Nailfold Capillary Abnormalities Research Articles

Calcinosis in dermatomyositis.

To provide the most recent literature on our understanding behind the pathogenesis and the treatment of calcinosis in dermatomyositis. Early diagnosis and controlling the overall disease activity are cornerstones to prevent calcinosis in juvenile dermatomyositis. Observational cohort studies showed that prolonged state of inflammation and features of vascular dysfunction like digital ulcers and abnormal nailfold capillaries are associated with calcinosis. Neutrophil activation and mitochondrial dysfunction have recently emerged as potential mechanistic pathways involved in calcinosis pathogenesis. Few recent case series have alluded to the efficacy of topical and intralesional sodium thiosulfate, while JAK inhibitors appear to be newer promising therapy in juvenile dermatomyositis. Calcinosis in dermatomyositis consists of deposition of insoluble calcium compounds in the skin and other tissues. It is prevalent in up to 75% of patients with juvenile dermatomyositis and up to 20% in adult dermatomyositis. While it leads to significant patient morbidity, we do not yet understand the pathogenesis in its entirety. Surgical excision although palliative is the mainstay of treatment and should be offered to patients. All available treatment options are only based on very low level of evidence.

A Medical Record-based Pilot Study on the Diffusing Capacity of the Lungs for Carbon Monoxide versus Nailfold Capillaroscopy as an Early Marker for Lung Involvement in Connective Tissue Disease-related Interstitial Lung Diseases

Abstract Background: Connective tissue disease (CTD)-related interstitial lung diseases (ILDs) are one of the most common ILDs which affect relatively younger populations and can lead to extensive lung damage and fibrosis. Early detection and initiation of anti-inflammatory therapy can curtail the extent of lung damage. The diffusing capacity of the lungs for carbon monoxide (DLCO) is a well-established early marker of ILD. While most of the studies have shown specific nailfold capillaroscopy (NFC) findings in patients with CTD-ILD, none of them has evaluated the relation between diffusion impairment and nailfold capillary changes. The present study was carried out to evaluate the relationship between DLCO and NFC as markers for lung involvement in CTD-ILDs. Materials and Methods: This was a medical record-based observational study wherein the medical records of 100 diagnosed patients of CTD-ILD attending the pulmonology and rheumatology outpatient department were screened. Data concerning NFC, spirometry, and DLCO were collected and analyzed. Results: In our study of 100 patients, the minimum age was 29 years, and the maximum age was 78 years. A majority of patients in the study were found to be males (58%) and were suffering from CTD-ILD for a duration ranging from 2 to 5 years (58%). Eighty-nine percentage had a functional impairment on spirometry in the form of reduced forced vital capacity, and 61% had impaired DLCO. Nailfold capillary abnormalities were observed in all but one patient. The most common abnormal finding was a loss of capillary density (95% of patients) and a decrease in the intercapillary distance (94% of patients). A significant correlation was seen between diffusion impairment and abnormal nailfold capillary length. However, there was no significant correlation between DLCO and overall nailfold capillary abnormalities. Conclusion: The study concludes that NFC cannot be used as a tool to monitor diffusion impairment of the lungs in CTD-ILD. DLCO is a better assessment method compared to NFC changes as an indicator of lung involvement in CTD-ILD.

Nailfold videocapillaroscopy in antineutrophil cytoplasmic antibody-associated vasculitis.

