Anti-neutrophil cytoplasmic antibody-associated Pauci-immune crescentic glomerulonephritis complicating Sjögren's syndrome.

Sjögren's syndrome is a chronic autoimmune disease, characterized by specific autoimmune antibodies anti-Ro and anti-La, and it can involve multiple organs, such as the kidneys, lungs, muscles, and nervous system. The most common renal complication of Sjögren's syndrome is tubulointerstitial nephritis, and glomerulonephritis is relatively uncommon. We report the case of an 86-year-old man presenting with recurrent fever, poor appetite, decreased salivary secretion, and body weight loss. Laboratory investigation revealed that serum creatinine was 4.2 mg/dL, proteinuria was 3+, and there was microscopic hematuria. Positive perinuclear anti-neutrophil cytoplasmic antibody, anti-Ro, and anti-La antibodies were detected. Renal biopsy showed crescentic glomerulonephritis with scanty immune complex deposition. The patient was diagnosed with primary Sjögren's syndrome complicated with rapidly progressive glomerulonephritis with positive anti-neutrophil cytoplasmic antibody. Unlike the patients of other case reports, our patient's renal function did not recover after immunosuppressant treatment, and he finally received long-term hemodialysis. Pauci-immune glomerulonephritis is a rare renal complication of Sjögren's syndrome, and progress to renal failure in such patients is possible.

Primary aldosteronism: changes in cystatin C-based kidney filtration, proteinuria, and renal duplex indices with treatment.

OBJECTIVES: To obtain information about the effect of prolonged aldosterone excess on kidney function. METHODS: We determined kidney function changes defined by cystatin C-based estimations of glomerular filtration rate (CysC-GFR). Pretreatment proteinuria and intrarenal Doppler velocimetric indices in primary aldosteronism were examined and followed after adrenalectomy or spironolactone treatment. RESULTS: This prospective, multicenter study included 130 primary aldosteronism patients (56 men; age, 49.9 ± 13.4 years: 100 with adenoma and 30 with idiopathic hyperaldosteronism) and 73 essential hypertension patients (36 men; age, 51.4 ± 14.8 years) as controls. Patients with primary aldosteronism had higher CysC-GFR (P < 0.05) and heavier proteinuria (0.042) than those with essential hypertension. With primary aldosteronism, a higher aldosterone-renin ratio (odds ratio, OR = 7.85, P = 0.008) was independently related to pretreatment CysC-GFR. The factors related to pretreatment proteinuria included CysC-GFR (OR, -0.006, P = 0.001), plasma aldosterone concentration (OR, 0.004, P = 0.002), and duration of hypertension (OR, 0.016, P = 0.032). Duration of hypertension was also independently correlated with the pretreatment resistive index among primary aldosteronism patients (OR, 0.004, P = 0.035). CysC-GFR (all, P < 0.05), proteinuria (P < 0.001), and resistive index (P < 0.001) decreased 1 year after adrenalectomy but not with spironolactone treatment. CONCLUSION: Our data suggest that prolonged hyperaldosteronism will cause relative kidney hyperfiltration and reversible intrarenal vascular structural changes, which disguise the consequent renal injury, including declining GFR and proteinuria. Adrenalectomy and spironolactone treatment exert different clinical impacts toward kidney damage even with a similar blood pressure-lowering effect.

Impact of timing of renal replacement therapy initiation on outcome of septic acute kidney injury.

