In our cohort, pSS patients developing eMZL were characterized by a combination of intermittent parotid enlargement, cryoglobulinemic vasculitis, high focal score (≥4) (13), anti-Ro, anti-La and low complement at time of sampling (seven years to three months before eMZL diagnosis), while most of these risk factors (except anti-Ro) were not reported in the controls (Supplementary Table 4). Here, CALR is linked to peeling skin syndrome.