Nailfold Capillaroscopy Patterns Research Articles

Re-evaluation of nailfold capillaroscopy in discriminating primary from secondary Raynaud’s phenomenon and in predicting systemic sclerosis: a randomised observational prospective cohort study

ABSTRACT Background Primary Raynaud’s phenomenon (pRP) is difficult to distinguish from secondary (sRP). Although nailfold capillaroscopy (NFC) may detect early alterations, no universal criteria yet discriminate between pRP from sRP. Objectives To create and validate two NFC scores that could distinguish pRP from sRP and that could predict systemic sclerosis (SSc), respectively. Methods We performed NFC on two separate cohorts with isolated RP, and recorded number of capillaries per field, enlarged/giant capillaries, crossed/bizarre patterns, microhemorrhages, neoangiogenesis, rarefaction, edema, blood flow velocity, stasis. By multivariate regression analysis, we evaluated the adjusted prognostic role of these features in a derivation cohort of 656 patients. Results were used to construct algorithm-based prognostic scores (A and B). These scores were then tested on a confirmation cohort of 219 patients. Results Score A was unable to discriminate sRP from pRP (low negative predictive values with high positive predictive values for any cut-point); score B was unable to discriminate progression to SSc or a SSc-spectrum disorder (low positive predictive values with high negative predictive values for lower cut-points). Conclusion NFC patterns, believed as specific, showed low discriminatory power and on their own are unable to reliably discriminate sRP from pRP or predict evolution to SSc.

Nailfold capillaroscopy in Egyptian systemic lupus erythematosus (SLE) patients: correlation with demographic features and serum levels of IL 17A and IFNs I

BackgroundIn SLE patients, cytokines are linked to endothelial cell damage. Nailfold capillaroscopy (NFC) is a simple method for evaluating micro-vascular abnormalities in different connective tissue diseases (CTDs). The study aimed to detect the levels of interleukin 17A (IL 17A), type I interferons (IFNs I) in the serum, and NFC changes in Egyptian SLE patients compared to a control group and to correlate NFC findings with patients’ demographic features and serum levels of IL 17A and IFNs I.ResultsSerum levels of IL 17A, IFN α, and IFN β were significantly higher in SLE patients than in control group (P < 0.0001). About thirty nine patients (73.6%) of the 53 SLE patients showed abnormal NFC changes. Egyptian SLE patients had a high prevalence of the NFC non-specific pattern, with 32 (60.4%) patients showing non-specific changes and 7 (13.2%) patients showing scleroderma pattern, including 3 (5.6%) patients with active scleroderma pattern and 4 (7.55%) patients with late scleroderma pattern. Furthermore, Raynaud’s phenomenon (RP) was observed in 8 (15.1%) SLE patients, with 3 (5.6%) having normal NFC pattern and 5 (9.4%) having scleroderma pattern. All controls (n = 20) showed normal hairpin shape capillaries. Except for SLEDAI (P = 0.03) and the presence of RP (P < 0.0001), there were no significant differences in demographic and laboratory parameters between the three NFC patterns (normal, non-specific, and scleroderma); additionally, NFC score correlated significantly with SLEDAI (P = 0.021).ConclusionAs a result of the high disease activity, Egyptian SLE patients had elevated serum levels of IL 17A and IFNs I. The most common NFC pattern in Egyptian SLE patients was a non-specific pattern. NFC abnormalities in Egyptian SLE patients were correlated with disease activity but not with patients’ ages, disease duration, or serum levels of IL 17A and IFNs I. SLE patients with scleroderma NFC pattern and RP should be closely followed for the possibility of appearance of anti-U1 RNP antibodies and MCTDS.

Nailfold capillaroscopy as a prognostic marker for connective tissue diseases: An observational clinico-epidemiological study.

