Scleroderma associated with ANCA-associated vasculitis.
Scleroderma and ANCA-associated vasculitides (AAV), such as microscopic polyangiitis, are distinct disease entities, but are rarely known to coexist with each other. We have reported on two cases of scleroderma patients for the first time in Korea, and these patients were initially known to have only limited type scleroderma with pulmonary fibrosis, but eventually they were found to be ANCA-positive with the associated clinical features of vasculitis. Both were treated with high-dose steroids and cyclophosphamide and remitted without major sequelae. When scleroderma patients exhibit atypical features such as normotensive renal failure with signs of active inflammation, the possibility of AAV should always be considered.
Rho
Young Hee
YH
Division of Rheumatology, Korea University Anam Hospital, 126-1, 5-ka Anam-dong, Seongbuk-Ku, Seoul, Korea, 136-705.
Choi
Seong Jae
SJ
Lee
Young Ho
YH
Ji
Jong Dae
JD
Song
Gwan Gyu
GG
D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood* / Q000276:immunology; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008875:Middle Aged; D012595:Scleroderma, Systemic / Q000150:complications*; D014657:Vasculitis / Q000150:complications* / Q000188:drug therapy
D019268:Antibodies, Antineutrophil Cytoplasmic; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide