Intravenous Methylprednisolone Pulse Research Articles

#2996 Diffuse alveolar haemorrhage in antiphospholipid syndrome—a rare case

Abstract Background and Aims Diffuse alveolar haemorrhage (DAH) is a serious manifestation of diseases such as systemic lupus erythematosus (SLE) and, apparently in a paradoxical manner, antiphospholipid syndrome (APS). Inflammation resulting from the deposition of immune complexes in alveolar capillaries appears to contribute to the pathophysiology of DAH, SLE and APS. Symptoms include cough, dyspnoea, haemoptysis or fever. It is associated with a drop in haemoglobin, respiratory failure and diffuse infiltrates on imaging exams. Bronchoscopy is diagnostic. It is important to exclude infection as treatment includes high-dose corticosteroid therapy and other immunosuppressive drugs. We present a challenging case regarding the diagnostic and therapeutic approach to this pathology. Case report This is the case of a 52-year-old Caucasian female, with a history of SLE, APS, splenectomy due to thrombocytopenia, IgM Kappa monoclonal gammopathy and indolent non-Hodgkin lymphoma. She was medicated with prednisolone 5 mg and warfarin (target INR between 2 and 3). The patient was observed due to a 1-day history of fever, nasal obstruction and odynophagia, associated with progressively worsening fatigue. On examination, she was pale, eupnoeic, tachycardic, with bibasal rales and a hyperaemic oropharynx. Lab workup results were the following: haemoglobin 7 g/dL (11.7 g/dL 3 months before), VGM 71 fL, presence of schistocytes, leukocytes 13.7 G/L, neutrophils 11.1 G/L, PCR 4.6 mg/dL, platelets 170 G/L, INR 3.6, aPTT 45 sec, negative D-Dimers, urea 54 mg/dL, creatinine 1.4 mg/dL, LDH 431 IU/L, normal haptoglobin, negative Coombs test. In arterial blood gas analysis, pO2 was 61 mmHg and lactate was 5 mmol/L. Chest X-ray showed bilateral basal cotton-wool infiltrates and chest CT showed bilateral ground-glass pattern. The rest of the investigation included no complement consumption, negative anti-ds-DNA antibody, positive lupus anticoagulant, anticardiolipin and anti-beta2-glycoprotein antibodies, negative cryoglobulins and no evidence of progression of the lymphoproliferative disease. She was admitted to the intensive care unit due to suspicion of atypical/viral pneumonia vs. DAH. Oxygen therapy, empirical antibiotic therapy with ceftriaxone and clarithromycin and oral methylprednisolone 1 mg/Kg/day were started. She needed blood transfusion, although without visible blood loss. Bronchofibroscopy was performed, with bronchoalveolar lavage suggestive of DAH, so it was decided to start intravenous pulses of methylprednisolone, plasmapheresis, and intravenous immunoglobulin (IVIG). Warfarin was changed to unfractionated heparin. The patient had clinical, analytical and radiological improvement. Due to the lack of evidence of infection, antibiotics were stopped after 7 days. The patient was discharged under weaning corticosteroid therapy and monthly IVIG (during 5 months), without recurrence in the following year. Conclusion This is a rare case of oligosymptomatic DAH in a patient with SLE and APS. The literature currently available on DAH in the context of SLE or APS is scarce and its treatment is mainly dependent on the experience of each medical centre. Corticosteroid therapy is consensual, with greater variability in other corticosteroid-sparing therapies. In the case of patients with APS, anticoagulation is a challenge, and it is important to resume it as soon as the bleeding is controlled due to the risk of thrombotic events.

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.

