Relapsing Tubulointerstitial Nephritis with Antimitochondrial M2 Antibody Accompanied by Pulmonary Involvement.

We herein report a 50-year-old woman who suffered from tubulointerstitial nephritis with antimitochondrial M2 antibody, distal renal tubular acidosis, and Fanconi syndrome. Our case also had interstitial pneumonia. After initially successful glucocorticoid therapy, tubulointerstitial nephritis and interstitial pneumonia relapsed. After the second successful round of glucocorticoid therapy, tubulointerstitial nephritis relapsed again and responded to glucocorticoid and azathioprine. This case might indicate (1) the association between pulmonary involvement and tubulointerstitial nephritis with antimitochondrial antibodies and (2) the need for a maintenance dose of glucocorticoid and immunosuppressants in tubulointerstitial nephritis with antimitochondrial antibodies.

Anti-mitochondria antibody-related tubulointerstitial nephritis accompanied by severe hypokalemic paralysis.

A 47-year-old man presented with severe hypokalemic paralysis and respiratory failure. A large amount of potassium was administered along with providing intensive care, and his condition improved. Hypokalemia was attributed to increased urinary potassium excretion. A kidney biopsy was performed to make a definitive histological diagnosis. It revealed acute tubulointerstitial nephritis (TIN). After the diagnosis, prednisolone was administered, and the TIN gradually improved. From the clinical course and laboratory findings, the TIN was presumed to be an autoimmune disorder. Further specific autoantibody tests were positive for anti-mitochondrial antibody (AMA), which has been gaining increasing attention in regard to TIN. In addition, all previous cases of TIN associated with AMA have affected females. The detailed pathogenetic mechanisms are as yet unclear and require further investigation.

Prediction of Acute Glomerular Filtration Rate Reductions Following Renin-angiotensin System Blockade in Chronic Kidney Disease: A Possible Application of Ultrasonography in Clinical Practice.

Objective Renal arteriolosclerosis is a risk factor for acute reductions in the glomerular filtration rate (GFR) when renin-angiotensin system (RAS) inhibitors are administered. Renal arteriolosclerosis can be detected by an increase in the resistive index (RI) on Doppler ultrasonography. The purpose of the present study is to determine whether or not the RI can predict acute GFR reductions following RAS blockade in chronic kidney disease (CKD). Methods We surveyed all CKD patients who were hospitalized in Otemae Hospital from January 2008 to December 2017. One hundred and eight patients who had been newly treated with RAS inhibitors were able to be followed for 14 weeks. The end point was an acute reduction in the GFR, defined as a decrease of ≥30%. Results Twenty-three of the 108 patients presented with acute GFR reductions. The cumulative probability of acute GFR reductions was 3.3% and 53% in patients with RI ≤0.70 and RI >0.70, respectively (p<0.001). A univariate Cox proportional-hazards analysis showed that the RI, age, GFR, systolic blood pressure, urinary protein excretion, diabetic kidney disease, coronary artery disease, and use of diuretics were significant variables. Multivariate hazard ratios were calculated from the RI and three established variables (age, GFR, diuretics), and the RI and use of diuretics were shown to be significant risk factors for acute GFR reductions. Conclusion These results suggest that an increase in the RI, as well as the use of diuretics, may be risk factors for acute GFR reductions following RAS blockade.

Chronological renal resistive index increases related to atherosclerotic factors, and effect of renin-angiotensin system inhibitors.

