Capillaroscopic pattern in inflammatory arthritis

BackgroundThere are limited data about the role of nailfold capillaroscopy in inflammatory arthritis.ObjectivesTo study the role of capillaroscopy in inflammatory arthritis — rheumatoid arthritis (RA), psoriatic arthritis (PsA) and early arthritis.MethodsPatients from the following groups were included in the study: 62 patients with RA; 34 patients with PsA with involvement of the joints of the hands; 9 women with early arthritis. Nailfold capillaroscopy was performed with videocapillaroscope.ResultsRaynaud's phenomenon (RP) was found in 30.6% (19/62) of RA patients, in 32.4% (11/34) of PsA patients and 44.4%, (4/9) of cases with early arthritis. The most frequent found capillaroscopic changes in RA patients were presence of elongated capillaries in 58% of cases (36/62) and prominent subpapillary plexus in 69% (43/62). Dilated capillaries were found in 78.9% (15/19) of patients with secondary RP and in 62.8% (27/43) of those without RP. “Scleroderma-like” capillaroscopic pattern was observed with low frequency in RA patients (14.5%/9/62). “Scleroderma-like” capillaroscopic pattern was also found in 11.1% (1/9) in the group of patients with early arthritis. The low frequency of the last type of capillaroscopic pattern in RA requires patients with such changes to be observed during regular follow-up for the development of systemic rheumatic disease different from inflammatory arthritis. In patients with PsA capillaries with specific morphology (tight terminal convolutions) were found in 58.8% (20/34) of cases.ConclusionsResults from the present study confirm the necessity for inclusion of the nailfold capillaroscopy in the diagnostic algorithm in patients with inflammatory arthritis.     

Nailfold capillaroscopy by digital microscope in an Indian population with systemic sclerosis

Aim: Nailfold capillaroscopy (NFC) is a simple, non‐invasive method with exceptional predictive value for the analysis of microvascular abnormalities, especially in systemic sclerosis (SSc) but remains underutilized due to cost factors of the nailfold videocapillaroscope, lack of expertise and availability issues. The aim of this study was to establish the utility of an inexpensive digital microscope to study NFC changes in SSc in correlation with disease subsets and extent of skin involvement. Methods: Twenty‐two diffuse cutaneous SSc (DSS), 20 limited cutaneous SSc (LSS) patients and 42 controls were evaluated with NFC using a digital microscope at 30× and 100× magnification. Digital micrographs were used to study qualitative and quantitative changes in microvasculature. Results: The capillary density was significantly less in all cases of SSc as compared to controls (5.3 ± 1.4 vs. 8.7 ± 1.2; P < 0.00001). Disorganized architecture was much more prevalent in DSS versus LSS (86.4%vs. 25%). The vascular deletion score (VDS) was significantly higher in DSS as compared to LSS (P < 0.0001). Scleroderma pattern (SP) was seen in 18 (81.9%) and 15 (75%) of patients with DSS and LSS, respectively. Only 4% of normal subjects showed non‐specific pattern and none showed SP. The mean modified Rodnan skin score (MRSS) was positively correlated with vascular deletion score (r = 0.572; P < 0.001) and negatively with capillary density (r = ?0.8; P < 0.001). Conclusion: Nailfold capillaroscopy changes in SSc are related to disease subset and MRSS. NFC with digital microscope is a simplified, inexpensive, outpatient procedure with results comparable to previous studies.     

Nailfold capillary microscopy in healthy children and in childhood rheumatic diseases: a prospective single blind observational study

