Hypertension and nephrology

[New development in the pathogenesis, diagnosis and treatment of IgA nephropathy]

NAGY Judit, VAS Tibor, KOVÁCS Tibor

DECEMBER 20, 2016

Hypertension and nephrology - 2016;20(06)

[IgA nephropathy is one of the leading cause of primary glomerulonephritis worldwide. IgA nephropathy is regarded as an immune mediated disease with a multi-hit pathogenesis starting with the production of poorly glycosylated IgA1 and glycan-specific IgG and IgA autoantibodies leading to the formation of IgA1 containing immune complexes. These immune complexes deposit in the glomerular mesangium followed by the onset of mesangioproliferative glomerulonephritis. The disease has variable clinical presentation and outcome. There is a need to identify patients who have the potential to progress to end-stage renal disease with the help of clinical, histological and biological markers. Treatment options for IgA nephropathy are largely based on opinion or weak evidence. It is true for the KDIGO Clinical Practice Guideline for Glomerulonephritis treatment recommendations containing low level of evidence for almost all recommendations related to IgA nephropathy. It is suggested to separate the patients into 3 groups on the basis of risk to progression and to give not-specific supportive treatment (especially angiotensin converting enzyme inhibitors or angiotensin receptor blocking agents) to all of them on the basis of the risk factors. We discuss the recommendations of the KDIGO Guideline about steroid and immunosuppressive treatment for moderate and high risk patients. Lastly, we provide our perspective on the existing other treatment options (tonsillectomy etc.) and on ongoing clinical trials.]



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Hypertension and nephrology

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Hypertension and nephrology

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Related contents

Hypertension and nephrology

[Prognostic role of arterial stiffness in IgA nephropathy]

SÁGI Balázs, KÉSŐI István, VAS Tibor, CSIKY Botond, KOVÁCS Tibor, NAGY Judit

[Background: Arterial stiffness has a prognostic role in chronic cardiovascular diseases. Pulse wave velocity (PWV) determined by the carotid-femoral pulse detection is accepted as a gold standard method. Further diagnostic procedures are in use to assess the arterial stiffness including the finger photoplethysmography. The prognostic role of this method is limited in chronic renal diseases. The goal of our investigation was to determine the prognostic significance of the stiffness index (SIDVP) measured by the photoplethysmographic method in IgA nephropathy. Patients and methods: One hundred and three histologically proved IgA nephropathy patients with chronic kidney disease stage 1-4 were investigated (67 male, 36 female, 45 ± 11 years) and followed for an average 65 (6-107) months. The stiffness index was determined by the volume alteration of the digital artery during the cardiac cycle (Pulse Trace system, Micro Medical, Gilingham, Kent, UK). The primary combined end point was total mortality, major cardiovascular events (stroke, myocardial infarction or cardiovascular procedure, for example revascularisation) plus achieving end stage renal disease. The secondary end points were cardiovascular and renal end points alone. Results: The patients with increased stiffness index (> 10 m/s) had significantly more combined primary end point events (10/60 vs. 19/43, P = 0.015). In case of the secondary end points the renal end points were significantly more frequent in patients with higher stiffness index. Stiffness index has also proved to be an independent predictor on survival from other cardiovascular risk factors (age, hypertension, diabetes, obesity, lipid disturbances and decrease of renal function) using the Cox regression model in IgA nephropathy. Every 1 m/s increase in stiffness index resulted a 17% gain in the occurrence of the combined primary end point. Conclusions: Stiffness index determined by finger photoplethysmography is an eligible parameter to assess the prognosis in IgA nephropathy. Increased stiffness index in IgA nephropathy seems to be a good prognostic tool for identification of higher risk patients.]

Hypertension and nephrology

[Prominents in Hungarian nephrology Professor Gyula Petrányi (1912–2000). I. part]


[A nation can only survive and keep its identity through its traditions. This is why the initiative to launch this series coming from professor János Radó is worthy of attention. Gyula Petrányi is an outstanding personality in 20th century internal medicine, to be more precise in nephrology and immunology, his activity being wide-ranging. The first part of the current summmary of his work deals with a tribute to his personality, and his role in immunomodularity treatment in glomerulonephritis. The second part shall cover his role in spreading renal biopsy, screening and caring kidney patients, dialysis, in developing kidney patients’ care, furthermore in clinicopharmacology and renal transplantation.]

Hypertension and nephrology

[Prognostic value of histopatologic classification in ANCA-association vasculitis]

FILE Ibolya, BIDIGA László, TRINN Csilla, UJHELYI László, BALLA József, MÁTYUS János

[Introduction: Rapidly progressive glomerulonephritis is life-threatening manifestation of antineutrophil cytoplasmatic antibody (ANCA)-associated vasculitis (AAV), often diagnosed only in advanced stage of renal failure. In 2010 a new histopathologic classification of ANCA- associated vasculitis was published by an international working group of nephropathologists. Vasculitis cases were classified in four groups: focal, crescent, mixed and sclerotic. Method: The aim of our study was to re-evaluate the predictive value of this new classification regarding renal outcome. From the 88 patients with ANCA-associated vasculitis treated in our department from 1996 to 2013, 39 were involved. Results: By retrospective evaluation of biopsy samples, patients were classified as 11 in focal, 12 in crescent, eight in mixed, eight in sclerotic group. There was no significant difference among the four groups regarding the mean age, sex, ANCAtype and initial eGFR. Due to the treatment, the eGFR values significantly increased in the focal and in the crescent groups. Eleven patients needed dialysis at presentation and three of them recovered, none of them belonged to the sclerotic group. The cumulative renal response to treatment was 100% in the focal, 87.5% in the mixed, 64% in the crescent and 62% in the sclerotic group. Renal response at one year treatment was 80%, thirty-one of the thirty-nine patients were dialysis independent. All patients were alive at one year, by year five two patients from the sclerotic group died. Conclusion: The new nephropathological classification of AAV is useful in predicting the renal response to treatment. Nephropathology can optimize the system by mentioning the specific percentage of normal glomeruli in the biopsy specimen.]

