Nailfold Capillaroscopy Abnormalities 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).

Microvascular dysfunction associated with reduced nailfold capillary density in patients with ischemia with no obstructive coronary artery disease

Abstract Funding Acknowledgements None. Introduction The impairment of microvascular function present in coronary microcirculation with chronic angina without obstructive coronary arteries. There are insufficient data in the literature regarding possible generalized microangiopathy in this population. Aim The aim of this study is to demonstrate whether patients with Angina with No Obstructive Coronary Artery Disease (ANOCA) compared to individuals without coronary microvascular dysfunction (CMD) are characterized by a different level of nailfold capillaroscopy abnormalities. Methods We examined 18 participants without CMD - non-CMD group [9 female, 50%, mean age: 54.4±8.1 years) and 26 patients with ANOCA - CMD group (22 female, 84,6%, mean age : 53.2±10.7 years). Functional coronary angiography was performed for the assessment of coronary microcirculation in all patients. Coronary flow reserve (CFR) and index of microvascular resistance (IMR) were measured in the left anterior descending coronary artery using a temperature/pressure sensor-tipped guidewire. In addition, the assessment of skin microcirculation was performed by capillaroscopy, a non-invasive technique to evaluate small vessels of the microcirculation in the nailfold, using stereomicroscope in all patients. Results In CMD group, mean CFR and IMR were 1.34±0.6 and 44.8±28, respectively. Out of the 26 MVD patients with abnormal CFR, 7 of them (27%) had a normal value of IMR, indicating functional microvascular dysfunction. On the other hand, 18 patients (69%) had an abnormal IMR, indicating structural microvascular dysfunction. Capillary density in patients with MVD was significantly decreased compared to the control group (7.6±2.2 vs 10.9±1.8 vessels/mm, p=0.04). The difference in capillary density between the two groups was statistically significant after adjustment for multiple comparisons (p<0.05). No significant difference was found in body mass index, renal function, medical history of dyslipidemia, diabetes mellitus and smoking status between the two groups (p<0.05). Conclusion Nailfold capillary density was reduced in ANOCA patients compared to control group. These data may provide new insights regarding possible generalized microangiopathy in CMD patients. These results suggest that there is an association between microcirculatory impairment at both heart and peripheral vascular bed level.

P151 Nailfold capillaroscopy for prediction of novel severe organ involvement and mortality in systemic sclerosis

Abstract Background/Aims Nailfold capillaroscopy (NFC) has been suggested as a potential biomarker of disease severity in systemic sclerosis (SSc). Several studies report the association between capillary loss and disease severity however, the association of NFC abnormalities with novel severe organ involvement/progression and mortality in SSc has not been evaluated. We aim to evaluate the association of nailfold capillaroscopy (NFC) abnormalities with novel major organ involvement/progression and mortality in SSc. Methods Follow-up data from patients with SSc registered between 2000 and 2022 were analyzed. Patients underwent NFC at baseline. Baseline demographic data, antibody status, baseline pulmonary function, novel severe organ involvement/progression and mortality were registered. Novel organ involvement/progression was defined as new or progressive involvement of peripheral vasculature, lungs, heart, skin, gastrointestinal, kidney, musculoskeletal at 12 and 24 months of follow-up. The following NFC parameters were evaluated: capillary density, hemorrhages, enlarged and giant capillaries, avascular areas, organization of capillary architecture and scleroderma pattern (early/active/late). Logistic regression modelling was run to assess associations between NFC and clinical parameters, the occurrence of novel severe organ involvement and/or progression and mortality. Results 113 patients were included: 28 (25%) were male, 86 (76%) lcSSc and 27 (23%) dcSSc. 70 patients (61%) developed novel overall severe organ involvement/progression and 40 patients died (36%) during follow-up. Table 1 and table 2 summarizes the associations between NFC and novel severe organ involvement/progression and mortality during follow-up. Loss of capillary density was associated with overall severe organ involvement (p = 0.002), peripheral vascular involvement (p = 0.03), new ILD (p = 0.04) and skin progression (p = 0.01); avascular areas were associated with overall severe organ involvement (p = 0.03), new ILD (p = 0.03) and progression of ILD (p = 0.02) and scleroderma pattern was associated with overall severe organinvolvement (p = 0.03), peripheral vascular involvement (OR p = 0.04), new ILD (p = 0.004), progression of ILD (p = 0.03) and skin progression (p = 0.04). Mortality was associated with ILD (OR 2.56 CI 95% 1.5-7.2, p 0.04), DLCO <70% (2.34 CI 93% 1.42-6.23, p 0.03), a late pattern in NFC (3.42 95% CI 1.42-7.25, p 0.002), presence of hemorrhages (OR 1.77 95% CI 1.24-8.23, p0.034) and avascular zones (OR 6.22 95% CI 2.54-10.3, p 0.021). Survival rates from diagnosis at 10 years were lower in patients with NFC with active a late pattern (40% and 20%) compared with those with normal, unspecific and early pattern (100%, 90% and 70%) (p = 0.008 by log rank test) (figure 1). Conclusion NFC may be a potential biomarker in SSc for predicting novel severe organ involvement and/or progression and mortality. Abnormal capillary density, avascular areas and scleroderma pattern are predictors of overall severe organ involvement, peripheral vascular involvement, novel and progression of ILD and skin progression. Disclosure C. Sieiro Santos: None. P. Pérez García: None. J. Ordas Martínez: None. C. Álvarez Castro: None. E. Díez Álvarez: None.

