Corticosteroid Pulse Therapy Research Articles

Characteristics associated with COVID-19 vaccine hesitancy: A nationwide survey of 1000 patients with immune-mediated inflammatory diseases

ObjectivesTo identify potential predictors of COVID-19 vaccine hesitancy (C19-VH) in adults with immune-mediated inflammatory diseases (IMID). MethodsA total of 1000 IMID patients were enrolled in this web-based cross-sectional study. A standardised and self-administered survey was designed by members of the Brazilian Society of Rheumatology Steering Committee for Infectious and Endemic diseases and distributed to IMID patients spread across Brazil. ResultsOf the 908 (90.8%) respondents eligible for analysis, 744 (81.9%) were willing to get vaccinated against COVID-19. In our multivariable logistic regression model, concurrent malignancy, fibromyalgia, hydroxychloroquine use, and recent corticosteroid pulse therapy were independently associated with higher odds of C19-VH. The short duration of COVID-19 vaccine clinical trials was the main reason for C19-VH. ConclusionWe identified novel characteristics potentially associated with C19-VH among adults with IMID. Greater awareness on the safety and efficacy of COVID-19 vaccines is needed for both IMID patients and attending physicians.

Spontaneous ARIA-like Events in Cerebral Amyloid Angiopathy-Related Inflammation: A Multicenter Prospective Longitudinal Cohort Study.

Background and ObjectivesThe goal of this work was to investigate the natural history and outcomes after treatment for spontaneous amyloid-related imaging abnormalities (ARIA)-like in cerebral amyloid angiopathy–related inflammation (CAA-ri).MethodsThis was a multicenter, hospital-based, longitudinal, prospective observational study of inpatients meeting CAA-ri diagnostic criteria recruited through the Inflammatory Cerebral Amyloid Angiopathy and Alzheimer's Disease βiomarkers International Network from January 2013 to March 2017. A protocol for systematic data collection at first-ever presentation and at subsequent in-person visits, including T1-weighted, gradient recalled echo–T2*, fluid-suppressed T2-weighted (fluid-attenuated inversion recovery), and T1 postgadolinium contrast-enhanced images acquired on 1.5T MRI, was used at the 3-, 6-, 12-, and 24-month follow-up. Centralized reads of MRIs were performed by investigators blinded to clinical, therapeutic, and time-point information. Main outcomes were survival, clinical and radiologic recovery, intracerebral hemorrhage (ICH), and recurrence of CAA-ri.ResultsThe study enrolled 113 participants (10.6% definite, 71.7% probable, and 17.7% possible CAA-ri). Their mean age was 72.9 years; 43.4% were female; 37.1% were APOEε4 carriers; 36.3% had a history of Alzheimer disease; and 33.6% had a history of ICH. A history of ICH and the occurrence of new ICH at follow-up were more common in patients with cortical superficial siderosis at baseline (52.6% vs 14.3%, p < 0.0001 and 19.3% vs 3.6%, p < 0.009, respectively). After the first-ever presentation of CAA-ri, 70.3% (95% confidence interval [CI] 61.6%–78.5%) and 84.1% (95% CI 76.2%–90.6%) clinically recovered within 3 and 12 months, followed by radiologic recovery in 45.1% (95% CI 36.4%–54.8%) and 77.4% (95% CI 67.7%–85.9%), respectively. After clinicoradiologic resolution of the first-ever episode, 38.3% (95% CI 22.9%–59.2%) had at least 1 recurrence within the following 24 months. Recurrence was more likely if IV high-dose corticosteroid pulse therapy was suddenly stopped compared to slow oral tapering off (hazard ratio 4.68, 95% CI 1.57–13.93; p = 0.006).DiscussionThese results from the largest longitudinal cohort registry of patients with CAA-ri support the transient and potentially relapsing inflammatory nature of the clinical-radiologic acute manifestations of the disease and the effectiveness of slow oral tapering off after IV corticosteroid pulse therapy in preventing recurrences. Our results highlight the importance of differential diagnosis for spontaneous ARIA-like events in β-amyloid–driven diseases, including treatment-related ARIA in patients with Alzheimer disease exposed to immunotherapy drugs.