Lege Artis Medicinae

[Polyneuropathy as a first sign of microscopic polyangiitis]

ZÖLD Éva, HORVÁTH Ildikó Fanny, TARJÁN Péter, BARTA Zsolt, ZEHER Margit

[INTRODUCTION - Microscopic polyangiitis (MPA) is a systemic autoimmun disease characterized by necrotizing small vasculitis. MPA belongs to the ANCA-associated vasculitides. The disease can affect many of the body’s organ systems. Major organs involved are kidneys, skin, peripheral nerves and lungs. In addition, generalized symptoms such as fever and weight loss are very common. CASE REPORT - In January 2013 a 56-year old woman presented with weight loss, lower leg numbness, walking difficulty and petechiae on the lower legs. One month later, laboratory examinations showed progressive kidney dysfunction, anemia, hypersedimentation and elevated C reactive protein level, but further tests and investigations for potential bacterial infection and tumors were all negative. In sum, clinical signs and symptoms suggested systemic vasculitis, which was proved by the kidney biopsy and ENG examination. From these findings, microscopic polyangiitis was diagnosed with polyneuropathy and glomerulonephritis. The patient was a Hepatitis B (HBV) virus carrier, which can be provoking factor for vasculitis. Corticosteroid and six treatment cycles of intravenous pulse cyclophosphamide were performed for induction of remission. After treatment her symptoms improved and kidney function was normalized. Antiviral treatment was started because of HBV reactivation in October 2013. As a new manifestation of MPA, pulmonary symptoms were appeared in November 2013 and the patient was treated with synchronization of plasmapheresis and pulse cyclophosphamide with good clinical effectivity. Now, she is treated with methotrexate as immunosuppressive treatment and control examinations indicate the remission of the disease with proper renal function. CONCLUSION - We draw attention to a rare case of vasculitis and underline the importance of both the early diagnosis and the early and effective immunosuppressive therapy. Peripheral neuropathy may occur as a result of having systemic vasculitis. Nevertheless, the exploration and elimination of provoking factors are also must be part of the management and the regular follow-up is essential to recognize the disease relapses, thus avoid permanent organ damage.]

Hypertension and nephrology

[Cardiovascular risk assessment in chronic kidney disease, significance of left ventricular myocardial mass index]

SÁGI Balázs, KÉSŐI István, VAS Tibor, CSIKY Botond, NAGY Judit, KOVÁCS Tibor

[Introduction: Earlier studies have shown that cardiovascular (CV) mortality and morbidity in chronic kidney disease (CKD) often exceed their average population, and left ventricular hypertrophy (LVH) is an independent risk factor for CV disease. However, in CKD, the relationship between LVH, arterial stiffness (AS) and renal function has not yet been fully elucidated. Little data is available on their prognostic role. Aims of our study a) cross-sectional examination of the relationship between left ventricular mass index (LVMI), arterial vascular stiffness, and renal function, b) in our follow-up study, clarification of the LVMI, the prognostic role of AS in patients with CKD, IgA nephropathy (IgAN). Methods: In our cross-sectional study, 79 IgAN patients were examined in our clinic. The myocardial mass index (LVMI) was determined using an estimation formula after echocardiographic measurements. Arterial stiffness was measured using a photoplethizmography technique (PulseTrace) and characterized by the stiffness index (SI). The MDRD formula was used to estimate renal function (GFR) (eGFR, ml/min/1.73 m2). In the prognostic study the primary combined endpoint was total mortality, the most important CV events (stroke, myocardial infarction or cardiovascular interventions such as revascularization) and end stage renal disease. Secondary endpoints were CV and renal endpoints separately. Results: Of the 79 patients included in our cross-sectional study, 50 were men, with an average age of 46 ± 11 years. The mean value of LVMI was 106.66 ± 22.98 g/m2. Patients were divided into groups of 115 g/m2 for males considered to be abnormal and 95 g/m2 for women. LVMI is closely correlated with SI and inversely with eGFR (corr. coeff: 0.358; p <0.05 or -0.526; p <0.001). In case of LVH, SI was significantly higher in both sexes (p = 0.005 in males, p = 0.04 in females). In case of higher LVMI, renal function was significantly lower (p = 0.002 in males, p = 0.01 in females). Metabolic syndrome occurred in several cases in both sexes with LVH, but the difference was only significant in male patients (males 6 vs. 10, p = 0.008; females 2 vs. 4, p = 0.29). In our follow-up study, the presence of LVH in men significantly reduced survival in both primary and secondary endpoints, whereas in women there was no significant difference. Conclusion: In IgAN decreasing of renal function is closely related to left ventricular hypertrophy and vascular stiffness, as well as a close relationship was found between LVMI and AS. Reduced renal function is associated with an increase in LVMI and an increase in AS, which may result in a worse prognosis for both CV and renal outcomes. The underlying role of all these can be assumed to be a common vascular and myocardial pathological remodeling.]