Intravenous Corticosteroid Pulse Research Articles

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.

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.

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.

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

Abstract&#x0D; Introduction &amp; 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&#x0D; 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&#x0D; 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)&#x0D; 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&#x0D; Introduction &amp; 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&#x0D; 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.&#x0D; 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.&#x0D; 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 &amp; 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.

Anti-leucine-rich Glioma-inactivated 1 Protein-antibody Positive Encephalitis with Extensive Unilateral Cerebral Cortex and White Matter Lesions.

Encephalitis caused by antibodies targeting the leucine-rich glioma-inactivated 1 protein receptor, which belongs to the anti-voltage-gated potassium channel receptor complex, is characterized by hyponatremia, progressive cognitive impairment, seizures, and psychiatric disorders. The patient initially presented with faciobrachial dystonic seizures and subsequently developed encephalopathy. Brain magnetic resonance imaging revealed atypical unilateral hyperintense signals in the cerebral cortex and white matter. Intravenous corticosteroid pulse therapy effectively improved faciobrachial dystonic seizures and brain lesions.

Protocols in pediatric rheumatology (PROKIND): treat-to-target in polyarticular juvenile idiopathic arthritis

The aims of the PROKIND protocols are improvement and harmonization of the diagnostics, monitoring, treatment decision and prognosis. This article reports the results of a prospective treat-to-target observational study of patients with polyarticular juvenile idiopathic arthritis (JIA) during the first year of treatment. Disease activity was assessed with the 10-joint juvenile arthritis disease activity score (JADAS-10), functional limitation with the childhood health assessment questionnaire disability index (CHAQ-DI) and with information on overall well-being, on pain, on fatigue and on global estimation of disease activity. Overall, 129 patients with polyarticular JIA (rheumatoid factor, RF, positive (+) polyarthritis n = 22, RF negative (-) polyarthritis n = 133 from 23pediatric rheumatology institutions in Germany and Austria were recruited. Patients with initial treatment with methotrexate formed cohort1, patients with additional repeated intravenous corticosteroid pulse therapy formed cohort2 and patients with concomitant intra-articular corticosteroid administration in at least 5joints formed cohort3. The mean JADAS10 showed adecrease in disease activity from 16.4 ± 6.1 to 2.8 ± 3.6 and the decrease in the CHAQ-DI from 1.0 ± 0.8 to 0.3 ± 0.5 showed the improvement in functional capacity. Similarly, improvements in quality of life, pain and fatigue were demonstrable. A JADAS inactive disease was achieved by 18.1% at month3, 47.7% at month6 and66.7% at month12. In cohort 1 a JADAS remission was achieved by 72.4%, by 50% in cohort 2 and by 69.2% in cohort 3. An escalation to treatment with biologics was necessary in 38% of patients in cohort 1, 60% in cohort 2 and 46% in cohort 3. Using atreat-to-target approach a dramatic improvement in disease activity, functional capacity and quality of life in polyarticular JIA could be achieved. Even after 12months an inactive disease was achieved in the majority of cases.

The Efficacy and Adverse Effects of Corticosteroid Pulse Therapy in Alopecia Areata: A Review Article.

Alopecia areata (AA) is a common, non-scarring, autoimmune hair loss disorder, varying in severity from small round hairless patches to the total loss of scalp or body hair. As steroid pulse therapy outcomes for AA vary, this study aimed to review the related literature regarding the efficacy, relapse rates, side effects, and prognostic factors associated with the response to different pulse corticosteroid treatments. We performed a literature search on August 29, 2022, to provide an overview of the efficacy of pulse steroid therapy in patients with AA. The terms "pulse steroid therapy AND alopecia areata" and "pulse corticosteroid therapy AND alopecia areata" were searched on PubMed and Google Scholar. A total of 24 articles were assessed. There was no difference in outcomes and side effects between intravenous and oral pulse corticosteroid therapy. The relapse rate and efficacy depended on the time of AA onset, age, and AA type: improved outcomes and decreased relapse were linked with recent onset (<6 months), a younger age (<10 years), and the multifocal type of AA. Patients with a past medical history of atopy, nail pitting, or thyroid disease and those with severe forms of AA like alopecia totalis and alopecia universalis had the least improvement. All kinds of mentioned systemic pulse corticosteroids effectively induce hair regrowth in AA. Betamethasone pulse seems to be the most effective agent (followed by intramuscular triamcinolone), especially in severe cases, but more side effects may accompany it. Combining this agent with other medications can reduce the dosage and side effects. Pulses of prednisolone and methylprednisolone are less effective but safer, as they have low relapse rates and adverse effects. A combination of them with other drugs can increase their efficacy.

