AB1437 NAILFOLD CAPILLAROSCOPY IN DEFICIENCY OF ADENOSINE DEAMINASE 2 (DADA2): A CASE-CONTROL STUDY

Background Deficiency of adenosine deaminase 2 (DADA2) is a rare monogenic autoinflammatory disease characterized by the presence systemic inflammation, vasculitis, early-onset stroke, and hematologic manifestations such as cytopenia immunodeficiency. Mucocutaneous peripheral microvascular manifestations, livedo racemosa, cutaneous ulcers Raynaud’s phenomenon (RP), can occur, in up to 50%, 22% 8 % DADA2 patients respectively [1]. Objectives To investigate architecture nailfold capillaries comparing them with adequate healthy controls (HCs) primary RP individuals. Methods The data 9 followed at Istituto Gaslini were retrospectively retrieved compared 11 HCs 7 RP. Clinical genetic collected for patients. Nailfold videocapillaroscopy (NVC) findings whole cohort, independently from their current treatment. capillaroscopic parameters classified according Fast Track Algorithm distinguished between scleroderma-pattern (encompassing specific NVC alteration, giant and/or loss combined abnormally shaped capillaries) non-scleroderma patterns (non-specific alterations) [2]. A validated semiquantitative scale (score 0–3 each parameter) was used compare three groups [3] Results diagnosed testing. onset early infancy, while mean age 18.8 years. Livedo racemosa present (78%), digital 1 patient, an ischemic stroke reported 5 (55%). None had (0%). showed non-specific alterations all (capillary dilations 100%, haemorrages 23% patients). capillary density normal (median = 9) no scleroderma-like pattern found. No significant differences rates finding detected DADA2, HCs. Conclusion This first report investigating Microvascular damage does not show alteration but might suggest disease-related endothelial damage. References [1] Meyts I et al. J Clin Immunol 2018 [2] Smith V Autoimmun Rev 2019 Sulli Ann Rheum Dis. 2008 Acknowledgements: NIL. Disclosure Interests Declared.