Epidemiology of Cutaneous Involvement in Sjögren Syndrome: Data from Three French Pss Populations (TEARS, ASSESS, Diapss)

Background: Cutaneous involvement is common during primary Sjogren’s Syndrome (pSS) but prevalence and characteristics are difficult to establish precisely because of the limited number of patients studied in most cohorts, the variability of the disorders evaluated in each cohort, the rarity of some of them, and the heterogeneity of evaluations from previous studies (1). Objectives: To determine the prevalence and significance of dermatological disorders in primary Sjogren Syndrome. Methods: We use 2 French cohorts (ASSESS, in which prevalence of skin disorders in 395 pSS patients was evaluated, and diapSS in which 91 consecutive pSS patients had an examination by a dermatologist) and baseline data of the TEARS randomized trial (110 patients with recent or active pSS, treated with rituximab or placebo, and evaluated for skin dryness using a visual analogue scale out of 100). Results: Skin manifestations included in the ESSDAI were rare in the ASSESS cohort (n=16/395, 4.1%, mainly purpuras; only 3 had high activity) but associated with activity in the other ESSDAI domains (peripheral neurological (p<0.001), muscular (p=0.01), hematological (p=0.017) and biological (p=0.017)), history of arthritis (p=0.008), splenomegaly (p=0.024) and higher gamma globulin level (p=0.008)) (Table). Compared to pSS patients not receiving a dermatological consultation, the pSS patients who had a dermatological consultation had significantly more dermatological involvement outside ESSDAI score [42% (29/69) versus 19.6% (11/56); p=0.008]. The TEARS study showed a high prevalence of cutaneous dryness (VAS>50; 48.2%) and that these dry skin patients had higher pain VAS (61.5+/-28.2 vs 46.8+/-27.0; p=0.003) and drought (79.4+/-15.2 vs 62.5+/-21.7; p<0.0001). Cutaneous involvement No Cutaneous involvement Total p values* Muscular 3/16 (18.8) 10/373 (2.7) 13/389 (3.3) 0.001 Peripheral nervous system PNS 4/16 (25) 34/373 (9.1) 38/389 (9.8) 0.00001 Biological 8/16 (50) 138/371 (37.2) 146/387 (37.7) 0.017 Hematologic 7/16 (43.8) 55/373 (14.7) 62/389 (15.9) 0.017 History of arthritis 12/16 (75) 154/374 (41.2) 166/390 (42.6) 0.008 History of splenomegaly 2/16 (12.5) 10/3762.7) 12/392 (3.1) 0.024 History of lymphoma 0/16 (0) 18/379 (4.7) 18/395 (4.6) 0.372 Mean (SD) ESSDAI score 14.5 (6.8) 4.4 (5.1) 0.00001 Mean (SD) ESSDAI score after excluding the points awarded by skin manifestations 8.1 (6.2) 4.4 (5.1) 0.014 Gammaglobulin levels (mean +/- SD) 23.1 +/-7.3 18.5 +/-8.1 - 0.006 Conclusion: The most common skin disorder is dryness, which is associated with a higher level of pain and overall subjective dryness. ESSDAI skin activity is rare, associated with hypergammaglobulinemia and ESSDAI activity. Systematic dermatological examination is informative for non-specific pSS lesions References: [1]Orgeolet L, Foulquier N, Misery L, Redou P, Pers J-O, Devauchelle-Pensec V, et al. Can artificial intelligence replace manual search for systematic literature? Review on cutaneous manifestations in primary Sjögren’s syndrome. Rheumatol Oxf Engl. 2019 Aug 31; Disclosure of Interests: Camille Villon: None declared, Laure Orgeolet: None declared, Anne-Marie Roguedas-Contios: None declared, Laurent Misery: None declared, Jacques-Eric Gottenberg Grant/research support from: BMS, Pfizer, Consultant of: BMS, Sanofi-Genzyme, UCB, Speakers bureau: Abbvie, Eli Lilly and Co., Roche, Sanofi-Genzyme, UCB, Divi Cornec: None declared, Sandrine Jousse-Joulin: None declared, Raphaèle Seror Consultant of: BMS UCB Pfizer Roche, Jean-Marie Berthelot: None declared, Philippe Dieudé: None declared, Jean-Jacques Dubost: None declared, anne-laure Fauchais: None declared, Vincent Goeb: None declared, Eric Hachlla: None declared, Pierre-Yves Hatron: None declared, Claire Larroche: None declared, Gilles Hayem: None declared, Véronique LE GUERN Grant/research support from: UCB for GR2 study (to our institution), Aleth Perdriger: None declared, Jacques Morel: None declared, Olivier VITTECOQ: None declared, Xavier Mariette Consultant of: BMS, Gilead, Medimmune, Novartis, Pfizer, Servier, UCB, Valerie Devauchelle-Pensec: None declared, alain saraux: None declared