A Case Series of Cat-Scratch Disease with Ocular Manifestations: Clinical Findings and Treatment Approach.

To present the clinical and demographic characteristics, imaging findings, diagnosis and treatment approach in cases of cat scratch disease (CSD) with ocular involvement. The records of 19 patients followed-up and treated between 2010 and 2020, including detailed ophthalmological examinations, imaging findings, and treatment approach, were evaluated retrospectively. Twenty-three eyes of 19 patients, 7 female (37%) and 12 male (63%), were included in the study. The mean age was 34.1±13.3 (range: 11-56) years, and the mean follow-up duration was 12.6±18.0 (range: 1-81) months. Unilateral involvement was observed in 15 cases (79%). Cat contact was reported in 14 cases (74%). In 6 cases (32%), flu-like symptoms were present before the ocular complaints. The mean visual acuity (VA) at presentation was 0.42±0.36 (range: 0.001-1.0). Anterior uveitis was observed in 3 eyes (13%). Posterior segment findings included neuroretinitis in 14 (61%), superficial retinal infiltrate(s) in 8 (35%), papillitis in 3 (13%), branch retinal artery occlusion in 2 (8%), and cilioretinal artery occlusion in 1 (4%) of the eyes. All cases were positive for Bartonella henselae immunglobulin (Ig)M and/or IgG. Systemic antibiotic therapy was administered to all patients. Intravenous pulse or oral corticosteroids were given, especially in cases with optic disc involvement. The mean final VA was 0.80±0.25 (range: 0.01-1.0). CSD may present with different ocular involvement patterns. Apart from the classical neuroretinitis and macular star appearance, patients may present with isolated optic disc edema, branch retinal artery occlusion, and retinal infiltrations. In such patients, cat contact history and Bartonella serology should be evaluated to differentiate CSD.

IL-10‒Producing Potency from Blood B Cells Correlates with the Prognosis of Alopecia Areata

IL-10‒Producing Potency from Blood B Cells Correlates with the Prognosis of Alopecia Areata

A Rapidly Fatal Case of Anti-GFAP Receptor Encephalitis Due to Acute Brain Edema and Herniation

Objective Glial fibrillary acidic protein antibody (GFAP) is a newly recognized biomarker for an immunotherapy responsive autoimmune meningo-encephalomyelitis with a wide variety of clinical presentations. We report the second GFAP antibody positive case in a young man who died despite appropriate and aggressive immunomodulatory treatment. Background 29 year old previously healthy male with childhood immune disorder presented with 3 week history of acute progressive worsening headaches, bloody emesis, nausea, blurry vision and generalized weakness. Exam was significant for downbeating nystagmus, limb ataxia and tremor and later progressing into inattention, confusion, urinary retention, asymmetric pupils, hyprreflexia and lack of motor or sensory response. Lumbar puncture revealed lymphocytic pleocytosis with elevated protein and opening pressure of 36 cm H20. MRI demonstrated areas of restricted diffusions symmetrically involving white matter of the corpus callosum, middle cerebellar peduncle, cerebellar white matter bilaterally as well as within the pons centrally. Patient was started on intravenous immunogammaglobulin (IVIG) and pulse corticosteroids along with broad spectrum antimicrobial therapy. After an initial apparent response to treatment, repeat head CT showed Diffuse Sulci effacement. Shortly after, He rapidly decompensated with clinical findings indicating brainstem herniation, cardiac arrest and brain death was diagnosed. CSF studies subsequently were reported as positive for GFAP antibodies. An autopsy reported the cause of death as cerebellar tonsillar herniation secondary to diffuse cerebral edema. all sections showed perivascular inflammation and gliosis. Design/Methods NA. Results NA. Conclusions This reported case of anti-GFAP meningo-encephalomyelitis is unusual for the rapid onset of cerebral edema and rapid progression to herniation and brain death occurring only 4 weeks after symptom onset. While this may be a rare complication of Anti GFAP encephalitis, clinicians should be vigilant for acutely increased intracranial pressure in patients with clinical findings of encephalitis in general.

Outcomes of patients with acute Vogt-Koyanagi-Harada disease treated with intravenous corticosteroid pulse followed by the slow tapering of oral corticosteroid therapy.

