How to perform and interpret capillaroscopy

Background: Nailfold capillaroscopy (NFC) is a valuable tool to detect microcirculation abnormalities in connective tissue diseases (CTDs). However, whether the universal serial bus (USB) digital microscopy used as onychoscopy is as effective as the videocapillaroscopy in determining the diagnostic and prognostic values of CTDs remains to be determined.Objective: This study aims to investigate NFC features of systemic lupus erythematosus (SLE), dermatomyositis (DM), and systemic sclerosis (SSc) patients and compare with normal controls as well as examine which feature could differentiate among CTDs. Furthermore, we aim to explore different capillaroscopic abnormalities and their association with disease activity.Methods: Nailfold images were taken from patients and healthy controls using a USB digital microscopy. Patterns on the capillary morphology, diameter, architecture, and density were recorded and compared. We further determined the NFC findings in SLE, DM, and SSc and corresponded to their respective disease activity scoring system.Results: A total of 245 participants, consisting of 54 SLE, 32 DM, and 51 SSc patients, as well as 108 controls, were enrolled. All capillaroscopic features, except for tortuous capillaries, were significantly more common in CTDs than healthy control (all p < 0.05). A multinomial logistic regression analysis revealed that bushy capillaries had significantly higher odds for both SLE and DM than SSc (OR: 4.10, 95% confidence interval (CI): 1.71–9.81, p = 0.002 and OR: 7.82, 95% CI, 2.86–21.38, p < 0.001, respectively). Elongated capillaries demonstrated significant odds for SLE compared with SSc (OR: 3.35, 95% CI: 1.005–11.20, p = 0.049), while prominent subpapillary plexus showed greater odds for SLE compared with both DM and SSc (OR: 2.75, 95% CI: 1.07–7.02, p = 0.03 and OR: 5.78, 95% CI: 2.29–14.58, p < 0.001, respectively). The presence of hemorrhage, enlarged capillaries, and the low-density index had significantly higher odds in favor of SSc than SLE. Bushy capillaries were the only pattern with a strong association for DM over SSc. The presence of enlarged capillaries indicated higher SLE severity, but no specific finding was related to DM or SSc skin scores.Conclusions: Nailfold capillaroscopic examination using a digital microscope is a valuable method for the diagnosis of SLE, DM, and SSc. Several morphologic patterns can help differentiate among CTDs; however, the prognostic significance of this method requires further investigations.

SP0127 Capillaroscopy for prediction and longitudinal monitoring of vasculopathy in connective tissue diseases

BackgroundNailfold capillary microscopy (NCM) has been shown to be associated with disease severity and internal organ involvement in non-consecutive systemic sclerosis (SSc) cohorts. NCM may help in early recognition of...

BackgroundInterstitial lung disease (ILD) is one of the most serious lung complications in patients with connective tissue disease (CTD), most commonly in patients with systemic sclerosis (SSc). High-resolution Computed Tomography...

To investigate the utility of an open-source Digital Imaging and Communication in Medicine viewer software-OsiriX-to assess pulmonary fibrosis (PF) in patients with systemic sclerosis (SSc). Chest high-resolution computed tomography (HRCT) examinations obtained from 10 patients with diagnosis of SSc were analysed by two radiologists adopting a standard semiquantitative scoring for PF. Pulmonary involvement was evaluated in three sections (superior, middle and inferior). For the assessment of the extension of PF, the adopted semiquantitative HRCT score ranged from 0 to 3 (0 = absence of PF; 1 = 1-20 % of lung section involvement; 2 = 21-40 % of lung section involvement; 3 = 41-100 % of lung section involvement). Further, a quantitative assessment (i.e. parameters of distribution of lung attenuation such as kurtosis and mean lung attenuation) of PF was independently performed on the same sections by a rheumatologist, independently and blinded to radiologists' scoring, using OsiriX. The results obtained were compared with those of HRCT semiquantitative analysis. Intra-reader reliability of HRCT findings and feasibility of OsiriX quantitative segmentation was recorded. A significant association between the median values of kurtosis by both the quantitative OsiriX assessment and the HRCT semiquantitative analysis was found (p < 0.0001). Moreover, kurtosis correlated significantly with the mean lung attenuation (Spearman's rho = 0.885; p = 0.0001). An excellent intra-reader reliability of HRCT findings among both readers was obtained. A significant difference between the mean time spent on the OsiriX quantitative analysis (mean 1.85 ± SD 1.3 min) and the mean time spent by the radiologist for the HRCT semiquantitative assessment (mean 8.5 ± SD 4.5 min, p < 0.00001) was noted. The study provides the new working hypothesis that OsiriX may be a useful and feasible tool to achieve a quantitative evaluation of PF in SSc patients.