Nailfold capillaroscopy abnormalities and pulmonary hypertension in mixed connective tissue disease and systemic sclerosis patients.

Pulmonary arterial hypertension (PAH) represents an important vascular complication of mixed connective tissue disease (MCTD) and systemic sclerosis (SSc). Microvascular involvement in these diseases can be investigated by means of nailfold capillaroscopy (NFC). Microvascular involvement detected in the nailfold bed is the mirror of the microvascular damage occurring in the entire body, further indicating the involvement of the target organs. The aim of this study was to evaluate the microvascular involvement in MCTD patients with or without PAH, compared to that found in SSc patients with or without PAH. This cross-sectional study was performed in the Department of Internal Medicine and Department of Rheumatology, Timișoara, Romania, during the time period between January 2017 and December 2022, on a group of 26 patients with MCTD and 26 SSc patients. Antinuclear antibodies, anti-U1-RNP, anti-Scl 70, anti-centromere, anti-cardiolipin antibodies (aCL) (IgM, IgG), anti-β2-glycoprotein I (aβ2GPI) (IgM, IgG) antibodies, and lupus anticoagulant (LAC) were determined in both the groups. PAH was evaluated through cardiac ultrasonography, determining the sPAP (systolic pulmonary artery pressure). Nailfold capillaroscopy was performed using a USB Digital Microscope and 2.0-megapixel digital camera recording capillaries density, giant capillaries, enlarged capillaries, capillaries hemorrhages, avascular areas, ramified/bushy capillaries scores. Data were recorded and presented as mean ± standard deviation. Statistical analyses were performed using the Student's t-test, ANOVA test, and Pearson's correlation. Differences were considered statistically significant if p-value < 0.05. Among the MCTD patients, PAH was identified in 12 patients (46.15%), while among the SSc patients PAH was identified in 14 patients (53.84%). Development of PAH in MCTD patients was associated with lower capillaries density (p-value < 0.00001), higher scores of giant capillaries, ramified/bushy capillaries, and capillary hemorrhages (p-value < 0.00001, for each of them). Anti-U1-RNP, aCL, aβ2GPI antibodies and LAC were also found to be involved in PAH-associated MCTD development. Unlike MCTD patients, SSc patients with PAH presented with lower capillaries density and ramified/bushy capillaries scores (p-value < 0.05). The MCTD patients who presented significant NFC abnormalities (especially active and late scleroderma-like capillaroscopic pattern) are prone to PAH development. Capillary density reduction is the most important factor associated with the occurrence of PAH. Differences in NFC findings (especially capillary density and ramified/bushy capillaries) were detected among patients with MCTD and SSc having PAH.

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.

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.

E34 Coexisting sickle cell disease and autoimmune diseases among Nigerian children