Evaluation of macular blood flow after intermittent intravenous infusion of high-dose corticosteroids (pulse therapy) in patients with thyroid-associated orbitopathy (TAO) using angio-OCT

PurposeThe aim of this study is to evaluate the changes in macular blood flow index (BFI) in patients with moderate to severe thyroid-associated orbitopathy (TAO) before and after pulse therapy and their relationship with clinical features and disease activity using angio-OCT technology.MethodsWe analyzed twenty-four eyes. Every patient underwent a complete eye examination and angio-OCT analysis (OCT Topcon ImageNet 6; DRI OCT Triton, Topcon Corporation) before (T0) and two months (T2) after pulse therapy. We analyzed macular vascular blood flow in four angiographic levels: superficial plexus (SP), deep plexus (DP), external retina (ER), and choriocapillaris (CC). We used the clinical activity score (CAS) score to define TAO as moderate or severe.ResultsMacular BFI significantly increased at T2 in the DP, ER, and CC (p < 0.01). CAS score (5.8 ± 0.8 vs. 3.9 ± 0.9, p < 0.01) and Hertel exophthalmometry values (22.6 ± 2.3 mm vs. 21.2 ± 2,5 mm, p < 0.01) improved for all patients at T2 compared T0. Mean IOP increased from 13.3 ± 2.8 mmHg to 14.3 ± 2.1 mmHg (p < 0.01). No correlation was found between CAS score and macular BFI in all the analyzed levels.ConclusionsPulse therapy treatment can change macular BFI. In particular, two months alter pulse therapy, all the patients show an increase in macular vascular blood flow in each angiographic level. According to our results, angio-OCT analysis of the macular BFI may be a useful tool in the follow-up of TAO patients after pulse therapy.

New-onset hypogammaglobulinaemia and infectious complications associated with rituximab use in childhood-onset rheumatic diseases.

To investigate the incidence and risk factors for hypogammaglobulinaemia and infectious complications associated with rituximab treatment in childhood-onset rheumatic diseases. We performed a single-centre retrospective study of patients (n = 85) treated at Boston Children's Hospital (BCH) from 2009 to 2019. Study subjects included patients (ages 6-24 years) who received rituximab for the treatment of a childhood-onset rheumatic disease. New-onset hypogammaglobulinaemia developed in 23 (27.1%) patients within 18 months of rituximab induction treatment. Twenty-two patients (25.9%) developed at least one infectious complication in the 18 months following the first rituximab infusion; of these, 11 (50%) had serious infections requiring inpatient treatment. After adjusting for potential confounders, exposure to pulse corticosteroid therapy in the month prior to rituximab use was a significant predictor of both new-onset hypogammaglobulinaemia (odds ratio [OR] 3.94; 95% CI: 1.07, 16.0; P = 0.044) and infectious complications (OR 15.3; 95% CI: 3.04, 126.8; P = 0.003). Post-rituximab hypogammaglobulinaemia was the strongest predictor of serious infectious complications (OR 7.89; 95% CI: 1.41, 65.6; P = 0.028). Younger age at rituximab use was also a significant predictor of new-onset hypogammaglobulinaemia (OR 0.83; 95% CI: 0.70, 0.97; P = 0.021). Compared with other rheumatic diseases, patients with vasculitis had a higher likelihood of developing infectious complications, including serious infections. Although rituximab was well tolerated in terms of infectious complications in the majority of patients with childhood-onset rheumatic diseases, a substantial proportion developed new-onset hypogammaglobulinaemia and infectious complications following treatment. Our study highlights a role for heightened vigilance of rituximab-associated hypogammaglobulinaemia and infections in paediatric patients with rheumatic conditions.

