Abstract
Sulfasalazine (SSZ) can induce serological and clinical autoimmune reactions but the occurrence of SSZ‐related Wegener's granulomatosis (WG) has not been reported before. We describe two patients with rheumatoid factor (RF)‐positive rheumatoid arthritis (RA) who developed biopsy‐proven WG with serious organ involvement during SSZ therapy. The pathogenetic mechanism that explains the relationship between SSZ and the occurrence of a de novo anti‐neutrophil cytoplasmic antibody (ANCA)‐related vasculitis or a flare is discussed. We propose that WG can be a rare complication of SSZ therapy and that this, like other autoimmune adverse events of this drug, is mediated by SSZ‐induced apoptosis.