Abstract Introduction Patients with sickle cell disease (SCD) do have a defective activation of the alternate complement pathway which increases their risk of having infections. This is thought to predispose to autoimmune disease (AID) in them. However, there is paucity of data on coexisting AID and SCD in children which may be due to under diagnosis as a result of overlapping symptoms of the two disease conditions. Objectives To highlight the clinical features, diagnostic challenge and importance of early recognition and treatment of autoimmune diseases in children with sickle cell disease. Methods Retrospective review of records of four children with sickle cell disease who were diagnosed with autoimmune diseases at the paediatric Rheumatology Clinic of Lagos State University Teaching Hospital (LASUTH) between May 2018 to February 2023. Results Case 1 15 –year –old girl known haemoglobin SC patient of haematology unit who was referred to rheumatology unit with history of worsening jaundice, abdominal swelling, headache, constitutional symptoms, left knee pain, and two episodes of blood transfusion within two months. Initally being managed for hyperhaemolytic and vaso-occlusive crisis but later referred to rheumatology unit on suspicion of autoimmune disease due to recurrent anaemia, and five episodes of blood transfusion within two months. ESR was 65 mm/h, ANA of 1:2560, positive anti-smith antibodies, elevated urine protein creatinine ratio (UPCR), low C3 and C4 and positive direct coomb’s test. Diagnosis was juvenile systemic lupus erythematosus (JSLE). She had pulse methylprednisolone, oral prednisolone, hydroxychloroquine and mycophenolate mofetil. She has improved clinically and being followed up in clinic. Case 2 10-year-old girl, known sickle cell anaemia patient who had unproductive cough of one-month duration and chest radiograph findings of bronchiectatic and fibrotic changes in the right upper/middle lobes with reduction in lung volume. Gene xpert was negative for tuberculosis but chest CT showed honeycombing in the right upper lobe implying Interstitial lung disease. Further investigations showed ESR of 5, ANA of 1:1280, borderline anti-dsDNA, positive anti-SM/RNP, normal urinalysis, normal renal function and restrictive lung disease on spirometry. She is being managed as a case connective tissue disorder with associated Interstitial Lung Disease. She is presently on mycophenolate mofetil and being followed up in clinic. Case 3 11-year-old female living with sickle cell anaemia who presented with inflammatory arthritis of the small joints of the hands, elbows and knees of nine-month duration, sclerodactyly, contractures of the PIP of the fingers, narrowing of oral aperture, generalized hypopigmented macules and abnormal nailfold capillaroscopy. ANA was 1:640, positive anti-Scl 70, negative anti-centromere, ESR of 130 mm/h, thrombocytosis, severe restriction on spirometry and features of interstitial lung disease on HRCT of the chest. She commenced mycophenolate mofetil, prednisolone and nifedipine. She later had 2 doses of rituximab due to slow clinical improvement and is being followed up. Case 4 13-year-old female with sickle cell anaemia who presented with recurrent fever &amp; severe haemolytic anaemia requiring frequent blood transfusions (13 pints of blood over 6 months!!!). Initial diagnosis was septicaemia, and autoimmune haemolytic anaemia. Following rheumatology review, ANA was 1:640, positive anti-DsDNA, negative anti-Smith antibodies, ESR of &amp;gt; 150mm/h, elevated UPCR and pancytopaenia. She was later diagnosed with Lupus nephritis. She had Mycophenolate mofetil, prednisolone and hydroxychloroquine. She died of acute lupus flare 4 weeks after discharge from the hospital. Conclusions Physicians should have high index of suspicion for possible association of sickle-cell disease and autoimmune diseases so as not to delay definitive diagnosis and initiation of appropriate treatment.

77 Case series of juvenile scleroderma from Nigeria

BackgroundThe word scleroderma means ‘hard skin’ that develops due to excessive accumulation of collagen. It is the third most frequent rheumatic disease in paediatric rheumatology after juvenile idiopathic arthritis and systemic lupus erythematosus. When it occurs in individual <16 years, it is called juvenile scleroderma. It is a rare disease that occurs in one per million children, documented to be more common in African adults with poorer survival states compared with Caucasians.Objectives To describe the clinical and laboratory characteristics of children with juvenile scleroderma seen in our clinic, thus, increasing its awareness.MethodsRetrospective review of records of three children diagnosed with juvenile scleroderma at the paediatric Rheumatology Clinic of Lagos State University Teaching Hospital(LASUTH) between May 2018 to April 2022.Results Case 1 A 12-year-old girl presented with skin tightness of the left hand and thigh of one year, contracture of the 3rd, 4th and 5th proximal interphalangeal joints, arthritis of the left wrist, skin induration of the dorsum of the left hand involving the 4th and 5th finger and extending to the wrist and skin and induration of the anterolateral aspect of the left thigh. Her blood tests showed an erythrocyte sedimentation rate (ESR) at 20 mm/h, an ANA titre of 1:2560, a negative anti-Scl 70/anti-centromere antibodies, a normal complete blood count and serum electrolytes/urea/creatinine. A diagnosis of linear scleroderma was made, the patient had prednisolone, methotrexate and folic acid, in addition to topical emollients. She improved clinically as observed during follow up visits six weeks after initiation of treatment but later defaulted from the clinic due to unknown reason. Case 2 A 4-year-old girl presented with constitutional symptoms, swollen hands and feet, sclerodactyly, narrowing of oral aperture, ulcers at tips of the fingers, inflammatory pain of the large joints, hypopigmented macules on the face, trunk, abdomen and back and also abnormal capillaroscopy. Erythrocyte sedimentation rate was 22 mm/h with normal levels of electrolytes/urea/creatinine, thrombocytosis, ANA titre of 1:640 and negative anti-centromere and anti-U1RNP antibodies. A diagnosis of diffuse systemic sclerosis was made. She started prednisolone, methotrexate, nifedipine and omeprazole and was asked to do an ECG, an Echocardiogram, a spirometry and a chest HRCT but she couldn’t afford to do these investigations due to severe financial constraints. She was clinically stable for four months until she presented at the emergency room with sudden loss of consciousness and congestive cardiac failure. She died during resuscitation attempt. Case 3 An 11-year-old girl known patient of haematology unit with sickle cell anaemia, presented with inflammatory arthritis of the small joints of the hands, elbows and knees of nine-month duration, sclerodactyly, contractures of the PIP of the fingers, narrowing of oral aperture, generalized hypopigmented macules and abnormal nailfold capillaroscopy. Investigation showed an ANA titre of 1:640, positive anti-Scl 70 antibodies, negative anti-centromere antibodies, ESR of 130 mm/h and thrombocytosis. The echocardiogram showed a normally structured heart with severe restriction on spirometry and features of interstitial lung disease on HRCT of the chest. She started mycophenolate mofetil, prednisolone and nifedipine. She later received 2 doses of rituximab due to slow clinical improvement. She is being followed up.ConclusionMost data on scleroderma are from adult studies, we reported these three cases due to the rare occurrence of scleroderma in children, thus increasing its awareness.

