was normal. Patient 2 was a 20-year-old female with symptoms of Raynaud\'s phenomenon who presented with white spots on the volar aspects of her hands for 2 years. The capillaroscopy showed abnormal findings with presence of homogenous enlarged capillaries.
CONCLUSIONS: Bier spots do not require any treatment. The associated conditions found should be monitored.

Diabetic sclerodactyly is a frequently recognized skin finding that may occur in patients with diabetes mellitus but coexistence of diabetes and systemic sclerosis is rare. We describe a case of coexistence of type 1 diabetes mellitus and systemic sclerosis in 42-year-old man with the history of Raynaud\'s phenomenon, progressive diffuse hardening of the skin and sclerodactyly, slowly worsening with time. The medical history included type 1 diabetes since childhood with microvascular complications. The patient presented a typical capillaroscopic scleroderma-like pattern, antinuclear antibodies and sclerotic lesions in gastrointestinal system. Summing up, our case represents the rare coexistence of autoimmune diseases like diabetes mellitus type 1 and systemic sclerosis.

Raynaud\'s phenomenon and digital ulcers (DUs) are frequent among systemic sclerosis (SSc) patients. Our aim was to investigate the diagnostic and predictive value for DU of endothelial dysfunction biomarkers (flow-mediated dilatation (FMD), serum levels of endothelin-1 (ET-1), and ADMA), angiogenic/angiostatic biomarkers (vascular endothelial growth factor (VEGF), endoglin, and endostatin), and nailfold videocapillaroscopy (NVC). We compared our results with a literature review. In a cohort study of 77 SSc patients, we followed two groups of patients: (i) naïve DU patients (39) and (ii) active DU at baseline (38 patients) for 3 years. Telangiectasia (p < 0.001) and diffuse disease subset (p = 0.001) were significantly more frequent in patients with active DU at enrolment. Additionally, NVC late scleroderma pattern (AUC 0.846, 95%CI 0.760-0.932), lower values of FMD (AUC 0.754, 95%CI 0.643-0.864), increased serum levels of ET-1 (AUC 0.758, 95%CI 0.649-0.866), ADMA (AUC 0.634, 95%CI 0.511-0.757), and endoglin as well as low VEGF serum levels (AUC 0.705, 95%CI 0.579-0.830) were significantly associated to new DU events in the 3-year follow-up. Cox regression analysis showed that FMD > 9.41 % (HR 0.37, 95%CI 0.14-0.99); ET-1 >11.85 pmol/L (HR 3.81, 95%CI 1.41-10.26) and late NVC pattern (HR 2.29, 95%CI 0.97-5.38) were independent predictors of DU recurrence. When estimating the probability of occurrence of first DU in naïve DU patients, only late NVC pattern (HR 12.66, 95%CI 2.06-77.89) was an independent predictor factor. In conclusion, late scleroderma patterns in NVC are the best independent predictors of SSc patients who are at risk of developing DU. Endothelial dysfunction assessed by FMD and ET-1 was also found to be an independent predictor of DU recurrence in a 3-year follow-up.

Vasculopathy has a major role in the pathogenesis and tissue injury in systemic sclerosis (SSc). Raynaud\'s phenomenon (RP) is frequently the first clinical manifestation of SSc preceding by years other clinical manifestations. RP in SSc patients is frequent, often very severe and long lasting. The repeated bouts of RP lead to prolonged digital ischemia that may progress to digital ulceration or in extreme to critical digital ischemia with gangrene. Digital ulcers (DU) are a true burden for all patients. They are very painful, with a long and slow healing course, have high risk of infection and are extremely disabling. In adults, up to 40-50% of patients will experience at least one DU in the course of the disease and of these 31-71% will have recurrent ulcers. In order to try to identify predictive risk factors for DU in SSc patients, an extensive literature review was conducted, according to the guidelines proposed at the PRISMA statement. MEDLINE database (PubMed) and Thomson Reuters Web of Knowledge platform were searched for articles published in peer-reviewed journals since 1990 with the last search run on June 2014 and published in English language. The keyword search terms included: digital ulcer/s, systemic sclerosis, scleroderma, digital scars, ischemic complications, autoantibodies, biomarkers, endothelium dysfunction, endothelin-1, vascular endothelial growth factor (VEGF), endostatin, ADMA, endoglin, angiostatin, and capillaroscopy. The following criteria were included: (1) cohorts of SSc patients including patients with DU, (2) endothelium dysfunction and angiogenesis biomarkers compared with a healthy control group, (3) autoantibodies, capillary morphology and distribution, endothelium dysfunction and angiogenesis biomarkers compared between patients with and without digital ulcers, (4) detailed description of the statistical methods used to conclude for predictive factors, and (5) English language. Our search provided a total of 376 citations. Of these, 297 studies were discarded for not meeting the criteria proposed.