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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 Background A causal relationship between acute pancreatitis and administration of glucocorticoids remains a matter of debate, since most of the reported cases were diagnosed with systemic vascular diseases (including systemic lupus erythematosus and polyarteritis nodosa) that may be responsible for the pancreatitis. Case presentation We report a case of a 51-year-old woman who developed acute pancreatitis after receiving methylprednisolone pulse therapy for the treatment of fulminant autoimmune hepatitis (AIH). She was admitted to our hospital because of overt jaundice and back pain. Since her liver dysfunction deteriorated progressively, a liver biopsy was performed and a diagnosis of AIH was established. She was given intravenous methylprednisolone pulse therapy at 1000 mg/day for 3 days, and oral prednisolone at 40 mg/day thereafter. While her liver function improved rapidly, she started complaining of mild back pain and serum amylase and lipase levels were elevated from 5 days after the initiation of steroid therapy. A CT scan revealed mildly edematous changes around the pancreas, leading to a diagnosis of acute pancreatitis. After tapering off prednisolone, back pain disappeared, and elevated serum amylase was normalized without exacerbation of AIH. A systematic literature review identified 8 cases of acute pancreatitis developing after administration of corticosteroid pulse therapy with a median latent period of 5 days. Conclusions The present case and reports in the literature suggest that steroid pulse therapy may cause acute pancreatitis in patients having no signs of systemic vasculitis.

Abstract We herein report a 50-year-old Japanese woman with breast cancer who complained of blurred vision and central scotoma in her left eye on the 12th day after surgery. Subsequently, the sudden-onset binocular visual disorder progressed, and she was diagnosed with cancer-associated retinopathy (CAR) based on the clinical findings. Although her visual acuity temporarily improved following the start of adjuvant chemotherapy, reductions in her visual acuity progressed once again. After two courses of steroid pulse therapy initiated from the 59th day following the onset of CAR, although her visual field was still constricted, her binocular visual acuity improved from finger movement to 0.8 2 months later. The shorter the period from onset to treatment, the better the prognosis of the visual function. However, a diagnosis is often delayed because the incidence of this disease is very rare. Therefore, it is important to suspect CAR whenever a sudden visual disorder develops in cancer patients. Furthermore, treatment is believed to be effective even if steroid therapy is started up to 2 months from onset.

Objective: The purpose of this study was to elucidate the rapid impact of high-dose intravenous methylprednisolone pulse therapy (1,000 mg/day for 3 days) on the eventual visual prognosis in patients with serum anti-aquaporin-4 immunoglobulin G (AQP4-IgG)–positive neuromyelitis optica spectrum disorders (NMOSDs) who had an attack of optic neuritis (ON). Methods: Data from 32 consecutive NMOSD patients (1 male and 31 female) with at least one ON attack, involving a total of 36 ON-involved eyes, were evaluated. The following variables at ON onset were evaluated: sex, age at the first ON episode, visual acuity at nadir, visual acuity after 1 year, duration from ON onset to treatment for an acute ON attack, cycles of high-dose intravenous methylprednisolone pulse therapy for the ON attack, and cycles of plasmapheresis for the ON attack. Among the 36 ON-involved eyes, 27 eyes were studied using orbital MRI with a short-T1 inversion recovery sequence and gadolinium-enhanced fat-suppressed T1 imaging before starting treatment in the acute phase. Results: In univariate analyses, a shorter duration from ON onset to the initiation of high-dose intravenous methylprednisolone pulse therapy favorably affected the eventual visual prognosis 1 year later (Spearman's rho = 0.50, p = 0.0018). The lesion length on orbital MRI was also correlated with the eventual visual prognosis (rho = 0.68, p < 0.0001). Meanwhile, the days to steroid pulse therapy and lesion length on orbital MRI did not show a significant correlation. These findings suggest that the rapidness of steroid pulse therapy administration affects the eventual visual prognosis independent of the severity of ON. In multivariate analysis, a shorter time from ON onset to the start of acute treatment (p = 0.0004) and a younger age at onset (p = 0.0071) were significantly associated with better visual outcomes. Conclusions: Rapid initiation of high-dose intravenous methylprednisolone pulse therapy is essential to preserve the eventual visual acuity in patients with serum AQP4-IgG-positive NMOSD. Once clinicians suspect acute ON with serum AQP4-IgG, swift administration of steroid pulse therapy before confirming the positivity of serum AQP4-IgG would be beneficial for preserving visual function. [ABSTRACT FROM AUTHOR]