Objective. To study the difference of interstitial lung diseases (ILDs) in high-resolution computerized tomography and pulmonary function test among different connective tissue diseases (CTDs). Methods. 209 patients with different CTDs were recruited and underwent lung HRCT and PFT. Eerythrocyte sedimentation rate (ESR), C-reactive protein (CRP), serum ferritin (SF), anti-SSA, and so on were tested. Based on HRCT, a patient was classified into ILD group (CTD+ILD) or non-ILD group (CTD-ILD). HRCT, PFT, and laboratory markers were compared according to CTDs and CTD-associated ILDs. Results. The incidences of ILD were 79.6%, 82.0%, 89.7%, and 97.1% respectively for Rheumatoid arthritis (RA), primary Sjogren’s symptom (pSS), dermatomyositis/polymyositis (DM/PM), and systemic sclerosis (SSc) groups. RA and pSS patients exhibited more nodules, patching, ground-glass opacity, and cord shadow foci in HRCT, DM/PM and SSc patients exhibited more reticular opacity and honeycombing foci. RA and pSS patients exhibited more obstructive ventilatory disorder, small airway dysfunction and emphysema in PFT, and DM/PM and SSc patients exhibited more restrictive ventilatory disorder, mixed ventilatory disorder. ESR, CRP and SF were significantly higher in total CTD+ILD group than in total CTD-ILD group (P = 0.047, 0.006, 0.004, respectively), and higher in different CTD+ ILD groups than in comparable CTD-ILD groups (P = 0.049, 0.048, and 0.023, pSS+ILD, SSc+ILD and RA+ILD compared to pSS-ILD, SSc-ILD and RA-ILD, respectively for ESR, CRP, SF). The positive rate of anti-SSA was significantly higher in DM/PM+ILD group than in DM/PM-ILD group (P = 0.025). Conclusions. The manifestations and incidences of ILDs differ among different CTDs in HRCT and PFT, and inflammation and anti-SSA are positively correlated with ILDs in different CTDs, which provide important evidences for judging disease condition and prognosis.

To determine the clinical significance of anti-centromere (ACA) and anti Scl-70 antibodies in an English population with connective tissue diseases, we examined the sera of 150 patients, including 40 with systemic sclerosis (SS), who were prospectively studied on the same clinical protocol in our connective tissue disease clinic. ACA was present in 44% of the CREST patients as opposed to only 12% of those with SS and diffuse skin involvement. Only two patients without SS had ACA. Anti Scl-70 was detected in 20% of the patients with SS and only two of those with other connective tissue diseases. We confirmed the specificity of these antibodies for SS. Either anti Scl-70 or ACA was present in half the patients with SS and their presence may represent a useful aid to diagnosis of this disease in patients presenting with Raynaud's phenomenon or an undifferentiated connective tissue disease. While less than half the CREST patients had ACA, this antibody appears to identify those patients within the CREST variant with skin involvement confined to sclerodactyly as opposed to those with acrosclerosis. These patients, however, did not differ in the degree of visceral involvement.

