Circulating antibodies to α-enolase and phospholipase A2 receptor and composition of glomerular deposits in Japanese patients with primary or secondary membranous nephropathy.

BACKGROUND: Phospholipase A2 receptor (PLA2R) is recognized as a target antigen in primary membranous nephropathy (MN); Anti-α-enolase antibody in primary and secondary MN has been proposed, however, little is known about the potential contribution of α-enolase to the pathogenesis of MN. METHODS: We evaluated circulating antibodies to α-enolase by a dot blotting system and PLA2R by indirect immunofluorescence, and glomerular deposition of these proteins in 25 patients with primary MN, 20 patients with secondary MN, 44 patients with collagen disease or severe infection, 60 patients with nephritis (each ten patients of IgA nephropathy, focal segmental gloemrulosclerosis, minimal change nephrotic syndrome, membranoproliferative glomeurlonephritis, diabetic glomerulosclerosis, and tubulointerstitial nephritis) as disease control, and 20 healthy subjects. RESULTS: In primary MN, 18 of 25 sera (72 %) showed anti-α-enolase antibody (IgG1 and IgG4, 11 pts; IgG4 alone, six pts; IgG1 alone, one pt). In secondary MN, 15 of 20 sera (75 %) contained anti-α-enolase antibody (IgG1 and IgG3, 13 pts; IgG3 alone, two pts). No circulating anti-α-enolase antibody was found in 44 collagen diseases or septic patients, 60 nephritis without MN, and 20 healthy subjects. Twelve of 25 sera (48 %) from patients with primary MN were positive for anti-PLA2R antibody, whereas all patients with secondary MN were negative. Eight of the 12 PLA2R-positive patients (67 %) with primary MN also had anti α-enolase antibody. Although PLA2R antigen was present in a subepithelial pattern in 10 of 19 (52 %) patients with primary MN, α-enolase was never detected in glomerular deposits in 19 and ten patients with primary and secondary MN, respectively. CONCLUSIONS: Circulating anti-α-enolase antibodies are highly present in both primary and secondary MN (about 70 %, respectively), while anti-PLA2R antibodies are specific for primary MN (48 %) with a prevalence apparently lower in the Japanese population than in Chinese and Caucasian populations. The absence of α-enolase from subepithelial immune deposits suggests that anti-α-enolase antibodies do not contribute directly to immune-deposit formation, although they may have other pathogenic effects.

Acid-base disturbances in nephrotic syndrome: analysis using the CO2/HCO3 method (traditional Boston model) and the physicochemical method (Stewart model).

BACKGROUND: The Stewart model for analyzing acid-base disturbances emphasizes serum albumin levels, which are ignored in the traditional Boston model. We compared data derived using the Stewart model to those using the Boston model in patients with nephrotic syndrome. METHODS: Twenty-nine patients with nephrotic syndrome and six patients without urinary protein or acid-base disturbances provided blood and urine samples for analysis that included routine biochemical and arterial blood gas tests, plasma renin activity, and aldosterone. The total concentration of non-volatile weak acids (ATOT), apparent strong ion difference (SIDa), effective strong ion difference (SIDe), and strong ion gap (SIG) were calculated according to the formulas of Agrafiotis in the Stewart model. RESULTS: According to the Boston model, 25 of 29 patients (90%) had alkalemia. Eighteen patients had respiratory alkalosis, 11 had metabolic alkalosis, and 4 had both conditions. Only three patients had hyperreninemic hyperaldosteronism. The Stewart model demonstrated respiratory alkalosis based on decreased PaCO2, metabolic alkalosis based on decreased ATOT, and metabolic acidosis based on decreased SIDa. We could diagnose metabolic alkalosis or acidosis with a normal anion gap after comparing delta ATOT [(14.09 - measured ATOT) or (11.77 - 2.64 × Alb (g/dL))] and delta SIDa [(42.7 - measured SIDa) or (42.7 - (Na + K - Cl)]). We could also identify metabolic acidosis with an increased anion gap using SIG > 7.0 (SIG = 0.9463 × corrected anion gap-8.1956). CONCLUSIONS: Patients with nephrotic syndrome had primary respiratory alkalosis, decreased ATOT due to hypoalbuminemia (power to metabolic alkalosis), and decreased levels of SIDa (power to metabolic acidosis). We could detect metabolic acidosis with an increased anion gap by calculating SIG. The Stewart model in combination with the Boston model facilitates the analysis of complex acid-base disturbances in nephrotic syndrome.

