Intravenous Methylprednisolone Pulse Therapy Research Articles

Patients with anti-small ubiquitin-like modifier activating enzyme-positive dermatomyositis resembling antisynthetase syndrome with poor prognosis: a bicentric international retrospective study and literature review.

This study aimed to describe adult Brazilian and Japanese patients with anti-small ubiquitin-like modifier activating enzyme (SEA)-positive dermatomyositis (DM), as there are few studies in the literature. A literature review was also conducted. This bicentric international retrospective study, conducted between 2012 and 2023, included patients with anti-SAE-positive DM (2017 European League Against Rheumatism/American College of Rheumatology classification criteria). All demographic features and clinical, laboratory, therapeutic, and follow-up data were collected from Brazilian and Japanese centers using pre-standardized and parameterized information. We included 17 adult patients with a median age of 65 (56-76) and a predominance of females (82.4%). Constitutional symptoms at baseline were present in 58.8% of the patients. In addition to classical cutaneous DM lesions, one-third of the patients had myalgia and significant muscle weakness, whereas half presented with dysphagia, interstitial lung disease, and joint manifestations. The first-line treatment consisted of intravenous methylprednisolone and immunoglobulin pulse therapy in 41.2% and 28.6% of the patients, respectively. The median follow-up duration was 20 (13-74) months; at the last medical evaluation, half had active disease and were still using oral glucocorticoids (median dosage, 10.0 mg/day). Approximately one-fifth to one-third of the patients were diagnosed with different types of cancer, had severe infections, or died. Patients with anti-SAE-positive DM not only resemble the phenotype of antisynthetase syndrome but are also associated with a poor prognosis.

Visual Field Improvement in Non-Arteritic Posterior Ischemic Optic Neuropathy in a Patient Treated with Intravenous Steroids

A 50-year-old man presented with a nasal visual field defect in the left eye. His medical history was unremarkable. On ophthalmic examination, his best-corrected visual acuity (BCVA) was 1.2 in both eyes. Fundoscopy revealed no abnormalities in either eye. Goldmann visual field test showed a nasal visual field defect in the left eye. The patient was diagnosed with NPION of the left eye and was followed up without treatment. However, four days after the initial visit, his BCVA decreased to counting finger in the left eye. Goldmann visual field test showed a complete nasal visual field defect with central absolute scotoma. Following admission, the patient was treated for 3 days with intravenous methylprednisolone pulse therapy. Three months later, his BCVA was improved to 0.8 in the left eye, but central relative scotoma was remained. Six months later, his BCVA was maintained at 0.8, and central relative scotoma was disappeared. However, the optic disc looked diffusely pale in the left eye. This case highlights that steroid therapy is an option for severe cases with posterior ischemic optic neuropathy.

Optic Neuritis After COVID-19 Vaccination: A Case Series and a Review of Literature.

The coronavirus disease (COVID-19) pandemic broke out in March 2020, causing tremendous damage to public health and more than 6 million deaths. After authorization for the emergency use of COVID-19 vaccines, various adverse events have been reported, including optic neuritis. COVID-19 vaccination was implemented in Taiwan in March 2021. We report patients who developed optic neuritis after COVID-19 vaccination at one university-affiliated tertiary hospital, between March 2021 and December 2022. We also provided a literature review of optic neuritis cases after COVID-19 vaccination. Five patients who developed optic neuritis after COVID-19 vaccination have been identified. Four brands of vaccine used were as follows: Moderna, Pfizer-BioNTech, Medigen, and Oxford AstraZeneca. Optic neuritis developed after the first dose of vaccination in 4 patients, whereas in 1 patient, it developed after the second shot. In the 3 patients with poor initial visual acuity, intravenous methylprednisolone pulse therapy achieved substantial improvement. Optic neuritis is a rare but potentially vision-threatening adverse effect of COVID-19 vaccination. We suggest early diagnosis and treatment to maximize visual outcomes.

