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Systemic lupus erythematosus was diagnosed in a patient at 43 years old. When proteinuria recurred at 57 years old, the first kidney biopsy was performed, and class IV-G (A) + V lupus nephritis was diagnosed. The prednisolone dose was increased to 40 mg/day, and cyclosporine A was introduced. After 1 year, proteinuria had decreased to 0.1 g/day. Prednisolone was discontinued three years later, and cyclosporine A was continued. Thereafter, proteinuria did not reoccur. At 67 years old, a second kidney biopsy showed complete remission of lupus nephritis. Cyclosporine A enabled permanent discontinuation of glucocorticoids in a patient with lupus nephritis.

A 42-year-old Japanese woman was admitted for the evaluation of proteinuria. She had a history of four habitual abortions and valvular heart disease, including severe mitral regurgitation and moderate tricuspid regurgitation. A kidney biopsy showed fibrointimal thickening of interlobular arteries, fibrin thrombosis, and associated focal segmental sclerosis. Although the standard test for antiphospholipid (aPL) antibodies was negative, the patient was diagnosed with antiphospholipid syndrome (APS)-related disease by testing for phosphatidylserine dependent anti-prothrombin anticardiolipin antibody, a non-criterial aPL antibody. A kidney biopsy may lead to a diagnosis of APS in patients with negative laboratory test findings for APS.

A 43-year-old Japanese woman with rheumatoid arthritis treated by infliximab and methotrexate for 11 years was admitted for proteinuria and purpura. A kidney biopsy revealed endothelial damage-dominant nephritis with IgA deposition. Infliximab and methotrexate were discontinued, and tocilizumab was started; however, proteinuria persisted. Therefore, tocilizumab was discontinued, and oral prednisolone and methylprednisolone pulse therapy were administered. After 6 months, urinary protein was less than 0.1 g/day, and purpura subsided. To our knowledge, this is the first case of endothelial damage-dominant nephritis related to IgA vasculitis involving the skin and kidney after long-term use of infliximab and methotrexate.

Objective High-dose melphalan and autologous stem cell transplantation (ASCT) therapy for AL amyloidosis are now associated with reduced mortality based on the application of strict criteria. However, there is no long-term evidence concerning the performance of induction therapy with newer agents, such as bortezomib or daratumumab. Concerns regarding long-term relapse despite treatment with ASCT exist, and missing the opportunity to perform ASCT might occur if induction proves to not be efficacious and cardiac amyloidosis progression deprives the patients of a chance to receive ASCT. We herein report good amyloid control by vincristine, doxorubicin, and dexamethasone (VAD) induction therapy and argue the importance of induction therapy before ASCT. Methods We compared patients who underwent VAD induction and ASCT (VAD+ASCT) with patients who underwent frontline ASCT in our hospital. Patients A total of 26 patients with histologically proven AL amyloidosis were included (18 in the VAD+ASCT group and 8 in the frontline ASCT). Results In the VAD+ASCT group, the 10-year overall survival and renal response rates were 82% and 43%, respectively. The renal response rate at two years in the VAD+ASCT group was significantly better than that in the frontline ASCT group. Although there was no significant difference in the survival rates between the two groups, the time to next treatment or death was significantly better in the VAD+ASCT group than in the the frontline ASCT group. Acute kidney injury was the most frequent reason for failure to receive two courses of VAD, and early mortality was mainly due to gastrointestinal complications. Conclusion Considering that only those who underwent 2 courses of VAD experienced a 10-year renal response, induction therapy was deemed to be directly related to the long-term control of AL amyloidosis.

Polycystic liver disease (PLD) is the most common extrarenal manifestation of autosomal dominant polycystic kidney disease (ADPKD). However, current treatments for PLD are only supportive. We experienced a case of enlarged kidneys and liver in a 53-year-old Japanese man with ADPKD who was on hemodialysis. He underwent renal transcatheter arterial embolization (TAE) for enlarged kidneys. His blood pressure (BP) decreased after renal TAE, and his liver volume decreased from 5,259 mL to 4,647 mL (11.6% reduction) within 1 year after renal TAE. This case suggests that rigorous blood pressure control may be beneficial for ameliorating enlarged PLD.

