High-dose Intravenous Corticosteroids Research Articles

549VP High dose intravenous corticosteroids in refractory immune checkpoint inhibitor-related myocarditis/myositis/myasthenia overlap syndrome

549VP High dose intravenous corticosteroids in refractory immune checkpoint inhibitor-related myocarditis/myositis/myasthenia overlap syndrome

Effect of Corticosteroid Use on the Occurrence and Progression of Osteonecrosis of the Femoral Head: A Nationwide Nested Case-Control Study

BackgroundAlthough it is very well known that corticosteroids cause osteonecrosis of the femoral head (ONFH), it is unclear as to which patients develop ONFH. Additionally, there are no studies on the association between corticosteroid use and femoral head collapse in ONFH patients. We aimed to investigate the association between corticosteroid use and the risk of ONFH among the general population and what factors affect ONFH occurrence. Additionally, we aimed to demonstrate which factors affect femoral head collapse and total hip arthroplasty (THA) after ONFH occurrence. MethodsA nationwide, nested case-control study was conducted with data from the National Health Insurance Service Physical Health Examination Cohort (2002 to 2019) in the Republic of Korea. We defined ONFH (N = 3,500) using diagnosis and treatment codes. Patients who had ONFH were matched 1:5 to form a control group based on the variables of birth year, sex, and follow-up duration. Additionally, in patients who have ONFH, we looked for risk factors for progression to THA. ResultsCompared with the control group, ONFH patients had a low household income and had more diabetes, hypertension, dyslipidemia, and heavy alcohol use (drinking more than 3 to 7 drinks per week). Systemic corticosteroid use (≥1,800 mg) was significantly associated with an increased risk of ONFH incidence. However, lipid profiles, corticosteroid prescription, and cumulative doses of corticosteroid did not affect the progression to THA. ConclusionsThe ONFH risk increased rapidly when cumulative prednisolone use was ≥1,800 mg. However, oral or high-dose intravenous corticosteroid use and cumulative dose did not affect the prognosis of ONFH. Since the occurrence and prognosis of ONFH are complex and multifactorial processes, further study is needed.

A Chinese survey of clinical practice on the management of thyroid eye disease.

The management of thyroid eye disease (TED) has undergone significant changes for decades. The study sought to investigate current clinical practice on the management of TED in China. An online questionnaire survey was conducted from April to May 2023. The questionnaire involved diagnostic criteria for TED, multidisciplinary treatment (MDT) collaboration, and treatment preference for mild, moderate, and severe TED. A total of 289 questionnaires were collected, with 165 from endocrinologists and 124 from ophthalmologists. Only 36.7% of participants claimed there was an MDT clinical pattern for TED in their institutions. The coverage of biological agents was around 10% or lower. These were distinctly lower than in Western countries. About 62.6% of participants believed the incidence of TED has increased in recent years. Imaging techniques were used widely to assist in the diagnosis of TED. However, there was still controversy regarding the definition of proptosis in the Chinese population. Most doctors managed risk factors and provided orbital supportive treatments of artificial tears and glasses. For mild active TED, endocrinologists (39.4%) were inclined to recommend therapy for hyperthyroidism alone, while ophthalmologists (43.6%) preferred orbital corticosteroid injections. Currently, the most widely used treatment for moderate to severe active TED was high-dose intravenous corticosteroid (94.8%), while orbital radiotherapy combined with immunosuppressive agents was the most recognized second-line therapy (43.6%). The study documented the consistency and differences between current clinical practices in the management of TED in China and the recently updated guidelines. There was a remarkable difference between ophthalmology and endocrinology departments, warranting management optimization.

Corticosteroid treatment for acute hydrocephalus in neurosarcoidosis: a case report

BackgroundNeurosarcoidosis occurs symptomatically in 5–10% of patients with sarcoidosis, and hydrocephalus is a rare complication of neurosarcoidosis, with either acute or subacute onset and presenting symptoms related to increased intracranial pressure. It represents a potentially fatal manifestation with a mortality rate of 22% (increased to 75% in case of coexistence of seizures) that requires a prompt initiation of treatment. High-dose intravenous corticosteroid treatment and neurosurgical treatment must be considered in all cases of neurosarcoidosis hydrocephalus.Case presentationHere we present a case of hydrocephalus in neurosarcoidosis, complicated by generalized seizures, in a 29-year-old Caucasian male patient treated with medical treatment only, with optimal response.ConclusionSince neurosurgery treatment can lead to severe complications, this case report underlines the possibility to undergo only medical treatment in selected cases. Further studies are needed to stratify patients and better identify those eligible for only medical approach.

