Intravenous Methylprednisolone Pulse Research Articles

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.

Wunderlich syndrome as a rare complication of polyarteritis nodosa: a case report.

Spontaneous subcapsular and perirenal hemorrhage, known as Wunderlich syndrome (WS), is a rare clinical manifestation of polyarteritis nodosa (PAN). We report a case of a 48-year-old male with a history of recurrent episodes of leg muscle tenderness and dysesthesia, bilateral flank pain, painful nodular skin lesions in the lower limbs, weight loss, and difficult-to-control arterial hypertension. The abdominopelvic computed tomography angiography showed a large left perirenal hematoma, leading to the patient's admission to the intensive care unit. After the exclusion of infectious or neoplastic foci, the patient was diagnosed with PAN and started intravenous methylprednisolone pulses with a good response. Since WS is a rare initial clinical manifestation of PAN, an early diagnosis and aggressive treatment will significantly improve clinical outcomes.

Hand knob sign in osmotic demyelinating syndrome

BackgroundOsmotic demyelinating syndrome, commonly recognized as a consequence of the rapid correction of hyponatremia, has been known to cause motor, neuropsychiatric, or extrapyramidal symptoms. We reported a patient with an unusual presentation involving bilateral hand weakness, and pseudobulbar affect. The imaging was compatible with osmotic demyelinating syndrome with bilateral hand knob lesions, despite no history of overcorrection of hyponatremia.Case presentationA 44-year-old female presented with three weeks of emotional lability, spastic dysarthria, and bilateral hand weakness following ankle surgery and a mild head injury. Physical examination revealed weakness in the intrinsic hand muscles, leading to a claw-like deformity of the hands, although sensation remained unimpaired. Magnetic resonance imaging (MRI) of the brain revealed several hyperintensities on fluid-attenuated inversion recovery imaging involving various areas, including the hand knob area of the bilateral precentral gyri, caudate, lentiform nuclei, and pons, suggestive of osmotic demyelinating syndrome. Clinical improvement was observed following a trial of intravenous pulse methylprednisolone and plasmapheresis.ConclusionsBilateral hand weakness is an unusual manifestation of osmotic demyelinating syndrome. The precentral gyrus, specifically in the hand knob area, is the vulnerable region that can result from osmotic demyelinating syndrome.

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.

Glucocorticoid-induced adrenal insufficiency after therapy with intravenous methylprednisolone in patients with moderate-to-severe and active Graves' orbitopathy: assessment with a low-dose corticotropin test.

We aimed to assess adrenal function following treatment of moderate-to-severe and active Graves' orbitopathy (GO) with intravenous methylprednisolone (IVMP) in weekly pulses in a cumulative dose of 4.5 or 7.5g. We evaluated the impact of IVMP pulses on adrenal reserve using a low-dose (1μg) ACTH stimulation test (LDT) for the first time. In this prospective study we evaluated adrenal function in 21 patients with moderate-to-severe and active GO treated with 12 weekly IVMP pulses according to the European Group on Graves' Orbitopathy (EUGOGO) recommendations. We assessed serum cortisol, plasma adrenocorticotropic hormone (ACTH), and dehydroepiandrosterone sulfate (DHEA-S) levels before the 1st and 12th IVMP pulse. We performed dynamic testing using LDT before the 12th IVMP pulse in all patients. In those who failed LDT, adrenal function was reassessed with LDT and the overnight metyrapone test after 4-7weeks. Two patients failed to achieve serum cortisol levels ≥ 18.1μg/dL at 30 and 60min in LDT and were diagnosed with glucocorticoid-induced adrenal insufficiency (GC-induced AI). They were recommended to take hydrocortisone in situations of acute stress. Both patients were reassessed within 4-7weeks after treatment cessation and showed an adequate response in LDT and overnight metyrapone test. We observed a statistically significant decrease in DHEA-S levels (p = 0.004) before the 12th IVMP pulse compared to baseline in all patients. For the first time, our research shows that administering IVMP in 12 weekly pulses can result in GC-induced AI. We suggest that patients should undergo careful evaluation for GC-induced AI, including LDT, after therapy with IVMP according to EUGOGO guidelines. Screening for altered adrenal reserve could prevent life-threatening complications, particularly during acute stress situations.

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.