Clinical features and death risk factors of pneumocystis jirovecii pneumonia in kidney disease patients with immunosuppressive therapy

To investigate the clinical features and death risk factors of pneumocystis jirovecii pneumonia (PJP) in kidney disease patients with immunosuppressive patients. A Retrospective case series study was performed in 52 PJP patients with kidney disease who received immunosuppressive therapy in Nephrology or Respiratory department of Peking University First Hospital from January 1, 2006 to August 31, 2021. Patients were divided into survival group (36 cases) and death group (16 cases) according to their clinical outcomes. Univariate analysis was performed to compare the differences of clinical features between the two groups. Multivariate logistic regression model was used to analyze the death risk factors. The results showed that the median serum creatinine was 192.5 (109.8, 293.7) μmol/L, and the incidence of acute kidney injury was 63.5% (33/52). Univariate analysis showed that age (t=1.197,P=0.030), C-reactive protein level (t=2.378,P=0.022), time from onset to diagnosis (χ2=6.62,P=0.010), PJP severity (χ2=5.482,P=0.019), complicated with septic shock (χ2=3.997,P=0.046), mechanical ventilation (χ2=11.755,P=0.001), and blood purification therapy (χ2=4.748,P=0.029) were statistically significant. There were no statistically significant differences between the two groups in gender, duration and dosage of hormone therapy before PJP onset, intravenous methylprednisolone pulse therapy, immunosuppressant use, and serum creatinine level before and after hospitalization for anti-PJP treatment (all P>0.05). Multivariate analysis showed that the time from onset to diagnosis of PJP was >10 days (OR=40.945, 95%CI: 1.738-451.214; P=0.021) and severe PJP (OR=25.502, 95%CI: 1.426-74.806; P=0.028) was an independent death risk factor for kidney disease complicated with PJP of immunosuppressive therapy. In conclusion, the time from onset to diagnosis of PJP and PJP severity are independent death risk factors in patients with kidney disease complicated with PJP of immunosuppressive therapy. Close attention should be paid to oxygenation condition and early diagnosis can prevent the aggravation of PJP and improve the prognosis.

Efficacy and Safety of Pulse Intravenous Methylprednisolone in Pediatric Epileptic Encephalopathies: Timing and Networks Consideration.

Background: Epileptic encephalopathies (EE) are characterized by severe drug-resistant seizures, early onset, and unfavorable developmental outcomes. This article discusses the use of intravenous methylprednisolone (IVMP) pulse therapy in pediatric patients with EE to evaluate its efficacy and tolerability. Methods: This is a retrospective study from 2020 to 2023. Inclusion criteria were ≤18 years at the time of IVMP pulse therapy and at least 6 months of follow-up. Efficacy and outcome, defined as seizure reduction > 50% (responder rate), were evaluated at 6 and 9 months of therapy, and 6 months after therapy suspension; quality of life (QoL) was also assessed. Variables predicting positive post-IVMP outcomes were identified using statistical analysis. Results: The study included 21 patients, with a responder rate of 85.7% at 6 and 9 months of therapy, and 80.9% at 6 months after therapy suspension. Variables significantly predicting favorable outcome were etiology (p = 0.0475) and epilepsy type (p = 0.0475), with the best outcome achieved in patients with genetic epilepsy and those with encephalopathy related to electrical status epilepticus during slow-wave sleep (ESES). All patients evidenced improvements in QoL at the last follow-up, with no relevant adverse events reported. Conclusions: Our study confirmed the efficacy and high tolerability of IVMP pulse therapy in pediatric patients with EE. Genetic epilepsy and ESES were positive predictors of a favorable clinical outcome. QOL, EEG tracing, and postural-motor development showed an improving trend as well. IVMP pulse therapy should be considered earlier in patients with EE.

