Received Methylprednisolone Pulse Therapy Research Articles

Hypertrophic Pachymeningitis Associated with Myeloperoxidase-anti-neutrophil Cytoplasmic Antibodies Induced by Propylthiouracil: A Case Report.

A 71-year-old man with hyperthyroidism complained of headache lasting two months. He had been using propylthiouracil (PTU) for 14 years. Treatment intensification did not improve the symptoms. Blood tests detected a positive myeloperoxidase-anti-neutrophil cytoplasmic antibody (MPO-ANCA). Diffuse dural thickening was identified by magnetic resonance imaging. The patient was diagnosed with hypertrophic pachymeningitis (HP) due to ANCA-associated vasculitis (AAV). He received methylprednisolone pulse therapy followed by prednisolone and methotrexate, which improved his headache. PTU-induced AAV-related HP is a rare and indiscernible disease. Therefore, the possibility of the disease should be proactively considered when a PTU user experiences refractory headaches.

Systematic surveillance of immune-related adverse events in clinical practice and impact of subsequent steroid medication on survival outcomes.

Recent advances in immune-checkpoint inhibitors (ICIs) have highlighted the need for effective management of immune-related adverse events (irAEs). This study aimed to conduct a systematic surveillance of real-world development of irAEs for understanding their characteristics and examine the prognostic impact of steroid use for these events. We retrospectively investigated cancer patients treated with ICIs between 2014 and 2021 and collected information about irAEs throughout their development, management, and clinical outcomes. Overall, 458 patients (45.4%) developed 670 irAEs. The prevalence of irAEs varied by cancer type, but it was increased in regimens with longer treatment durations. Severe irAEs were more common in the nivolumab + ipilimumab and pembrolizumab + axitinib regimens. Patients who received steroids for irAEs at a dosage of < 2mg/kg had comparable prognosis to those who did not receive steroids; however, patients who received methylprednisolone pulse therapy, primarily for severe pneumonitis and hepatitis, had shorter overall survival than those who did not receive steroids (7.8 versus 23.4months, p = 0.016). Furthermore, methylprednisolone pulse therapy for irAEs was a poor prognostic factor in multivariate analysis (hazard ratio: 2.19, 95% confidence interval: 1.34-2.86, p < 0.001). Steroid treatment for irAE does not affect prognosis and should thus be used promptly to control inflammation. However, pulse therapy for severe cases is a poor prognostic factor, and early detection remains the key to managing such irAEs. The irAE characteristics in each regimen should be clarified to establish and provide more sophisticated irAE management, and the current findings will be beneficial to this goal.

Changing etiology of sympathetic ophthalmia: A 10-year study from a tertiary referral center in Taiwan

PURPOSE: In this study, we describe our experience of sympathetic ophthalmia (SO) at a tertiary referral center in Taiwan. MATERIALS AND METHODS: We retrospectively analyzed records of patients diagnosed with SO from January 2011 to December 2020. RESULTS: We collected data of 15 patients diagnosed with SO (eight males, seven females). Six patients developed SO after ocular penetrating trauma, and nine developed SO after ocular surgery, including seven with vitrectomy, one with penetrating keratoplasty, and one with cataract surgery. Penetrating ocular trauma was the primary cause of SO in the first 5 years (four of six cases), but the proportion was much lower in the last 5 years (two of nine cases). The interval between trigger events and SO ranged from 0.2 to 120 months. Nine patients received oral steroids alone, five patients received methylprednisolone pulse therapy followed by oral steroids, and immunosuppressants were added in four cases. Visual acuity of sympathetic eyes and exciting eyes improved after treatment. The initial visual acuity of sympathetic eyes in trauma related and ocular surgery related revealed no significant difference, but the final visual acuity of sympathetic eyes was better in ocular surgery-related cases than in the trauma-related cases (mean ± standard deviation, 1.01 ± 1.33 versus 0.49 ± 0.68 logarithm of the minimum angle of resolution, P = 0.021). CONCLUSIONS: With the increased number of eye surgeries performed in recent years, eye surgery has emerged as the dominant etiology of SO in our 10-year study. Early detection and proper treatment help to maintain vision in most of the SO patients.

