Intravenous Methylprednisolone Pulse Therapy Research Articles

Increased heart rhythm in response to high-dose intravenous methylprednisolone pulse therapy of moderate-to-severe Graves’ orbitopathy.

ABSTRACT
 Background: Intravenous glucocorticoids pulses administration is the main therapeutic option in the treatment of Graves’ orbitopathy. Such therapy could relate to the multiple adverse effects. The aim of the study is evaluation the influence of intravenous methylprednisolone (IVMP) pulse therapy on the heart rhythm (HR) changes in patients with active, moderate-to-severe Graves’ Orbitopathy (GO).
 Methods: We studied 20 patients with moderate-to-severe GO. All patients received 12 IVMP pulses (6x500 mg plus 6x250mg) at equal time intervals in a weekly schedule. We performed Holter ECG monitoring for 3 consecutive days (the day before, the day of IVMP and day after IVMP) to monitor HR and arrhythmias. We compared changes in HR between these 3 days and set time interval when the alteration was significant. This evaluation was performed during the 1st, 6th and 12th IVMP pulse.
 Results: Increased HR, in comparison with the day before, was registered on the day of IVMP administration. The most significant increase in HR started 5 hours (h) after a pulse administration and lasted 12 h. There were no significant differences in HR between the day before and the day after IVMP. We did not notice any major adverse cardiac events including severe arrhythmias.
 Conclusions: IVMP therapy is associated with increased HR, that occurs a few hours after infusion, lasts several hours and is transient. 
 Keywords: Graves’ ophthalmopathy; Graves’ disease; glucocorticoids; heart rate

Intravenous methylprednisolone pulse therapy and the risk of in-hospital mortality among acute COVID-19 patients: Nationwide clinical cohort study

BackgroundSteroids are widely used to modulate the inflammatory reactions associated with coronavirus disease 2019 (COVID-19); however, the optimal upper limit dose of steroid use for acute COVID-19 care remains unclear and currently available data may suffer from a time-dependent bias of no effectiveness or reversed causation given the desperate situation of treatment during this pandemic. Accordingly, the aim of this study was to elucidate the impact of intravenous pulse therapy with methylprednisolone (500 mg or greater per day) on the risk of in-hospital mortality among patients with COVID-19 by controlling for time-dependent bias.MethodsWe performed a prospective cohort study with 67,348 hospitalised acute COVID-19 patients at 438 hospitals during 2020–2021 in Japan. The impact of intravenous methylprednisolone pulse therapy on the risk of in-hospital mortality was examined based on hazard ratios (HRs) and 95% confidence intervals (95% CIs), with stratification according to the status of invasive mechanical ventilation (iMV). Time-dependent bias was controlled for in a marginal structural model analysis, with reference to patients without methylprednisolone therapy.ResultsDuring the study period, 2400 patients died. In-hospital mortality rates of iMV-free patients without or with methylprednisolone pulse therapy were 2.3% and 19.5%, and the corresponding values for iMV-receiving patients were 24.7% and 28.6%, respectively. The marginal structural model analysis showed that intravenous pulse therapy with methylprednisolone was associated with a lower risk of in-hospital mortality among patients receiving-iMV (HR 0.59; 95% CI 0.52–0.68). In contrast, pulse therapy with methylprednisolone increased the risk of in-hospital mortality among iMV-free patients (HR 3.38; 95% CI 3.02–3.79). The benefits of pulse therapy for iMV-receiving patients were greater than in those treated with intermediate/higher doses (40–250 mg intravenously) of methylprednisolone (HR 0.80; 95% CI 0.71–0.89).ConclusionThe results of our study suggest that intravenous methylprednisolone showed dose–response efficiencies, and pulse therapy may benefit critically ill patients with acute COVID-19, such as those requiring iMV.