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of illnesses that cause inflammation and alterations to small vessels in the body. Some of the most common and detrimental manifestations, including alveolar hemorrhage and glomerulonephritis, are caused by this capillary inflammation. We sought to clarify whether patients with AAV would have abnormal nailfold capillaries when evaluated with nailfold videocapillaroscopy. Patients with a current diagnosis of AAV and a control group were identified for enrollment. Nailfold videocapillaroscopy images were used for a semiquantitative analysis on capillary density, morphology, dilation, and microhemorrhage after review by 2 rheumatologists. Disease characteristics, occurrence of recent disease flare, and presence of ANCA were recorded. Thirty-three patients with a diagnosis of AAV and 21 controls were recruited. The AAV group had a median age of 59 and 17 (52%) were women. Granulomatosis with polyangiitis was the most common diagnosis (19 [58%]), followed by eosinophilic granulomatosis with polyangiitis (7 [21%]) and microscopic polyangiitis (7 [21%]). Twenty-seven patients (82%) had positive ANCA tests. After assessment of capillary density, dilation, morphology, microhemorrhages, and disorganization, there were no statistically significant differences between the 2 groups. There was no evidence of differences in nailfold capillaroscopy abnormalities between those diagnosed with AAV and the control group. While this cohort was relatively small, we did not find a high enough prevalence or specific phenotype of capillary abnormalities that could aid in diagnosis or prognostication of these diseases in the clinical setting.

Utility of nailfold capillary assessment for predicting pustulotic arthro-osteitis in palmoplantar pustulosis based on a prospective cohort study

Pustulotic arthro-osteitis (PAO) is 1 of the most serious comorbidities associated with palmoplantar pustulosis (PPP). Risk factors of PAO development are not well-known. To evaluate the clinical significance of nailfold capillary (NFC) changes in patients with PPP. We conducted a prospective cohort study in a population of 102 PPP patients. Correlations of NFC abnormalities, including nailfold bleeding and enlarged capillaries, with the prevalence of PAO, the incidence of new PAO, and serum levels of cytokines were analyzed. Detailed examination revealed that of 102 PPP patients, 52 without PAO and 50 with PAO. Both nailfold bleeding and enlarged capillaries were significantly more frequent in patients with PAO (50.0% vs 92.0%, P<.0001; 50.0% vs 94.0%, P<.0001). In addition, PPP patients without PAO were prospectively observed before they developed PAO (mean 28months [1-52months]). Multivariate analysis suggested that these NFC abnormalities were predictors of PAO development (hazard ratio 3.37, 95% confidence interval 1.13-10.07; 3.37, 1.13-10.07) and guselkumab prevent PAO development (0.093, 0.012-0.76). The degree of NFC abnormalities correlated with the severity of PAO and serum cytokine levels. All participants were Japanese. NFC abnormalities could be predictors of PAO in PPP patients, and their degree indicators of disease severity.

Increased oxidative stress response in circulating blood of systemic sclerosis patients – relation to disease characteristics and inflammatory blood biomarkers

BackgroundSystemic sclerosis (SSc) is a rare connective tissue disorder of unknown etiology characterized by organ fibrosis and microcirculation dysfunction. Emerging evidence suggests that SSc is related to increased oxidative stress, which contributes to further tissue and vascular damage. MethodsOxidative stress response in the peripheral blood was assessed in patients with SSc (n = 55) and well-matched controls (n = 44) using real-time monitoring of protein hydroperoxide (HP) formation by the coumarin boronic acid (CBA) assay. We also analyzed the relationship between HP generation and SSc clinics, systemic inflammation, and cellular fibronectin, an emerging biomarker of endothelial damage. ResultsSSc was characterized by a significantly faster (2-fold) fluorescent product generation in the CBA assay and higher cumulative HP formation (3-fold) compared to controls (p<0.001, both). The dynamics of HP generation were not associated with the form of the disease (diffuse vs. limited SSc), current immunosuppressive therapy use, presence of abnormal nailfold capillaries, and autoantibody profile. Still, it was enhanced in patients with more severe illness and certain clinical manifestations (i.e., pulmonary hypertension, digital ulcers, and cyclophosphamide treatment) and in smokers (current or past). Higher serum CRP, blood eosinophil count, and cellular fibronectin with lower hemoglobin levels were independent determinants of increased HP formation. ConclusionsOur data indicate a pro-oxidant imbalance in SSc, likely related to systemic inflammation and endothelial injury. However, extensive prospective studies are needed to verify whether it is also associated with clinical disease progression.

Epidemiology and Survival of Systemic Sclerosis-Sarcoidosis Overlap Syndrome.