Clinical manifestations, outcomes, and antibody profile of Brazilian adult patients with dermatomyositis: a single-center longitudinal study

BackgroundCurrently, only a few retrospective cohort or cross-sectional studies have described the general characteristics of Brazilian patients with classical dermatomyositis (DM). In contrast, we aimed to longitudinally assess a large sample of these patients, and several myositis autoantibodies.MethodsThis single-center longitudinal study included 91 Brazilian adults with defined DM (EULAR/ACR 2017) who underwent follow-up appointments in our tertiary center from 2012 to 2021. Myositis autoantibody analysis was performed using a commercial kit.ResultsThe mean age of the patients was 47.3 ± 15.4 years, with a predominance of female (67.0%) and White (81.3%) patients. As an initial treatment, 76.9% of the patients received methylprednisolone pulse therapy, 59.3% received intravenous immunoglobulin, and 54.9% received both drugs. The median follow-up duration was 44 months (interquartile 17–67) months. There were 92 severe episodes of infection, and neoplasms were identified in 20 patients (22.0%). Hypertension was identified in 46.2% of patients, whereas diabetes mellitus and myocardial infarction occurred in 19.8% and 4.4%, respectively. Nine patients died during the follow-up. At the last visit, one-third of the patients had disease activity, half had a complete clinical response, and one-fifth were in disease remission. In a univariate logistic regression, anti-aminoacyl-tRNA synthetase antibodies (n = 13) were associated with interstitial lung disease, “mechanic’s hands”, and anti-Ro-52, and had an inverse association with “V”-neck and “shawl” signs. Anti-MDA-5 (n = 10) were associated with male gender, digital ulcers, vasculitis, arthritis, anti-Ro-52, and active disease. Anti-Ro-52 (n = 26) were associated with “mechanics’ hands”, arthritis, interstitial lung disease, anti-tRNA synthetases, and anti-MDA-5. No association was found for anti-Mi-2 (n = 10).ConclusionsThis study shows the general profile of a significant sample of Brazilian patients with DM as well as the association of some antibodies with clinical and laboratory manifestations of this myositis.

Identifying risk and prognostic factors in polyarteritis nodosa patients with digital gangrene.

Many patients with polyarteritis nodosa (PAN) complicated by digital gangrene have poor outcomes and related research information is limited. Our aim is to identify the associated risk and prognostic factors in PAN patients with digital gangrene. We conducted a retrospective study of 148 PAN patients admitted to Peking Union Medical College Hospital from Octorber 2001 to December 2018. Forty-seven (31.8%) PAN patients had digital gangrene. The average age was 40.4 ± 17.9 years. The presence of digital gangrene was correlated with current smoking (P=.008, odds ratio [OR] 2.99, 95% CI, 1.33-6.73), eosinophil elevation (P=.003, OR 4.21, 95% CI, 1.62-10.91) and elevated leukocytes (P=.001, OR 4.26, 95% CI, 1.86-9.78). Thirty-two (68.1%) gangrene patients received methylprednisolone pulse therapy and all of these patients were treated with cyclophosphamide. Nine patients suffered irreversible organ injury and 2 died. Survival analysis showed higher serum C-reactive protein (CRP) was associated with poor prognosis in patients with gangrene (log-rank P=0.042 and generalized Wilcoxon P=.020). PAN patients with current smoking and eosinophil elevation were more prone to digital gangrene and a high serum CRP level predicted poor outcomes. The CRP level should be efficiently controlled to ensure a good prognosis.