To elucidate the intravenous corticosteroid pulse treatment outcomes of patients with acute Vogt-Koyanagi-Harada (VKH) disease and assess the differences between patients with no inflammation worsening and those with persistent or worsening inflammation. Potential factors responsible for eyes with low visual outcomes were also investigated. We retrospectively reviewed the clinical records of patients with acute VKH disease who first visited us between 2009 and 2018 and were followed up for > 300days. Clinical characteristics, treatments, and posttreatment conditions were assessed. Patients were classified into no inflammation worsening (acute-resolved [AR]) and inflammation worsening (chronic-recurrent [CR]) groups based on conditions after 6months from disease onset. This study included 60 eyes from 30 patients (mean age: 52.7years). Patients were treated with methylprednisolone pulse followed by the slow tapering of oral prednisolone; 73% of patients developed AR and 27% CR, and the best-corrected visual acuity (BCVA) was ≥ 1.0 in 83% of eyes at 6months following the introduction of treatment. Although the total prednisolone dose was higher in patients with CR disease, no significant difference was noted in the final BCVA. Among the patients, five eyes had a final BCVA of ≤ 0.5 due to anisometropic amblyopia, diabetic maculopathy, pre-existing macular hole, epiretinal membrane, and ellipsoid zone loss. Patients with acute VKH disease treated with corticosteroid pulse appear to demonstrate good visual outcomes, including patients with CR; the majority of eyes with low visual outcomes have pre-existing conditions that explain the low vision.

Anti-NMDAR Encephalitis with Poor Recovery on Steroid Pulse and IVIg: Practical Approach to Intensive Immunotherapy

Anti-N-methyl-D-aspartate receptor encephalitis (NMDARE) is a well-defined autoimmune encephalitis that is responsive to early intensive immunotherapy. Recent international consensus regarding treatment of NMDARE provides a practical treatment algorithm for immunotherapy escalation, while considering a patient's age, disease severity, and other background information. First-line immunotherapy, which includes an intravenous (IV) corticosteroid pulse with the addition of either intravenous immunoglobulins (IVIg) or plasma exchange, should be offered to all NMDARE-diagnosed patients as soon as possible. In cases where insufficient improvement follows a repeat of the first-line combination therapy (assessed on day 14 after the initial treatment), second-line immunotherapy comprising rituximab or an IV cyclophosphamide pulse (IVCPA) is considered. Per the recent expert consensus, rituximab is preferred to IVCPA as the second-line drug of choice, although the use of either drug in the treatment of NMDARE is off-label. Most patients show gradual improvement in the first few weeks following the introduction of second-line therapy, although repeated and alternating use of both drugs is often required. Some patients, whose NMDARE was refractory to the aforementioned therapies, have also been successfully treated with tocilizumab or bortezomib. Moreover, several international clinical trials involving rituximab, inebilizumab, bortezomib, and rozanolixizumab in the treatment of autoimmune encephalitis (AE, which includes NMDARE) are being conducted to establish high-grade evidence for the treatment of AE.

Long-term changes of choroidal blood flow velocity in Vogt-Koyanagi-Harada disease.

To evaluate changes in choroidal blood flow in patients with Vogt-Koyanagi-Harada (VKH) disease after initiation of corticosteroid treatment. Fourteen patients (10 men and 4 women) with acute VKH disease followed for 2years were retrospectively reviewed; only right eyes were included in the analysis. Mean blur rate (MBR) in the macula was measured by laser speckle flowgraphy (LSFG) and central choroidal thickness (CCT) was measured by optical coherence tomography (OCT), both prior to treatment and over 2years after initiation of corticosteroid treatment. Of 14 patients included in this study, 13 received initial treatment consisting of intravenous corticosteroid pulse therapy and one patient was treated using bilateral sub-Tenon injections of triamcinolone acetonide. Mean percentage change in MBR was significantly increased after initiation of treatment compared to pretreatment values (P < 0.001). Mean CCTs were significantly decreased after initiation of treatment, compared to pretreatment thicknesses (P < 0.001). There was no significant change in either MBR change or CCT at 1month after initiation of treatment through 2years of follow-up. The mean MBR percentage change was significantly higher in eyes with sunset glow fundus (SGF) compared to eyes without SGF at 1year. With initiation of corticosteroid treatment in VKH disease patients, choroidal blood flow improved and was maintained for 2years. However, the presence of SGF should be taken into consideration when interpreting MBR results in VKH disease patients.

Managing Antiphospholipid Syndrome in Children and Adolescents: Current and Future Prospects.

