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Background: The effects of tonsillectomy combined with steroid pulse (TSP) therapy for IgA nephropathy (IgAN) are little known. Therefore, we examined the effects of TSP therapy on the kidney outcomes of IgAN in a large, nationwide cohort study in Japan. Methods: Between 2002 and 2004, 632 IgAN patients with ≥ 0.5 g/day proteinuria at diagnosis were divided into three groups with mild (0.50–0.99 g/day; n = 264), moderate (1.00–1.99 g/day, n = 216), or severe (≥ 2.00 g/day; n = 153). Decline in kidney function and urinary remission were compared among the three groups after TSP therapy, corticosteroid (ST) therapy, or conservative therapy during a mean follow-up of 6.2 ± 3.3 years. 10.6% and 5.9% of patients in the ST and conservative therapy group underwent tonsillectomy. Results: The rate of urinary remission at the final observation was significantly higher in the TSP therapy group than in the ST or conservative therapy groups (mild proteinuria: 64%, 43%, and 41%; moderate proteinuria: 51%, 45%, and 28%; severe proteinuria: 48%, 30%, and 22%, respectively). In contrast, the rate of a 50% increase in serum creatinine was lower in groups TSP therapy, than ST or conservative therapy (mild proteinuria: 2.1%, 10.1% and 16.7%; moderate proteinuria: 4.8%, 8.8% and 27.7%; severe proteinuria: 12.0%, 28.9% and 43.1%, respectively). In multivariate analysis, TSP therapy significantly prevented a 50% increase in serum creatinine levels compared with conservative therapy in groups with moderate and severe proteinuria (hazard ratio, 0.12 and 0.22, respectively). Conclusion: TSP significantly increased the rate of proteinuria disappearance and urinary remission in IgAN patients with mild-to-moderate urinary protein levels. It may also reduce the decline in kidney function in patients with moderate-to-severe urinary protein levels. [ABSTRACT FROM AUTHOR]

Background: Tonsillectomy with steroid pulse therapy (TSP) and tonsillectomy monotherapy (T) have improved the prognosis of patients with immunoglobulin A nephropathy (IgAN). However, a consensus has not been reached on the best treatment for these patients. This study aimed to compare the efficacies of TSP and T. Methods: Data of patients with IgAN who received TSP or T were retrospectively analyzed. The exclusion criterion was a serum creatinine level > 1.5 mg/dL. The clinical remission and renal survival rates were compared. Results: Patients were divided into groups based on the treatment method: the TSP (n = 82) and T groups (n = 41). No significant differences were observed in patient characteristics, except for the observation period (TSP: 60 months, T: 113 months). The log-rank test revealed that the clinical remission rate was significantly higher in the TSP group than in the T group (p < 0.05). The superiority of TSP was also observed in the urinary protein excretion (> / = or < 1 g/day) of the two subgroups. According to the Cox proportional-hazards model, the treatment method and daily urinary protein extraction were independent factors affecting clinical remission. The 10-year renal survival rates in the TSP and T groups were 100% and 92.5%, respectively. The log-rank test revealed a tendency for a higher renal survival rate in the TSP group than in the T group (p = 0.09). Conclusion: The clinical remission rate was significantly higher with TSP than with T, regardless of urinary protein levels. TSP tended to have a better renal survival rate than T. [ABSTRACT FROM AUTHOR]

Key Clinical Message: A T217M heterozygous mutation in the SLC22A12 gene caused renal hypouricemia; this patient with IgA nephropathy had no findings other than IgA nephropathy on renal biopsy. Hypouricemia was susceptible to oxidative stress, but IgA nephropathy in the patient with hypouricemia could be treated with steroid pulse therapy without adverse events. [ABSTRACT FROM AUTHOR]

Background: Although intravenous methylprednisolone pulse (IVMP) therapy has been recommended for ocular myasthenia gravis (OMG), the evidence is limited. Aim: We aimed to investigate the efficacy and safety of IVMP for OMG. Methods: The patients with OMG were chosen retrospectively from May 2010 to December 2022. The therapeutic effects between IVMP‐treated and non‐IVMP‐treated groups were determined by ∆ocular quantitative MG (QMG) score and the ∆ocular MG activities of daily living profile (MG‐ADL) score, which is the disparities of the scores between before and after 1 month of drug administration; and Myasthenia Gravis Foundation of America post‐intervention status at 1, 3, 6, and 12 months. Results: There were 26 patients with OMG included, with the mean age of 67.2 ± 13.1 years. 13 of the 26 people with OMG received IVMP and subsequent low‐dose immunotherapies. The ∆ocular QMG and ∆ocular MG‐ADL scores were significantly higher in the IVMP group of 3 (1.5, 4) and 3 (2, 4.5) than in the non‐IVMP group of 1.5 (0, 3) and 1 (0, 2), p = 0.038 and p = 0.0027, respectively. The rates of minimal manifestation or better status in post‐intervention status at 1 and 12 months were also considerably higher in the IVMP group of 77% and 92% compared with the non‐IVMP group of 0% and 23%, p = 0.0001 and 0.001. There were no serious side effects discovered. Conclusion: IVMP induction therapy can quickly and safely improve symptoms of OMG, and subsequent low‐dose immunotherapy can keep symptoms at bay for 12 months. [ABSTRACT FROM AUTHOR]

Acquired idiopathic generalized anhidrosis is a rare disease characterized by systemic anhidrosis or hypohidrosis without other systemic diseases. However, its etiology remains unclear. Autoimmune mechanisms seem to be involved in the development of acquired idiopathic generalized anhidrosis. Although steroid pulse therapy is the most commonly used therapy, it lacks a high level of evidence. On the other hand, pilocarpine, a muscarinic receptor agonist that stimulates exocrine glands, increases saliva and tear secretion as well as sweating. Here, we report treatment progresses of steroid pulse therapy and oral pilocarpine in our department. Between 2012 and 2021, we treated 10 patients of acquired idiopathic generalized anhidrosis. All patients were administered oral pilocarpine as the first therapy, three (30%) of whom had increased sweating. Minor side effects were observed, however, no serious side effects were observed. Five patients who did not respond to oral pilocarpine were subsequently treated with steroid pulse therapy to which four (80%) showed significant response. Two patients were subsequently administered oral pilocarpine as post-therapy, and remission was maintained for up to 81 months. Oral pilocarpine could be used to relieve symptoms in patients with acquired idiopathic generalized anhidrosis prior to steroid pulse therapy and as maintenance therapy after steroid pulse therapy. [ABSTRACT FROM AUTHOR]

Tonsillectomy with steroid pulse therapy (SPT) has been established as an effective treatment for immunoglobulin A nephropathy (IgAN) in Japan. However, the underlying mechanisms supporting tonsillectomy remain unclear. This study assessed palatine tonsils from 77 patients with IgAN, including 14 and 63 who received SPT before and after tonsillectomy, respectively. Tonsils from 21 patients with chronic tonsillitis were analyzed as controls. Specific tonsillar lesions were confirmed in patients with IgAN, correlating with active or chronic renal glomerular lesions and SPT. T-nodule and involution of lymphoepithelial symbiosis scores in tonsils correlated with the incidence of active crescents and segmental sclerosis in the glomeruli, respectively. The study revealed an essential role of the tonsil–glomerular axis in early active and late chronic phases. Moreover, the SPT-preceding group demonstrated no changes in the T-nodule score, which correlated with active crescent formation, but exhibited a considerable shrinkage of lymphatic follicles that produced aberrant IgA1. The study underscores the involvement of innate and cellular immunity in IgAN and advocates for tonsillectomy as a necessary treatment alongside SPT for IgAN, based on a stepwise process. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Systemic prednisolone including steroid pulse therapy would be safe in 32 pregnant women, who developed Vogt‐Koyanagi‐Harada disease in the literature. Prednisolone administration would be shortened by monitoring of serous retinal detachment with optical coherence tomography. A 30‐year‐old woman in 31 weeks of pregnancy with metamorphopsia and headache was diagnosed Vogt‐Koyanagi‐Harada disease. She underwent steroid pulse therapy and oral prednisolone 20 mg daily for 3 weeks until complete resolution of serous retinal detachment monitored by optical coherence tomography. Oral prednisolone was tapered and discontinued until uneventful delivery. [ABSTRACT FROM AUTHOR]

