Intravenous Methylprednisolone Pulse Therapy Research Articles

The role of thyrotropin receptor antibodies in Grave’s Ophthalmopathy treatment

Background. Graves' ophthalmopathy (GO) is an autoimmune inflammatory disorder affecting the retroorbital tissues. Although the role of TRAb in GO is now accepted by many researchers and clinicians, their use in the disease management of GO is less well studied than the role of TRAb for the diagnosis and therapy monitoring of Graves’ disease.Aim: to evaluate the relation between TRAb level and the activity of GO, the course of GO and the effectiveness of the treatment.Materials and methods. We have studied 26 patients with GO and Grave’s Disease. Activity of GO was measured with the clinical activity score (CAS), we defined active GO as a CAS≥3. TSH, FT4 and TRAb were evaluated. All patients had received intravenous methylprednisolone (ivMP) pulse therapy in cumulative dose 6000 mg. We observed patients for 1 year after pulse therapy. TRAb level was evaluated before, 3, 6 and 12 months after pulse therapy.Results: At the time of initial treatment all patients had active GO, 60% with CAS 3-4 and 40% with CAS 5-7. On year after the pulse therapy of GO, all patients were classified into responders (69,2%) and non-responders (30,8%) according to their clinical manifestations. Pulse therapy considered as effective if GO activity decreased with CAS ≤ 2. Serum TRAb level was significantly higher in patients who non-responded to therapy – 34,8 U/L vs 17,5 U/L (p≤0,01). This level was significantly decreased in patients responded to treatment – 1,6 U/L vs 12,4 U/L (p≤0,01). TRAb level above 28,8 U/L before treatment (p≤0,01), 10,1 U/L after 3 months of treatment (p≤0,01), 5,1 U/L after 6 months of treatment (p≤0,01) and 8,2 U/L after 3 months of treatment (p≤0.01) was associated with higher risk of non-responding.Conclusion: We conclude, that TRAb level may serve not only as predictor of GO activity and severity, but changes in the level of antibodies could be of additional help for the disease management with ivMP.

Dermatomyositis complicated with asymmetric peripheral neuritis on exacerbation: A case report and literature review

AbstractWe describe a 69‐year‐old woman with dermatomyositis who presented with asymmetric peripheral neuritis. The patient first developed dermatomyositis at 39 years‐of‐age; her symptoms improved several years later with oral prednisolone treatment. A year and a half before admission to our hospital, she developed a sensory disturbance in her feet that gradually expanded to both legs with left dominance. Six months later, bilateral leg muscle weakness and proximal muscle tenderness appeared. Neurological examination at admission revealed proximal muscle weakness and atrophy of all four limbs. Ankle jerk was absent bilaterally. There was sensory impairment in the left hand and both lower limbs, with more severe involvement of the left side and the medial side. Electrophysiological tests showed decreased sensory nerve action potentials in the left superficial fibular nerve and bilateral sural nerves, and decreased motor action potentials in the bilateral fibular nerves. We diagnosed mononeuritis multiplex as a complication of dermatomyositis. Her symptoms of mononeuritis multiplex and dermatomyositis were successfully treated with intravenous methylprednisolone pulse therapy followed by oral prednisolone therapy. Although our literature search found nine cases of dermatomyositis with polyneuropathy/polyradiculoneuropathy, most of which involved axonal neuropathy, this is the first case report of dermatomyositis presenting with mononeuritis multiplex.

Graves' orbitopathy treated with immunosuppressive therapy and orbital radiotherapy in a tertiary care endocrine centre: case series

Graves’ Orbitopathy (GO) is a localized entity of Graves’ disease, which is a challenging disease to treat. We describe 6 cases of moderate to severe GO who presented to our endocrine centre, with a mean age of 48.6 years and equal gender distribution. All of them were thyrotoxic before the presentation and were treated with antithyroid drugs. CT scans of orbits in 5 patients were consistent with GO. All of the patients were treated with intravenous methylprednisolone pulse therapy for a duration of 12 weeks and orbital radiotherapy for two weeks according to European Groups on Graves' Orbitopathy (EUGOGO) guideline. All six patients went into remission and remained so after one year of follow-up. Combination therapy seems to be effective in treating moderate to severe GO.