Corticosteroid Pulse Therapy for Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Patients With Acute Ocular Involvement

To investigate the long-term effects of corticosteroid pulse therapy (CPT) for the treatment of acute-stage Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) patients with severe ocular involvement. Retrospective, comparative case series. This study retrospectively reviewed the medical records of 116 SJS/TEN patients who developed the disease between 2002 and 2018. Patients with severe ocular disorders (ie, ocular surface epithelial defect, pseudomembranous formation, or both) at the acute stage and who were followed for >1 year post SJS/TEN onset were enrolled. In those patients, the treatments administered for acute-stage SJS/TEN and associated ocular sequelae were examined, including the best-corrected visual acuity (BCVA) in the patient's worse eye and the incidence of the ocular complications at the final follow-up examination. A total of 85 patients were enrolled. Of those, 36 received CPT within 4 days post disease onset (group A) and 49 patients did not receive CPT within 4 days post disease onset (group B). The percentage of eyes with a BCVA in the worse eye of ≥ 1.0 were 52.8% in group A and 14.3% in group B. Severe ocular sequelae (ie, a worsening of BCVA and corneal and conjunctival complications) were significantly less in group A than in group B. In SJS/TEN patients with acute ocular involvement, CPT initiated within 4 days from disease onset may help reduce severe ocular sequelae.

Rheumatoid meningitis: A systematic review and meta-analysis.

Rheumatoid meningitis (RM) is a neurological complication of rheumatoid arthritis (RA). Current evidence is based on case reports and partial reviews. This is a systematic review and meta-analysis following the PRISMA statement. The aim is to describe the characteristics of the disease, including clinical, imaging and laboratory findings, treatment, outcomes and prognosis reported in the literature. In all, 103 studies with 130 cases were included. RM affected adults with an average age of 62years, with or without a previous RA diagnosis. RA activity and time with the disease were associated with a worse prognosis. Most common clinical manifestations were transient focal neurological signs (64.6%), systemic symptoms (51.3%), episodic headache (50.4%) and neuropsychiatric alterations (47.7%). Joint manifestations were present in only 27.4% of cases. Brain magnetic resonance imaging showed unilateral or bilateral involvement, predominantly frontoparietal. Both pachymeninges and leptomeninges were affected, the latter more frequently (82.88%). The laboratory findings included increased levels of rheumatoid factor (89.71%), anti-cyclic citrullinated peptide (89.47%), C-reactive protein (82.54%) and erythrocyte sedimentation rate (81.81%). Cerebrospinal fluid analysis showed an increase in the protein level (76.14%), with pleocytosis (85.19%) of mononuclear predominance (89.19%). Biopsy was performed in 72.52% of the patients. Corticosteroid pulse therapy was the main induction therapy. Disease relapse occurred in 31.17% of patients, whilst 54.54% had a full recovery. Rheumatoid meningitis must be considered in adult patients with or without RA diagnosis, high-dose corticosteroid induction therapy should be installed and maintenance therapy plays a key role. It is not recommended to use anti-TNF as an induction therapy. Nowadays, RM has a significantly better outcome. These findings may aid clinicians in timely RM diagnosis and treatment, thus improving its outcomes.

Thymic Neoplasm: A Rare Disease With Unusual Neurologic Manifestations

Thymoma is a rare tumor that is commonly associated with autoimmune diseases. Of these, myasthenia gravis (MG) is widely considered as the most common paraneoplastic condition. On the other hand, dermatomyositis (DM) has been rarely reported as the first presentation of non-invasive thymoma. Hereby, we describe a patient with non-invasive thymoma who initially presented with painful symmetric proximal muscle weakness with no signs of ptosis or diplopia. The needle electromyography (EMG) revealed spontaneous/insertional activity in proximal muscles. The patient was finally diagnosed with DM by muscle biopsy. Spiral chest computed tomography (CT) scan coupled with pathological assessment confirmed a non-invasive thymoma with diffuse reaction for P63 and pancytokeratin (Panck). Corticosteroids pulse therapy was initiated and the patient was referred for thymectomy. A few months later, patient began to display other neurological symptoms such as ptosis and diplopia with fluctuating pattern. As coexistence of MG was presumed, nerve conduction study (NCS) study was performed and slow repetitive nerve stimulation in proximal muscles showed more than 10% decrement in compound muscle action potential (CMAP) amplitude in repetitive nerve stimulation (RNS). Further workup revealed a positive anti-acetylcholine receptor antibody with high titer. Thus, MG was confirmed. No more treatment options were planned. Low dose corticosteroids were continued and azathioprine and pyridostigmine were prescribed. During follow-ups, symptoms were fully controlled. J Neurol Res. 2021;11(1-2):32-36 doi: https://doi.org/10.14740/jnr625

