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Statin‐induced autoimmune necrotizing myositis is a rare but important cause muscle weakness. Withdrawal of the statin and steroid treatment alone may be insufficient treatment for SIANM. Targeted immunosuppression may be needed and can be effective.

Background and Aims Imaging tools for predicting pancreatic atrophy after steroid therapy in autoimmune pancreatitis (AIP) have not been established. As delayed equilibrium‐phase contrast enhancement in computed tomography (CE‐CT) may reflect interstitial fibrosis, we evaluated the ability of equilibrium‐phase CT imaging for predicting pancreatic atrophy. Methods Forty‐six steroid‐treated AIP patients who underwent contrast‐enhanced CT at our university hospital were included in this retrospective study. CT attenuation (Hounsfield units [HU]) values in noncontrast images (NC) and equilibrium‐phase images (EP) and the differences in HU values between NC and EP images (SUB) were measured. Pancreatic volume was measured in CE‐CT before (Volpre) and after (Volpost) steroid therapy. The volume reduction rate was calculated. The relationships of CT values with pancreatic atrophy, Volpost, volume reduction rate, and diabetes exacerbation were investigated. Results CT values in the EP and SUB images before steroid therapy were associated with pancreatic atrophy after steroid therapy (atrophy vs nonatrophy 114.5 ± 12.8 vs 99.5 ± 11.1, P = 0.0002; 70.9 ± 14.72 vs 57.2 ± 13.1, P = 0.003, respectively), but CT values in NC images were not (P = 0.42). CT values in EP and SUB images before steroid therapy were correlated with Volpost (EP images r = −0.70, P = 0.002; SUB images r = −0.68, P = 0.03) and volume reduction rate after steroid therapy (EP images: r = −0.55, P, Pancreatic atrophy is often observed after steroid therapy for AIP and is associated with exacerbation of diabetes. However, it has not been possible to predict pancreatic atrophy before the start of therapy. This time, we have found a method to predict pancreatic atrophy using CT values, and we introduce it here.

Curcumin, when used in a combination regimen in multiple myeloma patients, has comparable progression‐free survival without the adverse effects of steroid‐based combination therapies that is curcumin may be a viable alternative to corticosteroids in combination with an immunomodulatory drug or proteasome inhibitor.

Introduction Tolosa-Hunt syndrome (THS), a condition characterized by painful ophthalmoplegia and accompanied by cranial nerve palsies, remains as a diagnosis of exclusion. Nevertheless, the 3rd Edition of the International Classification of Headache Disorders (ICHD) has refined its diagnostic criteria to require the demonstration of granulomatous inflammation on magnetic resonance imaging or biopsy. We sought to assess the effectiveness of the new criteria in arriving at accurate diagnoses. Methods We extracted all patient charts from our institution's electronic medical record associated with ICD-9 code 378.55 (external ophthalmoplegia). We then completed the retrospective diagnostic workups to determine if subjects met ICHD-3 criteria for THS and compared our findings with their final diagnoses. Results Of 62 patients associated with ICD-9 code 378.55, 10 (16%) was identified to have presenting symptoms concerning THS. The average age at the first onset of THS-like symptoms was 58 years. Five of the 10 (50%) met ICHD-3 criteria for THS. Two of the 5 (40%) meeting ICHD-3 criteria for THS were discovered to have other diagnoses. Two of the 5 (40%) patients not meeting ICHD-3 criteria for THS nevertheless received a final diagnosis of THS. Discussion Our false-negative rate of 40% is consistent with previous case series found in the literature. Our false-positive rate of 40% is, to our knowledge, a new contribution to the literature as no other case series includes more than a single false-positive. The false-positive rate is most concerning, as a preliminary misdiagnosis of THS can delay treatment tailored to the true etiology. Furthermore, infectious etiologies can be exacerbated with steroid treatment. Conclusion Our case series suggests that ICHD-3 criteria are suboptimal for the accurate diagnosis of THS. We recommend a close follow-up for all patients with symptoms concerning THS until a definitive responsible etiology is discovered and we encourage further studies assessing ICHD-3 guidelines to optimize their sensitivity and specificity in the diagnosis of THS.

