Received Methylprednisolone Pulse Research Articles

A Prospective Single-Arm Cohort Study of Immune-mediated Seizures/Epilepsy Without Encephalopathy (ISEWE) in Children from a Tertiary Care Hospital in South India.

Acute-onset seizures in children pose a diagnostic and therapeutic dilemma. Some epilepsy cases presenting with seizures but without encephalopathy, though treatable with immunotherapy, are often missed due to lack of suspicion of immune mechanism in the context of absent encephalitis. A prospective study was conducted on premorbidly normal children with new-onset seizures occurring in clusters, with normal neuroimaging. Investigations included electroencephalogram (EEG), cerebrospinal fluid examination, and testing for autoantibodies. All cases received methylprednisolone pulse therapy along with antiseizure medications (ASMs), followed by monthly dexamethasone oral pulse therapy. Fifteen cases were enrolled (11 males, four females). Mean age at seizure onset was 4.6 years. Focal seizures were present in 13/15 (86.7%) cases. Magnetic resonance imaging of the brain was normal in all. EEG showed interictal epileptiform discharges in 12 cases (focal in nine) and ictal record in two cases. On monthly oral steroids, number of ASMs could be reduced. Eight cases never had a relapse, while seven did have it. Relapses were more common if more than three ASMs were tried and less if steroids were started within 30 days. Immune-mediated seizures/epilepsy without encephalopathy is a new, important, and treatable entity. Early diagnosis and institution of immunotherapy results in significant improvement in seizure control and also reduces the need for long-term polytherapy. Awareness of this entity is crucial, especially when premorbidly normal children present with new-onset clusters of refractory seizures.

COMPARATIVE EFFICACY OF METHYLPREDNISOLONE AND TOCILIZUMAB IN PATIENTS WITH A SEVERE FORM OF COVID-19

Background. Recently, we have again noted an increase in the incidence of COVID-19. The treatment of patients with severe coronavirus infection poses a significant medical challenge. Objectives. The purpose of this research was to compare the efficacy of standard therapy and pulses of methylprednisolone in combination with or without tocilizumab in patients with a severe form of COVID-19. Patients and methods. In a retrospective study, the medical charts of 220 patients with a severe course of COVID-19 were reviewed. Patients were divided into four groups: those on daily methylprednisolone at a dose of 32 mg enterally; patients who received methylprednisolone pulses (500 mg daily intravenously for three consecutive days, with a subsequent change to the 32 mg of methylprednisolone daily); patients who received a single dose of 400 mg tocilizumab in combination with a 32 mg of methylprednisolone daily; patients who received a single dose of 400 mg tocilizumab in combination with methyl­prednisolone pulse therapy. At the end of therapy, 28-day mortality and the number of intubations in each group one week after the end of therapy were analyzed. Results. Patients treated with a combination of tocilizumab and pulse methylprednisolone therapy had the lowest risk of death (p<0.001), OR=0.03 (95 % CI 0.01-0.16), compared to patients treated only with 32 mg of methylprednisolone. Conclusions. Methylprednisolone pulses therapy is more effective than therapy with methylprednisolone at a daily dose of 32 mg. The combination of methylprednisolone and tocilizumab is more effective than the isolated administration of methylprednisolone. The combination of tocilizumab with methylprednisolone pulse therapy had the highest therapeutic effect.

Tumefactive Demyelination Lesions: Report on Three Cases

Abstract Purpose: Tumefactive demyelination (TD) lesion and its subtype Balo’s concentric sclerosis (BCS), are rare manifestations of central nervous system demyelinating disease. Because of its rarity, physicians might hesitate in reaching a diagnosis or initiating steroid pulse therapy. This study aims at pinpointing the key neuroimaging features to distinguish TD lesions from surgical conditions, and illustrating the clinical outcomes of patients with TD lesions. Case report: Two of the three patients had solitary TD lesions, one 47-year-old man presenting with newly onset seizure and another 54-year-old women suffering from progressive hemiparesis. The male patient underwent craniotomy for mass excision without further steroid therapy, while the female patient received methylprednisolone pulse therapy only. Both patients remained free of clinical and radiological relapses over the past 6-7 years, leading to the diagnosis of clinically isolated syndrome. The third case is a 30-year-old woman with subacute onset of dysarthria and hemiparesis. She had two BCS lesions along with other demyelinating lesions in the juxtacortical and periventricular regions, cerebellar peduncles, and spinal cord, fulfilling dissemination in time and space. Her neurological deficits resolved after pulse therapy, and she received long-term disease modifying therapy for multiple sclerosis. Conclusion: This study underscores the diverse neuroimaging and clinical presentations of patients with TD lesions, and emphasizes the importance of clinical vigilance regarding this rare condition.

