The clinical characteristics of Takayasu's arteritis with glomerulonephropathy / 中华内科杂志

Objective To investigate the clinical features of Takayasu's arteritis (TAK)with glomerulonephropathy and to improve physicians' understanding of this complication in patients with TAK. Methods Clinical data were retrospectively collected including manifestations, laboratory tests, image findings and treatment of 8 patients diagnosed as Takayasu's arteritis with glomerulonephropathy from January 2002 to January 2017 in Peking Union Medical College Hospital. Results Glomerulonephropathy was confirmed based on percutaneous renal biopsy. There were 6 women and 2 men. The median onset age and median disease duration were 24 (18-37) years and 42 (3-360) months, respectively. Five patients had hypertension. The 24 hour urinary protein was 0.18-14.91 g. Red blood cells and casts in urine were tested among 4 and 2 patients, respectively. Three patients had renal artery stenosis. Three patients demonstrated mesangial proliferative glomerulonephritis, two with IgA nephropathy, two with minimal change disease and one with membranoproliferative glomerulonephritis. Seven patients received glucocorticoid combined with cyclophosphamide therapy (glucocorticoid 40-60 mg/d, prednisone or equivalent; cyclophosphamide 0.4 g/week iv. or cyclophosphamide 0.1 g/d po.). Uninary blood cells removed and 24 hour urinary protein decreased from 1.65 g to 0.90 g after treatment for 12 months in one patient. The other 7 patients were missing. Conclusion Glomerulonephropathy is occasionally observed among TAK patients. Mesangial proliferative glomerulonephritis is the most common pathological subtype. Glucocorticoid combined with cyclophosphamide therapy could be an optional therapy for Takayasu's arteritis with glomerulonephropathy.

Pathology of C3 Glomerulonephritis

C3 glomerulonephritis (C3GN) is a recently described entity that shows a glomerulonephritis on light microscopy, bright C3 staining and the absence of C1q, C4, and immunoglobulins on immunofluorescence microscopy and mesangial and/or subendothelial electron-dense deposits on electron microscopy. The term 'C3 glomerulopathy' is often used to include C3GN and dense deposit disease (DDD), CFHR5 nephropathy, those of which result from dysregulation of the alternative pathway of complement. C3GN shares some aspects of atypical hemolytic uremic syndrome, MPGN, late stage of post infectious glomerulonephritis and other glomerulonephrtis. When C3GN is considered, measurement of serum complement proteins including C3, CFH, CFI, CFB and testing for the presence of C3 nephritic factor, anti-factor H autoantibodies are necessary. To screening for mutations, genes that encode complement regulators should be evaluated. This disorder equally affected all ages, both genders, and typically presented with hematuria and proteinuria. In both the short and long term, renal function remained stable in the majority of patients.

Secondary Membranous Nephropathy with Salivary Gland Cancer / 대한신장학회지

We report here on a rare case of membranous nephropathy occurring concurrently with salivary gland cancer. A 67-year-old woman was admitted to our department for nephrotic syndrome. She was diagnosed with membranous nephropathy and this was initially managed with steroids and cyclophosphamide, which started in 2003 and continued for about 10 months, without deriving any therapeutic benefits. During the treatment, the patient was diagnosed with salivary gland cancer when an incidental salivary gland mass was discovered in 2005. The patient's urinary protein level began to decrease following resection of the malignant mass. The clinical findings represent an interesting case of secondary membranous nephropathy that was diagnosed prior to the incidental discovery of malignancy.

Mesenchymal Stem Cells Ameliorate Adriamycin Induced Proteinuric Nephropathy

PURPOSE: Glomerulonephropathy (GN) often manifests as proteinuria and progresses to chronic renal failure without specific therapy. Mesenchymal stem cell (MSC) has been tried as a therapeutic agent in experimental GN, and previous studies showed that administration of MSC concomitantly to the insult inducing GN or via intra-renal administration ameliorated proteinuria. The purpose of this study was to test the therapeutic potential of MSC administered via intravenous route at the time of clinically evident proteinuria. METHODS: MSCs were administered intravenously via tail vain into the mice with adriamycin (ADR) induced nephropathy (ADR-GN), two weeks after ADR injection when massive proteinuria was evident. To test the capacity of MSC modulate the cytokine production in the inflammatory milieu, the concentrations of IFN-gamma and IL-10 were measured in the supernatant of in vitro mixed lymphocyte culture (MLC) with or without additional MSC. RESULTS: MSCs administered intravenously into the proteinuric mice with ADR-GN accelerated the recovery of this experimental GN with disappearance of proteinuria in two weeks when the saline treated (control) mice still showed significant proteinuria. The mice treated with MSC also had a tendency of better survival. Addition of MSC decreased IFN-gamma and increased IL-10 in the supernatant of MLC. CONCLUSION: This study showed that MSC had a therapeutic potential even when administered in a more clinically relevant setting into a proteinuric glomerulonephropathy model. Further study to verify the mechanism and long-term safety of this phenomenon is required.