JS-NG-5: THROMBOTIC MICROANGIOPATHY OR MALIGNANT HYPERTENSION: WHICH COMES FIRST? A CASE REPORT FROM A TERTIARY CARE HOSPITAL

A 66-year-old woman with a history of arterial hypertension was admitted to the emergency department (ED) due to disorientation. She reported a severe headache, nausea, vomiting in the evening prior the admission; her blood pressure (BP) when the ambulance arrived was 220/100 mmHg. During admission, BP was 158/91 mmHg, pulse 95 beats/minute, oxygen saturation 88% on room air, body temperature 36.6°C. The urine examination showed that urine protein was 3+, red blood cell count 10 mg/L. The whole blood cell analysis revealed neutrophilic leukocytosis, thrombocytopenia (Table). There was 1 schistocyte seen in the peripheral blood smear. The serum creatinine and urea values were raised (Table) with elevated level of D-dimers (1690 mcg/L (< 250 mcg/L)). Head CT showed no signs of hemorrhage or ischemia. Ceftriaxone, ampicillin, and acyclovir were initiated for suspected neuro-infection, she also underwent transfusion with 4 units of platelets due to thrombocytopenia. Patients GCS fell from 11 to 9 over 13 hours, therefore she was transferred to the intensive care unit (ICU). Cerebrospinal fluid analysis showed cytosis 15 cells/mcL (predominance of lymphocytes) and protein level of 1,073 g/L; PCR panel for the most common bacterial and viral meningitis agents came back negative. On day 2, lactate dehydrogenase level was raised at 3086 U/L (125–243 U/L), haptoglobin was < 0.08 g/L (0.3–2.0 g/L) and Coombs test was negative. Due to worsening renal function (Table) hemodialysis was initiated. The differential diagnosis included thrombotic thrombocytopenic purpura, atypical hemolytic-uremic syndrome (HUS), secondary thrombotic microangiopathy (TMA), immune thrombocytopenic purpura (ITP) and disseminated intravascular coagulation (DIC). ADAMTS-13, CH50, SC5b-9 levels were tested (0.53 TV/ml (0.40–1.50 TV/ml), 60 U Eq/ml (55–75 U Eq/ml), 4689 ng/ml (200–325 ng/ml), respectively). The patient started on plasmapheresis. On day 3, fundoscopy showed cotton wool spots (ischemia) with hemorrhages and soft exudates next to the optic nerve disc; intravenous methylprednisolone pulse therapy was administered (1 g daily for three days followed by oral methylprednisolone 32 mg daily). Renal biopsy on day 5 revealed active TMA damaging the glomeruli and acute tubular necrosis. After 15 days in the ICU, she remained hypertensive and anuric despite given treatment. Moreover, pulmonary edema occurred on day 15. Malignant hypertension (MH) is characterized by severe hypertension and multi-organ ischemic complications. It can cause TMA and the overall presentation may imitate primary TMA. The approach of renal TMA in MH remains challenging. Herein, we reported a case of MHT with biopsy-proven TMA in the kidney.

Wernicke-Korsakoff Syndrome in a Young Adult on Dialysis Who Showed Bilateral Ganglia Lesions: A Case Report.

A 30-year-old man admitted with renal dysfunction (serum creatinine, 8.19 mg/dL) was diagnosed with immunoglobulin A nephritis through a renal biopsy. He was treated with intravenous methylprednisolone pulse therapy and urgent hemodialysis, and eventually, he underwent maintenance hemodialysis. On day 108, he developed amnesia. Magnetic resonance imaging revealed bilateral basal ganglia lesions. Wernicke encephalopathy (WE) was diagnosed based on decreased serum thiamine concentration (12.8 μg/dL; reference range, 24-66 μg/dL). Thiamine replacement therapy was initiated, but the Wernicke-Korsakoff syndrome persisted. Careful monitoring of thiamine is required in patients undergoing dialysis. In addition, patients with WE may exhibit bilateral basal ganglia lesions.

Melanocyte-specific CD49a+ CD8+ T cells in vitiligo lesion potentiate to maintain activity during systemic steroid therapy.

Vitiligo is a common depigmenting skin disease that is often difficult to treat. Even if repigmentation is achieved by treatment, recurrence in the same lesion is often found within a year after stopping treatment. As a background of these issues, a subset of CD8+ T cells that recognize melanocyte-specific antigens or CD49a+ tissue-resident memory T cells that reside in the vitiligo lesion are thought to be involved. We investigated the MHC class I-restricted tyrosinase pentamer-positive CD8+ skin T cells in a progressive generalized vitiligo patient with HLA-A*02:01 who showed resistance to intravenous methylprednisolone pulse therapy. We found that HLA-A*02:01-restricted tyrosinase pentamer-positive CD8+ T cells remained in the lesions after the treatment and expressed IFN-γ and granzyme B. Interestingly, the expression of these cytokines in the pentamer-negative CD8+ T cells was decreased after intravenous methylprednisolone pulse therapy. These findings suggest that, in vitiligo patients, melanocyte-specific CD49a+ CD8+ T cells are in a potent activation state that is uncontrolled despite systemic immunosuppressive treatment, which may contribute to treatment resistance and local recurrence.