Background Nailfold capillaroscopy (NFC) is useful in the diagnosis and assessment of various connective tissue diseases. In this study, NFC findings were studied in patients of systemic sclerosis (SS), systemic lupus erythematosus (SLE) and dermatomyositis. Aims To study the nailfold capillaroscopic findings in patients with connective tissue disorders, their correlation with disease severity, and changes in findings following treatment or disease progression. Methods This observational prospective time-bound clinico-epidemiological study was conducted in 43 patients over 20 months at Topiwala National Medical College and BYL Nair Ch. Hospital, Mumbai. NFC was performed using the polarising mode of a USB 2.0 video-dermatoscope in all 10 fingernails at 50X and 200X. It was repeated at three follow-up visits to look for changes in findings. Results Among SLE patients, eleven (52.4%) had non-specific NFC patterns and eight (38.1%) had SLE patterns. Among systemic sclerosis patients, eight (42.1%) cases had active and late SS patterns respectively, while one (5.3%) each had SLE, non-specific and early SS patterns. After three follow-ups, 10 out of 11 (90.9%) cases with improvement in NFC also showed clinical improvement; this figure was significantly more than 11 out of 23 (47.8%) cases who had no change in NFC but showed clinical improvement. Two of the three dermatomyositis patients showed a non-specific pattern while one showed a late SS pattern at baseline. Limitations A larger sample size would have yielded results with more validity. Standardisation of the interval between baseline and last follow-up to equal to or more than six months would have yielded results with more accuracy. Conclusion Capillary findings change significantly over time and reflect changes in the clinical status of patients of both SLE and systemic sclerosis, and hence are an important prognostic marker. Rather than an overt change in NFC pattern, reduction or increase in abnormal capillaries is a better predictor of change in disease activity.

Comparison of predictive value of FRAX, trabecular bone score, and bone mineral density for vertebral fractures in systemic sclerosis: A cross-sectional study

Assessing fracture risk is important for managing patients with systemic sclerosis (SSc). Vertebral fracture (VF) is the most common fracture and is associated with future VF and non-VF. We aimed to evaluate the predictive value of FRAX, trabecular bone score (TBS), and bone mineral density (BMD) for VFs, compared to rheumatoid arthritis (RA) patients and postmenopausal women, and to identify risk factors for VFs in SSc. In this cross-sectional study, prevalent VFs, 10-year probability of major osteoporotic fracture by FRAX (FRAX-MOF), TBS, and BMD were assessed in women with SSc (n = 69) and RA (n = 58), and postmenopausal women (n = 38). Risk factors for osteoporosis, modified Rodnan total skin score (mRSS), organ involvement, and patterns of nailfold capillaroscopy (NFC) were also evaluated. The accuracy of BMD (T-score ≤ -2.5), TBS and FRAX-MOF, with and without TBS adjustment, to detect prevalent VF was assessed by determining the area under the receiver operating characteristic (ROC) curve. Patients with SSc (14.5%) and RA (17.2%) had significantly more VFs than postmenopausal women (0%) (P = .031). Non-significant differences were observed in TBS and BMD of all groups. The FRAX-MOF were higher in RA (9.2%) than SSc group (6.1%) and postmenopausal women (5.5%) (P < .001). Based on the ROC curve, TBS-adjusted FRAX-MOF (0.803) showed largest area under curve (AUC) to detect the prevalent VFs, followed by FRAX-MOF (0.796), TBS (0.765), and BMD (0.588) in the SSc group. In the RA group, FRAX-MOF had the largest AUC (0.896), followed by TBS-adjusted FRAX-MOF (0.863), TBS (0.736), and BMD (0.686). The cutoffs for FRAX-MOF and TBS-adjusted FRAX-MOF for detecting VFs were 8.95% and 9.7% for SSc, and 14.5% and 14% for RA. No association between VFs and SSc subtypes, organ involvement, mRSS or NFC patterns was found. FRAX-MOF, with or without TBS, had better predictive value for VFs than BMD and TBS in SSc. However, FRAX-MOF underestimated the probability of VFs in SSc compared with RA.

Cutaneous Features, Autoantibody Profile, and Nailfold Capillaroscopy of Systemic Sclerosis: A Study of 60 Cases.