OBJECTIVES: To develop an objective method of nailfold capillaroscopy (NFC), applicable to a wide age range of paediatric patients. To compare the morphological characteristics of the nailfold capillaries in different rheumatology patient groups and controls. METHODS: A colour digital video camera attached to a stereomicroscope was used to capture nailfold capillary images. Computerised image processing was used to analyse and store data. Subsequent quantitative and qualitative morphological analysis was performed in the following paediatric patient and control groups: 18 children with connective tissue diseases (CTD: juvenile dermatomyositis, systemic sclerosis, and undifferentiated connective tissue disease), eight with systemic lupus erythematosus, nine with primary Raynaud's disease, three with primary vasculitis, 15 with juvenile idiopathic arthritis, 17 healthy children and 20 healthy adults. Images were analysed by a single assessor who was unaware of the patient details. RESULTS: The NFC technique was simple to perform and gave reproducible results, although some intra- and intersubject variation was noted. Capillary density and width was age related, with younger children having fewer and wider capillaries than older children and adults. Linear capillary density was significantly higher in healthy adults (mean (SD) 8.6 (1.6) capillaries/mm) compared with healthy children (HC 6.9 (0.9) capillaries/mm). The group with CTD had the most abnormal findings, with lower linear density (4.9 (1.7) capillaries/mm) and increased capillary loop width (10.7 (7.3) mm) compared with HC (3.5 (1.7) mm). In addition, 11/18 (61%) patients in the CTD group had more than two definitely abnormal capillaries in at least two nailfolds, an abnormality not seen in other subjects. Two qualitative measures, the degree of avascularity and general disarrangement of capillary pattern, were more commonly observed in the CTD group than in HC. The proportion of tortuous capillaries did not differ significantly between study groups. CONCLUSIONS: This study is unique in measuring objective quantitative and qualitative parameters of the nailfold vasculature across a wide spectrum of age and disease. Differences in capillary morphology and frequency in children with CTD compared with other paediatric diseases and healthy controls were demonstrated. In the clinical situation, an assessment of the general degree of disarrangement may offer a fast tool for assessment of the nailfold vasculature which correlates well with NFC data.     

Clinical utility of nailfold capillaroscopy

Nailfold capillaroscopy (NFC) is a simple noninvasive microscopic technique used to identify characteristic morphological abnormalities in the nailfold capillaries. The presence of this microvasculopathy appears to be of fundamental importance in the pathological processes that underlie the scleroderma spectrum disorders (including dermatomyositis and antisynthetase myositis). This review discusses the different methodologies and techniques in performing NFC and stresses the diagnostic utility achieved with simple ‘bedside’ techniques utilising the ophthalmoscope, dermatoscope or smart phone. Recent advances in reporting abnormal microvascular patterns and vascular metrics (e.g. capillary density and dropout) are discussed. The aetiopathogenesis of the microvasculopathy is currently unknown but its close association with Raynaud Phenomena and specific autoantibodies together with recent observations from sequential NFC allows speculations on its possible mechanism. Finally, future developments in the use of NFC as a possible biomarker in the management of the scleroderma spectrum disorders are discussed, with a recommendation that NFC becomes more widely available, particularly in rheumatological, immunological and dermatological practice. NFC provides a clinically accessible window on the pathologic process fundamental to scleroderma-related disease.     

The capillaroscopic findings in idiopathic pernio: is it a microvascular disease?

Objectives

Pernio is a disorder that affects the unprotected skin regions of individuals who are exposed to nonfreezing, damp cold. We aimed to examine nailfold capillaries by video capillaroscopy and evaluate the vascular involvement in patients with idiopathic pernio.

Methods

Fifty-three patients with idiopathic pernio (male/female ratio 35:18, mean age 25 ± 9 years) and 38 age- and sex-matched healthy volunteers (male/female ratio 30:8, mean age 24 ± 4 years) were included in the study. Forty-seven of the 53 patients and all the healthy volunteers were evaluated by nailfold video capillaroscopy.

Results

In the patient group, the mean capillary diameter and the mean apical capillary diameter were 56 ± 15 and 24 ± 7 μm, respectively. In the control group, the mean capillary diameter and the mean apical capillary diameter were 37 ± 8 and 15 ± 4 μm, respectively (both p < 0.001). Both of these differences were independent of the disease activity, smoking, and the number of pernio episodes. There were no architectural derangements, avascular areas, or hemorrhages.

Conclusions

In the present study, increased nailfold capillary diameter and increased apical capillary diameter were found in patients with pernio regardless of the disease activity. These findings suggest organic damage of the microcirculation.     