A case of retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations: Long‐term treatment with repeated courses of immunotherapy

AbstractWe present here the case of a 48‐year‐old man who first developed retinopathy, and motor deficit and aphasia occurred 8 years later. A tumor‐like lesion was seen by brain MRI. He was diagnosed with retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations (RVCL‐S) because a three prime repair exonuclease 1 (TREX1) gene abnormality was identified. After intravenous methylprednisolone pulse (IVMP) and intravenous immunoglobulin (IVIg) therapy, cerebrospinal fluid (CSF) test results and clinical manifestations improved. However, a new lesion appeared 3 years later. Immunotherapy became ineffective, and repeated relapses occurred. We evaluated the results of laboratory tests and brain MRI for 6 years after the first treatment. Coagulation tests, including measurements of vWF activity, remained high, so immunotherapy was thought to have a limited effect.

Decrease in Bone Formation and Bone Resorption during Intravenous Methylprednisolone Pulse Therapy in Patients with Graves' Orbitopathy.

Background: Treatment with glucocorticoids (GCs) is associated with side effects. In contrast to the well-known negative impact on bone tissue exerted by oral GCs, few data are available regarding intravenous GCs. We investigated the influence of intravenous methylprednisolone (IVMP) on bone turnover markers (BTM): amino-terminal propeptide of type I procollagen (P1NP) and the C-terminal telopeptide of type I collagen (CTX), and on calcium metabolism parameters: 1,25-dihydroxyvitamin D (1,25(OH)2D), 25-hydroxyvitamin D (25(OH)D), calcium (Ca), phosphate (P), and intact parathormone (iPTH). Methods: In a prospective study, 23 consecutive subjects with Graves’ orbitopathy were included and treated with IVMP according to the European Group on Graves’ Orbitopathy recommendations. We evaluated effects on BTM occurring during the first 7 days after 0.5 g IVMP, and after the therapy with 12 IVMP pulses with a cumulative dose of 4.5 g. Results: We observed prompt but transient decrease of P1NP (p < 0.001) and the reduction of CTX (p = 0.02) after the first IVMP pulse. Following the full course of IVMP therapy, both P1NP and CTX were found decreased (p < 0.05 and p < 0.01, respectively). Conclusions: A single pulse of 0.5 g IVMP already decreases bone formation and resorption; however, this change is transient. The full therapy is associated with suppression of bone turnover.

Pneumocystis jirovecii-associated immune reconstitution inflammatory syndrome-like phenomenon in a child with leukaemia: a case report and literature review

BackgroundImmune reconstitution inflammatory syndrome (IRIS) refers to the phenomenon of intense immune responses against pathogens in patients with AIDS undergoing antiretroviral therapy to reconstitute immune function, resulting in functional impairment of multiple organs. Non-AIDS immunosuppressed hosts may also develop similar manifestations to IRIS during immune recovery.Case presentationAn 8-year-old girl presented with acute lymphoblastic leukaemia was admitted for scheduled chemotherapy treatment. During chemotherapy, she experienced pancytopenia and Pneumocystis jirovecii pneumonia, which was diagnosed based on the abnormal shadows observed on chest computed tomography, the elevation of serum β-D-glucan, and the positive mNGS results of Pneumocystis jirovecii in both sputum and blood. After treatment with Granulocyte Colony-Stimulating Factor, sulfamethoxazole, and caspofungin, aggravation of lung lesions was discovered and severe interstitial lung disease developed in a short period along with a rapidly increasing leukocyte count. Intravenous methylprednisolone pulse therapy was given, but lung function did not improve, and she finally died after the withdrawal of medical care.ConclusionsFor patients with acute lymphocytic leukaemia infected with Pneumocystis jirovecii, the rapid aggravation of pulmonary lesions in the process of blood recovery and immune reconstitution should raise vigilance against the possibility of IRIS-like reactions. The use of granulocyte stimulating factors may aggravate the inflammatory response in the lungs. The timing, dosage, and duration of treatment of glucocorticoids and the impact of high-dose methylprednisolone pulse therapy on the prognosis of patients should be explored in further research.