We evaluated the epidemiology, manifestations, serology, comorbidities, and survival among patients with systemic sclerosis (SSc) with and without sarcoidosis. We conducted a retrospective cohort study comparing patients with SSc with and without sarcoidosis. All patients fulfilled the American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for SSc. Sarcoidosis was based on physician diagnosis and/or confirmatory biopsy. The primary outcome was time from diagnosis to all-cause mortality. Survival was evaluated using Kaplan-Meier curves. We included 1977 patients (1971 with SSc, 6 with SSc-sarcoidosis) with a SSc-sarcoidosis prevalence of 0.30%. Sarcoidosis frequently preceded SSc (66.66%). The most frequent sarcoidosis manifestations were pulmonary (66.66%), lymphadenopathy (66.66%), arthritis (50%), cutaneous (33.33%), and hepatic (16.66%). Patients with SSc and SSc-sarcoidosis had female to male sex ratios of 4.5:1 vs 5:1 and median ages of SSc onset of 48.3 vs 43.8 years, respectively. Interstitial lung disease (35% vs 66.66%) and pulmonary hypertension (24.91% vs 50%) tended to occur more frequently whereas abnormal nailfold capillaries (34.7% vs 16.66%) and digital ulcers (33.33% vs 16.66%) tended to occur less frequently among patients with SSc-sarcoidosis, but the differences were not significant. There was an increased frequency of stroke among the patients with SSc-sarcoidosis (relative risk 8.59, 95% CI 1.02-72.00). The median survival times were 23.4 years for SSc-sarcoidosis and 18.6 years for SSc, with no differences in survival curves (log-rank test, P = 0.55). Sarcoidosis in SSc is rare but appears to occur more frequently than in the general population. It is associated with pulmonary, lymph node, cutaneous, joint, and hepatic involvement. Stroke occurs more frequently in patients with SSc-sarcoidosis but with no differences in survival.

The clinical features of juvenile dermatomyositis: A single-centre inception cohort

IntroductionJuvenile Dermatomyositis (JDM), a severe and rare autoimmune disease, is the most common idiopathic inflammatory myopathy in children. We describe the clinical features of a large single-centre cohort. MethodsWe studied an inception cohort (0-18 years old) referred for diagnosis to the JDM clinic at The Hospital for Sick Children (SickKids), between January 1989 and September 2017. Probable or definite diagnosis of JDM was done according to the 2017 ACR/EULAR Criteria. We excluded children who had treatment started at another hospital. The data were collected retrospectively from clinical charts and the SickKids JDM database. Results172/230 (74.8%) patients were included. They were most often female (female:male = 1.8:1); the age at diagnosis was 8.5±4.3 years. There was a positive family history for autoimmune disease in 52%, mainly rheumatoid arthritis. No patient died. The most common signs at inception were muscle weakness (85.5%), nailfold capillary abnormalities (83.4%), Gottron papules (78.5%), heliotrope rash (66.3%), abnormal gait (55.8%), and malar/facial rash (54.7%). The prevalence of Gottron papules, heliotrope rash, facial/malar rash, nailfold capillary abnormalities, Raynaud phenomenon, dysphonia/dysphagia (a frequent cause of hospitalization), mouth ulcers, calcinosis, eye problems, joint involvement, acanthosis nigricans and lipodystrophy increased during follow-up. Muscle enzymes, namely CK, ALT, AST, were often normal or only slightly raised despite active muscle disease; conversely LD was often high. Anti-Nuclear Autoantibodies were positive in 49.7% of patients at diagnosis. The course of the disease was: 29.1% monocyclic, 5.3% polycyclic, 33.1% chronic. The course of 56 patients (32.5%) was not classifiable due to length of follow-up. Corticosteroids were used as treatment in almost all our patients and 30% required intravenous therapy due to the severity of the presentation; methotrexate was added in 64%, more often in recent years. Unresponsive patients were treated mostly with intravenous immunoglobulins (IVIG). ConclusionsThe information obtained from this relatively large number of patients adds to the growing knowledge base of this rare disease. Trial registrationSickKids Research Ethics Board approved the study.

Predicting the Progression of Very Early Systemic Sclerosis: Current Insights.

