Quand penser à un syndrome de Sjögren primitif ?

Abstract

Primary Sjögren's syndrome (SS) or Sjögren disease is an autoimmune disorder with a high female ratio (9:1) with a peak of frequency around 50-year-old, but there are also cases with early onset before the age of 20. Forms with the classic triad of dryness, fatigue and pain are a common mode of entry into the disease and account for about 30% of patients with primary SS. Systemic involvement with one or more visceral manifestations is seen in about 70% of cases. Late Raynaud's phenomenon, bronchiolar involvement, interstitial lung disease, synovitis, neuropathies (particularly sensory neuropathies), tubulointerstitial nephritis, and parotid enlargement are all possible entry points to the disease since the dryness may be in the background. Finally, SS should also be considered in cases of cryoglobulinemic vasculitis, which often combines membranoproliferative glomerulonephritis, purpura, livedo and multiple mononeuropathy. More rarely, it is the discovery of a MALT lymphoma or the occurrence of a congenital atrioventricular heart block that will lead to the diagnosis of SS.