BACKGROUND: Renal resistive index (RI) calculated using renal Doppler ultrasonography (RDU) has recently been considered a clinically important indicator of renal outcome, survival, and systemic arteriosclerotic disorders. However, the cause of RI elevation remains unclear. The present study was an effort to first, identify the factors related to RI elevation, and second, understand the effect of renin-angiotensin system inhibitors (RAS-Is) on renal RI elevation. METHODS: We carried out this single-center case-control study among 100 CKD patients, recruited from outpatients who underwent RDU more than twice, at least a year apart. The rate of renal RI change per year (dRIpy) was chosen as the dependent variable: [(last examined renal RI-initial examined renal RI)/(initial examined renal RI × period of observation) × 100 (%/year)]. We examined the association between dRIpy and other clinical and biological data. RESULTS: Among 100 CKD patients, the average serum creatinine and eGFR were 1.76 ± 0.84 mg/dL and 37.0 ± 18.2 ml/min/1.73 m2, respectively. The average dRIpy in all patients was 1.8 ± 1.4%/year. The linear multiple regression demonstrated that dRIpy was positively associated with the presence of diabetes mellitus (DM) and high low-density lipoprotein cholesterol (LDL) levels, and negatively with eGFR and RAS-I use. CONCLUSIONS: This study demonstrated that the elevation of RI was related to DM, eGFR, high LDL, and the use of RAS-Is. In particular, RAS-Is could contribute towards suppressing the elevation of RI in CKD patients and towards preventing the development of renal failure in CKD patients.

Nephrotic Syndrome with Thrombocytopenia, Lymphadenopathy, Systemic Inflammation, and Splenomegaly.

Nephrotic syndrome can be caused by various diseases, from primary kidney diseases to systemic diseases. A kidney biopsy is useful for confirming the causes of nephrotic syndrome and in its management. We herein describe a case of nephrotic syndrome with thrombocytopenia, lymphadenopathy, systemic inflammation, splenomegaly, kidney enlargement, and progressive renal insufficiency. A kidney biopsy showed endothelial swelling with mild interstitial fibrosis and tubular atrophy. This case met the diagnostic criteria for TAFRO syndrome. Little is known about TAFRO syndrome, especially in relation to the associated kidney pathophysiology. The accumulation of a greater number of cases in which the kidney biopsy findings are investigated is needed to clarify the pathogenesis of kidney involvement in this condition.

A case report of proliferative glomerulonephritis with monoclonal immunoglobulin M-kappa deposits without associated lymphoproliferative disorder or detectable paraproteinemia.

A 53-year-old man presented with proteinuria and hematuria. No significant abnormality was detected in his physical examination or laboratory tests, including evidence of paraprotein in serum and urine. Renal biopsy revealed mesangial proliferation, thickened glomerular basement membranes, and spike formation. Immunofluorescence revealed deposition of immunoglobulin (Ig) M heavy chain, kappa (κ) light chain, and complement component C3 along capillary walls in the glomeruli. Light chain staining indicated significant restriction, because only κ chain, not lambda chain, was present in glomeruli. Aggregated electron dense deposits were observed in the subepithelial area and within the lamina densa on electron-microscopic examination. Cryoglobulinemia and amyloidosis were ruled out. Clinically, steroid therapy was not initiated due to patient preference, and the only prescribed medication was an angiotensin II receptor blocker. At the approximately 3-year follow-up, estimated glomerular filtration rate had decreased very mildly. The present case demonstrates that deposition of monoclonal IgM-κ may be associated with membranoproliferative glomerulonephritis-like changes in the glomeruli. Although no underlying hematological abnormality or paraproteinemia was observed in this case within the range of limited clinical examination, the patient's condition is consistent with proliferative glomerulonephritis with monoclonal IgM deposits, similar to the recently established proliferative glomerulonephritis with monoclonal IgG deposits. Further elucidation of the pathophysiology and effective treatments of the disorder should be expected in the future through the accumulation of similar cases.

Tuberculous Fasciitis in Polymyositis: A Rare Case of Extrapulmonary Tuberculosis.

A 71-year-old woman with polymyositis presenting with left thigh pain and an intermittent fever was admitted to Osaka Rosai Hospital. We initially diagnosed that her pain and fever were caused by a soft tissue infection because her polymyositis was controlled. She did not respond to various antibiotic therapies. Chest computed tomography demonstrated miliary tuberculosis (TB). Ziehl-Neelsen staining of liver biopsy specimens revealed epithelioid cell granuloma and acid-fast bacilli. Therefore, we finally diagnosed the lesion as TB fasciitis that improved with anti-TB drug therapy. The atypical presentation of TB fasciitis demonstrates the clinical importance of eliminating TB infections in immunocompromised hosts.

[Effectiveness of a practical protocol for the prevention of contrast-induced nephropathy: improved prevention of contrast-induced nephropathy].

