Validation of the International IgA Nephropathy Prediction Tool in the Greek Registry of IgA Nephropathy.

BACKGROUND: Immunoglobulin A nephropathy (IgAN) is among the commonest glomerulonephritides in Greece and an important cause of end-stage kidney disease (ESKD) with an insidious chronic course. Thus, the recently published International IgAN prediction tool could potentially provide valuable risk stratification and guide the appropriate treatment module. This study aimed to externally validate this prediction tool using a patient cohort from the IgAN registry of the Greek Society of Nephrology. METHODS: We validated the predictive performance of the two full models (with or without race) derived from the International IgAN Prediction Tool study in the Greek Society of Nephrology registry of patients with IgAN using external validation of survival prediction models (Royston and Altman). The discrimination and calibration of the models were tested using the C-statistics and stratified analysis, coefficient of determination ( R D 2 ) for model fit, and the regression coefficient of the linear predictor (ßPI), respectively. RESULTS: The study included 264 patients with a median age of 39 (30-51) years where 65.2% are men. All patients were of Caucasian origin. The 5-year risk of the primary outcome (50% reduction in estimated glomerular filtration rate or ESKD) was 8%. The R D 2 for the full models with and without race when applied to our cohort was 39 and 35%, respectively, and both were higher than the reported R D 2 for the models applied to the original validation cohorts (26.3, 25.3, and 35.3%, respectively). Harrel's C statistic for the full model with race was 0.71, and for the model without race was 0.70. Renal survival curves in the subgroups (<16th, ~16 to <50th, ~50 to <84th, and >84th percentiles of linear predictor) showed adequate separation. However, the calibration proved not to be acceptable for both the models, and the risk probability was overestimated by the model. CONCLUSIONS: The two full models with or without race were shown to accurately distinguish the highest and higher risk patients from patients with low and intermediate risk for disease progression in the Greek registry of IgAN.

IgA nephropathy in Greece: data from the registry of the Hellenic Society of Nephrology.

BACKGROUND: Natural history, predisposing factors to an unfavourable outcome and the effect of various therapeutic regimens were evaluated in a cohort of 457 patients with immunoglobulin A nephropathy (IgAN) and follow-up of at least 12 months. METHODS: Patients with normal renal function and proteinuria <1 g/24 h as well as those with serum creatinine (SCr) >2.5 mg/dL and/or severe glomerulosclerosis received no treatment. Patients with normal or impaired renal function and proteinuria >1 g/24 h for >6 months received daily oral prednisolone or a 3-day course of intravenous (IV) methylprednisolone followed by oral prednisolone per os every other day or a combination of prednisolone and azathioprine. The clinical outcome was estimated using the primary endpoints of end-stage renal disease and/or doubling of baseline SCr. RESULTS: The overall 10-year renal survival was 90.8%, while end-stage renal disease and doubling of baseline SCr developed in 9.2% and 14.7% of patients, respectively. Risk factors related to the primary endpoints were elevated baseline SCr, arterial hypertension, persistent proteinuria >0.5 g/24 h and severity of tubulointerstial fibrosis. There was no difference in the clinical outcome of patients treated by the two regimens of corticosteroids; nevertheless, remission of proteinuria was more frequent in patients who received IV methylprednisolone (P = 0.000). The combination of prednisolone with azathioprine was not superior to IV methylprednisolone followed by oral prednisolone. Side effects related to immunossuppressive drugs were observed in 12.8% of patients. CONCLUSION: The clinical outcome of patients with IgAN was related to the severity of clinical and histological involvement. The addition of azathioprine to a corticosteroid-based regimen for IgAN does not improve renal outcome.

Nocturnal hypertension: poor correlation with office blood pressure but strong prognostic factor for target organ damage.

INTRODUCTION: We investigated the correlation between systolic and diastolic nocturnal blood pressure (BP) values and office BP values, as well as parameters of 24-hour ambulatory BP monitoring, in patients with hypertension. In addition, we compared nocturnal hypertensives with nocturnal normotensives regarding their demographic, clinical, and laboratory characteristics, as well as other data from 24-hour BP monitoring. METHODS: The study included 182 consecutive patients who had newly diagnosed, never treated, uncomplicated arterial hypertension. Blood samples were obtained from all patients for the determination of glycaemic and lipidaemic profiles. All underwent a complete echocardiographic examination, including tissue Doppler imaging, measurement of carotid intima-media thickness, measurement of carotid-femoral pulse wave velocity, and determination of the augmentation index of reflected waves (Aix@75), as well as 24-hour ambulatory BP monitoring. The population was divided into nocturnal normotensives (NN, n=77) and nocturnal hypertensives (NH, n=105, nocturnal BP >120/70 mmHg). RESULTS: Although the NH did not differ from the NN as regards the classical cardiovascular risk factors, they showed an excessive inotropic response to exercise (61.9% vs. 22.7%, p=0.028), higher levels of serum uric acid (5.5 ± 1.56 mg/dl vs. 4.7 ± 1.36 mg/dl, p=0.003), as well as greater arterial stiffness, as expressed by a higher carotid-femoral pulse wave velocity (8.6 ± 1.6 m/s vs. 7.9 ± 1.4 m/s, p=0.009), and a greater carotid intima-media thickness (0.74 ± 0.17 mm vs. 0.68 ± 0.15 mm, p=0.007). In addition, although the two groups did not differ significantly as regards office BP values and did not show strong correlations between nocturnal and office BP, both nocturnal diastolic and, especially, systolic BP showed strong correlations with levels of serum uric acid and with subclinical lesions in the heart, central aorta, peripheral vessels, and renal vasculature. CONCLUSIONS: Nocturnal BP is poorly correlated with office BP values. However, the presence of nocturnal hypertension is associated with morphological and functional disturbances of the cardiovascular net. 24-hour ambulatory BP monitoring is an essential tool for revealing this subgroup of hypertensive patients who are at increased cardiovascular risk.

A 31 year old woman with essential hypertension grade III and branch retinal vein occlusion with homozygous C677T MTHFR hyperhomocysteinemia and high Lp(a) levels.

We report a 31-year old woman with essential hypertension grade III and history of branch retinal vein occlusion in the setting of hyperhomocysteinemia due to homozygous MTHFR gene mutation and elevated Lp(a). The patient was treated successfully with antihypertensive treatment, acetylsalicylic acid and multivitamin complex supplementation.