Pulmonary fibrosis presenting as an early manifestation of microscopic polyangiitis

Microscopic polyangiitis (MPA) is a systemic small vessel vasculitis that is included in the pulmonary-renal syndromes. Although glomerulonephritis represents the major clinical feature of MPA indicative of renal involvement, diffuse alveolar haemorrhage is the classic manifestation of pulmonary involvement. However, pulmonary fibrosis is a less frequently reported pulmonary manifestation. Herein we describe a patient who was diagnosed with MPA presenting with radiographic evidence of pulmonary interstitial fibrosis as an early clinical manifestation accompanied by constitutional symptoms such as fever and weight loss. We also include a short literature review focusing on the association between pulmonary fibrosis and MPA

Heat-Shock Protein 27 kinetics in end stage renal disease patients

Atherosclerosis is one of the most significant yet unresolved problems in patients on regular Hemodialysis (HD) with major impact on morbidity and mortality of patients with end-stage renal disease (ESRD). There is growing evidence that atherosclerosis may-at least in part- result from autoimmune processes, in which the family of Heat-Shock Proteins (HSP) may be involved [1].</p

The role of pure diffuse mesangial hypercellularity in patients with proteinuria

Background. Pure diffuse mesangial hypercellulality (DMH), in its primary form, is a relatively rare histological finding, and scant data exist in the literature regarding its clinical course and prognosis in nephrotic adults with this diagnosis. Methods. We retrospectively analyzed the clinical and histological data of 8 out of 41 patients with the above diagnosis in regard to response to the treatment, outcome and prognostic indicators. Results. Six patients received oral prednisolone as initial therapy, five of whom receiving it as monotherapy at first. The two other patients did not receive anything at all. Three out of the above six patients received prednisolone either with cyclophosphamide or with cyclosporine (CyA). Three patients responded with complete remission, two showed partial remission, and one did not respond at all. During follow-up, none of the patients with complete response appeared to have relapse. The two patients with initial partial response to steroids received CyA in combination with low dose of oral prednisolone. The other patient who did not respond at all from the beginning did not receive anything more due to his bad general condition. Plasma creatinine remained stable in those with complete or partial response to treatment. None of the clinical characteristics was found to be predictive of the degree of renal function impairment at the time of renal biopsy. The three patients with partial or no response were characterized by the severity of mesangial hypercellularity. Patients with complete or partial response to therapy did not differ with regard to age, plasma creatinine, and severity of proteinouria at biopsy. Presence of mesangial IgM was not associated with poor or satisfactory response. In general, no clinical feature at the time of biopsy was predictive of a response to therapy. Conclusions. At present, it seems that adult patients with DMH and proteinuria represent a heterogeneous group with different clinical courses despite a similar morphological appearance in initial biopsies. We conclude that serial biopsies taken at regular intervals coupled with a longer follow-up may provide answers concerning the real intentity of DMH

Treatment with cinacalcet in hemodialysis patients with severe secondary hyperparathyroidism, influences bone mineral metabolism and anemia parameters

Background: Due to the premium rate of Chronic Kidney Disease, we have increased our knowledge with respect to diagnosis and treatment of Bone Mineral Disease (BMD) in End-Stage Renal Disease (ESRD). Currently, various treatment options are available. The medication used for Secondary Hyper-Parathyroidism gives promising results in the regulation of Ca, P and Parathormone levels, improving the quality of life. The aim of the present study was to investigate the relation of cinacalcet administration to not only parathormone, Ca and P but also to anemia parameters such as hematocrit and hemoglobin. Materials and Methods: A retrospective observational study was conducted in a Chronic Hemodialysis Unit. One-hundred ESRD patients were recruited for twenty-four months and were evaluated on a monthly rate. Biochemical parameters were related to medication prescribed and the prognostic value was estimated. Cinacalcet was administered to 43 out of 100 patients in a dose of 30-120 mg. Results: Significant differences were observed in PTH, Ca and P levels with respect to Cinacalcet administration. Ca levels appeared to be higher at 30mg as compared to 60mg cinacalcet. Furthermore, a decreasing age-dependent pattern was observed with respect to cinacalcet dosage. A positive correlation was observed between dry weight (DW) and cinacalcet dose. Finally, a positive correlation between Hematocrit and Hemoglobin and cinacalcet was manifested. Conclusions: Cinacalcet, is a potential cardiovascular and bone protective agent, which is approved for use in ESRD patients to assist SHPT. A novel information was obtained from this study, regarding the improvement of the control of anemia. © 2020 Bentham Science Publishers

Effect of cyclosporine therapy with low doses of corticosteroids on idiopathic nephrotic syndrome