Pediatric antiphospholipid syndrome (APS) is a rare acquired multisystem autoimmune thromboinflammatory condition characterized by thrombotic and non-thrombotic clinical manifestations. APS in children and adolescents typically presents with large-vessel thrombosis, thrombotic microangiopathy, and, rarely, obstetric morbidity. Non-thrombotic clinical manifestations are frequently seen in pediatric APS and may be present even before the vascular thrombotic events occur. We review insights into the pathogenesis of APS and discuss potential targets for therapy. The identification of multiple immunologic abnormalities in patients with APS reveals molecular targets for current or future treatment. Management strategies, especially for APS in adolescents, require screening for additional prothrombotic risk factors and consideration of counseling regarding contraceptive strategies, lifestyle recommendations, treatment adherence, and mental health issues associated with this autoimmune thrombophilia. The main goal of therapy in pediatric APS is the prevention of thrombosis. The management of acute thrombosis events in children and adolescents is the same as for primary APS, which involves isolated occurrences, and secondary APS, which is seen in association with another autoimmune disease, e.g., systemic lupus erythematosus. A pediatric hematologist should be consulted so other differential thrombophilic conditions can be eliminated. Therapy includes unfractionated heparin or low-molecular-weight heparin followed by vitamin K antagonists. Treatment of catastrophic APS involves triple therapy (anticoagulation, intravenous corticosteroid pulse therapy, and plasma exchange) and may include intravenous immunoglobulin for children and adolescents with this condition. New drugs such as eculizumab and sirolimus seem to be promising drugs for APS.

Clinical Case of a Child with Optic Neuritis, Associated with Epstein-Barr Virus

We present a clinical case of a child with bilateral papillitis, associated with EBV-infection in a 5-year-old female child, who was diagnosed and treated at the Aleksandrovska University Hospital. The initial reason for the eye-exam was a new-onset squint. On admission, she had discrete Eidon confocal scan, decreased visual acuity and bilateral edema and hyperemia of the optic disks. MRI of brain and orbits was evaluated on emergency to rule out a demyelinating or intracranial process. The child had persistent lymphadenopathy and laboratory abnormalities and tested positive for EBV serology. She was treated with intravenous corticosteroid pulse therapy and visual acuity was restored to 100%. Papillitis in children is frequently bilateral, with viral genesis and has a good prognosis. Isolated papillitis (without inflammation of other eye structures) is difficult to distinguish clinically from papilledema. When papilledema is suspected, urgent MRI has to be carried out.

Back to the Basics: Response of Alopecia Areata Universalis to Intravenous High-Dose Pulse Corticosteroid Therapy.

Back to the Basics: Response of Alopecia Areata Universalis to Intravenous High-Dose Pulse Corticosteroid Therapy.

Alopecia areata: Current understanding of the pathophysiology and update on therapeutic approaches, featuring the Japanese Dermatological Association guidelines.

Alopecia areata (AA) is a relatively common nonscarring hairloss disease characterized by an autoimmune response to anagen hair follicles (HFs). Accumulated evidence suggests that collapse of the HF immune privilege subsequent to triggering events, represented by viral infection, leads to autoimmune response in which autoreactive cytotoxic CD8+NKG2D+ T cells mainly target exposed HF autoantigens. AA had been recognized as type 1 inflammatory disease, but recent investigations have suggested some roles of type 2- and Th17-associated mediators in AA pathogenesis. The significance of psychological stress in AA pathogenesis is less emphasized nowadays, but psychological comorbidities, such as depression and anxiety, attract greater interest in AA management. In this regard, the disease severity may not solely be evaluated by the extent of hair loss. Use of trichoscopy markedly improved the resolution of the diagnosis and evaluation of the phase of AA, which is indispensable for the optimization of treatment. For the standardization of AA management, the establishment of guidelines/expert consensus is pivotal. Indeed, the Japanese Dermatological Association (JDA) and other societies and expert groups have published guidelines/expert consensus reports, which mostly recommend intralesional/topical corticosteroid administration and contact immunotherapy as first-line treatments, depending on the age, disease severity, and activity of AA. The uniqueness of the JDA guidelines can be found in their descriptions of intravenous corticosteroid pulse therapy, antihistamines, and other miscellaneous domestically conducted treatments. Considering the relatively high incidence of spontaneous regression in mild AA and its intractability in severe subsets, the importance of course observation is also noted. Evidenced-based medicine for AA is currently limited, however, novel therapeutic approaches, represented by JAK inhibitors, are on their way for clinical application. In this review, the latest understanding of the etiopathogenesis and pathophysiology, and update on therapeutic approaches with future perspectives are summarized for AA, following the current version of the JDA AA management guidelines.

Bowel Perforation During Haemophagocytic Lymphohistiocytosis Treatment with Corticosteroids and Anakinra.

Anakinra can potentially augment bowel inflammation.The concomitant use of anakinra and corticosteroids may increase the risk of bowel perforation.Use of anakinra and corticosteroids in patients with pre-existing gastrointestinal diseases requires vigilant observation for abdominal symptoms.