Timing of Initiation of Methylprednisolone Pulse Therapy in Patients with COVID-19

High-dose methylprednisolone pulse therapy is widely used in patients with severe COVID-19. This therapy is known to have sufficient clinical effectiveness, but the optimal administration method is not known. In this study, we assessed the deterioration of oxygenation after methylprednisolone pulse therapy in patients with COVID-19 according to disease severity (oxygen requirement) at initiation of therapy. Ninety-nine patients with COVID-19 who received methylprednisolone pulse therapy at Saitama Medical University Hospital in Japan between October 2020 and October 2021 were retrospectively reviewed. Clinical outcomes were compared according to the fraction of inspired oxygen as a measure of disease severity at initiation of methylprednisolone pulse therapy. Based on the FIO2 level at initiation of methylprednisolone pulse therapy, patients were classified into an early treatment group (FIO2 ≤ 0.39; n = 21), a middle treatment group (FIO2 0.40–0.69; n = 38), and a late treatment group (FIO2 ≥ 0.70; n = 40). The frequency of administration of mechanical ventilation and the days of oxygen therapy in the middle group were lower than in the other groups. The frequency of adverse events was also lower in the middle group. Both late and early methylprednisolone pulse therapy may lead to further deterioration of COVID-19 and an increase in adverse events.

Identification of patients with COVID-19 who are optimal for methylprednisolone pulse therapy.

BackgroundCorticosteroids have been reported to reduce the mortality rates in patients with coronavirus disease 2019 (COVID-19). Additionally, the role of high-dose methylprednisolone pulse therapy in reducing mortality in critically ill patients has also been documented. The purpose of this study is to identify patients with COVID-19 who are suitable for methylprednisolone pulse therapy.MethodsThis was a retrospective study that included patients with COVID-19 receiving methylprednisolone pulse therapy (≥250 mg/day for 3 days) with subsequent tapering doses at our hospital between June 2020 and January 2021. We examined the differences in background clinical factors between the surviving group and the deceased group.ResultsOut of 156 patients who received steroid therapy, 17 received methylprednisolone pulse therapy. Ten patients recovered (surviving group) and seven patients died (deceased group). The median age of the surviving and deceased groups was 64.5 years (range, 57-85) and 79 years (73-90), respectively, with a significant difference (p=0.004). Five of the deceased patients (71%) had developed serious complications associated with the cause of death, including pneumothorax, pneumomediastinum, COVID-19-associated pulmonary aspergillosis, cytomegalovirus infection, and bacteremia. On the other hand, out of the 10 survivors, only one elderly person had cytomegalovirus infection and the rest recovered without complications.ConclusionAdministration of methylprednisolone pulse therapy with subsequent tapering may be an effective treatment in patients with COVID-19 up to the age of early 70s; however, severe complications may be seen in elderly patients.

Pulmonary angiosarcoma presenting with diffuse alveolar hemorrhage: a case report

This study aimed to analyze the diagnosis and treatment of one case of pulmonary angiosarcoma (PPA) retrospectively. The main manifestation of this female patient was cough, hemoptysis and dyspnea. Computed tomography (CT) of the chest revealed multiple small nodules and ground-glass patches in both lungs suggesting of diffuse alveolar hemorrhage (DAH). Laboratory examination revealed decreased hemoglobin and platelet counting, normal coagulation function. Results of rheumatic markers testing including antinuclear antibody (ANA), anti-extractable nuclear antigen antibody (ENA), vasculitis marker, and antiphospholipid antibody were negative. Tumor markers were negative. Sputum smear, sputum culture, and alveolar lavage fluid culture showed negative results. The bone marrow smear was essentially normal. The patient received methylprednisolone pulse therapy (250 mg daily × 5 days) and immunoglobin (20 d daily × 7 days) treatment, but her hemoptysis persisted. Bilateral pleural effusion drainage found a large amount of bloody effusion, but cytology of the pleural fluid showed negative results. The clinical symptoms, laboratory results, imaging findings, and pathological features of the patient were summarized, and problems in diagnosis and treatment were discussed. A thoracoscopic lung biopsy was performed and the diagnosis of PPA was confirmed by pathology and immunohistochemistry (IHC) staining. This case suggested that the possibility of PPA should be considered in patients with DAH, but with negative findings in routine examinations, lung biopsy is usually required.