Patients with acquired idiopathic generalized anhidrosis (AIGA) demonstrate a sudden loss of sweating function without neurological or endocrine abnormalities. The main treatment is steroid pulse therapy. However, the number of courses required for improvement has been unclear. This study aims to clarify the factors associated with AIGA disease severity and with AIGA patients' responses to steroid pulse therapy. We retrospectively analysed the clinical information of 28 patients with AIGA in our department from the last 10 years. Univariate analysis revealed that patients with a large anhidrotic area need multiple courses of steroid pulse therapy. [ABSTRACT FROM AUTHOR]

Background: COVID-19 disease can manifest as a wide spectrum ranging from asymptomatic patients to severe COVID pneumonia with the latter leading to morbidity and even death. The human physiological response to COVID infection mainly presents as a hyperacute inflammatory reaction which leads to multi organ damage especially of the lungs. Corticosteroids reduce morbidity and mortality in COVID pneumonia by suppressing the inflammatory reaction and minimizing organ damage. High dose corticosteroids delivered early in the form of pulse therapy have shown an enhanced benefit over routine therapeutic doses. Aim: This study aimed to compare the clinical outcomes in patients with COVID-19 pneumonia with standard therapeutic doses of methylprednisolone (MPPT 1000 mg/day for three days) admitted to an intensive care unit(ICU). Methods: A profile318 patients were selected retrospectively for standard corticosteroid dose regimen on the basis of severity of the disease and lab parameters. The patients were divided into two cohorts with one receiving MPPT, Group A (N=192)and the other standard corticosteroid dose Group B (N=126). Data was compiled from the hospital records and compiled in Excel. MPPT was given as 1000mg of the drug daily intravenously for 3 days. In almost all these patients, the drug was given early in the morning in the form of an infusion of 250 ml of Normal Saline over a period of 2 hours.Patient records and electronic data were assessed in the hospital and values related to clinical outcome was compiled. Results: It was found that there was a significant reduction in mortality as compared to the other group p<0.01. Patients receiving pulse therapy of steroids had markedly higher improvement in clinical parameters as well as a quicker reduction in levels of inflammatory cytokines which translated into much lesser morbidity later on with significant differences among the study groups p<0.01. Conclusion: Hence this study reaffirms the notion of high dose pulse therapy being beneficial in Severe Covid-19 disease. [ABSTRACT FROM AUTHOR]

Severe fever with thrombocytopenia syndrome (SFTS) is an emerging tick-borne infectious disease caused by the SFTS virus. Effective treatment for SFTS has not been established, but steroid pulse therapy is often used. This study aimed to verify whether steroid pulse therapy for SFTS improves prognosis or not. Data of SFTS patients were obtained from the Japanese Diagnosis Procedure Combination Database from April 2013 to March 2021. Patients treated with steroid pulse therapy were compared with untreated patients, using overlap weighting and traditional multivariable regression analysis to evaluate the impact of steroid pulse therapy on prognosis. 412 SFTS patients were included in this study, and 66 (16%) underwent steroid pulse therapy within 3 days of admission and were allocated to the steroid pulse therapy group. After overlap weighting, patients in the steroid pulse therapy group had a significantly higher in-hospital mortality rate than patients in the control group (31.1% vs. 20.5%; difference: +10.6%; 95% confidence interval: +2.2% to +19.0%). There were no statistically significant differences in hospitalization cost and length of hospital stay between the two groups. The results of the sensitivity analysis using traditional multivariable regression were similar to those of the main analysis. In the analysis of SFTS patients using the Japanese Diagnosis Procedure Combination inpatient data, steroid pulse therapy did not improve patient prognosis. The evidence does not support the universal use of steroid pulse therapy in patients with severe SFTS. [ABSTRACT FROM AUTHOR]

We present here the case of a 48‐year‐old man who first developed retinopathy, and motor deficit and aphasia occurred 8 years later. A tumor‐like lesion was seen by brain MRI. He was diagnosed with retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations (RVCL‐S) because a three prime repair exonuclease 1 (TREX1) gene abnormality was identified. After intravenous methylprednisolone pulse (IVMP) and intravenous immunoglobulin (IVIg) therapy, cerebrospinal fluid (CSF) test results and clinical manifestations improved. However, a new lesion appeared 3 years later. Immunotherapy became ineffective, and repeated relapses occurred. We evaluated the results of laboratory tests and brain MRI for 6 years after the first treatment. Coagulation tests, including measurements of vWF activity, remained high, so immunotherapy was thought to have a limited effect. [ABSTRACT FROM AUTHOR]

Context: Few reports focused on the sex differences in alopecia areata (AA) treated with steroid pulse therapy. Aims: This study aimed to investigate the association between the clinical outcomes and distinction of gender in AA patients treated with steroid pulse therapy. Settings and Design: This study retrospectively investigated 32 cases (15 males and 17 females) treated with steroid pulse therapy at the Department of Dermatology at the Shiga University of Medical Science between September 2010 and March 2017. Methods and Material: The administration of corticosteroid was 500 mg methylprednisolone intravenous infusion for 3 consecutive days. Patients were followed up approximately once a month until March 2017. Statistical Analysis Used: The respective data were analyzed by examining and comparing males and females. Statistical analysis was performed using t-test and χ2 test. Results: There were no significant differences in periods from the onset of AA to the administration of steroid pulse therapy (P = 0.2), the degree of severity (P = 0.37) and improved rate (P = 0.0772) between males and females. In contrast, the remission rate was 20% (3 of 15 males) and 71% (12 of 17 females), which was statistically significant (P = 0.0042). Previous reports have also shown a significant difference in the remission rate between males and females (males: 32 of 114; females: 51 of 117; P = 0.014). Conclusions: Despite the limitations of a small sample size including the previous reports (n = 261), female patients with AA would have better outcomes than male patients after steroid pulse therapy. [ABSTRACT FROM AUTHOR]

This prospective, observational study examined changes in dry eye status after steroid pulse and orbital radiation therapies in 16 patients (32 eyes) with active thyroid eye disease (TED). TED status was evaluated through clinical activity score (CAS), margin reflex distance (MRD)-1 and 2, presence or absence of Graefe's sign/lid lag, and Hertel exophthalmometric value. Dry eye status was quantified through presence or absence of superior limbic keratoconjunctivitis, corneal fluorescein staining (AD score), tear break-up time, Schirmer test I results, tear meniscus height, and dry eye-related quality of life score. Meibomian gland dysfunction (MGD) was evaluated through Marx line score, eyelid abnormalities (MGD score), meibum expression score, and meibomian gland loss score. Those items were measured before and 6 months after treatment, and the results were statistically compared. Consequently, CAS significantly improved, and MRD-1 significantly decreased after treatment (p < 0.050). Although a part of MGD status improved (p < 0.050), all items regarding dry eye status did not change significantly after treatment (p > 0.050). Steroid pulse and orbital radiation therapies did not largely alter most items regarding dry eye and MGD status. [ABSTRACT FROM AUTHOR]

