Intravenous Methylprednisolone Pulse Research Articles

A Rare Case of Anti-N-Methyl-D-Aspartate Receptor Encephalitis in an Infant Presenting with Regression and Movement Disorder.

A Rare Case of Anti-N-Methyl-D-Aspartate Receptor Encephalitis in an Infant Presenting with Regression and Movement Disorder.

Posterior reversible encephalopathic syndrome in systemic lupus erythematous: A case report

Posterior reversible encephalopathy syndrome (PRES) implies to a reversible subcortical vasogenic brain edema, which leads to some acute neurological symptoms. PRES occurs usually in the setting of renal failure, blood pressure fluctuations, cytotoxic drugs, autoimmune disorders, and pre-eclampsia or eclampsia.This is a 40 year old female patient with history of systemic lupus erythematous (SLE) from twenty years ago, admitted to our neurology ward due to severe and refractory headache with multiple convulsive events.Brain MRI showed vasogenic brain edema in occipito-parieto-frontal lobes white matter compatible with PRES. Treatment is omitting of the etiology, but in these patients, discontinuation of immunosuppressive drugs is not possible and probably intravenous methylprednisolone pulse is a good treatment option especially in patient without response to aggressive control of blood pressure.

1125. Effect of intravenous pulses of methylprednisolone 250 mg versus dexamethasone 6 mg in hospitalized adults with severe COVID-19 pneumonia: an open-label randomized trial.

Abstract Background Pulse glucocorticoid therapy is used in COVID-19 infection. We evaluated the effectiveness of methylprednisolone 250 mg/d for 3 days vs. dexamethasone 6 mg/d for 10 days in patients with severe but not critical COVID-19 pneumonia. Methods A multicentre, randomized, open-label, controlled trial was conducted between February 2021 and August 2021 at 4 hospitals in Spain and included 128 hospitalized adults with confirmed COVID-19 pneumonia needing oxygen therapy but not critically ill. Patients were randomly assigned in a 1:1 ratio to receive dexamethasone 6 once daily for 10 days or methylprednisolone 250 mg once daily for 3 days. The primary outcome was 28-day mortality. Results Of the 128 randomized patients, 125 were analysed (mean age 60 ± 17 years; 82 males [66%]). Mortality at 28 days was 4.8% in the 250 mg methylprednisolone group vs. 4.8 % in the 6 mg dexamethasone group (absolute risk difference, 0.1% [95% CI, -8.8 to 9.1%]; P=0.98). The post-hoc added composite outcome of mortality at 90 days or intubation was 15.9% in the 250 mg methylprednisolone group vs. 15% in the 6 mg dexamethasone group (absolute risk difference, -0.9% [95% CI, -13.8 to 12.3%];P=0.83). Hyperglycaemia was more frequent in the methylprednisolone group, at 27.0 vs. 8.1 % (absolute risk difference, -18.9% [95% CI, -31.8 to - 5.6%]; P=0.007). Conclusion Among severe but not critical patients with COVID-19, 250 mg/d for 3 days of methylprednisolone compared with 6 mg/d for 10 days of dexamethasone did not result in a decrease in mortality or intubation. Disclosures Carlos Dueñas Gutierrez, MD, ViIV, GILEAD, Jannsen, MSD: Lecture fees.

Melanocyte-specific CD49a+ CD8+ T cells in vitiligo lesion potentiate to maintain activity during systemic steroid therapy.

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.

ODP531 Worsening of pretibial myxedema following repeated doses of radioiodine treatment in a patient with hyperthyroid Graves’ disease