Methylprednisolone Pulse Therapy or Additional IVIG for Patients with IVIG-Resistant Kawasaki Disease.

There have been no robust data from clinical trials to guide the clinician in the choice of therapeutic agents for the child with intravenous immunoglobulin (IVIG) resistance. The treatment regimen for IVIG-resistant patients varies between institutions, and the best option has not yet been established. Therefore, in this trial, a total of 955 patients with Kawasaki disease (KD) were selected and were initially treated with IVIG (2 g/kg), of whom 80 (8.38%) assessed as IVIG resistant were randomly divided into two groups: Group A received the second IVIG treatment (n = 40), and Group B received methylprednisolone pulse therapy (MPT, n = 40). The whole fever time, duration of fever after retreatment, hospital days, medical costs, readmission rate, and laboratory examination difference (△) were calculated. Coronary artery lesion (CAL) outcomes were followed up over two years. Patients in the MPT group had shorter fever after retreatment and lower medical costs; more rapid declines in C-reactive protein (CRP), neutrophils (N%), and platelet (PLT) levels; and more rapid rise in sodium. However, they also probably had a higher incidence of treatment failure and CALs than the additional IVIG treatment group in the long-term follow-up. Caution is still required in the use of MPT to treat IVIG-resistant KD.

Advantages and disadvantages of corticosteroid use for acute exacerbation of interstitial pneumonia after pulmonary resection.

Acute exacerbation of interstitial pneumonia (AE-IP) is the top cause of 30-day mortality in surgery for lung cancer patients. The general treatment for AE-IP is corticosteroid; however, there are some disadvantages of corticosteroid use after surgery. This study was conducted to report the clinical course of AE-IP after surgery and evaluate the effect of corticosteroid use. This retrospective study was performed on 337 patients with interstitial pneumonia who underwent surgical resection for lung cancer at our institute between 2009 and 2018. AE-IP were observed in 14 patients (4.2%) and their management and clinical outcome were investigated. All patients received methylprednisolone pulse therapy. Six patients (42.9%) became convalescent after pulse therapy and eight (57.1%) died within 90days after surgery due to lack of therapeutic efficacy. Oxygenation and ground-glass opacities of the survivors improved within 3days after starting pulse therapy. Patients who responded to the first pulse also responded to the second pulse. Four patients developed complications including two with bronchopulmonary fistulas that may be related to steroid treatment. Even if the corticosteroid was effective, all AE-IP patients died within 1year after surgery. Corticosteroid therapy is effective for AE-IP after surgery; however, it may lead to severe complications after surgery.

Recovery from hypoxemia and Hypercapnia following noninvasive pressure support ventilation in a patient with statin-associated necrotizing myopathy: a case report

BackgroundStatin-associated necrotizing myopathy (SANM) is a rare autoimmune disorder caused by administration of statins. SANM is characterized by weakness due to necrosis and regeneration of myofibers. Here we report the first case of SANM with acute respiratory failure treated with noninvasive pressure support ventilation in addition to immunosuppressants.Case presentationA 59-year-old woman who had been treated with 2.5 mg/day of rosuvastatin calcium for 5 years stopped taking the drug 4 months before admission to our hospital due to elevation of creatine kinase (CK). Withdrawal of rosuvastatin for 1 month did not decrease the level of CK, and she was admitted to our hospital due to the development of muscle weakness of her neck and bilateral upper extremities. Anti-3-hydroxy-3-methylglutaryl coenzyme A reductase antibodies were positive. Magnetic resonance imaging showed myositis, and muscle biopsy from the right biceps brachii muscle showed muscle fiber necrosis and regeneration without inflammatory cell infiltration, suggesting SANM. After the diagnosis, she received methylprednisolone pulse therapy (mPSL, 1 g/day × 3 days, twice) and subsequent oral prednisolone therapy (PSL, 30 mg/day for 1 month, 25 mg/day for 1 month and 22.5 mg/day for 1 month), leading to improvement of her muscle weakness. One month after the PSL tapering to 20 mg/day, her muscle weakness deteriorated with oxygen desaturation (SpO2: 93% at room air) due to hypoventilation caused by weakness of respiratory muscles. BIPAP was used for the management of acute respiratory failure in combination with IVIG (20 g/day × 5 days) followed by mPSL pulse therapy (1 g/day × 3 days), oral PSL (30 mg/day × 3 weeks, then tapered to 25 mg/day) and tacrolimus (3 mg/day). Twenty-seven days after the start of BIPAP, she was weaned from BIPAP with improvement of muscle weakness, hypoxemia and hypercapnia. After she achieved remission with improvement of muscle weakness and reduction of serum CK level to a normal level, the dose of oral prednisolone was gradually tapered to 12.5 mg/day without relapse for 3 months.ConclusionsOur report provides new insights into the role of immunosuppressants and biphasic positive airway pressure for induction of remission in patients with SANM.

