Corticosteroid Pulse Research Articles

Telitacicept for refractory cystitis associated with severe systemic lupus erythematosus: A case report.

Lupus cystitis, a severe complication of systemic lupus erythematosus (SLE), presents considerable treatment challenges. This case report describes the use of telitacicept in treating severe SLE with lupus cystitis. A single patient with lupus cystitis. A patient with symptoms including frequent urination, urgency, and acute urinary retention. Initial treatments included corticosteroid pulse therapy, immunoglobulin, and cyclophosphamide, which improved laboratory indicators but failed to alleviate symptoms of urinary retention. The patient was then treated with telitacicept. Significant alleviation of urinary retention was observed shortly after incorporating telitacicept into the treatment regimen. The patient's condition remained stable with no relapse during the subsequent 10 months of follow-up. This case highlights the therapeutic potential of telitacicept for SLE patients who are unresponsive to conventional therapies, particularly those with severe manifestations such as lupus cystitis.

Corticosteroid-responsive narcolepsy type II after COVID-19: A relevant differential diagnosis of post-COVID syndrome (a case report).

Excessive daytime sleepiness is a possible symptom of post-COVID syndrome and is also the cardinal symptom of narcolepsy, a rare life-long sleep disorder with a possible autoimmune background. Recent reports indicate that COVID-19 infection may trigger narcolepsy. However, it remains unclear how best to identify and treat such cases. A 25-year-old male developed daytime sleepiness after COVID-19 infection. A diagnosis of narcolepsy type II was made based on pathologically shortened sleep latencies in polysomnography and multiple sleep latency tests (MSLT) together with several sleep-onset REM-sleep periods (SOREMs). Pupillography and neuropsychological testing revealed reduced alertness levels. Hypocretin levels in the cerebrospinal fluid were borderline. Based on the postulated autoimmune background of narcolepsy, we performed an intravenous high-dose corticosteroid pulse therapy with methylprednisolone. Narcoleptic symptoms immediately and consistently remitted after the corticosteroid pulse. Follow-up after 4 months revealed normalisation of sleep latencies, no further SOREMs in the MSLT, and increased alertness in pupillography and neurocognitive testing. No further wakefulness promoting drug therapy was required. Narcolepsy should be considered in the differential diagnosis of post-COVID syndrome with leading symptoms of daytime sleepiness. Furthermore, immunosuppressive therapy may offer a treatment option in managing an otherwise lifelong disorder in select cases.

Comparative Efficacy and Safety of Baricitinib Against Traditional Therapies in Severe Alopecia Areata: ARetrospective Cohort Study.

Alopecia areata is a common autoimmune disease which results in reversible hair loss. Janus kinase inhibitors are prescribed for severe alopecia areata with encouraging results. There are no studies comparing the efficacy and safety of Janus kinase inhibitors to traditional treatment options, such as topical immunomodulators and traditional immunosuppressants. To retrospectively compare the efficacy and safety of baricitinib, an approved Janus kinase inhibitor, to other treatments for severe AA during a 6-month treatment period. We included patients with newly presenting, relapsing or treatment-resistant alopecia areata with Severity of Alopecia Tool (SALT) score ≥ 50, for the period between July 2021 and July 2023. Medical histories were reviewed and possible side effects were recorded. Primary endpoints were SALT ≤ 20 and SALT ≤ 10 after 6 months of treatment. Seventy-five patients (53 females) were divided into three groups: topical immunomodulators (51 patients); baricitinib (19 patients); and a group receiving pulsed intramuscular corticosteroids or traditional immunosuppressants (11 patients). Twenty-one patients received more than one treatment options within 2 years. After 6 months, the baricitinib group showed superior efficacy with 32% and 26% of patients achieving SALT ≤ 20 and SALT ≤ 10, compared to 12% and 9% in both other groups. Baricitinib demonstrated better secondary outcomes (50% and 90% reduction from initial SALT scores). All treatments exhibited mild-to-moderate and expected side effects. Weight gain, which had not been reported in clinical trials for alopecia areata, was observed in three baricitinib-treated patients. Baricitinib was superior to traditional treatments for severe alopecia areata after 6 months. Weight gain concerned 16% of patients receiving baricitinib.

