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Demyelinating optic neuritis (DON) is a rare but sight-threatening ophthalmic condition which occasionally occurs after human papilloma virus (HPV) vaccination. We herein report a case of previously healthy 13-year-old girl who developed a bilateral refractory DON three days after the first dose of bivalent HPV vaccine. The patient experienced bilateral severe visual loss three days after HPV vaccination, and her vision was quickly deteriorated to no light perception one day after the onset of DON. Ophthalmic examination revealed sluggish pupillary light reflex and swollen optic disc in both eyes, and an emergent orbital MRI examination revealed bilateral hyperintensity and enlargement of the intraorbital optic nerve with contrast enhancement. Serological tests for aquaporin-4 IgG antibody, myelin oligodendrocyte glycoprotein IgG antibody, and other common autoantibodies were all negative. The patient showed poor response to 10 days of intravenous methylprednisolone pulse therapy (500 mg, 250 mg, and 125 mg twice per day for 4, 4, and 2 days, respectively). Hence, three-dosed subcutaneous satralizumab was used in the acute stage of DON as an adjunct therapy. Her vision gradually improved after satralizumab therapy, and increased to 20/20 and 20/32 in the right and left eye at the 3-month follow-up. To the best of our knowledge, this is the first case report of satralizumab therapy in the AQP-4 Ab and MOG-Ab dual seronegative isolated DON. Our study indicates that satralizumab may be a safe and efficient adjunct therapy which can be used in the acute stage of the refractory DON poorly responding to steroid pulse therapy. [ABSTRACT FROM AUTHOR]

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]

Demyelinating optic neuritis (DON) is a rare but sight-threatening ophthalmic condition which occasionally occurs after human papilloma virus (HPV) vaccination. We herein report a case of previously healthy 13-year-old girl who developed a bilateral refractory DON three days after the first dose of bivalent HPV vaccine. The patient experienced bilateral severe visual loss three days after HPV vaccination, and her vision was quickly deteriorated to no light perception one day after the onset of DON. Ophthalmic examination revealed sluggish pupillary light reflex and swollen optic disc in both eyes, and an emergent orbital MRI examination revealed bilateral hyperintensity and enlargement of the intraorbital optic nerve with contrast enhancement. Serological tests for aquaporin-4 IgG antibody, myelin oligodendrocyte glycoprotein IgG antibody, and other common autoantibodies were all negative. The patient showed poor response to 10 days of intravenous methylprednisolone pulse therapy (500 mg, 250 mg, and 125 mg twice per day for 4, 4, and 2 days, respectively). Hence, three-dosed subcutaneous satralizumab was used in the acute stage of DON as an adjunct therapy. Her vision gradually improved after satralizumab therapy, and increased to 20/20 and 20/32 in the right and left eye at the 3-month follow-up. To the best of our knowledge, this is the first case report of satralizumab therapy in the AQP-4 Ab and MOG-Ab dual seronegative isolated DON. Our study indicates that satralizumab may be a safe and efficient adjunct therapy which can be used in the acute stage of the refractory DON poorly responding to steroid pulse therapy.

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 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.

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]

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. Abstract 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 Background Immune checkpoint inhibitors have been reported to have excellent therapeutic effects on various malignant tumors. However, immune‐related adverse events can occur, targeting various organs. Case presentation A 49‐year‐old male with lung carcinoma was started on carboplatin + pemetrexed + nivolumab (every 3 weeks) + ipilimumab (every 6 weeks), and nivolumab/ipilimumab was administered in the 3rd course. Subsequently, fever and fatigue developed, and grade 3 liver damage was also noted, so he was admitted to Kindai University Hospital. A bone marrow aspirate examination was performed on the third day of illness, and a definitive diagnosis of hemophagocytic lymphohistiocytosis (HLH) was made. It was determined that immediate therapeutic intervention was necessary, and pulse therapy with methylprednisolone was started on the third day of illness. After 3 days of pulse treatment, a rapid recovery of platelet values, a decrease in ferritin levels, and a decrease in lactate dehydrogenase were observed. Subjective symptoms such as fever and fatigue also quickly improved. Conclusion Early diagnosis and treatment for HLH resulted in a positive response. The number of HLH cases may increase in the future due to the expansion of immune checkpoint inhibitor indications.