Changes of immunoglobulins and CD series in patients with Graves′ orbitopathy treated with high-dose methylprednisolone

Objective To study the effect of high-dose methylprednisolone intravenous pulse therapy on immunoglobulins and CD series in patients with active moderate-to-severe Graves′ orbitopathy. Methods Twenty-seven patients with active moderate-to-severe Graves′ orbitopathy were enrolled in this study. All the patients received iv methylprednisolone pulse therapy for 12 weeks according to the 2016 European Thyroid Association/European Group on Graves′Orbitopathy(EUGOGO) Guidelines. Serum thyroidal autoantibodies, such as thyroid-stimulating hormone receptor antibody (TRAb), anti-thyroperoxidase antibody (TPOAb), and serum immunoglobulins, such as IgG, IgE, IgA, IgM were evaluated at the baseline, at the end of 4th and 12th week. Percentages of CD3+ T cells, CD4+ T cells, CD8+ T cells and CD19+ B cells, CD16+ or CD56+ NK cells were also evaluated at each time point. Results TRAb, TPOA and IgE, IgG, IgA were significantly decreased both after 4th week and after 12th week (all P 0.05). Conclusion High-dose methylprednisolone may exert an immunosuppressive effect not only by modifying humoral and cellular immune functions, but also by decreasing serum immunoglobulins. Key words: Graves′ orbitopathy; Methylprednisolone; Pulse therapy; Immunoglobulin; CD series

Clinical characteristics and relationship between myasthenia gravis and premature ovarian failure: report of two cases

Myasthenia gravis (MG) and premature ovarian failure (POF) are rare. MF and POF greatly affect patients’ health. Combined occurrence of MF and POF in young women can have serious consequences. We report two cases of MG with POF. Case 1 was a 20-year-old woman who presented with myasthenic crisis and menopause in September 2015 and November 2015, respectively. The patient’s estradiol and follicle-stimulating hormone levels were abnormal. She was administered plasmapheresis and methylprednisolone pulse therapy. She improved and was discharged with normal restoration of menstruation after 3 months. Case 2 was a 21-year-old woman who had right eyelid droop and double vision in June 2014. She presented with menstrual disorder and menopause in August 2014 and September 2014, respectively. Estradiol and follicle-stimulating hormone levels were abnormal. She underwent progesterone therapy. She was admitted to hospital again in March 2016 with a myasthenic crisis. She received methylprednisolone pulse therapy and underwent thymectomy, but menstruation was not restored. In conclusion, there is comorbidity of POF in MG, and there is a close relationship between these two diseases. MG may subsequently lead to development of POF, and timely immunotherapy for MG may normalize POF.

Chinese Systemic Lupus Erythematosus Treatment and Research Group (CSTAR) Registry XI: gender impact on long-term outcomes.