Genetic factors associated with changes in muscle strength and mass during corticosteroid therapy

BACKGROUND: Corticosteroid use is associated with loss of muscle mass and strength, which is associated with an unfavorable prognosis of the underlying disease, as well as an increased risk of cardiovascular disease. Patients with multiple sclerosis (MS) have a higher baseline susceptibility to cardiovascular disease and a higher risk of adverse effects due to corticosteroid use. In clinical practice, different degrees of severity of side effects of corticosteroids are reported. This may be related to the presence of a genetic predisposition, the identification of which should allow a personalized approach to therapy. AIM: To evaluate the association of polymorphic variants of the glucocorticoid receptor gene NR3C1, the FTO gene, and the melatonin receptor genes MTNR1A, MTNR1B with the development of changes in muscle mass and strength in the context of corticosteroid use in patients with MS. MATERIALS AND METHODS: The study included 80 patients (mean age 36.3±10.0 years) with MS receiving pulse corticosteroid therapy: 4 (3; 4) g methylprednisolone. Patients were enrolled over the course of one year. To assess the primary endpoint (reduction in muscle mass and strength), muscle strength and mass were monitored before and after therapy using wrist dynamometry, the time-up-and-go test, and bioimpedancemetry. Polymorphic variants of the glucocorticoid receptor gene NR3C1, the FTO gene, and the melatonin receptor genes MTNR1A and MTNR1B were identified by real-time polymerase chain reaction. Intergenic interactions were evaluated using the multifactor dimensionality reduction (MDR) method. R (v. 3.2, R Foundation for Statistical Computing, Austria) was used for statistical analysis. RESULTS: In women with loss of muscle mass after corticosteroid therapy, there was an increased incidence of depression (p=0.01) for polymorphic variant BclI (rs41423247) of the NR3C1 gene (p=0.005). Reduced dominant arm strength in women was associated with a variant of the BclI gene (rs41423247) (p=0.02), genotypes including BclI (rs41423247) and N363S (rs56149945) (p=0.03). Men with decreased strength in the non-dominant arm were more likely to be smokers (p=0.03). Combinations of genotypes of the NR3C1, FTO, MTNR1B genes were identified with increased and decreased risk of muscle mass loss in women after corticosteroid therapy. Analysis of intergenic interactions showed a strong synergism between the polymorphic variant rs993960 of the FTO gene and rs10830963 of the MTNR1B gene. CONCLUSION: The presence of polymorphic variants BclI (rs41423247), N363S (rs56149945) of the glucocorticoid receptor gene NR3C1 is associated with loss of muscle strength and mass in women receiving corticosteroid therapy for MS. Combinations of genotypes of the NR3C1, FTO, MTNR1B genes have been identified with an increased risk of muscle mass loss in women after corticosteroid therapy. Modifiable factors associated with loss of muscle mass and strength and cardiovascular risk (depression, smoking) were identified. The data obtained can be used to personalize corticosteroid therapy and prevent cardiovascular and metabolic disorders.

A neuropsychiatric systemic lupus erythematosus presenting with acute confusional state: a case report

