Presence Of Giant Capillaries Research Articles

Use of nailfold video capillaroscopy in polycythemia vera.

In this study, we aimed to investigate capillary vessel diameters and structural changes of capillaries by using nailfold video capillaroscopy (NVC) in patients with polycythemia vera (PV). This cross-sectional study included a total of 24 patients (19 males, 5 females; mean age: 59.8±12.9 years; range, 50.2 to 68 years) who were diagnosed with PV and 15 healthy controls (11 males, 4 females; mean age: 40.7±5.1 years; range, 36 to 44 years) between June 2016 and February 2017. Nailfold video capillaroscopy was performed by an experienced rheumatologist who was blinded to clinical data. The apical, arterial, and venous limb diameters of capillaries were measured and microvascular changes of capillaries were scored. When capillaries were evaluated in terms of morphological structures, giant capillary was detected in 67% of the patients with PV and 0% in the control group (p<0.05). The arterial, venous, and apical diameters of the capillaries were significantly higher in the patients with PV compared to the control group (p<0.001). The presence of giant capillaries and the marked increase of arterial, venous, and apical diameters of capillaries seem to be related to PV. As it additionally plays an important role in diagnosis, prognosis, and treatment monitoring of certain diseases, capillaroscopy can be considered to be a promising microcirculation biomarker.

Associations between nailfold capillaroscopy findings and interstitial lung disease in patients with mixed connective tissue disease.

IntroductionMixed connective tissue disease (MCTD) is a chronic immune-mediated disorder defined by the combined presence of serum anti-RNP antibodies and distinct clinical features including progressive lung fibrosis. The aim of this study was to evaluate potential associations between lung fibrosis in MCTD and specific clinical and laboratory findings including results of nailfold capillaroscopy (NFC) examination.Material and methodsPatients with MCTD who were admitted to the Departments of Allergy and Immunology or Dermatology at the University Hospital of Krakow (Poland) in 2015–2018 were identified based on comprehensive individual record review. Diagnosis of MCTD required fulfilment of at least one of the four widely accepted sets of diagnostic criteria. Clinical and laboratory data were collected, and statistical analysis was performed to identify potential predictors of interstitial lung disease (ILD).ResultsThirty patients (90% females) aged 22–81 years met the study inclusion criteria. The mean duration of symptoms associated with MCTD was 7.3 months. Photosensitivity and Raynaud’s phenomenon were the most common clinical manifestations (90% and 70%, respectively). Hand oedema, sclerodactyly and the presence of giant capillaries in NFC correlated significantly with the risk of lung involvement. In multivariate analysis, the presence of enlarged loops in NFC (giant capillaries) was identified as an independent factor for ILD (R 2 = 0.82, p < 0.0000001).ConclusionsThe NFC examination should be considered in all patients with a diagnosis of MCTD. The presence of giant capillaries may be a promising marker for interstitial lung disease in these patients, especially among those with a short duration of disease (i.e. < 1 year).

Nailfold capillaroscopy-how many fingers should be examined to detect abnormality?

Nailfold capillaroscopy is being increasingly used by rheumatologists in the diagnosis of SSc. However, assessment of all nailfolds can be time-consuming in a busy outpatient clinic. Our aim was to answer the question as to how many (and which) fingers a clinician should routinely assess to capture accurately the true state. A total of 2994 assessments (by an international panel of expert observers) of 1600 images from 173 participants (101 with SSc, 22 with primary RP and 50 healthy controls) were included in this analysis. Seven single-finger or finger combinations (derived from the middle and ring fingers) were then tested for sensitivity for the presence of two markers of capillary abnormality [presence of giant capillaries and an SSc grade (early, active or late)] compared with assessment of all eight fingers. For the eight-finger gold standard, sensitivity against the diagnostic criteria was 74.6% (53.0% for the presence of giants alone and 73.1% for image grade alone). Examining only one finger gave low sensitivity (ranging from right middle 31.7% to left ring 46.6%). Examining both ring fingers gave a sensitivity of 59.8%, whereas examining the four-finger combination of both ring and both middle fingers gave a sensitivity of 66.7%. During routine capillaroscopic examination, ideally all eight nailbeds (excluding thumbs) should be examined, otherwise some abnormalities will be missed. Examining only four fingers reduces capillaroscopy sensitivity.