Systemic sclerosis (SSc) is a complex autoimmune connective tissue disease with distinct pathological hallmarks (ie, inflammation, vasculopathy, fibrosis) that may predominate at different stages in the disease course with varying severity. Initial efforts to classify patients with SSc identified a subset of patients with very early SSc. These patients possessed signs of SSc (eg, Raynaud phenomenon, SSc specific autoantibodies and/or nailfold capillary abnormalities) without fulfilling complete SSc classification criteria. Recognizing the inherent value in early diagnosis and intervention in SSc, researchers have endeavored to identify risk factors for progression from very early SSc to definite SSc. The present review summarizes the clinical phenotype of patients with very early and early SSc. Through a scoping review of recent literature, this review also describes risk factors for progression to definite SSc with a focus on the specific clinical features that arise early in the SSc disease course (eg, diffuse cutaneous sclerosis, interstitial lung disease, esophageal dysfunction, renal crisis, cardiac involvement). In addition to clinical risk factors, this review provides evidence for how biological data (ie, serological, genomic, proteomic profiles, skin bioengineering methods) can be integrated into risk assessment models in the future. Furthering our understanding of biological features of very early SSc will undoubtedly provide novel insights into SSc pathogenesis and may illuminate new therapeutic targets to prevent progression of SSc.

223 Evaluation of nailfold capillaries in dermatomyositis using a dermatoscope

Nailfold capillary (NC) abnormalities are increasingly utilized in the evaluation of rheumatic conditions. Their presence can distinguish primary Raynaud’s phenomenon from secondary etiologies and are used in the diagnostic criteria of scleroderma. Dermoscopy is a convenient method of evaluating NC changes with similar efficacy to capillaroscopy. We imaged all ten nailfolds of subjects with dermatomyositis (DM) using a dermatoscope with a smartphone adapter. Fingernails were cleaned with an alcohol swab prior to evaluation. Images were assessed for loop dilation (LD), abnormal morphology (AM), capillary bed disorganization (CBD), capillary hemorrhage (CH), capillary dropout (CD), decreased capillary density (DCD), and subpapillary plexus visibility (SP). Cutaneous Dermatomyositis Area and Severity activity (CDASI-A) scores and systemic activity (lung, muscle, or joint involvement) were collected. Analysis used Student’s t-test and chi-square test. Subjects with hypertension, hyperlipidemia, diabetes, glaucoma, or other primary rheumatic disease were excluded. 44 subjects, 17 with amyopathic DM (ADM), were included. Mean (standard error) CDASI activity and damage scores were 13 (1.05) and 4 (0.39), respectively. 89% of subjects demonstrated capillary abnormalities. LD (77%), followed by CBD (73%), were the two most common findings. NC changes were most often found on 4th and 5th fingers. Thumbs demonstrated the fewest abnormalities. Mean CDASI-A was higher in patients with AM (CDASI 16 vs 10, p=0.003), CD (15 vs 10, p=0.016), DCD (17 vs 10, p<0.001), and trended towards significance for LD and CBD. There was a trend towards a higher percentage of CH in patients with ADM compared to those with muscle disease (no difference in CDASI-A). Subjects with skin limited disease (n=23) vs active systemic disease demonstrated more AM (74% vs 38%, p=0.02; no difference in CDASI-A). Given its prevalence, NC changes are useful in the evaluation of DM. AM, CD, and DCD may be markers of cutaneous disease activity.

Bilateral popliteal artery occlusion in a young woman as a rare complication of systemic sclerosis: A case report

Systemic sclerosis (SSc) is an autoimmune rheumatic disorder characterized by vascular damage, sclerotic skin changes, and multi-system involvement of internal organs. Digital ischemic lesions, nailfold capillary abnormalities, and secondary Raynaud’s phenomenon are common manifestations of microvascular injuries. Recent findings have revealed that macrovascular involvement in SSc more prevalent than previously believed. There have been reports of large vessel involvement, such as the ulnar, femoral, carotid, renal, and pulmonary arteries. We report here a case of a young woman with progressive SSc who complained of lower limb pain, dysphagia secondary to esophageal dysfunction, and urinary incontinence.She had symptoms of Raynaud’s phenomenon, skin sclerosis, sclerodactyly, digital pitting ulcers,nailfoldcapillary abnormalities, and digital ischemia. On special examination, the anti-centromere antibody was detected. She developed bilateral popliteal artery occlusion as a complication of SSc.

