Nailfold Videocapillaroscopic Pattern Research Articles

Peripheral microangiopathy\xa0in precapillary pulmonary hypertension: a nailfold video capillaroscopy prospective study

BackgroundAlthough pulmonary vascular bed has been the main subject of research for many years in pulmonary hypertension (PH), interest has recently started to divert towards the possibility of a co-existing peripheral microangiopathy. The aim of the current study was to investigate the presence of nailfold video-capillaroscopic (NVC) structural changes in patients with precapillary PH and to identify possible associations of NVC measurements with markers of disease severity.MethodsΑ prospective case–control study was performed in 28 consecutive patients with precapillary PH [14 with idiopathic pulmonary arterial hypertension (IPAH) and 14 with chronic thromboembolic pulmonary hypertension (CTEPH)] and 30 healthy controls. NVC quantitative and qualitative parameters were evaluated using Optilia Digital Capillaroscope. To ensure inter-observer repeatability capillaroscopic images were reviewed by two independent investigators. For multiple comparisons among continuous variables, one-way ANOVA or the Kruskal–Wallis test were used. Differences between the groups were tested with post-hoc analysis with adjustment for multiple comparisons (Bonferroni test).ResultsBoth IPAH (71.4% were women, mean age 53.1 ± 13.4 years) and CTEPH (64.3% women, mean age 60.9 ± 14.4 years) groups presented reduced capillary density compared to healthy controls (8.4 ± 1.2 loops/mm and 8.0 ± 1.2 loops/mm vs. 9.7 ± 0.81 loops/mm, p < 0.001) and increased loop width (15.7 ± 3.9 μm and 15.8 ± 1.9 μm vs. 11.5 ± 2.3 μm, p < 0.001). More than half of patients with IPAH presented microhaemorrhages on capillary nailfold, while increased shape abnormalities in capillary morphology and more capillary thrombi per linear mm were detected in patients with CTEPH compared to patients with IPAH and healthy controls. All PH patients presented a non-specific NVC pattern compared to controls (p < 0.001).ConclusionThe findings of the study reveal a degree of significant peripheral microvascular alterations in patients with IPAH and CTEPH, suggesting a generalized impairment of peripheral microvasculature in pulmonary vascular disease.

FRI0392 Relationships between bone loss and dickkopf-1 (DKK-1) serum levels in patients with systemic sclerosis.

BackgroundSystemic sclerosis (SSc) patients might present an increased risk of osteoporosis as a result of the chronic inflammatory state, immobilization, use of glucocorticoids and other causes. The Wnt/β-catenin pathway is...

Very low levels of vitamin D in systemic sclerosis patients

Vitamin D displays many extraosseous immunomodulatory effects. The aim of the study was to evaluate the level of vitamin D in patients with systemic sclerosis (SSc) and to analyze the associations between the concentration of the vitamin and clinical manifestations. In March-April 2009, 65 consecutive SSc patients underwent evaluation of vitamin D concentrations by the LIAISON immunoassay (normal 30-100 ng/ml). Serum levels between 10 and 30 ng/ml were classified as vitamin D insufficiency, while concentrations <10 ng/ml as vitamin D deficiency. None of the patients were receiving vitamin D supplementation at the time of or during the year prior to study entry. The mean level of vitamin D was 15.8 ± 9.1 ng/ml. Only three cases showed normal values; vitamin D insufficiency and deficiency were found in 43 and 19 cases, respectively. Patients with vitamin D deficiency showed longer disease duration (13.1 ± 6.8 versus 9.4 ± 5.5 years, P = 0.026), lower diffusing lung capacity for carbon monoxide (63.7 ± 12.4 versus 76.4 ± 20.2, P = 0.014), higher estimated pulmonary artery pressure (28.9 ± 9.9 versus 22.8 ± 10.4, P = 0.037) and higher values of ESR (40 ± 25 versus 23 ± 13 mm/h, P = 0.001) and of CRP (7 ± 7 and 4 ± 2 mg/l, P = 0.004) in comparison with patients with vitamin D insufficiency; moreover, late nailfold videocapillaroscopic pattern was more frequently found (52.6% versus 18.6%, P = 0.013). None of the patients showed evidence of overt mal-absorption. Low levels of vitamin D are very frequent in patients with SSc. Intestinal involvement is not likely the cause of vitamin D deficit; other factors such as skin hyperpigmentation and reduced sun exposition for psychological and social reasons may be implicated. Patients with vitamin D deficiency showed more severe disease in comparison with patients with vitamin D insufficiency, above all concerning lung involvement. Further trials are awaited to determine whether vitamin D could represent a modifiable factor able to interfere with SSc evolution.

