T cells and in situ cryoglobulin deposition in the pathogenesis of lupus nephritis.
We discuss a 53-year-old woman with systemic lupus erythematosus who presented with vasculitis, hypocomplementemia and nephritis. Although her serum complement 4 (C4) levels were zero, she had four copies of C4 gene. Renal biopsy revealed membranoproliferative glomerulonephritis and the presence of cryoglobulins, detected by electron microscopy, and significant numbers of T cells in the interstitium. Cryoglobulins were considered responsible for the complete consumption of C4 in the serum the levels of which improved gradually after treatment. T cells in the kidney were found to express CD44 and phosphorylated ezrin/radixin/moiesin which explain why they homed to the kidney inappropriately. The contribution of cryoglobulins and T cells in the expression of kidney pathology is discussed.
Department of Medicine, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA 02115, USA.
Robert A
RA
Cohen
George
G
Bayliss
Jose C
JC
Crispin
Gwen F
GF
Kane-Wanger
Christine A
CA
Van Beek
Vasileios C
VC
Kyttaris
Ingrid
I
Avalos
C Yung
CY
Yu
George C
GC
Tsokos
Isaac E
IE
Stillman
D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D003181:Complement C4 / Q000172:deficiency; D003450:Cryoglobulins / Q000378:metabolism*; D005260:Female; D005455:Fluorescent Antibody Technique; D006801:Humans; D018960:Hyaluronan Receptors / Q000378:metabolism; D008181:Lupus Nephritis / Q000276:immunology* / Q000473:pathology* / Q000503:physiopathology*; D046529:Microscopy, Electron, Transmission; D008875:Middle Aged; D009173:Mycophenolic Acid / Q000031:analogs & derivatives / Q000627:therapeutic use; D011241:Prednisone / Q000627:therapeutic use; D013601:T-Lymphocytes / Q000276:immunology* / Q000378:metabolism
D000893:Anti-Inflammatory Agents; C497483:CD44 protein, human; D003181:Complement C4; D003450:Cryoglobulins; D018960:Hyaluronan Receptors; D009173:Mycophenolic Acid; D011241:Prednisone