Anti-OJ antibody-positive anti-synthetase syndrome following SARS-CoV-2 infection: a case report and literature review

BackgroundCOVID-19 can induce a systemic inflammatory response with variable clinical manifestations. Similar to various viruses, COVID-19 has been implicated in the pathogenesis of autoimmune diseases. This article highlights the potential for infections including the SARS-CoV-2 virus to induce exacerbations of pre-existing autoimmune diseases or even potentially unmask de novo autoimmune diseases in particular anti-synthetase syndrome (ASSD) in predisposed individuals. Although there are other case reports of ASSD following SARS-CoV-2 infection, here we present the first reported case of a gentleman with a newly diagnosed anti-OJ positive anti-synthetase syndrome following SARS-CoV-2 infection.Case presentationDescribed is a case of a 70-year-old man presenting to the emergency department with worsening dyspnea in the context of a recent COVID-19 infection. CT-chest revealed changes suggestive of fibrotic lung disease, consistent with usual interstitial pneumonitis (UIP) pattern. Despite recovery from his COVID-19 illness, the patient subsequently developed proximal myopathy with cervical flexion weakness on further assessment with persistently elevated creatinine kinase (CK). Myositis autoantibodies found a strongly positive anti-OJ autoantibody with MRI-STIR and muscle biopsy performed to further confirm the diagnosis. The patient received pulse methylprednisolone 1 g for 3 days with a long oral prednisolone wean and in view of multiple end-organ manifestations, loading immunoglobulin at 2 g/kg administered over two days was given. In addition, he was then commenced and escalated to a full dose of azathioprine given a normal purine metabolism where he remains in clinical remission to this date. At least 267 cases of rheumatic diseases has been associated with SARS-CoV-2 infection as well as COVID-19 vaccination. A literature search on PubMed was made to determine the amount of case reports describing myositis associated with SARS-CoV-2 infection. We found 3 case reports that fit into our inclusion criteria. Further literature searches on diagnostic approach and treatment of ASSD were done.ConclusionAlthough SARS-CoV-2 infection itself can cause a directly mediated viral myositis, this case report highlights the possibility of developing virus-triggered inflammatory myositis through multiple aforementioned proposed mechanisms. Therefore, further studies are required to explore the relationship and pathophysiology of SARS-CoV-2 infection and the incidence of inflammatory myopathies.

P022 Unmasking the storm: hemophagocytic lymphohistiocytosis in systemic lupus erythematosus - a case series