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.

Neuromyelitis Optica Spectrum Disorder (NMOSD) or Chronic-Relapsing Inflammatory Optic Neuropathy (CRION)?: A Challenge in Diagnosis and Treatment

Abstract
 Introduction : Neuromyelitis optica spectrum disorder (NMOSD) is a chronic inflammatory, immune-mediated central nervous system disease targeting the AQP4-water channel membrane protein of the optic nerve and the spinal cord. This case report aims to present a case of seroconverting NMOSD which was initially diagnosed as chronic relapsing inflammatory optic neuropathy (CRION).
 Case Illustration : 15-year-old female with recurrent, steroid-dependent optic neuritis of the LE. During the first attack, brain MRI showed optic nerve inflammation with normal brain findings. Initial test for AQP4-IgG, MOG-Ab and ANA profile were negative. The diagnosis of CRION was made. Treatment with corticosteroid and immunosuppressant agent was maintained for one year. After treatment cessation, however, LE optic neuritis recurred with bilateral lower limb weakness. Spinal MRI showed signs of longitudinal extensive transverse myelitis. Re-testing for AQP4-IgG showed positive result. Diagnosis of NMOSD was made, and the patient was treated with high-dose intravenous corticosteroid followed by oral dose, and immunosuppressant. Final visual acuity of the LE was 1.0, with minimal residual visual field defect and good motor function.
 Discussion : During the initial attack, the patient did not meet the 2015 NMOSD international consensus diagnostic criteria. The AQP4-IgG was negative and no other corresponding signs and symptoms. The criteria was met one year after: optic neuritis, LETM and positive seroconversion of AQP4-IgG.
 Conclusion : Diagnosing NMOSD is at times challenging especially during initial attack. Retesting of the AQP4- IgG is at times necessary in seronegative patients, especially in children showing ADEM-like event with optic neuritis and LETM to achieve diagnosis and proper treatment strategy.

A peculiar case of idiopathic orbital myositis in adolescence

Abstract
 Introduction : Idiopathic orbital myositis, an inflammation localized at the extraocular muscles, is rarely reported in pediatrics. Until now, the prevalence and incidence of orbital myositis remain unknown. This paper aims to report a case of idiopathic orbital myositis in a young girl with headache.
 Case Illustration : A-16-year-old presented with a droopy eyelid of the left eye (LE) and double vision since 1 week before admission. She also experienced swollen eyelid and cluster headache for 5 months with no neurological abnormalities. Ophthalmic examinations showed exotropia with limited eye movement of LE. Ptosis of LE was noted with margin reflex distance 1 (MRD-1) +2 mm, while MRD-1 +6 mm for the right eye. Other examinations were unremarkable with normal funduscopy and negative RAPD. Non-contrast orbital sinus CT-Scan revealed an enlargement of the superior rectus and lateral rectus muscle. The laboratory result showed slight leucocytosis and normal ESR. High-dose intravenous corticosteroid was administered and tapered with oral steroid. The orbital clinical findings showed improvements, better ocular movements, and diminished headache complaints.
 Discussion : As a rare case, the diagnosis of orbital myositis in adolescence can be challenging and requires detailed examination, and proper imaging to exclude other diagnosis such as ophthalmoplegic migraine, infections, malignancies, or congenital lesions (hemangioma). The first-line therapy is a systemic corticosteroid, which well responded in this patient.
 Conclusion : In this patient, intravenous corticosteroid represent an effective treatment. Close monitoring of the disease progression is required after switching to oral therapy to prevent relapse.

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.

Analysis of 9 cases of drug induced hypersensitivity syndrome related hemophagocytic lymphohistiocytosis