Background: Immune checkpoint inhibitors can cause various adverse effects. Recently it has been shown that Vogt-Koyanagi-Harada (VKH) disease-like uveitis can occur in patients treated with nivolumab.Case Presentation: A 69-year-old man developed bilateral panuveitis after nivolumab treatment for recurrent hypopharyngeal cancer. Slit lamp examination revealed bilateral granulomatous keratic precipitates, anterior chamber cells and partial synechiae. Fundus examination revealed bilateral optic disc edema and diffuse serous retinal detachment. His human leukocyte antigen (HLA) typing showed HLA-DRB1*04:05 allele. A lumbar puncture did not demonstrate pleocytosis. Bilateral sub-tenon injections of triamcinolone acetonide were initiated. As his panuveitis did not regress completely, steroid pulse therapy was administered. That therapy led to the resolution of his serous retinal detachment and to rapid improvement in his vision. Following this, we treated him with 50 mg/day of prednisolone for 1 week and then reduced it by 5 mg every week. No bilateral uveitis relapse had occurred by his 3-month follow-up; however, he subsequently died because of his cancer.Conclusion: To our knowledge, this is the first report of a patient with NVKH who underwent a lumbar puncture. Unlike VKH, our case did not show meningismus or pleocytosis. NVKH may, therefore, have a different etiology from VKH. In cases of NVKH with posterior uveitis, steroid pulse therapy may be considered as a treatment option, as it is in VKH. [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: Myelin-oligodendrocyte glycoprotein antibody-positive optic neuritis (MOGON) is usually responsive to the steroid, but, for some patients, steroid pulse therapy alone may be inadequate. This study aimed to investigate the factors predicting the response to steroid pulse therapy in MOGON., Methods: This study included 17 patients (24 eyes) with MOGON, who received single steroid pulse therapy as initial treatment. Best corrected visual acuity (BCVA) and mean deviation (MD) values after treatment were examined concerning findings at onset., Results: No correlation was found between BCVA at onset and after treatment, but a correlation was observed between MD values at onset and after treatment (correlation coefficient 0.48, p=0.01, Spearman's rank correlation coefficient). Age, gender, duration from onset to treatment, magnetic resonance imaging findings, and optical coherence tomography findings did not affect visual function after treatment., Conclusions: Severe visual field impairment at onset may indicate that additional treatment may be necessary., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2024, Takai et al.)

A 26-year-old Indian man who had arrived in Japan 24 days prior presented to our hospital with abdominal pain and a fever. A blood test revealed marked hepatic dysfunction, and imaging tests confirmed a diagnosis of acute hepatitis. The patient's liver function and coagulability deteriorated, and his general condition was poor. Given the possibility of fulminant hepatic failure, we initiated steroid pulse therapy. Following the initiation of steroid therapy, the patient's liver function and subjective symptoms rapidly improved. Testing revealed positive findings for IgA-hepatitis E virus, and a genetic analysis of hepatitis E identified genotype 1, which is not endemic to Japan, leading to a definitive diagnosis of imported hepatitis E infection from India. The successful response to steroid therapy highlights the potential benefit of this approach in managing severe cases of acute hepatitis E, a rare occurrence in Japan. This case underscores the importance of considering hepatitis E infection in individuals with a recent travel history to regions with high prevalence and the potential benefits of steroid therapy in managing severe cases of acute hepatitis E.

Purpose: To evaluate the efficiency of plasma exchange (PE) add-on on optic neuritis (ON) in neuromyelitis optica spectrum disorders (NMOSD). Methods: Our ambispective, nonrandomized study was performed in Southwest Hospital, Southwest Eye Hospital, Army Medical University. We studied 31 consecutive NMOSD patients characterized by ON and hospitalized from September 2015 to May 2018. Their clinical features were assessed, and efficiency of PE add-on treatment in ON of NMOSD was evaluated. Correlation was assessed between the effect of steroid pulse therapy (SPT) and the number of ON episodes in NMOSD. Results: All 31 NMOSD patients accepted SPT; 15 patients of them accepted SPT and PE add-on. In these 15 patients, after PE add-on treatment, the patients' visual acuity was further significantly improved (P = 0.000, N = 23), including 3 no light perception (NLP) patients. After the treatment, the visual function recovered quickly in the first 2 months and then gradually slowed down; the visual function remained stable about 6 months later. The correlation coefficient between visual acuity improvement of SPT and the number of ON episodes was − 0.311 (P = 0.030, N = 49). Conclusion: One clinical feature of NMOSD can be repeated vision impairment. In NMOSD patients characterized by ON, efficacy of SPT is limited as the number of episodes increased, and PE add-on is more effective. Even though the visual acuity of NMOSD patients decreases to NLP during episodes, there is still a chance to restore vision by PE add-on treatment. [ABSTRACT FROM AUTHOR]

