Importance of Awareness and Careful Follow-Up of Suspected IgG4-Related Periaortitis.

IgG4-related disease may cause large vessel vasculitis, which often affects males in their 60s. Here, we report a case of suspected IgG4-related periaortitis in a 76-year-old man with lower left-side chest pain and hypertension based on computed tomography findings of thickened lesions surrounding the abdominal aorta and mesenteric arteries after ruling out acute cardiovascular diseases. His serum IgG4 levels were high, but the C-reactive protein and D-dimer levels were within normal limits. Because IgG4-related periaortitis was suspected, the patient was carefully monitored for blood pressure control, inflammatory markers, and renal function. Steroid therapy was not initiated, however, due to the difficulties performing a biopsy targeting periaortitis to obtain a definitive diagnosis and possible severe complications. During follow-up observation, IgG4-related kidney disease was suspected based on a slight increase in the serum creatinine levels and a renal biopsy was considered. Just before performing the renal biopsy, we observed left renal hydronephrosis caused by spreading retroperitoneal fibrosis. Immediate ureteral stent implantation and initiation of steroid therapy successfully improved the renal function and decreased the serum IgG4 level, respectively. Although relatively rare, IgG4-related periaortitis/periarteritis and retroperitoneal fibrosis should be considered in the differential diagnosis of aortic diseases, even after ruling out serious major acute cardiovascular diseases. Cardiologists should also be aware of the possible progression and systemic spread of this disease.

A Retrospective Analysis of Risk Factors for Mortality during Hemodialysis at a General Hospital That Treats Comprehensive Diseases.

Objective We analyzed adverse events retrospectively during a three-year follow-up of patients undergoing hemodialysis at the dialysis center of our general hospital that can treat comprehensive diseases and conducted an exploratory study focusing on the risk factors that determine the prognosis of hemodialysis patients. Methods A total of 132 hemodialysis patients at our dialysis center as of June 2017 were included in the study. Data on event incidence, including death and various clinical indicators, were collected in the electronic medical record for three years until June 2020. Results Between June 2017 and June 2020, 33 of the 132 patients died. The mortality group had a lower body mass index (BMI) and a longer duration of hemodialysis already carried out with more preexisting upper gastrointestinal (GI) bleeding, infections, ischemic heart disease (IHD), and malignancy than the survival group. Furthermore, the mortality group took more warfarin, aspirin, proton pump inhibitors and less H2 blockers than the survival group. Occurrence of upper or lower GI bleeding was similar between the mortality and survival groups. In a univariate analysis for mortality, the odds ratio was significantly higher for a low BMI (<18), long duration of hemodialysis, history of upper GI bleeding, and presence of IHD. Multivariable-adjusted odds ratios for mortality were significantly higher for cases with a history of upper GI bleeding and BMI <18. Conclusion A history of upper GI bleeding and low BMI may be poor prognostic factors of hemodialysis patients. Careful management of upper GI bleeding and a low BMI are required during the initiation of hemodialysis.

Low Body Mass Index without Malnutrition Is an Independent Risk Factor for Major Cardiovascular Events in Patients with Hemodialysis.

We retrospectively analyzed major cardiovascular events (MACE), a composite of cardiac death, nonfatal myocardial infarction, unplanned revascularization, heart failure leading to hospitalization, and stroke during a 3-year follow-up of patients with hemodialysis at the dialysis center of our general hospital that can treat comprehensive diseases. Moreover, we conducted an exploratory study that focuses on the risk factor for MACE in patients with hemodialysis.A total of 132 patients with hemodialysis at our dialysis center as of June 2017 were included in the study. Data on event incidence, including death and various clinical indicators, were collected in the electronic medical record for three years until June 2020. Between June 2017 and June 2020, of the 132 patients with hemodialysis, 31 patients experienced MACE (10 cardiovascular deaths, 3 nonfatal myocardial infarction, 11 unplanned revascularizations, 5 heart failure leading to hospitalization, and 2 stroke). The patients with MACE had a lower body mass index (BMI), longer duration of dialysis with more preexisting gastrointestinal (GI) bleeding, and took more aspirin compared to the MACE-free patients. Malnutrition markers (serum total protein, serum albumin, and serum total cholesterol) were similar in both groups. In a univariate analysis for MACE, the odds ratio was significantly higher for BMI < 18.5, duration of hemodialysis, and history of GI bleeding. Multivariable-adjusted odds ratios for MACE were significantly higher for BMI < 18.5.In conclusion, BMI < 18.5 without malnutrition may be an independent risk factor for MACE in patients with hemodialysis.

A case report of nephrotic syndrome due to collapsing focal segmental glomerulosclerosis treated with low-density lipoprotein apheresis.