IntroductionIt is known, that course of the disease differs between overlap syndromes (OS) and systemic sclerosis (SSc) group.AimTo compare the prevalence of OS in limited cutaneous systemic sclerosis (lcSSc) and diffuse cutaneous SSc (dcSSc) and to analyze the presence of different manifestations in the SSc and OS group.Material and methodsThe study included 126 European Caucasian SSc patients (99 females and 27 males) hospitalized consecutively in the Department of Rheumatology and Connective Tissue Diseases. Patients fulfilled the American College of Rheumatology (ACR) classification criteria of SSc (57 – dcSSc and 69 – lcSSc). The study groups were determined according to the subtype of SSc, coexistence of other connective tissue diseases (CTDs), and incidence of clinical and serological manifestations.ResultsIn our SSc study group, 28/126 patients (22%) were affected by more than one CTD. The prevalence of OS was significantly higher in the lcSSc group – 33% (23/69) compared to the dcSSc group – 8% (5/57). We found that mortality and digital ulcers were significantly higher, whereas kidney involvement and arthritis were significantly lower in the SSc group compared to the OS group. The prevalence of anti-topoisomerase I (a-Scl-70) was significantly higher, and prevalence of anti-PM/Scl, anti-Ro-52 antibodies was significantly lower in the SSc group compared to the OS group.ConclusionsOverlap syndromes were more common in lcSSc than in dcSSc. The course of the disorder and internal organ involvement were different in OS compared to SSc patients.

FRI0560 Quantitative ct indexes in the evaluation of interstitial lung disease related to rheumatoid arthritis

Microvascular lesions are a predominant feature in systemic sclerosis (SSc) and seem to play a central pathogenic role. The presence of nailfold capillary abnormalities is useful in diagnosing SSc. Capillaroscopy, however, usually requires special equipment and may be time consuming. Dermatoscope has been presented as a new diagnostic tool for quick and efficient examination of nailfold capillaries for circumstances when standard microscope equipment is not available. To assess the practical utility of dermatoscope for assessment of capillary morphology in patients with SSc, 83 Japanese patients with SSc (68 women, 15 men) and 68 healthy controls were examined in the study. Twenty-one patients (16 women, 5 men) had diffuse cutaneous SSc and 62 (52 women, 10 men) had limited cutaneous SSc. Enlarged capillaries and hemorrhages were evaluated in all 10 fingers with either naked eyes or DermLite(®) DL100 dermatoscope. Enlarged capillaries and hemorrhages were significantly more frequently detected with dermatoscope than without it. These findings were observed most frequently in the fourth finger. The presence of two or more enlarged capillaries in one or more fingers showed 83.1% sensitivity and 100% specificity for SSc. Among patients with SSc with anti-topoisomerase I antibody, the disease duration correlated negatively with the dermatoscopic number of enlarged capillaries and hemorrhages. Dermatoscope allows the easy and rapid identification of capillary nailfold morphological changes in SSc and should be routinely used for diagnosing SSc.

Sir, We have read the letter by Belloli L et al. [1], who commented on our paper concerning low levels of vitamin D in patients affected by systemic sclerosis (SSc) [2] and showed further data on hypovitaminosis D in SSc. We agree with Belloli L et al. that in Italy, hypovitaminosis D is very frequent not only in systemic rheumatic diseases as SSc but also in very different unrelated diseases like osteoarthritis [1] as well as in elderly women [3], but not in premenopusal women, in whom low concentrations of vitamin D were found in 27.8% and 3.4% of the cases in winter and in summer, respectively [4]. But in our opinion, the interest concerning hypovitaminosis D in SSc and in other connective tissue diseases was that low levels of vitamin D may influence clinical manifestations; in SLE hypovitaminosis D has been associated with disease activity and severity [5–7] and in undifferentiated connective tissue diseases with a high risk of developing a well-defined connective tissue disease [8]. In rheumatoid arthritis, the serum concentration of vitamin D correlated negatively with disease activity (DAS28) and disability as evaluated by Health Assessment Questionnaire and mobility activities of daily living score [9]. In SSc, an inverse correlation between the serum level of vitamin D has been reported with disease activity and with acutephase reactants [10]; vitamin D deficiency was also associated with more severe diseases [2], supporting both the immunomodulatory properties [11] and the antifibrotic action of the vitamin [12, 13]. Moreover, very recently, it has been reported that vitamin D plays an antiproliferative effect on mesenchymal multipotent cells [14] revealing possible new perspectives on the pleiotropic properties of the molecule. As recently reviewed, the development of autoimmune rheumatic diseases is dependent on the interaction between genetic background and many environmental factors, which include hypovitaminosis D [15]. Therefore, low vitamin D status may represent an easily modifiable factor, whereby treatment may have a beneficial impact on both the development and the clinical phenotype of connective tissue diseases.

