[Tubulointerstitial nephritis with IgM-positive plasma cells in an elderly woman diagnosed by a renal biopsy and treated with corticosteroid therapy].

An 81-year-old female was referred to our department 1 year ago due to a worsening renal function. Her manifestations met the criteria of Sjögren syndrome, suggesting renal failure likely resulting from tubulointerstitial nephritis (TIN) due to Sjögren syndrome. However, at her request, she was followed up with no further investigation or treatment. The following July, since her renal function deteriorated again, a renal biopsy was performed. Using IgM-CD138 dual staining, the renal pathology showed the infiltration of accumulated IgM-positive plasma cells within the renal insterstitium, so she was diagnosed with tubulointerstitial nephritis with IgM-positive plasma cells (IgMPC-TIN).IgMPC-TIN, proposed by Takahashi et al. in 2017, as a type of TIN, is characterized by the pathological infiltrations of IgM-positive plasma cells within the renal insterstitium and is effectively treated with corticosteroid therapy. Despite her old age, corticosteroid therapy was performed, resulting in the improvement in her renal function according to blood and urine tests and an improved pulmonary involvement, although renal dysfunction remained.Elderly patients often have multiple underlying medical conditions and take numerous medications, so differentiating renal disorders is challenging. However, a renal biopsy, even in an elderly patient, can aid in identifying the cause of renal disorders and predicting the prognosis. IgMPC-TIN is a condition in which renal function can be expected to improve if treated. It is thus important to make a diagnosis of IgMPC-TIN without overlooking and to consider proper treatment.

Hypertensive Crisis and Left Ventricular Thrombi after an Upper Respiratory Infection during the Long-term Use of Oral Contraceptives.

A 34-year-old woman who had been using oral contraceptives for 10 years developed hypertensive crisis with papilloedema after an upper respiratory infection. Laboratory data showed hyperreninemic hyperaldosteronism and elevated levels of fibrinogen, fibrin, and fibrinogen degradation products. Echocardiography demonstrated two masses (18 mm) in the left ventricle. On the fourth hospital day, cerebral infarction, renal infarction, and upper mesenteric artery occlusion suddenly occurred despite the blood pressure being well-controlled using anti-hypertensive drugs. Echocardiography revealed the disappearance of the left ventricular masses, which suggested left ventricular thrombi. Cessation of the contraceptives and administration of heparin, warfarin, and anti-platelets drugs improved her general condition.

Morganella morganii Peritonitis Associated with Continuous Ambulatory Peritoneal Dialysis (CAPD) after Colonoscopy.

A 79-year-old man on continuous ambulatory peritoneal dialysis (CAPD) developed abdominal pain and cloudy peritoneal fluid two days after colonoscopy that revealed multiple diverticula. The white blood cell count was 9,000 cells/µL, C-reactive protein level was 6.86 mg/dL, and the white blood cell count of the peritoneal fluid was 7,800 cells/µL, suggesting acute peritonitis. Empiric therapy consisting of cefazolin and ceftazidime slowly improved the patient's symptoms. The initial microbiological examination of the peritoneal fluid demonstrated Morganella morganii. He was changed from CAPD to hemodialysis. It is important to consider M. morganii peritonitis in patients with colonic diverticula.

[Successful treatment of high-dose methotrexate-induced oliguric acute renal failure by using a combination of hemodialysis filtration and direct hemoperfusion].

A 64-year-old man with central nervous system metastases from systemic non-Hodgkin lymphoma was treated with high- dose intravenous methotrexate(MTX 3.5 g/m2). The patient subsequently developed oliguric acute renal failure 12 hours after MTX initiation, and his serum MTX level was 163 mM at 26 hours. Hemodialysis filtration(HDF)combined with direct hemoperfusion(DHP)was initiated at 45hours. Seven sessions of combined HDF and DHP and 2 courses of HDF alone were performed, and the mean MTX extraction rates were 68.2% and 74.3%, respectively. The patient experienced severe respiratory failure, febrile neutropenia, myelosuppression, and oral mucositis. However, his urine output began to improve on day 7 after MTX initiation, and his renal function gradually recovered. His serum MTX level declined to 0.04 mM on day 23 after MTX initiation. In the present case, we immediately initiated HDF and DHP and successfully treated the patient for MTX-induced renal failure.

Fat embolism syndrome: an autopsy-proven case involving a patient on dialysis and systemic scleroderma.