Intravenous Methylprednisolone Pulse Therapy Versus Intravenous Immunoglobulin in the Prevention of Coronary Artery Disease in Children with Kawasaki Disease: A Randomized Controlled Trial.

Background: Kawasaki disease (KD) is often complicated by coronary artery lesion (CAL), including dilatation or aneurysms. Intravenous immunoglobulin (IVIG) is used with aspirin to prevent CAL in KD.Objective: Given that the primary treatment for other vasculitis is the use of corticosteroids, this study has been performed to evaluate the effect of intravenous methylprednisolone pulse (IVMP) therapy in preventing CAL in KD.Method: A randomized, single-blind clinical trial was conducted on 40 KD patients aged six months to five years. Patients were randomized into two groups according to the main treatment plan in addition to aspirin: case group (IVMP for three consecutive days and then oral prednisolone for three days) and control group (intravenous immunoglobulin 2 g/kg). Echocardiography was performed for all children at least three times, during the acute phase, two weeks, and two months later.Results: Data analysis at the end of the study was done on 40 patients (20 patients in each group). There were no significant differences in age and sex distribution, mean fever, and acute phase duration, as well as baseline echocardiography in the two groups. The frequency of CAL was 20% in the case group and 45% in the control group, after two weeks (p<0.05), but there was no significant difference between two groups in types of coronary artery lesion after two weeks and the frequency and severity of CAL after two months.Conclusion: IVMP as initial line therapy effectively control systemic and vascular inflammation and decrease coronary artery damage in KD.

MOG antibody‐related disorder associated with peripheral facial nerve palsy

A 44-year-old Japanese woman exhibited neurological symptoms including unsteadiness of gait, headache, hypoesthesia of the left face, and left-sided peripheral facial nerve palsy. Brain MRI revealed abnormal intensities in the hemispheres, brainstem, and corpus callosum. Although routine blood and cerebrospinal fluid studies were unremarkable, serum anti-myelin oligodendrocyte glycoprotein (MOG) antibody was positive. With two-time intravenous methylprednisolone pulse therapy, her symptoms gradually improved. We report here a rare case of a MOG antibody-related disorder with peripheral facial nerve palsy as a predominant symptom. We should be aware of this disease even in a case of peripheral facial nerve palsy.

Intracranial hemorrhage in posterior reversible encephalopathy syndrome due to corticosteroid pulse therapy

We report the case of a 51-year-old woman who developed intracranial hemorrhage due to posterior reversible encephalopathy syndrome (PRES) after intravenous methylprednisolone pulse therapy for Graves’ ophthalmopathy. Corticosteroid therapy very rarely induces PRES. To our best knowledge, no cases of intracranial hemorrhage due to PRES caused by corticosteroid therapy have been reported. The majority of case reports about corticosteroid-induced PRES have been hypothesized to be caused by corticosteroid-induced hypertension. However, this patient did not have high blood pressure until the onset of the intracranial hemorrhage. Corticosteroids themselves can directly induce PRES. Furthermore, although corticosteroids are frequently used to treat vasogenic edema, corticosteroid therapy can aggravate vasogenic edema in PRES. Thus, they are not recommended for the treatment of PRES and should be discontinued immediately upon the onset of PRES.

Hypertrophic pachymeningitis with co-occurrence of anti-neutrophil cytoplasmic antibodies and immunoglobulin G4 seropositivity: Case report and literature review

We report an extremely rare case of hypertrophic pachymeningitis in which a 71-year-old man presented with an intractable recurrent headache for >1 year. During this period, he became positive for immunoglobulin G4 and proteinase 3-antineutrophil cytoplasmic antibodies. Contrast-enhanced magnetic resonance imaging showed characteristic diffuse thickening of the dura. Symptoms were improved by intravenous methylprednisolone (500 mg per day for 5 days) and cyclophosphamide pulse therapy during corticosteroid withdrawal; he remained symptom-free during 1-year follow-up. This case suggests that this disease can be treated by corticosteroids combined with immunosuppressive agents.