Systemic sclerosis (SSc) is an autoimmune chronic multisystem disorder with a plethora of cutaneous manifestations. These manifestations often may be the only presenting complaint. Early identification of these help in diagnosing grievous systemic manifestations and their prompt and appropriate treatment. To study the clinical profile of SSc, modified Rodnan's skin scoring (mRSS), nailfold capillaroscopy (NFC) patterns, antibody profile in the western India population, and their association with cutaneous manifestations. Patients of SSc fulfilling the European League Against Rheumatism (EULAR) 2013 classification of SSc criteria, who attended dermatology outpatient department (OPD) between January 2017 and September 2018 were included in the study. The demographic data, cutaneous features, autoantibody profile, mRSS, and NFC pattern were noted Results: A total of 60 patients (57 females and 3 males; mean age years) of SSc were evaluated. Clinical subtypes were 40 diffuse cutaneous SSc and 20 limited cutaneous SSc. The most common presenting symptoms were Raynaud's phenomenon (RP) (95%) and skin tightening (90%). The common cutaneous findings were sclerodactyly (86.7%), stellate scars (78.3%), parrot-beaked nose (76.7%), mask-like facies (75%), microstomia (56.7%), salt and pepper pigmentation (55%), puffy finger (46.7%), telangiectasia (46.7%), digital ulcer (38.3%), fixed flexion deformity (33.3%), and calcinosis cutis (8.33%). Limited cutaneous systemic sclerosis (lcSSc) had mRSS score of 8.3 ± 4.1 and diffuse cutaneous systemic sclerosis (dcSSc) subset had a score of 28 ± 10.4. Antinuclear antibody (ANA), Anti-topoisomerase antibody (ATA), and anti-centromere antibody (ACA) were positive in 59, 49, and 7 patients, respectively. The NFC patterns were early (23.3%), active (45%), and late (18.3%). The sample size of the study was small. We were not able to determine the significance of other less common autoantibodies with scleroderma. The study highlights the importance of identifying early cutaneous findings and the role of a useful diagnostic and prognostic reproducible scoring system (mRSS) and NFC.

The relationship of nailfold capillaroscopy patterns with clinical features, functional status, pain and fatigue in patients with systemic sclerosis

Aim: To identify the frequency of scleroderma-type capillaroscopic patterns and evaluate the association of capillaroscopic patterns with clinical parameters, functional status, fatigue, and pain in systemic sclerosis (SSc).&#x0D; Material and Method: This cross-sectional study included SSc patients consecutively between January 2017 and January 2019. Cutaneous involvement was evaluated with the modified Rodnan skin score (mRSS). The presence of digital ulcers, Raynaud phenomenon, interstitial lung disease, pulmonary hypertension, cardiac, gastrointestinal system (GIS), renal, joint and muscle involvement were recorded. The severity of the Raynaud phenomenon, fatigue, pain, and patient global assessment (PGA) was assessed on the Visual Analogue Scale (VAS). The Health Assessment Questionnaire (HAQ) and the Duruoz Hand Index (DHI) were used to assess physical disability and hand function, respectively. Nailfold videocapillaroscopic examinations of the patients were performed, and they were classified into four groups, including normal/non-specific, early, active, and late scleroderma patterns.&#x0D; Results: The mean age of 32 patients with SSc (31 female, one male) was 48.93±12.77. Anormal capillaroscopic examination findings were detected in 93.7% of the patients, and the most common capillaroscopic pattern was the active pattern. The comparison of scleroderma pattern groups revealed no difference in age (p=0.224), but disease duration was shorter in the early pattern group (p=0.005). The duration and severity of the Raynaud phenomenon, and mean mRSS were lower in the early pattern group (p=0.004, p=0.009, and p=0.001, respectively). The digital ulcer (p=0.011) and diffuse cutaneous SSc (p=0.016) were more common in the late pattern group. The percentage of pulmonary hypertension (p=0.011), GIS involvement (p

Nailfold Capillaroscopic Changes as a Marker of Interstitial Lung Disease in Systemic Sclerosis: A Cross-Sectional Study in a Tertiary Care Hospital in Eastern India.