Objective: To analyze the clinical features,treatment and prognosis of drug induced hypersensitivity syndrome related hemophagocytic lymphohistiocytosis (DIHS-HLH). Methods: This was a retrospective case study. Clinical characteristics, laboratory results, treatment and prognosis of 9 patients diagnosed with DIHS-HLH in Beijing Children's hospital between January 2020 and December 2022 were summarized. Kaplan-Meier survival analysis was used to calculate the overall survival rate. Results: Among all 9 cases, there were 6 males and 3 females, with the age ranged from 0.8 to 3.1 years. All patients had fever, rash, hepatomegaly and multiple lymph node enlargement. Other manifestations included splenomegaly (4 cases), pulmonary imaging abnormalities (6 cases), central nervous system symptoms (3 cases), and watery diarrhea (3 cases). Most patients showed high levels of soluble-CD25 (8 cases), hepatic dysfunction (7 cases) and hyperferritinemia (7 cases). Other laboratory abnormalities included hemophagocytosis in bone marrow (5 cases), hypofibrinogenemia (3 cases) and hypertriglyceridemia (2 cases). Ascending levels of interleukin (IL) 5, IL-8 and interferon-γ (IFN-γ) were detected in more than 6 patients. All patients received high dose intravenous immunoglobulin, corticosteroid and ruxolitinib, among which 4 patients were also treated with high dose methylprednisolone, 2 patients with etoposide and 2 patients with cyclosporin A. After following up for 0.2-38.6 months, 7 patients survived, and the 1-year overall survival rate was (78±14)%. Two patients who had no response to high dose immunoglobulin, methylprednisolone 2 mg/(kg·d) and ruxolitinib died. Watery diarrhea, increased levels of IL-5 and IL-8 and decreased IgM were more frequently in patients who did not survive. Conclusions: For children with fever, rash and a suspicious medication history, when complicated with hepatomegaly, impaired liver function and high levels of IL-5 and IL-8, DIHS-HLH should be considered. Once diagnosed with DIHS-HLH, suspicious drugs should be stopped immediately, and high dose intravenous immunoglobulin, corticosteroid and ruxolitinib could be used to control disease.

Fatal iatrogenic cerebral β-amyloid-related arteritis in a woman treated with lecanemab for Alzheimer’s disease

We report the case of a 79-year-old woman with Alzheimer’s disease who participated in a Phase III randomized controlled trial called CLARITY-AD testing the experimental drug lecanemab. She was randomized to the placebo group and subsequently enrolled in an open-label extension which guaranteed she received the active drug. After the third biweekly infusion, she suffered a seizure characterized by speech arrest and a generalized convulsion. Magnetic resonance imaging revealed she had multifocal swelling and a marked increase in the number of cerebral microhemorrhages. She was treated with an antiepileptic regimen and high-dose intravenous corticosteroids but continued to worsen and died after 5 days. Post-mortem MRI confirmed extensive microhemorrhages in the temporal, parietal and occipital lobes. The autopsy confirmed the presence of two copies of APOE4, a gene associated with a higher risk of Alzheimer’s disease, and neuropathological features of moderate severity Alzheimer’s disease and severe cerebral amyloid angiopathy with perivascular lymphocytic infiltrates, reactive macrophages and fibrinoid degeneration of vessel walls. There were deposits of β-amyloid in meningeal vessels and penetrating arterioles with numerous microaneurysms. We conclude that the patient likely died as a result of severe cerebral amyloid-related inflammation.

Relentless Placoid Chorioretinitis Responsive to Intravitreal Triamcinolone and Systemic Adalimumab

Purpose: We describe a case of relentless placoid chorioretinitis that was responsive to intravitreal triamcinolone (IVT) and systemic adalimumab, but suboptimally responsive to oral prednisone. Methods: Interventional case report. Results: A 23-year-old woman presented with macular placoid chorioretinitis in the left eye and visual acuity of finger counting at 3 feet. Despite a 2-week course of 60mg oral prednisone, the choroiditis expanded dramatically in the mid-periphery. She was admitted to the hospital for high-dose intravenous corticosteroids, which stopped progression of disease. After discharge, the patient was started on adalimumab and restarted oral prednisone, however upon resumption of oral prednisone 60mg, the disease started to progress again over the next 2 weeks. An intravitreal injection of triamcinolone (IVT) 2mg was given, which halted progression of the disease for at least 9 weeks, at which time it was felt that the adalimumab started to also have an effect. Visual acuity 9 weeks after IVT had improved to 20/20. The patient was lost to follow-up after this point. Conclusion: This is a case of relentless placoid chorioretinitis that continued to progress despite high-dose oral corticosteroids, but which responded to intravitreal triamcinolone and systemic adalimumab. This indicates that intravitreal corticosteroids may represent an alternative treatment option to oral corticosteroids in the short-term management of macula-threatening non-infectious chorioretinitis, particularly in patients with a sub-optimal response to oral corticosteroids.