Long-Term Visual Prognosis in Patients With Aquaporin-4-Immunoglobulin G–Positive Neuromyelitis Optica Spectrum Disorder

To identify the factors associated with visual prognosis for functional and structural outcomes of optic neuritis (ON) in patients with aquaporin-4-immunoglobulin (AQP4-IgG)-positive neuromyelitis optica spectrum disorder (NMOSD). We included the eyes that experienced at least 1 episode of ON and were followed for at least 2 years after the first attack of ON in patients with AQP4-IgG-positive NMOSD. We performed a retrospective review of clinical data, including ophthalmological examination and orbital MRI, of 34 eyes of 22 patients. Functional outcomes were measured as final visual acuity, visual field index, and mean deviation and structural outcomes as final retinal nerve fiber layer (RNFL) and ganglion cell-inner plexiform layer (GCIPL) thickness. The mean age at onset of the first ON was 42.7 ± 13.7, and all patients were female. The poor visual acuity was significantly associated with the worse final visual acuity and thinner RNFL and GCIPL. Older age also showed a negative correlation with RNFL thickness. The number of attacks was not statistically significant for functional and structural outcomes. The lesion involving the intracanalicular optic nerve to the chiasm on orbital MRI showed worse visual acuity and a thinner GCIPL. Rapid high-dose intravenous methylprednisolone pulse therapy within 3 days was statistically significant, with better visual acuity and more preserved GCIPL thickness. Our results indicate that the severity of ON rather than the number of recurrences might be critical for the visual prognosis of patients with AQP4-IgG-positive NMOSD. Rapid treatment within 3 days may improve visual outcomes, and a younger age at onset may have better visual outcomes.

Short-term outcome of intravenous methylprednisolone pulse therapy versus oral prednisolone in patients with epileptic spasms

BackgroundInfantile spasm (IS) is an age-specific epileptic disorder of early infancy that typically presents with epileptic spasms occurring in clusters. International League Against Epilepsy (ILAE) also recommends the term “IS syndrome” for clustered spasms occurring during infancy (~ 1 year, rarely after 2 years of age) accompanied by electroencephalography (EEG) hypsarrhythmia. Older clinical studies comparing efficacy of adrenocorticotrophic hormone (ACTH) and corticosteroids preferred ACTH, but recent studies did not observe a significant difference between both nor showed better efficacy of oral prednisolone compared to intramuscular synthetic one. Participants were 53 patients with epileptic spasms divided into two groups: 26 patients treated by intravenous methylprednisolone (group A) and 27 treated by oral prednisolone (group B). Both groups were matched in age, sex, and age of spasm onset. The outcome measures were spasms cessation at 7 and 14 days and recurrence at 6 weeks after tapering, EEG improvement at 2 and 6 weeks, and frequency of side effects.ResultsWe found that both groups had similar results regarding frequency of children achieving spasms cessation at day 7 and day 14 (53.8% vs 51.9% and 38.5% vs 40.7%, p = 1.000 each), respectively. After drug tapering, there was insignificant difference between both groups regarding recurrence of spasm at 6 weeks (14.8% vs 11.5%, p = 1.000) and EEG improvement at 2 weeks (42.3% vs 33.3%, p = 0.577) with nearly similar improvement in both groups at 6 weeks (46.2% vs 48.1%, p = 1.000). With regard to adverse effects of both therapies, no difference observed between both groups.ConclusionsNo significant difference was found between oral prednisolone and intravenous methylprednisolone in treatment of epileptic spasm regarding short-term outcomes. The only advantage of intravenous therapy is decreased time to get response and the only advantage of oral therapy is its applicability at home without hospitalization. Further studies are warranted to explore the long-term outcomes.

Bilateral Optic Neuritis Caused by Meningococcal Meningoencephalitis

AbstractIn this article, we described a case of pathogen-induced bilateral optic neuritis accompanying meningococcal meningoencephalitis in an adolescent male. A 15-year-old boy presented to our emergency room due to progressive severe headache, stiff neck, diffuse extremity pain, fever, and nausea concerning meningoencephalitis. Intravenous ceftriaxone, metamizole, and ondansetron were started immediately. Due to acute autonomic dysregulation and development of petechiae, he was transferred to the pediatric intensive care unit. Next morning, the patient developed severe visual impairment. Investigations revealed elevated C-reactive protein, procalcitonin and leucocyte count, decreased platelet count, and clotting activation. Cerebrospinal fluid (CSF) analysis revealed increased leucocyte count, protein, and decreased glucose concentration with pathological CSF cytology. Brain magnetic resonance imaging revealed an increased contrast enhancement in the optic nerve sheath, consistent with acute bilateral optic neuritis. He was started on high-dose intravenous pulse methylprednisolone therapy. After treatment with ceftriaxone und pulse steroids, the patient was discharged in good condition without any visual sequel.