Hepatic IgA nephropathy is a complication of chronic liver disease. IgA nephropathy after liver transplantation is rare, especially in children, and carries a significant risk factor for chronic renal failure and mortality. In cases without viral hepatitis, steroid therapy may be useful for IgA nephropathy associated with liver dysfunction.

This case report highlights the clinical efficacy of endoscopic transpapillary drainage for gallbladder perforation in a high-risk surgical patient with a history of steroid treatment for interstitial pneumonia. The usefulness of endoscopic transpapillary gallbladder drainage in high-risk surgical patients with acute cholecystitis has not been established. In difficult cases of emergent surgery, such as described here, endoscopic transpapillary drainage is a promising method to manage gallbladder perforation and acute cholecystitis recurrence.

Key Clinical Message Anabolic steroids and androgenic steroids (AAS) can have long‐term effects on the female voice. These changes are clinically relevant since they are difficult to treat and therefore should be disclosed to patients using AAS or receiving androgenic steroid therapy.

Key Clinical Message Organizing pneumonia (OP) and pulmonary alveolar proteinosis (PAP) are rare complications in patients with hematologic disorders. We herein report a case of PAP that developed during steroid treatment for OP in a patient with atypical chronic myeloid leukemia. Physicians should pay close attention to these complications in patients with hematologic malignancies.

Isolated cardiac sarcoidosis is a generally accepted disease condition, and the low yield of endomyocardial biopsy because of patchy involvement is also well known. However, current guidelines still require histologic confirmation of granuloma for the diagnosis of cardiac sarcoidosis, either in myocardial or extra-cardiac tissues. Therefore, only a presumptive diagnosis of chronic multifocal myocarditis of unknown origin can be made in a large number of patients in whom the only considerable diagnosis is cardiac sarcoidosis based on current knowledge. Even if these patients are treated with the same treatment scheme as that for cardiac sarcoidosis, which may not cause harm in the absence of a definite diagnosis, the true spectrum of cardiac sarcoidosis could not be determined for deciding the optimal treatment strategy. In addition, the current recommendations for dose, duration of initial steroid therapy, and treatment in patients who did not respond to initial steroid therapy are not easy to follow in real-world practice. We would like to propose a scoring system for the diagnosis of cardiac sarcoidosis and suggest our adoption or modification of the diverse current recommendations.

We describe the case of a 29‐year‐old female non‐smoker who was treated with steroid therapy for a subacute exacerbation of multisystem Langerhans cell histiocytosis (MS‐LCH) with worsening lung, skin, and oral mucosal lesions. The patient developed pneumonia, and computed tomography (CT) showed multiple thin‐walled cavities. Transbronchial lung cryobiopsy (TBLC) specimens revealed Langerhans cells, which were positive for CD1a and S‐100 expression. Similar histological findings were detected in the submandibular gland, skin, and tooth. On the basis of these findings, the patient was diagnosed with MS‐LCH and subsequently treated with steroid therapy. From the literature review, case reports of non‐smokers with pulmonary lesions that worsened and required treatment are rare. Almost all cases recurred and needed additional treatment. This case study contributes to our understanding of the potential role of steroid therapy in MS‐LCH treatment. Additionally, TBLC is a novel, potentially safer, diagnostic tool that has not been previously described for LCH., Multisystem Langerhans cell histiocytosis in an adult non‐smoker treated with steroid therapy for a subacute exacerbation: a case report and review of the literature.