To assess the prevalence of autoimmune diseases in first-degree relatives of patients with systemic sclerosis (SSc), and to compare those results with control families in order to identify patterns of autoimmune diseases in relatives. A retrospective case-control postal questionnaire survey was performed in France to recruit patients with SSc belonging to an association of patients with SSc and unrelated age-matched and sex-matched controls. Each participant was asked to self-report on the existence of autoimmune diseases in their first-degree relatives. The prevalence of autoimmune diseases in the families of patients with SSc was compared with the corresponding prevalence in the families of controls. A total of 121 families out of 373 (32.4%) with a member having SSc reported at least 1 autoimmune disease in 1 or more first-degree relatives. The most frequent autoimmune diseases in SSc families when adjusted for family size were autoimmune thyroid disease (AITD; 4.9%), rheumatoid arthritis (4.1%), psoriasis (3.9%), and type 1 diabetes mellitus (2.9%). Compared with control families, AITD and connective tissue diseases (SSc, systemic lupus erythematosus, or Sjögren's syndrome) were more likely to occur in families with SSc (p = 0.01 and p = 0.01, respectively), with OR of 3.20 (95% CI 1.25-8.18) and 5.20 (95% CI 1.22-21.8). In contrast, inflammatory bowel disease was less likely to occur within families with SSc (p = 0.02, OR 0.29, 95% CI 0.11-0.80). In addition, the coexistence of more than 1 autoimmune disease in the index SSc case was associated with familial aggregation of autoimmune diseases. Our results show that autoimmune diseases cluster within families of patients with SSc. This supports the notion that these diseases might arise on a shared genetic basis underlying several autoimmune phenotypes.

BackgroundMixed connective tissue disease (MCTD) is a systemic auto-immune disorder, being probably the least common among the connective tissue diseases. Symptoms can be severe and could affect health-related quality of...

SAT0394 Patients with Rheumatoid Arthritis in Comparison to Other Connective Tissue Diseases are Mostly Influenced by Concomitant Fibromyalgia

Connective tissue diseases (CTDs) are associated with several interstitial lung diseases. The aim of this study was to describe the recently individualized syndrome of combined pulmonary fibrosis and emphysema (CPFE) in a population of patients with CTD. In this multicenter study, we retrospectively investigated data from patients with CTD who also have CPFE. The demographic characteristics of the patients, the results of pulmonary function testing, high-resolution computed tomography, lung biopsy, and treatment, and the outcomes of the patients were analyzed. Data from 34 patients with CTD who were followed up for a mean±SD duration of 8.3±7.0 years were analyzed. Eighteen of the patients had rheumatoid arthritis (RA), 10 had systemic sclerosis (SSc), 4 had mixed or overlap CTD, and 2 had other CTDs. The mean±SD age of the patients was 57±11 years, 23 were men, and 30 were current or former smokers. High-resolution computed tomography revealed emphysema of the upper lung zones and pulmonary fibrosis of the lower zones in all patients, and all patients exhibited dyspnea during exercise. Moderately impaired pulmonary function test results and markedly reduced carbon monoxide transfer capacity were observed. Five patients with SSc exhibited pulmonary hypertension. Four patients died during followup. Patients with CTD and CPFE were significantly younger than an historical control group of patients with idiopathic CPFE and more frequently were female. In addition, patients with CTD and CPFE had higher lung volumes, lower diffusion capacity, higher pulmonary pressures, and more frequently were male than those with CTD and lung fibrosis without emphysema. CPFE warrants inclusion as a novel, distinct pulmonary manifestation within the spectrum of CTD-associated lung diseases in smokers or former smokers, especially in patients with RA or SSc.