Cryptogenic new-onset refractory status epilepticus successfully treated with intravenous methylprednisolone pulse therapy and intravenous immunoglobulin

Cryptogenic new-onset refractory status epilepticus successfully treated with intravenous methylprednisolone pulse therapy and intravenous immunoglobulin

Prostate abscess with acute urinary retention as the initial presentation of granulomatosis with polyangitis

A 50-year-old male presented with acute urinary retention. He also had a history of cough, haemoptysis, exertional dyspnoea, loss of appetite, generalised weakness and left-ear discharge of 1-month duration. Prior episode of acute urinary retention had occurred 2 months ago; diagnostic evaluation revealed prostate abscess, and he had received treatment elsewhere for the same. He also had a history of hypertension. Physical examination revealed fever, pallor, bilateral parotid enlargement and left-ear serous discharge. Clinical examination, imaging of chest revealed consolidation with cavitation on the left side, bilateral pleural effusion. Laboratory testing revealed normocytic normochromic anaemia (haemoglobin 6.6 g/dL); neutrophilic leucocytosis (total leucocyte count 13,100 cells/mm3; polymorphs 87%); raised erythrocyte sedimentation rate (110 mm at the end of the first hour); elevated serum creatinine (2.69 mg/dL) and an active urine sediment. Flexible fibreoptic bronchoscopy showed alveolar haemorrhages. Bronchoalveolar lavage fluid Xpert MTB/RIF testing, cytopathology were negative. Computed tomography-guided biopsy from the lesion was suggestive of granulomatous vasculitis. Cytoplasmic antineutrophil cytoplasmic antibodies (C-ANCA) tested positive. The patient was diagnosed to have granulomatosis with polyangitis (GPA). As arterial hypoxaemia was evident, tracheal intubation and mechanical ventilatory support were initiated. Renal replacement therapy, intravenous (iv) methyl prednisolone and cyclophosphamide pulse therapy and five sessions of plasmapheresis were administered. As remission could not be achieved, iv rituximab was started. On the 36th day of admission, the patient developed septic shock and died. The present case documents the uncommon association of GPA with prostate abscess, acute urinary retention.

Factors influencing intravenous methylprednisolone pulse therapy in Chinese patients with isolated optic neuritis associated with AQP4 antibody-seropositive neuromyelitis optica

This study investigated the factors influencing intravenous methylprednisolone pulse (IVMP) therapy for recovering visual acuity in Chinese patients with aquaporin-4 (AQP4) antibody-seropositive neuromyelitis optica-related optic neuritis (NMO-ON). This retrospective case series included 243 affected eyes of 182 patients (36 male, 146 female) diagnosed with NMO-ON in the Neuro-Ophthalmology Clinic of Beijing Tongren Hospital from September 2012 to September 2020. All patients with AQP4-antibody seropositivity had clinical manifestations of acute ON, excluding other diagnoses and received IVMP treatment at 500 mg/day or 1000 mg/day for 3 days. Primary outcome was the extent of improvement in logMAR visual acuity after IVMP treatment. The therapeutic influences of sex, age, baseline visual acuity, therapeutic intervals, and IVMP dose on acute NMO-ON were analysed. Chi-square tests, Mann–Whitney U-tests, Kruskal–Wallis tests, Spearman’s correlation coefficients, and multiple linear regression were used for statistical analysis. Age ranged between 7 and 80 years (median age, 44; interquartile range [IQR], 29–52) years. Among the 243 eyes, the median improvement in logMAR visual acuity was 0.3 (IQR, 0–0.9). Therapeutic efficacy of IVMP was significantly higher in female than in male patients (Z = 2.117, P = 0.034). The treatment effect gradually decreased with increase in age at onset (Rs = 0.157, P = 0.015), and visual improvement was significantly lower in patients aged > 50 years than in those ≤ 50 years (Z = 2.571, P = 0.010). When patients had low visual acuity at onset, improvements were more obvious (rho = − 0.317, P < 0.001); however, final visual acuity was still low (rho = 0.688, P < 0.001). Therapeutic effect was negatively correlated with therapeutic intervals (rho = 0.228, P = 0.001). Dosage of methylprednisolone (1000 mg/day or 500 mg/day) did not significantly influence treatment efficacy (Z = 0.951 P = 0.342). Therefore, IVMP therapy can improve visual acuity in the affected eyes of patients with AQP4 antibody-seropositive NMO-ON with similar effect at 500 mg/day and 1000 mg/day doses. Sex, age at onset, and therapeutic intervals may influence the efficacy of IVMP in patients with NMO-ON.