To describe the long-term effect of steroid treatment on weight in nonambulatory males with Duchenne Muscular Dystrophy (DMD), we identified 392 males age 7–29 years with 4,512 weights collected after ambulation loss (176 steroid-naïve and 216 treated with steroids ≥6 months) from the Muscular Dystrophy Surveillance, Tracking, and Research Network (MD STARnet). Comparisons were made between the weight growth curves for steroid-naïve males with DMD, steroid-treated males with DMD, and the US pediatric male population. Using linear mixed-effects models adjusted for race/ethnicity and birth year, we evaluated the association between weight-for-age and steroid treatment characteristics (age at initiation, dosing interval, cumulative duration, cumulative dose, type). The weight growth curves for steroid-naïve and steroid-treated nonambulatory males with DMD were wider than the US pediatric male growth curves. Mean weight-for-age z scores were lower in both steroid-naïve (mean = −1.3) and steroid-treated (mean = −0.02) nonambulatory males with DMD, compared to the US pediatric male population. Longer treatment duration and greater cumulative dose were significantly associated with lower mean weight-for-age z scores. Providers should consider the effect of steroid treatment on weight when making postambulation treatment decisions for males with DMD.

Key Clinical Message Sjögren's syndrome concurrent with protein‐losing gastroenteropathy can develop into secondary systemic capillary leak syndrome. Thus, it is important to diagnose the condition as soon as possible and simultaneously administer treatment for Sjögren's syndrome, protein‐losing gastroenteropathy, and systemic capillary leak syndrome.

Objective To investigate the role of intratympanic (IT) therapy in the treatment of idiopathic sudden sensorineural hearing loss (ISSNHL). Methods This study was a retrospective review. Patients were treated for ISSNHL from January 1, 2011 to April 12, 2015 with the following: pre/posttreatment audios, treatment initiated ≤90 days and idiopathic etiology. Fifty-three ISSNHL patients were analyzed in the following subgroups: oral steroids (n = 8), combination oral+IT (n = 39), and IT (n = 6). Main outcomes measured were pre/posttreatment pure tone average (PTA) scores. Results The PTA changes for all treatment groups improved by 8.0 ± 19.5 dB (P = .004); for 31 patients treated ≤2 weeks after onset, PTA improved by 13.8 ± 16.6 dB (P

Key Clinical Message We report a case of 37‐year‐old man admitted with acute motor and sensory axonal neuropathy (AMSAN) which was treated with pulse‐steroid therapy after the plasmapheresis and intravascular immunoglobulin. The improvement of the symptoms of AMSAN after pulse‐steroid therapy may represent a therapeutic alternative for this variant of Guillain‐Barré syndrome.

Background The aim of this systematic review was to assess the efficacy of photodynamic therapy (PDT) in the treatment of symptomatic oral lichen planus (OLP). Methods This systematic review aimed to address the following focused question: "Is PDT effective in the treatment of symptomatic OLP?" Indexed databases such as MEDLINE, EMBASE, and CENTRAL were searched up to and including August 2017. Results Six clinical studies were included. The risk of bias was considered high in 5 studies and moderate in one study. Parameters of PDT such as wavelengths, energy fluence, power density and exposure time ranged between 320-660 nm, 120 J/cm2 , 130 mW/cm2 , and 70-150 seconds, respectively. The follow-up period ranged from 4 to 48 weeks. All included studies reporting clinical scores showed that PDT was effective in the treatment of OLP in adult patients at follow-up. However, PDT did not show significant improvement when compared with steroid therapy. Conclusion Photodynamic therapy appears to have some effect in the symptomatic treatment of OLP in adult patients. However, further randomized controlled trials with long follow-up period, standardized PDT parameters, and comparing the efficacy of PDT with steroid therapy are warranted to obtain strong conclusions in this regard.