Systemic lupus erythematosus (SLE) is a chronic multisystem autoimmune disease characterized by variable clinical symptoms. It disproportionately affects African-American women and women from other ethnic groups. The complexity of SLE, stemming from its genetic and phenotypic diversity, poses challenges in diagnosis, treatment, and development of novel therapies. Neuropsychiatric systemic lupus erythematosus (NPSLE) encompasses a spectrum of neurological syndromes involving the central, peripheral, and autonomic nervous systems, as well as psychiatric syndromes diagnosed in SLE patients after ruling out other causes. Neuropsychiatric manifestations typically emerge in the later stages of SLE (10–75%). The acute onset of neuropsychiatric symptoms as the primary presentation of NPSLE is exceptionally rare and can be diagnostically elusive when other organs are unaffected. In this article, we present the case of a 53-year-old woman who was admitted to the hospital due to disorientation and an acute confusional state. Following the diagnosis of NPSLE, she received treatment consisting of three doses of pulse corticosteroids and one dose of cyclophosphamide. She exhibited a favorable clinical response, leading to partial resolution of her symptoms and subsequent discharge from the hospital. Practical Implications. Due to the rarity and diverse clinical presentations of NPSLE, particularly with the sudden onset of neuropsychiatric symptoms, there is a significant risk of delayed or incorrect diagnosis, which can lead to ineffective treatment. Therefore, maintaining a high index of clinical suspicion is paramount for achieving early and accurate diagnosis, which is crucial for initiating timely and effective treatment strategies.

Immune checkpoint inhibitor-induced autoimmune limbic encephalitis with positivity for anti-Hu antibodies in a patient with small-cell lung cancer: A case report and literature review.

In recent years, there has been an increasing number of studies on neurological symptoms induced as paraneoplastic neurological syndrome (PNS) or neurological immune-related adverse events (irAEs) in patients treated with immune checkpoint inhibitors (ICIs). Herein, we report a 68-year-old male patient with small-cell lung cancer who developed memory impairment and autonomic nervous system dysfunction after three courses of carboplatin, etoposide, and durvalumab therapy. Brain magnetic resonance imaging revealed hyperintense areas restricted to the bilateral temporal lobes. Moreover, based on the blood test results, the patient was strongly positive for anti-neuronal nuclear antibodies. Hence, he was diagnosed with autoimmune limbic encephalitis (ALE). Corticosteroid pulse therapy was administered. After treatment, the patient exhibited gradual improvement in memory impairment. However, while tapering the prednisolone dose, the patient exhibited relapse of memory disturbance owing to ALE. It is challenging to distinguish PNS from neurological irAEs. However, ICI-induced ALE with positivity for anti-Hu antibodies has an extremely poor prognosis.

The impact of high dose corticosteroid treatment affect on cardiac abnormalities in patients with multiple sclerosis

Aims: High-dose corticosteroids to control acute relapses of multiple sclerosis, leveraging their anti-inflammatory effects. However, these treatments can lead to cardiovascular side effects. Understanding the pathophysiology of corticosteroid induced bradycardia is paramount for healthcare providers. Our aim in this study are to identify risk factors for cardiac side effects and assess the timing of cardiac complications relative to treatment. Methods: Patients who met the McDonald's criteria for definite MS and patients requiring admission for pulse steroid treatment with an acute recurrence were included.Individuals taking cardiac medications, or with a heart illness were excluded.Patients were given 1 g IV methylprednisolone in 2 hours for five to seven days in order to treat acute relapses. Results: We studied with 23 patients(6 males and 17 females, 26.1/73.9% respectively).The mean±SD age of the patients was 34.6±9.9 (18-43) years and the mean±SD duration of disease was 5.5±4.9 years.Most of the patients were relapsing–remitting MS in 73.9%, primary progressive in 4.4% and secondary progressive in 21.7%.The most common cardiac arhythmia during corticosteroid pulse therapy was sinus bradycradia(n=6). Conclusion: The combination of direct effects on cardiac myocytes, electrolyte disturbances, autonomic dysfunction, and individual genetic factors can contribute to the development of bradycardia in MS patients treated with high-dose methylprednisolone. Close monitoring and prompt intervention are crucial to manage this adverse effect and optimize patient safety.

Juvenile Dermatomyositis: Updates in Pathogenesis and Biomarkers, Current Treatment, and Emerging Targeted Therapies.