Abstract Background/Aims Hemophagocytic lymphohistiocytosis (HLH), a critical illness of uncontrolled immunological activity and hyperinflammation, manifests as primary/familial or secondary form. Secondary HLH can be of multi-faceted etiology including infections, cancer, autoimmune and immunodeficiencies. HLH in context of systemic lupus erythematosus (SLE) is a rare entity and mirrors the clinical picture of SLE flare, adding to the diagnostic dilemma. Methods Our objective is to consider HLH as a key differential in unidentified autoimmune diseases. We present a trio of cases of undiagnosed SLE manifesting as HLH. Results A 13-year-old male presented with fever, rash, oral ulcers, and jaundice for a month with passage of dark coloured urine for last seven days. On examination, he had generalized lymphadenopathy, erythematous maculopapular rashes, palpable purpura, non-scarring alopecia, and hepatosplenomegaly. The labs revealed pancytopenia (Hb 9.8g/dL, WBC 2000/mm3, Platelets 23000/mm3), deranged creatinine (1.6mg/dL), transaminitis and hyperbilirubinemia, hyper-ferritinemia (2420µg/dL), hypertriglyceridemia (280mg/dL), hypofibrinogenemia (142mg/dL), and nephrotic range proteinuria (3.7g/24h). Diagnosis of SLE was confirmed by high titres of antinuclear antibody (ANA) and anti-double stranded DNA (dsDNA) and anti-Smith positivity. Renal biopsy showed class II lupus nephritis and acute tubular necrosis. Bone marrow biopsy revealed hemophagocytosis and the diagnosis of SLE complicated by HLH was made. A 16-year-old girl presented with fever and headache for three weeks, worsening over last ten days with irritable behaviour for one day, and respiratory distress that necessitated endotracheal intubation. The presence of pancytopenia (9.1/1940/41000), transamintis, hyper-ferritinemia (4040µg/dL), hypertriglyceridemia (250mg/dL), and low fibrinogen (133mg/dL) in laboratory evaluation raised a compelling suspicion of HLH confirmed by hemophagocytosis on marrow biopsy. Cerebrospinal fluid analysis was unremarkable, however MRI detected features of vasculitis with vasculitic infarcts. Direct Coomb’s test was positive. Autoimmune profiling unveiled positive results for ANA, anti-dsDNA, and anti-Smith antibodies consistent with SLE. A 21-year-old female presented with fever and arthralgias for two months. Investigations were suggestive of pancytopenia (7/1050/36000), raised ESR (126mm/hr) and CRP (54 mg/dL), elevated ferritin (4320 µg/dL), lactate dehydrogenase (1068U/L) and triglycerides (439mg/dL). The marrow biopsy was negative for hemophagocytosis while her soluble interleukin - 2 receptor levels were elevated (2740 U/ml). ANA and dsDNA were positive in significant titres, complement levels were low and Coombs test was positive, thereby confirming the primary diagnosis of SLE. All patients received methylprednisolone pulse along with cyclophosphamide in case 1 and IVIG in case 2 and 3, resulting in remarkable improvement. Conclusion With a prevalence of 0.9-4.6%, HLH secondary to SLE is an uncommon entity. The diagnosis of HLH related to SLE is challenging since the symptoms overlap, however findings of hyperferritinemia, hypofibrinogenemia, and hypertriglyceridemia favour HLH over SLE. Once HLH is proven, immunological testing for SLE is warranted to prevent diagnostic delay. Early treatment is needed to combat such a fulminant life-threatening illness. Disclosure R. Mishra: None. A. Rohatgi: None. S. Anuradha: None. N. Kumar: None. S. Singhla: None. S.T. Mathews: None. S. Kumar: None. P. Gautam: None.

Clinical characteristics of hypertrophic cranial pachymeningitis in granulomatosis with polyangiitis: a retrospective single-center study in China.

Hypertrophic cranial pachymeningitis (HCP) is uncommon but a poorly understood complication of granulomatosis with polyangiitis (GPA). We conducted this retrospective study to elucidate the clinical characteristics and factors independently associated with granulomatosis with polyangiitis (GPA) complicated by hypertrophic cranial pachymeningitis (HCP) in China. We collected the medical records of 78 patients diagnosed with GPA who were admitted to the inpatient department of Peking Union Medical College Hospital between January 2003 and September 2021. Clinical features, laboratory and radiological findings, and Birmingham Vasculitis Activity Scores (excluding meningitis score) were recorded. A binary logistic regression analysis was performed to analyze factors independently associated with GPA-related HCP. Headache (100%) and cranial nerve palsy (61.5%) were common manifestations of HCP. Compared to 52 GPA patients without HCP, 26 patients with HCP required more time from initial symptoms to diagnosis, with a lower ratio of pulmonary and renal involvement, a higher ratio of myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) positivity, conductive or sensorineural hearing loss, mastoiditis, and decreased vision or sudden visual loss. Binary logistic regression analysis indicated that proteinase 3-antineutrophil cytoplasmic antibody (PR3-ANCA) negativity (OR 10.698, p = 0.001), conductive or sensorineural hearing loss (OR 10.855, p = 0.005), and decreased vision or sudden visual loss (OR 8.647, p = 0.015) were significantly associated with GPA-related HCP. Of the 26 patients, 18 received methylprednisolone pulse treatment, and 18 received intrathecal injections of dexamethasone and methotrexate. HCP was a severe manifestation of GPA in our study. Independent factors associated with the occurrence of HCP in patients with GPA included PR3-ANCA negativity, conductive or sensorineural hearing loss, and decreased vision or sudden visual loss. Furthermore, GPA-related HCP was associated with higher disease activity, requiring more intensive treatments.