OBJECTIVES To evaluate the effectiveness of interventional sialendoscopy alone or combined with outpatient intraductal steroid irrigations in patients with sialadenitis due to Sjogren's syndrome (SS). DESIGN A pilot therapeutic study. SETTING ENT Clinics, Universities of Milan and Pavia. STUDY POPULATION We included 22 patients with SS of whom 12 underwent interventional sialendoscopy followed by intraductal steroid irrigations (group A), and 10 interventional sialendoscopy alone (group B). OUTCOMES MEASURES The following outcome measures were considered and recorded before and after the therapeutic intervention: (i) number of episodes of glandular swelling, (ii) cumulative prevalence of patients with glandular swelling assessed by the specific domain, the EULAR SS Disease Activity Index (ESSDAI), (iii) severity of pain by means of a 0-10 pain visual analogue scale (VAS), (iv) severity of xerostomia and other disease symptoms assessed by the EULAR SS Patient Reported Index (ESSPRI) and the Xerostomia Inventory questionnaire. RESULTS The postoperative reduction in the mean number of episodes of glandular swelling was 87% (95% CI: 77-93) and 75% (95% CI: 47%-88%) in the groups A and B, respectively. The percentage of patients with glandular swelling decreased from 41.7% to 0.0% in the group A and from 30.0% to 0.0% in the group B, respectively. Most of the patients experienced a subjective clinical improvement documented by the statistically significant reductions in the postoperative mean pain VAS (group A P

Aim Since Kawasaki disease (KD) was first reported in 1967, its aetiology has been intensively investigated, but remains unclear. We investigated the incidence of recurrent KD in Japan in 2003-2012, compared with previous data from our team, and assessed the potential risk factors for disease recurrence. Methods Data from nationwide KD surveys were retrospectively analysed and 81 310 patients were observed. Incidence rates were compared between different years. Results The incidence rates for recurrent KD were 3.89 per 1000 person-years (January 2003 to December 2012), 4.95 (July 2003 to December 2010), 6.48 (January 2003 to December 2006) and 6.51 (January 2007 to December 2010). These rates were almost consistent with our previous data, which started in the 1980s. The incidence rate for recurrence was statistically higher among males, patients under the age of three years and those who received intravenous immunoglobulin or steroid therapy at the initial episode. The presence of cardiac sequelae during the initial episode did not affect the recurrence incidence. Conclusion Although the incidence of KD has increased, the incidence of recurrent KD has remained largely unchanged over the past 30 years. Risk factors for recurrence included male sex, young age and initial resistance to immunoglobulin therapy.

Aim To investigate the effect of low-dosage steroid therapy in patients with severe aristolochic acid nephropathy (AAN). Methods Forty-three chronic AAN patients in the Peking Union Medical College Hospital and the First Affiliated Hospital of Xinxiang Medical College were included in this study from November 1998 to October 2013. According to the treatment method, the patients were divided into a steroid group (SG, n = 25) and a control group (CG, n = 18). The serum biochemical indicators at the basement in the two groups exhibited no obvious statistical differences. In comparison with the baseline data, the levels of serum creatinine at 3, 6, 9, and 12 months were analyzed. The blood pressure, haemoglobin, serum biochemical indicators, and the side-effects of steroid application were also observed. Urinary macrophage chemoattractant protein-1 (MCP-1) and transforming growth factor-1 (TGF-1) amounts were measured as well. Results (i) The serum creatinine content in the CG group was significantly higher than the baseline level during the follow-up(6, 9, and 12 months later), whereas in the SG group it decreased during the 3-6 month period and remained stable within 1 year. (ii) The biochemical indicators, blood pressure, and haemoglobin persisted stable. (iii) The side-effects of low-dosage steroid therapy were not severe and were tolerated by the AAN patients. (4) Urinary MCP-1 and TGF-1 concentrations were positively correlated with serum creatinine and decreased in the SG group. Conclusion Low-dosage steroid therapy reversed or delayed the renal failure progression in severe chronic AAN patients, which may be associated with the suppression of MCP-1 and TGF-β1 activities.