Abstract Introduction The development of new-onset pretibial myxedema following radioiodine (RAI) treatment has been occasionally reported from the probable influences of thyrotropin receptor antibodies (TRAbs) similar to the RAI-associated Graves’ ophthalmopathy (GO). However, the worsening of pre-existing pretibial myxedema after RAI treatment has never been reported. Herein, we report an interesting case of fluctuating clinical course of pretibial myxedema following treatments of hyperthyroid Graves’ disease for over 3 years. Case Report A 49-year-old Thai woman with underlying hyperthyroid Graves’ disease with GO and pretibial myxedema for 1 year came to our hospital for relapsed hyperthyroidism and active, moderate-to-severe GO in 2019. A plague form of pretibial myxedema with indurated hyperpigmented lesion was found on both legs. Intravenous pulse methylprednisolone (IVMP) was given to control her active GO. Both GO and pretibial myxedema significantly improved after IVMP treatments. Then, RAI (30 mCi) was administered at 1 month later as a definitive treatment. Low dose of oral prednisolone (20 mg/ day) was also given before and after RAI treatment. Transient hypothyroidism developed at 4 months post-RAI and the estimated weights of the thyroid decreased from 60 grams to 30 grams. However, hyperthyroidism relapsed again within 6 months post-RAI treatment. At that time, her pretibial myxedema flared up slightly but her GO was still inactive. Eventually, a second dose of RAI (20 mCi) was given at 11 months apart from the first RAI administration. Low dose of oral prednisolone was also given. She developed post-radiation thyroiditis at 4 months after RAI with worsening of pretibial myxedema and mild exacerbation of GO. Topical corticosteroid ointment was given for pretibial myxedema and only local supportive measures was applied to treat GO. Finally, permanent hypothyroidism was achieved at 6 months after the second course of RAI. Currently, her pretibial myxedema is stable without further flareup. Conclusion Our case highlights the potential of RAI treatment to precipitate both GO and pretibial myxedema in patients with pre-existing skin lesions. Low dose prednisolone prophylaxis might not ensure preventative effect in exacerbation. Whether the risk of worsening of pretibial myxedema is higher in patients with persistent hyperthyroidism requiring a second dose of RAI is a question that warrants further studies. Presentation: No date and time listed

Juvenile dermatomyositis associated with autoantibodies to small ubiquitin-like modifier activating enzyme: a report of 4 cases from North India and a review of literature.

Juvenile dermatomyositis (JDM) is the commonest inflammatory myositis in children. The clinical phenotype is often characterized by the presence of myositis-specific antibodies (MSA). Antibodies to small ubiquitin-like modifier activating enzyme (SAE) are amongst the rarest MSA reported in children. A review of medical records of all patients diagnosed to have JDM during the period January 1992-April 2022 in our institute was done. Case records of children with JDM who had significant positivity for anti-SAE antibody by myositis immunoblot were analysed in detail. Of the 140 children with JDM, MSA immunoblot was carried out in 53 patients-4 (7.5%) amongst these had significant positivity for anti-SAE antibodies. Median age of onset of symptoms was 5.5years (range: 5-11years). Clinical features at presentation included fever, photosensitivity, heliotrope rash, and Gottron papules. Clinically significant proximal muscle weakness was noted in 3/4 patients; 1 had no discernible weakness but had radiological evidence of myositis. None of the 4 patients had evidence of interstitial lung disease or calcinosis. All patients were treated with intravenous pulse methylprednisolone: subcutaneous weekly methotrexate and hydroxychloroquine. One patient received mycophenolate mofetil because of a relapse of muscle disease, while none received cyclophosphamide or biologics. Median follow-up duration was 21.5months (range: 6-39months). Anti-SAE antibodies were found in 4/53 (7.5%) of North Indian children with JDM. All 4 patients had predominant cutaneous manifestations followed by muscle disease. Response to treatment was brisk and sustained. None had developed calcinosis in the follow-up. 1. We report high prevalence of anti-SAE antibody positivity (7.5%) in North Indian cohort of JDM. 2. Cutaneous disease precedes muscle weakness in children with anti-SAE positive JDM. 3. No evidence of interstitial lung disease/calcinosis was seen in these children.

