Intravenous Methylprednisolone Pulse Research Articles

Effect of pulsed intravenous methylprednisolone with alternative low-dose prednisone on high-risk IgA nephropathy: a 18-month prospective clinical trial

Full-dose prednisone (FP) regimen in the treatment of high-risk immunoglobulin A nephropathy (IgAN) patients, is still controversial. The pulsed intravenous methylprednisolone combined with alternative low-dose prednisone (MCALP) might have a more favorable safety profile, which has not been fully investigated. Eighty-seven biopsy-proven IgAN adult patients and proteinuria between 1 and 3.5 g/24 h after ACEI/ARB for at least 90 days were randomly assigned to 6-month therapy: (1) MCALP group: 0.5 g of methylprednisolone intravenously for three consecutive days at the beginning of the course and 3rd month respectively, oral prednisone at a dose of 15 mg every other day for 6 months. (2) FP group: 0.8–1.0 mg/kg/days of prednisone (maximum 70 mg/day) for 2 months, then tapered by 5 mg every 10 days for the next 4 months. All patients were followed up for another 12 months. The primary outcome was complete remission (CR) of proteinuria at 12 months. The percentage of CR at 12th and 18th month were similar in the MCALP and FP groups (51% vs 58%, P = 0.490, at 12th month; 60% vs 56%, P = 0.714, at 18th month). The cumulative dosages of glucocorticoid were less in the MCALP group than FP group (4.31 ± 0.26 g vs 7.34 ± 1.21 g, P < 0.001). The analysis of the correlation between kidney biopsy Oxford MEST-C scores with clinical outcomes indicated the percentages of total remission was similar between two groups with or without M1, E1, S1, T1/T2, and C1/C2. More patients in the FP group presented infections (8% in MCALP vs 21% in FP), weight gain (4% in MCALP vs 19% in FP) and Cushing syndrome (3% in MCALP vs 18% in FP). These data indicated that MCALP maybe one of the choices for IgAN patients with a high risk for progression into ESKD.Trial registration: The study approved by the Chinese Clinical Trial Registry (registration date 13/01/2018, approval number ChiCTR1800014442, https://www.chictr.org.cn/).

Bilateral Optic Neuritis Caused by Meningococcal Meningoencephalitis

AbstractIn this article, we described a case of pathogen-induced bilateral optic neuritis accompanying meningococcal meningoencephalitis in an adolescent male. A 15-year-old boy presented to our emergency room due to progressive severe headache, stiff neck, diffuse extremity pain, fever, and nausea concerning meningoencephalitis. Intravenous ceftriaxone, metamizole, and ondansetron were started immediately. Due to acute autonomic dysregulation and development of petechiae, he was transferred to the pediatric intensive care unit. Next morning, the patient developed severe visual impairment. Investigations revealed elevated C-reactive protein, procalcitonin and leucocyte count, decreased platelet count, and clotting activation. Cerebrospinal fluid (CSF) analysis revealed increased leucocyte count, protein, and decreased glucose concentration with pathological CSF cytology. Brain magnetic resonance imaging revealed an increased contrast enhancement in the optic nerve sheath, consistent with acute bilateral optic neuritis. He was started on high-dose intravenous pulse methylprednisolone therapy. After treatment with ceftriaxone und pulse steroids, the patient was discharged in good condition without any visual sequel.

Management skin manifestation of multisystem inflammatory syndrome associated with SARS-CoV-2

