Association of ANCA-associated vasculitis-rheumatoid arthritis overlap syndrome in four patients: rituximab may be the right choice?

Autoimmunity. 2012 Jun;45(4):304-9. doi: 10.3109/08916934.2012.677078. Epub 2012 Apr 10.

Abstract

Objectives: There have been few reports on the association of rheumatoid arthritis (RA) with anti-neutrophil cytoplasmic antigen (ANCA)-associated systemic vasculitides.

Methods: Here we present four cases of RA overlapping with Wegener's granulomatosis (WG), microscopic polyangiitis (MPA) and Churg-Strauss syndrome (CSS). All these cases had both the diagnosis of RA and that of ANCA-associated vasculitis.

Results: After we tried corticosteroids, multiple immunosuppressive drugs, sometimes plasmapheresis, rituximab was introduced to 3 out of 4 cases. Rituximab therapy was found to be effective in these three overlap cases. In the fourth case, rituximab was and will be considered; however, the patient has not given consent.

Conclusion: To our knowledge, this is the first report on RA+CSS association. Common pathogenic background, such as genetic predisposition and ANCA may account for the development of RA+vasculitis overlaps. In DMARD-refractory cases, such as the ones presented here, biologics, especially rituximab may be highly effective.

MeSH terms

  • Adult
  • Aged
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / diagnosis
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / drug therapy*
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / immunology
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / pathology
  • Antibodies, Antineutrophil Cytoplasmic
  • Antibodies, Monoclonal, Murine-Derived / therapeutic use*
  • Arthritis, Rheumatoid / drug therapy*
  • Arthritis, Rheumatoid / immunology
  • Churg-Strauss Syndrome / diagnosis
  • Churg-Strauss Syndrome / immunology
  • Churg-Strauss Syndrome / pathology
  • Female
  • Granulomatosis with Polyangiitis / diagnosis
  • Granulomatosis with Polyangiitis / immunology
  • Granulomatosis with Polyangiitis / pathology
  • Humans
  • Male
  • Microscopic Polyangiitis / diagnosis
  • Microscopic Polyangiitis / immunology
  • Microscopic Polyangiitis / pathology
  • Middle Aged
  • Rituximab
  • Syndrome

Substances

  • Antibodies, Antineutrophil Cytoplasmic
  • Antibodies, Monoclonal, Murine-Derived
  • Rituximab