A multicenter randomized controlled trial of tonsillectomy combined with steroid pulse therapy in patients with immunoglobulin A nephropathy.

BACKGROUND: The study aim was, for the first time, to conduct a multicenter randomized controlled trial to evaluate the effect of tonsillectomy in patients with IgA nephropathy (IgAN). METHODS: Patients with biopsy-proven IgAN, proteinuria and low serum creatinine were randomly allocated to receive tonsillectomy combined with steroid pulses (Group A; n = 33) or steroid pulses alone (Group B; n = 39). The primary end points were urinary protein excretion and the disappearance of proteinuria and/or hematuria. RESULTS: During 12 months from baseline, the percentage decrease in urinary protein excretion was significantly larger in Group A than that in Group B (P < 0.05). However, the frequency of the disappearance of proteinuria, hematuria, or both (clinical remission) at 12 months was not statistically different between the groups. Logistic regression analyses revealed the assigned treatment was a significant, independent factor contributing to the disappearance of proteinuria (odds ratio 2.98, 95% CI 1.01-8.83, P = 0.049), but did not identify an independent factor in achieving the disappearance of hematuria or clinical remission. CONCLUSIONS: The results indicate tonsillectomy combined with steroid pulse therapy has no beneficial effect over steroid pulses alone to attenuate hematuria and to increase the incidence of clinical remission. Although the antiproteinuric effect was significantly greater in combined therapy, the difference was marginal, and its impact on the renal functional outcome remains to be clarified.

Estimated glomerular filtration rate and daily amount of urinary protein predict the clinical remission rate of tonsillectomy plus steroid pulse therapy for IgA nephropathy.

BACKGROUND: This retrospective study was designed to estimate the clinical remission (CR) rate of tonsillectomy plus steroid pulse (TSP) therapy in patients with IgA nephropathy. METHODS: Based on 292 of 302 patients with IgA nephropathy treated at 11 Japanese hospitals, we constructed heat maps of the CR rate at 1 year after TSP with the estimated glomerular filtration rate (eGFR), grade of hematuria, pathological grade, number of years from diagnosis until TSP, and age at diagnosis on the vertical axis and the daily amount of urinary protein (urinary protein) on the horizontal axis. We compared subgroups usinge Student's t test, the chi-square test with Yates correction, or Fisher's exact probability test. RESULTS: The first heat map of eGFR and urinary protein showed that the CR rate was 71 % (CR vs. non-CR, 96 vs. 40) in patients with eGFR greater than 30 ml/min/1.73 m(2) and 0.3-1.09 g/day of urinary protein. However, the CR rate in patients with more than 1.50 g/day of urinary protein was approximately 30 %. The second heat map of grade of hematuria and urinary protein revealed that the CR rate is 72 % (CR vs. non-CR, 93 vs. 37) in patients with more than 1+ hematuria and 0.3-1.09 g/day of urinary protein; however, it was 28.6 % in patients with no hematuria. The third heat map of pathological grade and urinary protein demonstrated that the highest CR rate was 83 % (CR vs. non-CR, 52 vs. 11) in patients with pathological grade I or II disease and less than 1.09 g/day of urinary protein, as opposed to 22 % (CR vs. non-CR, 9 vs. 32) in patients with pathological grade III or IV disease and more than 2.0 g/day of urinary protein. The fourth heat map of the number of years from diagnosis until TSP and urinary protein revealed that the former did not influence the CR rate in patients with less than 1.09 g/day of urinary protein. However, in patients with more than 1.10 g/day of urinary protein, the CR rate of the subgroup with less than 6 years was 43 % (CR vs. non-CR; 23 vs. 54) compared to 23 % (CR vs. non-CR, 11 vs. 48; P = 0.01) in the subgroup with more than 6 years. The fifth heat map of age at diagnosis and urinary protein showed that the CR rate is approximately 72 % (CR vs. non-CR, 73 vs. 28) in patients older than 19 years at diagnosis with 0.3-1.09 g/day of urinary protein. CONCLUSIONS: The daily amount of urinary protein is an important predictor of the CR rate after TSP in IgA nephropathy patients. Heat maps are useful tools for predicting the CR rate associated with TSP.