Evaluation of red blood cell distribution width in patients with psoriatic arthritis

Aim of the workTo evaluate the red blood cell distribution width (RDW) values in psoriatic arthritis (PsA) patients and to study the relationship between these values and disease activation.Patients and methodsForty seven patients with PsA and 56 age- and sex matched healthy controls were included in this study. Laboratory test results of both groups were retrospectively collected from medical records; these included levels for white blood cell count, hemoglobin, platelet, RDW, erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). Disease activity score (DAS28) was used to evaluate disease activity.ResultsThe study comprised 47 PsA patients (32 females and 15 males) (F:M 2.1:1) with a mean age of 39.2 ± 9.9 (20–54) years and mean disease duration was 3.3 ± 1.94 (1–8) years. 39 (83%) patients were receiving monotherapy, 8 (17%) were receiving combined therapy. The RDW values were significantly higher when comparing active disease period (16 ± 3.9) of PsA patients versus inactive disease period (14.2 ± 1.04) and controls (14.03 ± 1.2) (p < 0.001). Otherwise, no significant differences were found when comparing inactive disease period of PsA patients versus controls (p = 0.18). RDW values of active disease period of PsA patients significantly correlated with ESR (r = 0.57, p < 0.001), CRP (r = 0.4, p = 0.006) and DAS28 (r = 0.42, p = 0.003).ConclusionsIncreased RDW is associated with active disease period of PsA patients. RDW seems to be a surrogate marker of the inflammation, like CRP and ESR. It is included in the complete blood count thus its measurement does not need any additional costs. RDW may be a potential marker to evaluate disease activity of PsA.     

Microvascular abnormalities in Sj?gren's syndrome: nailfold capillaroscopy.

OBJECTIVE: To describe microvascular abnormalities by nailfold capillaroscopy in patients with primary Sj?gren's syndrome (SS) with or without Raynaud's phenomenon (RP) and those with anticentromere antibodies (ACA). METHODS: Forty patients with SS (14 without RP, 16 with RP, 10 with ACA), 20 patients with scleroderma (SSc) (10 with limited and 10 with diffuse disease) (disease control group) and 40 healthy controls (control group) were evaluated by nailfold capillaroscopy. RESULTS: Capillaroscopic abnormalities in SS ranged from non-specific findings (crossed capillaries) to more specific findings (confluent haemorrhages and pericapillary haemorrhages) or scleroderma-type findings. SS patients with RP presented capillary abnormalities in higher frequency than patients without RP. The majority of SS patients with ACA (80%) presented scleroderma-type findings. CONCLUSION: Nailfold capillaroscopy can be used as a simple non-invasive method to evaluate the microvascular abnormalities in SS patients, especially in those with RP and those with ACA.     

Labial capillary microscopy in systemic sclerosis.

OBJECTIVES--To investigate whether in vivo capillary microscopy of the lower lip mucosa can be used to assess microvascular disease in systemic sclerosis. METHODS--Thirteen patients with systemic sclerosis and 11 healthy control subjects were studied by conventional nailfold capillary microscopy and labial capillaroscopy. The following parameters were analysed: loop length; loop width (maximum distance between the arteriolar and venular limbs); loop density (number of capillaries/mm2); venular plexus visibility; megacapillaries; and the architectural arrangement of the capillary network. RESULTS--A typical 'scleroderma pattern' at the nailfold was observed in 12 of 13 (92%) patients with systemic sclerosis. Labial capillaroscopy showed a different morphological pattern of microangiopathy. A diffuse architectural derangement of the capillary network was the most striking abnormality in 12 (92%) patients. Labial capillaries in the patients with systemic sclerosis were shorter (mean (SD) loop length 133 (32.2) microns) than in healthy controls (211 (48.4) microns) and showed an increased loop width (41.7 (13.1) v 27.6 (5.5) microns in controls. The loop density was 10.5 (4.6) capillaries/mm2 in patients with systemic sclerosis and 9 (1.7) capillaries/mm2 in controls. Labial capillaroscopy in patients with systemic sclerosis did not provide definite evidence of enlarged capillaries or avascular areas, or both, even where such abnormalities were clearly evident at the nailfold. CONCLUSIONS--This study shows that labial capillary microscopy is a simple, non-invasive technique which allows a careful morphological assessment of the mucosal microcirculation. Labial capillaroscopy in patients with systemic sclerosis showed significant microvascular changes with respect to the controls. The results of labial and nailfold capillaroscopy are not superimposable, even if some common findings, such as architectural derangement, are present.     

Morphologic study of microcirculation in acromegaly by capillaroscopy.