The objective of this paper is to assess the role of gender on survival rate and causes of death and organ damage in systemic lupus erythematosus (SLE) patients in China from 2009 to 2015. We conducted a multicenter cohort study to analyze the differences in outcome data between male and female SLE patients. A group of 1494 SLE patients who fulfilled the 1997 American College of Rheumatology SLE classification criteria were enrolled in the Chinese Systemic Lupus Erythematosus Treatment and Research Group (CSTAR) registry from April 2009 to February 2010. All enrolled patients were followed up at least once per year from 2009 to 2015. For patients who could not attend the outpatient clinic, follow-up was conducted by telephone interview. We collected demographic data, clinical manifestations and damage scores (System Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI)). Survival rates were evaluated using the Kaplan-Meier method. This study included 1352 women and 142 men. The five-year survival rates were 92.0% for men and 97.6% for women. The survival rates of males were significant lower than for females ( p = 0.019). Male patients received methylprednisolone pulse therapy and cyclophosphamide significantly more than female patients ( p = 0.010). During follow-up, 12 male patients and 66 female patients died. The most common cause of death was infection (41.7%) for men and active SLE disease (27.3%) for women. At the end of the study, the major accumulated organ damages included renal (8.5%) and musculoskeletal (7.7%), and nervous system (5.6%) for men and renal (8.8%) and musculoskeletal (6.7%) for women. There were no significant differences in SDI scores between the two groups at baseline and at the end of the study. Male SLE patients had lower survival rates than female patients. Male patients received more methylprednisolone pulse and cyclophosphamide therapy. The most common causes of death were infection for male patients and active SLE disease for female patients. The major accumulated organ damages were renal, musculoskeletal, and nervous system both for male and female SLE patients in China.

Metastatic Thymoma-Associated Myasthenia Gravis: Favorable Response to Steroid Pulse Therapy Plus Immunosuppressive Agent.

BackgroundOur study retrospectively reviewed the therapeutic effect of steroid pulse therapy in combination with an immunosuppressive agent in myasthenia gravis (MG) patients with metastatic thymoma.Material/MethodsMG patients with metastatic thymoma that underwent methylprednisolone pulse therapy plus cyclophosphamide were retrospectively analyzed. Patients initially received methylprednisolone pulse therapy followed by oral methylprednisolone. Cyclophosphamide was prescribed simultaneously at the beginning of treatment. Clinical outcomes, including therapeutic efficacy and adverse effects of MG and thymoma, were assessed.ResultsTwelve patients were recruited. According to histological classification, 4 cases were type B2 thymoma, 3 were type B3, 2 were type B1, and 1 was type AB. After combined treatment for 15 days, both the thymoma and MG responded dramatically to high-dose methylprednisolone plus cyclophosphamide. The symptoms of MG were improved in all patients, with marked improvement in 6 patients and basic remission in 4. Interestingly, complete remission of thymoma was achieved in 5 patients and partial remission in 7 patients. Myasthenic crisis was observed in 1 patient and was relieved after intubation and ventilation. Adverse reactions were observed in 7 patients (58.3%), most commonly infections, and all were resolved without discontinuation of therapy. During the follow-up, all patients were stabilized except for 1 with pleural metastasis who received further treatment and another 1 who died from myasthenic crisis.ConclusionsThe present study in a series of MG patients with metastatic thymoma indicated that steroid pulse therapy in combination with immunosuppressive agents was an effective and well-tolerated for treatment of both metastatic thymoma and MG. Glucocorticoid pulse therapy plus immunosuppressive agents should therefore be considered in MG patients with metastatic thymoma.