Alopecia areata (AA) is a common, nonscarring type of alopecia that can cause chronic and severe hair loss in some cases. Although there is no standard established protocol for the use of corticosteroid pulse therapy, it has been reported to be an effective and well-tolerated treatment option for patients with severe AA. This study retrospectively analyzed 29 patients who were diagnosed with severe AA (>50% hair loss) from January 2010 to December 2020 and treated with 3 monthly sessions of intravenous corticosteroids. Methylprednisolone (500 mg/day) was administered for 3 consecutive days during the first session, followed by a 1-day treatment regimen for the second and third sessions. The treatment had an overall satisfactory response rate of 58.6% and a complete response rate of 13.8%. Eleven of the 13 patients who achieved a satisfactory response experienced relapse. A subgroup analysis showed that patients with a shorter duration of AA (≤6 months) had a significantly better response to corticosteroid pulse therapy. This study highlights that 3 monthly sessions of corticosteroid therapy are sufficient to induce satisfactory but temporary response. [ABSTRACT FROM AUTHOR]

Myocarditis is an adverse event associated with coronavirus disease 2019 (COVID‐19) mRNA vaccination. A 50‐year‐old man presented with dyspnea and resting chest pain after receiving the second dose of the COVID‐19 mRNA vaccine and developed cardiogenic shock. Fulminant myocarditis was diagnosed by endomyocardial biopsy and treated with intravenous corticosteroids. [ABSTRACT FROM AUTHOR]

Background: Henoch–Sch€onlein purpura nephritis often resolves spontaneously, without treatment, making decisions regarding therapeutic interventions difficult. Methods: Fifty-four patients who were diagnosed as having Henoch–Sch€onlein purpura nephritis between April 2004 and March 2018, and developed hematuria and/or proteinuria, were studied retrospectively. The observation period ended at the disappearance of hematuria or proteinuria, or the last observation date before December 2019 for each patient. Twenty-four of the patients received no treatment (Group A), 19 underwent renin-angiotensinaldosterone system inhibitors only (B), 4 experienced steroid pulse therapy and combination therapy only (C) and the remaining 7 received steroid pulse therapy and combination therapy following renin-angiotensin-aldosterone system inhibitors (C). Clinical characteristics were examined according to the treatment method. Survival analysis for persistent urinary abnormalities was performed according to treatment modality, with multiple treatment records created per subject, if necessary. Results: The highest urine protein/creatinine levels were significantly higher in groups B and C than in group A. The lowest estimated glomerular filtration rate was not significantly different among the three groups. In groups A and B, proteinuria resolved in >90% of patients. Survival analysis showed that steroid pulse therapy and combination therapy was not related to the better resolution of hematuria or proteinuria than renin-angiotensin-aldosterone system inhibitors. Conclusions: Several patients with Henoch–Sch€onlein purpura nephritis went into remission either without treatment or with renin-angiotensin-aldosterone system inhibitors alone. The treatment plan for patients with Henoch– Sch€onlein purpura nephritis needs to be determined carefully. [ABSTRACT FROM AUTHOR]

Background: Critical coronavirus disease 2019 (COVID-19) has a high fatality rate, especially in hemodialysis (HD) patients, with this poor prognosis being caused by systemic hyperinflammation; cytokine storms. Steroid pulse therapy or tocilizumab (TCZ) have insufficient inhibitory effects against cytokine storms in critical cases. This study evaluated the clinical effects and safety of combining steroid pulse therapy and TCZ. Methods: From September 2020 to May 2021, 201 patients with COVID-19 were admitted to our hospital. Before February 2021, patients with an oxygen demand exceeding 8 L/min were intubated and treated with standard therapy (dexamethasone and antiviral therapy). After February 2021, patients underwent high-flow nasal cannula oxygen therapy and were treated with TCZ (8 mg/kg) and methylprednisolone (mPSL) (500 mg/day [≤ 75 kg], 1000 mg/day [> 75 kg]) for 3 days. We compared background characteristics, laboratory findings, and prognosis between non-HD and HD patients and between patients who received and did not receive TCZ and mPSL pulse therapy. Results: Among non-HD patients, the TCZ + mPSL pulse group had significantly higher survival rates and lower secondary infection rates (p < 0.05), than the standard therapy group. All HD patients in the standard therapy group with oxygen demand exceeding 8 L/min died. Contrastingly, all patients in the TCZ + mPSL pulse group survived, with their oxygen demand decreasing to 0–1 L/min within 3 weeks post-administration. Conclusion: TCZ combined with mPSL pulse therapy improved the survival rate without significant adverse events in critical HD and non-HD patients with COVID-19 by strongly suppressing systemic hyperinflammation. [ABSTRACT FROM AUTHOR]

Perioperative COVID‐19 infections in patients suffering from end‐stage renal disease (ESRD) are more likely to become severe, with a high mortality rate, than those in other patients. For such patients, corticosteroid therapy is one of the limited number of treatment options. We experienced a case of ESRD in which COVID‐19 infection immediately followed arteriovenous graft surgery. Although the respiratory condition deteriorated following dexamethasone administration, requiring invasive mechanical ventilation, intravenous methylprednisolone pulse therapy (pulse therapy) improved it dramatically, suggesting that pulse therapy may be effective against severe COVID‐19 infection in patients suffering from ESRD. [ABSTRACT FROM AUTHOR]

Pemphigus vulgaris which is an autoimmune dermatological disorder characterized by vesiculobullous lesions over the skin and mucosae may also give rise to cardiac disease. Global Longitudinal Strain (GLS) measurement may help to predict cardiac dysfunction in these patients. In this prospective study, the GLS was measured before and after steroid pulse therapy in pemphigus vulgaris patients using 2 D speckle-tracking echocardiography. Moreover, blood pressure, pulse rate, and corrected QT (cQT) interval were recorded before and after steroid pulse therapy. We included 23 pemphigus patients. The mean age of patients was 34.5(±6.4). Mean GLS decreased significantly after the administration of steroid pulse therapy. The mean GLS decreased regardless of age, however, the patients with the age range of 40–50 years showed the highest change in GLS. There was no interaction between gender and GLS change. Mean cQT increased significantly after pulse therapy (p=.007). Heart rate increased significantly from 88.5 ± 10.8 to 97.0 ± 13.4 (p<.001). Mean systolic and diastolic blood pressure did not show any significant change. The steroid pulse therapy may associate with the progression of cardiac dysfunction in pemphigus patients. GLS, as a predictor, may be a valuable marker of cardiac subclinical dysfunction in this condition. [ABSTRACT FROM AUTHOR]

Generalized disabling pansclerotic morphea is a rare and mutilating form of morphea involving the dermis, subcutaneous tissue, fat, muscle, and even bone. It most often occurs in young children but adults can also be affected. We report a case of rapidly progressing and disabling adult onset generalized pansclerotic morphea, which responded well to methotrexate and steroid pulse therapy. [ABSTRACT FROM AUTHOR]

The use of plasma filtration with dialysis (PDF) may be considered when treating the acute phase of capillary leak syndrome (CLS). To the best of our knowledge, this is the first report using PDF for CLS. [ABSTRACT FROM AUTHOR]