Juvenile diabetes and systemic sclerosis: just a coincidence?

BackgroundLimited joint mobility (LJM), previously known as cheiroarthropathy, refers to the presence of reduced extension at the finger joints in people with diabetes and may be associated with scleroderma-like syndromes such as diabetic sclerodactyly.While scleroderma-like syndromes and LJM have been observed in patients with long-term diabetes and associated complications, the coexistence of diabetes with Juvenile systemic sclerosis (jSSc) is rarely described.Case presentationWe describe the case of a 14-year-old boy with long-lasting type 1 diabetes (T1D) and suspected LJM associated with Raynaud phenomenon, sclerodactyly and tapering of the fingertips. A comprehensive work-up showed positive autoantibodies (ANA, anti-Ro-52, anti-Mi-2b), abnormal nailfold capillaroscopy with a scleroderma pattern, interstitial lung disease and cardiac involvement. The overall clinical picture was consistent with the diagnosis of jSSc.ConclusionsLJM can be the initial sign of underlying systemic sclerosis. Nailfold capillaroscopy may help differentiate jSSc from classical LJM in pediatric patients with T1D and finger contractures or skin induration of no clear origin.This case report provides a starting point for a novel hypothesis regarding the pathogenesis of jSSc. The association between T1D and jSSc may be more than a coincidence and could suggest a relationship between glucose metabolism, fibrosis and microangiopathy.

Absence of association between nailfold capillary findings and mild cognitive dysfunction in systemic lupus erythematosus.

The role of vascular damage in cognitive dysfunction (CD) in SLE is not entirely understood. Nailfold capillaroscopy (NFC) is a noninvasive method that may aid the description of further vascular contributions to CD in SLE. Therefore, the aim of our study was to examine and compare finger nailfold capillary morphology in subjects with SLE with and without CD. We conducted a cross-sectional study in patients with SLE. Demographic, clinical, and laboratory characteristics were collected. We evaluated nailfold capillary findings including avascular zones, hemorrhage, dilated and tortuous capillaries, disarrangement, crossing, subpapillary venular plexus, branched loops, and shortened loops by NFC. The Montreal Cognitive Assessment (MoCA) scale was used to screen cognitive function. CD was defined as a score < 26/30. Sixty-five females (97.0%) and 2 males (3%) with SLE were analyzed. Means of age and disease duration were 44.3 ± 12.0years and 15.5 ± 7.6years, respectively. Thirty-five (54.7%) patients had CD. The rate of patients with ≥ 1 NFC abnormality was 50% in both patients with and without CD (P = 0.14). Eight (22.8%) patients with CD compared to 1 without (3.5%) displayed dilated capillaries (P = 0.036). Other NFC abnormalities differed between patients with and without CD, but the possible relationships between dilated capillaries and CD disappeared after adjusting by age, diabetes, and hypertension. NFC findings were not associated with mild CD in patients with SLE. Our exploratory data do not support systemic microvasculopathy measured by NFC related to CD in patients with SLE.

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.

Progression of patients with Raynaud's phenomenon to systemic sclerosis: a five-year analysis of the European Scleroderma Trial and Research group multicentre, longitudinal registry study for Very Early Diagnosis of Systemic Sclerosis (VEDOSS).

