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Abstract

Y. Mori, H. Yamashita, Y. Umeda,Y.U. Uchiyama-Tanaka, A. Nose,N. Kishimoto, Y. Kijima, T. Nagata,M. Mori, H. Matsubara, H. Yoshida and T. Iwasaka

¹Department of Medicine II, Kansai Medical University, Osaka, and
²Department of Clinical Laboratory Medicine, Fukui Medical University,
Fukui, Japan
An otherwise healthy 20-year-old woman presented with an erythematous rash on her face as well as arthralgia and anemia. She also had systemic edema, proteinuria and hypertension. Laboratory data on admission showed hypocomplementemia, human parvovirus B19 (HPV) DNA and both immunoglobulin (Ig) M and IgG antibodies to HPV in her serum. Renal biopsy specimens showed features of endocapillary glomerulonephritis under light microscopy. Electron microscopy showed massive subendothelial electron-dense deposits. No cause was probable other than immune complex-mediated glomerulonephritis associated with HPV infection. In a review of this and similar cases reported in the literature, several characteristic features come to light: female dominance, onset in the second or third decade of life, hypocomplementemia, histologic renal endocapillary and/or mesangioproliferative glomerulonephritis with subendothelial deposits and spontaneous recovery.

Abstract

Y. Uchiyama-Tanaka, Y. Mori, N. Kishimoto, A. Nose, Y. Kijima, T. Nagata, Y. Umeda, H. Masaki, H. Matsubara and T. Iwasaka

Renal Division and ²Cardiovascular Center, Department of Medicine II, Kansai Medical University, Osaka, Japan
We describe the case of a 51-year-old man with hepatitis C virus (HCV) infection and a 3-month history of facial edema. Laboratory tests upon admission for renal biopsy showed normal renal function and normocomplementemia. Serum HCV antibody (Ab) and cryoglobulin were positive. Renal biopsy specimens showed features of membranous glomerulonephritis. The likely cause was immune complex-mediated glomerulonephritis associated with HCV infection. Reports of similar cases in the literature show the normocomplementemia and negative or slightly positive cryoglobulins observed in our case as well as seropositivity for circulating immune complexes containing HCV RNA. In our case, electron microscopic examination of the subepithelial glomerular lesions revealed massive virus-like particles within unusual multilayers of electron-dense deposits (EDDs), suggesting the existence of HCV in the glomeruli. In the addition to the unique histopathological feature the presence of La/SS-B antibody in his serum indicated an abnormal immune response associated with HCV. We advise him to undergo the therapy with new type of IFN such as pegIFN-a2a and/or anti-viral agent like ribavirin to achieve clinical and histopathological improvement.

Abstract

N. Kishimoto, Y. Mori, H. Yamahara, Y. Kijima, A. Nose, Y. Uchiyama-Tanaka, T. Tokoro, T. Nagata, Y. Umeda, N. Takahashi, H. Yoshida and H. Matsubara

¹Division of Cardiology and Nephrology, Department of Medicine, Kyoto Prefectural University of Medicine, Kyoto, ²Department of Medicine II, Kansai Medical University, Osaka, and ³Division of Nephrology, University of Fukui Hospital, Fukui, Japan
Renal deterioration often occurs in cases of infectious endocarditis (IE), but, IE- associated nephritis with rapidly progressive glomerulonephritis (RPGN) is rare. Patients with severe infection (e.g., IE) sometimes show positivity for cytoplasmic antineutrophil cytoplasmic antibodies (C-ANCA). Therefore, diagnosis and treatment are very difficult in cases of RPGN with IE and positivity for C-ANCA. Such cases are rare, only 12 have been reported in the English literature. Herein, we describe the case of a 50-year-old man who presented with RPGN with IE and tested positively for C-ANCA. He was referred to our hospital because of leg edema, purpura and renal dysfunction. Laboratory tests revealed serum creatinine elevation and positivity for C-ANCA and proteinase 3-specific (PR3)-ANCA. RPGN and acute renal failure were diagnosed. Hemodialysis and steroid therapy were started. Streptococcus oralis was isolated by blood culture. Transthoracic echocardiography revealed grade III mitral valve insufficiency with two vegetations. Therefore, IE was diagnosed. The steroid therapy was stopped, and antibiotic therapy was begun. Because there was no improvement, surgical therapy was performed. The operation was successful, but the patient died of brain hemorrhage. Our experience in this case indicates C/PR3-ANCA positive RPGN must be ruled out in patients with infectious disease, particularly IE, together with renal symptoms, and renal biopsy should be performed. Correspondence to:
Y. Mori, MD Division of Cardiology and Nephrology Department of Medicine Kyoto Prefectural University of Medicine 465 Kajii-cho Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto, 602-8566, Japan
Email: [email protected]

Abstract

T. Kusaba, T. Hatta, K. Sonomura, Y. Mori, T. Tokoro, T. Nagata, Y. Umeda, K. Nagata, T. Yasuda, T. Sato, K. Kimura and H. Matsubara

¹Division of Hypertension and Nephrology, Department of Medicine, Kyoto Prefectural University of Medicine, Kyoto, ²Division of Internal Medicine II, Kansai Medical University, Osaka, ³Division of Nephrology and Hypertension, Department of Internal Medicine, St. Marianna University School of Medicine, Kanagawa and ⁴Division of Cardiology and Vascular Regenerative Medicine, Department of Medicine, Kyoto Prefectural University of Medicine, Kyoto, Japan
Idiopathic nodular glomerulosclerosis (ING) is characterized as diffuse nodular glomerulosclerotic lesions, closely resembling Kimmelstiel-Wilson lesions without diabetic mellitus. We report here three Japanese cases of ING and discuss the previous reports. The patients were 75-, 48- and 84-year-old males with a history of long-term hypertension. Laboratory examination revealed moderate proteinuria and mild renal dysfunction. Diabetes mellitus was excluded by repeated clinical and laboratory investigations. Renal histology revealed nodular glomerulosclerosis, and both afferent and efferent arteriolosclerosis in all patients. In electron microscopy, the glomerular basement membrane was markedly thick in all patients. A low-protein diet and potent anti-hypertensive treatment using angiotensin-converting enzyme inhibitors were initiated in all patients and urinary protein excretion significantly reduced without the progression of renal dysfunction. We reviewed 42 previously reported cases and our three cases. The analysis revealed that common clinical features of ING are being male (82.2%) of relatively advanced age (mean age 61.3 years), with hypertension (82.2%), mild renal dysfunction (mean serum creatinine 2.9 mg/dl) and moderate urinary protein excretion (mean 4.05 g/day). Common histopathological findings of ING are nodular glomerulosclerosis (100%), arterio-arteriolosclerosis (91.2 and 89.7%) and glomerular basement membrane thickening (85.7%). In conclusion, ING is one of the phenotypes of arteriosclerotic renal disease without diabetes mellitus. Severe arterio-arteriolosclerosis may contribute to the progression to glomerular nodular formation in ING. The combination of renin-angiotensin system inhibition and a low protein diet can be beneficial for the reduction of urinary protein excretion.Correspondence to:
T. Kusaba, MD Division of Nephrology and Hypertension Department of Internal Medicine Kyoto Prefectural University of Medicine 456 Kajii-cho, Kamigyo-ku, Kyoto-city, 602-8566 Japan
Email: [email protected]

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