Connective tissue disease

The connective tissue diseases consist of systemic lupus erythematodes, Sjögren`s syndrome, systemic sclerosis, poly- and dermatomyositis, anti-U1-RNP positive mixed connective tissue disease as well as undifferentiated connective tissue diseases and so called overlap syndroms (two connective tissue diseases existing at the same time).

Symptoms

The symptoms of connective tissue diseases are heterogeneous and dependent on the specific connective tissue disease. Frequent early manifestations include Raynaud’s phenomenon (color changes of the fingers following cold exposure) and arthritis. As connective tissue diseases are systemic autoimmune diseases, common symptoms also include fatigue, fever and specific organ dysfunctions. 

Diagnostics

The overall aim of any treatment approach is the prevention of irreversible organ damage. Therefore, early recognition of organ involvement is of key importance in the care of patients with connective tissue diseases. Due to the heterogeneity of organ involvements of the connective tissue diseases, the diagnostic work-up is often challenging and assessments have to be individualized to the specific patient. In the common outpatient clinic for connective tissue diseases, patients with all kind of connective tissue diseases are taken care of. Depending on the specific needs specialized diagnostic tests like nailfold capillaroscopy, validated muscle strength tests and other screening tests for organ involvement including echocardiography and lung function tests are ordered. We aim to perform the different organ screening tests within one day. Patients with multiple organ involvement often require assessments by different specialized disciplines. These patients are assessed in the interdisciplinary outpatient clinic for connective tissue diseases and are discusses in a plenary presentation. Specific assessments exist for patients with systemic sclerosis and inflammatory myopathies.

Therapy

Connective tissue diseases are chronic autoimmune diseases and therefore often require the treatment with immunosuppressive agents. If the immunosuppressive treatment is necessary and what kind of immunosuppressive medication is most adequate, is dependent on the stage and activity of the connective tissue disease. Specific therapies exist for the different connective tissue diseases and patients are informed about the treatment options during the consultations.