Steroid pulse therapy prevents restenosis following balloon dilatation for esophageal stricture.

This study aimed to evaluate the effectiveness of intravenous steroid pulse therapy following balloon dilatation for esophageal stenosis and stricture in children. The study enrolled six children, including three with congenital esophageal stenosis and three with anastomotic strictures after surgery for esophageal atresia, all of whom were treated by balloon dilatation combined with high-dose intravenous methylprednisolone pulse therapy. Methylprednisolone was injected intravenously at a dose of 20mg/kg/day for 2days, starting from the day of dilatation, followed by 10mg/kg/day for 2days, for a total of 4days. Esophageal stricture recurred in all three patients with congenital esophageal stenosis despite repeated balloon dilatation without methylprednisolone. However, the symptoms of dysphagia improved and did not recur after systemic steroid pulse therapy following balloon dilatation. Symptoms also resolved in all three patients with anastomotic strictures following balloon dilatation with systemic steroid pulse therapy. All six patients remained asymptomatic after 6-21months follow-up, with no complications. Intravenous methylprednisolone pulse therapy following balloon dilatation is safe and effective for the treatment of esophageal stenosis and strictures in children.

Efficacy of intravenous methylprednisolone pulse therapy in patients with multiple sclerosis and neuromyelitis optica

Background: No large-scale studies have compared the efficacy of intravenous methylprednisolone pulse therapy (IVMP) for multiple sclerosis (MS) and neuromyelitis optica (NMO). Objective: To explain differences in treatment responses of MS and NMO patients to IVMP. Methods: Changes in neurological symptoms/signs and Expanded Disability Status Scale (EDSS) scores before and within 1 week of IVMP completion were obtained in 2010 at 28 institutions, and retrospectively collated from 271 MS (478 courses) and 73 NMO (118 courses) cases. Results: In MS patients, decreased EDSS score was significant after the first (−0.8 ± 0.9), second (−0.7 ± 0.9), and third (−0.7 ± 0.8) courses (p < 0.05), but not after the fourth (−0.3 ± 0.7) and fifth (−0.5 ± 0.6). However, decreased EDSS score was only significant after the first course (−0.5 ± 1.5, p < 0.05) in NMO patients. EDSS score was significantly decreased in MS compared with NMO patients at the first course (p < 0.05), but not thereafter. Model analysis for EDSS score improvement at the first course, adjusting for covariates, showed significantly greater decreases in MS compared with NMO patients (p < 0.05). Conclusion: IVMP is effective in MS from the first to third courses, and in NMO at the first course. Additionally, IVMP is more efficacious in MS than NMO patients, even at the first course.

Short and long-term effects of high-dose intravenous methylprednisolone pulse therapy on thyroid-associated ophthalmopathy.

The majority of previous studies on high-dose intravenous methylprednisolone pulse (IVMP) therapy have observed the clinical conditions of patients prior to and following treatment without any long-term follow-up, and these studies have predominantly focused on combined treatment. The present prospective clinical study aimed to assess the long-term effects and safety of high-dose IVMP therapy in thyroid-associated ophthalmopathy (TAO), as well as the significance of thyrotropin receptor antibody (TRAb) and soluble intercellular adhesion molecule-l (sICAM-1) during IVMP therapy. A total of 58 patients with TAO were treated with high-dose IVMP therapy, and their clinical characteristics and indices were recorded before, during and after therapy, with a 12–57 month (mean, 28.4 months) follow-up. Before treatment and on the second day after each IVMP therapy, serum TRAb and sICAM-1 levels were evaluated in 23 patients with TAO via a competitive radioimmunoassay and enzyme-linked immunosorbent assay, respectively. The results of the present study demonstrated that the symptoms of eyelid swelling, ophthalmodynia, photophobia, lacrimation and diplopia, and visual acuity, ocular motility, proptosis and clinical activity score (CAS) indices were all significantly improved after IVMP therapy. In addition, analysis of covariance demonstrated that alterations in the levels of serum TRAb during the course of treatment were associated with CAS of TAO, whereas the change in serum sICAM-1 was not. In conclusion, high-dose IVMP therapy is an effective, safe, stable and well-tolerated treatment for TAO, which is associated with rare, minor adverse effects. Furthermore, serum TRAb levels are correlated with the CAS of TAO and may serve as a predictor of the response to methylprednisolone therapy.