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]

Chronic kidney disease (CKD) is a worldwide public health problem that affects millions of people from all racial and ethnic groups. Although CKD is not one specific disease, it is a comprehensive syndrome that includes IgA nephropathy. As reported by the Japanese Society of Nephrology, 13.0 million people have CKD. In Japan, major causes of end-stage kidney disease are type 2 diabetic nephropathy, chronic glomerulonephritis, especially IgA nephropathy, hypertensive nephrosclerosis, and polycystic kidney disease. IgA nephropathy is characterized by polymeric IgA1 with aberrant galactosylation (galactose-deficient IgA1) increased in the blood and deposited in the glomerular mesangial areas, as well as partially in the capillary walls. The tonsils are important as one of the responsible regions in this disease. The clarification of the mechanism of galactose-deficient IgA1 production will pave the way for the development of novel therapies. The results of future research are eagerly awaited. At present, the most important therapeutic goals in patients with IgA nephropathy are the control of hypertension, the decrease of urinary protein excretion, and the inhibition of progression to end-stage kidney disease. Several investigators have reported that renin–angiotensin–aldosterone system inhibitors reduce levels of urinary protein excretion and preserve renal function in patients with IgA nephropathy. In Japan, tonsillectomy and steroid pulse therapy are more effective for patients with IgA nephropathy.

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]

Background Optic nerve diseases include inflammatory optic nerve diseases such as vasculitis, metabolic optic neuropathy, ischemic optic neuropathy, and optic neuritis. In this study, patients with acute optic neuritis are classified with better and poor visual acuity based on visual acuity after one month of steroid pulse therapy. To determine prognosis, initial visual acuity and critical fusion frequency (CFF) values will be compared with those recorded one month after treatment and at the last visit. Methods Visual acuity and CFF were evaluated one month after the start of treatment in patients diagnosed with acute optic neuritis, and follow-up was available for at least three months at Hiroshima University Hospital. Results All patients received steroid pulse therapy as initial treatment. After one month of treatment, visual acuity and CFF at the last visit were significantly improved in the group with improved visual acuity compared to the group with impaired visual acuity. Conclusions Visual acuity at the initial visit did not affect treatment outcome, and final visual acuity and CFF after one month of treatment for acute optic neuritis were better in patients with better visual acuity. Therefore, visual acuity values one month after treatment initiation may affect treatment outcomes., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Tsumura et al.)

Objective Steroid pulse therapy is a regimen involving the intravenous administration of supra-pharmacological doses of corticosteroids in the short term. It is used to treat various inflammatory and autoimmune conditions. However, the strengths and limitations of steroid pulse therapy for induction of remission in type 1 autoimmune pancreatitis (AIP) are unknown. Methods Depending on the steroid therapy regimen administered, the 104 patients with type 1 AIP included in this retrospective study were divided into three groups: conventional oral prednisolone (PSL) regimen (PSL group), intravenous methylprednisolone (IVMP) pulse followed by oral PSL regimen (Pulse+PSL group), and IVMP pulse-alone regimen (Pulse-alone group). We then examined the relapse rate and adverse events among the three groups. Results The Kaplan-Meier estimates of the relapse rate at 36 months after steroid therapy were 13.6% in the PSL group, 13.3% in the Pulse+PSL group, and 46.2% in the Pulse-alone group. The log-rank test revealed that the relapse-free survival in the Pulse-alone group was significantly shorter than that in the PSL (p=0.024) and Pulse+PSL groups (p=0.014). The exacerbation of glucose tolerance after steroid therapy was less frequently observed in the Pulse-alone group (0%) than in the PSL group (17%, p=0.050) and Pulse+PSL groups (26%, p=0.011). Conclusion Although treatment with IVMP pulse alone resulted in unsatisfactory relapse prevention outcomes compared with conventional steroid therapy, the IVMP pulse-alone regimen might be an alternative treatment strategy for type 1 AIP from the perspective of avoiding adverse events from steroids.