BackgroundMultisystem inflammatory syndrome (MIS), which develops after a past covid-19 infection. MIS can be described in different tissue inflammation, including the heart, lung, kidney, brain, skin, eye, and or gastrointestinal organs at the presence of COVID-19. Initially, MIS was described in Europe in children infected with SARS-CoV-2, then it was recently seen in the USA in 2020. MIS is a rare but serious disease condition associated with COVID-19 that can affect children (MIS-C) and adults (MIS-A).Case presentationA 44-year-old male who showed MIS-A in 59-day after his first covid-19 contact history. The patient presented to our emergency department with complaints of high fever, nausea, weakness, redness of the eyes, headache, and joint pain. On the second day of his hospitalization, a maculopapular skin lesion was seen in most of the skin. His fever could not be controlled even given paracetamol and broad effective antibiotics. His clinical, radiological, and laboratory findings showed that he had MIS-A. The patient was given intravenous pulse methylprednisolone and intravenous immunoglobulin (IVIG). These treatments, then, resulted in improvement of his clinical conditions, including fever and skin lesions, on the second day of the treatment. The patient was discharged in 14 days after the treatment.ConclusionThis report indicated that diagnosis and treatment of MIS-A could result in reducing patient morbidity and mortality.

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.

Prostate abscess with acute urinary retention as the initial presentation of granulomatosis with polyangitis

A 50-year-old male presented with acute urinary retention. He also had a history of cough, haemoptysis, exertional dyspnoea, loss of appetite, generalised weakness and left-ear discharge of 1-month duration. Prior episode of acute urinary retention had occurred 2 months ago; diagnostic evaluation revealed prostate abscess, and he had received treatment elsewhere for the same. He also had a history of hypertension. Physical examination revealed fever, pallor, bilateral parotid enlargement and left-ear serous discharge. Clinical examination, imaging of chest revealed consolidation with cavitation on the left side, bilateral pleural effusion. Laboratory testing revealed normocytic normochromic anaemia (haemoglobin 6.6 g/dL); neutrophilic leucocytosis (total leucocyte count 13,100 cells/mm3; polymorphs 87%); raised erythrocyte sedimentation rate (110 mm at the end of the first hour); elevated serum creatinine (2.69 mg/dL) and an active urine sediment. Flexible fibreoptic bronchoscopy showed alveolar haemorrhages. Bronchoalveolar lavage fluid Xpert MTB/RIF testing, cytopathology were negative. Computed tomography-guided biopsy from the lesion was suggestive of granulomatous vasculitis. Cytoplasmic antineutrophil cytoplasmic antibodies (C-ANCA) tested positive. The patient was diagnosed to have granulomatosis with polyangitis (GPA). As arterial hypoxaemia was evident, tracheal intubation and mechanical ventilatory support were initiated. Renal replacement therapy, intravenous (iv) methyl prednisolone and cyclophosphamide pulse therapy and five sessions of plasmapheresis were administered. As remission could not be achieved, iv rituximab was started. On the 36th day of admission, the patient developed septic shock and died. The present case documents the uncommon association of GPA with prostate abscess, acute urinary retention.

Effect of High-Dose Glucocorticoids on Markers of Inflammation and Bone Metabolism in Patients with Primary Glomerular Disease

Background: Glucocorticoids are one of the most commonly used drugs for treatment of inflammatory and autoimmune diseases. Sirtuin-1 (SIRT-1) belongs to the family of proteins involved in protection against inflammation and oxidative stress. A role of SIRT-1 in regulation of bone metabolism during high-dose steroid therapy is unknown. Objectives: The study assessed influence of high doses of intravenous corticosteroids on plasma inflammation and bone markers in patients with primary glomerular disease. Methods: The study included 40 patients (25 M, 15 F; mean age 53.1 ± 14 years) with chronic kidney disease (mean estimated glomerular filtration rate, 58.9 ± 31.3 mL/min). The main inclusion criterion was clinical and histopathological diagnosis of primary glomerular disease and urine protein excretion >2.0 g/24 h. The patients received intravenous pulses of methylprednisolone 20–30 mg/kg/day for three consecutive days followed by oral prednisone 0.8–1.0 mg/kg/day. The blood was taken before administration of methylprednisolone to assess plasma SIRT-1, sclerostin, calcium, phosphate, and parathormone; and first-morning urine sample was taken for measurement of calcium, phosphate, and albumin to creatinine ratio. The same laboratory tests were repeated after 4, 7, and 30 days during steroid therapy. Results: Plasma SIRT-1 increased significantly during steroid administration. Plasma sclerostin did not change significantly. There was a significant linear negative correlation between changes in SIRT-1 levels and sclerostin throughout the study. In a multiple regression model, changes of plasma sclerostin induced by steroid therapy explained the largest part of variance of respective changes of plasma SIRT-1. Conclusions: Plasma SIRT-1 increase during high-dose corticosteroid therapy is negatively related to the change of plasma sclerostin that may suggest a protective role of SIRT-1 against the negative effects of steroid therapy on bone.