Background: Steroid pulse therapy with tonsillectomy is known as a major treatment for IgA nephropathy (IgAN). However, its protocol was different among institutions and the effects of varying the number of steroid pulses remain unclear. Methods: From a total of 1,174 IgAN patients in a multicenter retrospective cohort analysis in Japan, 195 patients were treated by tonsillectomy combined with corticosteroid. They were divided into four groups based on the number of administered steroid pulses from 0 to three (TSP0-3), and remission of urinary abnormalities and renal survival until 1.5-fold increase in serum creatinine level from baseline were analyzed among the four groups and between TSP1 and TSP3. Results: Among the four groups, renal function was relatively good when the estimated glomerular filtration rate was approximately 80–90 mL/min/1.73m2 and proteinuria was relatively mild (< 1.0 g/gCre). The ratio of patients who developed renal dysfunction was < 5% in all groups, and the cumulative renal survival rate by Kaplan–Meier analysis was similar among groups (log-rank test, p = 0.37), despite varying clinical backgrounds and treatments. After adjustment of the background variables between TSP1 and TSP3, the remission rates of urinary abnormalities were similar and the renal survival rate also remained similar (66.8 vs. 85.4%, p = 0.45). Conclusions: In patients with mild proteinuria and good renal function, the number of steroid pulses did not affect the renal outcome in steroid pulse therapy with tonsillectomy. The adaptation and protocols, such as the number of steroid pulses, should be determined for each IgAN patient's background. [ABSTRACT FROM AUTHOR]

Background: Preventing progression to end-stage renal disease (ESRD) in advanced IgA nephropathy (IgAN) patients with impaired renal function remains challenging. We analyzed the efficacy of tonsillectomy combined with steroid pulse therapy (TSP). Methods: In this retrospective analysis, IgAN patients with proteinuria > 0.5 g/day and estimated glomerular filtration rate (eGFR) < 60 mL/min/1.73 m2 were divided into three groups: patients treated with TSP (TSP group; n = 23), oral prednisolone (oPSL group; n = 41), and conservative therapy (CONS group, n = 51). We analyzed the clinical and histological backgrounds, remission of urinary findings, and renal survival rate to a 25% decline in eGFR from baseline, and incidence of ESRD. Results: There were significant differences in the patients' backgrounds among the groups. Therefore, we adjusted the background using propensity score marching between TSP group and oPSL or CONS group. The 5-year remission rate of hematuria was significantly higher in the TSP group than in the oPSL group, and that of both hematuria and proteinuria was significantly higher in the TSP group than in the CONS group. The 10-year renal survival rate was significantly higher in the TSP group than in the oPSL and CONS groups. In a multivariate Cox regression analysis, TSP was found to be an independent factor for the 25% decline in eGFR in entire cohort. The adverse effect frequency in the TSP group was similar to the CONS group. Conclusions: TSP can effectively induce remission of urinary abnormality and improve the prognosis without frequent adverse effects in IgAN patients with impaired renal function. [ABSTRACT FROM AUTHOR]

Purpose: To report a case of large sarcoid choroidal granuloma that was successfully treated with steroid pulse therapy. Case Report: A 38-year-old man presented with the primary complaint of decreased visual acuity (VA) in his left eye. Upon examination, a large white protruding lesion of 10 × 8 papilla diameter in size was observed in the macular region, and slightly temporal to it, in the patient's left eye. Whole-body contrast-enhanced computed tomography performed for differential diagnosis detected numerous enlarged lymph nodes throughout the body, including the bilateral hilar regions. Sarcoidosis was diagnosed by biopsy of the right cervical lymph nodes showing noncaseating epithelioid cell granuloma. The fundus lesion was found to be a choroidal granuloma caused by sarcoidosis, and steroid pulse therapy was started. The granuloma was considerably decreased, and the VA in the left eye improved to 0.7 after 2 months. Conclusion: Steroid pulse therapy was found to be effective as an initial treatment for a large sarcoid choroidal granuloma. [ABSTRACT FROM AUTHOR]

Alexander disease (AxD) is a rare fatal leukodystrophy caused by a dominant missense mutation in the glial fibrillary acidic protein. In a mouse model of AxD, the pathological astrocyte causes a pronounced immune response. The inflammatory environment in the brain might play an important role in the neuronal dysfunction of AxD. A 3-month-old girl diagnosed with infantile AxD presented with severe intractable seizures and a deteriorated neurological state. Steroid pulse therapy was effective at preventing the epileptic activity and progressive white matter abnormalities on magnetic resonance images, but the effect was temporary. Levels of interleukin (IL)-6, IL-8, and macrophage chemotactic protein 1 (MCP-1) in the cerebrospinal fluid were high at onset and reduced transiently after steroid pulse therapy. These results suggest that inflammatory responses of astrocyte and microglia can contribute to the neuropathology of AxD. Robust immunomodulation that targets activated astrocytes and microglia may be a novel therapeutic strategy to improve neurological prognosis in AxD. [ABSTRACT FROM AUTHOR]

As a result, 14 patients underwent TSP, 2 patients CT (1 patient SP), 1 patient tonsillectomy, whereas 2 patients wanted neither tonsillectomy nor CT. Keywords: IgA nephropathy; Tonsillectomy; Steroid pulse therapy; Elderly EN IgA nephropathy Tonsillectomy Steroid pulse therapy Elderly 804 806 3 05/28/21 20210701 NES 210701 To the Editor Immunoglobulin A nephropathy (IgAN) is a major cause of primary glomerulonephritis worldwide. The beneficial effects of corticosteroid therapy (CT) for IgAN were first described by Kobayashi et al. [[1]], and Pozzi et al. reported steroid pulse therapy (SP) as an effective treatment regimen [[2]]. [Extracted from the article]

Purpose: High-dose postoperative steroid therapy after Kasai portoenterostomy is reported to improve jaundice clearance and a strong anti-inflammatory activity might prevent fibrous tissue formation which is often observed at the porta hepatis in revision surgery. We started steroid pulse therapy for the patients with cessation of decrease in jaundice and aimed to evaluate the efficacy in this study.Methods: The demographics and outcomes of patients who underwent laparoscopic Kasai portoenterostomy and received steroid pulse therapy within 2 months postoperatively between September 2014 and December 2018 were retrospectively reviewed; the therapy was determined successful when the serum total bilirubin level decreased to or below two-thirds of the pre-therapy level after 2 weeks. Patient data in the successful group were compared with those in the unsuccessful group.Results: Steroid pulse therapy was successful in seven of 16 patients (43.8%). The percentage of patients whose serum total bilirubin level decreased to normal was significantly higher in the successful group at 3 months (85.7% vs. 11.1%, P = 0.0028) and after all (100% vs. 33.3%, P = 0.011).Conclusions: Steroid pulse therapy was effective for some patients. Unsuccessful cases may have little chances of jaundice clearance; revision Kasai portoenterostomy would be a good option. [ABSTRACT FROM AUTHOR]

Lymphocytic hypophysitis (LYH) is a rare autoimmune inflammatory disease of the pituitary gland. In this study, we aim to characterize LYH at presentation and focus on the management and prognosis of LYH. A retrospective study of patients with LYH was conducted between 2011 and 2018 at a single institute. The patients were included by pathologic conformation and strict exclusion criteria. Clinical profile, imaging, and management data were collected. Twenty patients with LYH (16 women and 4 men) were included. Ten patients were diagnosed histologically and the remaining 10 patients were confirmed clinically of exclusion criteria. The median age at diagnosis was 37 years (range, 16–58 years). Presenting symptoms were followed by polyuria/polydipsia (11, 55%), vision changes (10, 50%), headache (8, 40%), menstrual irregularities and amenorrhea (4, 20%), diplopia (1, 5%), or sexual dysfunction (1, 5%). Eight patients had partial anterior pituitary hormone dysfunction. The thyroid-stimulating hormone axis was most involved. Ten patients received transsphenoidal surgery, 5 patients experienced steroid pulse therapy, and observation was performed on 5 patients. Only 5 patients (25%) showed improvement of anterior pituitary dysfunction after initial management. Recovery of diabetes insipidus occurred in 2 patients (18%). The overall recurrence rate was 22.2%. Nonoperative treatment is a better option for most patients with LYH because it is effective and noninvasive. Surgery is recommended for definitive diagnosis, severe or rapid progression of neurologic impairment, and glucocorticoid insensitivity. Periodic follow-up is mandatory in a patient's long-term management. [ABSTRACT FROM AUTHOR]