Preliminary criteria for the very early diagnosis of systemic sclerosis (VEDOSS) have been previously proposed to identify signs and symptoms in patients with Raynaud's phenomenon. Patients with all signs or symptoms of the VEDOSS criteria already fulfil the 2013 American College of Rheumatology-European League Against Rheumatism (ACR-EULAR) classification criteria for systemic sclerosis. However, prospective data for the evolution to fulfilling these criteria do not exist. We therefore aimed to determine the clinical value of the VEDOSS criteria to identify patients with Raynaud's phenomenon who progress to systemic sclerosis within 5 years. The VEDOSS project was a multicentre, longitudinal registry study done in 42 European Scleroderma Trial and Research group centres located in 20 countries in Europe, North America, and South America. Patients with Raynaud's phenomenon were eligible for enrolment. Those who had fulfilled the 1980 ACR or the 2013 ACR-EULAR classification criteria for systemic sclerosis, as well as of any other ACR or EULAR classification criteria for other definite connective tissue diseases at enrolment were excluded. Data were recorded each year during follow-up visits and included the four VEDOSS criteria (ie, positivity for antinuclear antibodies [ANAs], puffy fingers, systemic sclerosis-specific autoantibodies, and abnormal nailfold capillaroscopy). The primary endpoint was the fulfilment of the 2013 ACR-EULAR classification criteria for systemic sclerosis (ie, progression from enrolment to follow-up). Proportion of progressors and VEDOSS criteria interaction were reported descriptively. Predictors of progression of the distinct VEDOSS criteria interactions were determined based on the point prevalence at 5 years. To investigate the intermediate course of progression of the distinct VEDOSS criteria and their combinations, Kaplan-Meier analysis was done. Between March 1, 2010, and Oct 4, 2018, we enrolled 1150 patients with Raynaud's phenomenon in the VEDOSS database. 764 (66·4%) of 1150 patients met the VEDOSS criteria for study inclusion. Of the 764 patients, 553 (72·4%) had at least one available follow-up visit and the median duration of follow-up was 3·6 years (IQR 1·7-5·8). The mean age was 45·9 years (SD 15·0), 507 (91·7%) of 553 participants were female, and the median time since the onset of Raynaud's phenomenon was 4·0 years (IQR 1·7-10·0). At baseline, 401 (73·7%) of 544 patients with Raynaud's phenomenon had detectable ANA, with 208 (39·5%) of 527 patients positive for systemic sclerosis-specific autoantibodies. Nailfold capillaroscopy abnormalities were present in 182 (36·0%) of 505 patients and puffy fingers were detected in 96 (17·8%) of 540 at baseline. 1885 follow-up visits were recorded. 254 (45·9%) of 553 patients completed the study with progression or a 5-year follow-up; of whom, 133 reached the primary endpoint, resulting in an overall progression rate of 52·4%. The absence of ANA at baseline was the factor most strongly associated with a lack of progression within 5 years, with only four (10·8%) of 37 ANA-negative patients progressing. Conversely, positivity at baseline for systemic sclerosis-specific autoantibodies and puffy fingers was the combination having the highest risk of progression (16 [94·1%] of 17). Our results from the VEDOSS project offers a useful tool for a stratified risk approach to patients with Raynaud's phenomenon. The absence of ANA is a strong protective factor that identifies patients with very low risk of developing systemic sclerosis whereas the presence of one or two VEDOSS criteria in patients with Raynaud's phenomenon confers a progressively higher risk for systemic sclerosis over time. This stratification tool can be used both for clinical management and to inform early interventional trials. European Scleroderma Trial And Research and World Scleroderma Foundation.

Anticentromere Antibody Levels and Isotypes and the Development of Systemic Sclerosis.

ObjectiveLittle is known on the disease course of very early systemic sclerosis (SSc). Among the information yet to be elucidated is whether anticentromere antibody (ACA) isotype levels can serve as biomarkers for future SSc development and for organ involvement. This study was undertaken to evaluate whether IgG, IgM, and IgA ACA levels in IgG ACA–positive patients are associated with disease severity and/or progression from very early SSc to definite SSc.MethodsIgG ACA–positive patients from 5 different cohorts who had very early SSc or SSc fulfilling the American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) 2013 criteria were included. A diagnosis of very early SSc was based on the presence of IgG ACAs and Raynaud’s phenomenon, and/or puffy fingers and/or abnormal nailfold capillaroscopy, but not fulfilling the ACR/EULAR 2013 criteria for SSc. Multivariable regression analyses were performed to determine the association between baseline ACA isotype levels and progression to definite SSc with organ involvement.ResultsSix hundred twenty‐five IgG ACA–positive patients were included, of whom 138 (22%) fulfilled the criteria for very early SSc and 487 (78%) had definite SSc. Levels of IgG ACAs (odds ratio 2.5 [95% confidence interval 1.8–3.7]) and IgM ACAs (odds ratio 1.8 [95% confidence interval 1.3–2.3]) were significantly higher in patients with definite SSc. Of 115 patients with very early SSc with follow‐up, progression to definite SSc occurred within 5 years in 48 (42%). Progression to definite SSc was associated with higher IgG ACA levels at baseline (odds ratio 4.3 [95% confidence interval 1.7–10.7]).ConclusionACA isotype levels may serve as biomarkers to identify patients with very early SSc who are at risk for disease progression to definite SSc.