Cryptogenic new-onset refractory status epilepticus successfully treated with intravenous methylprednisolone pulse therapy and intravenous immunoglobulin

Cryptogenic new-onset refractory status epilepticus successfully treated with intravenous methylprednisolone pulse therapy and intravenous immunoglobulin

An Unwonted Cause of Acute Cerebellar Ataxia in a Young Lady: A Rare Path to Lupus

Neuropsychiatric manifestations are commonly observed in systemic lupus erythematosus (SLE) patients. In particular, neurological involvement is known to be more common in patients with positive anticardiolipin antibodies and lupus anticoagulants. Nevertheless, cerebellar ataxia has rarely been reported, especially as the first clinical manifestation of neuropsychiatric systemic lupus erythematosus (NPSLE). Cerebral vascular infarction or ischemia, vasogenic oedema and antibody-mediated cerebral vasculopathy or vasculitic process have been supposed as possible aetiologies of acute cerebellar ataxia related to SLE. We report the clinical and radiological features of a 38 years old diabetic lady who developed a rapidly progressive cerebellar syndrome as first sign of SLE; no other cause explaining her cerebellar ataxia was found. Imaging of brain in this patient revealed prominent cerebellar atrophy. She was treated with initial pulse intravenous methylprednisolone later oral prednisolone and oral mycophenolate mofetil. There was no further progression of her neurological signs after the initiation of therapy. The appearance of a cerebellar syndrome with unknown aetiology with associated features of possible systemic autoimmune dysfunction, should be taken into account in clinical practice for appropriate diagnostic workup in order to provide effective therapeutic options.

Acrodystrophic axonal polyneuropathy with celiac disease: a case report

BackgroundPatients with celiac disease present with not only gastrointestinal symptoms but also extraintestinal manifestations such as anemia, osteopathy, dermatitis herpetiformis, and celiac neuropathy. Despite a fairly wide range of celiac neuropathies, we report a case of the acrodystrophic variant of celiac polyneuropathy, which has not been previously described.Case presentationA 41-year-old Ukrainian male suffered from symmetric, sensorimotor axonal polyneuropathy and encephalopathy associated with celiac disease, which is characterized by severe trophic disorders in the lower extremities (trophic ulcers, hyperkeratosis, and anhidrosis). Acrodystrophic changes in the lower extremities were due to both neurogenic and direct immunoinflammatory damaging effects. Clinical–electrophysiological dissociation was also noted, which was represented by a gross axonal lesion with the preservation of muscle strength. The absence of enteropathic manifestations was accompanied by the pronounced histological changes in the duodenal mucosa by IIIb stage of Marsh. A gluten-free diet in combination with membrane plasma exchange and intravenous pulse methylprednisolone was prescribed to reduce the severity of sensory disorders and regression of encephalopathy within 7 months.ConclusionCeliac disease may be a potential cause of neuropathy and encephalopathy in adult patients. Further immunosuppressive treatment protocols for both intestinal and extraintestinal manifestations of celiac disease are required.