Clinical efficacy of combined rituximab treatment in a woman with severe Graves' ophthalmopathy.

The present study reports the case of a female Chinese patient with Graves' disease (GD) and severe Graves' ophthalmopathy (GO) in its active phase, who was treated with propylthiouracil and oral prednisolone for 2 months at a local hospital. However, a lack of improvement in symptoms meant that the patient was transferred to the First Affiliated Hospital of Xi'an Jiaotong University (Xi'an, China), whereupon the patient received high-dose intravenous methylprednisolone pulse therapy, although with limited efficacy. Subsequently, rituximab (RTX; anti-CD20 monoclonal antibody) combined with orbital irradiation treatment was initiated. The patient responded positively to the combined treatment; the clinical symptoms and enlargement of the extraocular muscles were ameliorated, and there were marked decreases in the clinical activity and NOSPECS grading scores. Furthermore, the serum levels of anti-thyrotropin receptor antibodies (TRAb) were markedly decreased at 2 months following RTX therapy. The patient was maintained in a euthyroid state by treatment with methimazole during and following RTX therapy. It was concluded that RTX treatment may attenuate severe GO by depleting lymphocytes, and may promote the recovery of GD by reducing the serum levels of TRAb.

Complete recovery of visual acuity as the main goal of treatment in patients with dysthyroid optic neuropathy.

To evaluate the effectiveness of methylprednisolone (MP) and surgical treatment in achieving complete reversal of dysthyroid optic neuropathy (DON) and predictive factors of this therapy. The group consisted of 10 patients (18 eyes) with DON. The diagnosis of DON was based on at least two criteria from the following: (i) deterioration of visual acuity (VA< 1.0), (ii) loss of colour vision, (iii) optic disc swelling, and/or (iv) signs of DON in magnetic resonance imaging (presence of apical crowding and/or optic nerve stretching). A complete recovery of DON was defined as the normalisation of VA (VA = 1.0), normal colour vision, and reversal of optic disc swelling. A significant improvement was defined as improvement of VA of at least 0.2. The consecutive steps of treatment of DON consisted of: (i) first-line treatment - intravenous MP pulse therapy (3 × 1 g); (ii) second-line treatment - endoscopic intranasal orbital decompression of medial wall; (iii) additional treatment - additional MP therapy and/or surgical decompression. A significant improvement in VA could be achieved in the majority of patients; a complete recovery was noted in 22.2%, 33.3%, and 66.7% of eyes after first-line, second-line, and additional treatment, respectively. Positive predictive factors were: younger age (p = 0.049), shorter duration of DON (p = 0.035), and a higher Graves' orbitopathy clinical activity score (p = 0.035). By using combination therapy (intravenous MP pulse therapy and surgical decompression), a complete recovery can be achieved in the majority of patients with DON.

How to Manage Electrical Status Epilepticus in Sleep.

Electrical status epilepticus in sleep is an age-dependent syndrome with the characteristic pattern of continuous spike and waves during non-rapid eye movement sleep. Most children can present developmental deterioration. The demonstration of the EEG pattern has to rely on all night long EEG recordings. A comprehensive neuropsychologic evaluation with periodic reassessment should be performed. For the idiopathic forms of electrical status epilepticus in sleep, clobazam could be considered as the first-line therapy; in the other cases, corticosteroids, in particular intravenous methylprednisolone pulse therapy, remain the most effective and should be considered the therapy of choice.

Successful Tocilizumab Therapy for Macrophage Activation Syndrome Associated with Adult-Onset Still's Disease: A Case-Based Review.