Background:Systemic sclerosis (SSc) is an autoimmune connective tissue disorder causing microvascular abnormality leading to Raynaud's phenomenon, skin tightening, and nailfold capillary changes. The patient may have systemic involvement, among them interstitial lung disease (ILD) and pulmonary arterial hypertension are mainly associated with disease-related mortality.Aims:To find out an association between nailfold capillaroscopic changes with ILD severity.Materials and Methods:An institution based cross-sectional study was performed among the patients of SSc above 18 years. Detailed history was taken. Clinical examinations, nailfold capillaroscopy (NFC) with a dermatoscope and high-resolution computed tomography (HRCT) scan of thorax were done. Data were analyzed in MedCalc statistical software version 20.Results:Out of total 43 patients, 74.4% (n = 32) were female. Mean age was 35.05 ± 7.24 years and mean duration 4.28 ± 2.81 years. Diffuse SSc was found in 88.37% (n = 38). On NFC, early, active, and late patterns were found in 30.2% (n = 13), 25.6% (n = 11), and 44.2% (n = 19) cases, respectively. On HRCT, early, active, and late ILD were present in 18.6% (n = 8), 37.2% (n = 16), and 30.2% (n = 13) cases, respectively. ILD changes were absent in 14% (n = 6) though NFC changes were present in them. Respiratory symptoms were absent in 20.93% (n = 9) patients though all had features of early NFC and among them 9.3% (n = 4) showed early ILD changes on HRCT. Significant associations were found between NFC changes and ILD severity (P = 0.0003), NFC changes and respiratory symptoms (P < 0.0001) and between ILD changes and respiratory symptoms (P < 0.0001).Discussion:NFC, an inexpensive procedure, can be performed in all patients of SSc to detect development of early ILD even before appearance of respiratory symptoms to prevent further progression.

Relationship between type of skin lesions and nailfold capillaroscopy pattern in mixed connective tissue disease

IntroductionMixed connective tissue disease (MCTD) is a rare disease with clinical picture consisted of multiple organ manifestations, including skin changes resembling systemic lupus erythematosus (SLE), systemic sclerosis (SSc), or dermatomyositis (DM). On the background of these manifestations are microvascular changes — alteration of endothelial function and impairment of endothelial progenitor cell. Nailfold capillaroscopy (NFC) is a simple, non-invasive technique for investigating microvascular involvement in rheumatic diseases.ObjectivesTo describe the relationship between type of skin lesions and NFC pattern in MCTD patients.MethodsWe analyzed the clinical picture and NFC patterns in 79 patients with MCTD. The NFC changes were classified into Normal, “Early,” “Active,” and “Late” scleroderma-like patterns (SD-like pattern) based on Cutolo classification.In all patients, subjective and physical examinations were carried out, specifically the occurrence of skin lesions in the course of MCTD was assessed (systemic sclerosis-like (Ssc-like), systemic lupus erythematosus-like (SLE-like), dermatomysitis-like (DM-like)).ResultsSkin changes were present in 64 (81%) patients, involving 43 (54%) SLE-like, 48 (61%) SSc-like, and 4 (5.1%) DM-like. NFC changes were observed in a total of 55 (69.6 %) patients with predominance of the “Early” pattern — 41 (51.9 %) patients. According to skin change phenotypes, NFC changes were observed in 31 (72%) patients with SLE-like and in 32 (66.7%) patients with SSc-like skin phenotypes. The “early” pattern predominated in both group.ConclusionsWe did not find any correlation between NFC pattern and the type skin changes.Key Points• The study did not show a correlation between the presence and absence of skin lesions and NFC pattern.• Scleroderma-like patterns were found in over 60% of patients with mixed connective tissue disease.• The “early” pattern is dominant regardless of the occurrence or absence of skin lesions in patients with MCTD.• Skin lesions, regardless of their type (SLE or SSc), do not correlate with type of lesion found in the NFC examination.

A Wide Spectrum of Ocular Manifestations Signify Patients with Systemic Sclerosis

ABSTRACT Objectives: Systemic sclerosis (SSc) is a rare, chronic connective tissue disease involving multiple organ systems, including the eye. We evaluated the detailed clinical ocular manifestations of outpatients with SSc. Methods: Demographics, disease duration and subtype, nailfold capillaroscopy (NFC) patterns and laboratory parameters encompassing the autoantibody profile of 51 SSc patients were evaluated, and a general ocular examination was performed for each participant. Results: Twenty-nine patients (56.86%) had eyelid skin alterations, 26 (50.98%) had retinal abnormalities, 26 (50.98%) had cataracts, 8 (15.69%) had conjunctival changes, 7 (13.73%) had iris abnormalities, 33 (64.71%) suffered from dry eye disease (DED), and 11 (21.57%) suffered from glaucoma. Significant positive correlations were found between NFC data and both tear breakup time and Ocular Surface Disease Index test values. Conclusions: Eyelid skin abnormalities, DED and retinal abnormalities are among the most common SSc-related ocular involvements. Diverse ophthalmic findings are attributed to the heterogeneity of SSc.