Although wide range investigations on the heart and great vessels have been reported in acromegaly, the field of microcirculation is still largely vacant. The nailfold is a window through which we can observe in vivo the vascular bed. This study investigates through nailfold capillaroscopy the morphology of cutaneous microcirculation in acromegaly in relationship with the usual hormonal parameters of disease activity. Twenty-five acromegalic patients and 26 normal subjects, age and sex matched, were studied. A subgroup of acromegalics (8 patients) was considered in stable remission, and the remaining 17 had active disease. Capillaroscopy was performed in each subject by in vivo computer aided stereomicroscopy (magnification, x400). The following morphological parameters were calculated: the number of tortuous loops, meandering capillaries, and capillaries per millimeter; avascular areas; visibility of subpapillary plexus; the capillary length; and intercapillary distance. We were unable to perform the exam in 4 of 25 patients because visibility was poor. The capillary number and length were significantly reduced in acromegalics compared to controls [8.9 +/- 1.5 vs. 10.3 +/- 1.2 no./mm (P = 0.0010) and 174 +/- 49 vs. 255 +/- 24 microm (P < 0.0001)]. Moreover, in acromegalics, the numbers of tortuous loops and meandering capillaries were significantly increased [19 +/- 8 vs. 13 +/- 5 (P = 0.0027) and 10 +/- 12 vs. 0.7 +/- 1.1 (P < 0.0001)]. The capillaroscopic alterations were still observed in a smaller group of 8 nondiabetic and nonhypertensive acromegalics. We found branch-like capillaries in 4 acromegalic patients, but not in the control group. Finally, we observed a meaningful different and ameliorated capillaroscopic morphology in acromegalic patients in stable remission compared to active disease patients as far as the total number (density) and meandering capillaries were concerned. In conclusion, our study shows that in acromegaly, morphological alterations also affect the peripheral microcirculation, which seems to be influenced by the activity of the disease. We believe that nailfold capillaroscopy may represent an additional useful tool in the follow-up of acromegalic patients.     

Decreased nailfold capillary density in Raynaud's phenomenon: a reflection of immunologically mediated local and systemic vascular disease?

Nailfold capillary patterns were studied in 107 patients with Raynaud's phenomenon (RP), including patients wih (n = 39) and without (n = 68) connective tissue disease (CTD). Capillary density was decreased in patients with sclerodactyly, digital ulcers, tuft resorption, and telangiectasia, compared with patients without these symptoms. In addition, an inverse relationship was found between the severity of RP at first presentation (as graded by photoelectric plethysmography during cooling) and the capillary density in patients with CTD (r = -0.45; p less than 0.05). In the total group of patients nailfold capillary density was inversely related to organ system involvement (r = -0.52; p less than 0.01). Decreased nailfold capillary density was observed, in particular, in patients with oesophageal hypomotility and in patients with chest x-rays compatible with interstitial fibrosis. As to factors supposedly involved in the pathogenesis of vascular changes in CTD, the presence of autoantibodies, increased levels of circulating immune complexes, and increased levels of acute phase reactants were all associated with a decreased number of nailfold capillaries. We conclude that loss of nailfold capillaries as observed by microscopy is a reflection of local and systemic vascular disease.     

Kapillarmikroskopie und rheumatische Erkrankungen: State of the art

Nailfold capillaroscopy (NVC) represents the best method for analyzing microvascular abnormalities in rheumatic diseases. Raynaud’s phenomenon (RP) represents the most frequent clinical aspect of microvascular involvement and is a key feature of several such diseases. Under normal conditions or in primary RP (exclusion by the cold-exposure test), the normal nailfold capillaroscopic pattern shows a regular disposition of the capillary loops within the nail bed. However, in subjects suffering from secondary RP, one or more alterations in the capillaroscopic findings should alert the physician to search for an underlying connective tissue disease. Architectural disorganization, giant capillaries, hemorrhages, loss of capillaries and avascular areas characterize more than 95% of patients with overt systemic sclerosis (sclerodema, SSc). Therefore, the term “scleroderma pattern”, includes all capillaroscopic changes typical of the microvascular involvement in SSc. The capillaroscopic aspects observed in dermatomyositis and in undifferentiated connective tissue disease are generally reported as “scleroderma-like patterns”. This peripheral microangiopathy can be effectively detected early in the course of the disease and studied in detail by nailfold capillaroscopy or, better, with NVC. In addition, early differential diagnosis between primary and secondary RP is the greatest advantage NVC has to offer. In addition, interesting capillaroscopic changes have been observed in systemic lupus erythematosus, antiphospholipid syndrome and Sjögren’s syndrome. However, further epidemiological and clinical studies are needed to better standardize NVC patterns.     