Clinical characteristics and prognosis of childhood-onset lupus mesenteric vasculitis as the initial presentation—a case–control study

BackgroundLupus mesenteric vasculitis (LMV) as initial presentation is rare, especially in childhood-onset systemic lupus erythematosus (cSLE). It is a critical complication of lupus. At present, the research on cSLE with LMV as the initial presentation is few. The aim of this study was to analyze the clinical characteristics and prognosis of cSLE with LMV in the Chinese population, compared with non-LMV cSLE.MethodsA retrospective case-controlled study was conducted on 55 cSLE patients between July 2018 and July 2021. The clinical data, laboratory findings, imaging, treatment, and follow-up data were collected and compared between the two groups of cSLE with LMV and non-LMV. Non-LMV cSLE patients were matched according to the age and sex of LMV patients.ResultsA total of 11 cSLE patients with LMV as the LMV group and 44 cSLE patients without LMV as the non-LMV group were included. The average age of onset was 12.55 ± 1.57 years old, the male-to-female ratio was 2:9, and high disease activity was observed in the LMV group. Abdominal pain was most common in LMV. Compared with the non-LMV, the percentage of abdominal pain, vomiting, abdominal distension, and diarrhea was higher, and gastrointestinal tract, serous cavity, kidney, and lung damage were higher in the LMV group (P < 0.05). In abdominal-enhanced CT, the percentage of intestinal wall thickening, peritoneal effusion, mesenteric vascular enhancement, hydronephrosis with ureteral dilatation, intestinal congestion, and gastric mucosa thickening in the LMV group were higher than those in the non-LMV group (P < 0.05). The percentage of receiving methylprednisolone pulse combined with cyclophosphamide pulse therapy in LMV was higher than in non-LMV. The clinical symptoms disappeared quickly, and there were no deaths in the LMV group. Compared with the non-LMV group, the 24-h urinary protein was higher, the complement C3 was lower, and the disease activity was higher in the LMV group (P < 0.05).ConclusionsLMV often occurs in 12 ~ 13-year-old girls with high disease activity of cSLE. Abdominal pain is the most common and more susceptible to damage to the kidney, serous cavity, and lung in cSLE with LMV. Methylprednisolone pulse combined with CTX pulse therapy is effective. After the treatment above, cSLE with LMV has a good prognosis, but the overall recovery is worse than non-LMV patients.

Hypertrophic Pachymeningitis Associated with Myeloperoxidase-anti-neutrophil Cytoplasmic Antibodies Induced by Propylthiouracil

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

Abstract 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.

5: PULSE METHYLPREDNISOLONE VERSUS DEXAMETHASONE IN COVID-19: A MULTICENTER COHORT STUDY

Introduction: Although high-dose corticosteroids can hypothetically curb the cytokine storm effectively, the clinical benefit of pulse methylprednisolone in coronavirus disease 2019 (COVID-19) remains inconclusive. We compared pulse methylprednisolone therapy with dexamethasone as a COVID-19 treatment. Methods: Using a Japanese multicenter database involving 350 acute care centers, we identified adults aged≥18 years admitted for COVID-19 and discharged between January 2020 and December 2021 who received pulse methylprednisolone (≥250 mg/day) or intravenous dexamethasone (≥6 mg/day) on the day of admission or the next day. One-to-one propensity score matching was performed with age, sex, comorbidities, disease severity, hospital size, and time of admission as covariates. The primary outcome was in-hospital mortality. Secondary outcomes were the length of hospital stay (LOS), insulin-requiring hyperglycemia, and fungal infection. Results: We included 1,202 (mean age, 62.4±16.3; male, 70.9%) and 7,669 (mean age, 61.6±16.3; male, 66.0%) patients in the pulse methylprednisolone and dexamethasone group, respectively. After propensity score matching (1,197 pairs), pulse methylprednisolone was associated with higher in-hospital mortality (12.0% vs 8.8%; p=0.011), longer LOS (13.0 [interquartile range: 9.0–22.0] vs 12.0 [8.0–18.0] days; p=0.002), and higher hyperglycemia incidence (16.3% vs 9.7%; p< 0.001), while fungal infection incidence (6.3% vs 4.6%; p=0.339) was not significantly different. In subgroup analysis, among patients who received mechanical ventilation (IMV) on the day of admission or the next day, in-hospital mortality was similar between the two groups (22.2% vs 20.5%; p=0.792). However, among patients without IMV, pulse methylprednisolone was associated with higher mortality (10.3% vs 7.0%; p=0.010). The sensitivity analysis involving patients who received ≥1 g/day of methylprednisolone vs 6 mg/day of dexamethasone showed consistent results. Conclusions: Compared to dexamethasone, pulse methylprednisolone may be associated with worse COVID-19 outcomes, especially in patients not on IMV. Providers should be aware of the potential consequences according to the type and dose of corticosteroid therapy and tailor the treatment for COVID-19.