Early diagnosis of giant cell arteritis - the basis of good outcome

Purpose: If untreated, giant cell arteritis can lead to blindness and stroke. The study objectives were to assess diagnostic procedures and treatment in early interventional clinic in University Clinical Centre Maribor in patients with temporal arteritis.&#x0D; Methods: Retrospective study (from 2012 to 2017) of patients diagnosed with temporal arteritis. We assessed epidemiological data, delay of diagnosis, and diagnostic procedures. Results were assessed with statistical methods (SPSS 22.0).&#x0D; Main goal: The main goal was to determinate the delay in days between symptom onset and admission to the interventional rheumatology clinic and to assess the causes of delay.&#x0D; Results: Fifty-three GCA (66 % female) patients with mean age 76.25 (from 63–89 years) years were included. Mean time duration of symptoms before admission to our early interventional clinic was 33.74 (0–180) days. The diagnostic procedure was completed in mean time of 2.04 days from the presentation at our interventional rheumatology clinic. The median time to the temporal artery biopsy (TAB) performed in 52 /53 patients was 2 days, with the median 2 days to the preliminary histological results from admission. TAB was positive in 43 (81.1%) of cases. The median time from admittance to colour Doppler sonography (CDS) of aortic arch branches was 2 days and it was positive in all 19 (35.8%) performed cases. 16 (30.2%) patients had polymyalgia rheumatica, 35 (66%) patients had visual disturbances, permanent one eye blindness occurred in 12 (22.64%) patients, and 2 (2.8%) patients experienced permanent blindness on both eyes. Seventeen patients (32.1%) were initially treated with intravenous methylprednisolone pulse. The mean initial dose of oral methylprednisolone was 45.55 (± 15.54) mg. All patients received low dose Aspirin.&#x0D; Conclusion: Early diagnosis and treatment of giant cell arteritis are very important as miss- or non-diagnosed GCA can lead to permanent blindness of the patient. With better education and public awareness, better access and better professional education of primary care physicians, and early admission to secondary interventional clinics we might spare these patients from the devastating consequences of the GCA.

Atypical Presentation of Dysphagia in a Patient Diagnosed Later With Dermatomyositis: A Case Report

Dysphagia has been reported in 10%-73% of patients with dermatomyositis. We present the case of a 58-year-old female patient who presented to the emergency department of Peterborough City Hospital with acute-onset difficulty in swallowing. Physical examination demonstrates proximal muscle weakness of the upper limbs and symmetrical skin rash over the face, chest, and thighs. Both clinical and laboratory findings pointed towards the diagnosis dermatomyositis. Oesophagogastroduodenoscopy identified no significant abnormality reducing the possibility of dysphagia due to an intrusive lesion, such as an abscess or a malignancy. MRI scan of the lower limbs revealed evidence of proximal myositis. CT neck, chest, abdomen and pelvis exclude any associated malignancy. The patient was treated initially with intravenous pulses of methylprednisolone for three days, and then switched to oral prednisolone and cyclophosphamide cycles and was considered for intravenous immunoglobulins as her symptoms had not completely resolved.

Central nervous system vasculitis in a patient with axial spondyloarthritis treated with infliximab: A case report and literature review.

Central nervous system (CNS) vasculitis is a rare form of vasculitis involving the blood vessels of the brain. It may be primary when it is confined to the CNS or secondary in the context of systemic inflammatory conditions such as systemic lupus erythematosus, rheumatoid arthritis, or infections. However, there is no known association with axial spondyloarthritis. Herein, we present the case of a 37-year-old man, with axial spondyloarthritis treated with infliximab for 9 years, who presented with persistent fevers, elevated inflammation markers, lateral medullary syndrome, and right-sided hemiparesis. Magnetic resonance imaging of the brain demonstrated multiple cerebral infarcts. Examination of cerebrospinal fluid showed mild lymphocytic pleocytosis and protein elevation. Digital subtraction angiography and transcranial ultrasonography of the cerebral blood vessels revealed luminal narrowing of the basilar and the left posterior cerebral artery. The diagnosis of CNS vasculitis was made and intravenous methylprednisolone and cyclophosphamide pulses were administered, leading to fever remission with gradual improvement and resolution of the neurological manifestations.