Intravenous methylprednisolone therapy for ocular myasthenia gravis: A retrospective study

AbstractBackgroundAlthough intravenous methylprednisolone pulse (IVMP) therapy has been recommended for ocular myasthenia gravis (OMG), the evidence is limited.AimWe aimed to investigate the efficacy and safety of IVMP for OMG.MethodsThe patients with OMG were chosen retrospectively from May 2010 to December 2022. The therapeutic effects between IVMP‐treated and non‐IVMP‐treated groups were determined by ∆ocular quantitative MG (QMG) score and the ∆ocular MG activities of daily living profile (MG‐ADL) score, which is the disparities of the scores between before and after 1 month of drug administration; and Myasthenia Gravis Foundation of America post‐intervention status at 1, 3, 6, and 12 months.ResultsThere were 26 patients with OMG included, with the mean age of 67.2 ± 13.1 years. 13 of the 26 people with OMG received IVMP and subsequent low‐dose immunotherapies. The ∆ocular QMG and ∆ocular MG‐ADL scores were significantly higher in the IVMP group of 3 (1.5, 4) and 3 (2, 4.5) than in the non‐IVMP group of 1.5 (0, 3) and 1 (0, 2), p = 0.038 and p = 0.0027, respectively. The rates of minimal manifestation or better status in post‐intervention status at 1 and 12 months were also considerably higher in the IVMP group of 77% and 92% compared with the non‐IVMP group of 0% and 23%, p = 0.0001 and 0.001. There were no serious side effects discovered.ConclusionIVMP induction therapy can quickly and safely improve symptoms of OMG, and subsequent low‐dose immunotherapy can keep symptoms at bay for 12 months.

DIAGNOSTIC CHALLENGE IN MIDDLE-AGED WOMAN WITH RECURRENT ISCHEMIC STROKES: A CASE OF PRIMARY CENTRAL NERVOUS SYSTEM VASCULITIS AND LITERATURE REVIEW

Primary vasculitides of the central nervous system are rare vascular disorders whose pathophysiology involves inflammatory infiltration exclusively in the vessels of the central nervous system. Its spectrum extends from primary angiitis of the central nervous system to cerebral amyloid angiopathy. This case portrays a 46-year-old woman with a history of multiple strokes. History of multiple comorbidities such as systemic arterial hypertension, prediabetes, and smoking. During follow-up after the second ischemic event, a patent foramen ovale was detected with positive microbubbles stand. Therefore, she was using oral anticoagulants. Transthoracic echocardiogram and magnetic resonance angiography without relevant changes. However, she resorted to the neurological deficit three more times. Cerebrospinal fluid (CSF) analysis was done twice; both were normal. Cerebral angiography was performed at the time of the last stroke and revealed suggestive signs of vasculitis. Discrimination of angiitis and reversible cerebral vasoconstriction syndrome was challenging and based on the demographic and clinical features of the patient. The characteristics of this case that corroborate the hypothesis of angiitis are the patient's age, which is in the median age range (40-60 years), chronic headache, and focal symptoms not temporally associated with the headache. Intravenous methylprednisolone pulse therapy (one gram/day) was performed for five days. Evolved with progressive improvement of the motor deficit. On hospital discharge, it was prescribed prednisone, and after ten days, Azathioprine 2.5mg/Kg/day was started. Diagnosis of primary central nervous system vasculitis requires high clinical suspicion due to the low specificity of the clinical features. This report reinforces the importance of investigating CNS vasculitis in young patients with a history of recurrent stroke.