Macular blood flow changes in dysthyroid optic neuropathy after high-dose intravenous pulse methylprednisolone (IVMP)

Objective: To investigate the changes in macular ໿vessel density (VD) of the superficial layer of retina (SLR) and deep layer of retina (DLR) in dysthyroid optic neuropathy (DON) after high-dose intravenous pulse methylprednisolone (IVMP). Materials and Methods: Eighteen DON patients (29 eyes) who completed high-dose IVMP and 16 healthy individuals (32 eyes) were enrolled in this study. Optical coherence tomography angiography (OCTA) image analysis and comprehensive ophthalmic examinations were performed, including the SLR macular whole-image VD (SLR-mwiVD) and DLR-mwiVD, best-corrected visual acuity (BCVA), the mean deviation of visual field (VF-MD), pattern standard deviation of visual field (VF-PSD) and the other parameters. Results: The SLR-mwiVD (41.39 ± 4.71 vs. 48.13 ± 3.68, p < 0.001) and DLR-mwiVD (40.77 ± 5.85 vs. 49.14 ± 7.02, p < 0.001) were decreased in DON compared to control eyes. After IVMP, visual function parameters were improved, and SLR-mwiVD (49.41 ± 3.18, p < 0.001) and DLR-mwiVD (50.41 ± 4.04, p < 0.001) were increased in the DON group compared to pretreatment. The increased SLR-mwiVD and DLR-mwiVD were significantly correlated with improvements in BCVA (Log MAR: from 0.62 ± 0.49 to -0.01 ± 0.03, p < 0.001), VF-MD (from – 6.89 ± 2.89 dB to – 1.75 ± 1.29 dB, p < 0.001) and VF-PSD (from 4.38 ± 2.52 dB to 2.32 ± 1.64 dB, p < 0.001). Conclusion: The increase in macular VD was significantly correlated with the improvement in visual function in DON after IVMP. Macular VD changes on OCTA may be a useful indicator for the response in DON after IVMP.

Anaphylactic reaction to intravenous pulse methyl prednisolone in a patient with multiple sclerosis

Anaphylactic reaction to intravenous pulse methyl prednisolone in a patient with multiple sclerosis

Optic neuritis in Turkish children and adolescents: A multicenter retrospective study

BackgroundVarious etiologies may underlie optic neuritis, including autoantibody-mediated disorders described in the last decade. We re-examined demographic, clinical, laboratory features and prognostic factors in pediatric patients with autoimmune optic neuritis according to current knowledge. MethodsCases of pediatric ON from 27 centers in Türkiye diagnosed between 2009 and 2022 were included for retrospective evaluation. ResultsThe study included 279 patients, 174 females and 105 males, with a female-to-male ratio of 1.65. The average age at onset was 12.8 ± 3.4 years, and mean follow-up, 2.1 years (range: 1–12.1 years). Patients <10 years old were grouped as "prepubertal" and those ≥10 years old as "others”. The diagnoses made at the end of follow-up were multiple sclerosis associated optic neuritis (n = 90, 32.3 %), single isolated optic neuritis (n = 86, 31 %), clinically isolated syndrome (n = 41, 14.7 %), myelin oligodendrocyte glycoprotein antibody associated optic neuritis (n = 22, 7.9 %), and relapsing isolated optic neuritis (n = 18, 6.5 %). Predominant diagnoses were myelin oligodendrocyte glycoprotein antibody associated optic neuritis and acute disseminated encephalomyelitis associated optic neuritis in the prepubertal group and multiple sclerosis associated optic neuritis in the older group. Recurrences were observed in 67 (24 %) patients, including 28 with multiple sclerosis associated optic neuritis, 18 with relapsing isolated optic neuritis, 11 with myelin oligodendrocyte glycoprotein antibody associated optic neuritis, 8 with aquaporin-4 antibody related optic neuritis, and 2 with chronic relapsing inflammatory optic neuropathy. Recurrences were more common among female patients. Findings supporting the diagnosis of multiple sclerosis included age of onset ≥ 10 years (OR=1.24, p = 0.027), the presence of cranial MRI lesions (OR=26.92, p<0.001), and oligoclonal bands (OR=9.7, p = 0.001). Treatment in the acute phase consisted of intravenous pulse methylprednisolone (n = 46, 16.5 %), pulse methylprednisolone with an oral taper (n = 212, 76 %), and combinations of pulse methylprednisolone, plasmapheresis, or intravenous immunoglobulin (n = 21, 7.5 %). Outcome at 12 months was satisfactory, with 247 out of 279 patients (88.5 %) demonstrating complete recovery. Thirty-two patients exhibited incomplete recovery and further combination treatments were applied. Specifically, patients with relapsing isolated optic neuritis and aquaporin-4 antibody related optic neuritis displayed a less favorable prognosis. ConclusionOur results suggest optic neuritis is frequently bilateral in prepubertal and unilateral in peri‑ or postpubertal patients. Age of onset 10 or older, presence of oligoclonal bands, and brain MRI findings reliably predict the development of multiple sclerosis. The risk of developing multiple sclerosis increases mostly during the second and third years of follow-up. Relapsing isolated optic neuritis remains a separate group where the pathogenesis and outcome remain unclear. Investigation of predisposing and diagnostic biomarkers and long follow-up could help to define this group.

