This line of research uses unique cohorts to study the interaction between immunological, clinical, and psychological (see collaborations) manifestations and mechanisms in these complex diseases. Both are considered to be generalized autoimmune diseases of unknown etiology.
SS is characterized by lymphocytic and plasma cell infiltration of exocrine glands, primarily salivary and lacrimal glands. Consequently, patients suffer from dryness of eyes and mouth, in addition to generalized symptoms including invalidating fatigue, reduced well-being, myalgia, and arthralgia. The course is usually relatively mild, but the disease carries the risk of developing malignant lymphoma. Extraglandular manifestations are present in 30-60% of the patients. A gold standard for the diagnosis of SS is not available. Serology (elevated ESR, serum IgG, and the presence of autoantibodies) and focal infiltration of inflammatory cells in exocrine, usually sublabial salivary glands, assumingly related to impaired exocrine function through destruction of gland tissue, are recognized as distinguishing.
Causative therapy is not available. Symptomatic therapy includes tear and saliva substitutes or secretagogues. In only a small part of patients with SS, extraglandular manifestations may require immune suppressive therapy.
SLE is characterized by a broad variety in clinical presentation and course. SLE may affect any organ system. Skin manifestations and non-erosive arthritis occur in most patients. Pericarditis and pleuritis may occur, whereas more than half of the patients present renal involvement necessitating immunesuppressive therapy. Manifestations the nervous system may lead to psychiatrical and neurological symptoms. Haematological involvement is frequently seen. Most patients have general symptoms such as anorexia and weight loss, fever and fatigue. SLE is characterized by exacerbations and periods without disease activity. Serologically striking are the presence of anti-nuclear antibody and antibodies to native DNA and the Sm(ith) antigen.
Prognosis has improved through more adequate diagnosis of especially milder cases of the disease, and through conscientious use of corticosteroids, hydroxychloroquine and the availability of immunosuppressive therapy including cyclophosphamide and mycophenolatemophetil.
For more information contact: Dr Ron Derksen (R.H.W.M.Derksen@umcutrecht.nl) for SLE or Dr Aike Kruize (email: A.Kruize@umcutrecht.nl) for SS