We report the case of a 71-year-old Japanese woman with adult-onset Still's disease (AOSD) in whom macrophage activation syndrome (MAS) developed despite therapy with oral high-dose prednisolone and intravenous methylprednisolone pulse therapy twice. She was successfully treated with tocilizumab (TCZ). Soon afterward, her fever ceased and high levels of both ferritin and C-reactive protein levels decreased. Her course was complicated by disseminated intravascular coagulation, cytomegalovirus infection, and Pneumocystis jirovecii pneumonia. After these were resolved, AOSD-associated MAS was well controlled. She was discharged on hospital day 87. Although biologics such as TCZ are becoming established for the treatment of AOSD, there is no recommended therapy for AOSD-associated MAS. Several biologics have been tried for this complication, but their efficacy and safety remain controversial. We reviewed reported cases of AOSD-associated MAS successfully treated with various biologics. TCZ initiation after adequate nonselective immunosuppressive therapy, such as methylprednisolone pulse therapy or a prednisolone-based combination of immunosuppressants, can be an effective treatment for AOSD-associated MAS. On the other hand, biologics given after insufficient immunosuppressive therapy may cause MAS. A strategy combining adequate immunosuppression and a biologic could be safe if special attention is given to adverse events such as opportunistic infections or biologic-associated MAS.

Multiple Cerebral Infarctions as an Initial Manifestation of Systemic Lupus Erythematosus with Concomitant Hypereosinophilic Syndrome

A 51-year old woman with drowsy consciousness and weakness in bilateral upper and lower extremities was admitted to our hospital. Laboratory test on admission showed eosinophilia, proteinuria, hypoalbuminemia, and elevated creatine kinase-MB. She also had positive antinuclear antibody, anti-ribonucleoprotein antibody and anti- Smith antibody as well as decreased levels of complements. Brain MRI showed multiple cerebral infarcts in both hemispheres. Renal biopsy determined class IV lupus nephritis. Vasculopathy and hypercoagulopathy induced by systemic lupus erythematosus rarely concomitant with hypereosinophilic syndrome were considered as main causes of multiple cerebral infarctions in this patient. The symptoms and laboratory data were gradually recovered with an intravenous methylprednisolone pulse therapy followed by oral prednisolone and monthly intravenous cyclophosphamide therapies.

Methylprednisolone Pulse Treatment of Graves' Ophthalmopathy Is Not Associated with Secondary Adrenocortical Insufficiency

Objective: Graves' ophthalmopathy (GO) is an inflammatory disease in the orbital region. The first-line medical treatment is glucocorticoids. An important potential side effect of glucocorticoid treatment is suppression of the hypothalamic-pituitary-adrenal (HPA) axis with impairment of endogenous cortisol production, implicating symptoms of adrenocortical insufficiency, especially in the period after cessation of therapy with possible risks in cases of intercurrent illness. The aim of this study was to evaluate HPA axis function before and after methylprednisolone pulse treatment of GO. Study Design: HPA axis function was evaluated by measurements of plasma ACTH and an ACTH stimulation test with plasma cortisol measurements at 0 and 30 min after an intravenous bolus of synthetic ACTH (Synacthen® 250 µg). This was done in 12 patients with GO before and at cessation of methylprednisolone pulse treatment (500 mg i.v. per week for 6 weeks followed by 250 mg i.v. per week for an additional 6 weeks). Results: All patients included fulfilled the criteria of intact HPA axis function before and at cessation of methylprednisolone pulse treatment. Data are given as medians (with ranges). Before glucocorticoid treatment basal plasma cortisol was 290 n<smlcap>M</smlcap> (196-579) and 786 n<smlcap>M </smlcap>(612-1,050) after ACTH stimulation. At cessation of therapy the corresponding values were 309 n<smlcap>M</smlcap> (88-718) and 852 n<smlcap>M</smlcap> (524-1,011), respectively. Thus, all patients passed a 30-min stimulated plasma cortisol of 500 n<smlcap>M</smlcap>. Before treatment plasma ACTH was 4.2 pmol/l (4-16) and at cessation of therapy the corresponding value was 4.8 pmol/l (2-9; p = 0.27). Conclusion: Transient suppression of the HPA axis with secondary adrenocortical insufficiency does not seem to be a common phenomenon after intravenous methylprednisolone pulse therapy for GO. Therefore, routine precautions are not necessary. However, our results do not exclude that transient secondary adrenocortical insufficiency might occur occasionally.

Serum concentrations of HGF and IL-8 in patients with active Graves' orbitopathy before and after methylprednisolone therapy.