Case report long segment myelitis secondary to neuro melioidosis

BackgroundNeuro-melioidosis, comprising 4% of all cases of melioidosis carries a risk of high morbidity and mortality. We describe two Sri Lankan patients presenting with long segment myelitis secondary to melioidosis.Case presentationCase 1: 47-year-old male presented with right side hemiparesis which progressed rapidly to quadriparesis. Initial cerebro spinal fluid (CSF) analysis revealed protein 76 mg/dl and glucose 72 mg/dl but without a cellular reaction. MRI spine revealed long segment myelitis with contrast enhancement. The patient was treated with intravenous methyl prednisolone pulses (IV MPP) and plasma exchanges(PLEX) on suspicion of an immune mediated myelitis but without success. A repeat MRI revealed high signal changes in the brain stem and along the entire spinal cord with contrast enhancement. MRI brain after treatment with MPP/PLEX showed enhancing hyper intensities along the corticospinal tracts. The repeat CSF revealed protein 1187 mg/dl, glucose 78 mg/dl, lymphocytes 1600/mm3 and neutrophils 10,200/mm3. CSF culture has become positive for Burkholderia pseudomallei. Serum melioidosis antibody titre was 1: 320. He was started on IV meropenem with oral cotrimoxazole for 12 weeks followed by oral co trimoxazole. But he had poor clinical recovery.Case 2: 47-year-old female presented with bilateral lower limb weakness for 1-week duration. On examination, she had flaccid paraparesis with a sensory level at T11. Inflammatory markers were elevated. CSF analysis revealed protein 50 mg/dl with lymphocytes 172/mm3. MRI pan spine revealed a long segment myelitis. Serum melioidosis antibody titre was 1: 640. She was treated with IV meropenem for 8 weeks followed by oral co-trimoxazole with an excellent clinical and radiological response.ConclusionNumerous neurological manifestations have been described with melioidosis, however long segment myelitis with a positive CSF culture is not yet reported. These cases signify the importance of considering melioidosis as a differential in patients with long segment myelitis especially in endemic areas.

Effect of intravenous pulses of methylprednisolone 250 mg versus dexamethasone 6mg in hospitalised adults with severe COVID-19 pneumonia: An open-label randomised trial.

BackgroundThe efficacy and safety of high vs. medium doses of glucocorticoids for the treatment of COVID‐19 patients has shown mixed outcomes in controlled trials and observational studies. We aimed to evaluate the effectiveness of methylprednisolone 250 mg bolus vs. dexamethasone 6 mg in patients with severe COVID‐19.MethodsA randomized, open‐label, controlled trial was conducted between February and August 2021 at four hospitals in Spain. The trial was suspended after the first interim analysis since the investigators considered that continuing the trial would be futile. Patients were randomly assigned in a 1:1 ratio to receive dexamethasone 6 mg once daily for up to 10 days or methylprednisolone 250 mg once daily for 3 days.ResultsOf the 128 randomized patients, 125 were analysed (mean age 60 ± 17 years, 82 males [66%]). Mortality at 28 days was 4.8% in the 250 mg methylprednisolone group vs. 4.8 % in the 6 mg dexamethasone group (absolute risk difference, 0.1% [95% CI, ‐8.8 to 9.1%], P=0.98). None of the secondary outcomes (admission to the intensive care unit, non‐invasive respiratory or high‐flow oxygen support, additional immunosuppressive drugs, or length of stay) or prespecified sensitivity analyses were statistically significant. Hyperglycaemia was more frequent in the methylprednisolone group at 27.0 vs. 8.1 % (absolute risk difference, ‐18.9% [95% CI, ‐31.8 to ‐ 5.6%], P=0.007).ConclusionsAmong severe but not critical patients with COVID‐19, 250 mg/d for 3 days of methylprednisolone compared with 6 mg/d for 10 days of dexamethasone did not result in a decrease in mortality or intubation.

Outcome of Pediatric Anti-N-Methyl-D-Aspartate (NMDA) Receptor Encephalitis in Rural Area of Thailand