Assessment of Microvascular Abnormalities by Nailfold Capillaroscopy in Juvenile Dermatomyositis After Medium‐ to Long‐Term Followup

Objective

In juvenile dermatomyositis (DM), microvascular abnormalities, measured by nailfold capillaroscopy (NFC), are common early in the disease course. We aimed to compare the presence of NFC abnormalities in patients with medium‐ to long‐term juvenile DM with that of controls, and to explore associations between NFC abnormalities and disease activity and other disease characteristics.

Methods

Fifty‐eight juvenile DM patients with a median disease duration of 16.8 (range 2–38) years were clinically examined and compared with matched controls. By NFC, we assessed nailfold capillary density (NCD), giant capillaries, scleroderma, and neovascular pattern (defined as scleroderma active or late pattern). NFC was analyzed with researchers blinded to patient/control identity and disease characteristics. We measured disease activity and damage by validated tools, and patients were categorized as having active or inactive disease according to the Paediatric Rheumatology International Trials Organisation criteria.

Results

Compared to controls, patients had decreased NCD (mean ± SD 6.4 ± 2.1/mm versus 7.6 ± 0.8/mm; P = 0.001) and showed more abnormality in all other NFC parameters; 36% of patients versus 4% of controls had NCD <6/mm (P < 0.001). Giant capillaries, scleroderma, and neovascular pattern were found in 9%, 84%, and 41% of patients, respectively. Patients with active disease (n = 30) presented more frequently with neovascular pattern than patients with inactive disease (n = 28) (P = 0.041). Decreased NCD and neovascular pattern were associated with higher levels of disease activity and impaired muscle function.

Conclusion

After medium‐ to long‐term followup, juvenile DM patients had decreased NCD and, often, neovascular pattern; both were associated with higher levels of disease activity and impaired muscle function. This suggests that NFC can be a biomarker for disease activity in longstanding juvenile DM too.

     

Synovial fluid levels of anti–cyclic citrullinated peptide antibodies and IgA rheumatoid factor in rheumatoid arthritis,psoriatic arthritis,and osteoarthritis

Objective

To assess the levels of anti–cyclic citrullinated peptide (anti‐CCP) and IgA rheumatoid factor (IgA‐RF) in synovial fluids of patients with rheumatoid arthritis (RA), psoriatic arthritis (PsA), and osteoarthritis (OA).

Methods

Knee effusions of 29 patients with RA (23 women, 6 men; mean ± SD age 60 ± 15 years), 20 with PsA (6 women, 14 men; mean age 51 ± 12 years), and 19 with OA (9 women, 10 men; mean age 73 ± 11.8 years) were aspirated, tested for white blood cell (WBC) counts, centrifuged, and stored at ?20°. Sera of 22, 11, and 12 of these patients with RA, PsA, and OA, respectively, were similarly stored. IgG anti‐CCP and IgA‐RF were detected by enzyme‐linked immunosorbent assay. Erythrocyte sedimentation rate and C‐reactive protein levels were used as measures of disease activity.

Results

Mean levels of synovial fluid anti‐CCP and IgA‐RF were significantly increased in RA joint effusions compared with PsA and OA (anti‐CCP: 150 ± 134, 34 ± 29, and 24 ± 26 units, respectively [P < 0.003]; IgA‐RF: 76 ± 77, 15.7 ± 10, and 18 ± 20 units, respectively). No significant difference was noted between OA and PsA. A significant correlation was found between synovial fluid anti‐CCP and serum anti‐CCP and IgA‐RF. In patients with RA, a significant correlation was found between synovial fluid WBC counts and IgA‐RF (P = 0.03) and serum IgA‐RF (P = 0.008), but not between synovial fluid and serum anti‐CCP levels. In RA patients, C‐reactive protein correlated with serum IgA‐RF.

Conclusion

Anti‐CCP and IgA‐RF were significantly increased in synovial fluid of RA in comparison with PsA and OA patients.