IntroductionGraves’ disease is the most common cause of hyperthyroidism, and orbitopathy is the most frequent extrathyroidal manifestation of Graves’ disease. The aims of this study were as follows: (1) to evaluate the serum concentration of HGF and IL-8 in the blood of newly diagnosed Graves’ disease patients with the first episode of active GO and healthy controls; (2) to estimate the influence of the thyroid function (euthyreosis vs. hyperthyreosis) on HGF and IL-8 blood levels in patients with active GO; (3) to evaluate the influence of intravenous (i.v.) methylprednisolone (MP) pulse therapy and additional oral MP treatment on HGF and IL-8 blood levels in patients with active GO.Patients and methodsThirty-nine Graves’ disease patients with the first episode of clinically active GO (Group A) were enrolled in the study. To estimate the influence of the thyroid function on serum concentrations of the studied proangiogenic factors, Group A was divided into Group A I (n = 18) in euthyroid and Group A II (n = 21) in hyperthyroid stage of Graves’ disease in moderate-to-severe stage of GO. The control group consisted of 20 healthy volunteers age- and sex-matched to the GO group. Concentrations of the studied proangiogenic factors in serum samples were measured by an enzyme-linked immunosorbent assay before (Group A) and after (Group A1) intensive pulse i.v.MP treatment and 1 month after the end of additional oral MP treatment (Group A2).ResultsWe found a significant increase in serum concentrations of studied factors in the GO group before immunosuppressive therapy when compared with the control group and decrease after i.v.MP treatment. One month after the end of additional oral MP treatment (Group A2), serum concentrations of HGF and IL-8 still decreased and no significant difference was observed in HGF and IL-8 concentrations when compared with the control group. We did not find the difference in serum concentration of the studied proangiogenic factors between patients in euthyroid and hyperthyroid stage of Graves’ disease before MP therapy.ConclusionsSerum HGF and IL-8 concentrations are elevated in Graves’ disease patients with active Graves’ orbitopathy as compared to the healthy control group. Successful management of active Graves’ orbitopathy with glucocorticoids is associated with a decrease in HGF and IL-8 serum concentrations.

Abstract 123: Long term Prognosis After Methylprednisolon pulse Combination Therapy For Refractory Kawasaki Disease

Background: We previously investigated the molecular mechanism and the clinical utility of intravenous methylprednisolone-pulse(IVMP) plus intravenous immunoglobulin (IVIG) combination therapy (IVMP+IVIG) for patients with Kawasaki disease (KD) in the acute phase (Pediatr Res 2009, Pediatrics 2012). However, the long-term prognosis after IVMP+IVIG still remains unclear. Objective: To examine the long-term prognosis after IVIG+IVMP. Methods: Forty-six patients (Age: 5-70 months, male: 27) who were enrolled in this study were treated with IVMP+IVIG during the period from 2007 to 2014 at Kitasato University Hospital who were all predicted to show resistance to initial IVIG according to their Egami score (J Pediatr 2006). The IVMP therapy (30mg/kg, 1dose) was administered for 2 hours before starting the IVIG therapy. Heparin (10U/kg/h, continuous infusion) was used concomitantly from 2 hours before the start of IVMP therapy and it was thereafter continued for 24 hours. The long-term prognosis of coronary artery lesions (CALs) and adverse events between the time of diagnosis and 5 years after IVMP +IVIG were determined. The “Z-score2.5-5” and “Z–score&gt;5” were defined as dilatation and an aneurysm, respectively, according to the AHA guidelines. Results: Dilatation cases based on the Z-score2.5-5, were identified in 15 of 46 patients (32.6%) at diagnosis, in 15 of 46 patients (32.6%) after 1 month, and in 3 of 36 patients (8.3%) after 1 year. All patients in whom coronary artery abnormalities were observed at 1 year later demonstrated regression.No patients showed a Z-score≧5 in all phases. Thirty-six patients (78.2%) showed prompt defervescence owing to the combination therapy. The patients had adverse event at acute phase, 16 of 46 patients (34.7%) for hypothermia, 5 patients (10.8%) for hypertension, 2 patients (4.3%) for bradycardia, and 4 patients (8.6%) for recurrent fever. No patients experienced any serious adverse events (short stature, hyperlipidemia, abnormal glucose tolerance) after IVMP +IVIG during the long-term follow-up. Conclusions: This study showed IVMP+IVIG combination therapy to therefore effectively prevent CALs in KD patients that are predicted to be resistant to intial IVIG according to their Egami score.