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]

Abstract Background Steroids are widely used to modulate the inflammatory reactions associated with coronavirus disease 2019 (COVID-19); however, the optimal upper limit dose of steroid use for acute COVID-19 care remains unclear and currently available data may suffer from a time-dependent bias of no effectiveness or reversed causation given the desperate situation of treatment during this pandemic. Accordingly, the aim of this study was to elucidate the impact of intravenous pulse therapy with methylprednisolone (500 mg or greater per day) on the risk of in-hospital mortality among patients with COVID-19 by controlling for time-dependent bias. Methods We performed a prospective cohort study with 67,348 hospitalised acute COVID-19 patients at 438 hospitals during 2020–2021 in Japan. The impact of intravenous methylprednisolone pulse therapy on the risk of in-hospital mortality was examined based on hazard ratios (HRs) and 95% confidence intervals (95% CIs), with stratification according to the status of invasive mechanical ventilation (iMV). Time-dependent bias was controlled for in a marginal structural model analysis, with reference to patients without methylprednisolone therapy. Results During the study period, 2400 patients died. In-hospital mortality rates of iMV-free patients without or with methylprednisolone pulse therapy were 2.3% and 19.5%, and the corresponding values for iMV-receiving patients were 24.7% and 28.6%, respectively. The marginal structural model analysis showed that intravenous pulse therapy with methylprednisolone was associated with a lower risk of in-hospital mortality among patients receiving-iMV (HR 0.59; 95% CI 0.52–0.68). In contrast, pulse therapy with methylprednisolone increased the risk of in-hospital mortality among iMV-free patients (HR 3.38; 95% CI 3.02–3.79). The benefits of pulse therapy for iMV-receiving patients were greater than in those treated with intermediate/higher doses (40–250 mg intravenously) of methylprednisolone (HR 0.80; 95% CI 0.71–0.89). Conclusion The results of our study suggest that intravenous methylprednisolone showed dose–response efficiencies, and pulse therapy may benefit critically ill patients with acute COVID-19, such as those requiring iMV.

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]

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. [ABSTRACT FROM AUTHOR]

Background: Steroids are widely used to modulate the inflammatory reactions associated with coronavirus disease 2019 (COVID-19); however, the optimal upper limit dose of steroid use for acute COVID-19 care remains unclear and currently available data may suffer from a time-dependent bias of no effectiveness or reversed causation given the desperate situation of treatment during this pandemic. Accordingly, the aim of this study was to elucidate the impact of intravenous pulse therapy with methylprednisolone (500 mg or greater per day) on the risk of in-hospital mortality among patients with COVID-19 by controlling for time-dependent bias. Methods: We performed a prospective cohort study with 67,348 hospitalised acute COVID-19 patients at 438 hospitals during 2020–2021 in Japan. The impact of intravenous methylprednisolone pulse therapy on the risk of in-hospital mortality was examined based on hazard ratios (HRs) and 95% confidence intervals (95% CIs), with stratification according to the status of invasive mechanical ventilation (iMV). Time-dependent bias was controlled for in a marginal structural model analysis, with reference to patients without methylprednisolone therapy. Results: During the study period, 2400 patients died. In-hospital mortality rates of iMV-free patients without or with methylprednisolone pulse therapy were 2.3% and 19.5%, and the corresponding values for iMV-receiving patients were 24.7% and 28.6%, respectively. The marginal structural model analysis showed that intravenous pulse therapy with methylprednisolone was associated with a lower risk of in-hospital mortality among patients receiving-iMV (HR 0.59; 95% CI 0.52–0.68). In contrast, pulse therapy with methylprednisolone increased the risk of in-hospital mortality among iMV-free patients (HR 3.38; 95% CI 3.02–3.79). The benefits of pulse therapy for iMV-receiving patients were greater than in those treated with intermediate/higher doses (40–250 mg intravenously) of methylprednisolone (HR 0.80; 95% CI 0.71–0.89). Conclusion: The results of our study suggest that intravenous methylprednisolone showed dose–response efficiencies, and pulse therapy may benefit critically ill patients with acute COVID-19, such as those requiring iMV. [ABSTRACT FROM AUTHOR]