A 72-year-old man was admitted for examination of proteinuria (9.14 g/day) and leg edema. Essential thrombocythemia (ET) was diagnosed because of thrombocytosis (platelet count, 57.9×10

We encountered an 86-year-old Japanese woman who presented with proteinuria (0.4 g/day) and hematuria (red blood cell sediment100/high-power field), a decreased renal function (serum creatinine, 1.51 mg/dL), and elevated myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA) levels (231 IU/mL) during treatment of rheumatoid arthritis with abatacept (a CTLA-4 agent) and adalimumab (a tumor necrosis factor-α agent). A kidney biopsy showed pauci-immune necrotizing glomerulonephritis, and ANCA-associated vasculitis was diagnosed. Treatment with tocilizumab (an interleukin 6 receptor antibody) monotherapy resulted in the improvement of renal findings and normalization of rheumatoid arthritis disease activity and serum ANCA levels. Tocilizumab can also suppress ANCA-associated vasculitis.

We herein report a 70-year-old man with malaise and muscle weakness that had developed within a month. The patient also had abdominal fullness due to polycystic kidney disease. Severe proximal skeletal muscle weakness and mild elevation of creatinine kinase to 301 IU/L were noted. A muscle biopsy of the right bicep showed polymyositis. Computed tomography showed a right renal mass, and an analysis after right nephrectomy identified clear cell carcinoma. The muscle weakness subsided one month after nephrectomy and intravenous immunoglobulin therapy. Therefore, we suspect that the development of polymyositis in this patient was closely related to renal cell carcinoma.

Renal cyst infection is a frequent and serious complication of autosomal dominant polycystic kidney disease (ADPKD) that is often difficult to treat and can be fatal. While nephrectomy is the standard therapy for severe refractory renal cyst infection, it can be associated with severe adverse events. We experienced a case of repetitive renal cyst infection in a 58-year-old Japanese man with ADPKD on dialysis. He underwent renal transcatheter arterial embolization (TAE) four months after the last episodes of renal cyst infection, and his renal cyst infection has not recurred since renal TAE. This case suggested that renal TAE is effective for preventing repetitive renal cyst infection.

Fabry disease (FD) is an X-linked lysosomal storage disorder resulting from a lack of alpha-galactosidase A (AGALA) activity in lysosomes. We herein report a patient with FD revealed by a renal biopsy who survived seven years after the introduction of peritoneal dialysis despite having severe heart failure due to left ventricular hypertrophy (LVH). FD was diagnosed based on a renal biopsy and biochemical analysis showing a low enzymatic activity of AGALA. A microscopic examination at the autopsy revealed marked hypertrophy and vacuolation of cardiac muscle cells. In our case, cardiac involvement determined the prognosis. Peritoneal dialysis is the modality of choice in the long-term management of dialysis patients with FD.

A 61-year-old woman exhibited right inguinal lymphadenopathy and right lower limb edema approximately 1 month prior to hospitalization. She was diagnosed with high grade B-cell lymphoma, and a lymph node biopsy and fluorescence in situ hybridization indicated MYC, BCL2, and BCL6 rearrangements (triple-hit lymphoma). She had progressive disease that was CD20-negative after two courses of rituximab, cyclophosphamide, doxorubicin, vincristine, methotrexate/ifosfamide, etoposide, high-dose cytarabine (R-CODOX-M/IVAC) therapy. Subsequent etoposide, prednisolone, vincristine, cyclophosphamide, doxorubicin (EPOCH) therapy was not effective. However, after two cycles of gemcitabine, dexamethasone, and cisplatin (GDP) therapy, she achieved a complete response and was able to undergo autologous peripheral blood stem cell transplantation. GDP therapy may be effective as salvage therapy for chemotherapy-resistant triple-hit lymphoma.

