Nailfold Capillaroscopy Pattern Research Articles

Relationship between nailfold capillaroscopy findings and the etiology and prognosis of interstitial lung disease

ObjectivesConnective tissue-associated interstitial lung diseases (CTD-ILD) are believed to be caused by microvascular damage. The objective of this study was to assess the nailfold capillaroscopy (NFC) pattern in patients diagnosed with both CTD-ILD and non-CTD-ILD to identify microvascular changes and determine the relation between capillaroscopic parameters, clinical variables, and disease-related measurements.Patients and methodsThis cross-sectional study included 95 patients with interstitial lung disease who applied to our Rheumatology and Chest Clinics between September 2021 and July 2023. The patients were divided into two groups based on their diagnosis: non-CTD-ILD (group 1) and CTD-ILD (group 2). Nailfold capillaroscopy was performed.ResultsNinety-five patients, 49 (51% female, mean age 62.31 ± 11.027 years) in group 1 and 46 (69.6% female, mean age 62.09 ± 10.887 years) in group 2, were included in the study. Abnormal capillary morphologies were both detected in the CTD-ILD group and the non-CTD-ILD groups. In patients with a usual interstitial pneumonia (UIP) pattern on chest computed tomography (CT), tortuosity was higher than in patients with non-specific interstitial pneumonia (NSIP) (P = 0.041), and the proportion of tortuosity increased significantly as the duration of the disease increased (P = 0.016).ConclusionOur study highlights capillaroscopic abnormalities alone may not be sufficient to differentiate CTD-ILD (other than systemic sclerosis) from non-CTD-ILD. The presence of NFC abnormalities in non-CTD-ILD may suggest that fibrotic lung disease could potentially play a role in the deterioration of the microvascular structure or abnormal angiogenesis. Our study demonstrated that a multidisciplinary approach, incorporating clinical, morphological, pathological, and serological evaluations, is necessary for interpreting ILD.Key Points• Capillaroscopic abnormalities can also be seen in non-CTD-ILD.• Capillaroscopy findings do not distinguish the non-Ssc etiology of ILD.• Nailfold capillaroscopy may have the potential to serve as a useful tool in predicting prognosis and monitoring the disease progression in patients with idiopathic pulmonary fibrosis (IPF).

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.

Microvascular damage evaluation based on nailfold videocapillarosopy in sarcoidosis.

Microvascular damage is thought to play a role in the pathogenesis of sarcoidosis. We aimed to evaluate the nailfold capillaroscopy (NVC) pattern to detect microvascular changes in patients with sarcoidosis and the relationship of capillaroscopic parameters with clinical variables and disease-related measurements. Forty-two patients with sarcoidosis and 42 age- and sex-matched patients with systemic sclerosis (SSc) and healthy individuals were included in this cross-sectional case-control study. Patients aged 18-80years who met the current American Thoracic Society criteria for sarcoidosis were included. NVC was performed by a digital microscope under a magnification of × 200. Capillary density, number of dilated, giant and neoangiogenic capillaries, capillary loop diameter, capillary shape, micro-hemorrhages, and number of avascular areas, were evaluated by an assessor who was blind to the groups allocation. Among the capillaroscopic parameters, neoangiogenesis and dilated capillaries, which can be seen in the pattern of scleroderma, were detected in patients with sarcoidosis but not significantly different from the control group (p = 0.055; p = 0.433, respectively). The rate of tortuosity and crossing capillaries of 50% and above was significantly higher in the sarcoidosis group than in SSc and healthy controls (p < 0.05). In patients with sarcoidosis, the only statistically significant finding was; forced expiratory volume (FEV1) in one second was lower in patients with a crossed capillary rate > 50% than in patients with a crossed capillary rate of less than 50% (FEV1; 87.21 ± 19.3, 102.5 ± 14.8, p = 0.04; respectively). Patients with a diagnosis of sarcoidosis have different capillaroscopic patterns. The presence of these nonspecific abnormal patterns may reflect microvascular damage in the pathophysiology of sarcoidosis. Key Points • Microvascular damage may play a role in the pathogenesis of sarcoidosis. • There may be some nonspecific abnormal findings in capillaroscopy findings in sarcoidosis. • Capillaroscopy may be valuable in reflecting sarcoidosis lung injury.

Emerging nailfold capillaroscopic patterns in COVID-19: from acute patients to survivors.

The SARS-CoV-2 infection causing the Coronavirus disease-19 (COVID-19) is characterized by a broad range of clinical manifestations, implicating microvascular damage with endothelial dysfunction and different organ involvement.

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.