Long-term Outcomes after Steroid Pulse Therapy in Patients with Type 1 Autoimmune Pancreatitis.

Objective Steroid pulse therapy is a regimen involving the intravenous administration of supra-pharmacological doses of corticosteroids in the short term. It is used to treat various inflammatory and autoimmune conditions. However, the strengths and limitations of steroid pulse therapy for induction of remission in type 1 autoimmune pancreatitis (AIP) are unknown. Methods Depending on the steroid therapy regimen administered, the 104 patients with type 1 AIP included in this retrospective study were divided into three groups: conventional oral prednisolone (PSL) regimen (PSL group), intravenous methylprednisolone (IVMP) pulse followed by oral PSL regimen (Pulse+PSL group), and IVMP pulse-alone regimen (Pulse-alone group). We then examined the relapse rate and adverse events among the three groups. Results The Kaplan-Meier estimates of the relapse rate at 36 months after steroid therapy were 13.6% in the PSL group, 13.3% in the Pulse+PSL group, and 46.2% in the Pulse-alone group. The log-rank test revealed that the relapse-free survival in the Pulse-alone group was significantly shorter than that in the PSL (p=0.024) and Pulse+PSL groups (p=0.014). The exacerbation of glucose tolerance after steroid therapy was less frequently observed in the Pulse-alone group (0%) than in the PSL group (17%, p=0.050) and Pulse+PSL groups (26%, p=0.011). Conclusion Although treatment with IVMP pulse alone resulted in unsatisfactory relapse prevention outcomes compared with conventional steroid therapy, the IVMP pulse-alone regimen might be an alternative treatment strategy for type 1 AIP from the perspective of avoiding adverse events from steroids.

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.

P08 Appearance matters: a case of adult-onset Parry-Romberg syndrome with linear scleroderma en coup de sabre and neurological involvement