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]

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]

We herein report a 50-year-old Japanese woman with breast cancer who complained of blurred vision and central scotoma in her left eye on the 12th day after surgery. Subsequently, the sudden-onset binocular visual disorder progressed, and she was diagnosed with cancer-associated retinopathy (CAR) based on the clinical findings. Although her visual acuity temporarily improved following the start of adjuvant chemotherapy, reductions in her visual acuity progressed once again. After two courses of steroid pulse therapy initiated from the 59th day following the onset of CAR, although her visual field was still constricted, her binocular visual acuity improved from finger movement to 0.8 2 months later. The shorter the period from onset to treatment, the better the prognosis of the visual function. However, a diagnosis is often delayed because the incidence of this disease is very rare. Therefore, it is important to suspect CAR whenever a sudden visual disorder develops in cancer patients. Furthermore, treatment is believed to be effective even if steroid therapy is started up to 2 months from onset. [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]

We herein report a rare case of acute hemorrhagic rectal ulcer (AHRU) complicated by cytomegalovirus enteritis following steroid pulse therapy for interstitial pneumonia. An 86-year-old woman underwent steroid pulse therapy for interstitial pneumonia. She was bedridden with dyspnea and suddenly developed melena. Colonoscopy revealed AHRU, which did not improve with conservative treatment, but did improve with ganciclovir administration for cytomegalovirus enteritis. This gastrointestinal complication has not received much attention by pulmonologists who perform steroid pulse therapy for interstitial pneumonia. Delayed treatment of this complications can be fatal. Caution should be taken when administering steroid pulse therapy to bedridden patients with interstitial pneumonia.

A 34-year-old male visited the hospital due to decreased visual acuity in the left eye following an injury from a car accident. In the left eye, best-corrected visual acuity (BCVA) was hand motion and intraocular pressure (IOP) was 8 mmHg. Choroidal vasodilation and chorioretinal folds were observed by spectral domain-optical coherence tomography (SD-OCT). Topical and systemic steroid treatments did not improve the chorioretinal folds. Twelve months after the injury, intra-vitreal triamcinolone (4 mg/0.1 ml) was injected. Six months after intra-vitreal triamcinolone injection, BCVA in the left eye had improved to 20/100. Fundus examination showed improvement in retinal vascular tortuosity and SD-OCT revealed improvements in choroidal vasodilation and chorioretinal folds. Intra-vitreal triamcinolone injection (IVTI) was effective against traumatic chorioretinal folds with no recurrence based on objective observation by fundus photography and SD-OCT.

According to the EUGOGO’s (European Group On Graves' Orbitopathy) last consensus, the treatment of choice for Graves’ Orbitopathy (GO) is pulses of glucocorticoid (GC). There is a lack of evidence for superiority of any intravenous (iv) GC schedules. As a rule this therapy is pretty safe. However, acute cardiovascular diseases and acute severe liver damage has been reported in sporadic cases during ivGC, resulting in fatal acute liver failure in a few patients. Design: we performed a prospective study in 36 Graves' patients with GO who were treated with 1000 mg of ivGC daily for 5 days (group I, n = 20) or weekly for 5 weeks (group II, n = 16). Results: ALAT and ASAT increases were much more prominent in group I than in group II especially during the treatment and in the first 4 weeks after the treatment. Increase of blood pressure, glycemia and negative ECG alterations in group I resulted to their management. And we revealed much more patients complains in group I. Conclusion: Both schedules seem to be pretty safe, but therapy once a week (for 5 weeks) is safer and allows to treat patients in outpatient clinics, reduce expenditures on inpatient care, accelerate of initial treatment and include more patients into treatment.