AbstractAnti-N-methyl-d-aspartate receptor (NMDAR) encephalitis has a very good outcome; however, there are few reports in developing countries regarding the outcome of pediatric anti-NMDAR encephalitis. We aimed to report the clinical outcome of pediatric anti-NMDAR encephalitis in the rural area of Thailand. This retrospective cohort study recruited children between the age of 1 month and 15 years with the diagnosis of anti-NMDAR encephalitis admitted at Maharat Nakhon Ratchasima Hospital from 1st May 2015 to 31th March 2020. Baseline characteristics and investigation were recorded. The first-line treatment was intravenous pulse methylprednisolone alone, pulse methylprednisolone plus intravenous immune globulin (IVIG), or IVIG alone. The second-line treatment was monthly intravenous cyclophosphamide or azathioprine. The modified Rankin Scale (MRS) was used for evaluation at 1, 3, 6, 12, 18, and 24 months after receiving first-line treatment to determine clinical outcomes. The factors that may affect the outcomes were evaluated. In total, 17 patients were recruited, with the mean age of 8 years, and 76.46% were female. 82.35% of patients developed seizures, and 52.94% turned to status epilepticus. 70.59% received first-line treatment as pulse methylprednisolone plus IVIG. 82.35% received complete follow-up at 12 months. According to MRS score, at first diagnosis 94.12% reported severe disability, at 1 month after receiving treatment, 47.06% improved to moderate–mild disability, and at 6 and 12 months, 40% and 78.57% had complete recovery, respectively. Different types of first-line and second-line treatments did not affect the clinical outcome. Comorbidity of status epilepticus affected MRS. Anti-NMDAR encephalitis has a very good prognosis; prompt diagnosis is necessary. Comorbidity as status epilepticus may affect the clinical outcome.

A case of retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations: Long‐term treatment with repeated courses of immunotherapy

AbstractWe 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.

Efficacy and Safety of Belimumab in Patients With Lupus Nephritis: Subgroup Analyses of a Phase 3 Randomized Trial in the East Asian Population

Belimumab improved kidney outcomes in patients with active lupus nephritis (LN) in BLISS-LN, leading to its approval in the United States and the European Union. As data on treatment of East Asian patients with LN are limited, we evaluated the efficacy and safety of belimumab in the BLISS-LN East Asian subgroup. Prespecified subgroup analysis of BLISS-LN, a phase 3, placebo-controlled, randomized 104-week trial. Adults with biopsy-proven, active LN were randomized (1:1) to belimumab or placebo, plus standard therapy. Patients were administered intravenous belimumab 10mg/kg, or placebo, plus standard therapy (oral glucocorticoids and either cyclophosphamide for induction followed by azathioprine for maintenance, or mycophenolate mofetil for both induction and maintenance). At the investigator's discretion, 1-3 intravenous pulses of methylprednisolone, 500-1,000mg each, could be administered during induction. The primary end point was primary efficacy renal response (PERR; ie, urinary protein-creatinine ratio≤0.7g/g, estimated glomerular filtration rate no more than 20% below preflare value or≥60mL/min/1.73m2, and no treatment failure) at week 104. Key secondary end points included complete renal response (CRR; urinary protein-creatinine ratio<0.5g/g, estimated glomerular filtration rate no more than 10% below preflare value or≥90mL/min/1.73m2, and no treatment failure) at week 104; PERR at week 52; time to kidney-related event or death; and safety. PERR and CRR were analyzed using a logistic regression model, and time to a kidney-related event or death was analyzed using a Cox proportional hazards regression model. 142 patients from mainland China, Hong Kong, South Korea, and Taiwan were included (belimumab, n=74; placebo, n=68). At week 104, more belimumab than placebo patients achieved PERR (53% vs 37%; OR, 1.76 [95% CI, 0.88-3.51]) and CRR (35% vs 25%; OR, 1.73 [95% CI, 0.80-3.74]). At week 52, more belimumab than placebo patients achieved PERR (62% vs 37%; OR, 2.74 [95% CI, 1.33-5.64]). Belimumab reduced the risk of a kidney-related event or death compared with placebo at any time (HR, 0.37 [95% CI, 0.15-0.91]). Safety was similar across treatment groups. Small sample size and lack of formal significance testing. Safety and efficacy profiles were consistent with BLISS-LN overall population, supporting benefits of belimumab treatment in the East Asian subgroup with LN. This study was funded by GSK (GSK study no. BEL114054). Registered at ClinicalTrials.gov with study number NCT01639339.