Nailfold Capillaroscopy Abnormalities Correlate With Disease Activity in Adult Dermatomyositis.

Objectives: The aim of this study was to determine the relationship between disease activity in adult patients with dermatomyositis (DM) and other biomarkers of disease activity such as C-reactive protein creatinine kinase and nailfold video capillaroscopy (NVC).Methods: We performed a prospective single center study of 15 adult patients with DM. Study participants underwent two assessments at least 9 months apart including clinical, laboratory and NVC evaluations. Patients received immunosuppressive medications for their dermatomyositis, and ongoing disease activity was measured by the Myositis Intention to Treat Index (MITAX). NVC evaluation included assessment of capillary density, capillary apical diameter (mm), and the number of microhemorrhages per digit.Results: Microvascular abnormalities were present in most DM patients. Of these, capillary density (4.71 vs. 6.84, p = 0.006) and mean apical diameter (56.09 vs. 27.79 μm, p = 0.003) significantly improved over the study period in concordance with improving disease control (MITAX 8.53 vs. 2.64, p = 0.002). Longitudinal analysis demonstrated that capillary density was independently associated with MITAX (β = −1.49 [CI −2.49, −0.33], p = 0.013), but not other parameters such as C-reactive protein and creatinine kinase.Conclusions: Nailfold capillary density is a dynamic marker of global disease activity in adult DM. NVC may be utilized as a non-invasive point-of-care tool to monitor disease activity and inform treatment decisions in patients with DM.

Nailfold capillaroscopy changes associated with anti-RNP antibodies in systemic lupus erythematosus.

Anti-U1RNP antibody is associated with distinct organ involvement in patients with systemic lupus erythematosus (SLE). Nailfold capillaroscopy (NFC) allows non-invasive assessment of microvascular abnormalities in several connective tissue diseases. The objective of this study is to determine the association of anti-U1RNP antibody with microvascular changes by NFC in RNP-positive SLE patients in comparison with RNP-negative SLE patients (negative disease controls) and mixed connective tissue disease (MCTD) cases (positive disease controls). NFC examination was performed in consecutive patients with SLE with or without anti-U1RNP positivity. MCTD patients were recruited as disease controls. Abnormalities noted in the three groups were compared using non-parametric tests. Ordinal logistic or linear regression was used wherever applicable. 81 patients were studied, of whom 28 were diagnosed as RNP-positive SLE (age 30.0 ± 10.37; 26 females), 26 were RNP-negative SLE (age 29.42 ± 9.20; 25 females) and 27 had MCTD (age36.5 ± 9.70; 25 females). RNP-positive SLE patients had more frequent giant capillaries, enlarged capillaries and ramified capillaries as compared to RNP-negative SLE (p = 0.05, < 0.01 and 0.03, respectively). The capillary density was lower in patients with MCTD as compared with patients withRNP-positive SLE (5.11 ± 1.69/mm vs 7.25 ± 1.38/ mm, p < 0.01) and RNP-negative SLE (8.92 ± 1.13/mm, p < 0.01). The reduction in capillary density was less severe in patients withRNP-negative SLE as compared with RNP-positive SLE (OR = 0.1058 [95% CI = 0.02-0.546], p < 0.01) which wasindependent of the presence of Raynaud'sphenomenon, interstitial lung disease and disease duration. Presence of anti-U1RNP antibody is associated with notable patterns of microvascular abnormalities in SLE. These NFC abnormalities are noted more profoundly in patients with MCTD and are less marked in RNP-negative SLE patients.

The aetiopathogenic significance, clinical relevance and therapeutic implications of vasculopathy in idiopathic inflammatory myopathy.