     

Nailfold capillaroscopy in Beh?et’s disease,analysis of 128 patients

The aims of this study were to find the characteristics and prevalence of nailfold capillary changes in a large series of patients with Behçet’s disease (BD) and to analyze their possible relation to other clinical characteristics of the disease. We performed nailfold capillaroscopy in 128 randomly selected patients fulfilling the international classification criteria for BD. Capillaroscopy was done in eight fingers with a ×3.2 microscopy. All patients were questioned for history of Raynaud's phenomenon, ischemic ulcers, smoking, and hypertension. A computerized form including demographic, clinical, and para-clinical features was used to collect data. Univariate and multivariate logistic regressions were used to analyze the relation between capillaroscopic findings and disease characteristics. Odds ratio and a confidence interval at 95% (CI) were calculated for each item. The mean age of the patients was 37?±?10 years, and the male to female ratio was 1.56:1. Capillaroscopy was abnormal in 51 patients (40%, CI 8.5). Enlarged capillaries were seen in 33 patients (26%, CI 7.6), hemorrhages in 21 (16%, CI 6.4), and capillary loss only in one patient. In univariate logistic regression analysis, the presence of enlarged capillaries was associated with lower age at disease onset (OR?=?0.9, CI 0.9–1; p?=?0.04), hypertension (OR?=?4.2, CI 1.5–11.4; p?=?0.006), superficial phlebitis (OR?=?5.5, CI 1.2–24.4; p?=?0.03), and negative pathergy test (OR?=?0.4, CI 0.2–0.9; p?=?0.04). The presence of hemorrhages tended to be associated with articular symptoms (p?=?0.05). Multivariate analysis also confirmed the association of enlarged capillaries with lower age at disease onset (p?=?0.01), hypertension (p?=?0.001), and superficial phlebitis (p?=?0.03). Nailfold abnormalities, mainly enlarged capillaries, are frequent in patients with BD. Our results suggest that these abnormalities may be related to other vascular features of the disease such as superficial phlebitis, but it does not seem to confer special risk for any other specific clinical symptom of the disease.     

Nailfold capillaroscopy is useful for the diagnosis and follow-up of autoimmune rheumatic diseases. A future tool for the analysis of microvascular heart involvement?

Raynaud's phenomenon (RP) represents the most frequent clinical aspect of cardio/microvascular involvement and is a key feature of several autoimmune rheumatic diseases. Moreover, RP is associated in a statistically significant manner with many coronary diseases. In normal conditions or in primary RP (excluding during the cold-exposure test), the normal nailfold capillaroscopic pattern shows a regular disposition of the capillary loops along with the nailbed. On the contrary, in subjects suffering from secondary RP, one or more alterations of the capillaroscopic findings should alert the physician of the possibility of a connective tissue disease not yet detected. Nailfold capillaroscopy (NV) represents the best method to analyse microvascular abnormalities in autoimmune rheumatic diseases. Architectural disorganization, giant capillaries, haemorrhages, loss of capillaries, angiogenesis and avascular areas characterize >95% of patients with overt scleroderma (SSc). The term 'SSc pattern' includes, all together, these sequential capillaroscopic changes typical to the microvascular involvement in SSc. The capillaroscopic aspects observed in dermatomyositis and in the undifferentiated connective tissue disease are generally reported as 'SSc-like pattern'. Effectively, and early in the disease, the peripheral microangiopathy may be well recognized and studied by nailfold capillaroscopy, or better with nailfold video capillaroscopy (NVC). The early differential diagnosis between primary and secondary RP is the best advantage NVC may offer. In addition, interesting capillaroscopic changes have been observed in systemic lupus erythematosus, anti-phospholipid syndrome and Sjogren's syndrome. Further epidemiological and clinical studies are needed to better standardize the NCV patterns. In future, the evaluation of nailfold capillaroscopy in autoimmune rheumatic diseases might represent a tool for the prediction of microvascular heart involvement by considering the systemic microvascular derangement at the capillary nailfold.     

Capillaroscopic observations in childhood rheumatic diseases and healthy controls