INTRAVENOUS METHYLPREDNISOLONE EFFECTS ON RECTUS MUSCLES IN THYROID EYE DISEASE

Introduction & Objective: Intravenous methylprednisolone has been used in management of Thyroid Eye Diseases (TED) which was an autoimmune inflammatory disease of the orbital tissues and the eyes. Previous study has shown benefits of intravenous methylprednisolone therapy on TED but there was need of quantitative measure to assess the effect of intravenous methylprednisolone therapy on extraocular rectus muscles. This study aimed to report the effect of high-dose intravenous methylprednisolone pulse therapy on inferior, medial, superior, and lateral rectus muscles and providing data distribution of active TED before and after therapy. Methods: Retrospective analyses were used in the data collection of patients with active Thyroid Eye Disease (TED) underwent high-dose intravenous methylprednisolone pulse therapy regiment 500mg every week for six consecutive weeks at Sardjito Eye Centre between January 2022 and December 2022. Ophthalmological investigation and quantitative computed tomography (CT) scan of the orbit were performed before and after methylprednisolone pulse therapy and demographics data were presented in tables and graphs. Results: This study included 62 eyes of 31 patients (61,3% female) with mean age of 37.8±13.47 years old. The mean thickness of Superior Rectus (SR) muscle before therapy were 4.12±2.20 mm and significantly decreased to 3.70±1.54 mm post therapy. There was no significant difference in delta mean Inferior Rectus (?IR) -0.18±1.69 (p=0.381), Medial Rectus (?MR) -0.34±1.68 (p=0.116), and Lateral Rectus (?LR) -0.33±1.94 (p=0.188) Conclusion: The treatment of Thyroid Eye Disease (TED) with high dose intravenous corticosteroid is safe and effective in reducing extraocular muscle tissue thickness with minimal side effects. Keywords: Thyroid Eye Disease, Extraocular Muscle, Intravenous Methylprednisolone Therapy.

INTRAVENOUS CORTICOSTEROID EFFECTS ON SUPERIOR AND INFERIOR RECTUS MUSCLE IN THYROID EYE DISEASE

Abstract
 Introduction & Objectives : Intravenous corticosteroid has been used in management of Thyroid Eye Diseases (TED) which was an autoimmune inflammatory disease of the orbital tissues and the eyes. Previous study has shown benefits of intravenous corticosteroid therapy on TED but there was need of quantitative measure to assess the effect of intravenous corticosteroid therapy on extraocular muscle. This study aimed to report the effect of high-dose intravenous corticosteroid pulse therapy on superior and inferior rectus muscle and providing data distribution of active TED before and after therapy
 Methods : Retrospective analyses were used in the data collection of patients with active TED underwent high- dose intravenous methylprednisolone pulse therapy regiment 500mg every week for six consecutive weeks at Sardjito Eye Centre between January and December 2022. Ophthalmological investigation and quantitative computed tomography (CT) scan of the orbit were performed before and after corticosteroid pulse therapy and demographics data were presented in tables and graphs
 Results : This study included 62 eyes of 31 patients (61,3% female) with mean age of 37.8±13.47 years old. The mean thickness of Superior Rectus (SR) muscle before therapy were 4.12±2.20 mm and decreased to 3.64±1.50 mm post therapy. The mean thickness of Inferior Rectus (IR) muscle before therapy were 4.45±1.80 mm and decreased to 4.26±1.63 mm post therapy. There was significant difference in mean ΔSR -0.47±1.84 mm (p=0.046) but not in ΔIR -0.18±1.69 (p=0.381)
 Conclusion : The treatment of Thyroid Eye Disease (TED) with high dose intravenous corticosteroid is safe and effective in reducing extraocular muscle tissue thickness with minimal side effects.

INVESTIGATING THE INTERPLAY: UNRAVELLING THE IL-6 CONNECTION AND INTRAVENOUS METHYLPREDNISOLONE'S IMPACT ON RECTUS MUSCLE IN THYROID EYE DISEASE

Abstract
 Introduction & Objectives : Intravenous corticosteroid has been used in management of Thyroid Eye Diseases (TED). Many previous studies have shown the therapeutic benefits of corticosteroid therapy. Recently, the efficacy of high-dose intravenous corticosteroid pulse therapy has been reported. Recent studies have highlighted the potential role of interleukin-6 (IL-6) in the pathogenesis of TED. However, the correlation between IL-6 levels and the effects of IVMP on rectus muscles in patients with TED remains unclear. This study aims to investigate the correlation between IL-6 levels and the effects of IVMP on rectus muscles in patients with TED
 Methods : Retrospective analysis were used in the data collection of patients with active Thyroid Eye Disease who undergone high-dose intravenous methylprednisolone pulse therapy (500mg per week for 6 successive weeks) at Sardjito General Hospital between January 2022 and December 2022. Data on the Level of IL-6 were obtained at the start of treatment, and quantitative computed tomography (CT) of the orbit and were performed before and after treatments. Demographics data were presented in tables and graphs.
 Results : This study included 62 eyes of 31 patients (61,3% female) with mean age of 37.8+13.47 years old. There was significant inverse correlation in levels of IL-6 and ?SR (P=0.037) which implies a reduced effect of IVMP on SR. However, other statistical analyses on different rectus muscle groups bare no statistical significance.
 Conclusion : The use of IL-6 as a single predictor of responsiveness of the treatment of high dose intravenous methylprednisolone is not endorsed by the result of this study. However, further studies with better established level of IL-6 both pre and post treatment, and more inclusive parameters are needed to better provide better understanding on the role of IL-6.