OP-22 - Efficacy and immune-related adverse events of ipilimumab and nivolumab combination therapy for a vaginal malignant melanoma

Vaginal malignant melanoma is a rare and aggressive tumor, and the standard treatment has not been established. Recently, immune checkpoint inhibitors via PD-1 and CTLA-4 have caused breakthroughs of standard therapy for cutaneous malignant melanoma. However, there are still limited reports of immune checkpoint inhibitors for vaginal malignant melanoma. 50-years-old woman, G1P1. She visited our hospital to evaluate abnormal genital bleeding for several months and vaginal tumors. Her pelvic examination and pelvic MRI showed a 4 cm blackish, polypoid tumor in the vagina. Histological examination of biopsy diagnosed malignant melanoma. Contrast-enhanced CT showed pelvic lymph node metastasis. Finally, she was diagnosed with vaginal malignant melanoma, cT1N1M0. The BRAF gene was wild type. She started ipilimumab (3 mg/kg, tri-weekly) plus nivolumab (1 mg/kg, tri-weekly) combination therapy. At the end of the 3rd course, autoimmune hepatitis (grade 4) and thyroiditis were observed. They were considered immune-related adverse events. Intravenous methylprednisolone pulse and Mycophenolate Mofetil slowly improved liver function. The 4 cm-sized vaginal tumor shrank to under 1 cm, and the enlarged lymph nodes disappeared. Currently, we are considering surgical intervention and/or maintenance chemotherapy with nivolumab alone. We experienced a combination therapy of ipilimumab + nivolumab for vaginal malignant melanoma. While the combination therapy was highly effective, severe immune-related adverse events were observed. There are also reports that cases with immune-related adverse events have a high response rate. It was considered necessary to accumulate experience of immune checkpoint inhibitors for vaginal malignant melanoma in the future.

Efficacy of pulse intravenous methylprednisolone in epileptic encephalopathy: a randomised controlled trial

BackgroundHigh-level evidence for using steroids in epileptic encephalopathy (EE), other than West syndrome (WS), is lacking. This study investigated the efficacy and safety of pulse intravenous methylprednisolone (IVMP) in EE...

Abdominal distension and vomiting in a lupus patient with a desire for pregnancy: think beyond the baby!

Intestinal pseudo-obstruction (IPO) is a rare enteric motility disorder with clinical features of intestinal obstruction in the absence of mechanical blockage. IPO usually presents as recurrent, self-limiting episodes of abdominal distension, postprandial pain, nausea and vomiting. Over time, symptoms can persist, leading to gut stasis with bacterial overgrowth, malabsorption and malnutrition. We present a case of IPO related to systemic lupus erythematosus, treated with intravenous pulses of high-dose methylprednisolone. Nutritional testing should be provided as insidious progression to intestinal failure may go undetected at an early stage, especially in situations with increased nutritional requirements, like pregnancy

Decrease in Bone Formation and Bone Resorption during Intravenous Methylprednisolone Pulse Therapy in Patients with Graves' Orbitopathy.

Background: Treatment with glucocorticoids (GCs) is associated with side effects. In contrast to the well-known negative impact on bone tissue exerted by oral GCs, few data are available regarding intravenous GCs. We investigated the influence of intravenous methylprednisolone (IVMP) on bone turnover markers (BTM): amino-terminal propeptide of type I procollagen (P1NP) and the C-terminal telopeptide of type I collagen (CTX), and on calcium metabolism parameters: 1,25-dihydroxyvitamin D (1,25(OH)2D), 25-hydroxyvitamin D (25(OH)D), calcium (Ca), phosphate (P), and intact parathormone (iPTH). Methods: In a prospective study, 23 consecutive subjects with Graves’ orbitopathy were included and treated with IVMP according to the European Group on Graves’ Orbitopathy recommendations. We evaluated effects on BTM occurring during the first 7 days after 0.5 g IVMP, and after the therapy with 12 IVMP pulses with a cumulative dose of 4.5 g. Results: We observed prompt but transient decrease of P1NP (p < 0.001) and the reduction of CTX (p = 0.02) after the first IVMP pulse. Following the full course of IVMP therapy, both P1NP and CTX were found decreased (p < 0.05 and p < 0.01, respectively). Conclusions: A single pulse of 0.5 g IVMP already decreases bone formation and resorption; however, this change is transient. The full therapy is associated with suppression of bone turnover.