Absence of Scleroderma pattern at nail fold capillaroscopy valuable in the exclusion of Scleroderma in unselected patients with Raynaud's Phenomenon.

BackgroundTo report the predictive value of nail-fold capillaroscopy (NFC) patterns of vasculopathy for systemic sclerosis (Scleroderma; SSc) in an unselected cohort of patients with Raynaud’s phenomenon (RP).MethodsPatients referred to a tertiary SSc clinic with RP were evaluated by light/video-NFC. Clinical diagnosis, details and serology were recorded. Primary RP was defined as RP with no features of connective tissue disease (CTD)/antibody. NFC patterns were determined: normal, non-specific, ‘early’, ‘active’ or ‘late’ SSc patterns. Fulfilment of the VEDOSS or 2013 ACR/EULAR criteria for SSc was determined following NFC assessment.ResultsThree hundred forty-seven patients were referred: mean (SD) age 47 (15.2) years. On clinical review, 54 (16 %) did not have RP, 69 (20 %) had primary RP, 52 (15 %) had SSc and 172 (50 %) had secondary RP. NFC SSc pattern was detected in 80 (23 %) patients; 37/52 with SSc, 30/172 with secondary RP, 9/69 with primary RP and 4/54 with no RP. For identifying patients who met either the VEDOSS or 2013 ACR/EULAR criteria for SSc, detection of a SSc NFC pattern had a sensitivity of 71 %, specificity 95 %, positive predictive value 84 % and negative predictive value 90 %.ConclusionsThe absence of SSc NFC pattern in patients with RP or suspected CTD is very valuable in the exclusion of SSc.

Assessment of nailfold capillaries with a handheld dermatoscope may discriminate the extent of organ involvement in patients with systemic sclerosis.

To investigate whether nailfold capillaroscopy (NFC) patterns assessed through an in-office handheld dermatoscope may reflect the extent of disease severity in systemic sclerosis (SSc). NFC patterns were evaluated with a non-contact, polarized light dermatoscope in 40 consecutive patients with SSc and graded in sequence as 0 = normal, 1 = early, 2 = active, or 3 = late patterns. Disease severity was measured according to a modified Medsger severity scale (MSS). For comparisons, patients were grouped in tertiles according to disease severity, and a numerical correlation between the NFC patterns and the composite MSS score was assessed. Twenty patients had normal or early NFC patterns, most of them (17 individuals, 85 %) having low to moderate disease severity. In contrast, 18 out of 20 (90 %) patients with active or late NFC patterns had moderate to high disease severity. Accordingly, patients with normal/early NFC patterns had a median MSS score of 4 (interquartile range (IQR), 3-5) as compared with 7 (4-8; P = 0.02) in those with active/late patterns. A Spearman's rho coefficient of 0.45 (95 % CI, 0.15-0.67; P = 0.003) was found between the graded scale of NFC patterns and the composite MSS score. A handheld dermatoscope is useful to visualize the NFC patterns in SSc patients, and it is efficient enough to reflect the extent of disease severity.