Kaposi Sarcoma in Patient with Membranous Glomerulonephropathy during Corticosteroid Therapy / 대한신장학회잡지

Membranous glomerulonephropathy is a common cause of nephrotic syndrome in adults. Kaposi sarcoma is a well-known entity with distinct clinical forms such as nodular cutaneous lesions, generalized lymphadenopathy and visceral involvement. Incidence of Kaposi sarcoma is greater in patients with immunosuppression, particularly those having undergone renal transplantation, but also in patients with other underlying disorders treated with immunosuppressive therapy, notably, corticosteroids. We present a case of Kaposi sarcoma in patient with membranous glomerulopathy during corticosteroid therapy. A 49- year-old man was admitted with a complain of facial and leg edema, 5-kg weight gain for 1 month, and foamy urine. Kidney biopsy showed membranous glomerulopathy. We started corticosteroid therapy to the patient. Two month later, his 24 hr urinary protein was decreased to 2.1 g/day. But, the well defined, various-sized, purple-colored papules and plaque appeared on the both hands and feet. He underwent skin biopsy, which revealed abnormally proliferated and dilated vessels, vascular slits, spindle-shaped cells and extravasated erythrocytes in the dermis. The findings were in accordance with Kaposi sarcoma. So he received cryotherapy with discontinuing corticosteroid. Four months after cryotherapy, skin lesions were cleared leaving slight hypopigmentation and amount of proteinuria was preserved without definite aggravation.

A Case of Membranous Glomerulonephropathy Associated with Idiopathic Autoimmune Thrombocytopenic Purpura / 대한신장학회잡지

Membranous glomerulonephropathy is the most common cause of nephrotic syndrome in adults and idiopathic autoimmune thrombocytopenic purpura is autoimmune disease caused by autoantibody to platelet membrane glycoprotein. Although there are some pathologic similarity between two diseases that 'membrane attack complex' play a role in pathologic process, but only 3 cases worldwide are reported about membranous glomerulonephropathy associated with idiopathic autoimmune thrombocytopenic purpura. So we report a case of sixty eight years of woman who had symptoms of generalized edema, foamy urine, anorexia and thrombocytopenia on admission and developed more severe symptomatic thrombocytopenia there after. She was diagnosed membranous glomeulonephropathy on renal biopsy and also diagnosed idiopathic autoimmune thrombocytopenic purpra on bone marrow biopsy and on the basis of exclusion.

Detection of Serum and Urinary TGF-?_1 in Patients with Primary Chronic Glomerulonephropathy and Its Clinical Significance / 中国医师杂志

Objective To explore change and significance of serum and urinary transforming growth factor-? 1(TGF-? 1)in the patients with primary chronic glomerulonephropathy (PCG). Methods 65 patients with PCG were divided into four groups according to the degree of renal function damage: normal renal function,renal compensatory,renal decompensatory and renal function failure groups. Levels of serum and urinary TGF-? 1 were determined with ELISA, and the levels of urinary protein and renal function were examined as well. Results Levels of serum and urinary TGF-? 1 in patients with PCG were singificantly higher than those in control, and the levels of serum and urinary TGF-? 1 among four groups were significantly different. The TGF-? 1 level of urinary was associated with the degree of renal function damage(r=0 28,P

Clinicopathologic Analysis of Membranous Glomerulonephropathy

Membranous glomerulonephropathy (MGN) is the most common primary cause of the nephrotic syndrome in adults, accounting for about 20% of the cases in most series. MGN is idiopathic in the majority of cases, however approximately 25% of adults have identifiable causes (secondary MGN). To evaluate the clinical and pathologic characteristics of MGN, we reviewed the clinical data and renal biopsies from 141 cases of MGN. The mean age of the patients at biopsy was 43 years old, but patients of all age were seen (range from 3 to 76 years of age). There were 88 males and 53 females. There were 99 idiopathic MGN cases and 42 secondary MGN cases. The associated causes of secondary MGN included hepatitis B infection (18 cases), SLE (10 cases), drugs (4 cases), post-transplantation MGN (5 cases), diabetes mellitus (4 cases), syphilis (1 case) and hepatitis B infection associated with rheumatoid arthritis. The prevalence of histologic stages by Ehrenreich and Churg was as follows. Stage I was 24 cases, stage II was 72 cases, stage III was 35 cases, and stage IV was 9 cases. All patients had proteinuria. Nephrotic syndrome was observed in 39%, edema in 73%, microscopic hematuria in 49%, gross hematuria in 28%, hypertension in 13%, and the serum creatinine level above 1.5 mg/dl was in 13%. Cases with glomerulosclerosis was observed in 45 cases with an increased percentage of glomerulosclerosis in the higher grade. Immunofluorescence (IF) examination showed predominantly granular IgG (118 cases) and C3 (84 cases) stainings along the glomerular capillary wall. In idiopathic MGN, sparse mesangial IF staining was noted up to 10% of the cases. However, mesangial IF staining in SLE was observed in 33%, hepatitis B infection in 28% and diabetes mellitus in 50%. An electron microscopic examination revealed subepithelial electron dense deposits of immune complex in all cases. The prevalence of mesangial and subendothelial electron dense deposit in idiopathic MGN was present in 19% and 6%, respectively. In SLE cases, mesangial and subendothelial deposits were observed in 78% and 56%, respectively. In hepatitis B infection, mesangial and subendothelial deposits were observed in 54% and 69%, respectively. In conclusion, immune deposits in the mesangium are scanty in idiopathic MGN, and if pronounced this should increase suspicion of underlying systemic diseases, such as SLE or other infectious diseases.