We present 2 cases of severe cutaneous adverse reactions (SCARs) during the tapering of corticosteroids, following several courses of high-dose pulse therapy for Vogt-Koyanagi-Harada disease. Their general symptoms and mucous membrane lesions, including those of the eye, were milder than those usually seen in Stevens-Johnson syndrome/toxic epidermal necrolysis. Based on their initial presentation, these cases were not initially identified as SCARs, but continued to progress over the course of a few days. The mechanism underlying the paradoxical response to steroid administration seen in these patients can be interpreted as immune reconstitution inflammatory syndrome in human immunodeficiency virus-negative patients. [ABSTRACT FROM AUTHOR]

Background: Invasive aspergillosis is often fatal. Here, we report a patient with invasive aspergillosis primarily involving the optic nerve diagnosed on autopsy. Case Presentation: A 77-year-old female with underlying diabetes mellitus, hyperlipidemia, and hypertension presented with disc swelling of the left eye. Although mini-pulse steroid therapy improved visual acuity (VA) of the left eye, it abruptly decreased to no light perception within a month, followed by a decrease in VA of the right eye to 0.5. At referral, VA was 0.3 in the right eye, and there was no light perception in the left eye. Results: Fundus examination revealed optic disc swelling of both eyes. Goldmann perimetry showed irregular visual field defects, whereas magnetic resonance imaging (MRI), general, and cerebrospinal fluid (CSF) examinations revealed no distinct abnormalities. We suspected anterior ischemic optic neuropathy and invasive optic neuropathy. As with the left eye, steroid pulse therapy temporarily improved VA of the right eye and then decreased to 0.2. Additional anticoagulant therapy did not improve VA. Concurrent to therapy, the patient became febrile with depressed consciousness. Repeat MRI identified suspected midbrain infarction, and CSF examination indicated cerebral meningitis. In spite of administering transfusions and antibiotics, she died on hospital day 40. Autopsy revealed large amounts of Aspergillus hyphae mainly localized in the dura mater of the optic nerve and destruction of the cerebral artery wall, suggesting an etiology of subarachnoid hemorrhage. Conclusions: When examining refractory and persistent disc swelling, we should rule out fungal infections of the optic nerve. [ABSTRACT FROM AUTHOR]

Hirayama disease (HD), or monomelic amyotrophy, is a benign neurologic disorder mostly affecting young Asian men. It usually presents with unilateral or bilateral muscular atrophy and weakening of the upper limbs. We treated a patient with HD with bilateral hand paresthesia and weakness in 1 hand and both legs. To our knowledge, this is the first HD case including lower extremity weakness and sensory abnormalities. We improved the patient's symptoms by administering steroids in parallel with conservative treatment. A 22-year-old man visited our clinic with bilateral hand paresthesia and weakness in the right hand and both legs. He had been affected 40 days. Results of blood and cerebrospinal fluid tests were normal. Evoked potential study and brain magnetic resonance imaging (MRI) were normal. In cervical MRI, however, a lesion with high signal intensity of the C6 level on T2-weighted images was confirmed, and gray and white matter were extensively invaded. We performed empirical steroid pulse therapy before the results of blood tests to differentiate spinal demyelinating disease. MRI with neck flexion showed HD-related findings, and autoantibody tests showed no specific findings. After steroid pulse therapy, his neurologic symptoms improved within 7 days, leaving only paresthesia of toes of both feet when discharged. HD occurs at a young age and therefore can damage quality of life. Although the patient had unusual symptoms, the condition was diagnosed quickly, and his symptoms improved with steroid therapy. If HD is suspected, additional tests such as MRI with neck flexion should be performed and early steroid treatment might be considered. [ABSTRACT FROM AUTHOR]

• There was not a significant difference in cumulative incidence rate of thrombotic events between patients with and without steroid pulse therapy. • Stratified analysis also showed no significant difference in thrombotic events between patients with and without Steroid pulse therapy. • Steroid pulse therapy was not associated with an increased risk of thrombotic events in lupus nephritis. [ABSTRACT FROM AUTHOR]

Objective: Tonsillectomy combined with steroid-pulse therapy is a widely accepted method for the treatment of IgA nephropathy (IgAN) in Japan. However, the indication of tonsillectomy for IgAN is still controversial, and the timing of tonsillectomy is not clearly defined for the protocol of this therapy. Based on the results of a randomized control trial in Japan, the Evidence-Based Clinical Practice Guidelines for IgA nephropathy 2014 (edited in Japan) recommended tonsillectomy combined with steroid-pulse therapy for Grade C1. However, this is not widely accepted worldwide. To clarify the validity and timing of tonsillectomy, we evaluated how the three-consecutive steroid-pulse therapy method affects the tonsil tissues of IgAN patients.Methods: We examined tonsil specimens from 35 IgAN patients and 8 chronic tonsillitis patients. We compared the proportion of follicular area to total tonsillar area and the number of germinal centers between each group on hematoxylin and eosin stained pathological specimens to clarify the histopathological characteristics of tonsils from IgAN patients. Based on these findings, we examined the tonsils of patients after three-consecutive steroid-pulse therapy treatments (n=34) to determine the influence of this therapy on the tonsil tissues of IgAN patients. Moreover, we observed chronological changes in tonsil tissues after steroid-pulse therapy.Results: The extrafollicular area was enlarged in IgAN patients before steroid-pulse therapy compared with chronic tonsillitis patients. Just after steroid-pulse therapy, the follicles became very small with blurry outlines, and the number of germinal centers was remarkably decreased. With a gradual decrease in oral prednisolone, the tonsil tissue structure was gradually restored.Conclusion: Tonsillectomy combined with steroid-pulse therapy is considered a reasonable treatment for IgAN. Steroid-pulse therapy-induced histological changes in tonsils were transient, indicating tonsillectomy should be performed before or just after steroid-pulse therapy. [ABSTRACT FROM AUTHOR]

A 61-year-old man with hyperthyroidism had exophthalmos with dilated conjunctival vessels in both eyes. Magnetic resonance imaging showed that the superior ophthalmic veins had a funicular-like appearance. Cerebral angiography showed no blood flow from both internal carotid arteries to the cavernous sinus, thus excluding a carotid-cavernous fistula. Blood tests showed an elevation of IgG4 (281 mg/dl), and a IgG4-related ophthalmic disease was considered. Steroid pulse therapy was performed, and all of the abnormal findings were improved. We concluded that this was a rare case of IgG4-related ophthalmic disease with perivascular lesions of the superior ophthalmic vein associated with optic nerve disturbance. [ABSTRACT FROM AUTHOR]