A comparison between nailfold capillaroscopy patterns in adulthood in juvenile and adult-onset systemic sclerosis: A EUSTAR exploratory study

ObjectiveQualitative capillaroscopy patterns in juvenile- and adult-onset systemic sclerosis (SSc) were studied in adulthood using data from the EULAR Scleroderma Trials and Research (EUSTAR) database. MethodsData collected between June 2004 and April 2013 were examined with focus on capillaroscopy. In this retrospective exploratory study, series of patients with juvenile-onset SSc were matched with series of adult-onset SSc having the same gender and autoantibody profile. Results30 of 123 patients with juvenile-onset and 2108 of 7133 with adult-onset SSc had data on capillaroscopy. Juvenile-onset SSc showed scleroderma pattern more frequently than adult-onset SSc (93.3% and 88%). The OR was 2.44 and 95% CI 0.57–10.41. An active scleroderma pattern was present in 58% of juvenile- and 61% of adult-onset SSc. The OR was 0.91 and 95% CI 0.28–2.93. The late scleroderma pattern was present in 61% of juvenile- and 55.5% of adult-onset SSc. The OR was 1.06 and 95% CI 0.34–3.56. ConclusionThis is the first exploratory study on the comparison of capillaroscopy between juvenile- and adult-onset SSc in adulthood. Juvenile-onset SSc had an increase prevalence of scleroderma pattern, but a similar distribution of the three patterns was suggested. Further studies are needed to define this issue.

Quantitative analysis of nailfold capillary morphology in patients with fibromyalgia.

Background/AimsNailfold capillaroscopy (NFC) has been used to examine morphological and functional microcirculation changes in connective tissue diseases. It has been demonstrated that NFC patterns reflect abnormal microvascular dynamics, which may play a role in fibromyalgia (FM) syndrome. The aim of this study was to determine NFC patterns in FM, and their association with clinical features of FM.MethodsA total of 67 patients with FM, and 30 age- and sex-matched healthy controls, were included. Nailfold capillary patterns were quantitatively analyzed using computerized NFC. The parameters of interest were as follows: number of capillaries within the central 3 mm, deletion score, apical limb width, capillary width, and capillary dimension. Capillary dimension was determined by calculating the number of capillaries using the Adobe Photoshop version 7.0.ResultsFM patients had a lower number of capillaries and higher deletion scores on NFC compared to healthy controls (17.3 ± 1.7 vs. 21.8 ± 2.9, p < 0.05; 2.2 ± 0.9 vs. 0.7 ± 0.6, p < 0.05, respectively). Both apical limb width (µm) and capillary width (µm) were significantly decreased in FM patients (1.1 ± 0.2 vs. 3.7 ± 0.6; 5.4 ± 0.5 vs. 7.5 ± 1.4, respectively), indicating that FM patients have abnormally decreased digital capillary diameter and density. Interestingly, there was no difference in capillary dimension between the two groups, suggesting that the length or tortuosity of capillaries in FM patients is increased to compensate for diminished microcirculation.ConclusionsFM patients had altered capillary density and diameter in the digits. Diminished microcirculation on NFC may alter capillary density and increase tortuosity.

Brief Report: Lysyl Oxidase Is a Potential Biomarker of Fibrosis in Systemic Sclerosis

Fibrosis is a major cause of morbidity and mortality in systemic sclerosis (SSc). Levels of lysyl oxidase (LOX), an extracellular enzyme that stabilizes collagen fibrils, have been found to be elevated in the skin of SSc patients, but have not been evaluated in the serum or correlated with the clinical parameters. We undertook this study to evaluate serum LOX levels in SSc patients and to correlate these levels with clinical parameters of SSc. SSc patients were evaluated for demographic features, clinical manifestations, routine laboratory tests, serum autoantibodies, serum LOX concentrations, and nailfold capillaroscopy patterns. They underwent pulmonary function testing, echocardiography, and high-resolution computed tomography scans of the lung, assessment of skin fibrosis by the modified Rodnan skin thickness score (MRSS), and assessment of disease severity and activity by the Medsger severity scale and the Valentini activity index. Twenty-six SSc patients were evaluated and compared with 25 healthy controls and with 9 disease control patients with primary myelofibrosis. Almost 62% of the SSc patients (16 of 26) had limited cutaneous SSc (lcSSc), while 38% had diffuse cutaneous SSc (dcSSc) (10 of 26); 31% of the patients (8 of 26) had lung involvement. The LOX concentration in SSc patients was higher than that in healthy controls and similar to that in disease controls (P < 0.0001), and it was significantly higher in patients with dcSSc than in those with lcSSc (P = 0.006). The LOX concentration correlated with the MRSS in patients without lung fibrosis. This study is the first to demonstrate high serum LOX levels in SSc patients that correlate specifically with skin fibrosis. These correlations suggest that LOX levels may serve as a novel biomarker of fibrosis. Future studies are warranted to determine whether LOX is a potential therapeutic target in SSc.

