[Primary Sjögren's Syndrome - News on Diagnostics and Therapy]

Primary Sjögren's Syndrome - News on Diagnostics and Therapy Abstract. Primary Sjögren's syndrome is an autoimmune disease that affects primarily the exocrine glands and is mainly characterized by sicca symptoms of the eyes and mouth, but also nose, throat, vagina and skin can be affected. Fatigue and pain are also very characteristic. Systemic manifestations can occur, e.g. in joints, muscles, lungs, kidneys, skin or the nervous system. A feared complication is the development of a lymphoma, the risk being especially high in case of positive anti-SSA-(Ro) antibodies, but also in case of a high ESSDAI score, hypocomplementemia, cytopenia or evidence of ectopic germinal centers in salivary gland biopsies. Diagnosis is principally made by verification of sicca symptoms (e.g. Schirmer's test) and detection of typical antibodies or a typical gland biopsy. Sicca symptoms are primarily treated symptomatically (e.g. artificial tears or saliva). In case of systemic manifestations glucocorticoids, conventional DMARDs or biologics (mostly rituximab) are treatment options. In severe cases, intravenous immunoglobulins or plasma exchange may be required.