Thymic Neoplasm: A Rare Disease With Unusual Neurologic Manifestations

Thymoma is a rare tumor that is commonly associated with autoimmune diseases. Of these, myasthenia gravis (MG) is widely considered as the most common paraneoplastic condition. On the other hand, dermatomyositis (DM) has been rarely reported as the first presentation of non-invasive thymoma. Hereby, we describe a patient with non-invasive thymoma who initially presented with painful symmetric proximal muscle weakness with no signs of ptosis or diplopia. The needle electromyography (EMG) revealed spontaneous/insertional activity in proximal muscles. The patient was finally diagnosed with DM by muscle biopsy. Spiral chest computed tomography (CT) scan coupled with pathological assessment confirmed a non-invasive thymoma with diffuse reaction for P63 and pancytokeratin (Panck). Corticosteroids pulse therapy was initiated and the patient was referred for thymectomy. A few months later, patient began to display other neurological symptoms such as ptosis and diplopia with fluctuating pattern. As coexistence of MG was presumed, nerve conduction study (NCS) study was performed and slow repetitive nerve stimulation in proximal muscles showed more than 10% decrement in compound muscle action potential (CMAP) amplitude in repetitive nerve stimulation (RNS). Further workup revealed a positive anti-acetylcholine receptor antibody with high titer. Thus, MG was confirmed. No more treatment options were planned. Low dose corticosteroids were continued and azathioprine and pyridostigmine were prescribed. During follow-ups, symptoms were fully controlled. J Neurol Res. 2021;11(1-2):32-36 doi: https://doi.org/10.14740/jnr625

Sequential occurrence of microscopic polyangiitis and anti-glomerular basement membrane disease in a patient with small cell lung cancer: a case report

BackgroundThe association between a preceding malignancy and the onset of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) has been reported in several studies. While the co-existence of ANCA and anti-glomerular basement membrane (GBM) antibodies in an individual patient is not a common occurrence, this double-positive disease currently has no optimal treatment method. Herein, we report a case of a double-positive disease involving the sequential development of acute kidney injury (AKI) and diffuse alveolar hemorrhage (DAH) in a patient with small cell lung cancer (SCLC).Case presentationA 75-year-old Japanese woman was diagnosed with small cell lung cancer (cT3N2M1b cStage IV) and received chemotherapy. After one cycle of chemotherapy, she experienced fever and malaise. Her serum creatinine level rapidly increased, and she tested positive for myeloperoxidase (MPO)-ANCA and anti-GBM antibody. She was diagnosed with AKI due to microscopic polyangiitis (MPA) based on renal biopsy. Corticosteroid therapy was initiated, which improved her renal dysfunction. Eight days after she was discharged from the hospital, she complained of dyspnea and bloody sputum, and her condition rapidly progressed to respiratory failure. Upon chest imaging, ground-glass opacities were seen in her bilateral lower lungs. Laboratory examinations after admission revealed a lower MPO-ANCA titer and an elevated anti-GBM antibody titer compared to her previous admission. We diagnosed her with DAH due to an anti-GBM disease. After corticosteroid pulse therapy, plasma exchange was performed five times; her oxygen saturation and chest radiologic findings improved gradually. Following five cycles of plasma exchange, her oxygen saturation recovered to 95% in room air.ConclusionsTo our knowledge, this is the first reported case of vasculitis caused by MPA and anti-GBM disease leading to the development of AKI and DAH during treatment of SCLC. SCLC, MPA, and anti-GBM disease may occur sequentially. A double-positive disease might have a worse prognosis; therefore, intensive therapy is more likely to achieve a better outcome.

High-dose corticosteroid pulse therapy increases the survival rate in COVID-19 patients at risk of hyper-inflammatory response.

