A case of dense deposit disease associated with a group A streptococcal infection without the involvement of C3NeF or complement factor H deficiency.
A 14-year-old girl presented with acute glomerulonephritis. Tests revealed hypocomplementemia and elevated Antistreptolysin-O titers, and renal biopsy revealed endocapillary and mesangial proliferative glomerulonephritis with double contours of the glomerular basement membrane (GBM). Despite methylprednisolone pulse therapy and the administration of oral prednisolone, overt proteinuria and hypocomplementemia persisted. A second renal biopsy 6 months later confirmed the initial diagnosis of dense deposit disease (DDD) based on electron-dense deposits in the GBM. C3 nephritic factor (C3NeF) and a deficiency of complement factor H (CFH) were not evident. A nephritis-associated plasmin receptor (NAPlr), nephritogenic group A streptococcal antigen, and the plasmin activity by in situ zymography were been in both the first and second biopsy specimens. The patient received combined immunomodulatory therapy with prednisolone and mizoribine, and the urinary protein decreased to a mild level at 27 months after disease onset. These findings suggest that persistent glomerular NAPlr deposition may be associated with the pathogenesis of DDD in some patients without the involvement of C3NeF or CFH mutation and that DDD patients of this type may respond to immunomodulatory treatment.
Suga
Kenichi
K
Department of Pediatrics, The Institute of Health Bioscience, The University of Tokushima Graduate School, Tokushima University, Tokushima city, Tokushima, 770-8503, Japan. aksugajp@yahoo.co.jp
Kondo
Shuji
S
Matsuura
Sato
S
Kinoshita
Yukiko
Y
Kitano
Etsuko
E
Hatanaka
Michiyo
M
Kitamura
Hajime
H
Hidaka
Yoshihiko
Y
Oda
Takashi
T
Kagami
Shoji
S
D000293:Adolescent; D001706:Biopsy / Q000009:adverse effects; D003178:Complement C3 Nephritic Factor / Q000378:metabolism; D017242:Complement Factor H / Q000378:metabolism; D005260:Female; D005500:Follow-Up Studies; D005921:Glomerulonephritis / Q000150:complications / Q000209:etiology / Q000473:pathology; D015432:Glomerulonephritis, Membranoproliferative / Q000150:complications / Q000473:pathology*; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000150:complications / Q000378:metabolism / Q000473:pathology; D056747:Immunomodulation; D007678:Kidney Glomerulus / Q000378:metabolism / Q000473:pathology; D009393:Nephritis / Q000150:complications / Q000378:metabolism / Q000473:pathology; D011239:Prednisolone / Q000378:metabolism; D011507:Proteinuria / Q000150:complications / Q000378:metabolism / Q000473:pathology; D018000:Receptors, Peptide; D013290:Streptococcal Infections / Q000150:complications / Q000276:immunology; D013997:Time Factors
D003178:Complement C3 Nephritic Factor; D018000:Receptors, Peptide; C065181:plasmin receptor; D017242:Complement Factor H; D011239:Prednisolone