Objectives: Systemic sclerosis (SSc) is a severe life-threatening autoimmune disease where little data on the effectiveness of anti-inflammatory and immunosuppressive therapy exist. It is not known to which extent patients are treated whith immunosuppressive agents in general practice. Therefore, within the German Network for Systemic Scleroderma, which gathers rheumatologists, dermatologists, pulmologists and nephrologists, the use of corticosteroid and immunosuppressive therapy in registered patients was investigated.
Methods: A one page questionnaire was developed which contained questions on duration and dosage of corticosteroids, type and dosage of immunosuppressants and type and dosage of UV-therapy.
Results: Data of 1556 patients from 21 centers were analyzed. Of these, 541 (34 %) received corticosteroid therapy and 805 (51 %) received no steroids. About 25 % of the patients receiving corticosteroids were treated with a dose exceeding 7.5 mg prednisone equivalents. Corticosteroid use was reported in 41.9 % of patients with diffuse cutaneous and 28 % of patients with limited cutaneous disease (p< 0.01). Among patients with overlap disease characteristics 55.8% received corticosteroids. Immunosuppressive therapy was conducted in 26.9 % of all patients. Again, among those with overlap symptoms a much higher number, 55.8%, was treated with immunosuppressants compared to 16.9 % of those with limited cutaneous disease and 36.5 % of those with diffuse cutaneous disease. The most commonly used compounds included methotrexate (7.6 %), azathioprine (6.3 %), cyclophosphamide (5.1 %), chloroquine (2.2 %), cyclosporine A (1.1 %) varying significantly in their use between medical subspecialties. High ESR, recent onset of disease, presence of lung fibrosis, joint involvement or male gender was associated with a significantly higher percentage of patients receiving steroid or immunosuppressive treatment . UV-therapy mainly consisting of UVA or PUVA therapy was reported in 10.1 % of patients.

Conclusion: This is the first study investigating the use of immunosuppressive and antifibrotic therapy in a large cohort of SSc patients in daily clinical practice. The study reveals considerable variations of therapeutic regimens among different medical disciplines and, in particular, a high percentage of corticosteroid use. These data will form the framework for following and comparing the outcome of the development of therapeutic guidelines on a national and international level.