Retrospective analysis of factors predicting end-stage renal failure or death in patients with microscopic polyangiitis with mainly renal involvement.

BACKGROUND: The aim of this study was to identify risk factors for end-stage renal failure (ESRF) or death in Japanese patients with microscopic polyangiitis (MPA) with renal involvement. METHODS: From 54 consecutive patients with systemic vasculitis based on Watt's algorithm, we retrospectively analyzed 39 MPA patients with renal involvement, including 19 (48.7 %) with renal-limited vasculitis. RESULTS: Thirty-three of 39 patients (84.6 %) demonstrated rapidly progressive glomerulonephritis, and 13 (33.3 %) developed ESRF; 8 of 13 required dialysis within 1 week. Thirteen (33.3 %) died during follow-up of more than 12 months, and 7 died during the first 6 months, mainly because of opportunistic infections. Overall survival at 6 and 12 months was 79.5 and 71.1 %, respectively. Serum creatinine levels did not differ significantly between survivors and non-survivors (P = 0.092). The mean Birmingham Vasculitis Activity Score, version 3 (BVAS v.3), was 16.2 ± 6.5, with a renal subscore of over 12 points in 82.1 %, and BVAS v.3 was marginally higher in non-survivors than survivors (P = 0.045). An age- and sex-adjusted Cox proportional hazards analysis demonstrated that neither the serum creatinine level (P = 0.277) nor BVAS v.3 (P = 0.188) at initial diagnosis was a risk factor for overall survival. The baseline serum creatinine cutoff value for discriminating between ESRF and non-ESRF was 4.6 mg/dl, with a sensitivity and specificity of 92.3 and 84.6 %, respectively. CONCLUSIONS: Survival rates do not relate to ESRF in MPA patients with mainly renal involvement. Although patients with ESRF required regular hemodialysis, longer survival can be achieved.

Glomerular tip adhesions predict the progression of IgA nephropathy.

BACKGROUND: Focal segmental glomerulosclerosis-like lesions have been proposed to be predictive factors for IgA nephropathy. This single center, retrospective cohort study was designed to clarify which clinical and pathological factors are predictive of decreased estimated glomerular filtration rate (eGFR) at 5 and 10 years in IgA nephropathy patients. METHODS: Of the 229 patients with IgA nephropathy who were admitted to Aichi Medical University Hospital between 1986 and 2010, 57 were included in this study during the 5 to 10 years after renal biopsy. Clinical, laboratory, and pathological parameters were analyzed by multiple linear regression analysis with backward elimination to determine independent risk factors. After identifying such factors, we compared patients with and without each factor using the Student's t test, Wilcoxon test, or Mann-Whitney U test. RESULTS: Four variables were identified as predictive factors for progression of IgA nephropathy: initial eGFR (p = 0.0002), glomerular tip adhesion (p = 0.004), global sclerosis (p = 0.019), and diastolic blood pressure (p = 0.024). The annual decrease in eGFR of patients with (n = 9) or without glomerular tip adhesions (n = 48) was 4.13 ± 3.58 and 1.49 ± 2.89 ml/min/1.73 m2, respectively (p = 0.015). Serum total cholesterol levels were 231 ± 45 mg/dl and 196 ± 42 mg/dl, respectively (two-sided p = 0.064; one-sided p = 0.032). CONCLUSIONS: The presence of glomerular tip adhesions predicts the progression of IgA nephropathy. High levels of serum total cholesterol may affect glomerular tip adhesions.