A rare presentation of acute myocarditis as a manifestation of adult-onset Still’s disease: a case report

Abstract Background Adult-onset Still’s disease (AOSD) is a rare systemic autoinflammatory condition characterized by a classical triad of symptoms that include prolonged fever, polyarthritis, and a characteristic salmon-pink skin rash. It can affect a variety of organ systems resulting in many different clinical presentations and is usually a diagnosis of exclusion. Myocarditis complicated by cardiogenic shock is a rare and life-threatening manifestation of AOSD, typically affecting younger patients. There is a limited experience and evidence in how best to manage this challenging patient cohort. Case summary A previously fit and well 22-year-old male presented with fever, arthralgia, and general malaise. On clinical examination, he was pyrexial and hypotensive, requiring vasopressor support for presumed septic shock. Subsequent transthoracic echocardiography and cardiac MRI findings were in keeping with fulminant myocarditis. Further septic and auto-immune screens were negative although he responded well to high-dose intravenous corticosteroids. Attempts to wean immunosuppression were unsuccessful, and his ferritin was markedly elevated (20 233 μg/L). A diagnosis of AOSD was suspected after exclusion of other possible causes. The successful addition of tocilizumab (an interleukin-6 receptor antagonist) therapy allowed for gradual de-escalation of steroid therapy and disease remission, with on-going remission at 18 months on maintenance therapy. Discussion This case highlights the importance of considering AOSD as a rare cause for myocarditis, especially when fever is present, or disease is severe. Failure to improve with first-line therapy involving high-dose corticosteroids, or inability to wean that therapy, should prompt consideration for escalation of therapy, with tocilizumab seemingly an effective treatment option.

Diagnosis and Treatment of Neuro-Behçet: A Clinical Update

Behçet's disease is a relapsing multisystemic inflammatory syndrome characterized by recurrent oral and/or genital ulcers, uveitis, arthritis, skin lesions, and gastrointestinal and neurological involvement. Neuro-Behçet corresponds to nervous system involvement and is one of the most severe complications of Behçet disease. It occurs in 3% to 30% of cases and is categorized into parenchymal (most common) or non-parenchymal disease. The most common manifestation of parenchymal neuro-Behçet is meningoencephalitis with involvement of the brainstem, where patients present with cranial neuropathies, encephalopathy, sensory-motor syndromes, epilepsy, or myelitis. The main non-parenchymal manifestation is cerebral venous thrombosis. Neuro-Behçet has a predominantly subacute course, with remission within weeks, or clinical progression in one third of the cases. The diagnosis is essentially clinical and diagnostic tests help to corroborate the suspicion, distinguish from differential diagnoses, and exclude complications. Brain magnetic resonance imaging allows the identification of acute lesions (hypointense or isointense on T2-weighted and hypointense on T1-weighted sequences) contrast-enhanced, and chronic lesions characterized by non-contrast enhanced small lesions and brainstem atrophy. If non-parenchymal involvement is suspected, cerebral veno-magnetic resonance imaging /computed tomography should be performed. Cerebrospinal fluid shows elevated proteinorachia and pleocytosis in parenchymal and no changes in non-parenchymal neuro-Behçet (except increased opening pressure). Outbursts of parenchymal disease should be treated with high dose intravenous corticosteroid therapy, with subsequent switch to oral corticoids, followed by biologic therapy, usually an anti-TNF. The treatment of cerebral venous thrombosis is controversial and may consist of a combination of corticosteroids and anticoagulation.

A Case Report on Pityriasis Rubra Pilaris (PRP) and Multisystem Inflammatory Syndrome in Children (MIS-C)

Pityriasis rubra pilaris (PRP) is a rare inflammatory papulosquamous skin disease of unknown etiology. There are six distinct types of PRP. A higher incidence was reported within the pediatric group, but treatment remains largely empiric, based on case reports and series. The condition is often refractory to treatment, especially initially, with topical corticosteroids and oral Acitretin the more commonly employed agents. biologic agents approved for psoriasis have been used with good effect in PRP. Multisystem Inflammatory Syndrome in Children (MIS-C) is a new disease entity that evolved as a result of the coronavirus disease 2019 (COVID-19) pandemic. Due to our incomplete understanding of the occurrence of MISC, new diagnostic and therapeutic issues have emerged. The American college of rheumatology (ACR) has recommended the use of intravenous immunoglobulin (IV IG) and/or high-dose corticosteroids as first-line treatment in these patients. In some cases of severe patients may receive such as a second dose of IV IG, Anakinra or Infliximab and Aspirin. Here we report the case of a 4-year-old boy presented with rashes. We describe the successful use of IV IG, corticosteroids, and Aspirin for treatment of MIS-C and PRP. Keywords: Pityriasis rubra pilaris, MIS-C, IV IG

Acute Severe Myocarditis as the Presenting Complaint of Adult-Onset Still's Disease: A Case Report