Test whether high dose corticosteroid pulse therapy (HDCPT) with either methylprednisolone or dexamethasone is associated with increased survival in COVID-19 patients at risk of hyper-inflammatory response. Provide some initial diagnostic criteria using laboratory markers to stratify these patients. This is a prospective observational study, 318 met the inclusion criteria. 64 patients (20.1%) were treated with HDCPT by using at least 1.5mg/kg/24h of methylprednisolone or dexamethasone equivalent. A multivariate Cox regression (controlling for co-morbidities and other therapies) was carried out to determine whether HDCPT (among other interventions) was associated with decreased mortality. We also carried out a 30-day time course analysis of laboratory markers between survivors and non-survivors, to identify potential markers for patient stratification. HDCPT showed a statistically significant decrease in mortality (HR = 0.087 [95% CI 0.021-0.36]; P < 0.001). 30-day time course analysis of laboratory marker tests showed marked differences in pro-inflammatory markers between survivors and non-survivors. As diagnostic criteria to define the patients at risk of developing a COVID-19 hyper-inflammatory response, we propose the following parameters (IL-6 > = 40 pg/ml, and/or two of the following: C-reactive protein > = 100 mg/L, D-dimer > = 1000 ng/ml, ferritin > = 500 ng/ml and lactate dehydrogenase > = 300 U/L). HDCPT can be an effective intervention to increase COVID-19 survival rates in patients at risk of developing a COVID-19 hyper-inflammatory response, laboratory marker tests can be used to stratify these patients who should be given HDCPT. This study is not a randomized clinical trial (RCT). Future RCTs should be carried out to confirm the efficacy of HDCPT to increase the survival rates of COVID-19.

Development of a scoring system to predict outcomes of i.v. corticosteroid pulse therapy in rapidly progressive alopecia areata adopting digital image analysis of hair recovery.

Alopecia areata (AA) is a common autoimmune disease manifesting varying degrees of hair loss. Rapidly progressive AA (RP-AA) is a severe subtype of AA and often resistant to skin-directed treatments. i.v. corticosteroid pulse therapy has been applied for RP-AA; however, the treatment outcome can only become evaluable several months after the intervention, discomposing the patients. In this study, we attempted to develop a scoring system to predict treatment outcomes based on statistical correlations between newly identified predictors and the recovery rates calculated by digital image analysis. Thirty RP-AA patients (15 men and 15 women) who underwent pulse therapy and demonstrated total hair loss during the clinical course were included. The percentages of hair regrowth (%HR) at 6months after the treatment were quantitatively calculated by image analysis software. The correlation between %HR and clinicopathological and immunological variables were statistically assessed. The analysis identified four confirmatory contributors including female sex (P=0.015), absence of previous AA history (P=0.02), lower peripheral blood eosinophil count (P=0.02) and mild to moderate cell infiltration around the hair bulb (P=0.034), together with a potential contributor, namely absence of atopic dermatitis in their medical history (P=0.08). The scoring system was developed by double counting confirmatory variables and single counting a potential variable. Importantly, the scores obtained by this system demonstrated significant correlation with %HR (r=0.61, P<0.001). The usefulness of this scoring system was further validated by assessing additional 20 cases of RP-AA. When combined with a recently published algorithm for early detection of self-healing subset, the current scoring system may help strategize the therapeutic approach for RP-AA.

Mortality in COVID-19 patients with acute respiratory distress syndrome and corticosteroids use: a systematic review and meta-analysis

ABSTRACT Objectives The acute respiratory distress syndrome (ARDS) secondary to viral pneumonitis is one of the main causes of high mortality in patients with COVID-19 (novel coronavirus disease 2019). We systematically reviewed mortality in COVID-19 patients with ARDS and the potential role of systemic corticosteroids in COVID-19 patients. Methods Electronic databases and country-specific healthcare databases were searched to identify relevant studies/reports. The quality assessment of individual studies was conducted using the Newcastle–Ottawa Scale. Country-specific proportion of individuals with COVID-19 who developed ARDS and reported death were combined in a random-effect meta-analysis to give a pooled mortality estimate of ARDS. Results The overall pooled mortality estimate among 10,815 ARDS cases in COVID-19 patients was 39% (95% CI: 23–56%). The pooled mortality estimate for China was 69% (95% CI: 67–72%). In Europe, the highest mortality estimate among COVID-19 patients with ARDS was reported in Poland (73%; 95% CI: 58–86%) while Germany had the lowest mortality estimate (13%; 95% CI: 2–29%) among COVID-19 patients with ARDS. The median crude mortality rate of COVID-19 patients with reported corticosteroid use was 28.0% (lower quartile: 13.9%; upper quartile: 53.6%). Conclusions The high mortality in COVID-19 associated ARDS necessitates a prompt and aggressive treatment strategy which includes corticosteroids. Most of the studies included no information on the dosing regimen of corticosteroid therapy, however, low-dose corticosteroid therapy or pulse corticosteroid therapy appears to have a beneficial role in the management of severely ill COVID-19 patients.