It is 120 years since 'angiomyositis' was included alongside 'polymyositis' and 'dermatomyositis' in an attempt to propose a taxonomy that reflected the major clinical characteristics of idiopathic inflammatory myopathy (IIM). Endothelial injury, perivascular inflammation and capillary loss are important histological findings in affected tissues in IIM. Overt vascular clinical features including RP and abnormal nailfold capillaroscopy (NC) are also common in IIM. Despite the presence of endothelial injury, perivascular inflammation and capillary loss in affected tissues in IIM, and the presence of clinical features such as RP and NC abnormalities, the pathogenic and therapeutic implications of vasculopathy in IIM have been somewhat overlooked. RP and NC abnormalities are not always present, providing a valuable opportunity to explore aetiopathogenic factors driving vasculopathy within autoimmune rheumatic disease. The present review examines the aetiopathogenic, prognostic and therapeutic significance of vasculopathy in IIM. We describe the prevalence and clinical relevance of vasculopathy in IIM, and consider how vasculopathy may be better utilized to support improved IIM diagnosis and disease classification. Areas of unmet research need are highlighted where relevant.

O10 A case of sarcoidosis mimicking Sjögren’s syndrome along with abnormal nailfold capillaroscopy

Case report - IntroductionSicca symptoms and Raynaud’s disease are amongst the common presentation of Sjögren’s syndrome (SS) which has an estimated prevalence of 1%. We are reporting a case that was initially considered as SS, but subsequent lip biopsy confirmed histological evidence of sarcoidosis in the presence of abnormal nailfold capillaroscopy and negative autoimmune profile. Sarcoidosis can be associated with various autoimmune diseases including systemic sclerosis, Hashimoto's thyroiditis, systemic lupus erythematosus, rheumatoid arthritis, Sjögren’s syndrome and spondyloarthropathy.Case report - Case descriptionA 29-year-old Caucasian man was referred to rheumatology clinic, suffering with ongoing sicca symptoms including dry itchy gritty eyes, sore red eyelids, persistent mouth dryness and cracking ulcers. He has history of Raynaud’s disease, dysphagia for solid, dry cough and photosensitive rash on his chest and arms. His other symptoms were polyarthralgia, myalgia, headache, persistent fatigue, poor memory, dry skin over the hands and tingling sensation at the feet. There was no history of joint swelling, uveitis, chest pain or shortness of breath. He has hypothyroidism secondary to radioiodine treatment and is currently on levothyroxine. He works in a factory, drinks alcohol occasionally and is non-smoker. There was no family history of autoimmune conditions.General and systemic examination was unremarkable, and there were no stigmata of connective tissue disease except for tenderness and crepitus at the right knee joint. His saliva flow rate was 0.4ml/5 mins and Schirmer’s test was 20mm/5 mins (both were normal values).Autoimmune profile was negative; ANA: 0.2 units, RF factor: <10, CCP antibodies (IgG): 1.1U/mL. Apart from mild lymphopenia, the other laboratory tests were at normal range including CK, CRP, adjusted calcium, ACE, immunoglobulins, serum protein electrophoresis and urine test for protein. Chest X-ray and lung function test were both normal.Nailfold capillaroscopy shows grade1 enlarged capillaries, prominent capillary ramifications in the form of bushy capillaries and some microhaemorrhages, a pattern suggestive of mixed connective tissue disease.Lip biopsy showed small non-caseating epithelioid granuloma within the parenchyma.The initial working diagnosis was a possible serology-negative SS, but the later results of lip biopsy confirmed sarcoidosis.He was commenced on Hydroxychloroquine and advised about cognitive-behavioural therapy for generalised ache and fatigue. As he was not responding to pilocarpine, a referral for ophthalmology review was requested.Case report - DiscussionSarcoidosis is a multisystem disease of unknown aetiology, known to exist worldwide with a variable prevalence of around 20 per 100,000 in the UK. It is characterized by the presence of multiple non-caseating granulomas, inflammation which may occur in any tissue in the body manifested as local symptoms with or without systemic features. Involvement of the salivary and lacrimal glands can result in xerostomia and xerophthalmia, respectively.Chronic sarcoid has an often subtle, insidious, progressive, and highly variable clinical course; it can be asymptomatic in a significant percentage of the cases.In addition to the clinical and radiological findings, the diagnosis of sarcoidosis should be based on the histological proof of non-caseating granulomas and ruling out other diseases that shares similar features.SS has many features in common with sarcoidosis; these may include sicca symptoms, arthralgia, myalgia, arthritis, erythematous rash, lymphadenopathy, peripheral neuropathy, fatigue and raised inflammatory markers. Moreover, positive rheumatoid factor, defective T suppressor cell regulation and HLA-DR3 are linked to both diseases. Pulmonary involvement in either of them may have similar clinical and radiographic manifestations, making it difficult for the clinician to distinguish between these diseases.In terms of management, hydroxychloroquine may help the skin and joint manifestations in both diseases whereas more severe diseases may require treatment with glucocorticoids, methotrexate, and azathioprine.Nevertheless, prompt confirmation of the diagnosis is crucial provided the difference in response to medication, complications, outcome, and prognosis.Based on the clinical and lip biopsy findings, the above case was diagnosed with sarcoidosis. The case did not meet the criteria for the diagnosis of SS provided the negative results of Schirmer’s test, saliva flow test and autoimmune profile.Case report - Key learning pointsRaynaud’s phenomenon (secondary Raynaud’s) with positive capillaroscopy findings was an unusual early presenting feature of sarcoidosis in the above case. There was one case report of similar presentation published in 2011. However, literature did not reveal specific information about abnormal nailfold capillaroscopy results in sarcoidosis.Dry eyes and dry mouth may occur in sarcoidosis mimicking the presentation of primary SS. In addition, true coexistence of sarcoidosis and SS has also been defined based on the histopathologic examination of the exocrine glands. Diagnostic criteria need to be applied for patients with suspected overlap of the two diseases.Clinicians will need to be vigilant and perform appropriate investigations for overlapping rheumatic conditions with sarcoidosis. Lip biopsy remains a crucial investigation for cases referred with sicca features, not only to establish the diagnosis of SS but also to exclude associated lymphoma or sarcoidosis.