Severe digital ischemia as an unrecognized manifestation in patients with antisynthetase autoantibodies: Case series and systematic literature review.

Severe digital ischemia, including digital ulcers and gangrene, is considered rare in patients with antisynthetase antibodies. This study aimed to elucidate the clinical features of antisynthetase-positive patients complicated with digital ulcers and/or gangrene using a systematic literature review and case series in a single-center cohort. A systematic literature review was conducted to identify reports describing antisynthetase-positive cases with digital ulcers and/or gangrene. Our cohort of consecutive patients with antisynthetase antibodies was stratified by the history of severe digital ischemia. Demographic and clinical features and outcomes in patients with severe digital ischemia identified in the systematic literature review and our cohort were compared with those in patients without severe digital ischemia in our cohort. The systematic literature review revealed 12 antisynthetase-positive patients with severe digital ischemia from one case series and eight case reports. Seven (7%) of 100 patients with antisynthetase antibodies in our cohort had a record of severe digital ischemia. Severe digital ischemia was often found at presentation and was associated with the classification of systemic sclerosis with or without myositis overlap. Clinical features associated with severe digital ischemia in antisynthetase-positive patients included Raynaud's phenomenon (p < 0.001), digital pitting scars (p = 0.001), and nailfold capillary abnormality (p = 0.02). Outcomes of severe digital ischemia were generally favorable with vasodilators. Severe digital ischemia is an overlooked complication in antisynthetase-positive patients. Antisynthetase antibodies should be measured in patients presenting with digital ulcers or gangrene, especially in those with systemic sclerosis phenotype and features associated with antisynthetase antibodies in the absence of systemic sclerosis-specific autoantibodies.

Dermatomyositis and juvenile dermatomyositis

Dermatomyositis (DM) is an inflammatory multisystem disease of unknown etiology, which can already occur in children but first onset can also be in older adulthood. Myalgia and muscle weakness can occur later in the course of the disease or even be completely absent in some forms. Classical signs on the skin include heliotrope rash, facial erythema, Gottron's papules and nailfold capillary abnormalities. For the diagnosis, screening for the presence of myositis-specific autoantibodies has become increasingly more relevant. Muscle enzymes may be elevated but not in approximately one third of patients. In the absence of typical clinical or serologic findings, additional examination methods such as nailfold capillaroscopy, magnetic resonance imaging, electromyography, skin or muscle biopsies may help to establish the diagnosis. Depending on the clinical and serological subtype, additional screening for gastrointestinal or cardiopulmonary involvement should be considered. In adults, an age-appropriate tumor screening should also be performed. Apart from corticosteroids as induction therapy, biologics and small molecule inhibitors are gaining in importance in addition to conventional disease-modifying anti-rheumatic drugs and intravenous immunoglobulins. The prognosis for DM and juvenile DM (JDM) has improved. Most patients recover at least to some extent; however, afew patients die and a minority develop persisting muscle atrophy or severe calcinosis.

Association between nailfold capillaroscopy abnormalities and autoimmune disease in pediatric populations.

