Three cases of C-ANCA-positive vasculitis treated with immunoadsorption: possible benefit in early treatment.
Wegener's granulomatosis is a vasculitic disease predominantly affecting the upper respiratory tract, lungs, and kidneys. Three patients with Wegener's granulomatosis and rapidly progressive glomerulonephritis were treated with an intensified regimen of immunoadsorption (IA) (Excorim or Therasorb) in addition to cyclophosphamide (CYC) and methylprednisolone (PRE). Patient A had been in remission under oral CYC/PRE. The first exacerbation was treated successfully with 4 IA treatments without changing medication. Patient B experienced 3 flares within 1 year, which were treated with 28 IA (3-7 IAs/course), intravenous CYC after each course, and PRE. A fall of creatinine levels from 120 to 190 micromol/L to 100 micromol/L was noted after IA and before administration of CYC. Patient C presented in uremia. Autoantibodies were eliminated by 11 IA treatments parallel to CYC/PRE therapy. They remained within a normal range for >1 year's follow-up; however, kidney function did not return. In conclusion, the observations in Patients A and B suggest a beneficial therapeutic effect of early IA in WG.
Labor Müller, Rostock, Germany.
G
G
Matic
A
A
Michelsen
D
D
Hofmann
R
R
Winkler
M
M
Tiess
J M
JM
Schneidewind
W
W
Müller
W
W
Ramlow
D000328:Adult; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D005921:Glomerulonephritis / Q000150:complications / Q000276:immunology / Q000628:therapy*; D005938:Glucocorticoids / Q000627:therapeutic use; D014890:Granulomatosis with Polyangiitis / Q000150:complications / Q000276:immunology / Q000628:therapy*; D006801:Humans; D007163:Immunosorbent Techniques; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D008875:Middle Aged; D010951:Plasma Exchange; D010956:Plasmapheresis
D019268:Antibodies, Antineutrophil Cytoplasmic; D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D008775:Methylprednisolone