Juvenile dermatomyositis is a rare systemic inflammatory autoimmune disease involving muscle, skin, and vessels. Most patients do not fully respond to initial therapy, instead having a chronic refractory or polycyclic disease course. Pathogenesis is not completely understood, but immune cell dysregulation, particularly of B cells, mitochondrial dysfunction, changes in neutrophils and neutrophil extracellular traps (NETs), and increased type I and type II interferon (IFN) signaling have been described. There are limited randomized controlled trials of drugs in juvenile dermatomyositis (JDM), and treatment is largely based on lower-quality data such as case series, retrospective studies, and open-label prospective studies. These data have been compiled into expert recommendations or consensus treatment plans, which help guide therapy. While initial therapy is more standard with most including corticosteroids (high-dose oral and/or pulse intravenous methylprednisolone) and methotrexate, for refractory patients, guidelines are more varied with multiple options or combinations, including biologic therapies. There is a clear need for more efficacious and personalized therapy in JDM. Emerging treatment options worthy of further study in JDMinclude targetingIFN-signaling (JAK, IFNAR1, IFN beta),B-cells (CD20, CD19, BAFF, TACI, CD38, BCMA)including Chimeric Antigen Receptor (CAR)-T cell therapy, mitochondrial dysfunction, and NETs.

Long-term outcomes of intravenous corticosteroid pulse therapy for rapidly progressive alopecia areata: A single-center retrospective analysis of 106 cases and usefulness evaluation of a scoring system originally designed for half-year efficacy prediction for extended periods.

Intravenous corticosteroid pulse therapy (IVPT) has been preferentially conducted for rapidly progressive alopecia areata (RP-AA); however, the evaluation of long-term outcomes has been insufficient. In this study, 106 IVPT-treated RP-AA patients (36 males and 70 females) who were followed up for more than 1 year and up to 6.8 years were retrospectively analyzed. The mean observation period was 1137.8 ± 587.9 days (range 380-2490). The mean severity of alopecia tool (SALT) score before IVPT was 21.3 ± 23.4 but whole-scalp hair loss was observed in all cases after the intervention, suggesting that IVPT was performed soon after the onset. With additional interventions represented by intralesional triamcinolone acetonide injection with or without topical potent corticosteroid for those who insufficiently responded at 6 months after IVPT, 64.2%, 14.2%, and 21.7% of the patients respectively achieved good response (GR; SALT score ≤25), moderate response (MR; 25 < SALT score <75), and poor response (PR; 75 ≤ SALT score) 1 year after IVPT. On the final evaluation, the proportions of patients with GR, MR, and PR were 79 (74.5%), 7 (6.6%), and 20 (18.9%). Sixteen patients achieved and maintained full hair regrowth with IVPT alone until the end of observation. A previously reported scoring system for the short-term outcome prediction was shown to be useful for distinguishing the final-point GR responders from PR responders (P = 0.003). Of note, 21 patients were found to have some symptoms suggestive of the existence of preceding infectious diseases and tended to relapse. The revised scoring system adding the absence of preceding infectious diseases as one factor successfully predicted the occurrence of the relapse in our cohort (P = 0.002). Taken together, previously unreported real-world efficacy of IVPT to RP-AA was elucidated with the invention of a tool putatively enabling optimal long-term management.

Angioinvasive Rhino-Orbital-Cerebral Mucormycosis in a Patient with Type 2 Diabetes Mellitus: A Complex and Lethal Cause of Stroke