Factors influencing intravenous methylprednisolone pulse therapy in Chinese patients with isolated optic neuritis associated with AQP4 antibody-seropositive neuromyelitis optica

This study investigated the factors influencing intravenous methylprednisolone pulse (IVMP) therapy for recovering visual acuity in Chinese patients with aquaporin-4 (AQP4) antibody-seropositive neuromyelitis optica-related optic neuritis (NMO-ON). This retrospective case series included 243 affected eyes of 182 patients (36 male, 146 female) diagnosed with NMO-ON in the Neuro-Ophthalmology Clinic of Beijing Tongren Hospital from September 2012 to September 2020. All patients with AQP4-antibody seropositivity had clinical manifestations of acute ON, excluding other diagnoses and received IVMP treatment at 500 mg/day or 1000 mg/day for 3 days. Primary outcome was the extent of improvement in logMAR visual acuity after IVMP treatment. The therapeutic influences of sex, age, baseline visual acuity, therapeutic intervals, and IVMP dose on acute NMO-ON were analysed. Chi-square tests, Mann–Whitney U-tests, Kruskal–Wallis tests, Spearman’s correlation coefficients, and multiple linear regression were used for statistical analysis. Age ranged between 7 and 80 years (median age, 44; interquartile range [IQR], 29–52) years. Among the 243 eyes, the median improvement in logMAR visual acuity was 0.3 (IQR, 0–0.9). Therapeutic efficacy of IVMP was significantly higher in female than in male patients (Z = 2.117, P = 0.034). The treatment effect gradually decreased with increase in age at onset (Rs = 0.157, P = 0.015), and visual improvement was significantly lower in patients aged > 50 years than in those ≤ 50 years (Z = 2.571, P = 0.010). When patients had low visual acuity at onset, improvements were more obvious (rho = − 0.317, P < 0.001); however, final visual acuity was still low (rho = 0.688, P < 0.001). Therapeutic effect was negatively correlated with therapeutic intervals (rho = 0.228, P = 0.001). Dosage of methylprednisolone (1000 mg/day or 500 mg/day) did not significantly influence treatment efficacy (Z = 0.951 P = 0.342). Therefore, IVMP therapy can improve visual acuity in the affected eyes of patients with AQP4 antibody-seropositive NMO-ON with similar effect at 500 mg/day and 1000 mg/day doses. Sex, age at onset, and therapeutic intervals may influence the efficacy of IVMP in patients with NMO-ON.

Clinical spectrum of pediatric drug refractory epilepsy secondary to parieto-occipital gliosis

BackgroundParieto-occipital (PO) gliosis secondary to perinatal insult, is often associated with neurologic sequelae such as epilepsy, which can be drug resistant. ObjectiveTo evaluate the spectrum of epilepsy among patients presenting with seizures in association with PO gliosis and to determine factors that influence the development of epileptic encephalopathy (EE) in these patients. MethodsWe retrospectively evaluated patients aged < 16 years with drug refractory epilepsy and PO gliosis who underwent video electroencephalography (Video EEG). We evaluated the clinical, electrophysiological and radiological profile including treatment responsiveness of subjects with EE. ResultsOne hundred one patients (M: F=3:1) with mean age of onset of epilepsy at 28.9 ± 33.1 months were recruited into the study. Based on video EEG findings, Based on video EEG findings, the commonest type of focal onset ictus was tonic seizures with impaired awareness (n = 26, 29.9%). Myoclonic jerks (n = 20, 23%) were the commonest type of generalised onset seizures. Ictal onset from parieto occipital region were observed in 28 patients. Ictal onset from frontal, temporal and fronto temporal region were observed in 6 (6.8%), 7(7.9%) and 9 (8.9%) patients, respectively. Comparison of the seizure types and ictal onset among subgroups of patients with occipital gliosis, parieto-occipital gliosis and parieto-occipital with frontal gliosis revealed that the extent of gliosis did not significantly affect seizure semiology or ictal onset. EE was significantly associated with presence of neonatal seizures (p = 0.04), hypoglycaemia (p = 0.005), longer duration of ICU stay (Z score = −3.55, p < 0.001) and younger age of onset of seizures (Z score = - 2.97, p = 0.03). Eleven out of eighteen (64.7%) subjects with EE showed greater than 50% improvement in seizure frequency following three months of pulse intravenous methylprednisolone therapy. ConclusionsAmong subjects with PO gliosis on MRI, the seizure semiology is unaffected by laterality, radiologic extension beyond the occipital cortex or presence of ulegyria. Patients with PO gliosis can have florid interictal epileptiform discharges anteriorly and can have seizures with ictal onset from frontal and temporal region. Development of EE is strongly related to the age of onset of seizures, neonatal seizures, prolonged NICU admission, rather than the radiological findings. Subjects with EE and PO gliosis show good response to intravenous pulse methylprednisolone.