A 66-year-old woman receiving continuous ambulatory peritoneal dialysis developed acute respiratory distress 12 hours after a fall. Blood gas analysis revealed hypoxia (PaO2 67.7 torr) and metabolic acidosis with an increased anion gap, consistent with lactic acidosis (lactate, 86.5 mg/dL; normal range, 4.0-16.0). Magnetic resonance imaging showed a lumbar vertebral body fracture. On the fourth hospital day, the patient died of multiorgan failure and disseminated intravascular coagulation. Postmortem studies revealed fat emboli in the systemic circulation, ie, fat embolism syndrome. Diagnosing fat embolism syndrome can be difficult in patients on dialysis or in those with collagen vascular or pulmonary diseases.

Systemic AA amyloidosis in a patient with lung metastasis from renal cell carcinoma.

AA amyloidosis occurs in patients with high levels of serum amyloid A protein (SAA), which is produced by liver cells in response to signals from several pro-inflammatory cytokines. Chronic inflammatory disease is a major cause of AA amyloidosis; however, malignant neoplasms are rarely reported to be associated with AA amyloidosis. We report herein a case of a solitary lung metastasis of renal cell carcinoma associated with systemic AA amyloidosis. Pathological specimens of the resected lung tumor demonstrated renal cell carcinoma, and the presence of IL-1ß, IL-6, and TNF-α in the lymphocytes and plasma cells surrounding the tumor cells, and AA amyloid in the vascular area, but not in metastatic clear cells. Four weeks after surgery, serum IL-6, SAA, and CRP levels normalized. Although this case is very rare, it is full of interesting suggestions about the pathogenesis of malignancy-related systemic amyloidosis.

Differential diagnosis of localized and systemic amyloidosis based on coagulation and fibrinolysis parameters.

BACKGROUND: A simple assay that can discriminate between localized and systemic amyloidosis is needed. METHODS: Coagulation and fibrinolysis parameters were measured in subjects with active or progressive systemic amyloidosis (Group A; 9 patients), systemic amyloidosis in complete remission (Group B; 6 patients), localized AL amyloidosis (Group C; 6 patients), monoclonal gammopathy of undetermined significance (Group D; 5 patients), chronic glomerulonephritis with proteinuria (Group E; 22 patients), or glomerulonephritis in complete remission (Group F; 11 patients). RESULTS: No significant differences were noted between Group A and the other groups in the international normalized ratio of prothrombin time, activated partial thromboplastin time, and levels of antithrombin and plasminogen. Levels of thrombin-antithrombin (TAT) complexes, fibrinogen, fibrinogen degradation product d-dimers, and plasmin-α2-plasmin inhibitor complexes (PIC) were significantly elevated in Group A. All patients that showed TAT complexes, fibrinogen, and PIC levels greater than 4.2 ng/mL, 399 mg/dL, and 1.4 µg/mL, respectively, had active or progressive systemic amyloidosis. All patients with TAT complex levels less than 3.6 ng/mL, fibrinogen levels less than 355 mg/dL, and PIC levels less than 0.9 µg/mL had localized AL amyloidosis. CONCLUSION: Analyses of TAT complexes, fibrinogen, and PIC can be used to differentiate localized AL amyloidosis from systemic amyloidosis.

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.

Hypocomplementemic urticarial vasculitis syndrome is associated with high levels of serum IgG4: a clinical manifestation that mimics IgG4-related disease.

A 58-year-old Japanese woman presented with recurrent abdominal pain, chronic urticaria, and petechiae on her extremities, and hypocomplementemia, findings that were consistent with hypocomplementemic urticarial vasculitis syndrome (HUVS). A laboratory examination revealed that she had markedly elevated IgG levels (4,448 mg/dL; normal range, 870-1,700 mg/dL) with particularly high IgG4 levels (1,050 mg/dL; normal range, 48-105 mg/dL) and a high IgG4/total IgG ratio (0.22; normal range, 0.02-0.05). A skin biopsy demonstrated leukocytoclastic vasculitis with IgG4 deposition in the vascular lumen and vascular walls. A lymph node biopsy revealed reactive lymphoid hyperplasia with numerous IgG4-positive cells in the perifollicular area, but no sclerotic findings. A chromosomal analysis of an enlarged lymph node, without phytohemagglutinin (PHA) stimulation, demonstrated that one in every three analyzed cells had abnormalities, such as 44, XX, -13, add(15)(p11), -17, -17, and mar.

Clinical correlations with dermatomyositis-specific autoantibodies in adult Japanese patients with dermatomyositis: a multicenter cross-sectional study.