BACKGROUND: In Japan, "Guidelines for iodinated contrast in a patient with chronic kidney disease (CKD) 2012" was published, but preventive protocols for specific contrast-induced nephropathy (CIN) have not been specified. Therefore, we developed a CIN preventive protocol, and validated its operation and renal protective effect. METHODS: In a retrospective cohort study, we determined eGFR within 3 months before contrast-enhanced computed tomography (CECT). We evaluated CKD stage 3b - 4 adult patients (eGFR 15 - 45 mL/min/1.73m2) who underwent CECT. We observed changes in renal function over 9 months and compared the changes between the pre-protocol group, which received CIN preventive measures from clinicians, and the post-protocol group, which received 500 mL 0.9% saline intravenously over 4 hours or drank 2,000 mL water over 36 hours. RESULTS: The numbers of CT and CECT patients after validation of the protocol were 5,450 and 2,037, respectively. Among the CECT patients, 310 (15.2%) and 77(3.8%)had eGFRs < 60 and 45 mL/min/1.73 m2, respectively. Among the CECT patients whose eGFRs were < 60 mL/min/1.73 m2, 74.5% were 70 years or older. Tumor scanning accounted for 77% of all CECT cases. The number of CECT patients after 3 months did not significantly differ between the groups (2,189 vs 2,037). The percentage of patients with CKD stage G3b - 4 showed no significant differences (3.3% vs 3.7%, p = 0.89). The proportion of patients whose eGFR did not deteriorate at 3, 6 and 9 months was significantly higher in the post-protocol group than in the pre-protocol group (p < 0.001), and the protocol was the only independently-significant predictor. CONCLUSIONS: Our protocol prevented CIN and provided a renal protective effect without reducing the number of CECT patients.

[A case of crescentic poststreptococcal acute glomerulonephritis (PSAGN) accompanied by membranous nephropathy].

In 2010, a 71-year-old man was referred to our hospital because of mild proteinuria and hematuria. At that time, he had been asymptomatic. Three months later he noticed macroscopic hematuria, followed by general malaise, and then anorexia. He was admitted for acute kidney injury (serum creatinine 2.7 mg/dL), marked proteinuria (4.35 g/gCr), and elevated C-reactive protein (7.21 mg/dL). Some vesicles were noted on the soft palate, and a throat culture yielded a growth of group A beta-hemolytic streptococci. Antistreptolysin O and antistreptokinase titers were elevated, but serum complement levels were within normal limits. Antineutrophil cytoplasmic antibodies (ANCA) directed against elastase and bactericidal permeability increasing protein (BPI)were positive. The renal function and inflammation did not improve despite oral antibiotic therapy. Pathological examination of a renal biopsy specimen revealed diffuse crescent formation, numerous subepithelial dome-shaped deposits (humps), and prominent endocapillary proliferation. Furthermore, a focal and segmental spike appearance was seen, with deposits smaller than humps. There was a striking clinical improvement after steroid pulse therapy followed by oral prednisolone. The features of this case strongly suggest crescentic PSAGN accompanied by pre-existing membranous nephropathy.

[A case of drug-induced granulomatous interstitial nephritis during the long course of Crohn's disease].

A 33-year-old man was diagnosed with Crohn's disease in 2001, and treated with mesalazine and ranitidine. Administration of infliximab was started in 2007 and led to a decrease in the activity of the Crohn's disease. He was referred to our department in the summer of 2011 following rapid progression of renal insufficiency, with serum creatinine levels increasing from 1.5 mg/dL to 4.3 mg/dL within 2 months. On admission, laboratory findings showed signs of inflammation, anemia, proteinuria, and hematuria. Renal biopsy results indicated the diagnosis of granulomatous interstitial nephritis. Neither clinical manifestations nor laboratory findings were suggestive of infectious disease, sarcoidosis, Wegener's granulomatosis or tubulointerstitial nephritis and uveitis. Mesalazine and ranitidine were discontinued in view of reports of drug-induced granulomatous interstitial nephritis. Levels of C-reactive protein immediately decreased, but renal function remained unimproved. Treatment with steroid pulse therapy was then initiated, followed by oral prednisolone at 40 mg/day, and his serum creatinine recovered to 2.3 mg/dL. Mesalazine and/or ranitidine appear to have been responsible for the granulomatous interstitial nephritis. In cases of Crohn's disease showing rapid deterioration of renal function, drug-induced renal disease should be considered, even if the drugs have been taken without apparent problems for a long duration.