Time to diagnosis of autoimmune disease in pediatric populations can take years but nailfold capillaroscopy (NFC) may identify early signs of autoimmune disease. The aim of this study is to assess the association between nailfold capillary abnormalities and autoimmune disease in children. A systematic search of PubMed, EMBASE, and Scopus was performed to identify all studies published before March 17, 2021. Observational studies reporting NFC outcomes in children with autoimmune disease and healthy controls (HC) were eligible for inclusion. Odds ratios (OR) and 95% confidence intervals (CI) were pooled using a random-effects meta-analytical model. Nine of 3665studies reporting on 641 patients (398subjects, 243 controls) were included. Pediatric patients with autoimmune disease were 9.88 (95% CI 3.16-30.87, I2 =80.1%) times more likely to have abnormal nailfold capillaries than HC. Of the capillaroscopic features, dilated capillaries (OR 27.90, 95% CI 2.17-349.05, I2 =59.9%) were the most likely abnormality observed on NFC. This was followed by the likelihood of reduced capillary density (<7capillaries/mm) (OR 19.91, 95% CI 3.79-105.52, I2 =0%), giant capillaries (OR 12.87, 95% CI 2.38-69.45, I2 =0%), hemorrhages (OR 13.89, 95% CI 5.34-36.16, I2 =0%), and avascularity (OR 10.38, 95% CI 2.20-49.04, I2 =0%). Children with autoimmune disease are significantly more likely to have nailfold capillary abnormalities. NFC may be useful in identifying early signs of underlying rheumatic disease and potentially decrease the time to diagnosis for this patient population.

Juvenile systemic sclerosis - observations of one clinical centre.

ObjectivesThe systemic form of scleroderma (SSc) in children is a very rare disease; therefore, it is recognized relatively late, which increases the risk of complications. The aim of the study was to assess the clinical symptoms of juvenile systemic sclerosis (JSSc) in our cohort patients.Material and methodsA group of (N = 22) scleroderma patients aged between 2 and 16 years were observed. Demographic data and all clinical results obtained during 16 years of observation in the clinic of rheumatic diseases of developmental age were collected and analysed.ResultsIn all observed children the major JSSc criterion was found, i.e. skin thickening proximal to the metacarpal phalangeal and/or metatarsophalangeal joints. Other symptoms are presented as follows: nailfold capillary abnormalities – 100%, Raynaud’s phenomenon – 90.9%, sclerodactyly – 27.3%, digital tip ulcers – 27.3%, dysphagia – 18.2%, gastroesophageal reflux – 27.3% (assessed in only 10 children), arrhythmias – 22.7%, heart failure – 9.1%, new-onset arterial hypertension – 9.1%, pulmonary fibrosis – 72.7%, pulmonary arterial hypertension – 9.1%, neuropathy – 13.6%, carpal tunnel syndrome – 4.5%, tendon friction rubs – 4.5%, arthritis – 22.7%, and myositis – 13.6%. There were no cases of renal crisis. Decreased diffusing capacity of oxygen was confirmed in 12 patients (58.3%). The presence of antinuclear antibodies was noticed in 86.7% of patients, and among SSc selective autoantibodies: anticentromere – 31.8%, anti-topoisomerase I – 18.2%, anti-PM-Scl 100 or 75 – 45.5%, anti-RP11, Th/To, PCNA in total in 27.3% were presented. In 4.5% of cases, apart from the presence of anti-PM-Scl autoantibodies, positive lupus band test, reduced concentration of complement, and antiphospholipid antibodies were also found. In 59% of studied children, the body mass index was below the 25th percentile.ConclusionsThe presented retrospective analysis shows that the occurrence of Raynaud’s phenomenon with changes in nailfold capillaroscopy is the best screening toll for the assessment of risk of JSSc. All patients of developmental age with Raynaud’s phenomenon, especially in the case of the appearance of antinuclear antibodies, should be monitored with capillaroscopy regardless of other laboratory or imaging tests.

Nailfold capillaries and myositis-specific antibodies in anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis.

This study aimed to quantify nailfold capillary (NFC) abnormalities in anti-melanoma differentiation-associated gene 5 (MDA5) -positive DM patients and to evaluate the association with clinical parameters, including serum biomarkers. In addition, we aimed to clarify the period leading to remission of NFC abnormalities during immunosuppressive treatment in patients with DM. A prospective observational study was conducted including patients (n = 10) who first visited Hiroshima University Hospital and were diagnosed with DM or clinically amyopathic DM with anti-MDA5 antibodies. We compared the NFC abnormalities detected by nailfold-video capillaroscopy (NVC), physical findings, blood tests, respiratory function tests, and vascular-related growth factors measured using a LEGENDplexTM Multi-Analyte Flow Assay Kit. NFC abnormalities improved in all patients from 2 to 17 weeks after the initiation of immunosuppressive treatment. The NVC scores were inversely correlated with anti-MDA5 antibody titres at baseline. NVC scores and forced vital capacity were positively correlated. Baseline values of M-CSF and stem cell factor were correlated with anti-MDA-5 titres. Our study suggested that NVC scores and disease activity were inversely correlated before treatment. Vascular-related growth factors, such as M-CSF and stem cell factor, may be associated with the disease mechanism in patients with anti-MDA5 antibody-positive DM.