Graves' ophthalmopathy constitutes a major therapeutic challenge, so specialists of various disci-plines have to combine their forces. According to the last consensus of EUGOGO (European Group On Graves' Orbitopathy) the treatment of choice for Graves' ophthalmopathy is pulses of glucocorticoid (GC). Evidence for the superiority of any of the different intravenous (iv) glucocorticoid schedules is lacking. Objective: to evaluate the efficacy of two different methylprednisolone pulse therapy schedules in Graves' ophthalmopathy. Materials and Methods: 30 Graves' patients with Graves' ophthalmopathy were subcategorized into two groups: Group A subjects (n = 14) were given iv glucocorticoid daily for 5 days (total 5 g) and Group B patients (n = 16) were given iv glucocorticoid weekly for 5 weeks (total 5 g). All patients were examined by the ophthalmologist before and in 1 and 12 weeks after the treatment. Results: We found that in group A visual acuity was significantly higher than in group B at 12 weeks. By 12 week proptosis, diplopia had improved as well as CAS had fallen in two groups but there were no significant differences between the two groups. Conclusion: Both schedules are effective on proptosis, soft tissue swelling. Daily pulse therapy appeared to be more effective on visual acuity. Thereby in case of active Graves' ophthalmopathy associated with impairment of visual acuity, daily pulse therapy is a treatment of choice. But, in spite of improvement, strabismus, diplopia, proptosis have a marked negative effect on quality of life, disturb a lifestyle. In these cases surgery should be considered. After end of our research two patients (3 orbits) had bone decompression with a good result. We consider that eye muscle surgery is next needed to restore their binocular vision.

A 50-year-old woman experienced cardiopulmonary arrest. Although the arrest lasted for 4 min, she could not be withdrawn from the mechanical ventilator because of low tidal volume, despite being awake and alert after admission. The results of the anti-acetylcholine receptor antibody and repetitive nerve stimulation tests were negative, and the anti-muscle-specific kinase antibody levels revealed myasthenia gravis. We recommended therapeutic plasma exchange; however, the patient refused the treatment as she did not want to use blood products. Consequently, we initially attempted steroid pulse therapy, which enabled the patient to be withdrawn from the mechanical ventilator. Thus, steroid pulse therapy was beneficial for the crisis associated with the anti-muscle-specific kinase antibody in the absence of therapeutic plasma exchange.

Vitiligo is a common depigmenting skin disease that is often difficult to treat. Even if repigmentation is achieved by treatment, recurrence in the same lesion is often found within a year after stopping treatment. As a background of these issues, a subset of CD8 + T cells that recognize melanocyte-specific antigens or CD49a + tissue-resident memory T cells that reside in the vitiligo lesion are thought to be involved. We investigated the MHC class I-restricted tyrosinase pentamer-positive CD8 + skin T cells in a progressive generalized vitiligo patient with HLA-A*02:01 who showed resistance to intravenous methylprednisolone pulse therapy. We found that HLA-A*02:01-restricted tyrosinase pentamer-positive CD8 + T cells remained in the lesions after the treatment and expressed IFN-γ and granzyme B. Interestingly, the expression of these cytokines in the pentamer-negative CD8 + T cells was decreased after intravenous methylprednisolone pulse therapy. These findings suggest that, in vitiligo patients, melanocyte-specific CD49a + CD8 + T cells are in a potent activation state that is uncontrolled despite systemic immunosuppressive treatment, which may contribute to treatment resistance and local recurrence., (© 2022 Japanese Dermatological Association.)

Chronic kidney disease (CKD) is a worldwide public health problem that affects millions of people from all racial and ethnic groups. Although CKD is not one specific disease, it is a comprehensive syndrome that includes IgA nephropathy. As reported by the Japanese Society of Nephrology, 13.0 million people have CKD. In Japan, major causes of end-stage kidney disease are type 2 diabetic nephropathy, chronic glomerulonephritis, especially IgA nephropathy, hypertensive nephrosclerosis, and polycystic kidney disease. IgA nephropathy is characterized by polymeric IgA1 with aberrant galactosylation (galactose-deficient IgA1) increased in the blood and deposited in the glomerular mesangial areas, as well as partially in the capillary walls. The tonsils are important as one of the responsible regions in this disease. The clarification of the mechanism of galactose-deficient IgA1 production will pave the way for the development of novel therapies. The results of future research are eagerly awaited. At present, the most important therapeutic goals in patients with IgA nephropathy are the control of hypertension, the decrease of urinary protein excretion, and the inhibition of progression to end-stage kidney disease. Several investigators have reported that renin–angiotensin–aldosterone system inhibitors reduce levels of urinary protein excretion and preserve renal function in patients with IgA nephropathy. In Japan, tonsillectomy and steroid pulse therapy are more effective for patients with IgA nephropathy. [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]