Adult-onset Still’s disease (AOSD) is a rare systemic autoinflammatory condition characterised by a classical triad of symptoms: prolonged fever, polyarthritis, and a characteristic salmon-pink skin rash. It can affect a variety of organ systems, resulting in many different clinical presentations. Myocarditis complicated by cardiogenic shock is a rare and life-threatening manifestation of AOSD, typically affecting younger patients. There is limited experience and evidence in how to best manage this challenging patient cohort. Case: A previously fit and healthy 22-year-old male presented with fever, arthralgia, and general malaise. On clinical examination he was pyrexial and hypotensive, requiring vasopressor support for presumed septic shock. Subsequent transthoracic echocardiography and cardiac magnetic resonance imaging findings were in keeping with fulminant myocarditis. Further septic and autoimmune screens were negative, although he responded well to high-dose intravenous corticosteroids. Attempts to wean immunosuppression were unsuccessful. His ferritin was markedly elevated (20,233 ug/L), prompting haematological and rheumatological opinions. A diagnosis of AOSD was suspected using Yamaguchi criteria. The successful addition of tocilizumab (an interleukin-6 receptor antagonist) therapy enabled gradual de-escalation of steroid therapy with ongoing disease remission at 6 months. This case highlights the importance of considering AOSD as a rare cause of myocarditis, especially when fever is present or disease is severe. Failure to improve with first-line therapy involving high-dose corticosteroids, or inability to wean that therapy, should prompt consideration for escalation of therapy, with tocilizumab seemingly being an effective treatment option.

Paediatric optic neuritis: experience from a tertiary referral centre in Malaysia

Background: Optic neuritis typically presents with acute or subacute onset of mild or profound blurring of vision. There are very limited reports regarding optic neuritis in the paediatric population compared to adults from the South Asian region. We report a series of 7 cases of paediatric optic neuritis.
 Methods: All optic neuritis cases aged less than 12 years old in 2016 were studied retrospectively.
 Results: Out of 44 patients with optic neuritis, 7 of them were of paediatric age. The mean age was 9.9 years. All patients had sudden onset profound vision loss (range 3/60 to hand movement). Four patients had bilateral involvement, all had reduced colour vision. Three had underlying acute disseminated encephalomyelitis. All were treated with high-dose intravenous corticosteroids. Five patients made full recovery, one patient had partial recovery, and one patient had no visual recovery. Interestingly, none of them had multiple sclerosis (MS) or neuromyelitis optica (NMO) at the time of diagnosis.
 Conclusion: Prognosis is generally good for isolated cases of paediatric optic neuritis. Outcome of cases secondary to ADEM depends on the degree and extent of demyelination. A diagnosis of chronic relapsing inflammatory optic neuropathy can be considered in recurrent cases that are steroid responsive. None of our cases had underlying MS or NMO during the study period.

Drug-related immune-mediated myelopathies.

Iatrogenic immune-mediated inflammatory disorders of the spinal cord are an uncommon but potentially severe complication of drug therapy for several human diseases. Particularly the introduction of novel biological agents in the treatment of systemic inflammatory disorders and cancer immunotherapy have led to a significant increase in immune-related adverse events of the central nervous system (CNS). The use of Tumor necrosis factor alpha (TNF-alpha) inhibitors in rheumatic and inflammatory bowel diseases has been associated with demyelinating and other inflammatory CNS conditions, including myelitis. The introduction of immune checkpoint inhibitors in the treatment of several human malignancies has led to an increase in drug-induced immune-related adverse events including in the CNS. Other drugs that have been associated with immune-mediated myelitis include tyrosine-kinase inhibitors and chimeric antigen receptor (CAR) T Cell therapy. A high degree of suspicion is necessary when diagnosing these conditions, as early diagnosis and treatment is crucial in preventing further neurological damage and disability. The treatment of drug-induced inflammatory myelitis typically involves administration of high-dose intravenous corticosteroids, however additional immunosuppressive agents may be required in severe or refractory cases. While most cases are monophasic and remit following discontinuation of the offending agent, chronic immunosuppressive therapy may be indicated in cases with a progressive or relapsing disease course or when a diagnosis of a specific underlying neuro-inflammatory disorder is made. Outcomes are generally favorable, however depend on the specific therapeutic agent used, the clinical presentation and patient factors. In this review we aim to describe the clinical characteristics, imaging findings and management for the most common forms of iatrogenic immune-mediated myelopathies.

CMV Infection Following mRNA SARS-CoV-2 Vaccination in Solid Organ Transplant Recipients.

CMV Infection Following mRNA SARS-CoV-2 Vaccination in Solid Organ Transplant Recipients.