AB0819 Clinical course and nailfold capillaroscopy patterns of 69 patients with mixed connective tissue disease (MCTD)

BackgroundMCTD is a rare connective tissue disease with many controversies regarding its recognition, clinical course and assessment. The clinical expression of MCTD is the consequence of its complex immunopathology. The...

Nailfold Capillaroscopy in Evaluation of Systemic Lupus Erythematosus with / without Positive Antiphospholipid Antibodies

Objective: The use of nailfold capillaroscopy (NC) in cases of systemic lupus erythematosus (SLE) and associated antiphospholipid syndrome (APS) has seldom been studied. NC has shown a very high specificity at pattern recognition in assistance of diagnosis of SLE in patients presented with Raynaud's phenomenon (RP). Herein we address the characteristic NC findings in SLE patients with/without positive antiphospholipid antibodies (aPL) in a retrospective study to evaluate the feasibility of NC in predication of SLE associated APS.Methods and Results: A total of 33 patients with SLE in a university hospital who had received an NC exam from 2007 to 2011 were enrolled in our study. The observed frequency of RP in SLE was 74 %. For antiphospholipid positivity association evaluation, the presence of SLE NC pattern in this study revealed 70% sensitivity, 76.9% specificity, 70% PPV (positive predictive value), and 76.9% NPV (negative predictive value). The two-tailed p value by Fisher's exact test was 0.0397. A statistically significant association of thromboembolic events between SLE NC patterns was also found (p=0.0075).Conclusion: The semi-quantitative parameters for the classification of a SLE pattern at NC were predictive of aPL positivity and thromboembolic complications in patients with SLE. The significant association between thromboembolism in patient with SLE and their NC findings in our study suggested that NC study would be helpful in the diagnosis of seronegative APS.

Nailfold capillary abnormalities are prevalent in sclerodermoid graft-versus-host disease and readily detected with dermatoscopy

Well-recognized videocapillaroscopic patterns have been described in systemic sclerosis (SS). However, no studies have described the capillary abnormalities of sclerodermoid chronic graft-versus-host disease (Scl GVHD) developed after allogeneic haematopoietic stem cell transplantation (allo-HSCT). The aims of this study were to find the characteristics of nailfold capillary changes in Scl GVHD after allo-HSCT. Eighteen patients affected by Scl GVHD and a control group of 15 patients with lichenoid GVHD were evaluated. Duration and type of sclerodermoid GVHD, Raynaud phenomenon (RP), dysphagia, joint contractures, antinuclear antibodies (ANA), anti-Scl-70 and anticentromere (ACA) antibodies were investigated parameters. A nailfold capillary examination using a standard dermatoscope was performed on all fingers of each subject. Twelve patients were male and six were female with a mean age of 37 +/- 11.6 years. Joint retractions and dysphagia developed in 27.8% and 38.9% of the patients, respectively. Three (16.7%) patients had RP. Autoimmune markers like anti-Scl-70 and ACA were negative in all. Capillaroscopy was abnormal in 15 patients with Scl GVHD. A regular disposition of the capillary loops along with avascular whitish linear areas at the level of the last row, neovascularization with reticular pattern, capillary disorganization, haemorrhages, enlarged capillaries and avascular areas were the main features. No capillary abnormalities were observed in patients with lichenoid GVHD. There was no statistically significant correlation between ANA positivity, RP, joint retractions, dysphagia, extensiveness of Scl GVHD, duration of sclerodermoid lesions and nailfold capillaroscopy analysis. This study shows the identification of distinct nailfold capillaroscopy patterns in patients with Scl GVHD but it does not confer special risk for any other specific clinical symptoms of the disease.