Children with Steroid-resistant Nephrotic Syndrome: Long-term Outcomes of Sequential Steroid Therapy

ObjectiveThis study aimed to investigate the long-term outcomes in children with steroid-resistant nephrotic syndrome (SRNS), who received methylprednisolone pulse therapy (MPT)-based sequential steroid therapy. In particular, we aimed to observe whether these patients had a high risk of adverse events. MethodsWe conducted a retrospective study over a 5-year period. The long-term outcomes for children with SRNS receiving sequential therapy were observed. ResultsSixty-three children were diagnosed with SRNS and underwent MPT-based sequential steroid therapy. Thirty-five (55.6%) achieved complete or partial remission, 19 (30.2%) of whom were in remission even after treatment cessation at last review. The mean time to initial remission after MPT was 24.3±13.1 days. Forty-nine children (77.8%) experienced relapses, of whom 31 (49.2%) demonstrated a frequent relapsing course. Adverse effects relevant to MPT were generally mild and infrequent. Five patients (7.9%) complained of vomiting or nausea during MPT infusion; 25 (39.7%) experienced excessive weight gain and developed an obvious Cushingoid appearance; and 26 (41.3%) had poor growth associated with long-term steroid use. Twenty-eight patients (44.4%) failed to respond to MPT, of whom 21 (33.3%) achieved complete or partial remission with immunosuppressive agents. ConclusionMPT-based sequential steroid therapy appears to be a safe and effective method for inducing rapid remission in childhood SRNS. Further clinical studies are needed to comprehensively evaluate this therapy.

Dermatomiositis y síndrome de Evans asociado a infección por HTLV-1

A 55-year-old female patient, born in Ayacucho, with a history of dermatomyositis for 3 years, who received irregular treatment with prednisone. Two months prior to admission, she presented with autoinmune hemolytic anemia and idiopathic thrombocytopenic purpura. The patient received methylprednisolone pulse therapy and packed red blood cells transfusions. Upon admission, she was drowsy, with a poor overall status, marked weight loss, dehydration, with presence of livedo reticularis in her lower extremities, and onychodystrophy and onycholysis on the toes of both feet. Western blot test was positive for human T-lymphotropic virus type 1 (HTLV-1). The patient evolved with recurrent hypoglycemia. Therefore, we report a case of dermatomyositis and Evans syndrome in the context of an HTLV-1 infection.

Retrospective study of primary IgA nephropathy with crescent formation and/or rapidly progressive glomerulonephritis in children

IgA nephropathy is the most common type of glomerulonephritis in the world. Its clinical and pathological manifestations vary. A few of the patients with IgA nephropathy present with rapidly progressive glomerulonephritis (RPGN) and/or crescent formation. Their conditions are serious and acute, but there are few reports on their characteristics, treatment and outcome. This study aimed to analyze the clinicalopathological features, treatment and prognosis of primary IgA nephropathy in children, to provide a reference for clinical diagnosis and treatment. A retrospective study was conducted in children with primary IgA nephropathy with crescent formation and/or rapidly progressive glomerulonephritis admitted to our department from 2000 to 2014. The patients meeting the inclusion and exclusion criteria were included. Patients were divided into RPGN group and non-RPGN group according to the clinical manifestations, crescent formation group and non-crescent group, crescentic IgA nephropathy group and non-crescentic IgA nephropathy group according to renal biopsy. Their clinical manifestations and pathological features, treatment and prognosis were compared. A total of 265 patients were recruited, 10 patients (3.8%) had RPGN, 151 patients (57.0%) had crescent formation, 19 cases (7.2%) showed crescentic IgA nephropathy.Compared with non-RPGN group, RPGN group showed more gross hematuria, higher serum creatinine, lower creatinine clearance correction at biopsy and follow-up, and more crescentic IgA nephropathy (P<0.05). The percent of patients who received methylprednisolone pulse and blood purification therapy in RPGN group is higher than that of non-RPGN group (P<0.05). Compared with non-crescent group, crescent formation group showed more gross hematuria at biopsy and follow-up, higher serum creatinine at biopsy, lower creatinine clearance correction, more 24-hour urinary protein at biopsy and higher serum creatinine at follow-up (P<0.05). The percentage of patients received more methylprednisolone pulse, oral steroids, cyclophosphamide pulse in crescent formation group was higher than that of non-crescent group (P<0.05). Compared with non-crescentic IgA nephropathy group, crescentic IgA nephropathy group showed more RPGN percent, higher serum creatinine, more 24-hour urinary protein at biopsy (P<0.05). The percentage of patients who received more methylprednisolone pulse and blood purification therapy in crescentic IgA nephropathy group was more than non-crescentic IgA nephropathy group (P<0.05). At follow-up, 20.0% of the patients with RPGN and crescent nephritis returned to normal renal function and the percent of crescent glomerulonephritis but not RPGN was 71.4%, RPGN but not crescent glomerulonephritis was 80.0%, crescent formation but not crescent nephritis was 87.5%. In primary IgA nephropathy with crescent formation and/or rapidly progressive glomerulonephritis, the patients with both RPGN and crescentic IgA nephropathy showed the worst clinical manifestations, its prognosis was worst while the patients with crescent formation showed the mildest clinical manifestations and best prognosis.