INVESTIGATING THE INTERPLAY: UNRAVELLING THE IL-6 CONNECTION AND INTRAVENOUS METHYLPREDNISOLONE’S IMPACT ON RECTUS MUSCLE IN THYROID EYE DISEASE

Introduction & Objective: Intravenous corticosteroid has been used in management of Thyroid Eye Diseases (TED). Many previous studies have shown the therapeutic benefits of corticosteroid therapy. Recently, the efficacy of high-dose intravenous corticosteroid pulse therapy has been reported. Recent studies have highlighted the potential role of interleukin-6 (IL-6) in the pathogenesis of TED. However, the correlation between IL-6 levels and the effects of IVMP on rectus muscles in patients with TED remains unclear. This study aims to investigate the correlation between IL-6 levels and the effects of IVMP on rectus muscles in patients with TED Methods : Retrospective analysis were used in the data collection of patients with active Thyroid Eye Disease who undergone high-dose intravenous methylprednisolone pulse therapy (500mg per week for 6 successive weeks) at Sardjito General Hospital between January 2022 and December 2022. Data on the Level of IL-6 were obtained at the start of treatment, and quantitative computed tomography (CT) of the orbit and were performed before and after treatments. Demographics data were presented in tables and graphs. Results : This study included 62 eyes of 31 patients (61,3% female) with mean age of 37.8±13.47 years old. There was significant inverse correlation in levels of IL-6 and ?SR (P=0.037) which implies a reduced effect of IVMP on SR. However, other statistical analyses on different rectus muscle groups bare no statistical significance. Conclusion : The use of IL-6 as a single predictor of responsiveness of the treatment of high dose intravenous methylprednisolone is not endorsed by the result of this study. However, further studies with better established level of IL-6 both pre and post treatment, and more inclusive parameters are needed to better provide better understanding on the role of IL-6.

Effect of high-dose intravenous methylprednisolone pulse (IVMP) therapy in the survival of patients with anti-melanoma differentiation-associated gene 5-related rapidly progressive interstitial lung disease: a retrospective analysis.

To assess the impacts of high-dose intravenous methylprednisolone pulse (IVMP) therapy in survival and the occurrences of treatment-related infection of patients with anti-melanoma differentiation-associated gene 5 antibody-related rapidly progressive interstitial lung disease (MDA5-RPILD). Patients with MDA5-RPILD from June 2017 to August 2022 in our hospital were retrospectively reviewed. IVMP therapy was defined as intravenous methylprednisolone (mPSL) 0.5g/day for 3 consecutive days during hospitalization or 7 days prior to admission and patients were divided into IVMP group and non-IVMP group based on who had ever received IVMP therapy. All-cause mortality and the incidence of adverse events during treatment were compared between the two groups. Sixty-four patients with MDA5-RPILD were enrolled. Among them, twenty-three (35.9%) patients had ever received IVMP therapy. The overall mortality was comparable between IVMP and non-IVMP group (IVMP group: 22/23, 95.7% vs. non-IVMP group: 38/41, 92.7%, p=0.11). And the incidence of treatment-related infections was also close (IVMP group: 21/23, 91.3% vs. non-IVMP group: 32/41, 78.0%, p=0.30). After adjustment for gender, age, smoking history, duration from symptom onset to diagnosis, and combination with steroid-sparing agent treatment, the Cox proportional hazards model showed that IVMP therapy was not associated with an improved survival (adjusted HR 1.10; 95% CI 0.57-2.13; p=0.77). Our study showed that the survival benefits and adverse events were comparable between IVMP-treated and untreated MDA5-RPILD patients. Future prospective trials are needed to investigate the optimal treatment regimen in MDA5-RPILD. Key Points • This observational study found that IVMP therapy may be not associated with an improved outcome in patients with MDA5-RPILD. • Treatment-related infections are common; however, the incidence of treatment-related infections had no difference between IVMP and non-IVMP group.