Abstract Introduction We describe the case of an adult patient who initially presented with neurological symptoms. Investigations were suggestive of a neuroinflammatory disorder but the diagnosis was unclear. It was only as time passed that the cutaneous signs and symptoms of Parry-Romberg syndrome (PRS) and linear scleroderma en coup de sabre (LSCS) became apparent to clinicians as the underlying cause for her presentation. Case description This female patient, with no past medical history, presented in 2015 when she was 43 years old. She reported a one-day history of malaise and lethargy, left-sided headache, numbness affecting the right face and right arm and dysphasia. Her symptoms gradually improved over the following few days. An MRI brain showed extensive T2 hyperintensity in the right frontal and frontoparietal regions. Cerebrospinal fluid (CSF) showed a lymphocytosis and oligoclonal bands, which were present in the CSF only. A repeat MRI brain a few weeks later showed slightly less extensive changes. This was treated by neurologists as a neuroinflammatory disorder with intravenous methylprednisolone pulses. At follow-up, she mentioned that she had noticed a change in her appearance, with the right side of her face becoming increasingly asymmetrical over the last few years. Neurologists were puzzled regarding the diagnosis, but given that she was almost asymptomatic at review, they decided to monitor her with serial MRI scans. There were no further neurological episodes and no interval changes on repeat scans. In 2020, she was referred to ophthalmology, as she had noticed that her right eye had become increasingly sunken. MRI of the orbits and sinuses was reported to show enophthalmos of no obvious cause. Around the same time, she was also referred to dermatology for an enlarging patch of alopecia over the right frontoparietal scalp. Autoimmune screening blood tests including ANA, ENA, dsDNA and centromere were negative. Scalp biopsy was inconclusive but was most in keeping with old alopecia areata. She was treated with intralesional steroids and had a partial response. It was only by 2021 that PRS with LSCS was recognised as the cause of her symptoms. We can be confident that her neuroinflammation is an extracutaneous manifestation, which occurred early on when cutaneous signs were subtle. Discussion PRS is a rare, acquired disorder characterised by progressive unilateral atrophy of facial skin, soft tissue, muscles, and underlying bony structures. LSCS is a subtype of linear scleroderma that occurs on the forehead or frontoparietal scalp. PRS and LSCS commonly coexist, and it is debated whether they are on a spectrum of the same disease. Both usually occur in childhood or adolescence, and adult-onset disease is very rare. The diagnoses are clinical and there are no specific diagnostic tests. Both are associated with neurological involvement, including seizures, headaches, focal neurological deficits and neuropsychiatric changes. Usually, the cutaneous lesion develops months to years before the onset of neurological symptoms. Our patient had noticed subtle changes in her appearance at least five years before her presentation in 2015. Neuroimaging abnormalities are predominately ipsilateral to the cutaneous lesions but may rarely be contralateral. Key learning points Given that adult-onset PRS and LSCS are very rare, there may be a lack of awareness of the conditions amongst adult physicians. This can lead to diagnostic delay. This case highlights that PRS and LSCS are not just conditions of the skin and connective tissues and that neurological involvement is a frequent extracutaneous manifestation. This case is also a reminder of the importance of a thorough examination of the skin, especially when the diagnosis is unclear.

BOB-ACG study: Pulse methylprednisolone to prevent bilateral ophthalmologic damage in giant cell arteritis. A multicentre retrospective study with propensity score analysis

IntroductionGiant cell arteritis (GCA) is complicated in 10 to 20% of cases by permanent visual ischemia (PVI). International guidelines advocate the use of intravenous pulse of methylprednisolone from 250 to 1000mg per day, for three days, followed by oral prednisone at 1mg/kg per day. The aim of this study is to assess whether this strategy significantly reduces the risk of early PVI of the second eye, compared with direct prednisone at 1mg/kg per day. MethodsWe conducted a multicentre retrospective observational study over the past 15 years in 13 French hospital centres. Inclusion criteria included: new case of GCA; strictly unilateral PVI, prednisone at dose greater than or equal to 0.9mg/kg per day; for the intravenous methylprednisolone (IV-MP) group, total dose between 900 and 5000mg, close follow-up and knowledge of visual status at 1 month of treatment, or earlier, in case of contralateral PVI. The groups were compared on demographic, clinical, biological, iconographic, and therapeutic parameters. Statistical analysis was optimised using propensity scores. ResultsOne hundred and sixteen patients were included, 86 in the IV-MP group and 30 in the direct prednisone group. One patient in the direct prednisone group and 13 in the IV-MP group bilateralised, without significant difference between the two strategies (3.3% vs 15.1%). Investigation of the association between IV-MP patients and contralateral PVI through classical logistic regression, matching or stratification on propensity score did not show a significant association. Weighting on propensity score shows a significant association between IV-MP patients and contralateral PVI (OR=12.9 [3.4; 94.3]; P<0.001). Improvement in visual acuity of the initially affected eye was not significantly associated with IV-MP (visual acuity difference 0.02 vs –0.28 LogMar), even in the case of early management, i.e., within the first 48hours after the onset of PVI (n=61; visual acuity difference –0.11 vs 0.25 LogMar). Complications attributable to corticosteroid therapy in the first month were significantly more frequent in the IV-MP group (31.8 vs 10.7%; P<0.05). DiscussionOur data do not support the routine use of pulse IV-MP for GCA complicated by unilateral PVI to avoid bilateral ophthalmologic damage. It might be safer to not give pulse IV-MP to selected patients with high risks of glucocorticoids pulse side effects. A prospective randomised multicentre study comparing pulse IV-MP and prednisone at 1mg/kg per day is desirable.

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&gt; 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.