Abstract Background Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infected patients are at high risk for developing severe conditions if other comorbidities are present, such as advanced cancer. Although the regulation of immune response is thought to play an important role in the treatment of coronavirus disease 2019 (COVID-19), physicians often have difficulties in selecting the most appropriate treatment. Furthermore, the impact that interrupting breast cancer treatment due to a COVID-19 infection has on patient outcomes is still unknown. Herein we report a case of advanced breast cancer in a patient whose COVID-19 acute respiratory failure was successfully treated with minimal interruption to their anticancer therapy for recurrent breast cancer. Case presentation A 48-year-old woman developed carcinomatous pleurisy after curative surgery for breast cancer. One month after the initiation of targeted therapy with palbociclib and fulvestrant, the pleural effusion decreased, but soon after she developed a COVID-19 infection. Dexamethasone (8 mg/day) was administered due to a prolonged fever, but her respiratory symptoms got worse and pneumonia appeared on a computed tomography (CT) scan 7 days after hospitalization. Thus, steroid pulse therapy (methylprednisolone 1000 mg/day) was administered for 3 days. Her respiratory condition rapidly improved. Two weeks after hospital discharge, complete regression of pneumonia was confirmed on CT scan, and her targeted therapy was resumed at the same dose and strength. More than 6 months later, her metastatic disease remains stable while on the same treatment. Retrospective analysis of the patient's neutralizing antibodies found the neutralizing activity was low in the early stages of infection, but became high after recovery. This suggests the patient acquired an immunity to SARS-CoV-2 through the infection, despite having a mild myelosuppression due to treatment for recurrent breast cancer. Conclusions Steroid pulse therapy is available worldwide, and may have an important role in cancer patients who develop severe pneumonia from SARS-CoV-2, by enabling them to avoid any long-term disruption to anticancer therapy. Moreover, it might also be useful when antiviral therapies lose their efficacy due to mutations of the virus, such as the Omicron variant. A critical element in cases such as this one is that treatment decisions are made by a team of specialists, including pulmonologists.

Abstract Background Steroid pulse (SP) therapy is one of the immunosuppressive therapies for immunoglobulin A nephropathy (IgAN). Although there are various protocols of SP therapy in IgAN, the intermittent SP (ISP) and consecutive SP (CSP) protocols are prevalently performed in clinical settings. However, there is a lack of evidence of comparisons of the effects on IgAN between these two protocols. Methods A total of 189 patients with IgAN who had received SP therapy were included in this study. They were divided into two groups according to the SP protocols into the intermittent SP (ISP) or consecutive SP (CSP) group as follows: ISP; three-times SP therapy in alternate months, CSP; three-times SP therapy in three consecutive weeks. Kidney function, remission of urinary findings, and side effects of SP therapy were compared between the two groups. The observational period was 12 months after the initiation of SP therapy. Results There was no significant difference in kidney function between the two groups during the observational period. The remission rate of proteinuria and hematuria at 12 months also did not significantly differ between the two groups. Furthermore, even after the adjustment of clinical characteristics using propensity score matching, the remission rate of proteinuria and hematuria at 12 months was similar between the two groups. At 2 months, the remission rate of proteinuria was significantly higher in the CSP group than in the ISP group. There were no critical side effects in both groups. Conclusion The effects of SP therapy on IgAN were similar between the ISP and CSP group at 12 months although CSP therapy could remit proteinuria faster than ISP therapy.

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.

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.