O28 A rare sight-threatening complication of a rare disease

Case report - IntroductionRelapsing polychondritis (RP) is a rare autoimmune disorder characterised by inflammation of cartilaginous structures throughout the body. It usually presents in the fourth and sixth decade, and commonly affected areas include the nasal and respiratory tracts, external ears and joints. Ocular involvement is reported in around 65% of RP patients during their lifetime but is rarely sight-threatening. However, we present an unusual case of recurrent ocular inflammation due to RP that resulted in unilateral posterior scleritis with sub-retinal exudation, and posed a high risk of retinal detachment. Prompt escalation of immunosuppressive treatment was required to prevent this.Case report - Case descriptionA 48-year-old man of south-east Asian descent presented to rheumatology in December 2020 with a typical history of new inflammatory arthritis of 4 weeks duration. He was known to have bilateral episcleritis and ocular hypertension for 3 years and took Brinzolamide and Latanoprost eye drops, as well as metformin for type 2 diabetes mellitus. The only other relevant history was treatment with antibiotics as an inpatient for bilateral pinna cellulitis 2 months prior, which was suspected to be related to his diabetes. Examination revealed mildly reduced hand grips but no definite synovitis. The most remarkable finding was bilateral painless red eyes. Both pinnae appeared inflamed on close inspection without obvious auricular lobe involvement. Blood tests showed raised C-Reactive Protein (38 mg/L), and Erythrocyte Sedimentation Rate (62 mm/hr), with normochromic normocytic anaemia but other blood counts were normal. Renal and liver function was normal. Detailed immunology was negative.Relapsing Polychondritis was suspected and confirmed following multidisciplinary team (MDT) discussions with the Ear Nose and Throat and ophthalmology teams. At the next visit, his disease progressed to florid polyarthritis in a rheumatoid distribution. This responded well to low-dose prednisolone and methotrexate. However, the patient attended eye casualty on multiple occasions over the next 6 weeks with alternating acute eye pain and redness, mildly reduced visual acuity and raised intraocular pressures. He developed sub-retinal fluid pockets suggestive of posterior scleritis in the right eye and retinal tomography demonstrated extensive pigment clumping especially in the macula with outer retinal disruption. Urgent pulses of intravenous methylprednisolone were given, followed by high-dose prednisolone tapering. The case was discussed with the regional uveitis MDT, and treatment was escalated to adalimumab. He is currently 1 month into treatment and has preserved visual acuity and stable intraocular pressures, with no other systemic involvement of his RP.Case report - DiscussionThe diagnosis of RP requires a high index of clinical suspicion given the lack of diagnostic markers. The clinical features are multisystem and might not occur simultaneously as in this case, where the eye disease preceded the ear and joint manifestations by a couple of years. Perichondritis might be more difficult to appreciate in patients with darker skin and can further delay diagnosis. The average time to diagnosis from the initial symptoms has been reported as 14 months from a case series of 158 patients with RP in China.Ocular complications are usually bilateral and the commonest are episcleritis and scleritis (over 50% of cases have one of these), followed by uveitis and retinopathy. Eyelid oedema, proptosis and optic neuritis can also occur but are rarer, and exudative retinal detachment has only been noted in a small number of case reports. Unfortunately, no current treatment guidelines exist for RP due to a lack of randomised controlled trials but treatment is usually guided by the severity of manifestations in conjunction with trends from case series and expert opinion. The aim is to halt or slow disease progression and glucocorticoids are often used first-line for moderate to severe disease, with or without the addition of steroid-sparing agents such as methotrexate, ciclosporin and azathioprine. A meta-analysis of biologics in RP conducted by Kemta et al. in 2012 has suggested anti-TNF (particularly Infliximab) might be beneficial as second-line treatment for severe or refractory cases of organ involvement due to RP such as central nervous system, nasal or pulmonary involvement. However, due to the extreme rarity of both RP and associated severe retinal disease, the optimum therapeutic choice in this setting is currently not known.Case report - Key learning pointsRP remains a challenging clinical diagnosis with a common delay in diagnosis of more than 1 year from the onset of symptoms. High index of suspicion and MDT input can aid earlier diagnosis and treatment to mitigate further complications.Eye disease occurs in up to two-thirds of patients with RP but exudative retinal or subretinal involvement is very rare. It can occur even when other manifestations of RP are well-controlled with immunosuppression as in this case.Anti-TNF treatment could be used to minimise visual loss in these severe cases of inflammatory eye disease due to RP, given reported benefit for other organ-threatening complications of RP. However, future case series are needed to guide evidence-based treatment choices.