Aşikâr pulmoner hipertansiyonu olmayan sistemik sklerozlu hastalarda tırnak kapilaroskopi bulguları ve modifiye Rodnan skoru sağ ventrikül fonksiyonlarında bozulma ile ilişkilidir

Objective: Although nailfold capillaroscopy (NC) and modified Rodnan skin score (mRSS) have already been studied in a variety of contexts related to Systemic Sclerosis (SSc) progression, there is limited data about the relationships between NC, mRSS, and right ventricle (RV) function in SSc patients without overt pulmonary arterial hypertension (PAH). In this study, we examined the relationship between RV function and clinical SSc parameters such as NC pattern and mRSS. &#x0D; Material and Method: Thirty two patients with SSc and twenty healthy participants as a control group were enrolled in this study. Patients with SSc were assessed for digital ulcers, Raynaud’s phenomenon, and severity of skin involvement by a rheumatology specialist. Also, all participants underwent echocardiographic examinations by cardiology specialists. The echo parameters were measured considering the criteria of the American Society of Echocardiography guidelines. &#x0D; Results: Systolic pulmonary arterial pressure (sPAP) was statistically higher in the SSc group (26.4±3.2 vs 30.8±3.6 mmHg, p

Ultrasonographic detection of enthesitis and its relation to clinical manifestations among Egyptian systemic sclerosis patients

BackgroundSystemic sclerosis is a complex multi-systemic autoimmune disease with a wide range of its clinical manifestations; many systemic sclerosis (SSc) patients develop musculoskeletal manifestations during their course of illness. The aim of the study is to assess the prevalence of sonographically detected entheseal alterations in a case-control study of systemic sclerosis patients and to evaluate the relationship between the presence of these alterations and the clinical systemic manifestations. Patients and controls were evaluated using B mode and power Doppler ultrasonography to detect presence of enthesitis and were scored using Madrid Sonography Enthesitis Index (MASEI).ResultsIn SSc patients, the MASEI score was significantly higher than in control (P < 0.0001). Enthesitis was more prevalent among SSc patients compared to healthy controls, SSc patients with enthesitis had significantly more prevalence of diffuse subtypes (P < 0.001).SSc patients had significantly more prevalence of interstitial pulmonary fibrosis (IPF) (P < 0.001), digital ulcers (P < 0.001), pulmonary hypertension (P < 0.001), and arthralgia and arthritis (P < 0.001). Regarding nailfold capillaroscopy pattern, late pattern was significantly more prevalent among patients with enthesitis (P = 0.008). Age, ESR level, and modified Rodnan skin score were predictors for MASEI score.ConclusionsUltrasound features of enthesopathy were frequently presented in systemic sclerosis patients. The enthesopathy was correlated with inflammation and disease complications.

Evaluation of Nailfold Capillaroscopy Pattern in patients with Primary Osteoarthritis of the Hands

Background: Hand osteoarthritis (OA) can be a distressing disorder that impairs a patients' quality of life. There is a relation between OA and vascular damage, especially atherosclerosis, and OA are both common chronic conditions associated with aging and obesity. Aim of the study: The objective of this research was to examine nailfold microcirculation in individuals with hand OA Patients and Methods: a cross-sectional, observational study, about 56 patients with hand OA were enrolled. All subjects underwent a standard radiology X-Ray examination of the hands and a Nailfold vascular examination was performed. Results: of the 56 patients studied were females, and 12 were males. Regarding the Kellgren and Lawrence scoring, it was mild (grade II) in one patient (50%) and severe (grade IV) in one patient (50%) in the male patients without pain, while in those with hand pain, it was moderate (grade III) in eight patients (80%) and severe (grade IV) in two patients (20%).it was mild (grade II) in eight patients (22.2%), moderate (grade III) in 21 patients (58.3%) and severe (grade IV) in seven patients (19.4%). Nailfold examination for studied subjects showed that there were about 41 patients (73.2%) with low capillary density and 32 patients (57.1%) with morphological changes. Conclusion: Our findings provided a general description of the NFC findings most likely to be seen in OA patients; Vascular anomalies may play a role in the illness progression and may assist in elucidating because the condition is frequently found in the distal digit joints.

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.