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]

Background: High-dose steroid administration is a common initial therapeutic approach for Vogt-Koyanagi-Harada disease (VKH). Nonetheless, administering substantial doses of steroids to pregnant women necessitates meticulous consideration due to the potential impacts on the mother and fetus. We present a case wherein steroid pulse therapy was administered to a patient who developed VKH during the late stages of pregnancy., Case Summary: The patient was a 26-year-old nulliparous woman. At 33 weeks and 1 day of her pregnancy, she experienced a decline in visual acuity and noticed metamorphopsia in her left eye. Examination revealed bilateral serous retinal detachment, leading to VKH diagnosis. A collaborative effort involving the departments of ophthalmology, internal medicine, and neonatology was initiated. Steroid pulse therapy was administered at 34 weeks and 1 day of pregnancy under hospital supervision. Complications, such as threatened preterm labor and gestational diabetes, emerged, necessitating the initiation of oral ritodrine hydrochloride and insulin therapy. Then, serous retinal detachment was resolved, and visual acuity was restored. Labor pains initiated 32 days post-initiation of steroid pulse therapy (at 38 weeks and 4 days of gestation), culminating in a normal delivery. Mother and newborn experienced an uneventful puerperal course and were discharged from the hospital on the 5th day following delivery., Conclusion: VKH management in pregnancy requires multidisciplinary coordination, emphasizing collaboration with ophthalmologists and specialists in internal medicine and neonatology., Competing Interests: Conflict-of-interest statement: All authors declare that they have no conflict of interest to disclose., (©The Author(s) 2024. Published by Baishideng Publishing Group Inc. All rights reserved.)

Introduction: Acute respiratory distress syndrome (ARDS) is considered a poor prognostic factor for miliary tuberculosis (MTB), but little is known about the effectiveness of steroid pulse therapy for MTB complicated by ARDS. Patients and methods: Medical records were used to retrospectively investigate the prognosis and clinical information of 13 patients diagnosed with MTB complicated by ARDS among 68 patients diagnosed with MTB at our hospital between January 1994 and October 2016. None of the patients had multidrug resistant tuberculosis (TB). MTB was diagnosed by 1 radiologist and 2 respiratory physicians based on the observation of randomly distributed, uniformly sized diffuse bilateral nodules on chest computed tomography and the detection of mycobacterium TB from clinical specimens. ARDS was diagnosed based on the Berlin definition of ARDS. The effect of steroid pulse therapy on death within 3 months of hospitalization was examined using Cox proportional hazards models. Variables were selected by the stepwise method (variable reduction method). Results: Six of 8 patients with MTB complicated by ARDS were alive 3 months after hospitalization in the steroid pulse therapy group, whereas only 1 of 5 patients was alive in the non-steroid pulse therapy group. Analysis of factors related to the survival of patients with MTB complicated by ARDS revealed that steroid pulse therapy was the strong prognostic factor (hazard ratio = 0.136 (95 % CI: 0.023–0.815)). Conclusion: Our findings suggest that steroid pulse therapy improves the short-term prognosis of patients with MTB complicated by ARDS.

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]