Intra-and inter-observer reliability of nailfold videocapillaroscopy — A possible outcome measure for systemic sclerosis-related microangiopathy

ObjectivesOur aim was to assess the reliability of nailfold capillary assessment in terms of image evaluability, image severity grade (‘normal’, ‘early’, ‘active’, ‘late’), capillary density, capillary (apex) width, and presence of giant capillaries, and also to gain further insight into differences in these parameters between patients with systemic sclerosis (SSc), patients with primary Raynaud's phenomenon (PRP) and healthy control subjects. MethodsVideocapillaroscopy images (magnification 300×) were acquired from all 10 digits from 173 participants: 101 patients with SSc, 22 with PRP and 50 healthy controls. Ten capillaroscopy experts from 7 European centres evaluated the images. Custom image mark-up software allowed extraction of the following outcome measures: overall grade (‘normal’, ‘early’, ‘active’, ‘late’, ‘non-specific’, or ‘ungradeable’), capillary density (vessels/mm), mean vessel apical width, and presence of giant capillaries. ResultsObservers analysed a median of 129 images each. Evaluability (i.e. the availability of measures) varied across outcome measures (e.g. 73.0% for density and 46.2% for overall grade in patients with SSc). Intra-observer reliability for evaluability was consistently higher than inter- (e.g. for density, intra-class correlation coefficient [ICC] was 0.71 within and 0.14 between observers). Conditional on evaluability, both intra- and inter-observer reliability were high for grade (ICC 0.93 and 0.78 respectively), density (0.91 and 0.64) and width (0.91 and 0.85). ConclusionsEvaluability is one of the major challenges in assessing nailfold capillaries. However, when images are evaluable, the high intra- and inter-reliabilities suggest that overall image grade, capillary density and apex width have potential as outcome measures in longitudinal studies.

SP0173 Manual and Semi Automatic Identification and Scoring of the Qualitative Capillaroscopic Patterns in Systemic Sclerosis

Microvascular damage is one of the most important clinical feature of systemic sclerosis (SSc), and nailfold videocapillaroscopy (NVC) is the recognized tool to study morphological capillary abnormalities. NVC may detect...

SP0115 Manual and Semi Automatic Identification and Scoring of the Qualitative Capillaroscopic Patterns in Systemic Sclerosis

Microvascular damage is one of the most important clinical feature of systemic sclerosis (SSc), and nailfold videocapillaroscopy (NVC) is the recognized tool to study morphological capillary abnormalities. NVC may detect...

SAT0329 Scleroderma in Men in Northwestern Spain: Differentiating Clinical Features

Background85-90% of patients with systemic sclerosis (SSc) are women, so there are few series that have evaluated distinguishing clinical features in men (1).ObjectivesTo describe clinical features of the male subgroup...

AB1017 What Are the Distinct Capillaroscopic Features in Connective Tissue Disease as Compared with Diseases of Social Importance – Arterial Hypertension and Diabetes Mellitus?

BackgroundThe diseases of social importance such as arterial hypertension (AH) and diabetes mellitus (DM) are frequent concomitant pathology in patients with rheumatic diseases. At present, the capillaroscopic findings in these...

Early Diagnostic and Predictive Value of Capillaroscopy in Systemic Sclerosis.

Nailfold microvascular impairment represents an early feature of systemic sclerosis (SSc) and its progression through different patterns of capillary damage and their validated scoring, is evaluable by nailfold videocapillaroscopy (NVC) in a safe and reliable manner. The presence of specific morphological microvascular alterations at the NVC (i.e., presence of giant capillaries) is fundamental and mandatory for the early diagnosis of SSc, together with the presence of the Raynaud's phenomenon. Furthermore, a recent longitudinal study showed a dynamic transition of microvascular damage through different NVC patterns of microangiopathy in almost 50% of SSc patients and clinical symptoms progressed in accordance with the NVC morphologic changes in 60% of the SSc patients. A pilot study was the first demonstrating an association between baseline NVC patterns and future severe, peripheral vascular and lung involvement with stronger odds according to worsening scleroderma patterns. Prognostic indexes for digital trophic lesions, especially for daily use in SSc clinics and simply limited to the mean score of capillary loss are now validated. Very recently, it has been described that efficacious potentially disease modifying therapies in SSc may interfere with progression of nailfold microvascular damage, as assessed by NVC, over long term at least in presence of digital ulcers. NVC is a safe and reliable tool for the early diagnosis of SSc and the different NVC scleroderma patterns have a predictive value for the clinical complications of the disease.

SP0044 How to Score the Qualitative Capillaroscopic Patterns in Systemic Sclerosis

Vascular damage is one of the key factors of systemic sclerosis (SSc), and nailfold videocapillaroscopy (NVC) is the recognized tool to study the microvascular bed, showing a high diagnostic sensitivity....

SP0153 How to Score the Qualitative Capillaroscopic Patterns in Systemic Sclerosis

Vascular damage is one of the key factors of systemic sclerosis (SSc), and nailfold videocapillaroscopy (NVC) is the recognized tool to study the microvascular bed, showing a high diagnostic sensitivity....