ABSTRACT: Aim: Recent studies have suggested that patients with post‐transplant immunoglobulin A nephropathy have poor graft survival. There is limited research on the therapeutic effectiveness for post‐transplant immunoglobulin A nephropathy, especially steroid pulse therapy. The present study evaluated the efficacy of steroid pulse therapy on post‐transplant immunoglobulin A nephropathy. Methods: We retrospectively analyzed patients diagnosed with de novo or recurrent immunoglobulin A nephropathy at Kyushu University Hospital between January 2013 and August 2015. Patients with moderate proteinuria (≥0.5 g/g creatinine) and/or cellular or fibrocellular crescents on a graft biopsy were treated with steroid pulse therapy. Steroid pulse therapy was 500 mg/day for 3 days in weeks 1 and 2, followed by 20 mg of oral prednisolone that was tapered after 6 months. Patients were followed for 2 years, and the estimated glomerular filtration rate, urinary findings, and adverse events were recorded. Results: Seven patients received steroid pulse therapy. The mean duration after kidney transplantation was 6.6 ± 4.7 years. After 2 years of treatment, 85.7% of patients reached complete remission of proteinuria, urinary protein excretion declined (0.82 ± 0.51 to 0.26 ± 0.22 g/g creatinine, P = 0.007), and the estimated glomerular filtration rate was maintained (48.7 ± 12.8 to 47.4 ± 14.0 mL/min per 1.73 m2, P = 0.98). Adverse events were observed in one patient who developed herpes zoster infection. Conclusion: Steroid pulse therapy for post‐transplant immunoglobulin A nephropathy effectively reduces proteinuria over 2 years. However, comparison of steroid pulse therapy and other regimens with a high‐quality design is required. [ABSTRACT FROM AUTHOR]

Background/Aims: Angiotensin receptor blockers (ARBs) may be beneficial for clinical remission during conventional therapy with tonsillectomy and steroid pulse (TSP) for active IgA nephropathy. Methods: Seventy-seven patients with active IgA nephropathy were randomly assigned to the control arm with conventional regimen (TSP followed by oral prednisolone) (n = 37) or the ARB arm with conventional regimen plus ARB candesartan for the first 6 months (n = 40). Patients not achieving proteinuria remission at 12 months in either arm were administered candesartan, which was titrated until the 24-month follow-up. The primary endpoints were remission of proteinuria (< 0.3 g/gCr) and hematuria at 12 months. Results: Baseline proteinuria (g/g Cr) were comparable between the control and ARB arm (1.02 vs. 0.97, P = 0.97). Similarly, cumulative remission rates at 6, 12, and 24 months were comparable between the control and ARB arms (37.8% vs. 35% [P = 0.80], 48.7% vs. 38.5% [P = 0.37], 71.4% vs. 51.3% [P = 0.08]). Proteinuria, which was slightly worse in the control arm than in the ARB arm at 6 months, was comparable afterwards (0.20 vs. 0.23 g/g Cr at 12 months; 0.12 vs. 0.13 g/g Cr at 24 months). Significant reductions observed in urinary angiotensinogen were almost comparable between the two treatment arms at both 6 and 12 months. Conclusion: Early candesartan treatment combined with TSP may not benefit clinical remission regardless of the blood pressure. ARB titration later during the treatment might provide benefit for patients with active IgA nephropathy. [ABSTRACT FROM AUTHOR]

Severe fever with thrombocytopenia syndrome (SFTS) is a tick-borne infectious disease caused by the SFTS virus (SFTSV). Clinical symptoms of SFTS often involve encephalopathy and other central neurological symptoms, particularly in seriously ill patients; however, pathogenesis of encephalopathy by SFTSV is largely unknown. Herein, we present case reports of three patients with SFTS, complicated by encephalopathy, admitted to Tokushima University hospital: one patient was a 63-year-old man, while the other two were 83- and 86-year-old women. All of them developed disturbance of consciousness around the 7th day post onset of fever. After methylprednisolone pulse therapy of 500 mg/day, all of them recovered without any neurological sequelae. SFTSV genome was not detected in the cerebrospinal fluid of 2 out of the 3 patients that were available for examination. In these patients, disturbance of consciousness seemed to be an indirect effect of the cytokine storm triggered by SFTSV infection. We propose that short-term glucocorticoid therapy might be beneficial in the treatment of encephalopathy during early phase of SFTSV infection. [ABSTRACT FROM AUTHOR]

Background: High circulating levels of soluble tumor necrosis factor receptors (TNFRs: TNFR1, TNFR2) predict renal function decline in a variety of kidney diseases. Tonsillectomy with steroid pulse (TSP) therapy has been reported as a remission induction therapy in IgA nephropathy (IgAN), mainly in Japan. However, little is known about whether TNFR levels change after TSP therapy in patients with IgAN. Methods: Two hundred twenty-three patients with IgAN were stratified according to the estimated glomerular filtration rate (eGFR): Group I (eGFR ≥ 60 mL/min/1.73 m, n = 172) and Group II (eGFR < 60 mL/min/1.73 m, n = 51). We measured serum TNFR levels with immunoassay in all patients at the time of renal biopsy, and also in patients whose samples just before the first (after tonsillectomy) ( n = 34) and/or the third steroid pulse therapy ( n = 77) were available. Results: The TNFR levels were significantly higher in Group II than in Group I. A significant negative correlation was observed between TNFR levels and eGFR at baseline (TNFRs: r > −0.50). In multivariate logistic regression analysis, both TNFRs were associated with renal function decline, independent of age and uric acid levels. Proteinuria and hematuria remarkably improved after TSP therapy, as expected. In comparison with baseline TNFR levels, the levels of TNFR2, but not TNFR1, decreased significantly just before the third steroid pulse therapy, although both levels did not change after tonsillectomy. Conclusions: The TNFR2 level did not change after tonsillectomy alone but decreased significantly after steroid pulse therapy in patients with IgAN. [ABSTRACT FROM AUTHOR]

Aim To evaluate the efficacy of intravenous high-dose pulses of methylprednisolone ( IVPM) for treatment of ocular involvement in Behcet's disease ( BD). Method In a double-blind control study, we randomized BD patients with posterior uveitis ( PU) and/or retinal vasculitis ( RV) into two groups. They received either IVPM (1000 mg methylprednisolone) or placebo for 3 consecutive days. Both groups received combination therapy with IV cyclophosphamide, azathioprine and prednisolone for 6 months. Visual acuity ( VA), Disease Activity Index ( DAI) based on the inflammatory state of each section of each eye, total inflammatory ( TIAI) and adjusted DAI ( TADAI) for each patient were calculated. The comparisons were done by paired t- and Mann-Whitney U-test. Results Seventeen patients in each group completed the treatment. The mean VA improved from 0.5 to 0.8 ( P < 0.000001) for the study and from 0.6 to 0.7 ( P < 0.02) for the placebo group. The difference was significant ( P = 0.01). The comparison showed no significant difference regarding DAI improvement in other items ( P > 0.2): PU, 1.9 to 0.5 ( P < 0.0006) versus 2.3 to 0.8 ( P < 0.0002); RV: 4.0 to 1.1 ( P < 0.0004) versus 3.1 to 1.1 ( P < 0.0005); TIAI: 23 to 5.7 ( P < 0.0002) versus 24.8 to 8.4 ( P < 0.003); TADAI: 24.1 to 7.3 ( P < 0.0002) versus 25.9 to 7.9 ( P < 0.004). We had one flare in the study versus seven in the placebo group ( P < 0.005). Conclusion Adding high-dose intravenous steroid pulse therapy to conventional combination therapy for severe ocular lesions of BD may cause better improvement on VA and fewer flares during the first 6 months of treatment. [ABSTRACT FROM AUTHOR]