OBJECTIVE: To describe, by using video nailfold capillaroscopy (NFC), microvascular abnormalities in children with rheumatic diseases and to evaluate the capillary changes over a follow up period. METHODS: 118 children suffering from rheumatic diseases: 55 juvenile idiopathic arthritis (JIA), 7 mixed connective tissue disease (MCTD), 6 primary Raynaud's phenomenon (PRP), 34 systemic lupus erythematosus (SLE), 8 juvenile systemic sclerosis (JSSc) and 8 juvenile dermatomyositis (JDM) were included in the study. Patients with major capillaries abnormalities or scleroderma pattern were followed up for at least 12 months. 70 age- and sex-matched healthy controls (HC) were also examined. RESULTS: In HC there was a significant correlation between age and capillary length (p = 0.001). JIA patients showed capillary number, size, shape and arrangement similar to HC. Minor abnormalities were frequently observed. The percentage of major abnormalities were significantly increased compared to HC in MCTD (p = 0.008), SLE (p = 0.0002) and JDM patients (p < 0.0001). 5/8 of JSSc had a scleroderma pattern from the onset of the disease. The serial observations in connective tissue diseases also showed that the evolution of capillaroscopic pattern was not unidirectional. In fact, in some nailfolds there was an increase in capillary loss and in avascular areas, whereas sometimes it remained stable on repeated examination. CONCLUSION: NFC can be used as a simple, inexpensive, non-invasive method to evaluate the microvascular abnormalities in childhood rheumatic conditions, and it may be useful in early recognition and monitoring scleroderma spectrum disorders.     

Functional and morphological evaluation of hand microcirculation with nailfold capillaroscopy and laser Doppler imaging in Raynaud's and Sjögren's syndrome and poly/dermatomyositis

OBJECTIVES: Nailfold capillaroscopy is widely used in autoimmune patients to determine capillary morphology. Laser Doppler imaging (LDI) is a relatively new method for measuring the microcirculation of cutaneous perfusion. The aim of this study was to investigate the capillary morphology and microcirculation among patients with Sj?gren's syndrome (SS) and poly/dermatomyositis (PM/DM) with these two non-invasive methods and to detect secondary Raynaud's syndrome (SRS) in these autoimmune diseases. METHODS: Thirty patients with primary SS, 30 patients with PM/DM, 30 patients with primary Raynaud's syndrome (PRS), and 30 healthy volunteers were included in the study. Nailfold capillaroscopy and LDI were performed on each patient. RESULTS: A comprehensive analysis was performed among the patients and healthy individuals. Among SS patients avascularity and among PM/DM patients avascularity and capillary morphology changes were most often detected by capillaroscopy. With LDI the mean steady-state cutaneous perfusion was 1.25 perfusion units (PU) in region of interest 1 (ROI1), 1.22 in ROI2, and 1.49 at the fingertips in PRS patients; the corresponding values were 1.2, 1.03, and 1.48 PU in SS, 0.91, 0.76, and 1.19 PU in PM/DM, and 1.79, 1.62, and 2.2 PU in the controls. The differences were significant between each autoimmune group compared to the control group (p<0.02, p<0.001, and p<0.001, respectively). CONCLUSIONS: By using nailfold capillaroscopy, abnormalities in capillary morphology can be detected, and by using LDI, the reduced blood flow in the capillaries can be detected. These investigations can be useful in the detection of SRS, or in distinguishing whether the reduced blood flow is due to primary/systemic autoimmune diseases.     

A study on soluble intercellular adhesion molecule-1 and selenium in patients with rheumatoid arthritis complicated by vasculitis

Clinical manifestations of vasculitis, as a complication of rheumatoid arthritis (RA), can be postulated as a consequence of immune response abnormalities and endothelial cell dysfunction. In this study we searched for the relationship between the extent of vascular involvement and either serum sICAM-1 or selenium concentrations. We also explored the possible interaction of serum selenium with sICAM-1 to provide a greater understanding of their role in rheumatoid vasculitis (RV). For the study, we measured the serum titers of sICAM-1 using an ELISA assay and the serum selenium concentrations using the ETAAS method in 37 women suffering from RA and 18 normal women serving as controls. All the RA patients were evaluated by extensive clinical, laboratory and capillaroscopic studies. In all patients with extra-articular manifestations, severe or moderate changes in nailfold capillaroscopy were found. Serum sICAM-1 titers in RA patients with mild vasculitis on nailfold capillaroscopy did not differ significantly from those of the healthy subjects, whereas a higher sICAM-1 level seemed to reflect the more intensive vascular changes in capillaroscopy. These data suggest that sICAM-1 serum levels may reflect the extent of the microvascular involvement in RA patients. Compared with controls, all the RA patients had markedly lower serum selenium concentrations, irrespective of the degree of the capillaroscopic vascular changes. A significant inverse correlation between sICAM-1 and selenium was found in the controls (r = –0.54; P<0.02). By contrast, no correlation was noted in RA patients (r=0.10, P not significant). This suggests that the sICAM-1 shedding in RV does not appear to be influenced by selenium, presumably owing to its low serum concentration.Abbreviations CRP Serum C-reactive protein - DAS Disease activity score - ESR Erythrocyte sedimentation rate - GSH-Px Glutathione peroxidase - NSAIDs Non-steroidal anti-inflammatory drugs - RA Rheumatoid arthritis - RV Rheumatoid vasculitis - TR Thioredoxin reductase     

