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Background:Sjögren Syndrome (SS) is a rheumatic connective tissue disease in which vascular involvement (e.g. Raynaud’s phenomenon) may occur. No systematic review exists elucidating on the role of nailfold capillaroscopy in SS.Objectives:To give a standardised overview of capillaroscopic changes and clinical associations in SS.Methods:The literature was searched through in three databases by two reviewers. All published original studies which assess patients with SS by capillaroscopy were revised. A quality assessment was applied, based on sample size, population description, presence of a control group, presence of instrumental specifications and/or standardly applied capillaroscopic methodology, presence of clear descriptions of capillaroscopic parameters and based on the used statistical analysis. The capillaroscopic findings were described in a EULAR consented standardised way (1). Significant associations of capillaroscopic parameters in SS-patients with clinical and laboratory variables were also reported.Results:The literature search resulted in 826 hits. Based on title and abstract screening 519 original studies were retained and of these, 12 full texts described an assessment by nailfold capillaroscopy in SS. Six studies (four case-control studies and two case-series) were retained after performing a critical quality assessment (fig 1). EULAR standardised description (table 1) attested conclusive results for capillary ‘morphology’, suggesting a not higher prevalence of abnormal shapes in SS than in healthy (2,3). Concerning clinical associations, capillary density was associated with Raynaud in two studies and with interstitial lung disease in one study (2-4). No association between serologic features (anti-nuclear antibodies, anti-SSA, anti-SSB and anti-RF) and capillaroscopic parameters were found (2,5).Table 1.Standardised description of capillaroscopic characteristics in Sjögren’s Syndrome vs Healthy Legend. Only studies mentioning p-values were considered in this table. *Tektonidou et al. did not report differences between healthy controls and the SS group as a whole, but rather reported differences between healthy controls and subgroups of patients with/without Raynaud’s and with/without centromere antibodies.AssessmentParameterSignificantNot significantConclusionQuantitativeDensity1 study [2]1 study [3]Not conclusiveDimensionDilationGiant0 studies0 studies0 studies1 study [2]Not conclusiveNormal morphologyHairpin shapedTortuosityCrossing0 studies0 studies0 studies0 studies1 study [3]1 study [3]Not conclusiveAbnormal morphology0 studies2 studies [2,3]Equal absence of abnormal morphology in SS vs healthy controlsHaemorrhages0 studies2 studies [2,3]Not conclusive*Semi quantitative0 studies0 studiesNo dataQualitativeNormalNon specific abnormalitiesScleroderma pattern0 studies0 studies0 studies1 study [3]1 study [3]0 studiesNot conclusive*Not conclusive*No dataConclusion:A small number of studies have investigated the role of nailfold capillaroscopy in SS. Prospective follow up studies with standard evaluation and capillaroscopy in SS are warranted.References:[1]Smith et al Autoimmun Rev 2020, DOI: 10.1016/j.autrev.2020.102458[2]Capobianco et al Clin Exp Rheumatol 2005;23(6):789-94[3]Tektonidou et al Rheumatology (Oxford, England) 1999;38(9):826-30[4]Cakmakci et al Tuberk Torakx 2015;63:22-30[5]Corominas et al Rheumatol Int 2016;36(3):365-9Disclosure of Interests:Karin Melsens: None declared, Maria C. Leone: None declared, Sabrina Paolino: None declared, Maurizio Cutolo Grant/research support from: Bristol-Myers Squibb, Actelion, Celgene, Consultant of: Bristol-Myers Squibb, Speakers bureau: Sigma-Alpha, Dirk Elewaut: None declared, Roberto Gerli: None declared, Isabelle Peene: None declared, Vanessa Smith Grant/research support from: The affiliated company received grants from Research Foundation - Flanders (FWO), Belgian Fund for Scientific Research in Rheumatic diseases (FWRO), Boehringer Ingelheim Pharma GmbH & Co and Janssen-Cilag NV, Consultant of: Boehringer-Ingelheim Pharma GmbH & Co, Speakers bureau: Actelion Pharmaceuticals Ltd, Boehringer-Ingelheim Pharma GmbH & Co and UCB Biopharma Sprl

The difference between a normal capillaroscopy and an “abnormal” capillaroscopy is important in the differential diagnosis between a primary Raynaud9s phenomenon (=not linked to any condition) from a secondary RP. A normal capillaroscopic pattern, by qualitative assessment, is characterized by a homogeneous distribution of hairpin shaped capillaries as a “comb-like structure”, with a density of between 9–14 capillaries per mm. Yet, there exists a wide intra- and inter-individual variety in a normal population (non-specific variations) which will be discussed during the workshop. An abnormal capillaroscopic image due to systemic sclerosis (SSc) is characterized by (a combination) of specific changes (giants, capillary loss, hemorrhages and abnormal shapes (ramifications)). Depending on their relative prevalence three SSc-patterns are described by Cutolo et