Hyponatremia associated with demyelinating disease of the nervous system.

A 63-year-old man was diagnosed with periodontitis and underwent tooth extraction. Several days later, he suffered a high fever, ischuria, a change in personality, and disorientation. A urologist examined him and found severe hyponatremia (117 mEq/L), and he was then transferred to our hospital. On admission, physical findings revealed dysfunction of the bladder and bowel, altered mental status, and hypovolemia. Blood chemistry showed serum sodium of 120 mEq/L, a serum urate of 1.4 mg/dL, urinary Na of 61 mEq/L, and fractional urate excretion of 16 %. Examination of the cerebrospinal fluid (CSF) showed monocytosis. Magnetic resonance imaging (MRI) of the brain and spinal cord showed multiple lesions characterized by hyperintensity on T2-weighted sequences, suggesting demyelinating disease. His sodium concentration normalized 3 days after volume replacement therapy, and his altered mental status along with the dysfunction of the bladder and bowel were promptly improved after the initiation of high-dose glucocorticoids. Additionally, the abnormal lesions on MRI markedly decreased. This clinical course led to the likely diagnosis of acute disseminated encephalomyelitis (ADEM). Hyponatremia in neurologically injured patients is usually attributed to the syndrome of inappropriate antidiuretic hormone (SIADH) or cerebral salt-wasting syndrome (CSWS). In the present patient, the uric acid level remained low and uric acid excretion remained elevated despite correction of the hyponatremia, which suggested CSWS. The differentiation of CSWS from SIADH is difficult but critically important due to the fact that the disorders are managed differently. Coexistence of ADEM and CSWS has rarely been reported.

[Clinical characteristics of adult-onset minimal change nephrotic syndrome in our hospital].

OBJECTIVE: Few findings are available regarding adult-onset minimal change nephrotic syndrome (MCNS) with respect to the disease course and complications, such as acute kidney injury (AKI). We therefore performed a retrospective review to characterize the clinical presentations, steroid responsiveness and complications of adult-onset MCNS patients in our hospital. PATIENTS AND METHODS: We retrospectively reviewed 40 cases of idiopathic adult-onset MCNS who had been investigated and treated at a single center. Patients between 18 and 50 years of age (Younger group) at the time of biopsy were compared with those older than 50 years (Older group) with regard to demographic data, clinical features and treatment outcome. RESULTS: Baseline characteristics of the 40 patients were: median age, 42 years (interquartile range: 28-63 years); male, 70%; mean (+/- standard deviation) systolic and diastolic blood pressures, 125 +/- 17 mmHg and 78 +/- 12 mmHg, respectively; estimated glomerular filtration rate (eGFR), 74 mL/min/1.73 m2 (range: 64-94 mL/min/1.73 m2); serum albumin, 1.8 +/- 0.3 g/dL; and urinary protein, 7.8 g/day (range: 3.9-10.4 g/day). All except for one patient received steroid pulse therapy. Time to complete response (CR) was 12 days (range: 8-21 days). Time to CR was significantly longer in the Older group (p = 0.011). The Late-responder group (time to CR > 2 weeks)was significantly older (p < 0.01), with a low eGFR (p < 0.001) and a higher prevalence of interstitial fibrosis in renal biopsy before the initiation of corticosteroid therapy (p < 0.05), compared with the Early-responder group. AKI was observed in 14 patients. Patients with an episode of AKI were significantly older (p = 0.005), with a lower eGFR (p < 0.002) and a higher prevalence of cellular casts (p < 0.05). At the follow-up, 19 patients (51%) had experienced relapses. The relapse rate was significantly lower in the Older group than in the Younger group (p < 0.05). CONCLUSION: The present study revealed that older patients had a longer period to CR and a higher risk of AKI at follow-up.