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.

Pulmonary Manifestations in Systemic Sclerosis: Hospital-Based Descriptive Study.

IntroductionPrevalence of systemic sclerosis (SSc)-related organ injury is difficult to estimate as it occurs early in SSc, even though patients are often asymptomatic. As the patients with organ damage have a poor prognosis, all the patients should be carefully evaluated and followed‑up in the initial periods. This facilitates the early identification and initiation of appropriate therapy. This study emphasizes on different clinical manifestations and early predictors of lung involvement by using clinical, radiological, and pulmonary function tests in a tertiary care centre. Materials and methodsA total of 53 SSc cases, who satisfied American College of Rheumatology (ACR) 2013 criteria, without any overlap syndromes were included in the study. All patients underwent thorough clinical examination along with Modified Rodnan Scoring (MRS) assessment, nailfold capillaroscopy (NFC), chest X-ray (CXR), HRCT thorax, 2D-echocardiography, spirometry and diffusion lung study by carbon monoxide (DLco).ResultsOut of 53 patients, four were male and 49 were female. Twenty-one patients had limited SSc (lcSSc) and 32 had diffuse SSc (dcSSc). Eighty-three per cent of subjects presented with skin manifestations and 34% with respiratory complaints. Reticulonodular opacities and ground glassing were the predominant radiological abnormalities suggestive of non-specific interstitial pneumonia (NSIP) followed by usual interstitial pneumonia (UIP). Pulmonary hypertension was predominant in patients with lcSSc. Thirty-eight patients had a restrictive pattern of spirometry. Forty-four patients showed deranged DLco, among which two patients showed an isolated decrease in DLco. Thirty-seven patients had abnormal NFC among which dropout pattern was predominant. MRS was significantly correlated with pulmonary involvement by DLco and HRCT.ConclusionsSSc can affect the lungs even before developing obvious clinical pulmonary manifestations. DLco and HRCT play a critical role in detecting early lung involvement and predicting the outcomes in SSc. Higher modified Rodnan’s score, which has a significant correlation with DLco and HRCT can be used to predict early visceral involvement in resource-limited settings.

The Comparison of Nailfold Capillaroscopy between Juvenile Systemic Lupus Erythematosus and Healthy Controls: Correlation with Laboratory and Clinical Parameters.

Background Nailfold capillaroscopy is a noninvasive technique to recognize peripheral microangiopathy, which is an important feature in systemic lupus erythematosus (SLE). The aims of the present study were to investigate the prevalence of nailfold capillaroscopy (NFC) changes in juvenile systemic lupus erythematosus (JSLE), find out patterns of these changes, and correlate findings with clinical and laboratory parameters. Methods Forty-nine patients with SLE, all meeting the 1997 revised ACR criteria for SLE classification, and 30 healthy controls were included. A digital video camera was used to capture nailfold capillary images. Computerized image processing was used for analysis. Results Different abnormal NFC changes were observed with abnormal morphology, the increased diameter and abnormal loop densities in 55.1%, 93.9%, and 26.5% of the patients, respectively. No statistically significant differences were depicted between capillaroscopy with age, gender, autoantibodies (APLs, anti-ds DNA), antiphospholipid antibody syndrome, thrombotic angiopathy, renal function tests (Bun, Cr), and abnormal urine analysis. However, a significant correlation was found between the branched pattern and the CNS involvement group (P value <0.03). Conclusions Different abnormal NFC changes are quite common among patients with SLE, and nailfold capillaroscopy is an effective method to monitor such changes. Treatment strategies may change in the branched pattern of nailfold capillaroscopy due to CNS involvement.