Over the last ten years various biologics have been introduced in the therapy of rheumatic diseases. The most successful approach so far has been the inhibition of TNF alpha, which is able to suppress systemic inflammation in an unprecedented manner. However, the early observation of newly developed antinuclear antibodies and some cases of drug-induced lupus has so far hampered the evaluation of this treatment option in larger clinical studies for collagen vascular diseases. Thus, ten years after treating the first RA patients with TNF alpha-Blockers we still only have reports of individual cases of patients with collagen vascular diseases treated with this principle. The following article reviews these single cases and summarizes the current knowledge about efficacy and safety of anti-TNF alpha-therapy for this indication.