Cyclosporine (CyA) has an immunosuppressive effect that might suggest a therapeutic role in idiopathic glomerular conditions. We focused on the optimization of CyA treatment control in patients with idiopathic nephrotic syndrome by using trough-level CyA measurements (C0) and the 2-h postdose levels (C2). Twenty-two patients (14 male, 8 female) with idiopathic nephrotic syndrome and the mean age of 51 ± 18 months (mean [M] ± standard deviation [SD]) were enrolled in our study during a period of 10 months (range: 3-18 months). All of the patients received CyA (2-3 mg/kg) in combination with methylprednisolone. In the present study protocol CyA concentrations (C0, C2), renal function, lipid profile, and degree of proteinuria were determined. The mean proteinuria of our patients before treatment was 11 972 ± 7953 mg/24 H (±SD) and the mean creatinine level (Cr) was 0.99 ± 0.37 mg/dL (±SD). Proteinuria decreased significantly already from the first month of therapy with CyA to 3578 ± 2470 mg/24 H (M± SD), and during the whole study period this reduction was significant (0.56 ± 0.37 gr/24 H (M ± SD), P &lt; 0.05). At the same time renal function preserved, 1.09 ± 0.48 mg/dL (M ± SD). The blood levels of C0 were 135.10 ± 97.36 ng/mL (M ± SD) and the blood levels of C2 were 725 ± 256 ng/mL (M ± SD) at the first month of therapy. At the same time renal function preserved, 1.09 ± 0.48 mg/dL (M ± SD). Total cholesterol levels reduced significantly during study period (276.89 ± 45.57 to 200.67 ± 40.27 mg/dL [M ± SD]). The mean number of antihypertensive medication remained the same. The whole therapeutic protocol did not provoke any kind of side effects and CyA was quite tolerated by our patients. Treatment of idiopathic nephrotic syndrome with low doses of CyA with methylprednisolone leads to remission of proteinuria without deterioration of renal function. Blood levels of C0 for monitoring and treatment of nephrotic syndrome agrees with recent literature, while our study focus on establishing the proper levels of C2 for the treatment of nephrotic syndrome. The efficacy of CyA is combined with safety and tolerance

HCV viraemia in anti-HCV-negative haemodialysis patients: Do we need HCV RNA detection test?

Background: Hepatitis C virus (HCV) infection is still common among dialysis patients, but the natural history of HCV in this group is not completely understood. The KDIGO HCV guidelines of 2009 recommend that chronic haemodialysis patients be screened for HCV antibody upon admission to the dialysis clinic and every 6 months thereafter if susceptible to HCV infection. However, previous studies have shown the presence of HCV viraemia in anti-HCV-negative haemodialysis patients as up to 22%. Objectives: To evaluate the presence of HCV viraemia, using HCV RNA detection, among anti-HCV-negative haemodialysis patients from a tertiary dialysis unit in Athens. Methods: We enrolled 41 anti-HCV-negative haemodialysis patients diagnosed with third-generation enzyme immunoassay. HCV viraemia was evaluated using a sensitive (cut-off: 12 IU/mL) reverse transcriptase polymerase chain reaction (COBAS AmpliPrep/TaqMan system) for HCV RNA. Results: None of the 41 anti-HCV-negative haemodialysis patients were shown to be viraemic. Conclusions: Routine HCV RNA testing appears not to be necessary in anti-HCV-negative haemodialysis patients. © The Author(s) 2018

Leptin concentrations in relation to body mass index and the tumor necrosis factor-alpha system in humans

The expression of leptin, an adipocyte-derived protein whose circulating levels reflect energy stores, can be induced by tumor necrosis factor (TNF)alpha in rodents, but an association between the TNF alpha system and leptin levels has not been reported in humans. To evaluate the potential association between serum leptin and the TNF alpha system, we measured the levels of soluble TNF alpha-receptor (sTNF alpha-R55), which has been validated as a sensitive indicator of activation of the TNF alpha system. We studied two groups: 1) 82 young healthy normal controls and 2) 48 patients with noninsulin dependent diabetes mellitus (NIDDM) and 24 appropriately matched controls. By simple regression analysis in controls, there was a strong positive association between leptin and 3 parameters: body mass index, sTNF alpha-R55, and insulin levels. In a multiple regression analysis model, leptin remained significantly and strongly associated with body mass index, and the association of leptin with both insulin and sTNF alpha-R55, although weakened, remained significant. Patients with NIDDM had leptin concentrations similar to controls of similar weight. Importantly, serum levels of sTNF alpha-R55 were also positively and independently associated with leptin in this group of diabetic subjects and matched controls. These data are consistent with the hypothesis that the TNF alpha system plays a role in regulating leptin levels in humans. Further elucidation of a possible role of the TNF alpha system in leptin expression and circulating levels may have important implications for our understanding of obesity and cachexia in humans