Les complications systémiques de la maladie de Sjögren (ou syndrome de Sjögren primitif)

Primary Sjögren syndrome (pSS) is a progressive autoimmune disease characterized by sicca and systemic manifestations that are frequent and potentially severe, especially when related to B cell activity or the presence of cryoglobulinemia, but the pathological role of the anti-SSA antibodies has never been demonstrated. The frequency of the systemic manifestations in pSS is differentially evaluated related to disease duration or the methods used to detect them, but they are supposed to concern 20 to 70% of the patients. The systemic manifestations can be artificially divided in two types: the life-threatening manifestations (lymphoma, vasculitis with cryoglobulinemia, central nervous system involvement, ganglioneuronopathy and membranoproliferative glomerulonephritis) and the others manifestations. However, the mortality rate in pSS is similar to the general population. The more frequent and easy to evaluate systemic manifestations have been gathered in the ESSDAI score (EULAR Sjogren's Syndrome Disease Activity Index), an international consensual score used in clinical trials and recently incorporated in the STAR index (Sjogren's Tool for Assessing Response).