Journal of nephropathology
Volume:4 Issue: 2, Apr 2015

Context: T helper (Th) cells as an important part of the immune is responsible for elimination of invading pathogens. But, if Th cell responses are not regulated effectively, the autoimmune diseases might develop. The Th17 subset usually produces interleukin-17A which in experimental models of organ-specific autoimmune inflammation is very important. Evidence Acquisitions: Directory of open access journals (DOAJ), Google Scholar, Embase, Scopus, PubMed and Web of Science have been searched. Fifty-six articles were found and searched. In the present review article, we tried to summarize the recently published data about characteristics and role of Th1 and Th17 cells and discuss in detail, the potential role of these T helpers immune responses in renal inflammation and renal injury, focusing on glomerulonephritis. Published papers in animal and human studies indicated that autoimmune diseases such as rheumatoid arthritis and multiple sclerosis, classically believed to be Th1-mediated, are mainly derived from a Th17 immune response. Identification of the Th17 subgroup has explained seemingly paradoxical observations and improved our understanding of immune-mediated inflammatory responses. Secretion of IL-17A, as well as IL-17F, IL-21, IL-22, suggests that Th17 subset may play a crucial role as a pleiotropic pro-inflammatory Th subset. There is experimental evidence to support the notion that Th1 and Th17 cells contribute to kidney injury in renal inflammatory diseases like glomerulonephritis.

Gitelman’s syndrome (GS) is a rare autosomal recessive renal tubular disorder that is characterized by episodic clinical manifestations and persistent biochemical abnormalities. The disorder manifests in adolescent or adult age and is characterized by transient episodes of muscle weakness and tetany. Its diagnosis requires a high index of suspicion and skillful interpretation of laboratory investigations. We herein present a case of a 20-year-old female patient who presented with generalized muscle weakness and mild renal insufficiency. Laboratory investigations revealed mild azotemia, high anion gap acidosis, hypokalemia, hypomagnesemia, and hypocalciuria. She recovered her renal functions and muscle power with appropriate management and is doing well seven months after her first presentation to our hospital. This case highlights the need to create high index of suspicion among the general practitioners about this syndrome and an early referral of such patients to nephrologists for an accurate diagnosis and appropriate management.

The co-existence of thin basement membrane nephropathy (TBMN) and another glomerular pathology portends a worse prognosis than TBMN alone. The purpose of our study was to investigate the prevalence of TBMN and associated glomerular pathologies at our institution. Patients and We reviewed all renal biopsies performed at Saint Louis University hospital over a 7-year period. We excluded all post transplant biopsies, and biopsies showing diabetic glomerulopathy, membranoproliferative glomerulopathy, membranous glomerulopathy, and biopsies where no electron microscopy or immunofluorescent studies were done. All other biopsies were included. A total of 634 biopsies were included in the study. The prevalence of TBMN was 47 (7.4%), of whom 17 (36.2%) had TBMN alone. In the remaining 30 (63.8%) patients TBMN was associated with other glomerular pathologies: IgAN 9 (19.1%) and FSGS 9 (19.1%). We found significantly higher prevalence of IgAN in patients with TBMN versus all biopsies (19.1% vs. 7.7%, respectively, P = 0.002). We found significant similarities in biopsy indications for TBMN and IgAN group. Around two thirds of the cases of TBMN were associated with other glomerular pathologies. The prevalence of IgAN, but not focal segmental glomerulosclerosis, was significantly higher in patients with TBMN as compared to the general renal biopsy specimens.

Inflammation has a major role in disease lead to renal failure and diabetes mellitus, controlling inflammation in diabetic kidney receivers could decrease morbidity and mortality. This study designed for evaluating the efficacy of pioglitazone on C-reactive protein and lipid profile in diabetic kidney transplant receivers. Patients and In this double blinded clinical trial, 58 diabetic renal transplant receivers, in first month after transplantation, randomized into two groups; receiving insulin and pioglitazone (15 mg tablet daily, group A); and insulin and placebo (group B). Blood pressure, weight, body mass index (BMI) and laboratory data compared in before and after 4-month treatment in two groups by SPSS. Fifty-eight patients with mean age of 44.15 ± 2 years included. There were no significant difference between groups in demographic data and other baseline measured variables (P > 0.05). The mean weigh and BMI were slightly increased in group A and decreased in group B. The mean hs-CRP was decreased 4.82 mg/dL in group A and 1.93 mg/dL in group B (P = 0.007). The mean total serum cholesterol was significantly decreased 34 mg/dL in group A and 18.07 mg/dL in group B (P = 0.027). The mean serum HDL-C was significantly increased 13.31 mg/dL in group A and 5.89 mg/dl in group B (P < 0.001). Pioglitazone seems to be a safe drug for reducing serum lipids and CRP in kidney transplant receivers with diabetes mellitus in short term. Long term effect of this drug could be evaluated in future studies.

The natural evolution of C1q nephropathy (C1qNP) during immunosuppressive treatment is relatively little studied or understood. A 30 year-old Caucasian female was referred to us for further management of biopsy-proven C1qNP and severe nephrotic syndrome. Serologic work-up remained negative, including complement C3 and C4 levels and repeated testing for antinuclear antibodies. A renal biopsy revealed minimal change nephropathy vs. focal sclerosis on light microscopy and C1qNP on immunopathology. She has failed trials of high-dose oral prednisone, mycophenolate mofetil 1,500 mg twice a day and a subsequent regimen of monthly IV cyclophosphamide 750 mg × 9 cycles. She also received the maximum tolerated angiotensin-converting enzyme inhibitor and spironolactone therapy. Random urine protein-to-creatinine (UPC) ratio predicted proteinuria in the range between 5-35 gm/day, while serum creatinine rose progressively from 1.0 mg/dL to 1.4 mg/dL (to convert to μmol/L, multiply by 88.4). A decision was made to repeat renal biopsy to reassess the underlying histology. The biopsy revealed focal sclerosis but no C1q deposition. Our case illustrates at least two points: first, an established pathologic diagnosis does not obviate the need for repeated renal biopsy later on, should diagnostic uncertainty persist. Second, histological diagnoses may evolve over time, especially in a patient receiving active and powerful immune-modulating treatment. In our case, the clinical nephrosis did not change with immunosuppressive therapy while C1q deposition ceased, making this latter entity likely the immunologically mediated process.