Background: IgA nephropathy (IgAN) is a chronic glomerular disease that causes end-stage renal disease in 20-40 % of patients within 20 years. The efficacy of tonsillectomy combined with steroid pulse (SP) administration (TSP) for clinical remission of IgAN has been reported. Particularly in Japan, TSP has been performed widely. However, the optimum method for steroid administration in TSP has not been established. Methods: We retrospectively compared clinical remission in IgAN patients treated with tonsillectomy combined with two different steroid administration methods: (1) three courses of SP therapy and oral prednisolone administered on alternate days (group 3A; n = 25); and (2) one course of SP therapy and oral prednisolone administered on consecutive days (group 1C; n = 22). Results: There was no significant difference in the clinical remission rates between the two groups at 12 (48.0 vs. 40.9 %, P = 0.77) and 24 months after starting treatment (68.0 vs. 72.7 %, P = 0.76) and at the final observation (76.0 vs. 81.8 %, P = 0.73). The mean period from starting treatment to remission of hematuria in group 3A was significantly shorter than that in group 1C (5.7 ± 4.4 vs. 9.9 ± 5.9 months, P = 0.03). Dyslipidemic patients treated for the first time with statin after the SP therapy were more present in group 3A at 24 months ( P = 0.02). Conclusions: In IgAN patients, treatment of group 3A may be effective for inducing rapid remission of hematuria. Further studies are needed to establish an appropriate protocol for TSP. [ABSTRACT FROM AUTHOR]

Background: In addition to renin-angiotensin system inhibition (RAS), corticosteroids are recommended for patients who have immunoglobulin A nephropathy (IgAN) with ≧1 g/day proteinuria. Tonsillectomy plus corticosteroid pulse therapy (TSP) had been reported as more effective in producing clinical remission of IgAN than just oral-corticosteroid (OS) or steroid-pulse (SP) therapy-but that remained unconfirmed. Accordingly, this study compared the effects of TSP, corticosteroid therapies, and RAS on a multicenter, large-scale, long-term cohort. Methods: 1127 biopsy-proven IgAN patients with chronic kidney disease (CKD), G1-3, treated in our hospitals March 1981-December 2013 with TSP ( n = 209), SP ( n = 103), OS ( n = 300), or RAS, alone ( n = 515), were followed until end-stage renal disease (ESRD) or death, renal survival compared by treatment and proteinuria level. Hazard ratios (HRs) of ESRD were analyzed after adjusting for sex, age, BMI, eGFR, albumin, proteinuria, hematuria, blood pressure, medications, and renal-biopsy year, with propensity-score-matched analyses performed. Results: With TSP as referent, the overall HRs of SP, OS, and RAS were, respectively, 1.33 (0.44-4.04), 3.56 (1.45-8.71), and 3.64 (1.48-8.96); with proteinuria ≧1.0 g/gCre, respective HRs were 2.99 (0.71-12.54), 5.04 (1.44-17.67), and 7.23 (1.98-26.40); with proteinuria <1.0 g/gCre, 0.42 (0.04-4.89), 3.24 (0.79-13.30), and 2.05 (0.52-8.05); and for patients with CKD G3, 0.37 (0.10-1.41), 2.14 (0.77-5.94), and 2.03 (0.72-5.72). Similar results were observed in models including pathological grading and/or propensity-score matching. Conclusion: TSP may decrease the risk of ESRD in IgAN patients better than other therapies in CKD G1-2, with proteinuria ≧1.0 g/gCre, while outcome was similar to SP in CKD G3, or with proteinuria <1.0 g/gCre. [ABSTRACT FROM AUTHOR]

We report a case of recurrent Henoch-Schönlein purpura nephritis (HSPN) treated successfully with a tonsillectomy and steroid pulse therapy in a kidney transplant patient. A 29-year-old woman was admitted to our hospital for an episode biopsy; she had a serum creatinine (S-Cr) of 1.0 mg/dL and 1.34 g/day proteinuria 26 months after kidney transplantation. Histological examination revealed increased amounts of mesangial matrix and mesangial hypercellularity with IgA deposition. Of note, one glomerulus showed focal endocapillary proliferation and tuft necrosis. We diagnosed active recurrent HSPN. Considering both the histological findings and refractory clinical course of the native kidney, she was treated for 3 consecutive days with steroid pulse therapy and a tonsillectomy. The patient's proteinuria decreased gradually to less than 150 mg/day 6 months later. A second biopsy 6 years after kidney transplantation showed an excellent response to treatment and revealed a marked reduction in both the mesangial matrix and mesangial hypercellularity, with trace IgA deposition. We conclude that a tonsillectomy and steroid pulse therapy appeared to be useful in this patient with active recurrent HSPN. This paper is the first to report a tonsillectomy and steroid pulse therapy as a therapeutic option for active recurrent HSPN. Further studies are needed to elucidate the efficacy and mechanisms of tonsillectomy with recurrent HSPN in kidney transplant patients. [ABSTRACT FROM AUTHOR]

Background: The IgA nephropathy (IgAN) Study Group in Japan conducted a multicenter, randomized, controlled trial of tonsillectomy with steroid pulse therapy (TSP) versus steroid pulse monotherapy in patients with IgAN (UMIN Clinical Trial Registry Number; C000000384). The effects of therapies in relation to pathological severity were analyzed in this study. Methods: The patients with IgAN, urinary protein 1.0-3.5 g/day, serum creatinine of 1.5 mg/dl or less were randomly assigned to receiving TSP (Group A) or steroid pulses alone (Group B). The primary endpoint was the disappearance of proteinuria and/or hematuria. Twenty-six biopsies in Group A and 33 in Group B were available. The histological grades (HG) according to the percentage of glomeruli with crescent or sclerosis and the Oxford classification were analyzed. Results: The patients in Group A had a 4.32- to 12.1-fold greater benefit of disappearance of proteinuria and 3.61- to 8.17-fold greater benefit of clinical remission (disappearance of proteinuria and hematuria) than those in Group B in patients with HG2-3, acute lesions (cellular or fibrocellular crescent) affecting more than 5 % of glomeruli, chronic lesions (fibrous crescents or sclerosis) affecting more than 20 % and S1. In contrast, odds ratios for disappearance of proteinuria or clinical remission in Group A to Group B were not significant in patients with HG 1, acute lesion in 5 % or less of glomeruli, chronic lesion in 20 % or less and S0. The disappearance of hematuria showed no relation to pathological severity. Conclusion: TSP might be better employed according to the pathological severity. [ABSTRACT FROM AUTHOR]

Background: Medical intervention for patients with IgA nephropathy and mild proteinuria (<1.0 g/day) is controversial, and the effectiveness of tonsillectomy plus steroid pulse therapy (TSP) for such patients remains obscure. Methods: Among 323 patients in our multicenter cohort study, 79 who had mild proteinuria (0.4-1.0 g/day) at diagnosis were eligible to participate in this study. We compared the clinicopathological findings at diagnosis, a decline in renal function defined as a 50 or 100 % increase in serum creatinine (sCr) and clinical remission (CR) defined as the disappearance of hematuria and proteinuria (<0.3 g/day) among groups given TSP ( n = 46), steroid therapy (ST) ( n = 9), and non-ST ( n = 24). Factors contributing to CR were also evaluated using multivariate analysis. Results: Background factors at diagnosis including age, ratio (%) of patients with hypertension, sCr, proteinuria, and histological severity did not significantly differ among the groups. Only two patients each in the TSP (4.3 %) and non-ST (8.3 %) groups achieved a 50 % increase in sCr during a mean follow-up period of 4.7 years. At the final observation, 71.7, 44.4, and 41.7 % of patients in the TSP, ST, and non-ST groups, respectively, achieved CR ( p = 0.032). Cox proportional hazards models revealed that TSP led to CR more effectively than non-TSP by a factor of about threefold (hazard ratio, 2.74; p = 0.008). Conclusion: TSP therapy has potential for inducing CR in patients with IgAN and mild proteinuria (<1.0 g/day). [ABSTRACT FROM AUTHOR]