Relation between nailfold capillaroscopic pattern (assessed using a dermoscope) and organ involvement in systemic sclerosis

Objectives: The objectives of the study were: (1) To document the nailfold capillary changes (using a dermoscope) in patients with systemic sclerosis attending a tertiary care center, (2) to study the relation between nailfold capillaroscopic pattern and skin sclerosis assessed by modified Rodnan skin score (mRSS), and (3) to study the relation between nailfold capillaroscopic pattern and organ involvement in systemic sclerosis. Materials and Methods: A cross-sectional study was conducted among 40 patients with systemic sclerosis who attended the dermatology outpatient department of a tertiary care center from January 1, 2018, to December 31, 2018. Nailfold capillaries were examined with the help of dinolite dermoscope AM4113ZT at 50× and 200× magnification, under polarized light. Results: Study participants included 34 (85%) females and 6 males (15%). The nailfold capillaroscopy showed “early scleroderma pattern” in 3 (7.5%) “active pattern” in 28 (70%) and “late pattern” in 9 (22.5%) patients. “Late scleroderma pattern” showed a significant association with disease duration, mRSS, and mean number of organs affected. Limitations: The study participants may be over-representing advanced cases since the study was conducted among patients attending a tertiary referral center. Conclusion: We found dermoscope to be a useful tool to study the nailfold capillary changes in patients with systemic sclerosis as reported by others. Late scleroderma pattern may serve as an indicator of high mRSS and involvement of more number of organs in systemic sclerosis.

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A randomised, open-label trial to assess the optimal treatment strategy in early diffuse cutaneous systemic sclerosis: the UPSIDE study protocol

IntroductionSystemic sclerosis (SSc) is a chronic, autoimmune connective tissue disease associated with high morbidity and mortality, especially in diffuse cutaneous SSc (dcSSc). Currently, there are several treatments available in early...

The evaluation of nailfold capillaroscopy pattern in patients with fibromyalgia.

Objectives This study aims to evaluate nailfold capillaroscopic pattern in patents with fibromyalgia and to assess the relation of capillaroscopic parameters with clinical variables and disease-related measures.Patients and methods This cross-sectional, case-control study included 60 participants (4 males, 56 females; mean age: 44.0±8.2 years; range, 26 to 64 years) between August 2019 and November 2019. All participants were divided into two groups as the primary fibromyalgia group (n=30) who met the 2016 modified American College of Rheumatology Diagnostic Criteria for Fibromyalgia and the control group (n=30) consisting of age- and sex-matched healthy individuals. Nailfold capillaroscopy was performed by a digital microscope under a magnification of 200X. Capillary density, capillary loop diameter, number of dilated, giant and neoangiogenic capillaries, capillary shape, number of avascular areas, micro-aneurysms and micro-hemorrhages were evaluated by an assessor who was blind to the group allocation. In the fibromyalgia group, Widespread Pain Index, Symptom Severity Scale scores, and Fibromyalgia Severity scores were calculated. Health status and presence of benign joint hypermobility syndrome (BJHS) were evaluated using the Fibromyalgia Impact Questionnaire (FIQ) and revised Brighton criteria, respectively.Results Of the capillaroscopic parameters, the mean capillary loop diameter, number of micro-aneurysms, avascular areas, and neoangiogenic capillaries were significantly higher in the patient group compared to the controls (p<0.001, p=0.016, p=0.038, and p=0.04, respectively). Nailfold capillaroscopic findings did not significantly differ between the patients with (n=16) and without concomitant BJHS (n=14). Of the disease-related measures, only FIQ score showed a weak correlation with the number of dilated capillaries (p=0.324).Conclusion Patients with fibromyalgia have distinct capillaroscopic patterns than healthy population. Capillaroscopic features, in general, are not related to clinical variables and disease-related measures.

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.

Progression of nailfold capillaroscopic patterns and correlation with organ involvement in systemic sclerosis: a 12 year study.

Objective: The aim of this observational study was to investigate the evolution of scleroderma microangiopathy throughout different nailfold videocapillaroscopy (NVC) patterns ('early', 'active', 'late') as well as the prevalence of organ involvement in SSc patients during a 12-year follow-up. Methods: Thirty-four SSc patients showing at baseline (first capillaroscopic analysis) the 'early' NVC pattern of microangiopathy were enrolled and followed for 12 years (s.d. 2). Complete NVC analysis and clinical and serological findings were collected. Patients were in a standard therapeutic care setting. Statistical analysis was carried out by non-parametric tests. Results: After a 12-year follow-up, the 'early' NVC pattern changed from baseline in 76% of the patients. The NVC pattern was found to be 'active' in 9 patients (26%), 'late' in 13 (38%) and characterized by non-specific capillary abnormalities in 4 (12%). In the subgroup whose microangiopathy progressed from the 'early' to the 'late' NVC pattern, the median time of progression from the 'early' to the 'active' pattern was significantly shorter (11 months) when compared with patients who progressed from the 'early' to the 'active' NVC pattern (55 months) (P = 0.002). The median time of progression between NVC patterns was significantly shorter in SSc patients showing either a nucleolar ANA pattern or Scl70 autoantibodies (P = 0.048). Organ involvement was progressively greater in SSc patients with 'early', 'active' and 'late' NVC patterns, respectively. Conclusions: This longitudinal study confirms over a 12-year follow-up the evolution of specific NVC patterns associated with the progressive severity of organ involvement in SSc patients in a standard clinical care setting.