Rhino-orbital-cerebral mucormycosis is a rapidly progressive and often fatal fungal infection caused by molds of the order Mucorales, particularly affecting immunocompromised individuals. This infection is notorious for its angioinvasive properties, enabling the fungi to invade blood vessels and leading to tissue necrosis. We report the clinical course of a 59-year-old Caucasian man with poorly controlled type 2 diabetes (HbA1c 16.8%) who presented with unilateral headache, left-sided facial numbness, and incomplete left ocular motor paresis. Initial presentation raised suspicion of orbital phlegmon, leading to antibiotic and later corticosteroid pulse therapy, which worsened the patient’s condition. Subsequent imaging demonstrated extensive inflammatory changes, including wall irregularities of the left intracranial internal carotid artery, accompanied by ocular protrusion and periorbital enhancement. New palatal lesions indicated mucormycosis, which was confirmed by molecular analysis of a palatal biopsy, leading to Amphotericin B treatment. Pre-surgery imaging revealed a malignant middle cerebral artery infarction, and the patient died 16 days after symptom onset and 12 days after initial presentation under palliative care due to a poor prognosis. This case of angioinvasive mucormycosis underscores the severe and often fatal course of rhino-orbital-cerebral mucormycosis in an immunocompromised individual. The rapid progression from initially vague and unspecific symptoms to extensive vascular involvement and stroke highlights the critical need for early and accurate diagnosis, as well as prompt intervention to prevent further disease progression. Additionally, this case also illustrates the potential risks associated with corticosteroid therapy in the presence of undiagnosed fungal infections, which can exacerbate the condition and lead to serious complications. Clinicians should maintain a high index of suspicion for mucormycosis in similar clinical scenarios, prioritizing adequate antifungal treatment and careful monitoring to improve patient outcomes. Early interdisciplinary collaboration is essential for the effective management of such complex cases.

Clinical Features and Electroencephalogram Analysis ofBrain Network Functional Connectivityin Anti-Leucine-RichGlioma-Inactivated1Antibody Encephalitis.

To summarize the clinical manifestations, laboratory findings, and magnetic resonance imaging (MRI) characteristics of anti-leucine-rich glioma-inactivated 1 (LGI1) antibody encephalitis (anti-LGI1 antibody encephalitis) and explore the electroencephalogram (EEG) features. We retrospectively analyzed the medical history of 16 patients diagnosed with anti-LGI1 antibody encephalitis at the First Hospital of Hebei Medical University from 2021 to 2023. EEGs of patients with anti-LGI1 antibody encephalitis and healthy individuals were analyzed. Based on Video-EEG signal analysis of EEG δ, θ, α, β frequency bands, weighted phase lag index values were calculated to form brain network matrices, studying differences in coherence between brain regions of patients with anti-LGI1 antibody encephalitis and healthy individuals. Patients with anti-LGI1 antibody encephalitis often presented with subacute onset seizures and cognitive decline. Routine test of cerebrospinal fluid was mostly normal. Serum testing revealed hyponatremia in 62.50% of patients, along with positive serum antinuclear antibodies, decreased vitamin B12, and abnormal cytokines such as interleukin-6. Head MRI revealed abnormal lesions related to the disease in seven cases (43.75%), mainly located in the unilateral or bilateral frontal and temporal lobes of the hippocampus. The EEG mainly showed generalized and focal slow waves, sometimes with focal discharges. Brain network functional connectivity analysis found a significant weakening of functional connections in the frontal-temporal lobe in the δ and β frequency bands. Intravenous pulse corticosteroids and intravenous immunoglobulin are first-line immunotherapies for anti-LGI1 antibody-related encephalitis. The disease recovery and cognitive decline improved in most patients. Anti-LGI1 antibody encephalitis is characterized by seizures and cognitive dysfunction. Serum may show abnormalities in immune indicators such as cytokines. Head MRI mainly reveals abnormal signals in the frontal-temporal lobes and the hippocampus. EEG brain network connectivity analysis reveals characteristic weakening of functional connections in the frontal-temporal lobe in the δ and β frequency bands.

Coexistence of anti-KS and anti-TIF1-γ antibodies in clinically amyopathic dermatomyositis presenting with rapid progression of interstitial lung disease.

Polymyositis/Dermatomyositis (PM/DM) is an idiopathic inflammatory myopathy (IIM) manifesting mainly as symmetrical proximal muscle weakness and/or typical cutaneous features due to autoimmune mechanisms. Clinically amyopathic dermatomyositis (CADM) is a subset of DM that exhibits only the typical cutaneous features without any clinical muscle symptoms. Several autoantibodies have been found specifically in patients with PM/DM, including CADM patients. Anti-KS antibody is one of a group of anti-aminoacyl transfer RNA (ARS) antibodies that are mainly associated with fever, Raynaud's phenomenon, polyarthritis, and interstitial lung disease (ILD), whereas anti-TIF1-γantibody is frequently found in DM patients with malignancy. Here, we report a CADM patient having both anti-KS antibody and anti-TIF1-γantibody. This patient developed an acute exacerbation of ILD and was successfully treated with high dose corticosteroid pulse therapy together with immunosuppressive agents. Although earlier experience had indicated that the seminal characteristic of anti-KS-positive ILD was slowly developing disease onset with little or no progression over the clinical course, the present patient suffered rapidly progressive disease.