A treatment dilemma in adult immunoglobulin A nephropathy: what is the appropriate target, preservation of kidney function or induction of clinical remission?

Immunoglobulin (Ig) A nephropathy is the most common type of glomerulonephritis worldwide. Data on its natural history suggest that approximately 40% of patients progress to end-stage renal failure after 20 years. Various therapies such as antiplatelet medication, fish oil, oral prednisolone, intravenous prednisolone, tonsillectomy, and tonsillectomy plus steroid pulse (TSP) have been proposed. Japanese nephrologists face challenging issues regarding this disease, such as the usefulness of the annual urinary screening system (kenshin) and kidney biopsies, the desire of patients and their families for treatment despite insufficient clinical evidence, and the risk of overtreatment with TSP versus the loss of a 'golden period' with late intervention. We review the current literature on tonsillectomy, steroid therapy, and TSP, which was first proposed in Japan, and present some perspectives on the treatment of IgA nephropathy.

A large-sized bubbling appearance of the glomerular basement membrane in a patient with pulmonary limited AL amyloidosis and a past history of lupus nephritis.

We report an unusual pathological finding, a large-sized bubbling appearance of the glomerular basement membrane (GBM), in a patient with pulmonary limited AL amyloidosis and a past history of lupus nephritis. The first renal biopsy specimen from 10 years ago, when systemic lupus erythematosus was diagnosed, demonstrated mild mesangial proliferation and subepithelial deposits (WHO classification: III + V). Light microscopy of the current biopsy using periodic acid methenamine silver (PAMS) stain demonstrated a large-sized bubbling appearance of the GBM; however, very weak immunoglobulin and complement deposition was observed in immunofluorescence studies. Routine electron microscopy demonstrated partial subendothelial expansion with electron-lucent materials, but no electron-dense deposits or amyloid fibrils. Electron microscopy with PAMS stain revealed electron-lucent endothelial scalloping, including some cellular components and microspheres in the GBM; however, it is not clear if these materials are derived from endothelial cells. One possibility is that these unique findings represent a recovery phase of lupus membranous nephritis; another is that these findings correspond to a new disease entity.

Acute peritonitis due to Corynebacterium ulcerans in a patient receiving continuous ambulatory peritoneal dialysis: a case report and literature review.

A 55-year-old Japanese woman receiving continuous ambulatory peritoneal dialysis (CAPD) was admitted to our service with abdominal pain and cloudy peritoneal fluid. Laboratory data revealed a white blood cell count of 7.20 × 10(9 )cells/L, hemoglobin 9.8 g/dl, hematocrit 29.0%, platelet count 284 × 10(9 )cells/L, and C-reactive protein (CRP) 0.109 g/L. Peritoneal fluid white blood cell count of 2,000 cells/µl suggested acute peritonitis. An empiric trial of cefazolin and ceftazidime, subsequently switched to meropenem, vancomycin, minocycline, and amikacin, did not improve the patient's symptoms. The peritoneal fluid collected before initiation of antibiotic therapy grew Corynebacterium ulcerans. Ampicillin/sulbactam was started based on the culture and sensitivity data. On hospital day 8, the CAPD catheter was removed due to no clinical improvement and persistently increased levels of CRP to 0.0174 g/L. A 14-day course of ampicillin/sulbactam improved her clinical condition and laboratory data. Microbiological analysis revealed that C. ulcerans isolated from this patient did not produce diphtheria toxin. C. ulcerans was not isolated from her dog's oral and nasal cavities during a search for the route of her infection. We recommend that in patients with peritoneal dialysis, special attention should be paid to Corynebacterium peritonitis, especially due to C. ulcerans, which may produce diphtheria toxin, be resistant to multiple antibiotics, and frequently become recurrent.

