| Summary: | Sjogren’s syndrome an autoimmune disease involving mainly the exocrine glands, resulting in dryness of the mucous surface (especially the oral and ocular ones). However, the clinical presentation can range from simpler manifestations such as mucosal dryness, arthralgias, and modest purpura, to even relevant systemic manifestations; association with lymphoma, especially non-Hodgkin lymphoma, is also frequent. Histologically, SS is characterized by a lymphocitic infiltration of tissues resulting in their destruction. Patho- genetic mechanisms are not fully understood but cellular B hyperactivity with auto-antibody production plays an important role. The main immunological markers are anti-nuclear antibodies (the most frequently detected), anti-Ro/SSA, and La/SSB (the most specific). It is also important to recognize cryoglobulinemia, hypergammaglobulinemia, hypocomplementemia, and rheumatoid factor positivity as prognostic markers; this may help define to whom to target more aggressive treatments. Indeed, this review aims to focus on the practical management aspects of the patient with SS, focusing on diagnosis and treatment. Referring to diagnosis, it is important to emphasize that although several classificatory criteria have been developed over the years, these do not represent diagnostic criteria; the diagnosis is up to the clinician, possibly aided by instrumental investigations including ultrasound, high-frequency ultrasound (useful as helping instru- ment in labial biopsy) and magnetic resonance of parotids. Instead, treatments (from the symptomatic ones to new biological therapies) should be modulated on the severity and the organ commitment of the disease, monitoring serologic changes and stratifying patients for the risk of developing NHL, in order to choose where to apply earlier and more aggressive therapies.
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