The diagnostic value of orbital magnetic resonance imaging T2-Mapping in patients with active Graves' ophthalmopathy

Objective To study the clinical value of orbital magnetic resonance imaging(MRI) in diagnosis and making a judgment about the curative effect of glucocorticoid in patients with Graves' ophthalmopathy(GO). Methods A total of 106 patients with GO were divided into active GO group or inactive GO group according to whether clinical activity score(CAS)≥3 or not, respectively. T2 relaxation times(T2RTs)(ms) and areas(mm2) of four extra-ocular muscles (EOMs) (superior, inferior, medial, and lateral rectus) were directly measured by MRI T2-Mapping. Correlation analysis of T2RTs and areas with eyeball motility, diplopia, exophthalmus, visual acuity and intraocular pressure(IOP) were also performed. The T2RTs and areas of EOMs before and after intravenous methylprednisolone(iv MP) pulse therapy were recorded, respectively. Results The mean T2RTs(88.9±13.8) and mean areas(58.1±23.8) of EOMs in active GO were significantly greater than those in inactive GO(80.6±10.0 and 46.2±18.6, respectively), bing both significantly greater than those in volunteers with normal eyes(76.2±4.7 and 30.3±6.1, respectively)(P<0.01). Maximal T2RTs and maximal areas of EOMs showed significant positive correlation(P<0.01), and both were positively correlated weakly with CAS, reduced movement of eyeball, diplopia, exophthalmus and loss of visual acuity(P<0.01). On the contrary, maximal T2RTs and maximal areas of EOMs showed negative correlation or no correlation with spontaneous retrobulbar pain, painful eye movement, redness of eyelids and abnormal IOP. Even if 1≤CAS<3, ophthalmic symptoms and signs also showed a positive responses to iv MP in patients with moderate to severe GO as long as prolonged T2RTs occurred at least in one of EOMs. The maximal T2RTs, maximal areas, mean T2RTs and mean areas of EOMs were significantly decreased at the end of iv MP treatment, meanwhile ophthalmic symptoms and signs were obviously improved. Conclusions Prolonged T2RTs, especially accompanied by enlarged area on the identical EOMs is an remarkable symbols of acute inflamed muscles. The findings suggested that the use of prolonged T2RTs can improve diagnostic sensitivity and predict the curative effect of iv MP in patients with moderate to severe GO and CAS≥1. (Chin J Endocrinol Metab, 2015, 31: 327-332) Key words: Graves' ophthalmopathy; Magnetic resonance imaging; Methylprednisolone

High Relapse Rates Despite Early Intervention with Intravenous Methylprednisolone Pulse Therapy for Severe Childhood Alopecia Areata.

Previous data suggest that early application of intravenous methylprednisolone pulse therapy (IV-MPPT) may improve the disease course of alopecia areata. The objective of this study was to investigate the outcome of IV-MPPT in severe childhood alopecia areata, predominantly with short disease duration. Eighteen children (10 girls, 8 boys) younger than 17years old (median age 7.7yrs, range 2.1-16.5yrs) treated with IV-MPPT for severe childhood alopecia areata in a referral center for pediatric dermatology over 3years (median disease duration 4mos, range 1-12mos) were retrospectively evaluated. Five patients had alopecia areata totalis or universalis and 13 had alopecia multilocularis. The median scalp area affected by alopecia was 60% (range 30%-100%). All patients underwent two or three cycles of IV-MPPT at monthly intervals (maximum 500mg/day on three consecutive days). Within 7months after the last IV-MPPT session, 10 of 18 children had good response (≥75% of hair regrowth), with eight showing improvement within the first 4 months. Of the remaining eight patients, one had moderate response (50%-74% regrowth), three had poor response (1%-49% regrowth), and four (all with alopecia areata universalis or totalis) had no response. Seven of the initial 10 good responders experienced relapses, with marked hair loss after the last IV-MPPT session. The estimated median time to relapse was 8months (95% confidence interval 7, 9mos). IV-MPPT, even early in the course of disease, did not affect long-term outcome of alopecia areata in our group of severely affected patients.