AB0805 Nailfold capillaroscopy findings are different between patients with U1RNP antibody and systemic sclerosis or patients with systemic lupus erythematosus and U1RNP antibody

BackgroundNailfold capillaroscopy (NC) is the best tool to study microcirculation in patients with Raynaud’s phenomenon (RF) or patients with connective tissue diseases. There are some characteristic capillaroscopy findings in systemic...

Capillaroscopy

Capillaroscopy is the most reliable way to distinguish between primary and secondary Raynaud's phenomenon (RP) through identification of an early pattern of systemic sclerosis (SSc). The presence of giant capillaries and microhaemorrhages on nailfold videocapillaroscopy (NVC) is sufficient to identify the scleroderma pattern (early), and an increase in these features and the addition of loss of capillaries (active pattern) is followed by neo-angiogenesis, fibrosis and 'desertification' (late pattern). The sensitivity of the American College of Rheumatology's classification criteria for SSc increases from 67% to 99% with the addition of these specific NVC abnormalities. Based on the appearance of the scleroderma pattern on NVC, almost 15% of patients shift from primary to secondary RP over a mean follow-up period of 29.4+/-10 months. Follow-up by NVC (every 6 months) is suggested for RP patients. A scoring system for NVC changes is available, and scores change significantly during follow-up of SSc patients. Several other NVC patterns have also been identified, such as in dermatomyosistis, systemic lupus eythaematosus, mixed connective tissue disease and antiphospholipid syndrome.

Predictive value of naitfold capillaroscopy in patients with Raynaud’s phenomenon

The objective of this study was to evaluate the long-term follow-up of patients with Raynaud's phenomenon (RP) and pathological nailfold capillaroscopy (NC) in order to analyse the predictive value of specific features of capillaroscopy for the development of a connective tissue disease (CTD). From 1992 to 2002, NC alone or combined with fluorescence videomicroscopy with sodium fluorescein (NaF) was performed in 1024 consecutive patients because of RP. We analysed the follow-up and pathological features of NC in all patients who had neither clinical nor serological signs of a CTD at the time of NC. Of 308 patients with neither serological findings nor clinical signs of CTD but with RP and pathological features in NC suspicious for CTD, follow-up data were available for 133 patients. An additional NaF test had been performed in 51 (38.4%) patients. After a mean follow-up of 6.5 years (range: 1-15 years), 109 patients had developed a CTD and 24 patients did not show any clinical signs or serological markers for a CTD after a mean follow-up of 8.5 years (range: 2-15 years). There were no differences in age, duration of RP or of follow-up in patients who developed a CTD compared to patients who did not. Significantly more giant capillaries (p=0.0001), avascular fields (p=0.02) and irregular architecture (p=0.0001) had been observed in patients who had developed a CTD during the follow-up of 6.5 years. The presence of giant capillaries, avascular fields and irregular architecture of nailfold capillaries is predictive for the development of a CTD in patients with RP.

Angiogenic and angiostatic factors in systemic sclerosis: increased levels of vascular endothelial growth factor are a feature of the earliest disease stages and are associated with the absence of fingertip ulcers.

To examine whether the lack of sufficient neoangiogenesis in systemic sclerosis (SSc) is caused by a decrease in angiogenic factors and/or an increase in angiostatic factors, the potent proangiogenic molecules vascular endothelial growth factor (VEGF) and basic fibroblast growth factor, and the angiostatic factor endostatin were determined in patients with SSc and in healthy controls. Forty-three patients with established SSc and nine patients with pre-SSc were included in the study. Serum levels of VEGF, basic fibroblast growth factor and endostatin were measured by ELISA. Age-matched and sex-matched healthy volunteers were used as controls. Highly significant differences were found in serum levels of VEGF between SSc patients and healthy controls, whereas no differences could be detected for endostatin and basic fibroblast growth factor. Significantly higher levels of VEGF were detected in patients with Scl-70 autoantibodies and in patients with diffuse SSc. Patients with pre-SSc and short disease duration showed significant higher levels of VEGF than healthy controls, indicating that elevated serum levels of VEGF are a feature of the earliest disease stages. Patients without fingertip ulcers were found to have higher levels of VEGF than patients with fingertip ulcers. Levels of endostatin were associated with the presence of giant capillaries in nailfold capillaroscopy, but not with any other clinical parameter. The results show that the concentration of VEGF is already increased in the serum of SSc patients at the earliest stages of the disease. VEGF appears to be protective against ischemic manifestations when concentrations of VEGF exceed a certain threshold level.