Cryoglobulinemic vasculitis (CV) presents with systemic manifestations, including renal disease, arthritis, peripheral neuropathy, and muscle weakness. We encountered two patients who developed severe nephrotic range proteinuria; however, extrarenal manifestations were not noted during the clinical course. A renal biopsy revealed typical membranoproliferative glomerulonephritis (MPGN) with huge thrombus-like endothelial deposits and predominant IgM positivity, but electron microscopy did not reveal any definite microtubules. Immunosuppressive therapy and plasmapheresis were only partially effective, and the improvement was not durable. Biological therapy with rituximab (RTX) had no effect. Renal-limited CV should be recognized as a subset of essential CV.

A 55-year-old man was admitted to our institute to undergo evaluation for proteinuria (5.4 g/day) with lambda-type Bence-Jones protein (BJP). Primary amyloid light chain (AL) amyloidosis and acquired factor X deficiency were diagnosed. High-dose melphalan combined with autologous stem cell transplantation was performed. After three years, the patient's proteinuria normalized, he was negative for urinary BJP, and his factor X activity improved to 105%. Serial renal biopsy showed no progression of amyloid deposition at a biopsy after 5 years, but showed a slight increase in the amyloid deposition after 11 years. This therapy can improve the prognosis of AL amyloidosis; however, there are limitations to the strategy.

A 58-year-old man was referred to our institution for an evaluation of nephrotic range proteinuria. Renal biopsy showed a marked expansion of the mesangial matrix and thickening of glomerular basement membrane (GBM) in periodic acid-silver methenamine (PAM). Immunofluorescence (IF) revealed strong staining for the monoclonal kappa light chain. EM demonstrated massive subendothelial and mesangial dense deposits. As a result, light chain deposition disease (LCDD) was diagnosed. Melphalan and prednisolone (MP) therapy was started, which was continued for 10 years with minimal complications. Serial evaluations of renal histology revealed the resolution of nodular lesions and the glomeruli became nearly normal. MP therapy can therefore be an effective therapeutic option for LCDD if it is continued over the long term.

We herein present a rare case of an autosomal dominant polycystic kidney disease (ADPKD) patient with Caroli's disease, a congenital embryonic biliary tree ductal plate abnormality often associated with autosomal recessive polycystic kidney disease. A 76-year-old woman with ADPKD on hemodialysis was admitted to our hospital with recurrent cholangitis and hepatobiliary stones. Caroli's disease was diagnosed according to typical imaging findings of cystic intrahepatic bile duct dilatation and the central dot sign. Hepatobiliary system abnormalities such as Caroli's disease should be considered in febrile ADPKD patients, even in the absence of typical clinical signs or symptoms.

A 52-year-old Japanese woman was admitted to our hospital with the renal pelvic mass lesion detected on a health screening examination. The surgical specimen contained a mass exhibiting the histological features of immunoglobulin (Ig)G4-related disease, including lymphoplasmacytic infiltration and sclerosis with numerous IgG4-producing plasma cells. Postoperatively, an elevation of the serum IgG4 level was confirmed at 403 mg/dL; however, there was no evidence of tubulointerstitial nephritis or glomerulopathy, including membranous nephropathy, and the urothelium of the renal pelvis was intact without inflammation. We herein report this case in which IgG4-related disease of the renal pelvic region presented with a mass lesion in the intrarenal sinus near the renal pelvis, not 'pyelitis' (as described by Stone).

Objective The prognosis of patients with hypertensive emergencies has recently improved dramatically owing to the development of effective antihypertensive therapy. We examined the histological and clinical features of patients with hypertensive emergency-related nephropathy. Methods Twelve patients (11 men and one woman) were diagnosed as having hypertensive emergencies with acute renal failure according to the Joint National Committee-7 classification of blood pressure for adults and underwent renal biopsies at our hospital between 1995 and 2008. These patients were enrolled in this retrospective study. Results The age of the subjects was 40.1±9.8 years. At presentation, the mean systolic/diastolic blood pressure was 232±32/146±12 mmHg and none of the patients were being treated with antihypertensive drugs, although 10 patients had histories of hypertension. The mean serum creatinine level was 6.1±4.7 mg/dL. All 12 patients showed left ventricular hypertrophy on echocardiography. On light microscopy of the renal biopsy specimens, all 12 patients showed onion skin patterns of the arterioles; however, no fibrinoid necrosis of the small arteries was found. Electron microscopy revealed electron-lucent widening of the subendothelial zone of the glomerular capillary walls in seven patients. One of the 12 patients did not respond to medical therapy and required regular dialysis. The other 11 patients responded to treatment. Conclusion An onion skin pattern of the arterioles is the most frequent histological finding in patients with hypertensive emergency-related nephropathy. Long-standing hypertension might contribute to this arteriolar change, since left ventricular hypertrophy was also seen in these patients. With strict control of hypertension using antihypertensive medications, the prognosis of patients with hypertensive emergency-related nephropathy can be improved.