Beneficial therapeutic plasma exchange response in the treatment of severe relapses in patients with multiple sclerosis.

Therapeutic plasma exchange (PLEX) is effective as a second-line treatment of severe relapses of multiple sclerosis (MS) that failed to respond to standard steroid therapy. Our objective was to evaluate the effectiveness of PLEX in the severe MS relapses in a cohort of patients treated at Neurology Clinic, University Clinical Centre of Serbia, Belgrade, from 2007 until 2020. This retrospective study comprised 107 MS patients with 127 severe relapses treated with PLEX. Majority of our patients suffered from relapsing remitting MS (83.2%), 12.1% had secondary progressive MS and 4.7% had primary progressive MS. Mean age was 39.2 years (range, 19-79 years), female/male ratio 2.3:1. Pulse corticosteroid treatment was used before PLEX in 99.3% of patients. Median EDSS score at nadire during relapse was 6.0 (range 2.0-10.0). After PLEX, 73.8% relapses showed a marked clinical improvement, 7.1% showed mild improvement and in 19.0% there was no improvement. Median EDSS at discharge was 4.0 (6.0 at nadir of relapse vs. 4.0 at discharge; p<0.0001) and it was sustained at the same level, 6 month after PLEX. Multivariate regression analysis showed that higher EDSS at nadir during relapse (OR=0.63, 95% CI 0.41-0.96, p=0.039) and older age (OR=1.07, 95% CI 1.02- 1.12, p=0.010) were significantly associated with poor treatment response after 6- month follow-up. Adverse events occurred in 17.3 of procedures and they were completely resolved. Our study in a large cohort of MS patients confirmed that PLEX is effective.

Treatment-induced anogenital melanosis is a very frequent finding in patients with vulvar lichen sclerosus.

Pigmented lesions such as melanosis have rarely been reported in patients with vulvar lichen sclerosus (VLS) that is typically characterized by hypopigmented lesions. We aimed to analyze systematically anogenital melanosis in a large cohort of VLS patients. We analyzed the clinical data of 198 female patients with VLS. The anogenital lesions of all patients were professionally photographed in a standardized position and illumination. Severity classification of architectural findings followed an easy-to-use clinical score. A modified Melasma Area and Severity Index and an image analysis software were used to evaluate the area and intensity of pigmentation. According to the clinical score, 79 (198/39.9%) patients showed grade 1 disease, 78 (198/39.4%) grade 2, 37 (198/18.7%) grade 3, and 4 (198/2%) grade 4 disease. About 111 (56.1%) of the 198 patients had anogenital melanosis with a median modified Melasma Area and Severity Index of 3.6 (0.4-14). Univariate analysis revealed that anogenital melanosis was positively correlated with the use of topical estrogens (P = .0018) and negatively correlated with the use of pulsed high-dose corticosteroids plus low-dose methotrexate (PHDC-LDM, P = .021). On multivariable analysis, the use of topical hormone therapy turned out to be a strong independent predictor for the presence of anogenital melanosis (odds ratio: 4.57, 95% confidence interval: 1.66-12.57, P = .0033), whereas PHDC-LDM use was an independent predictor for the absence of anogenital melanosis (odds ratio: 0.35, 95% confidence interval: 0.15-0.84, P = .018). The study includes the retrospective monocentric design. Anogenital melanosis is a very frequent and so far, under-reported clinical finding in VLS patients. It is likely caused by the use of topical estrogens employed for VLS treatment. In contrast, patients with more severe disease and PHDC-LDM treatment appear to develop less likely anogenital melanosis.