al. with a 200 magnification videocapillaroscope: the “early”, “active” and “late” scleroderma pattern. The combination of the presence of a scleroderma pattern on capillaroscopy with the presence of an SSc-specific antibody in a patient with Raynaud9s phenomenon allows the “early” diagnosis of SSc. When the patient also has “puffy” fingers he/she meets the VEDOSS criteria for very early diagnosis of SSc. In patients with connective tissue diseases “other than” SSc and diseases of the scleroderma spectrum there are no “unique” capillary patterns. A variety of capillary abnormalities (change in morphology, dimension, presence of hemorrhages) have been observed. These abnormalities by themselves are not predictive of any defined condition and may be referred to as non-specific abnormalities. Disclosure of Interest None declared

The importance of distinguishing normal capillaroscopy findings form (pathognomonic) abnormal (pathological) findings, lies in the fact that this distinction allows the differentiation between a primary Raynaud’s phenomenon characterized by normal capillaroscopic findings from a secondary RP due to systemic sclerosis (SSc) and diseases of the scleroderma spectrum, characterized by (patognomonic) abnormal findings on capillaroscopy.[[1][1]] Diverse terminology had been described for RP, LeRoy and Medsger proposed criteria for the strict definition of primary RP and of secondary RP due to SSc. Amongst others these criteria encompass capillaroscopy.[[2][2]]To be able to speak of a primary RP, these criteria require a normal capillaroscopic pattern. In the normal capillary pattern capillaries of the distal row of the nailfold have an open hairpin shape, a homogenous size, are regularly arranged in a parallel fashion and their number ranges between 6 to 14 per mm with a mean of 9. The morphological pattern of nailfold capillaries is remarkably constant in normal individuals.[[3][3]] However, across the population there exists a wide variation in the ranges of the normal morphology. Andrade et al. reported capillaroscopic findings on the largest described healthy persons study population (800 healthy persons). In this study, also morphological anomalies were seen in 34% of the whole healthy person population but few in each individual. However, certain features, such as giant capillaries or extensive avascularity seem not to be present in a normal population and should be considered as pathological. On the contrary, patients with a secondary Raynaud’s phenomenon due to SSc show pathognomonic abnormal (pathological patterns) patterns on capillaroscopy. The majority of patients with clinically recognizable SSc show, very characteristic combination of capillary abnormalities (giant capillaries, loss of capillaries in the nailfold, hemorrhages and abnormal shapes [= neoangiogenesis], such as ramifications), the “scleroderma-type” changes, which can easily be assessed through pattern recognition.[[4][4]] Recently, Cutolo et al. qualitatively assessed the nailfolds of an SSc cohort with patients fulfilling the American College of Rheumatology (ACR) criteria for SSc with the nailfold videocapillaroscopy (NVC) technique (magnification X200). According to the relative prevalences of those abnormalities he classified the patterns found in SSc into the “early”, the “active” and the “late” pattern. Besides this, the detection of pathognomonic abnormal capillaroscopic patterns on capillaroscopy also allows us to diagnose SSc “early”, before skin involvement has occurred. In this way LeRoy and Mesdger proposed in 2001 criteria for the “early” diagnosis of SSc, with RP as single criterion. These criteria incorporate SSc-specific autoimmune antibodies and microvascular techniques, more specifically the “scleroderma-type” changes on capillaroscopy. These criteria have been recently validated in a large prospective study.[[5][5]] Of note, also in more recently proposed criteria for very early diagnosis of SSc (VEDOSS) these pathological patterns on capillaroscopy play an important role. 1. Smith V, Cutolo M. When and how to perform the capillaroscopy. In: Cutolo M, ed. Atlas of capillaroscopy in Rheumatic diseases. Milano: Elseviern Srl. 2010 2. LeRoy EC, Medsger TA, Jr. Raynaud’s phenomenon: a proposal for classification. Clin Exp Rheumatol . 1992; 10: 485-8. 3. Andrade LE, Gabriel Junior A, Assad RL, Ferrari AJ, Atra E. Panoramic nailfold capillaroscopy: a new reading method and normal range. Semin Arthritis Rheum . 1990; 20: 21-31. 4. Cutolo M, Sulli A, Pizzorni C, Accardo S. Nailfold videocapillaroscopy assessment of microvascular damage in systemic sclerosis. J Rheumatol . 2000; 27: 155-60. 5. Koenig M, Joyal F, Fritzler MJ, Roussin A, Abrahamowicz M, Boire G, et al. Autoantibodies and Microvascular Damage Are Independent Predictive Factors for the Progression of Raynaud’s Phenomenon to Systemic Sclerosis A Twenty-Year Prospective Study of 586 Patients, With Validation of Proposed Criteria for Early Systemic Sclerosis. Arthritis Rheum Disclosure of Interest None Declared [1]: #p-3 [2]: #p-4 [3]: #p-5 [4]: #p-6 [5]: #p-7