Treatment Options for Infantile Spasms Syndrome with SCN8A: A Case Report and Literature Review

Abstract“Infantile spasms syndrome (IS),” previously known as “West syndrome (WS),” is characterized by epileptic spasms (ES), abnormal electroencephalography (EEG) patterns such as hypsarrhythmia, and developmental stagnation or regression in infancy. IS has various etiologies, including genetic abnormalities. SCN8A variants are associated with developmental and epileptic encephalopathy, characterized by developmental delay, seizures starting from infancy, and refractory epilepsy with multiple seizure types. However, previous studies have not focused on the treatment of IS caused by SCN8A variants. We report a case of a previously healthy boy who presented ES and developmental regression at 6 months of age. His EEG revealed hypsarrhythmia, leading to the diagnosis of IS. After admission, the patient was treated with hormonal therapy using intravenous methylprednisolone pulse therapy (MPT). ES and hypsarrhythmia on EEG disappeared in the early stages of MPT administration with no observed treatment complications. Furthermore, we observed no recurrence of EEG abnormalities or seizures at 17 months of age. Genetic testing revealed a novel de novo SCN8A variant (NM_001177984.2:c.2882T > G:p. M961R). The literature review confirmed that 13 patients, including our described patient, were reported to have ES owing to missense variants of SCN8A. While the previous articles do not mention intravenous MPT for ES with SCN8A, our case findings suggest that intravenous MPT therapy may be effective for short-term suppression of ES caused by the SCN8A variant in IS.

Juvenile dermatomyositis with central nervous system involvement: two case reports from a retrospective single-center cohort, with literature review.

Juvenile dermatomyositis (JDM) is a systemic autoimmune disease primarily involving the muscles and skin; it can also affect the central nervous system (CNS). The relevant literature provides limited information regarding the characteristics of JDM with CNS involvement. We reviewed patients with JDM who were hospitalized at our center between January 2016 and August 2023, with a focus on those with CNS involvement. The aim was to provide detailed case reports on these patients, and to summarize the relevant literature about the characteristics of similar cases. Among 193 hospitalized patients with JDM, two (1.03%) had CNS involvement. Two patients, a 5.5-year-old girl and an 11-year-old boy, were admitted with severe proximal muscle weakness and seizures, and presented with active cutaneous vasculitis. Both were ultimately diagnosed with JDM, with CNS involvement. Both patients had confirmed presence of anti-NXP2 antibody through myositis-specific antibody analysis. Additionally, they all exhibited hyperferritinemia and thrombocytopenia. Salvage therapies like intravenous methylprednisolone (IVMP) pulse therapy and/or plasma exchange were administered successfully. At final follow-up, both patients had achieved complete clinical response and full neurological recovery. Our literature review identified nine similar case studies. CNS involvement usually occurred within the first 10 months of the disease course, and most of these patients had fatal outcomes, with a mortality rate of 66.6% (6/9). Including the two patients described herein, the median age for disease onset is 10.5 years (range 4-17 years), and the male: female ratio is 6:5. Seizures are the most common neurological symptom, accompanied by active cutaneous vasculitis. The brain biopsies showed two distinct pathological presentations: one was central nervous system vasculitis, and the other was cerebral macrophage activation syndrome. CNS involvement is a rare but life-threatening JDM complication. Herein, our cases and the literature indicate that it typically occurs within the first 10 months of the disease course and manifests as seizures, often accompanied by active cutaneous vasculitis, with fatal outcomes. Timely implementation of salvage therapies, like IVMP pulse therapy and plasma exchange, may significantly impact patient outcomes.