High-Dose Corticosteroid Pulse Therapy&amp;nbsp;Increases the Survival Rate in COVID-19 Patients at Risk of Cytokine Storm

Background: COVID-19 is a disease caused by SARS-CoV-2, which can develop into a hyper-inflammatory late phase or “cytokine storm” that poses high risk of death. It has been proposed that the management of this cytokine storm is crucial to increase the survival rate of COVID-19 patients. However, to date there are no diagnostic criteria based on inflammatory markers that could be used to select the COVID-19 patients at risk of developing a cytokine storm or effective interventions that can decreased mortality in these patients. Methods: We carried out a quasi-experimental study to test whether high dose corticosteroid pulse therapy with either dexametaxona or methylprednisolone were associated with reduced mortality in patients at risk of cytokine storm and assess which laboratory markers can be used to pre-select these patients. To control the development and progression of this hyper-inflammtory phase, 64 patients (20.1%) were treated with high dose corticosteroid pulse therapy (HDCPT) by using either dexametaxona or methylprednisolone daily for 2-5 days at doses of at least 30mg or 125mg respectively. To determine diagnostic criteria that can be used to defined patients at risk of cytokine storm, we carried out a 30-day time course analysis of laboratory markers between survivors and non-survivors. A multivariate Cox regression (controlling for co-morbidities and therapies) was carried out to determine whether HDCPT (among other interventions) was associated with decreased mortality in both the full cohort, and in a subgroup containing the patients with the derived criteria. Findings: Out of 500 patients originally recruited, 381 met the inclusion criteria of SARS-COV-19 detection by PCR or serology (n=272, 71.2%) or high clinical suspicion (n= 46, 16.9 %), defined as having bilateral pulmonary infiltrate or lymphopenia with according clinical profile. HDCPT was the only intervention that showed a significant decrease in mortality (odds ratio = 0.093 [95% CI 0.023 – 0.38]; P = 40 pg/ml and/or two of the following: C-reactive protein >= 100 mg/L, D-dimer >= 1000 ng/ml, ferritin >= 500 ng/ml and lactate dehydrogenase >= 300 U/L. Multivariate Cox regression by pre-selecting the patients using this criteria evidenced also a decreased mortality rate with HDCPT with a lower odds ratio (odds ratio = 0.081 [95% CI 0.011 – 0.56]; P = 0.011). Conclusions: Preventing and/or controlling the development of the cytokine storm in patients at high-inflammatory risk with HDCPT is a widely available therapy to increase survival rate in these patients. We estimate a 14.2% [95% CI 0.792 - 0.975] increase of survival rate. We also suggest some initial clinical variables that can aid with the identification of the patients who will benefit from this intervention.Funding Statement: There were no funding sources in this study.Declaration of Interests: The authors declare no competing interests.Ethics Approval Statement: This study was approved by the Ethics Committee of the Complejo Hospitalario de Jaén (Hospital of Jaén), Spain (0946-N-20). According to the local ethics committee regulations, verbal consent was obtained from all the patients that joined the study.

Corticosteroid Pulse Therapy for Graves' Ophthalmopathy Reduces the Relapse Rate of Graves' Hyperthyroidism.