Successful Treatment of Cardiac Tamponade with Systemic Lupus Erythematosus using Belimumab and Hydroxychloroquine

Cardiac tamponade with systemic lupus erythematosus (SLE) is a rare but fatal complication. Although cardiac tamponade is typically managed with pericardiocentesis and medication, there is no definitive treatment for this condition. Herein, we report a case of cardiac tamponade with SLE in a 15-year-old girl. The patient had fever, chest pain, and fatigue for 1 month. She was diagnosed with SLE, and hence, prednisolone treatment was initiated. Her symptoms had alleviated on the 4th day of treatment; however, the following day, she was admitted to our hospital with fever and vomiting. The patient did not show chest pain or dyspnea. She developed hypotension, chest pain, and dyspnea the day after admission. She was diagnosed with cardiac tamponade using echocardiography. After pericardiocentesis, she was administered belimumab and hydroxychloroquine following intravenous methylprednisolone pulses and was maintained with complete remission for 2 years after the discontinuation of prednisolone and mycophenolate mofetil. This case highlights the potential for successful treatment of lupus cardiac tamponade with intravenous methylprednisolone pulses followed by administration of belimumab and hydroxychloroquine.

Acute Antibody-mediated Rejection Coexisting With T Cell-mediated Rejection in Pediatric ABO-incompatible Transplantation.

Background.The management and outcome of ABO-incompatible (ABO-I) liver transplantation (LT) has been improving over the past few decades. Recently, the introduction of a pathological evaluation of acute antibody-mediated rejection (AMR) for liver allograft has provided a new recognition of allograft rejection in LT.Methods.One hundred and one pediatric ABO-I LTs performed in our institute were retrospectively analyzed. We assessed the clinical manifestations, diagnosis, and treatment of acute AMR, focusing on the recipient age and pathological findings.Results.Twelve cases (11.9%) of acute AMR related to ABO-I were observed. Nine cases developed mixed T cell–mediated rejection (TCMR)/AMR. These consisted of 6 patients in the younger age group for whom the preconditioning treatment was not indicated and 4 patients in the older age group to whom rituximab was administered as planned. Two patients in the older age group to whom preoperative rituximab was not administered as planned developed isolated AMR. Acute AMR in the older group required plasma exchange for treatment, regardless of the coexistence of TCMR. In contrast, those in the younger group were successfully treated by intravenous methylprednisolone pulse and intravenous immunoglobulin without plasma exchange, accounting for mild immune reaction.Conclusions.Acute ABO-I AMR can develop simultaneously with TCMR, even in young patients with a compromised humoral immune response following ABO-I LT. Establishing the accurate diagnosis of AMR with a pathological examination, including component 4d staining, is crucial for optimizing treatment.

Long-Term Efficacy and Low Adverse Events of Methylprednisolone Pulses Combined to Low-Dose Glucocorticoids for Systemic Sclerosis: A Retrospective Clinical Study of 10 Years' Follow-Up.

BackgroundPatients with systemic sclerosis (SSc) have poor prognosis without cure methods. We began, 10 years ago, to relieve active SSc using short-term intravenous high-dose methylprednisolone pulse (MP-Pulse) and then maintain remission using long-term and low-dose oral glucocorticoids (LTLD-GC).MethodsTotal 46 of SSc patients with interstitial lung disease (ILD) and induration of skin during January 2006 to December 2019 were analyzed retrospectively, who were followed up for 10 years or more. The patients were treated with MP-Pulse (15 mg/kg/day, 4 days/week, for 2 weeks) with (n=21) or without (n=25) LTLD-GC (prednisone 5–10 mg/day or methylprednisolone 4–8 mg/day). The biographic and clinical data, including occurrence of infection or any adverse reactions, were collected at baseline, 6 months, 1 year, and annually through 10 years after treatment.ResultsFrom baseline to 10 years, compared with MP-Pulse alone, MP-Pulse/LTLD-GC significantly reduced skin and lung fibrosis and improved lung function: Rodnan skin score (mRSS: 22.1±12.4 to 8.16±2.5, P<0.001), forced vital capacity (FVC: 71.7% to 89.83%, P<0.001), forced expiratory volume in the first second (FEV1: 75.7% to 87.88%, P<0.001), diffusing capacity of the lung for carbon monoxide (DLCO: 63.4% to 87.73%, P<0.001), and high-resolution chest computerized tomography scan (HRCT score: 3.96±2.81 to 1.42±0.83, P<0.001). None of the 46 patients had femoral head necrosis, compression fracture, death, or life-threatening adverse events.ConclusionThese outcomes indicate that intravenous MP-Pulse combined with oral LTLD-GC could achieve significant remission and better long-term (10 years) efficacy without severe adverse effects in SSc patients with ILD and induration of skin.