Comparative Study of Intravenous Methylprednisolone Pulse Therapy versus Oral Prednisolone Minipulse Therapy in Progressive Vitiligo

Abstract Background: Vitiligo is an idiopathic acquired illness characterized by limited depigmented macules and patches. We aim to compare intravenous (IV) methylprednisolone pulse therapy with oral prednisolone minipulse therapy in the treatment of progressive vitiligo. Objective: The assessment of the efficacy of both treatment methods in the arrest of the progression of vitiligo and repigmentation of the existing lesions. Materials and Methods: A total of 60 patients, 30 in each group, were enrolled for the study. Each patient underwent a detailed clinical, general physical, systemic, and thorough dermatological examination. A set of routine investigations, consisting of hemoglobin%, total leukocyte count, differential leukocyte count, urine routine examination, blood sugar (fasting and postprandial), liver function tests, renal function test, serum electrolytes, Montoux test, chest X-ray posteroanterior view, and thyroid profile were carried out before the analysis. Clinical photographs were taken before the start of therapy and after each month thereafter were used for the analysis after taking written consent. Results: The age of vitiligo patients considered for the study ranged from 13 to 70 years. The ratio of male-to-female patients considered for the study was 22:38 (36.6%: 63.3%). The duration of instability in vitiligo cases studied varied from 6 months to 2 years. In both Group A and Group B, the maximum number of patients (66.7% and 86.7%, respectively) had unstable vitiligo for 6 months to 1 year. Percentage repigmentation was better in Group A (IV) than Group B (OMP). In Group A, 17 out of 20 (85%) patients had shown different degrees of repigmentation, while 20 out of 30 (66.7%) patients of Group B had shown the same. This was statistically insignificant. Types of repigmentation observed in patients were of the following types: perifollicular, marginal, and combined. In both groups, all patients showed perifollicular regimentation. Conclusions: In progressive vitiligo, it was observed that oral mini pulse with prednisolone is superior to IV methylprednisolone pulse therapy for the arrest of progression. Considering the cost, mode of administration, hospital admission, loss of man-hours, and patients’ compliance, OMP was considered simpler and cost-effective.

Neuromyelitis Optica Spectrum Disorder (Device’s Disease): - A Case Report Of Full Recovery With Corticosteroids, Therapeutic Plasmapheresis And Immunotherapy

Neuromyelitis optica is a severely disabling inflammatory disorder of autoimmune etiology of the central nervous system, mainly affecting the optic nerves and spinal cord. Here, we present a case report of neuromyelitis optica in a 28-year-old Indian woman who presented with sudden painless loss of vision with tingling followed by weakness of all four limbs with bowel involvement that developed over a period of one week, with a significant history of recent vaccination with COVISHIELD (ChAdOx1 nCoV- 19) vaccine . Clinical evaluation showed diminished vision in both eyes, which progressed to loss of power across all major muscle groups in all limbs and bowel involvement. Magnetic resonance imaging of brain with contrast was done which showed T2W/FLAIR hyperintense non enhancing signal in bilateral thalami extending caudally in mid brain on left side and in midline anterior medulla with subtle T2 hyperintense signal in body of corpus callosum, similar foci are seen in bilateral frontoparietal white matter suggestive of ADEM or demyelination. Also T2 hyperintense signal in both optic nerves [left> right] in posterior part of orbit suggestive of optic neuritis. MRI whole spine showed multiple patchy hyperintense signals on T2 and hypointensity on T1 all over cervical and dorsal vertebrae through whole segment in contiguous manner. The patient was immediately started on intravenous methylprednisolone pulse therapy followed by plasmapheresis and immunomodulator therapy. The diagnosis of neuromyelitis optica was later confirmed with strongly positive neuromyelitis optica immunoglobulin G. Since, treatment was started early patient responded very well and made full recovery. Hence, early initiation of aggressive immunosuppressive treatment is essential in such cases.

