Journal of Pathology and Translational Medicine

Case Study

Concurrent Anti-glomerular Basement Membrane Nephritis and IgA Nephropathy: Kwang-Sun Suh, Song-Yi Choi, Go Eun Bae, Dae Eun Choi, Min-kyung Yeo; J Pathol Transl Med. 2019;53(6):399-402. Published online September 16, 2019; DOI: https://doi.org/10.4132/jptm.2019.08.05

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Anti–glomerular basement membrane (GBM) nephritis is characterized by circulating anti-GBM antibodies and crescentic glomerulonephritis (GN) with deposition of IgG along the GBM. In a limited number of cases, glomerular immune complexes have been identified in anti-GBM nephritis. A 38-year-old female presented azotemia, hematuria, and proteinuria without any pulmonary symptoms. A renal biopsy showed crescentic GN with linear IgG deposition along the GBM and mesangial IgA deposition. The patient was diagnosed as concurrent anti-GBM nephritis and IgA nephropathy. Therapies with pulse methylprednisolone and cyclophosphamide administration were effective. Concurrent cases of both anti-GBM nephritis and IgA nephropathy are rare among cases of anti-GBM diseases with deposition of immune complexes. This rare case of concurrent anti-GBM nephritis and IgA nephropathy with literature review is noteworthy.

Citations

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Original Articles

An Anion Site Change of the Glomerular Basement Membrane on Various Glomerular Diseases.: Yu Na Kang, Kwan Kyu Park, Seung Pil Kim, Sung Bae Park, Hyun Chul Kim, Eun Sook Chang, In Soo Suh; Korean J Pathol. 1997;31(8):765-772.

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Abstract PDF: We studied the ultrastructural alteration of glomerular anionic sites in 6 patients with minimal change nephrotic syndrome, 5 patients with membranous glomerulonephritis, 4 patients with focal segmental glomerulosclerosis, and 4 patients with IgA nephropathy by staining with polyethyleneimine (PEI) as a cationic probe. The control study was examined by using a nephrectomy specimen of non-glomerular disease which had no proteinuria. This method seems to selectively stain heparan sulphate in the basement membranes and has been widely used to evaluate changes in basement membrane charge in various human diseases as well as in experimental studies. The anionic sites in the lamina rara interna and lamina densa of normal glomerular basement membrane were always less numerous and less regularly distributed than those in the lamina rara externa. Characteristic common findings in these glomeruli showed a marked decrease of glomerular anionic sites in the regions with immune-complex deposits and normal distribution in the regions with focally those being absorbed and newly forming glomerular basement membrane. They were not detected in the gap of the basement membrane and on the area of the detached overlying epithelium using the PEI method. But the foot process fusion of epithelial cells seems not to influence the loss of anionic sites on the glomerular basement membrane.

The Role of MIB-1 Expression and Apoptosis in Experimental Crescentic Glomerulonephritis.: Nam Hoon Kim, Wan Seop Kim, Jung Woo Noh, Moon Hyang Park; Korean J Pathol. 1999;33(4):231-242.

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Abstract PDF: It has been postulated that programmed cell death via apoptosis may be critical for remodelling of glomeruli after inflammatory injury. To understand the regulatory mechanism of apoptosis in experimental crescentic glomerulonephritis (CGN), we examined the MIB-1 score (proliferation index, PI) and apoptotic index during the progression of experimental CGN to end-stage renal failure. CGN was induced in New Zealand White rabbits by administration of guinea pig anti-GBM IgG after sensitization with guinea pig IgG and their kidneys were analyzed for the development of crescents through sequential renal biopsies. Serum creatinine levels progressively increased in a time course until day 45. The PI in glomeruli, tubular epithelial cells, and interstitium progressively increased during the progression of experimental CGN. The mean numbers of MIB-1 positive intraglomerular nuclei (PI) were significantly correlated with degrees of crescent formation and the numbers of apoptotic cells in the glomeruli, tubules, and interstitium. Significant apoptosis was present from day 1 (15.8 10.16 cells/glomerular cross section) and increased in number with the proliferative lesions as glomerular inflammation continued. Moreover, apoptosis increased during the resolution of the glomerular inflammation, and many apoptotic cells were present in the sclerotic lesions in day 17 (18.6 12.99 cells/glomerular cross section). As glomerular inflammation subsided, cellular crescents progressed to fibrous crescents with a reduction of cellularity by day 45. On day 45, the glomerular PI and the numbers of apoptotic cells were markedly decreased. The correlations found in CGN between the creatinine level and the percentage of crescents, between the percentage of crescent and PI, and between the PI and number of apoptotic cells support the hypothesis that there is a change in the glomerular and tubulo-interstitial apoptosis under pathologic conditions. These findings indicate that apoptosis plays an essential role in the resolution of intra- and extraglomerular inflammation and in the elimination of glomerular cells within the sclerotic regions for progressive CGN. The regulation of the apoptotic phenomenon and increased PI during CGN may be important in the progression of glomerular inflammation and the development of pathologic glomerular sclerosis.