We herein report the case of a 73-year-old woman with steroid and cyclosporine resistant collapsing focal segmental glomerulosclerosis (FSGS) whose refractory proteinuria and hypoproteinemia were controlled with low-density lipoprotein apheresis (LDL-A). She was initially treated with steroid therapy, including methylprednisolone pulse and cyclosporine therapy. However, her hypoproteinemia, accompanied with renal insufficiency, persisted despite these therapies. We treated her using LDL-A and found improvement in her urine protein excretion, hyperlipidemia, hypoproteinemia, and renal function as a result of this treatment. This suggests that LDL-A may therefore be an effective therapy for nephrotic syndrome due to collapsing FSGS.

Role of inducible costimulator in the development of lupus in MRL/lpr mice.

Inducible costimulator (ICOS) is a costimulatory molecule expressed in activated T cells and plays an important role in T-cell-dependent immune responses. We investigated the role of ICOS in the development of autoimmune diseases in MRL/Mpj-lpr/lpr (MRL/lpr) mice. ICOS was expressed on CD4(+) T cells from adult MRL/lpr mice. ICOS-deficient MRL/lpr mice showed mild lymphoadenopathy and a decreased memory type CD4(+) T cells in the spleen. The anti-dsDNA antibody levels were decreased. CD4(+) T cells from ICOS-deficient MRL/lpr mice showed less of a bias to Th1 and an enhanced production of IL-4 in response to anti-CD3 antibody in comparison to those from wild-type MRL/lpr mice. Although ICOS-deficiency abrogated renal vasculitis completely, the severity of glomerulonephritis was not altered. ICOS is considered to play a role in CD4(+) T cell activation, autoantibody production, and renal vasculitis. However, it is not essentially required in the development of glomerulonephritis.

Different effects of castration and estrogen administration on glomerular injury in spontaneously hyperglycemic Otsuka Long-Evans Tokushima Fatty (OLETF) rats.

AIM: Non-insulin-dependent diabetic mellitus model rats, Otsuka-Long-Evans-Tokushima-Fatty (OLETF), develop diabetic nephropathy presenting with mesangial expansion leading to glomerular sclerosis and thickening of the glomerular basement membrane (GBM), especially in elderly males. The effects of sex hormones and castration on the incidence of diabetes mellitus (DM) have been studied in this strain rat. However, there have been no detailed studies on the effects of castration and sex hormone in the development of diabetic nephropathy. METHODS: In this study we examine the effect of castration or estrogen on the development of glomerular injury in OLETF rats. Thirty male OLETF rats and 10 male long-Evans Tokushima Otsuka (LETO) rats as a normal control were used. OLETF rats were divided into three groups: group 1 received sham-operation, group 2 was castrated at 6 weeks, and group 3 was administered 0.1 mg estrogen subcutaneously once a month from 6 weeks to 58 weeks of age and LETO rats were assigned to group 4. Body weight, urinary protein and fasting blood glucose, serum albumin and other serum constituents were investigated every 12 weeks from 12 weeks to 60 weeks of age. In groups 1-3, glucose tolerance test was performed at 38 weeks. Each group was studied morphologically at the end of the experiment (60 weeks of age). RESULTS: Castration attenuated proteinuria and glomerular sclerosis accompanied by an amelioration of glucose tolerance, a decrease in mesangial expansion and an attenuation of the GBM thickening. In contrast, although estrogen equally ameliorated glucose tolerance and attenuated the mesangial expansion and the GBM thickening, estrogen failed to attenuate proteinuria and glomerulosclerosis. A significant increase in glomerular tuft volume, and serum levels of growth hormone, total cholesterol and triglycerides was observed in the estrogen-treated rats as compared with the castrated rats. CONCLUSION: Besides the mechanisms involved in the development of diabetic nephropathy, other mechanisms may be involved and contribute to the development of glomerulosclerosis in the estrogen-treated rats, leading to a difference in glomerular injury between the castrated and estrogen-treated OLETF rats.

Nephrotic syndrome due to immunologically mediated hypocomplementic glomerulonephritis in a patient of Waldenström's macroglobulinemia.

A 72-year-old man was diagnosed as having nephritic syndrome complicated by Waldenström's macroglobulinemia (WM). A monoclonal IgM lambda protein and decreased serum complements were observed. The renal biopsy disclosed the capillary occluded by thrombi which was stained with IgG, IgA, IgM, C4, lambda light chain and slight kappa light chain in a granular pattern. Electron dense deposits were noted in the subendothelial spaces. An unusual case of WM who developed nephrotic syndrome due to immunologically mediated hypocomplementic glomerulonephritis is described.

Crescentic glomerulonephritis in a patient with heterozygous Fabry's disease.

A 58-year-old woman who suffered from a heterozygous Fabry's disease and immune complex crescentic glomerulonephritis (GN) is reviewed. The diagnosis was made on the basis of the pathologic findings and peripheral leukocyte alpha-galactosidase activity. Light microscopy revealed a vacuolization of epithelial cells and electron microscopy showed myelin figures in the cytoplasm of visceral epithelial cells typical of Fabry's disease at the first renal biopsy. During the following 4 months she developed progressive renal failure and a second renal biopsy disclosed the formation of cellular crescents in 7 of 11 glomeruli observed. A rare case of combined Fabry's disease and crescentic glomerulonephritis is discussed.