Myeloperoxidase-antineutrophil cytoplasmic antibody-related crescentic glomerulonephritis after treatment for clinically amyopathic dermatomyositis: a coincidental combination or not?

A 60-year-old Japanese man exhibited rapidly progressive glomerulonephritis 10 years after receiving prednisolone therapy for clinically amyopathic dermatomyositis (CADM). Upon admission, there were no signs of dermatomyositis. Laboratory analyses revealed the presence of myeloperoxidase-antineutrophil cytoplasmic antibodies (MPO-ANCA) at 1,280 EU in the absence of anti-glomerular basement membrane antibody and anti-melanoma differentiation-associated gene 5 antibodies, which are typically expressed in CADM. A renal biopsy demonstrated that 14 of 29 glomeruli showed global sclerosis, and the remaining 15 glomeruli exhibited fibrotic and fibrocellular crescent formation without immunoglobulin and complement. Following treatment with 500 mg/day methylprednisolone pulse therapy for 3 days, the patient was started on 30 mg/day of prednisolone orally. On the third day of hospitalization, we began hemodialysis for uremia and anuria with three treatments of plasma exchange starting on the tenth hospital day. Unfortunately, the patient's renal function did not recover, despite decreases in CRP and MPO-ANCA levels to the normal range. This case is the first English language report of MPO-ANCA-related crescentic glomerulonephritis in a patient who had recovered from CADM.

Progressive renal failure despite long-term biweekly enzyme replacement therapy in a patient with Fabry disease secondary to a new α-galactosidase mutation of Leu311Arg (L311R).

A 37-year-old Japanese man affected by Fabry disease secondary to a novel mutation of Leu311Arg (L311R) in α-galactosidase demonstrated progressive renal failure despite biweekly enzyme replacement therapy (ERT) for approximately 10 years. Kidney biopsy revealed foamy glomerular epithelial cells, compatible with the typical pathologic features of Fabry disease. The patient entered a phase III study of Replagal (agalsidase alfa) in 2001, allowing him to continue ERT with biweekly dosing for almost 10 years. During 2 years of that period, he was continued on Fabrazyme (agalsidase beta) biweekly dosing. His estimated GFR was calculated to decrease by 9.9 mL/min/1.73 m(2) per year. Patients with Fabry disease have been reported to have a mean decrease in GFR of 12.2 ± 8.1 mL/min/1.73 m(2) per year. This result suggests that biweekly ERT is only mildly effective at preventing loss of kidney function.

Torsades de Pointes induced by a combination of garenoxacin and disopyramide and other cytochrome P450, family 3, subfamily A polypeptide-4-influencing drugs during hypokalemia due to licorice.

We report an 82-year-old man who developed ventricular tachycardia and Torsades de Pointes (TdP) after oral administration of garenoxacin, a novel quinolone antibiotic agent that differs from the third-generation quinolones, for pneumonia. He had hypokalemia (K 2.3 mmol/L) induced by licorice and also had received disopyramide for arrhythmia, bicalutamide for prostate cancer, and silodosin for prostate hypertrophy. After taking him off all drugs and administering spironolactone supplemented with potassium, his low serum potassium level was ameliorated. Therefore, although garenoxacin reportedly causes fewer adverse reactions for cardiac rhythms than third-generation quinolone antibiotics, one must be cautious of the interference of other drugs during hypokalemia in order to prevent TdP.

An IgA1-lambda-type monoclonal immunoglobulin deposition disease associated with membranous features in a patient with chronic hepatitis C viral infection and rectal cancer.

A 61-year-old man infected with hepatitis C virus developed urinary protein. Two-dimensional electrophoresis and immunoblotting of sera revealed no monoclonal proteins. Light microscopy and immunofluorescence of a kidney biopsy specimen demonstrated bubbling appearance and formation of spikes, associated with predominantly IgA1-lambda deposition, but not IgG, along glomerular capillary walls. Electron microscopy showed electron-dense deposits without any fibrillary structure located in the glomerular basement membrane. Seven months after the kidney biopsy, the patient had a surgical operation for rectal cancer. One year later, the urinary protein was still present. The present case is the first report of an IgA1-lambda-type monoclonal immunoglobulin deposition disease associated with membranous features.