Early Short-Term Use of Different Doses of Corticosteroid in Hospitalized Pediatric Patients with Coronavirus Disease 2019 Pneumonia

Abstract Objective Encouraged by reports of favorable outcomes following the use of corticosteroids in patients with moderate-to-severe coronavirus 2019 (COVID-19) pneumonia, we aimed to present our experience with early short-term corticosteroid use at our center in pediatric patients with COVID-19 pneumonia. Methods One hundred and twenty-nine pediatric patients were included in the study. Patients were divided into four groups according to the type and dose of corticosteroids given: Group 1 (those receiving dexamethasone 0.15 mg/kg/d); Group 2 (those receiving methylprednisolone 1 mg/kg/d); Group 3 (those receiving methylprednisolone 2 mg/kg/d); and Group 4 (those receiving pulse methylprednisolone 10–30 mg/kg/d). Results Of 129 patients, 19 (14.7%) patients were assigned to Group 1, 30 (23.3%) patients to Group 2, 30 (23.3%) patients to Group 3, and 50 (38.8%) patients to Group 4. Thirty-two (24.8%) patients were followed in the pediatric intensive care unit (PICU), of whom 13 (10%) required mechanical ventilation, and 7 (%5.4) died. In Group 4, the hospitalization length was significantly longer than in other groups (p &lt; 0.001, p &lt; 0.001). No significant difference was found among the groups in terms of mortality (p = 0.15). The most common comorbidity was obesity (33%). A significant association was found between the presence of comorbidity and mortality (p &lt; 0.001). All patients who died had an underlying disease. Cerebral palsy was the most common underlying disease among the patients who died. Worsening of lymphopenia was significant in patients with severe COVID-19 pneumonia at the time of transfer to the PICU (p = 0.011). Conclusion Although children usually have a milder course of COVID-19 than adults, underlying diseases and obesity increase the severity of disease manifestations also in children. Further studies are needed to define the exact role of corticosteroids in COVID-19 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.

Factors Associated with Pulse Methylprednisolone Treatment Failure in COVID-19-Related Multisystem Inflammatory Syndrome in Children (MIS-C)

Abstract Objective This article determines the occurrence and variables associated with pulse methylprednisolone treatment failure in children with coronavirus disease 2019 (COVID-19)-related multisystem inflammatory syndrome in children (MIS-C). Methods This prospective observational study was undertaken at a tertiary care teaching hospital in Kerala, India. Children admitted with COVID-19-related MIS-C who were treated with pulse methylprednisolone as first-line therapy were included in the study. Depending on the response to the treatment, they were divided into two groups. The clinical, laboratory parameters, and follow-up findings at 3 months were compared between the two groups Results Seventy-six patients were admitted with MIS-C during the study period. Sixty received pulse methylprednisolone as the first-line therapy. Of the 60 patients who received pulse methylprednisolone, 50 responded to treatment, while 10 required repeat immunomodulation. Need for noninvasive or invasive ventilation (relative risk [RR]: 13.14, 95% confidence interval [CI]: 3.147–54.88), six or more organ involvement (RR: 4.667, 95% CI: 1.349–16.149), thrombocytopenia (RR: 6.43, 95% CI: 0.87–47.6, p 0.003), and abnormal chest X-ray findings at admission (RR: 4.5, 95% CI: 1.46–13.8), were found to be associated with increased risk of treatment failure with pulse methylprednisolone therapy. Note that 88% of patients with coronary artery involvement showed resolution at 3-month follow-up. Conclusion More than 80% of children with MIS-C can be treated successfully with corticosteroids. The need for ventilator support, abnormal chest X-ray findings, and thrombocytopenia at admission were found to be factors associated with pulse methylprednisolone treatment failure.