Profile of Multisystem Inflammatory Syndrome in Children (MIS-C) in Infants in North India during the Second Wave of SARS-CoV-2 Pandemic

Background: Multisystem inflammatory syndrome in children (MIS-C) secondary to severe acute respiratory syndrome coronavirus-2 has affected not only older children, adolescents, and adults but also infants, more so during the second wave of the global pandemic. Materials and Methods: All sequentially admitted infants hospitalized during 6 months who fulfilled the World Health Organization criteria for MIS-C or American Heart Association 2017 criteria for Kawasaki disease (KD) (and positive coronavirus disease-2019 [COVID-19] serology) were included in the present study. Results: A total of 19 infants were studied. Thirteen (68.3%) had evidence of recent COVID-19 infection. The median age of presentation was 2 months (interquartile range 25–75th, 0.5–3). The most common presenting symptoms were fever (68.4%), gastrointestinal complaints (63.1%), and edema (36.8%). Other predominant signs were shock (78.9%), myocarditis (52.6%), and neurological complaints (26.3%). Incomplete KD was present in 21% of patients. Elevated C-reactive protein, ferritin, D-Dimer, N-terminal prohormone of brain natriuretic peptide, and reduced fibrinogen were markers of severe illness. All subjects received intravenous immunoglobulin (IVIG) (100%), 31.5% received a second dose of IVIG, and 63.1% received pulse intravenous methylprednisolone. A total of 5 (26.3%) died as a result of the disease process. Conclusion: The spectrum of MIS-C in infants can be varied and is different from older children. A high index of suspicion is therefore needed in infants who present with critical illness and do not respond appropriately to conventional antibiotics and supportive care. The addition of IVIG and corticosteroids to the treatment regimen leads to a favorable outcome.

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.

Effectiveness of intravenous methylprednisolone pulse in patients with severe microscopic polyangiitis and granulomatosis with polyangiitis.

To evaluate the effectiveness and safety of two different intravenous methylprednisolone (IVMP) pulse doses in patients with severe microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA). We emulated a target trial using observational data from the nationwide registry in Japan. Patients with severe glomerulonephritis or diffuse alveolar haemorrhage were selected and pseudo-randomised into three groups using propensity score-based overlap weighting as follows: non-IVMP, IVMP 0.5 g/day, and IVMP 1.0 g/day. The primary outcome was all-cause death, and the secondary outcomes were composite all-cause death and kidney failure, severe relapse, and serious infection from 2 to 48 weeks after treatment initiation. To estimate the treatment effects, the Cox proportional hazard model and Fine-Gray subdistribution hazard model were used. In this emulated target trial, of 201 eligible patients (MPA, 175; GPA, 26), 6 (2.8%) died, 4 (2.0%) had kidney failure, 11 (5.3%) had severe relapse, and 40 (19.8%) had severe infections. Hazard ratios (HR) for IVMP 0.5 g/day and IVMP 1.0 g/day pulse groups compared with non-IVMP pulse were as follows: all-cause death = 0.46 (95% confidence interval [95%CI]: 0.07-2.81) and 0.07 (95%CI: 0.01-0.41); all-cause death/kidney failure = 1.18 (95%CI: 0.26-5.31) and 0.59 (95%CI: 0.08-4.52); subdistribution HRs for severe relapse = 1.26 (95%CI: 0.12-13.70) and 3.36 (95%CI: 0.49-23.29); and serious infection = 1.88 (95%CI: 0.76-4.65) and 0.94 (95%CI: 0.28-3.13). IVMP 1.0 g/day pulse may improve 48-week mortality in patients with severe MPA/GPA.

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 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.

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.

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.

Reduced Likelihood of Infarction in Crescendo Transient Ischemic Attack Caused by Vasculitic Middle Cerebral Artery Stenosis.

A 53-year-old woman was admitted to our hospital because of increasingly frequent transient speech disturbance and left upper limb weakness. Brain magnetic resonance imaging and angiography revealed multiple intracranial stenoses, including the proximal right middle cerebral artery (MCA), without evidence of infarction. The diagnosis of primary angiitis of the central nervous system was established based on circumferential vascular wall thickening with contrast enhancement observed in the right MCA. Following the administration of dual antiplatelet therapy and intravenous methylprednisolone pulse therapy, the patient experienced complete cessation of symptoms, and the stenosis gradually improved without infarction. The risk of infarct development in crescendo transient ischemic attacks may differ between inflammatory vascular stenosis and atherosclerosis.

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.