Cross cultural adaptation and validation of an Arabic version of selected PROMIS measures for use in rheumatoid arthritis patients

Aim of the workTo cross culturally adapt and validate an Arabic version of selected patient reported outcome information system (PROMIS) measures for use in rheumatoid arthritis (RA).Patients and MethodsOne-hundred RA patients were recruited. They were asked to complete a validated Arabic health assessment questionnaire (HAQ), visual analogue scale (VAS) of patient global assessment (PGA) and the translated Arabic version of selected PROMIS measures for use in RA. The items of PROMIS were translated and cross culturally adapted using general guidelines. A pretest sample of twenty RA patients was used for cognitive evaluation of translated version and estimation of internal consistency reliability with cronbachs-alpha. Convergent validity was assessed. Disease activity score-28 (DAS28) and clinical disease activity index (CDAI) were considered. Results: Patients were mostly females (94%) with a mean age of 41.5 ± 11.1 years and disease duration 6.3 ± 7.3 years. The level of education varied and extra-articular manifestations were present in 12%, rheumatoid factor was positive in 72%, the mean tender (8 ± 3.3) and swollen (5 ± 2.1) joint counts, the PGA was 6.7 ± 1.8, HAQ was 1.4 ± 0.7, the DAS28 was 5.4 ± 1.3 (2.7–8.5) while the CDAI was 27.1 ± 12.6 (5–64). The translated Arabic PROMIS showed good internal consistency and reliability (Cronbach's-α 0.7–0.9). There was a significant correlation between different domains of PROMIS (p < 0.01, r = 0.37–0.68) and of PROMIS domains with HAQ (p < 0.01, r = 0.37–0.85), PGA (p < 0.01, r = 0.3–0.67), (p < 0.01, r = 0.29–0.65) and CDAI (p < 0.01, r = 0.28–0.72).ConclusionThese data provide preliminary evidence of reliability and construct validity of the Arabic translated selected PROMIS measures for use in RA patients.     

Rheumatoid factor and anti-citrullinated protein antibodies (ACPA) in psoriatic arthritis (PsA), and skin psoriasis: Relevance and clinical implications

Aim of the workAnti-citrullinated protein antibodies (ACPAs), is a highly specific markers for rheumatoid arthritis, can also be found in psoriatic arthritis (PsA). This work aimed to look into the frequency of rheumatoid factor (RF) and ACPA in PsA and psoriasis patients without clinical evidence of arthritis (PSO), and to correlate findings with demographics, disease characteristics, laboratory findings, and radiological damage in PsA. Patients and methods: The study included 78 PsA patients, 47 PSO patients, and 69 normal controls. Full clinical assessments, RF and ACPA assays, and modified radiological Steinbroker score (Mss) for PsA were performed. Results: The age of the patients was 45.6 ± 8.7 years and the M:F 59:66 (M:F 0.9:1). Positive RF was not significantly different between PsA and PSO groups (p = 0.35), but positive ACPA was significantly more common in PsA patients (p < 0.001) than in PSO and controls. No significant difference was observed between the PSO and controls (p = 0.08). In PsA, RF titers correlated significantly with ESR (p = 0.002), swollen joint count (SJC)(p = 0.02), tender joint count (TJC)(p = 0.02), and mSS (p = 0.001), while in the PSO RF correlated significantly with ESR (p = 0.011) and CRP levels (p = 0.02). In the PsA, ACPA titer significantly correlated with CRP levels (p < 0.001), SJC (p = 0.002), TJC (p = 0.003), mSS (p < 0.001) and PASI (p < 0.001), but with none of these in the PSO. Conclusion: PsA patients have more frequent positive RF and ACPA than PSO patients. ACPA values are significantly correlated with markers of inflammation, swollen and tender joint count and radiological damage in PsA and not in PSO patients.