INTRODUCTION: Sepsis is the leading cause of acute kidney injury (AKI) in critical patients. The optimal timing of initiating renal replacement therapy (RRT) in septic AKI patients remains controversial. The objective of this study is to determine the impact of early or late initiation of RRT, as defined using the simplified RIFLE (risk, injury, failure, loss of kidney function, and end-stage renal failure) classification (sRIFLE), on hospital mortality among septic AKI patients. METHODS: Patient with sepsis and AKI requiring RRT in surgical intensive care units were enrolled between January 2002 and October 2009. The patients were divided into early (sRIFLE-0 or -Risk) or late (sRIFLE-Injury or -Failure) initiation of RRT by sRIFLE criteria. Cox proportional hazard ratios for in hospital mortality were determined to assess the impact of timing of RRT. RESULTS: Among the 370 patients, 192 (51.9%) underwent early RRT and 259 (70.0%) died during hospitalization. The mortality rate in early and late RRT groups were 70.8% and 69.7% respectively (P > 0.05). Early dialysis did not relate to hospital mortality by Cox proportional hazard model (P > 0.05). Patients with heart failure, male gender, higher admission creatinine, and operation were more likely to be in the late RRT group. Cox proportional hazard model, after adjustment with propensity score including all patients based on the probability of late RRT, showed early dialysis was not related to hospital mortality. Further model matched patients by 1:1 fashion according to each patient's propensity to late RRT showed no differences in hospital mortality according to head-to-head comparison of demographic data (P > 0.05). CONCLUSIONS: Use of sRIFLE classification as a marker poorly predicted the benefits of early or late RRT in the context of septic AKI. In the future, more physiologically meaningful markers with which to determine the optimal timing of RRT initiation should be identified.

Combining body mass index and serum potassium to urine potassium clearance ratio is an alternative method to predict primary aldosteronism.

BACKGROUND: Though aldosterone-renin ratio (ARR) is the current routine screening method for suspicious primary aldosteronism, we hypothesized that the simple formula combining body mass index (BMI) and serum potassium to urine potassium clearance (PUKC) ratio was comparable to ARR. METHODS: Records of patients who were referred to the National Taiwan University Hospital for investigation of primary aldosteronism from January 1995 through December 2007 were retrieved. Primary aldosteronism was diagnosed based on the modified 4-corners criteria, otherwise essential hypertension was diagnosed. In both groups, the PUKC/BMI ratio was determined as well as the ARR. Bland-Altman and mountain-plot analysis were used to validate the agreement between ARR and PUKC/BMI. Receiver operating characteristic (ROC) curves were used to compare the sensitivity and specificity of PUKC/BMI and ARR. RESULTS: The records for urinary potassium were analyzed for 177 hypertensive patients (134 patients with primary aldosteronism). ROC curves showed comparable areas under the curves of both methods (95% CI: -0.029 to 0.183; p=0.186). Bland-Altman analysis further supported the agreement between ARR and PUKC/BMI ratio. CONCLUSIONS: We found that the screening power of PUKC/BMI was as good as that of conventional ARR. With the quick and extensive availability of the PUKC/BMI method and its equivalence to ARR, this screening strategy would be a good first-line tool for massive community-based primary aldosteronism surveys.

Verification and evaluation of aldosteronism demographics in the Taiwan Primary Aldosteronism Investigation Group (TAIPAI Group).

OBJECTIVE: Current data on primary aldosteronism (PA) from Asian populations are scarce. This cohort study clarifies the attributes of patients with PA in a typical Chinese population. DESIGN: An observational cohort study. METHODS: The records of patients referred to the Hypertension Clinic from a multi-centre registration in Taiwan from January 1995 to December 2007 were reviewed. All patients with PA were classified into two subtypes: aldosterone-producing adenomas (APA) and idiopathic hyperaldosteronism (IHA); their characteristics were compared. RESULTS: Our cohort consisted of 346 patients with PA, 255 with APA and 91 with IHA. The initial hypokalaemia (59% in APA vs. 27.5% in IHA, p < 0.0001) and transtubular potassium gradient (TTKG) (6.30 ± 2.41 in APA vs. 4.91 ± 2.03 in IHA, p = 0.01) were higher in the APA group. Baseline plasma aldosterone concentration (PAC) was also significantly different between the two subgroups (49.96 ± 38.15 ng/dl in APA vs. 34.24 ± 21.47 in IHA, p < 0.0001). CONCLUSIONS: In typical Chinese PA patients, the APA subgroup had a higher proportion of hypokalaemia with elevated TTKG and higher PAC as compared with the IHA subgroup. This largest Asian database also demonstrated major differences between the Caucasian and Chinese populations including female predilection, frequent hypokalaemia, and common paralytic myopathy.