Research perspective in the clinical management of Kawasaki disease.

This study examines research perspective in the clinical diagnosis, treatment, and prevention of cardiovascular complications in Kawasaki Disease (KD). Starting with an overview of the disease, it introduces KD's clinical manifestations, etiology, epidemiological features, and its impact on the cardiovascular system. Subsequently, the study discusses in detail the diagnostic methods, pathological mechanisms, and treatment strategies for KD, including foundational and emerging approaches such as high-dose intravenous immunoglobulin and aspirin therapy, biologic therapy, and corticosteroid pulse therapy. Additionally, it outlines strategies for preventing cardiovascular complications, including early risk assessment and long-term management. The study also explores the intersection of the COVID-19 pandemic with an increase in KD-like symptoms, emphasizing the need for further studies on the association between SARS-CoV-2 and KD. Lastly, it explores future research directions to enhance understanding of KD and improve patient outcomes and quality of life. This study provides valuable insights into the comprehensive treatment and management of KD and highlights avenues for future research.

Epidemiology of childhood interstitial lung disease in France: the RespiRare cohort

IntroductionInterstitial lung disease in children (chILD) are rare and mostly severe lung diseases. Very few epidemiological data are available in limited series of patients. The aim of this study was...

Improvement of the Ocular Prognosis of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A National Survey in Japan

To investigate the incidence and prognostic factors of ocular sequelae in Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) cases arising between 2016 and 2018 in Japan, and compare the findings with those presented in the previous 2005-2007 survey. Retrospective, national trend survey. Dermatologic case report forms (CRFs) (d-CRFs) were sent to 257 institutions that treated at least 1 SJS/TEN case, and 508 CRFs were collected from 160 institutions. Ophthalmologic CRFs (o-CRFs) regarding patient demographic data, onset date, ocular findings (first appearance, day of worst severity, and final follow-up), topical treatment (betamethasone), outcome (survival or death), and ocular sequelae (visual disturbance, eye dryness) were sent to the ophthalmologists in those 160 institutions. The results of this survey were then compared with that of the previous 2005-2007 survey. A total of 240 cases (SJS/TEN: 132/108) were included. The incidence of ocular sequelae incidence was 14.0%, a significant decrease from the 39.2% in the previous survey (SJS/TEN: 87/48). In 197 (82.1%) of the cases, systemic treatment was initiated within 3 days after admission, an increase compared to the previous survey (ie, treatment initiated in 82 [60.7%] of 135 cases). Of the 85 cases with an Acute Ocular Severity Score of 2 and 3, 62 (72.9%) received corticosteroid pulse therapy and 73 (85.9%) received 0.1% betamethasone therapy; an increase compared to the 60.0% and 70.8%, respectively, in the previous survey. Ocular-sequelae-associated risk factors included Acute Ocular Severity Score (P < .001) and specific year in the survey (P < .001). The ophthalmologic prognosis of SJS/TEN has dramatically improved via early diagnosis, rapid assessment of acute ocular severity, and early treatment.

Serious neurological adverse events in immunocompetent children and adolescents caused by viral reactivation in the years following varicella vaccination.

Serious adverse events following vaccination include medical complications that require hospitalisation. The live varicella vaccine that was approved by the Food and Drug Administration in the United States in 1995 has an excellent safety record. Since the vaccine is a live virus, adverse events are more common in immunocompromised children who are vaccinated inadvertently. This review includes only serious adverse events in children considered to be immunocompetent. The serious adverse event called varicella vaccine meningitis was first reported in a hospitalised immunocompetent child in 2008. When we carried out a literature search, we found 15 cases of immunocompetent children and adolescents with varicella vaccine meningitis; the median age was 11years. Eight of the children had received two varicella vaccinations. Most of the children also had a concomitant herpes zoster rash, although three did not. The children lived in the United States, Greece, Germany, Switzerland, and Japan. During our literature search, we found five additional cases of serious neurological events in immunocompetent children; these included 4 cases of progressive herpes zoster and one case of acute retinitis. Pulses of enteral corticosteroids as well as a lack of herpes simplex virus antibody may be risk factors for reactivation in immunocompetent children. All 20 children with adverse events were treated with acyclovir and recovered; 19 were hospitalised and one child was managed as an outpatient. Even though the number of neurological adverse events remains exceedingly low following varicella vaccination, we recommend documentation of those caused by the vaccine virus.