A clinical study of mycophenolate mofetil combined with low-dose steroids in the management of pediatric systemic lupus erythematosus

Purpose: To investigate the efficacy and safety of mycophenolate mofetil (MMF) combined with lowdose steroids in the treatment of pediatric systemic lupus erythematosus (pSLE).Methods: A total of 76 children were diagnosed and admitted with SLE, lupus nephritis (LN) and type IV diffuse proliferative glomerulonephritis at Hainan Women and Children’s Medical Center, Haikou, China from March 2017 to December 2018, had their clinical data analyzed retrospectively. Among them, 38 children received methylprednisolone pulse combined with MMF and intermittent oral low-dose glucocorticoids (GC), labelled MMF group, while the remaining 38 children, which served as control group, were treated with oral GC and transitional reduction. Pertinent biochemical parameters were evaluatedResults: Compared with control group, MMF group showed a notably lower level of erythrocyte sedimentation rate (ESR), a higher level of complement C3, lower level of serum creatinine (Scr) and 24-h urine protein level 6 months after treatment (p &lt; 0.05). Albumin level was higher in MMF group at 6 months and 12 months after treatment than in the control group. Compared to control group, the SLEDAI score in MMF group was significantly lower at 6 months and 12 months after treatment (p &lt; 0.05). Body mass index, triglyceride, fasting blood glucose and intraocular pressure levels in the MMF group were significantly lower than those in the control group (p &lt; 0.05). Post-treatment, peripheral blood CD3+ and CD4+ T lymphocytes, CD4+/CD8+ ratio and NK cell levels in the two groups were significantly increased, while CD8+ T lymphocyte level declined.Conclusion: MMF combined with low-dose methylprednisolone controls symptoms early and mitigates renal injury in the treatment of pSLE. It is also safe, and effectively regulates the patient's cellular immune function.

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.

Treatment with 3-day methylprednisolone pulses in severe cases of COVID-19 compared with the standard regimen protocol of dexamethasone

Since the outbreak of COVID-19, research has been focused on establishing effective treatments, especially for patients with severe pneumonia and hyperinflammation. The role and dose of corticosteroids remain obscure. We...

A video-based discussion of movement disorders in paediatric anti NMDAR encephalitis: A case series from Eastern India.

The spectrum of movement disorders associated with anti N-Methyl-d-Aspartate-Receptor (NMDAR) encephalitis is myriad, particularly in children, possibilities of which were investigated from two tertiary care centres. A retrospective study was conducted in two tertiary referral centres in Eastern India, analysing data of 8 paediatric patients diagnosed as anti NMDAR encephalitis, presenting with one or more movement disorders (MDs). All the patients were of Bengali ethnicity with a median age of 9 years (3-16 years) and with female predilection (62.5%). CSF pleocytosis was a common feature in all. Seizures were described in 62.5%% of patients with a solitary patient exhibiting abnormalities on brain imaging. 3 out of 8 (37.5%) of patients presented with a single MD while the remaining had more than one type. Oro-linguo-facial dyskinesias and dystonia (37.5% each) were the most common movement type followed by chorea (12.5%). Complex stereotypies, myoclonus and facial tics were noted in one patient each. All patients received pulse methyl prednisolone. Escalation to second line therapy in form of rituximab was done for 5 patients (62.5%). Following immunotherapy, hyperkinetic movements resolved in 50% of patients, with persistence of movements in one (12.5%). A mortality of 37.5% was noted. Median duration of follow up was 26 months, during which none of the patients had evidence of systemic neoplasm. MDs are a core feature of anti NMDAR encephalitis, particularly in the paediatric age group, understanding and characterization of which, is the key to early diagnosis and effective therapy.

Spontaneous subcapsular renal hematoma in a patient with pauci-immune glomerulonephritis

Spontaneous subcapsular renal hematoma is a rare occurrence. We report a 42-year-old male with rapidly progressive glomerulonephritis who developed sudden-onset right flank pain. Imaging showed a large subcapsular collection in the right kidney, for which urgent nephrectomy was done due to hemodynamic instability. Histopathology from the nephrectomy specimen was suggestive of pauci-immune glomerulonephritis with crescents. The patient received pulse methylprednisolone followed by injectable cyclophosphamide.