Immunoglobulin gamma3-heavy-chain deposition disease: report of a case and relationship with hypocomplementemia.
The authors describe a 54-year-old woman presenting with proteinuria, hematuria, and hypocomplementemia whose renal biopsy results showed diffuse increase in mesangial matrix and nodular formations in several glomeruli with the deposition of immunoglobulin gamma3-heavy-chain and complement components C1q and C3 in the glomeruli and on the tubular basement membranes, without associated light-chain deposits. Staining for the constant domains of gamma-heavy-chain showed a deletion of the first constant domain (CH1). These findings were consistent with those of gamma-heavy-chain deposition disease (gamma-HCDD). The patient was treated monthly with melphalan and prednisolone although a bone marrow aspirate did not show findings suggestive of plasmacytoma. Six courses of melphalan and prednisolone therapy resulted in a marked reduction of urinary protein excretion and marked rise of complement levels. The current case is the fourth HCDD patient reported featuring gamma3-heavy-chain deposition who showed severe hypocomplementemia and responded to chemotherapy with improved renal parameters and complement levels. A review of previously reported cases of HCDD showed that some but not all HCDD cases were associated with hypocomplementemia. The authors also discuss here the relationship of HCDD to hypocomplementemia.
Soma
Jun
J
Department of Nephrology, Iwate Prefectural Central Hospital, Morioka, Japan. sjun@chuo-hp.pref.iwate.jp
Sato
Kozo
K
Sakuma
Tsutomu
T
Saito
Hirotaka
H
Sato
Hiroshi
H
Sato
Toshinobu
T
Abbas
Acha
A
Aucouturier
Pierre
P
D003172:Complement C1 / Q000032:analysis; D003176:Complement C3 / Q000032:analysis; D003165:Complement System Proteins / Q000172:deficiency*; D005260:Female; D005921:Glomerulonephritis; D005938:Glucocorticoids / Q000627:therapeutic use; D006362:Heavy Chain Disease / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D006417:Hematuria / Q000209:etiology; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D007142:Immunoglobulin gamma-Chains / Q000032:analysis; D007668:Kidney / Q000276:immunology / Q000473:pathology* / Q000648:ultrastructure; D008875:Middle Aged; D011507:Proteinuria / Q000209:etiology
D003172:Complement C1; D003176:Complement C3; D005938:Glucocorticoids; D007074:Immunoglobulin G; D007142:Immunoglobulin gamma-Chains; D003165:Complement System Proteins