Macrophage activation syndrome triggered by systemic lupus erythematosus flare: successful treatment with a combination of dexamethasone sodium phosphate, intravenous immunoglobulin, and cyclosporine: a case report

BackgroundMacrophage activation syndrome is classified as a secondary form of hemophagocytic lymphohistiocytosis. It is a hyperinflammatory complication observed to be comorbid with a variety of autoimmune diseases, including adult-onset Still’s disease and systemic juvenile idiopathic arthritis. Macrophage activation syndrome is less commonly detected in adult patients with systemic lupus erythematosus, which, if untreated, can be fatal, though determining the optimum treatment strategy is still a challenge.Case presentationHerein, we report a case of macrophage activation syndrome in a 33-year-old Egyptian female as an unusual complication of a systemic lupus erythematosus flare in adult patients. Our patient was initially treated with a combination of intravenous methylprednisolone pulse therapy and intravenous immunoglobulin therapy, which was followed by a course of oral prednisolone and oral cyclosporine with little response. Switching from oral prednisone to intravenous dexamethasone sodium phosphate showed a more favorable clinical and biochemical response.ConclusionMacrophage activation syndrome is less commonly detected in adult patients with systemic lupus erythematosus. Our case demonstrates that dexamethasone sodium phosphate can be a successful alternative treatment for patients with systemic lupus erythematosus complicated by macrophage activation syndrome in whom the response to pulse methylprednisolone was inadequate to manage their illness, proving to be remarkably effective in a relatively short time frame.

Therapeutic Response and Possible Biomarkers in Acute Attacks of Neuromyelitis Optica Spectrum Disorders: A Prospective Observational Study.

ObjectiveTo explore the outcomes of NMOSD attacks and investigate serum biomarkers for prognosis and severity.MethodPatients with NMOSD attacks were prospectively and observationally enrolled from January 2019 to December 2020 at four hospitals in Guangzhou, southern China. Data were collected at attack, discharge and 1/3/6 months after acute treatment. Serum cytokine/chemokine and neurofilament light chain (NfL) levels were examined at the onset stage.ResultsOne hundred patients with NMOSD attacks were included. The treatment comprised intravenous methylprednisolone pulse therapy alone (IVMP, 71%), IVMP combined with apheresis (8%), IVMP combined with intravenous immunoglobulin (18%) and other therapies (3%). EDSS scores decreased significantly from a medium of 4 (interquartile range 3.0–5.5) at attack to 3.5 (3.0–4.5) at discharge, 3.5 (2.0–4.0) at the 1-month visit and 3.0 (2.0–4.0) at the 3-month visit (p<0.01 in all comparisons). The remission rate was 38.0% at discharge and 63.3% at the 1-month visit. Notably, relapse occurred in 12.2% of 74 patients by the 6-month follow-up. Higher levels of T helper cell 2 (Th2)-related cytokines, including interleukin (IL)-4, IL-10, IL-13, and IL-1 receptor antagonist, predicted remission at the 1-month visit (OR=9.33, p=0.04). Serum NfL levels correlated positively with onset EDSS scores in acute-phase NMOSD (p<0.001, R2 = 0.487).ConclusionsOutcomes of NMOSD attacks were generally moderate. A high level of serum Th2-related cytokines predicted remission at the 1-month visit, and serum NfL may serve as a biomarker of disease severity at attack.Clinical Trial Registrationhttps://clinicaltrials.gov/ct2/show/NCT04101058, identifier NCT04101058.