Background: A course of anti-thyroid drugs (ATD) is the most common first line treatment for Graves' hyperthyroidism. However, hyperthyroidism relapse is frequent (30–70%). Due to the autoimmune nature of Graves' disease, the immunosuppressive treatment used for active Graves' orbitopathy (GO) may reduce the relapses after ATD discontinuation.Objective: To evaluate the recurrence rate in Graves' patients who, in addition to standard ATD, were treated or not treated with parenteral methylprednisolone (MPDS) for GO.Methods: Single-center retrospective study in a continuous series of 162 newly diagnosed Graves' patients, with or without GO, all gone into remission and followed-up until hyperthyroidism recurrence or at least 4 years after ATD discontinuation. Patients with moderate-severe active GO underwent middle dose MPDS treatment according to the EuGoGo guidelines. Cox proportional-hazard model was used to comparatively evaluate the risk of recurrence and the predictive factors in patients treated or not treated with MPDS pulse therapy.Results: MPDS treatment was the most significant factor that independently correlated with a reduced risk of hyperthyroidism relapse (HR = 0.53, 95% C.I. = 0.31–0.89). FT3 and female sex were also independent protective factors, while age almost reached the significance level, p = 0.062. The efficacy of MPDS was very high in patients aged <40 years (42.1% decrease in relapses, p < 0.01) but it was not significant in older patients.Discussion: Our study found that after ATD discontinuation the frequency of Graves' hyperthyroidism relapse was reduced in patients treated with MPDS pulse therapy for GO. This effect was more marked in young patients.

Pseudo-inflammatory manifestations of choroidal lymphoma resembling Vogt-Koyanagi-Harada disease: case report based on multimodal imaging

BackgroundHematologic malignancies occasionally cause serous retinal detachment (SRD); however, its pathogenesis remains unclear. Here we present the imaging characteristics of metastatic choroidal lymphoma masquerading as Vogt-Koyanagi-Harada (VKH) disease.Case presentationA 45-year-old Japanese woman was referred to our clinic because of bilateral SRD with blurred vision. Fluorescein angiography revealed multiple pinpoint leakage followed by pooling OU. Enhanced depth imaging optical coherence tomography showed marked choroidal thickening OU. Laser speckle flowgraphy detected choroidal circulation impairment OU. Although these results totally agreed with the inflammatory manifestations of acute VKH disease, indocyanine green angiography demonstrated various sizes of sharply marginated hypofluorescent lesions that seemed atypical for the finding of VKH disease, i.e., vaguely marginated hypofluorescent small dots. Cerebrospinal fluid pleocytosis was not detected. Blood tests revealed leukocytosis together with elevation of lactate dehydrogenase and soluble interleukin-2 receptor levels. Corticosteroid pulse therapy did not improve any ocular findings. Bone marrow biopsy was then performed, leading to a definite diagnosis of diffuse large B-cell lymphoma. After starting systemic chemotherapy, both SRD and choroidal thickening resolved rapidly with visual recovery. However, choroidal hypoperfusion persisted, which contrasted distinctly with the inflammatory pattern of VKH disease, i.e., the restoration of choroidal blood flow in parallel with normalization of choroidal thickness.ConclusionsOur detailed multimodal observations highlighted the differential imaging features of choroidal lymphoma despite close resemblance to VKH disease especially at the initial stage. Impaired circulation in the thickened choroid marked the pseudo-inflammatory pathogenesis of SRD due to choroidal involvement with neoplastic, but not inflammatory cells.

Outcome and prognostic factors of interstitial lung disease patients with acute respiratory failure in the intensive care unit.

Background:There are few studies reporting the clinical characteristics and outcomes of interstitial lung disease (ILD) patients with acute respiratory failure (ARF). The goal of this study is to investigate the clinical features, management, mortality, and associated factors in ILD patients with ARF requiring mechanical ventilation (MV).Methods:This was a retrospective, observational study conducted in a 24-bed intensive care unit (ICU) of a medical center in Taiwan during a 3-year period. Patients admitted to the ICU with a diagnosis of ILD with ARF needing MV were included for analysis. Patient characteristics, including demographics, critical-illness factors, and outcome data, were collected and analyzed.Results:A total of 82 patients with ILD who developed ARF were admitted to the ICU during the study period. At the onset of ARF, 38 patients received invasive MV, while 44 patients were treated with noninvasive MV. Overall in-hospital mortality was 65.9%, and 90-day and 1-year mortality were 69.5% and 76.8%, respectively. The independent risk factors for in-hospital mortality were worse oxygenation on days 5 and 7 after the onset of ARF. Invasive MV patients had significantly lower albumin levels, had higher Acute Physiology and Chronic Health Evaluation (APACHE) II scores at the onset of ARF, and received more vasopressors, sedatives, and corticosteroid pulse therapy during hospitalization compared with noninvasive MV patients.Conclusion:High in-hospital and long-term mortality rates were observed in ILD patients with ARF requiring MV. Poor oxygenation during hospitalization could serve as a predictive factor of poor prognosis. The reviews of this paper are available via the supplemental material section.