Background: In 2011, the Japanese Society of Nephrology (JSN) published new clinical guidelines for IgA nephropathy (IgAN) with a new risk stratification based on clinical and histological severity. For classification, patients are divided into four groups (low, medium, high, and very high risk). However, differences in responsiveness to each treatment among different groups remain unclear. We evaluate the responsiveness of tonsillectomy plus steroid pulse (TSP) therapy using the new risk stratification. Methods: We retrospectively reviewed 111 IgAN patients with TSP therapy between January 2003 and January 2013. Study patients were divided into three groups [low- ( n = 40), medium- ( n = 43) and high-/very high-risk group ( n = 28)]. The primary outcome was clinical remission (CR). The observation period was 1 year following tonsillectomy. Results: 57 out of 111 patients (51.4 %) reached CR. The CR incidence was 70.0, 41.9 and 39.3 % (the low-, the medium- and the high-/very high-risk group, respectively). The incidence of CR was significantly higher in the low-risk group ( P = 0.013). In a multivariate logistic regression analysis, both the medium- and the high-/very high-risk group showed significantly lower incidence of inducing CR than the low-risk group [(odds ratio 0.324; 95 % confidence interval 0.106-0.939, P = 0.041) (odds ratio 0.239; 95 % confidence interval 0.058-0.910, P = 0.040), respectively]. Conclusions: The new risk stratification in the 2011 JSN clinical guidelines for IgAN had a positive impact on early CR of TSP therapy. [ABSTRACT FROM AUTHOR]

Background: In recent years, tonsillectomy and steroid pulse (TSP) therapy have been widely performed in Japan. However, there is no consensus about the treatment protocol and indication. Methods: In this retrospective analysis, we compared patients who received tonsillectomy plus intermittent steroid pulse (SP) therapy three times in 6 months (ISP group, n = 44) with patients who received tonsillectomy plus 3 weeks of consecutive SP therapy (CSP group, n = 46) within 1 year after renal biopsy. These two different protocols were performed at two different institutions. We analyzed the clinical and histological background and clinical remission (CR), defined as disappearance of urine abnormalities at 18 months after starting treatment. Results: Before treatment, there was no significant difference in the clinical findings except for sex between the two groups. In ISP group and CSP group, mean estimated glomerular filtration rate was 82.1 ± 20.9 and 85.9 ± 19.1 ml/min/1.73 m, median proteinuria was 0.55 and 0.56 g/day, and median urinary red blood cells were 20 (10-20) and 20 (6-30)/high power filed. The histological (H) grade was lower in the CSP than the ISP group ( p = 0.022). The remission rate of proteinuria, hematuria, and rate of CR by the Kaplan-Meier method and logrank test were significantly higher in the CSP group than in the ISP group (CSP vs. ISP group; proteinuria: 97.8 vs. 77.3 %, p < 0.001, hematuria: 97.8 vs. 75.0 %, p = 0.005, CR: 95.6 vs. 63.6 %, p < 0.001). In the Cox proportional hazard model (forced entry), SP protocol and proteinuria before treatment were significantly associated with CR [SP protocol: hazard ratio (HR) 2.50, 95 % confidence interval (CI) 1.46-4.30, p = 0.001, proteinuria: HR 0.81, 95 % CI 0.68-0.96, p = 0.013)]. However H-grade was associated with remission of proteinuria (H-grade: hazard ratio (HR) 0.56, 95 % confidence interval (CI) 0.37-0.85, p = 0.006), and this result meant histological bias affected the remission of proteinuria. Conclusions: The difference of the protocol of TSP therapy may have some effect on the CR of IgAN, though the histological bias was observed in this study. The appropriate protocol and indication of TSP therapy must be analyzed and determined in the randomized controlled trial. [ABSTRACT FROM AUTHOR]

We report the case of a 64-year old man who presented memory disturbance, low-grade fever, weight loss, and bilateral hand tremors for three months. He was diagnosed with nonherpetic acute limbic encephalitis (NHALE). Follow-up magnetic resonance imaging (MRI) revealed new lesions after symptomatic improvement following steroid pulse therapy. This may indicate that there is a time lag between the disturbance or recovery of neurons and astrocytes. Thus, other lesions might occasionally appear during convalescence in patients with NHALE, even if only minimal lesions were found on the initial MRI. [ABSTRACT FROM AUTHOR]

Patients with Kawasaki disease (KD) who did not respond to the initial IVIG are known to have higher risk for developing coronary arterial lesions (CALs). Our aim is to clarify whether patients with initial IVIG resistant KD may benefit from methylprednisolone pulse therapy (MPT) in comparison with re- treatment of IVIG (2nd IVIG). A total of 237 patients (median age: 2 years 2 months; range 1 months-10 years) with KD were initially treated with IVIG (2 g/kg). Among them, 41 patients (22 %) were assessed as IVIG resistance: these patients were allocated to either group A receiving MPT ( n = 14) or group B receiving the 2nd IVIG ( n = 27). Patients with resistant to the additional therapy (MPT or 2nd IVIG) were received second IVIG (group A) or MPT (group B). Changes in leukocyte count, C-reactive protein and albumin before and after an additional therapy were significantly greater in group A than those in group B. However, the prevalence of CALs did not differ between the groups (36 % in group A and 26 % in group B, p > 0.05). There was no significant difference in the medical cost between the groups (median cost: 92,032 JPY in group A and 97,331 JPY in group B). MPT does not reduce the risk of development to CAL and does not seem to be beneficial as single agent therapy for IVIG resistant KD. [ABSTRACT FROM AUTHOR]

Background Autoimmune hypophysitis very rarely spreads to nearby organs outside the pituitary tissue, for unknown reasons, with only 5 reported cases of hypophysitis spreading over the cavernous sinus. Case presentation Three patients presented with cases of non-infectious hypophysitis spreading outside the pituitary tissue over the cavernous sinus. All three cases were diagnosed with histological confirmation by transsphenoidal surgery, and the patients showed remarkable improvement with postoperative pulse dose steroid therapy, including disappearance of abnormal signal intensities in the bilateral hypothalami on magnetic resonance imaging, resolution of severe stenosis of the internal carotid artery, and normalization of swollen pituitary tissues. Two of 3 cases fulfilled the histological criteria of immunoglobulin G4-related disease, although none of the patients had high serum immunoglobulin G4 level. Conclusion The true implications of such unusual spreading of hypophysitis to nearby organs are not fully understood, but the mechanism of occurrence might vary according to the timing of inflammation in this unusual mode of spreading. Pulse dose steroid therapy achieved remarkably good outcomes even in the patient with progressive severe stenosis of the internal carotid artery and rapid visual deterioration. [ABSTRACT FROM AUTHOR]

Background: The most serious side effects of systemic steroids include osteoporosis and suprarenal suppression. Many steroid regimens have been suggested to minimize these side effects; one of them is oral steroid pulse therapy. Objective: To compare the side effects of a daily oral steroid regimen versus a weekly oral steroid pulse regimen on bone mineral density and suprarenal suppression. Methods: Thirty patients with different skin diseases were divided into two groups: 15 for oral daily steroids (ODS) (group 1) and 15 for weekly oral pulse steroids (WOPS) (group 2). They were evaluated for bone mineral density (measured by DEXA) and suprarenal suppression (measured by serum cortisol level), morphological changes and blood sugar. Treatment was continued for 6 months to 3 years. Results: Cushingoid features in group 1 were observed in 73%, yet they were not detectable in group 2. Disturbed blood sugar in group 1 was 33% and 0% in group 2. The serum cortisol level was lower in patients on ODS than those on WOPS. The effect of WOPS on bone mineral density was very limited in comparison with the ODS. Conclusion: Weekly oral steroid pulse therapy induces no significant bone loss and no suprarenal suppression and can be an alternative option in the treatment of chronic disorders requiring long-term oral steroid therapy. [ABSTRACT FROM AUTHOR]