Encephalitis with Extensive Cortical Brain Magnetic Resonance Imaging Changes Secondary to Myelin Oligodendrocyte Glycoprotein Antibody Disease

Patient: Male, 17-month-old Final Diagnosis: Fluid attenuated inversion recovery (FLAIR) imaging showing cortical hyperintense lesions in myelin-oligodendrocyte-glycoprotein (MOG) associated encephalitis with seizures (FLAMES Symptoms: Encephalopathy • fever • left sided weakness • seizures Medication: — Clinical Procedure: Plasmapharesis Specialty: Neurology Objective: Rare disease Background:The relatively new autoimmune disorder, anti-myelin oligodendrocyte glycoprotein (MOG) disease is particularly interesting because of its broad range of presentations. This entity’s appearance on magnetic resonance imaging (MRI) of the brain often makes identifying this disease a challenging process. Younger patients tend to present with an acute disseminated encephalomyelitis picture, with encephalopathy and multifocal neurological signs, while older patients are more likely to present with optic neuritis. We, however, report an atypical case of a patient who presented with encephalopathy, seizures, and significant cortical and subcortical gray matter involvement and was found to have anti-MOG positivity in serum.Case Report:A 17-month-old previously healthy boy presented to Emergency Department with fever, lethargy, vomiting, and left-sided weakness. Eventually, he required intubation due to a prolonged seizure. Continuous electroencephalogram captured several focal seizures, and MRI of the brain showed cortical and subcortical T2 hyperintensities. After extensive laboratory evaluation, he tested positive for anti-MOG antibody. He was empirically started on high-dose intravenous pulse methylprednisolone, followed by plasma exchange, given the poor response to the intravenous steroids. At the 5-month follow-up, the results of the neurological examination had dramatically improved, and MRI findings had largely resolved.Conclusions:This case highlights the importance of suspecting anti-MOG antibody-mediated encephalitis, even while ruling out infectious etiologies, in children presenting with encephalopathy, seizures and MRI abnormalities. Prompt recognition would allow for less delay in treatment and hopefully improve prognosis.

Therapy With Intravenous Methylprednisolone Pulses Is Associated With Loss of Bone Microarchitecture in Trabecular Bone Score -Assessment Among Patients With Moderate-to-Severe Graves' Orbitopathy: A Pilot Study.

BackgroundTherapy with intravenous glucocorticoids (GCs) is associated with various side effects, however, the impact on bone remains elusive. Trabecular bone score (TBS) is a diagnostic tool providing information on bone microarchitecture based on images obtained from dual-energy X-ray absorptiometry. We investigated the influence of the intravenous methylprednisolone (IVMP) pulse administration on TBS in patients with moderate-to-severe Graves’ orbitopathy (GO).MethodsFifteen patients with GO were treated with 12 IVMP pulses (6x0.5g, 6x0.25 g on a weekly schedule). They received supplementation with 2000 IU of vitamin D and 1.0 g of calcium throughout the study period. TBS was assessed at baseline and after last IVMP pulse. To determine the difference between values at baseline and after treatment the least significant change (LSC) methodology was used. We compared pre- and posttreatment mean TBS values.ResultsWe found a significant decrease of TBS in 5 out of 15 (33%) patients. Mean TBS value decreased becoming 2.4% lower than at baseline (p<0.05).ConclusionsIVMP pulse therapy exerts negative effect on bone microarchitecture in TBS assessment. The analysis of the clinical risk factors for osteoporosis and the evaluation of bone mineral density and TBS should be considered before initiating IVMP therapy.