Introduction: We conducted a study to reveal trends in steroid prescription for Duchenne muscular dystrophy (DMD) patients in Japan. Methods: We asked patients (ages 5–20 years) identified in the patient registry and their clinicians about steroid therapy experiences. Regimen, dose, and starting age were compared among 3 subgroups according to prednisolone initiation year (2000–2004, 2005–2009, and 2010–2013). Results: Among 157 prednisolone users, 4 different regimens were used. Dose frequencies were: every other day (98 patients), daily (44 patients), 10 days on 20 days off (14 patients), and weekly (1 patient). Median starting age was 6 years, and median dose was 0.42 mg/kg/day. There was an increase in daily regimen use from 2005–2009 (n = 9, 16%) to 2010–2013 (n = 33, 36%). Conclusions: This study revealed a transition over time in steroid use from expert opinion to evidence‐based recommendation. Clinical research should be encouraged to optimize medication worldwide. Muscle Nerve 54: 673–680, 2016

Objectives To verify the role of interventional sialendoscopy and steroidal ductal irrigation in patients with recurrent sialadenitis. Design A prospective, cross-sectional pilot study. Setting University of Milan. Participants Fifty-four patients with sine causa recurrent sialadenitis who underwent interventional sialendoscopy (group A, 36 patients) or interventional sialendoscopy followed by a intraductal steroidal irrigations (group B, 18 patients). Main outcomes measures The number of episodes of sialadenitis three and 6 months before and after sialendoscopy, and their severity assessed by means of a 0–10 pain visual analogue scale. Results In the population as a whole, a significant post-treatment reduction in the number of episodes of 30.7 ± 5.5 after 3 months and 34.6 ± 10.2 after 6 months (P < 0.001) and a significant reduction in pain visual analogue scale values of 4.7 ± 0.4 after 6 months (P < 0.001) occurred. There was a statistically significant reduction in both parameters at the same time points in both treatment groups (P ≥ 0.001), with no significant between-group difference in pain visual analogue scale values, an albeit non-significant trend in favour of group B in terms of the number of episodes 3 months after therapy that became significant after 6 months (11.0 ± 9.9 versus 20.5 ± 9.5; P = 0.05). Conclusions Interventional sialendoscopy is effective for the treatment of recurrent sialadenitis; the addition of intraductal steroidal irrigations seems to increase its value in the medium term. Further studies of larger case series with longer follow-up are needed to establish the possibly primary role of steroid therapy in blocking inflammation.

Summary Background Acute severe ulcerative colitis is categorised using the Truelove & Witts criteria. The Travis and the Ho scores are calculated following 72 h of steroid treatment to identify patients at risk of failing steroid therapy who require colectomy or second-line medical therapy. Aim To compare the Travis and the Ho scores in a large unselected cohort to determine which might be more clinically relevant. Methods We analysed 3049 patients with ulcerative colitis from the 2010 round of the UK IBD audit of which 984 had acute severe ulcerative colitis. 420 patients had sufficient data for analysis. Patients were allocated into either a Travis high- or low-risk group and either a Ho high-, intermediate- or low-risk group. We assessed whether further medical or surgical intervention and outcomes varied between groups. Results High-risk patients in Travis and the Ho groups, when compared to lower risk groups, were more likely to fail steroid therapy: 64.5% (131/203) vs. 38.7% (84/217) (P < 0.0001) for Travis and 66.2% (96/145) vs. 46.7% (85/182) vs. 36.6% (34/93) (P < 0.0001) for Ho. They were also more likely to undergo surgery 34.0% (69/203) vs. 9.7% (21/217) for Travis and 33.1% (48/145) vs. 17.0% (31/182) vs. 11.8% (11/93) (P < 0.0001) for Ho. Travis high patients were more likely to be refractory to second-line medical therapy: 44.6% (37/83) vs. 20.0% (9/45) (P = 0.01). Conclusions Patients identified as high risk using the Travis or the Ho scoring systems are more likely to be resistant to IV steroids and require surgery. Risk of surgery in both high-risk populations is lower than previously reported.