Nailfold capillary abnormalities in childhood-onset systemic lupus erythematosus: a cross-sectional study compared with healthy controls.

ObjectivesFor selection of high-risk systemic lupus erythematosus (SLE) patients it is necessary to obtain indicators of disease severity that predict disease damage. As in systemic sclerosis, nailfold capillary abnormalities could be such a biomarker in SLE. The primary objective of this cross-sectional study is to describe capillary abnormalities in childhood-onset SLE (cSLE) cohort (onset < 18 years) and compare them with matched healthy controls. The secondary objective is to correlate the observed capillary abnormalities with demographical variables in both cohorts and with disease-specific variables in cSLE patients.MethodsHealthy controls were matched for ethnic background, age and gender. Videocapillaroscopy was performed in eight fingers with 2-4 images per finger. Quantitative and qualitative assessments of nailfold capillaroscopy images were performed according to the definitions of the EULAR study group on microcirculation in Rheumatic Diseases.ResultsBoth groups (n = 41 cSLE-patients and n = 41 healthy controls) were comparable for ethnic background (p = 0.317). Counted per mm, cSLE-patients showed significantly more ‘giants’ (p = 0.032), ‘abnormal capillary shapes’ (p = 0.003), ‘large capillary hemorrhages’ (p < 0.001) and ‘pericapillary extravasations’ (p < 0.001). Combined ‘abnormal capillary shapes and pericapillary extravasations’ (in the same finger) were detected in 78% (32/41 patients). By qualitative analysis, ‘microangiopathy’ was detected in 68.3% (28/41) and a ‘scleroderma pattern’ in 17.1% (7/41) of the cSLE-patients (without scleroderma symptoms). The difference of percentage positive anti-RNP antibodies in the group with or without a scleroderma pattern was not significant (p = 0.089). The number of ‘abnormal capillary shapes per mm’ was significantly correlated with treatment-naivety. The number of ‘large pathological hemorrhages per mm’ was significantly correlated with SLEDAI score and presence of nephritis. Compared to healthy controls, ‘pericapillary extravasations’ were found in significantly higher numbers per mm (p < 0.001) as well as in percentage of patients (p < 0.001).ConclusionsOur observations confirm that giants, abnormal capillary morphology and capillary hemorrhages are also observed in cSLE, as was already known for adults with SLE. Number of capillary hemorrhages in cSLE was significantly correlated with disease activity. A high frequency and total amount of “pericapillary extravasations” was observed in cSLE patients, possibly revealing a new subtype of capillary hemorrhage that might reflect endothelial damage in these pediatric patients.

Abnormal Nailfold Capillaries in Patients after Hand Transplantation.

Background: The development of graft vasculopathy may play a role in the long-term deterioration of hand grafts. The aim of study was to examine the patterns of the nailfold capillaries in hand transplant recipients. Methods: the study was performed on six patients who received hand transplantation. To normalize for the effect of immunosuppression an age- and sex-matched group of 12 patients with active kidney transplant was selected. As an additional control group, 12 healthy volunteers were recruited. Nailfold videocapillaroscopy was performed in all participants. Additionally, serum concentrations of vascular endothelial growth factor (VEGF) were measured. Results: Videocapillaroscopic examination of the hand allografts revealed significant abnormalities: including capillary disorganization and microhaemorrhages. The number of capillaries was reduced, the vessels were enlarged and branched. Surprisingly, similar, albeit slightly less pronounced, changes were seen in the nailfolds of healthy hands of the limb transplant recipients. In kidney transplant recipients the capillaroscopic pattern was general normal and comparable to healthy individuals. Moreover, serum concentrations of VEGF in all participants correlated with average capillary diameter in capillaroscopy. Conclusions: in hand transplant recipients advanced microvascular abnormalities are found in nailfold capillaroscopic pattern in both transplanted and own extremities connected with elevated levels of VEGF.

