Morphologic variations of dense deposit disease: light and electron microscopic, immunohistochemical and clinical findings in 10 patients.
Twenty-one renal biopsy specimens obtained from 10 patients with dense deposit disease (DDD) were investigated using light microscopy, electron microscopy and immunohistochemistry. The patients included four females and six males aged 6 to 35 years (mean 16.1 years). A morphological diagnosis of DDD was made following the ultrastructural detection of continuous intramembranous dense deposits (CIMDD) in some capillary loops of at least one of the series of the repeated biopsies from each patient. With light microscopy, six patients showed membranoproliferative glomerulonephritis (MPGN). The other four patients showed diffuse proliferative glomerulonephritis (DPGN) with acute lesions showing intraglomerular neutrophilic infiltration, hump formation and endothelial swelling in three and minor glomerular abnormalities in one. Follow-up biopsies were obtained in six patients. Two patients progressed from DPGN to MPGN within 7 months, whereas three patients with MPGN showed morphologic improvement that featured increased capillary patency and regional disappearance of dense deposits along with the reduction of proteinuria. Dense deposit disease did not always feature typical amorphous and osmiophilic CIMDD spreading across the whole width of the lamina densa. This classical ultrastructural manifestation was mainly found in the patients with histologic non-MPGN and a linear peripheral pattern of complement component (C3) deposition. The MPGN patients with a granular peripheral pattern of C3 deposition also had CIMDD, but also additionally featured less dense subepithelial deposits superimposed on the CIMDD to produce an appearance simulating membranous transformation.(ABSTRACT TRUNCATED AT 250 WORDS)
Joh
K
K
Department of Pathology, Jikei University School of Medicine, Tokyo, Japan.
Aizawa
S
S
Matsuyama
N
N
Yamaguchi
Y
Y
Kitajima
T
T
Sakai
O
O
Mochizuki
H
H
Usui
N
N
Hamaguchi
K
K
Mitarai
T
T
D000293:Adolescent; D000328:Adult; D002648:Child; D003094:Collagen / Q000032:analysis; D015922:Complement C1q / Q000032:analysis; D003176:Complement C3 / Q000032:analysis; D003181:Complement C4 / Q000032:analysis; D005260:Female; D005340:Fibrinogen / Q000032:analysis; D005353:Fibronectins / Q000032:analysis; D005500:Follow-Up Studies; D015432:Glomerulonephritis, Membranoproliferative / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D007124:Immunoenzyme Techniques; D007070:Immunoglobulin A / Q000032:analysis; D007074:Immunoglobulin G / Q000032:analysis; D007075:Immunoglobulin M / Q000032:analysis; D007678:Kidney Glomerulus / Q000737:chemistry; D008297:Male; D008854:Microscopy, Electron
D003176:Complement C3; D003181:Complement C4; D005353:Fibronectins; D007070:Immunoglobulin A; D007074:Immunoglobulin G; D007075:Immunoglobulin M; D015922:Complement C1q; D005340:Fibrinogen; D003094:Collagen