Pattern of Nailfold Capillaroscopy in Patients With Systemic Lupus Erythematosus.

Objectives This study aims to assess the nailfold capillary changes in patients with systemic lupus erythematosus (SLE), particularly among those with Raynaud’s phenomenon (RP), and the correlation between nailfold capillary changes and autoantibodies and disease activity.Patients and methods A total of 85 patients (9 males, 76 females; median age 31 years; range, 15 to 58 years) with newly diagnosed SLE were selected between July 2016 and July 2018 from our hospital. Disease activity was scored by the SLE Disease Activity Index. Nailfold capillaroscopy (NFC) was performed in all patients.Results Normal pattern, non-specific pattern, and scleroderma pattern were found in 13 (15.3%), 64 (75.3%), and eight (9.4%) patients, respectively. There was no significant difference between anti-double stranded deoxyribonucleic acid, anti-Smith antibodies, and low complements (all p>0.05), while significant differences of NFC pattern were found between low disease activity and high disease activity (p=0.002). RP was present in 31.7% of SLE patients, and the NFC findings in SLE patients with and without RP were significantly different in dilatation (81.5% vs. 14.0%).Conclusion The results of our study showed that capillary changes were very common in patients with SLE, which seem to associate with disease activity and RP condition.

Microangiopathy and forearm arterial blood flow in systemic sclerosis: a controlled study.

The aim of the study was to evaluate the interrelationship between the micro- and macrovasculature. This is a cross-sectional study that examined SSc patients and fibromyalgia (FM) patients as controls. We assessed forearm peripheral vascular status and nailfold capillaroscopy. We evaluated the association between nailfold capillaroscopy pattern of microvasculopathy reflected as microangiopathy evolution score and macrovascular changes in the forearm vessels examined by color Doppler ultrasound. We assessed relevant clinical and laboratory data, as well as intima-media thickness (IMT) and internal diameter (ID) in the radial and ulnararteries in millimeters, and calculated the ratio IMT\ID peak systolic velocity and end-diastolic velocity were used for the calculation of the resistance index. We examined 73 patients: 50 patients with SSc and 23 patients with FM. Ten patients with SSc had arterial occlusions compared to 1 among FM patients (p = 0.082). The SSc group had a statistically significantly higher mean IMT to ID ratio (p < 0.001). There was no correlation between microangiopathy evolution score for both hands, RI, or mean IMT/ID ratio. Total microangiopathy evolution score was not associated with arterial occlusions. Our study demonstrated a high prevalence of macrovascular disease in SSc; no correlation was found between microvasculopathy and macrovascular disease, suggesting that different pathogenic mechanisms might operate in different vessels size. Key Points • This study demonstrated a high prevalence of macrovascular arterial forearm disease in systemic sclerosis patients. • This study found no correlation between capillaroscopic microangiopathy evolution score (MES) and macrovascular abnormalities. • Our findings suggest that different pathogenic mechanisms might operate in different vessels size.

Progression of nailfold capillaroscopic patterns and correlation with organ involvement in systemic sclerosis: a 12 year study.

The aim of this observational study was to investigate the evolution of scleroderma microangiopathy throughout different nailfold videocapillaroscopy (NVC) patterns ('early', 'active', 'late') as well as the prevalence of organ involvement in SSc patients during a 12-year follow-up. Thirty-four SSc patients showing at baseline (first capillaroscopic analysis) the 'early' NVC pattern of microangiopathy were enrolled and followed for 12 years (s.d. 2). Complete NVC analysis and clinical and serological findings were collected. Patients were in a standard therapeutic care setting. Statistical analysis was carried out by non-parametric tests. After a 12-year follow-up, the 'early' NVC pattern changed from baseline in 76% of the patients. The NVC pattern was found to be 'active' in 9 patients (26%), 'late' in 13 (38%) and characterized by non-specific capillary abnormalities in 4 (12%). In the subgroup whose microangiopathy progressed from the 'early' to the 'late' NVC pattern, the median time of progression from the 'early' to the 'active' pattern was significantly shorter (11 months) when compared with patients who progressed from the 'early' to the 'active' NVC pattern (55 months) (P = 0.002). The median time of progression between NVC patterns was significantly shorter in SSc patients showing either a nucleolar ANA pattern or Scl70 autoantibodies (P = 0.048). Organ involvement was progressively greater in SSc patients with 'early', 'active' and 'late' NVC patterns, respectively. This longitudinal study confirms over a 12-year follow-up the evolution of specific NVC patterns associated with the progressive severity of organ involvement in SSc patients in a standard clinical care setting.