Autonomic dysfunction and microvascular damage in systemic sclerosis

Systemic sclerosis (SSc) is a connective tissue disease characterized by vascular damage and interstizial fibrosis of many organs. Our interest was focused on the evaluation of cardiac autonomic function by measurements of heart rate variability (HRV) and microvascular damage detected by nailfold capillaroscopy (NC) in SSc patients. We examined 25 consecutive outpatients affected by systemic sclerosis and 25 healthy controls. Exclusion criteria were the presence of cardiac disease, hypertension, diabetes mellitus, or neurological diseases. All subjects underwent 24-h ambulatory ECG Holter recording and NC examination. Heart rate variability was evaluated in the time domain, using appropriate software, computing the time series of all normal-to-normal (NN) QRS intervals throughout the 24-h recording period. A semiquantitative rating scale was adopted to score the NC abnormalities, as well as a rating system for avascular areas and morphological NC patterns. In SSc patients, HRV analysis showed significantly lower values of SDNN (standard deviation of all NN intervals) (p=0.009), SDANN (standard deviation of the averages of NN intervals in all 5-min segments of the entire recording) (p=0.01), and pNN50 (the percentage of adjacent NN intervals that differed by more than 50 ms) (p=0.02), compared to the control group. These parameters in SSc patients significantly decreased with the worsening of semiquantitative capillaroscopy score. In conclusion, an abnormal autonomic nervous control of the heart might contribute to identify subclinical cardiac involvement in SSc patients. The coexistence of autonomic dysfunction with a more severe microvascular damage could be considered a potential prognostic tool in the identification of those patients particularly at risk for cardiac mortality.

Evaluation of Nailfold Videocapillaroscopic Abnormalities in Patients With Systemic Lupus Erythematosus

Nailfold capillaroscopy (NFC) has been shown to have a remarkable value in the differential diagnosis of connective tissue diseases. In fact, NFC patterns reflect the microvascular changes that may play a significant role in pathogenesis. The aims of this study were to determine in patients with systemic lupus erythematosus (SLE) the prevalence of NFC patterns, to evaluate any association with clinical features and laboratory parameters. One hundred twenty-three patients with SLE were included in this retrospective study. Video NFC parameters were analyzed in each patient. In all cases, the following parameters were evaluated: capillary arrangement, density, size, and shape. In patients with SLE, major capillary abnormalities were frequently observed (44 of 123 = 35.8%); however, no specific pattern was noted. There was a significant correlation between the SLEDAI index and the severity of capillary abnormalities (P < 0.0001). Pathologic capillary abnormalities were also significantly increased in SLE with positive anti-U1-RNP antibodies (P < 0.05). NFC may be a useful method to evaluate the microvascular changes in patients with SLE, and the presence of major capillary abnormalities seems to herald a more severe clinical course of the illness.

Interleukin-13 in systemic sclerosis: relationship to nailfold capillaroscopy abnormalities.

The aim of this study was to investigate whether interleukin-13 (IL-13) serum levels correlate to different nailfold capillaroscopy (NC) findings in patients with systemic sclerosis (SSc). IL-13 serum levels were measured using an ELISA method. The following NC abnormalities were considered: the presence of giant loops, haemorrhages, loss of capillaries, disorganisation of the vascular array, ramified/bushy capillaries and sludging of blood. A semiquantitative rating scale was adopted to score these changes, as well as a rating system for avascular areas and three morphological NC patterns ('early', 'active' and 'late'). Mean capillary density was determined by counting the total number of capillaries in a 1 mm length, and the arterial and venous diameters of the capillary as well as the total loop diameter were measured. In SSc patients IL-13 serum levels were significantly higher than in controls ( P < 00.1), whereas in patients with ( n=8) and without ( n=24) abnormal IL-13 serum levels (>17 pg/ml) the comparison of the NC features showed significantly relevant differences concerning a more frequent 'active' NC pattern ( P < 0.02), the presence of haemorrhages ( P < 0.0037) and sludging of blood ( P < 0.038), as well as larger total loop ( P < 0.036) and arterial ( P < 0.03) diameters, in those patients with elevated IL-13 serum levels. The study confirmed that IL-13 serum levels are higher in the sera of patients with SSc, and shows for the first time the significant correlations between this serological finding and some of the main relevant SSc capillaroscopic features, leading us to believe that this cytokine not only seems to sustain the immunological and fibrotic process of SSc, but might have a role in determining the more severe microvascular lesions in this disease.