Thrombotic thrombocytopenic purpura in a patient with rapidly progressive glomerulonephritis with both anti-glomerular basement membrane antibodies and myeloperoxidase anti-neutrophil cytoplasmic antibodies.

A 61-year-old Japanese woman with rapidly progressive glomerulonephritis exhibited both anti-glomerular basement membrane (GBM) antibodies (920 EU) and myeloperoxidase anti-neutrophil cytoplasmic antibodies (MPO-ANCA; 66 EU). Multiple plasma exchanges with fresh frozen plasma preceded by 500 mg/day intravenous methylprednisolone and 30 mg/day oral prednisolone decreased anti-GBM antibody and MPO-ANCA antibody titers to 106 EU and below 10 EU (normal ranges), respectively. Thrombotic thrombocytopenic purpura (TTP) manifests itself as a moderate decrease in the activity of disintegrin-like and metalloproteinase with thrombospondin type 1 motif, 13 (ADAMTS13) protein levels to 35% of normal; ADAMTS13 deficiency is only symptomatic when levels are less than 50%. This patient's disease was resistant to extensive plasma exchange, leading to her death from respiratory distress and perforation of the alimentary tract secondary to cytomegalovirus infection. Autopsy demonstrated severe crescentic glomerulonephritis associated with linear IgG, IgA, IgM, and C3 deposits along the glomerular capillary walls. This case is an uncommon example of combined double antibody-positive crescentic glomerulonephritis and refractory TTP.

A case of chronic kidney disease with thrombotic microangiopathy in a hematopoietic stem cell transplant recipient.

A 23-year-old Japanese man who had undergone hematopoietic stem cell transplantation for acute lymphocytic leukemia from an HLA-identical sibling 6 years earlier developed proteinuria and impaired kidney function. Kidney biopsy revealed thrombotic microangiopathy with a moderate increase in mesangial matrices and glomerular microaneurysm featuring retention of red blood cells. The patient's kidney function gradually deteriorated, requiring the institution of treatment with angiotensin II receptor blockers and angiotensin-converting enzyme inhibitors, and progressing to continuous ambulatory peritoneal dialysis 4 years after the initial kidney biopsy. Eventually, kidney transplantation was performed with his mother as the donor. His kidney function is stable on immunosuppressive drugs at 2 years after transplantation. This report reflects the growing number of patients with chronic kidney disease with thrombotic microangiopathy all over the world.

Tonsillectomy and steroid pulse (TSP) therapy for patients with IgA nephropathy: a nationwide survey of TSP therapy in Japan and an analysis of the predictive factors for resistance to TSP therapy.