Background: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infected patients are at high risk for developing severe conditions if other comorbidities are present, such as advanced cancer. Although the regulation of immune response is thought to play an important role in the treatment of coronavirus disease 2019 (COVID-19), physicians often have difficulties in selecting the most appropriate treatment. Furthermore, the impact that interrupting breast cancer treatment due to a COVID-19 infection has on patient outcomes is still unknown. Herein we report a case of advanced breast cancer in a patient whose COVID-19 acute respiratory failure was successfully treated with minimal interruption to their anticancer therapy for recurrent breast cancer. Case presentation: A 48-year-old woman developed carcinomatous pleurisy after curative surgery for breast cancer. One month after the initiation of targeted therapy with palbociclib and fulvestrant, the pleural effusion decreased, but soon after she developed a COVID-19 infection. Dexamethasone (8 mg/day) was administered due to a prolonged fever, but her respiratory symptoms got worse and pneumonia appeared on a computed tomography (CT) scan 7 days after hospitalization. Thus, steroid pulse therapy (methylprednisolone 1000 mg/day) was administered for 3 days. Her respiratory condition rapidly improved. Two weeks after hospital discharge, complete regression of pneumonia was confirmed on CT scan, and her targeted therapy was resumed at the same dose and strength. More than 6 months later, her metastatic disease remains stable while on the same treatment. Retrospective analysis of the patient's neutralizing antibodies found the neutralizing activity was low in the early stages of infection, but became high after recovery. This suggests the patient acquired an immunity to SARS-CoV-2 through the infection, despite having a mild myelosuppression due to treatment for recurrent breast cancer. Conclusions: Steroid pulse therapy is available worldwide, and may have an important role in cancer patients who develop severe pneumonia from SARS-CoV-2, by enabling them to avoid any long-term disruption to anticancer therapy. Moreover, it might also be useful when antiviral therapies lose their efficacy due to mutations of the virus, such as the Omicron variant. A critical element in cases such as this one is that treatment decisions are made by a team of specialists, including pulmonologists. [ABSTRACT FROM AUTHOR]

Abstract 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.

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]

Drug‐induced hypersensitivity syndrome/drug reaction with eosinophilia and systemic symptoms is a life‐threatening severe cutaneous adverse reaction, characterized by multiple organ involvement and reactivation of herpes viruses. Although the mainstay of treatment is a high dosage of corticosteroids delivered by pulse therapy or conventional oral administration, it remains debatable which mode is better. To clarify this issue, we reviewed publications in Japan of 299 cases of drug‐induced hypersensitivity syndrome/drug reaction with eosinophilia and systemic symptoms treated with corticosteroids, to evaluate safety concerns with regards to these two modes of treatment. As a result, we found that patients treated with pulse therapy more frequently suffered cytomegalovirus reactivation, persistency, and high mortality but less frequently experienced herpesvirus 6 reactivation or type 1 diabetes compared with those undergoing conventional treatment, suggesting that the administration mode may differentially modulate inflammatory responses toward distinct consequences. This is the first statistical analysis revealing that steroid pulse therapy frequently resulted in severe sequelae with high mortality. In terms of the risk of serious consequences, we consider that steroid pulse therapy should be eschewed for the treatment of drug‐induced hypersensitivity syndrome/drug reaction with eosinophilia and systemic symptoms. [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]

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 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.

Aim This study compared the clinical outcomes of critically ill patients with coronavirus disease (COVID‐19) pneumonia treated with high‐dose methylprednisolone and other steroids. Methods This retrospective observational study included critically ill COVID‐19 pneumonia adult patients with tracheal intubation treated between April 1, 2020, and September 15, 2021. Of the 46 patients who met the inclusion criteria, 36 received steroid pulse therapy (Group P) and 10 received steroids without pulse therapy (Group NP). Subgroup analyses in Group P by methylprednisolone dose of 1000 or 500 mg for 3 days during intensive care unit stay were carried out. The primary and secondary outcomes were 28‐day mortality and steroid‐associated complications, respectively. Results In the Kaplan–Meier curve analysis, there was no difference in the 28‐day survival between P and NP groups (log–rank P = 0.046). Univariate Cox proportional hazard model also showed that Group P had a decreased 28‐day mortality (hazard ratio 0.30; [95% confidence interval, 0.20–0.44]; P

Abstract 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.

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]

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.

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.

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]

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.

Although some prospective studies provided the evidence of corticosteroids for critically ill patients with COVID-19, the optimal dosage or timing of corticosteroids is still unknown. This is a case series of four patients on methyl-prednisolone pulses for the late phase of Coronavirus disease 2019 (COVID-19) with respiratory failure in our hospital. All patients needed invasive mechanical ventilation and had bimodal worseness of their respiratory status with consolidation and volume loss after intubation. All cases could successfully discontinue oxygen therapy without any severe adverse events after this pulse therapy in the late phase of COVID-19. This therapy is believed to be effective on some optimal patients. Hence, further studies to explore this efficacy and safety were needed.