OBJECTIVE: To clarify the association of clinical and prognostic features with dermatomyositis (DM)-specific autoantibodies (Abs) in adult Japanese patients with DM. DESIGN: Retrospective study. SETTING: Kanazawa University Graduate School of Medical Science Department of Dermatology and collaborating medical centers. Patients A total of 376 consecutive adult Japanese patients with DM who visited our hospital or collaborating medical centers between 2003 and 2008. MAIN OUTCOME MEASURES: Clinical and laboratory characteristics of adult Japanese patients with DM and DM-specific Abs that include Abs against Mi-2, 155/140, and CADM-140. RESULTS: In patients with DM, anti-Mi-2, anti-155/140, and anti-CADM-140 were detected in 9 (2%), 25 (7%), and 43 (11%), respectively. These DM-specific Abs were mutually exclusive and were detected in none of 34 patients with polymyositis, 326 with systemic sclerosis, and 97 with systemic lupus erythematosus. Anti-Mi-2 was associated with classical DM without interstitial lung disease or malignancy, whereas anti-155/140 was associated with malignancy. Patients with anti-CADM-140 frequently had clinically amyopathic DM and rapidly progressive interstitial lung disease. Cumulative survival rates were more favorable in patients with anti-Mi-2 compared with those with anti-155/140 or anti-CADM-140 (P < .01 for both comparisons). Nearly all deaths occurred within 1 year after diagnosis in patients with anti-CADM-140. Conclusion Dermatomyositis-specific Abs define clinically distinct subsets and are useful for predicting clinical outcomes in patients with DM.

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.

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.

Effects of combination therapy with angiotensin II type I receptor blockers and calcium channel blockers on renal function in hypertensive patients/a retrospective, "real-world" comparative study.

BACKGROUND: Combination therapies with angiotensin II type I receptor blockers (ARBs) and calcium channel blockers (CCBs) are frequently administered to hypertensive patients, because these regimens have renoprotective and antihypertensive effects. However, few studies have focused on the renoprotective effects of individual CCBs when combined with ARBs for hypertension. METHODS: Two hundred eighty-six outpatients prescribed three different CCBs (benidipine [CAS 91599-74-5], amlodipine [CAS 111470-99-6] and controlled release nifedipine (nifedipine CR) [CAS 21829-25-4]) for hypertension in combination with ARBs during a 4-year period were registered in a retrospective comparative study. The factors that influenced the appearance of renal events defined as doubling of serum creatinine were investigated. RESULTS: The renal event rate was significantly lower in the benidipine than in the amlodipine (p < 0.05) and nifedipine CR (p < 0.01) groups. Multivariate analysis revealed hazard ratios for renal events to be significantly higher with chronic kidney disease (CKD) and lower with benidipine. Moreover, among patients with CKD, the benidipine group showed a significantly lower renal event rate than the amlodipine (p < 0.05) and nifedipine groups (p < 0.05). CONCLUSION: In hypertensive patients treated with ARB and CCB, benidipine exhibits a better renoprotective effect than other drugs of this class (amlodipine and nifedipine CR).

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.

Membranous nephropathy (bubbling appearance and spike formation) without immunoglobulin deposition in a patient with systemic lupus erythematosus.

A 53-year-old Japanese man with systemic lupus erythematosus developed proteinuria and hematuria after a urinary stone episode. A light microscopic study of a kidney biopsy specimen demonstrated a bubbling appearance and spike formation of the basement membrane. Immunofluorescent studies revealed that there were no significant depositions of immunoglobulins, such as IgG (-), IgA (-), IgM (+/-), kappa light chain (+/-), lambda light chain (+/-), or C3 (-) in the glomerular capillary wall, though C1q was present as one-plus positive staining in mesangial areas. Electron microscopic studies showed that the thickness of the basement membrane varied from thin to thick without electron dense deposits, and that the cellular components of the podocyte were irregularly present in the basement membrane. Urinary protein decreased after the usage of prednisolone and mizoribine; however, proteinuria aggravated after an episode of urinary stone during the same treatment.

[An IgA nephropathy case with highly reduced urinary protein concomitant with reduced obesity].

We reported a case of a 38-year-old woman with both massive proteinuria and severe obesity. We diagnosed her as metabolic syndrome from her waist size of over 90 cm around her umbilicus, hyperlipidemia (high TG level) and hypertension. The urinary protein was more than 3.5 g/day and body mass index was 38.7 at admission. The renal biopsy specimen revealed IgA nephropathy. According to Clinical guidelines of IgA nephropathy 2nd version, Committee of IgA Nephropathy-the Special Study Group on Progressive Glomerular Disease, the Ministry of Health, Labor and Welfare of Japan-, her prognosis belonged to a rather poor group. We planed to administer steroid treatment first, however considering the adverse effects of steroid therapy, such as hyperlipidemia and diabetes mellitus, we tried to decrease her body weight as much as possible, and then treated her with both angiotensin converting enzyme inhibitor and anti-platelet drug. After her body mass index (body weight) was approximately 30.1 % (30 kg) less than that on admission, a parallel reduction of urinary protein was observed, and the final level was approximately 0.18 g/day. Decline in the body weight, diet and exercise were the chief measures that reduced the urinary protein without corticosteroid therapy.