BACKGROUND: Tonsillectomy and steroid pulse (TSP) therapy was proposed as a curative treatment for IgA nephropathy by Hotta et al. (Am J Kidney Dis 38:736-742, 2001) based on data that about 50% of patients achieved clinical remission (CR) of urinary abnormalities. MATERIALS AND METHODS: As a primary survey, we sent a questionnaire and letter to 848 hospitals in Japan, each of which employed a Fellow of the Japanese Society of Nephrology between October and December of 2006, in order to gather information about the prevalence and efficacy of TSP therapy for patients with IgA nephropathy. As a secondary survey, we collected data from both low- and high-CR-rate groups to determine which factors predicted resistance to TSP therapy. RESULTS: A total of 2,746 patients received TSP therapy between 2000 and 2006. The CR rates, calculated by measuring urinary criteria 6 and 12 months after TSP therapy, were 32.0% (347/1,085) and 45.6% (452/991), respectively. Analysis of the 30 hospitals in which TSP therapy had been performed on at least ten patients revealed that the CR rates varied from below 10% to 100%. A secondary survey of ten hospitals revealed that, after correction of the CR rate from each hospital, patients could be categorized into three groups: those with a low CR rate (122 patients in four hospitals), a middle CR rate (78 patients in four hospitals), and a high CR rate (103 patients in two hospitals). The CR rate of all patients (N = 303) was 54.1%. A comparison of patient data between the low- and high-CR-rate groups showed a significant difference in age at onset (years; P = 0.05), amount of proteinuria (g/day; P = 0.02), total protein (g/dl; P = 0.02), pathological grade (P = 0.009), and prognostic score as described by Wakai et al. [Nephrol Dial Transplant 21:2800-2808, 2006, (P = 0.04)]. Univariate analysis revealed that there was a significant difference between non-CR and CR subgroups in duration from diagnosis until TSP therapy (6.9 +/- 6.8 versus 5.3 +/- 5.2 years; P = 0.02), amount of proteinuria (1.5 +/- 1.6 versus 0.8 +/- 0.8 g/day; P < 0.0001), serum creatinine (0.99 +/- 0.40 versus 0.87 +/- 0.34 mg/dl; P = 0.006), pathological grade (P = 0.0006), and Wakai et al.'s prognostic score (37.4 +/- 17.8 versus 28.1 +/- 15.1; P < 0.0001). A multivariate logistic analysis demonstrated that resistance to TSP therapy depends on age at onset, amount of proteinuria, hematuria grade, and pathological grade, and a score predicting resistance to TSP therapy could be derived by the formula: [(-0.0330) x (age) + (0.4772) x log (amount of proteinuria) - (0.0273) x (hematuria grade: 0, 1, 2, and 3) + (0.7604) x (pathological grade: 1, 2, 3, and 4) - 0.1894]. A receiver operating characteristic (ROC) curve showed that patients with a resistance score of greater than -0.02 easily resist TSP therapy (sensitivity 69%, specificity 75%, positive likelihood ratio 2.76). CONCLUSION: TSP therapy shows promise as a treatment that can bring about CR of urinary abnormalities, but unfortunately the average CR rate is about 50% at 1 year after treatment. Predictive factors for resistance to TSP therapy are age at onset, amount of proteinuria, hematuria grade, and pathological grade. The present study suggests that patients with either early-stage or mild to moderate IgA nephropathy easily achieve CR following TSP therapy, whereas patients with late-stage or severe disease are prone to TSP therapy resistance.

Analysis of finger vein variety in patients with various diseases using vein authentication technology.

In finger vein authentication technology, near-infrared rays penetrate the finger and are absorbed by the hemoglobin in blood. The veins appear as dark areas. The finger vein pattern images of patients with various diseases were acquired; a new evaluation method applying image processing technique ("E value") was developed, and it was examined whether the patterns have any characteristics differentiating them from those of healthy volunteers. As a result, low E values appeared in systemic sclerosis, mixed connective tissue disease, Sjögren's syndrome, and polymyositis/dermatomyositis. No statistical reduction in E value was shown in patients with rheumatoid arthritis, pernio (without rheumatic diseases), arteriosclerosis obliterans, diabetes, hypertension, hypothyroidism and alopecia areata. This technology could be used for screening and evaluation of some diseases and their conditions with impaired peripheral venous circulation. E value may be useful as an indicator of venous circulation.

Light Chain Deposition Disease Diagnosed with Laser Micro-dissection, Liquid Chromatography, and Tandem Mass Spectrometry of Nodular Glomerular Lesions.

A 42-year-old man developed nephrotic syndrome and rapidly progressive renal failure. Kidney biopsy demonstrated nodular glomerulosclerosis, negative Congo red staining, and no deposition of light or heavy chains. Laser micro-dissection and liquid chromatography with tandem mass spectrometry of nodular lesions revealed the presence of a kappa chain constant region and kappa III variable region, which signified light chain deposition disease. Dexamethasone and thalidomide were effective in decreasing the serum levels of free kappa light chain from 147.0 to 38.0 mg/L, eliminating proteinuria, and halting the worsening of the kidney dysfunction, with serum creatinine levels stable around 4.0 mg/dL for 3 years.