Clinical Analysis of Pediatric Opsoclonus-Myoclonus Syndrome in One of the National Children's Medical Center in China.

Objective: To study the clinical characteristics and treatment of pediatric opsoclonus-myoclonus syndrome (OMS).Methods: We analyzed the clinical data of nine children OMS between June 2017 and Nov 2020.Results: Nine children (M/F = 3:6, median onset age was 18 months) diagnosed with OMS were included in the study. Before onset, human rhinovirus and respiratory syncytial virus were seen in one patient, respectively. And one patient received Japanese encephalitis vaccination. Three patients had neuroblastoma, and one patient had ganglioneuroblastoma. All patients' symptoms were improved after receiving surgery (for four patients with tumor), intravenous human immunoglobulin and pulsed methylprednisolone. However, four patients without mass relapsed and became relapse free after rituximab treatment. The relapse rate was 44.4% (4/9). The OMS severity score at the last follow-up was significantly lower than the OMS severity score at onset (3.0 ± 1.0 vs. 11.0 ± 2.2, paired-samples t-test, P < 0.001). All patients had at least one item of neurological symptoms or neuropsychological disturbances.Conclusion: For pediatric OMS, human rhinovirus infection and respiratory syncytial virus infection can be seen before onset. Rituximab is effective in reducing relapse. Improving recognition and long-term prognosis in OMS is urgent.

Macrophage activation syndrome triggered by systemic lupus erythematosus flare: successful treatment with a combination of dexamethasone sodium phosphate, intravenous immunoglobulin, and cyclosporine: a case report

BackgroundMacrophage activation syndrome is classified as a secondary form of hemophagocytic lymphohistiocytosis. It is a hyperinflammatory complication observed to be comorbid with a variety of autoimmune diseases, including adult-onset Still’s disease and systemic juvenile idiopathic arthritis. Macrophage activation syndrome is less commonly detected in adult patients with systemic lupus erythematosus, which, if untreated, can be fatal, though determining the optimum treatment strategy is still a challenge.Case presentationHerein, we report a case of macrophage activation syndrome in a 33-year-old Egyptian female as an unusual complication of a systemic lupus erythematosus flare in adult patients. Our patient was initially treated with a combination of intravenous methylprednisolone pulse therapy and intravenous immunoglobulin therapy, which was followed by a course of oral prednisolone and oral cyclosporine with little response. Switching from oral prednisone to intravenous dexamethasone sodium phosphate showed a more favorable clinical and biochemical response.ConclusionMacrophage activation syndrome is less commonly detected in adult patients with systemic lupus erythematosus. Our case demonstrates that dexamethasone sodium phosphate can be a successful alternative treatment for patients with systemic lupus erythematosus complicated by macrophage activation syndrome in whom the response to pulse methylprednisolone was inadequate to manage their illness, proving to be remarkably effective in a relatively short time frame.