Caution to Poor Adherence With Immunosuppressant Medication That Causes Coma-Onset Autoimmune Encephalitis: A Case Report and Literature Review

Autoimmune encephalitis after liver transplantation (LT) is a rare disorder. This is because patients are usually in an immunosuppressed state after LT. Here, we report a rare case of autoantibody-negative autoimmune-encephalitis-induced coma after living-donor (LD) LT. A 45-year-old woman who underwent LDLT for primary biliary cholangitis (PBC) was brought to our hospital with the chief complaint of cognitive deficiency and an episode of memory loss. Physical examination, laboratory tests, and cerebrospinal fluid analysis revealed no significant findings. However, diffusion-weighted magnetic resonance imaging showed hyperintensity in the bilateral hippocampus. No autoantibodies associated with autoimmune encephalitis were detected. The diagnosis of antibody-negative autoimmune encephalitis was made on the basis of low immunosuppressive drug levels in the blood (indicative of poor adherence) and the presence of PBC as the autoimmune disease. The patient regained consciousness after intravenous methylprednisolone pulse therapy and plasma exchange. This case highlights that when examining patients with impaired consciousness after LDLT, it is important to consider autoimmune encephalitis as a potential diagnosis.

Duplication Cysts and Autoimmune Hemolytic Anemia: An Association or a Coincidence?

Duplication cysts are rare congenital anomalies of the gastrointestinal tract and the occurrence of multiple such cysts is an extremely rare occurrence. Autoimmune hemolytic anemia is an extremely unusual manifestation of these duplication cysts. Herein, we report a case with two duplication cysts (one cyst in the thorax; another in the abdomen) complicated with autoimmune hemolytic anemia. This patient was a five year-old-male child who presented with congestive cardiac failure due to severe anemia (which, on evaluation, was diagnosed as autoimmune hemolytic anemia). Imaging studies (chest radiograph and computed tomography) revealed duplication cysts in the chest and abdomen. Anemia flare-up was noted in the patient despite two months of prednisolone therapy (2 mg/kg/day) and two high dose intravenous methylprednisolone pulse therapy courses (30 mg/kg/day for 5 days each). The steroid refractory autoimmune hemolytic anemia responded to rituximab (375 mg/ m2 body surface area/week for 4 weeks). Four months after tapering off the steroid therapy, the patient did not experience flare-up of the anemia. To the best of our knowledge, this is the youngest and first pediatric age group patient to be reported, with a possible association between gastrointestinal duplication cysts with autoimmune hemolytic anemia.

MRI of fatal course of acute hemorrhagic leukoencephalitis in a child with SARS-CoV-2 omicron BA 2.0 infection.

We present a pediatric case of acute hemorrhagic leukoencephalitis associated with SARS-CoV-2 Omicron BA 2.0 infection. A previously healthy girl presented with ataxia and diplopia three weeks after the COVID-19 confirmation from a nasopharyngeal swab. Acute and symmetrical motor weakness and drowsiness ensued within the following 3days. She then became spastic tetraplegic. MRI revealed multifocal lesions in the cerebral white matter, basal ganglia, and brainstem, with hemorrhagic changes confirmed with T1-hyperintensity and hypointensity on susceptibility-weighted images. Peripheral areas of decreased diffusion, increased blood flow, and rim contrast enhancement were noted in the majority of lesions. She was treated with a combination of intravenous immunoglobulin and methylprednisolone pulse therapy. Neurological deterioration ensued with coma, ataxic respiratory pattern and decerebrate posture. Repeated MRI performed on day 31 revealed progression of abnormalities, hemorrhages and brain herniation. Despite the administration of plasma exchange, she died two months after admission.

504 A single-center retrospective study evaluating the efficacy of intravenous methylprednisolone pulse therapy for 113 patients with alopecia areata

504 A single-center retrospective study evaluating the efficacy of intravenous methylprednisolone pulse therapy for 113 patients with alopecia areata