Синдром Толоса — Ханта в педиатрической практике

Tolosa-Hunt syndrome (THS) is a rare disease manifested with one or more cranial nerve disorders and orbital pain. In children, THS is diagnosed very rarely due to the lack of vigilance among physicians. Careful examination including MRI or helical CT help diagnose this disease. This paper discusses case history of THS in a child in the first ten years of life who has previously experienced similar episodes of ptosis and right-sided orbital pain; however, the cause of these symptoms has remained elusive. Disease anamnesis, clinical signs, neurological status, and additional diagnostic data are described in detail. Clinical course of THS with its relapses and remissions, rapid symptom regress after corticosteroid pulse therapy, and no specific granulomatous inflammation of the lateral wall of the cavernous sinus as demonstrated by MRI suggest idiopathic THS. The importance of in-depth neurological and general examination of patients with clinical signs of THS due to a variety of the causes of this syndrome is highlighted. KEYWORDS: pediatrics, Tolosa-Hunt syndrome, ophthalmoplegia, superior orbital fissure, cavernous sinus, corticosteroids. FOR CITATION: Shishkina E.V., Bazilevskaya T.N., Izokhvatova R.F. et al. Tolosa-Hunt syndrome in pediatric practice. Russian Journal of Woman and Child Health. 2020;3(4):336–339. DOI: 10.32364/2618-8430-2020-3-4-336-339.

Оценка эффективности местной кортикостероидной пульс-терапии

Оценка эффективности местной кортикостероидной пульс-терапии

The safety of pulse corticosteroid therapy- Systematic review and meta-analysis

ObjectiveTo amass all available evidence from randomized controlled trials regarding the safety of pulse corticosteroids therapy, in order to establish its safety. Patients and MethodsAll electronic databases from 1/1966 up to 02/2019 were reviewed to find all randomized controlled trials comparing pulse corticosteroids to oral corticosteroids or to placebo/no treatment. Two reviewers independently extracted and recorded data regarding type of corticosteroid treatment, dosages, length of treatment and follow-up. Risk ratios (RR) with 95% (CI) for differences between pulse corticosteroids and comparator were pooled using a fixed effect meta-analysis. The primary outcome was occurrence of severe adverse events (SAEs). Secondary outcomes included any adverse events (AEs), AEs requiring discontinuation, AEs per system involved and all-cause mortality. ResultsA total of 64 trials were included: 18 trials which compared pulse corticosteroids to oral corticosteroids and 46 trials which compared pulse corticosteroids to placebo/no intervention. Pulse corticosteroids was not associated with increased risk for SAEs for both comparators: RR 0.77 (95% CI 0.52-1.14), and RR 0.99 (95% CI 0.93-1.06), respectively. Sensitivity analysis based on adequate allocation concealment and use of a valid AE grading did not alter the results. Subgroup analysis revealed no increased risk of specific SAEs or AEs with pulse corticosteroids compared to oral corticosteroids. ConclusionPulse corticosteroids was not associated with an increase risk of SAEs and should be regarded as safe.

A case of metageria with a review of literature

Progeroid syndromes, one of which is metageria, are characterized by signs of premature aging with multiple systemic and skin symptoms. Here we describe an 8-year-old girl with no historyof specific underlying disease in her family, no hair growth at the scalp since she was born, and taller and thinner than her peers. We noticed diffuse subcutaneous fat atrophy. The patientwas referred to Sina Hospital, Tabriz because of the appearance of pseudomilia lesions throughout the body following pulse corticosteroid therapy. Proper and timely diagnosis of progeroid syndromes is important in preventing undesirable side effects. Introducing this case is important as it prevents other patients from undergoing corticosteroid therapy due to misdiagnoses, such as alopecia areata.