Glomerular Basement Membrane Thickness in Minimal Change Disease.: Yoon Mee Kim, Soon Hee Jung, Hyeon Joo Jeong; Korean J Pathol. 2000;34(12):994-1000.

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Abstract PDF: The thickness of the glomerular basement membrane may vary not only in glomerular disease, but also in normal persons according to age and sex. But there has been no data on the normal thickness of the basement membrane in Korea. This study was designed to determine the glomerular basement membrane thickness as a reference value according to age and sex, in 50 cases of minimal change disease obtained from patients aged 2~67 years. Measurement of glomerular basement membrane was made on electron micrograph using an image analyzer. The thickness of each case was estimated by the arithmetic and harmonic mean methods. The mean thickness of the glomerular basement membrane was 291.9 47.9 nm by harmonic mean method and 284.2 43.7 nm by arithmetic mean method. And the harmonic mean thickness of the glomerular basement membrane according to age was 249.1 32.5 nm (1~5 years), 256.6 45.3 nm (6~10 years), 279.2 57.9 nm (11~15 years), 303.2 43.8 nm (16~20 years), 335.3 37.5 nm (21~30 years), and 291.1 22.5 nm (over 30 years), respectively. There was a trend that the thickness of glomerular basement membranes increased with the age till 30 years of age. There was no significant sex-related difference. In conclusion, the mean glomerular basement membrane thickness is comparable to the data from western people and shows a trend of increasing thickness according to the age.

Morphologic Changes of the Parenchymal-Stromal Junction in Infiltrating Duct Carcinoma of the Breast: Immunohistochemical and Ultrastructural Features of Myoepithelial Cell, Basement Membrane.: Min Cheol Lee; Korean J Pathol. 1988;22(1):42-56.

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Abstract PDF: The morphologic study of noninfiltrating and infiltrating duct carcinoma of the breast disclosed profound alterations along the parenchymal-stromal junction. But fate of myoepithelial cell, changes of basement membrane and the relationship of fibroblast to myofibroblast remain uncertain. To study the morphologic changes of myoepithelial cell, basement membane and stromal fibroblast, a series of 32 not otherwise specified (NOS) type of infiltrating duct carcinoma of the breast with regional lymph node metastases was examined light microscopically after S-100 protein immunoperoxidase staining by biotinavidin system (BAS) and ultrastructurally. The results were as follows. 1) In 18 out of 32 cases, S-100 protein positive myoepithelial cells were observed individually in the parenchyma at the periphery of some carcinomatous duct-like structures or cancer cell nests. The cells were noted in 7 cases of metastatic regional lymph nodes. In 5 cases contained with 2 cases of infiltrating duct carcinoma with focal sarcomatous metaplasia, S-100 protein positive cells were seen in fibroblast-like spindle cells in stroma adjacent to cancer nests. 2) Ultrastructural features of myoepithelial cells showed significant loss of fine microfilament and hemides-mosomes and relative imcrease of coarse large filaments. Morphologic transformation of myoepithelial cells to neoplastic epithelial cells or stromal fibroblast-like spindle cells were suggested in 3 NOR type and 2 metaplastic type carcinomas. 3) The ultrastructural changes of basement membrane disclosed some variations from case to case and even within a single tumor if large number of blocks were studied. Focal destruction, splitting, segmentation and extensive loss of basement membrane arround cancer nests were noted. On the other hand, basement membrane material surrounded cancer nests or individual cancer cells irregularly. 4) Most stromal fibroblasts in infiltrating duct carcinoma had abundant rough endoplasmic reticulum with enlarged plump cytoplasm. Some of them were transformed to myofibroblasts which had perinuclear rough endoplasmic reticulum and peripheral microfilaments with dense bodies in their cytoplasm.

Light and Electron Microscopical Studies on the Stroma of Hydatidiform Mole.: Jong Tae Park, Sang Woo Juhng, Kyu Hyuk Cho; Korean J Pathol. 1987;21(4):240-248.

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Abstract PDF: Many investigators were interested in the pathogenesis and the relationship between microscopical features and clinical behavior of hydatidiform mole. Trophoblastic cells in the trophoblastic disease were intensively examined histologically, ultrastructurally, immunohistochemically, and with hormone assay method, etc. But ultrastructural study on the stroma of hydatidiform mole was scarcely reported. In this paper, hydatidiform mole was examined at light and electron microscopic levels, with emphasis on the stroma. The results were as follows: 1) Hydropic degeneration of H-mole is more severe in the center of stroma and is not related with the degree of trophoblastic proliferation. Hofbauer cell and vascular structure are extremely rarely observed in the periphery of stroma which has relatively preserved cellular components. 2) Basement membrane is sometimes separated from trophoblastic layer. Degenerated cells in the stroma contain vacuoles, autophagosomes, and lipid droplets. Collagen is abundant in the loose interstitium. Hofbauer cells have no lysosome or phagosome. Vascular lumen is patient and endothelial cells are degenerated. From the above results, H-mole may be produced due to abnormal changes of trophoblasts and stromal changes may be a secondary process, so called autolysis. Hofbauer cells are not engaged in the stromal degeneration and may be different from usual tissue macrophages.

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