Cyclophosphamide treatment improves microvessel damage in systemic sclerosis

The objective of this study is to evaluate nailfold videocapillaroscopy changes in scleroderma patients treated regularly on cyclic basis with iloprost and to find associations with clinical, serologic, and pharmacological variables. Forty-nine patients affected by systemic sclerosis (44 women and five men, mean age 52.4 years, mean disease duration 8.0 years, 31 patients with limited cutaneous subset and 18 with diffuse cutaneous form of the disease) underwent two nailfold videocapillaroscopies at a distance of 3 years from each other; the examinations were performed by an operator blinded to clinical features and to drug treatment. Six patients showed an amelioration of nailfold videocapillaroscopic abnormalities who changed from active to early pattern; five of these cases (83.3%) had been given cyclophosphamide therapy and the remaining case methotrexate plus azathioprine. Cyclophosphamide administration was significantly associated with amelioration of nailfold videocapillaroscopic pattern (p<0.001). None of the patients who received cyclophosphamide demonstrated worsening of the microvascular lesions; the progression of nailfold videocapillaroscopic pattern was inversely correlated to cyclophosphamide treatment (p=0.02). In our study, cyclophosphamide treatment demonstrated to be effective for scleroderma microvascular damage as directly observed by nailfold videocapillaroscopy.

Correlation between homocysteine plasma levels and nailfold videocapillaroscopic patterns in systemic sclerosis

The aim of the study is to investigate the relationship between microangiopathy as assessed by nailfold videocapillaroscopy (NVC) and plasma level of homocysteine (Hcy) in systemic sclerosis (SSc). As known, Hcy is a nonessential amino acid that interferes with normal properties of a vascular tree. Sixty patients affected by SSc (4 men and 56 women, mean age 54.6) underwent the determination of plasma Hcy level; at the same time, NVC was performed. Hcy level was also determined in 30 sex- and age-matched controls. In patients affected by SSc the plasma Hcy level was significantly higher than in healthy controls (11.8 and 6.5 micromol/l, respectively; p < 0.001). A significant correlation was found between plasma Hcy concentration and the pattern of NVC with a progressive increase from early to active and above all to late pattern (10.7, 11.8, and 17.4 micromol/l, respectively; p < 0.001). Subjects with high Hcy level (i.e., >75th percentile of Hcy level in controls and in patients considered altogether) were mostly represented in the scleroderma patients with late nailfold videocapillaroscopic pattern; the crude odds ratio was 9.0 (significant; 95% CI from 2.1 to 38.8). In conclusion, Hcy plasma level is related to microvascular involvement in patients affected by SSc; the concentration increases with the progression of the nailfold videocapillaroscopic pattern. Hyperhomocysteinemia may represent an aggravating factor among the complex mechanisms involved in scleroderma damage contributing to the injury of endothelium.

Plasma D-dimer concentration in patients with systemic sclerosis

BackgroundSystemic sclerosis (SSc) is an autoimmune disorder of the connective tissue characterized by widespread vascular lesions and fibrosis. Little is known so far on the activation of the hemostatic and fibrinolytic systems in SSc, and most preliminary evidences are discordant.MethodsTo verify whether SSc patients might display a prothrombotic condition, plasma D-dimer was assessed in 28 consecutive SSc patients and in 33 control subjects, matched for age, sex and environmental habit.Results and discussionWhen compared to healthy controls, geometric mean and 95% confidence interval (IC95%) of plasma D-dimer were significantly increased in SSc patients (362 ng/mL, IC 95%: 361–363 ng/mL vs 229 ng/mL, IC95%: 228–231 ng/mL, p = 0.005). After stratifying SSc patients according to disease subset, no significant differences were observed between those with limited cutaneous pattern and controls, whereas patients with diffuse cutaneous pattern displayed substantially increased values. No correlation was found between plasma D-dimer concentration and age, sex, autoantibody pattern, serum creatinine, erythrosedimentation rate, nailfold videocapillaroscopic pattern and pulmonary involvement.ConclusionWe demonstrated that SSc patients with diffuse subset are characterized by increased plasma D-dimer values, reflecting a potential activation of both the hemostatic and fibrinolytic cascades, which might finally predispose these patients to thrombotic complications.