The leading symptoms of this autoimmune disease are xerostomia and xerophthalmia. Both are characterised by a lymphoplasmacellular destructive inflammation of salivary and lacrimal glands and the detection of autoantibodies against Ro/SS-A and La/SS-B nuclear antigens. In the long run of the disease, a polyclonal hypergammaglobulinemia is evolving as a consequence of chronic B-cell stimulation which can also lead to B-cell lymphoma which underlines the need for periodical clinical and laboratory examinations. Further extraglandular manifestations of the disease are non-erosive polyarthritis, serositis, leukocytoclastic cutaneous vasculitis and polyneuropathy, which make a clear differentiation from different forms of autoimmune connectivitis even more difficult, in particular if sicca syndrome is also present which may represent a secondary Sjögren's syndrome. The treatment is mostly symptomatic and immunosuppressive drugs are only given in case of severe and life-threatening organ involvement.