This issue of ACTA, printed and electronic

This issue of ACTA, printed and electronic

Association of Nailfold Capillary Abnormalities With Primary Open-angle Glaucoma and Glaucomatous Visual Field Loss.

Nailfold capillary abnormalities are associated with primary open-angle glaucoma (POAG) and increased severity of global and central glaucomatous visual field (VF) loss. The purpose of this study was to investigate whether nailfold capillary abnormalities are associated with POAG and the severity of glaucomatous VF loss. A cross-sectional study of 83 POAG cases and 40 controls was conducted. Nailfold capillaroscopy images were assessed by masked graders for dilated capillaries >50 μm, crossed capillaries, tortuous capillaries, hemorrhages, avascular zones >100 μm, capillary density, and capillary distribution. VF loss in glaucoma cases was quantified using mean deviation and mean central pattern standard deviation (PSD) from the worst-affected eye. Logistic regression analyses of cases and controls showed that avascular zones [odds ratio (OR)=1.24; 95% confidence interval (CI): 1.06, 1.47; P=0.005], capillary density (OR=0.63; 95% CI: 0.46, 0.83; P<0.001), and capillary distribution (OR=7.88; 95% 95% CI: 2.53, 28.40; P=0.001) were associated with POAG. Simple linear regression analysis of cases only showed that nailfold hemorrhages were associated with mean deviation (β=-5.10; 95% CI: -9.20, -1.01; P=0.015) and mean central PSD (β=-4.37; 95% CI: -8.18, -0.57; P=0.025), and this remained significant in the multiple linear regressions. After controlling for demographic and clinical factors, avascular zones were associated with both mean deviation (β=-0.76; 95% CI: -1.48, -0.04; P=0.040) and mean central PSD (β=-0.78; 95% CI: -1.45, -0.10; P=0.024), whereas capillary distribution was only associated with mean deviation (β=-4.67; 95% CI: -7.92, -1.43; P=0.017). Nailfold capillary abnormalities are associated with POAG as well as increased global and central vision loss.

Nail Fold Capillaroscopy Abnormality in Behcet's Disease and Relation to Disease Activity among Egyptian Patients

Background: Behcet’s disease is a systemic autoimmune disease. Treatment mainly is immunosuppression. It has no sure diagnostic tests and relies mainly on clinical diagnosis. There are few scores to assess Behcet's disease activity (Behcet's Disease Current Activity Form) BDCAF score. Nailfold Capillaroscopy is a technique to visualize capillaries in nailfold area. It can correlate many diseases with the abnormalities in shape of blood capillaries. Objectives: The aim of the work was to find out Nail Fold Capillaroscopy abnormality in Behcet's Disease and its relation to the disease activity. Patients and Methods: A cross sectional-case control study which was done on 50 Behcet’s patients and 30 healthy controls. We assessed nailfold capillaroscopic changes in Behcet’s patients in comparison to controls and the correlation of BDCAF score with nailfold capillary abnormalities. Results: we found that 70% of Behcet's Disease patients had nailfold capillaroscopy abnormalities , a highly statistically significant changes in the capillaries in the form of capillary dilatation (24%), hemorrhage (54%), tortuosity (64%) in comparison to the control group (P<0.001). However, pattern was not specific as BDCAF Score wasn’t related to the capillary changes. Conclusion: Nailfold capillaroscopy showed nonspecific pattern in 70% Behcet's disease patients. Tortuosity, hemorrhages, dilation were not related to disease activity. Capillaroscopy is a good primary test for Bechet’s disease and can reflect the presence and extent of microvascular involvement and thereby might have diagnostic and prognostic value.