Post-infectious bronchiolitis obliterans in children: CT features that predict responsiveness to pulse methylprednisolone.

Intravenous pulse methylprednisolone therapy (IPMT) is an important treatment option for post-infectious obliterative bronchiolitis (OB), although it must be used carefully and only in selected patients because of its drawbacks. This study evaluated whether CT and clinical features of children with post-infectious OB can predict their responsiveness to IPMT. We searched the medical records for patients (less than 18 years of age) who were diagnosed with post-infectious OB between January 2000 and December 2011. 17 children who received IPMT were included in this study. All underwent chest CT before and after IPMT. The radiological features seen on pre-treatment CT were recorded. The air-trapping area percentages on pre- and post-treatment CT images were determined. The nine patients who exhibited decreased air trapping on post-treatment CT scans relative to pre-treatment scans were classed as responders. The patient ages and time from initial pneumonia to IPMT were recorded. All responders and only four non-responders had thickened bronchial walls before treatment (p = 0.029). The two groups did not differ significantly in terms of bronchiolitis, bronchiectasis or the extent of air trapping, although the responders had a significantly shorter median interval between initial pneumonia and IPMT (4 vs 50 months; p = 0.005) and were significantly younger (median, 2.0 vs 7.5 years; p = 0.048). Immediate IPMT may improve the degree of air trapping in children with post-infectious OB if they show a thickened bronchial wall on CT. Children with post-infectious OB may respond favourably to IPMT when pre-treatment CT indicates bronchial-wall thickening.

Liver Dysfunction Associated with Intravenous Methylprednisolone Pulse Therapy in Patients with Graves' Orbitopathy.

Intravenous methylprednisolone (IVMP) pulse therapy is the first-line treatment for the active phase of moderate to severe Graves' orbitopathy (GO). However, acute and severe liver damage has been reported during and after IVMP therapy. In this retrospective study, we investigated risk factors for liver dysfunction during and after IVMP therapy based on 175 Japanese patients with moderate to severe GO and treated at our center between 2003 and 2011. The results showed that seven patients developed severe liver dysfunction with elevated serum alanine aminotransferase (ALT > 300 U/L). Mild (40–100 U/L) and moderate (100–300 U/L) increases of ALT occurred in 62 patients (35%) and 10 patients (6%), respectively. Liver dysfunction was more frequently observed in males, in patients receiving high-dose methylprednisolone, and patients aged over 50 years. Preexistent viral hepatitis was significantly associated with liver dysfunction (65% in patients positive for hepatitis B core antibody and patients positive for hepatitis C virus antibodies). Our study confirmed the association of liver dysfunction with IVMP during and after treatment. It suggests that, in patients with GO, evaluation of preexisting risk factors—including viral hepatitis—and careful weekly monitoring of liver function during IVMP therapy and monthly thereafter for 12 months are warranted.

Intravenous methylprednisolone pulse therapy for children with epileptic encephalopathy.

The aim of this retrospective study of children affected by epileptic encephalopathy was to evaluate seizure frequency, electroencephalographic pattern and neuropsychological status, before and after intravenous methylprednisolone therapy. Eleven children with epileptic encephalopathy were administered one cycle of intravenous methylprednisolone (15-30 mg/kg/day for three consecutive days, once a month for four months) in addition to constant dosages of their regular antiepileptic drugs. The treatment resulted in statistically significant reductions of generalized slow spike-and-wave discharges (p<0.0028) and seizure frequency (p<0.013), which persisted even after methylprednisolone pulse therapy was stopped. A globally positive outcome was noted in 9/11 patients (81.8%). This methylprednisolone treatment regimen did not cause significant or persistent adverse effects. We suggest that children with epileptic encephalopathy without an underlying structural lesion could be the best candidates for intravenous methylprednisolone pulse therapy.

Three Cases of Myasthenia Gravis Treated Effectively with High-dose Intravenous Methylprednisolone Pulse Therapy for the Extraocular Muscle Paralysis

Three Cases of Myasthenia Gravis Treated Effectively with High-dose Intravenous Methylprednisolone Pulse Therapy for the Extraocular Muscle Paralysis