Anti-synthetase syndrome in a child with pneumomediastinum: a case report and literature review

BackgroundAnti-synthetase syndrome (ASS) is a group of rare clinical subtypes within inflammatory myopathies, predominantly affecting adult females. Instances of critical illness associated with ASS in children are even rarer.Case presentationWe report the case of a 7-year-old boy finally diagnosed with ASS, combined with pneumomediastinum. He presented with intermittent fever persisting for 12 days, paroxysmal cough for 11 days, chest pain, and shortness of breath for 4 days, prompting admission to our hospital. Pre-admission chest CT revealed diffuse pneumomediastinum, subcutaneous pneumatosis in the neck and bilateral chest wall, consolidation, atelectasis, and reticular nodular shadowing in both lungs, as well as pericardial effusion and bilateral pleural effusions. Laboratory tests revealed a positive result for serum MP immunoglobulin M (MP-IgM) and MP immunoglobulin G (MP-IgG). The patient was initially diagnosed with mycoplasma pneumoniae (MP) infection, and following 3 days of antibiotic treatment, the patient's tachypnea worsened. Positive results in muscle enzyme antibody tests included anti–PL-12 antibody IgG, anti–Jo-1 antibody IgG, and anti–RO-52 antibody IgG. Ultrasonography detected moderate effusions in the right shoulder, bilateral elbow, and knee joints. Corticosteroids pulse therapy was initiated on the 27th day following disease onset, and continued for 3 days, followed by sequential therapy for an additional 12 days. The child was discharged on the 43rd day, and subsequent follow-up revealed a significant improvement in consolidation and interstitial lesions in both lungs.ConclusionsASS in children may combine with rapidly progressive interstitial lung disease (RPILD) and pneumomediastinum. It is crucial to promptly identify concurrent immunologic abnormalities during the outbreak of MP, particularly when the disease exhibits rapid progression with ineffective conventional antibiotic therapy.

The clinical and radiological features and prevalence of Neuro-Behçet's Disease: A retrospective cohort multicenter study in Saudi Arabia

BackgroundNeuro-Behçet's disease (NBD) is defined as primary neurological involvement in patients with systemic symptoms of BD. The variety of clinical presentations seen in NBD and the long list of similar conditions make diagnosis challenging. This retrospective study aimed to estimate the prevalence and describe neurological involvement in patients with Behçet's disease who presented to King Abdulaziz Medical Cities in Jeddah and Riyadh, Saudi Arabia. MethodsThis was a retrospective, cohort study which utilized a non-probability consecutive sampling technique to include all patients diagnosed with NBD patients. All patients with BD (215) were screened for neurological symptoms. Thirty-five patients were found to be diagnosed with NBD. Outcomes were estimated using the modified Rankin scale (mRS). ResultsIn our cohort, one in six patients with BD was diagnosed with NBD. A total of 35 patients were diagnosed with NBD (mean age 27.56 ± 10.36 years; [2.88:1; Male: Female]). The main clinical features of NBD were headaches, weakness, unsteadiness, and dysarthria. The most commonly involved sites on imaging were the brainstem, diencephalon, cerebellum and basal ganglia. Oligocolonal bands were negative in all patients. Maintenance therapy most commonly included oral corticosteroids, azathioprine, and/or infliximab. Most patients received pulse corticosteroids alone when presenting with acute relapse. Half of our cohort was asymptomatic and three in four had favorable outcomes. ConclusionNBD is common among patients with BD in our population with most patients having favorable outcomes. Patients might have a wide array of symptoms which might make the diagnosis challenging.