Abstract 130: Clinical Efficacy Of Modified RAISE Protocol In Acute Kawasaki Disease

Backgrounds and Objectives: RAISE protocol demonstrated that IVIG plus prednisolone (PSL) therapy reduced the incidence of coronary artery abnormalities (CAAs) in children with high risk of refractory Kawasaki disease (KD). However, this protocol has longer hospital stay because PSL is tapered over the next 15 days when CRP was under 5mg/L. We proposed modified RAISE protocol which PSL is tapered over 9 days. The aim of this study was to evaluate clinical utility of modified RAISE protocol. Methods: This is a retrospective study to evaluate clinical data from KD between May, 2012 and August, 2014. Composite outcomes included incidence of relapse and coronary arterial abnormalities. Using Kobayashi score, children were divided into high and low risk group. Results: We enrolled 36 KD patients with high risk group in total 147 patients with KD. Median of age was 33 months (range 2-83 months), 13 males. Median days of illness were 4.0 (range 2-7) and Kobayashi score was 6.0 (range 5-9). Overall, 31 children (86.1%) were responders, whereas 5 children were non-responders (resistance; 1 (2.8%) relapse; 4 (11.1%)). Among 5 non-responders, 4 patients received methylprednisolone pulse therapy (mPSL) and the remaining one patient administrated two times mPSL and infliximab. During follow-up (1-29 months), 3 children (8.3%) had CAAs (dilatation: 3 patients). Modified RAISE protocol was not inferior to conventional RAISE protocol (CAAs 3.3%, p=0.68, responder 84.3%, p=1.0, resistance 5.0%, p=0.92, relapse 10.7%, p= 0.66). Conclusions: Clinical safety and efficacy of modified RAISE protocol (3days х3) were similar to conventional RASIE protocol(5days х3).

Membranoproliferative glomerulonephritis and C3 glomerulonephritis: frequency, clinical features, and outcome in children.

C3 glomerulonephritis (C3GN) is a recently described disease that is related to membranoproliferative glomerulonephritis (MPGN). We retrospectively compared the frequencies, clinical characteristics, treatment modalities, and outcomes of C3GN and MPGN in a cohort of Japanese children. Children who were pathologically diagnosed with MPGN (type I or III) in our hospital were divided into two groups based on immunofluorescence imaging of renal biopsies: children with MPGN induced by classical complement pathway activation (classical MPGN) and children with C3GN. Of 14 children with MPGN (five boys), four had classical MPGN, eight had C3GN, and two had unclassifiable glomerulonephritis. Four children with classical MPGN and seven with C3GN received methylprednisolone pulse therapy followed by oral prednisolone for 2 years (MPT+PSL therapy). Subsequently, six of seven children with C3GN received combined therapy (prednisolone, azathioprine, and anticoagulants) for 2 years because they responded poorly to MPT+PSL therapy. At the last follow-up visit, two children with classical MPGN and seven with C3GN had not achieved remission. One child with classical MPGN and five with C3GN had hypocomplementaemia at the last follow-up. None of the children had renal impairment. More than half of the patients previously diagnosed with MPGN fulfilled the criteria for C3GN in children. C3GN may be more refractory than classical MPGN to immunosuppressant therapy.