A 68-year-old Japanese woman with asthma of recent onset and a long history of membranous glomerulonephropathy (MN) was admitted because of multifocal pulmonary infiltrates, marked eosinophilia, mild renal dysfunction, a rash on her feet, and right median nerve paralysis. Although MPO- and PR3-ANCA were negative, skin biopsy demonstrated leukocytoclastic vasculitis and Churg-Strauss Syndrome (CSS) was diagnosed. She also had salivary gland swelling and a high serum IgG4 level. Renal biopsy revealed MN with eosinophil-rich tubulointerstitial nephropathy. Her symptoms resolved after the start of corticosteroid therapy. The present case shows that ANCA-negative CSS can have a clinical condition similar to IgG4-related kidney disease.

A 61-year-old Japanese woman on hemodialysis with autosomal dominant polycystic kidney disease (ADPKD) was admitted to the hospital with gluteal pain. Radiographs demonstrated a fracture of the left pubis. The serum 1,25(OH)(2)-vitamin D and 25(OH)-vitamin D levels were low. A biopsy of the right iliac crest disclosed osteomalacia. Active vitamin D sterol was administered in conjunction with dietary modification. Her gluteal pain was resolved three years later, and healing of the fracture was confirmed by radiology. This case emphasizes that vitamin D deficiency and malnutrition can cause osteomalacia in dialysis patients, even if calcium (Ca) and phosphate (P) levels are controlled by calcium carbonate.

Background Patients who have been on continuous ambulatory peritoneal dialysis (CAPD) for over 10 years are known to have a risk of developing encapsulating peritoneal sclerosis (EPS). However, the prognosis of patients on CAPD for over 10 years remains unclear. Methods To better understand the efficacy of a variety of treatments for EPS, we retrospectively reviewed 25 patients who started CAPD at Toranomon Hospital from 1981 to 1997 and continued it for longer than 10 years. Results The CAPD catheter was removed without peritoneal lavage in the initial 3 patients and they developed massive ascites. They all died of infection without resolution of the ascites. Accordingly, in the remaining 13 patients who did not undergo kidney transplantation, peritoneal lavage therapy was performed for 12 months before removing the CAPD catheter. As a result, 4 patients did not develop EPS. However, 9 patients had EPS with ascites, among whom 4 died of EPS-related diseases and 5 are alive. Five patients underwent cadaveric donor kidney transplantation. At the time of surgery, the CAPD catheter was removed without peritoneal lavage; 1 patient suffered from massive ascites immediately, although this subsided within 3 months after kidney transplantation, and 4 patients remain free from EPS-related symptoms and are doing well. Conclusion Kidney transplantation may be an option for preventing EPS. This study showed that improvement of the uremic state as well as treatment with immunosuppressants including corticosteroids may contribute to preventing EPS.

A 68-year-old woman was referred for evaluation of nephrotic-range proteinuria and a course suggesting rapidly progressive glomerulonephritis. Serum anti-neutrophil cytoplasmic antibody against myeloperoxidase (MPO) was 204 U/ml. A renal biopsy specimen revealed necrotizing glomerulonephritis with crescent formation. However, immunofluorescence showed staining with IgG and C3 along capillary walls. IgG positivity included both IgG1 and IgG4. Electron microscopic examination disclosed both paramesangial and subepithelial deposits. This case suggests that rarely, MPO-ANCA-related crescentic glomerulonephritis may present nephrotic-range proteinuria and show immune deposits along capillary walls.