Comparison of Tocilizumab and High-dose Methylprednisolone Pulse on Outcomes in Severe Corona Virus Disease-2019: TAME-COVID, a Retrospective Multicentric Study.

Background:India recently encountered fierce second wave of coronavirus disease (COVID-19), and scarcity of novel medications added to the management challenges. Various studies have highlighted the effectiveness of tocilizumab and high-dose steroids in severe COVIDs, but none has compared their efficacy.Materials and Methods:This retrospective multi-centric analysis compares intravenous tocilizumab (8 mg/kg/day, maximum dose-800 mg), and intravenous Methylprednisolone Pulse (MPS-1 g/day for 3 days) in severe COVID-19. Both the groups had additionally received the standard of care COVID treatment as per protocol. Outcomes were assessed at 30 days.Results:A total of 336 patients, with 249 receiving MPS and 87 receiving tocilizumab were compared. Majority of these were males (72.9%) with a mean age of 57.4 ± 13.6 years. Diabetes was the most common comorbidity. Patients in both groups had comparable age distribution, comorbidities, presenting mean-arterial pressures, d-Dimer levels, serum ferritin, serum leukocyte-dehydrogenase, and procalcitonin. However, the tocilizumab group had more number of males, higher incidence of coronary artery disease, more tachypnea and leukocytosis, more number of patients with severe acute respiratory disease syndrome (PaO2/FiO2 ratio <100), and higher C-reactive protein levels at presentation. Both groups had comparable adverse events' profile. Tocilizumab group had lesser requirement of invasive ventilation than MPS group (17% vs. 29%, P = 0.038), however mortality at the end of 30 days follow-up was similar (36% vs. 34% respectively; P = 0.678).Conclusions:Tocilizumab decreased the need for invasive ventilation in severe COVID-19; however, it did not translate to improved survival. A planned prospective randomized study is recommended in this respect to compare their efficacy.

Observational study on effect of pulse intravenous methylprednisolone in drug refractory epilepsy

Treatment options for management of Drug Refractory Epilepsy are limited and associated with poor results. One of the hypotheses being forwarded for genesis of drug refractoriness in epilepsy is inflammation. In this regard, corticosteroids, well known for their anti-inflammatory and anti-immune properties, have been used with variable results in different epilepsy syndromes. Steroids have also been seen to have a blood brain barrier repair thus helping in better penetration of Anti Epileptic Drugs. Thus, we aim to study the effect of pulse intravenous methylprednisolone in drug refractory epilepsy.

Juvenile Generalized Myasthenia Gravis: Presented as Unilateral Blepharoptosis and Successfully Managed with Pulse Intravenous Methylprednisolone

Myasthenia Gravis is a rare autoimmune disorder of childhood and this is rarer in South Asia. We present a pre-pubertal 7 year old female child of seropositive Generalized Juvenile Myasthenia Gravis. She presented with unilateral blepharoptosis and later generalized symptoms appeared. Ice-pack test, Neostigmine challenge test and acetylcholine receptor antibody test were positive. Serum muscle specific tyrosine kinase antibody test was normal. She did not have thymic abnormalities. She did not respond to high dose (26 mg/kg/day) of Pyridostigmine and oral Prednisolone (2 mg/kg/day), but was successfully treated with a combination of pulse intravenous Methylprednisolone (30 mg/kg once a month for 6 months) and daily doses of oral Prednisolone (2 mg/kg/day) along with Pyridostigmine without significant side effects. This combination can be considered a potential inexpensive treatment for Juvenile Myasthenia Gravis in a resource limited area where other immunosuppressive treatments such as intravenous immunoglobulin is expensive and unaffordable.