Objectives The purpose of this multicentre study was to determine the incidence of oral candidiasis in patients treated with topical steroids for oral lichen planus (OLP) and to determine whether the application of a concurrent antifungal therapy prevented the development of an oral candidiasis in these patients. Materials and Methods Records of 315 patients with OLP seen at four Oral Medicine practices treated for at least 2 weeks with steroids with and without the use of an antifungal regimen were retrospectively reviewed. Results The overall incidence of oral fungal infection in those treated with steroid therapy for OLP was 13.6%. There was no statistically significant difference in the rate of oral candidiasis development in those treated with an antifungal regimen vs those not treated prophylactically (14.3% vs 12.6%) (P = 0.68). Conclusions Despite the use of various regimens, none of the preventive antifungal strategies used in this study resulted in a significant difference in the rate of development of an oral candidiasis in patients with OLP treated with steroids.

This is the peer reviewed version of the following article: Kamada, S., Hanazono, A., Sanpei, Y., Inoue, T., Suzuki, S., Sugawara, M. and Iijima, K. (2019), Alternative to steroid therapy for myasthenia gravis and myositis occurring as immune-related adverse events. Clin Exp Neuroimmunol, 10: 190-191,which has been published in final form at https://doi.org/10.1111/cen3.12526. This article may be used for non-commercial purposes in accordancewith Wiley Terms and Conditions for Use of Self-Archived Versions.

SummaryBackground One of the important and distressing cutaneous side effects of steroid therapy is skin atrophy, which has no definite and effective treatment. To the best of our knowledge, laser therapy for steroid-induced atrophic scars has not been investigated to date. Objective The aim of this study was to evaluate the efficacy and safety of pulsed dye laser in the treatment of steroid-induced atrophic scars. Methods In this pilot study, 15 patients with at least one atrophic patch were treated with the 585-nm pulsed dye laser at 4-week interval sessions until achieving complete improvement or until patient were lost to follow-up. Clinical outcome was assessed via standard photographic method before each treatment session and after the final visit. An independent dermatologist evaluated the photographs. Result All of the patients (13 females and two males) with 25–59 years of age experienced some degree of improvement, except one patient who withdrew from the treatment after three sessions. The treatment was well tolerated. Conclusion The results of our study indicated that pulsed dye laser therapy could be employed as a new method in the treatment of steroid-induced atrophic scars. Pulsed dye laser might affect the lesions through inducing collagen deposition and production of more superficial dermal elastin as well as less unidirectional collagen in clusters.

Aim Drug-induced liver injury (DILI) is becoming a worldwide problem with its still unexplained properties. Methods The data of patients who were diagnosed with DILI between January 2008 and December 2013 were assessed. Results Five patients had been diagnosed with intrinsic and 82 patients with idiosyncratic DILI. The most common causative agents were antimicrobial drugs. The most common injury pattern was hepatocellular. When patients with bilirubin levels of more than 5 mg/dL were divided into two groups according to receiving steroid therapy (n = 11) or not (n = 40), there was not any significant difference according to their clinical results (P > 0.05). Five of the idiosyncratic DILI patients were diagnosed with drug-induced autoimmune hepatitis (DI-AIH). In histopathological examination, hepatic rosette formation and emperipolesis were observed to be more common among patients with DI-AIH when compared with ones without (P

Objectives/Hypothesis To evaluate in patients with idiopathic sensorineural hearing loss whether pulse therapy with methylprednisolone leads to better recovery of hearing than traditional oral prednisolone therapy. Study Design Randomized controlled trial. Methods Sixty-seven patients diagnosed with idiopathic sensorineural hearing loss were randomly divided into two groups based on therapy. The study group received 500-mg daily intravenous methylprednisolone for 3 consecutive days, followed by 1 mg/kg (maximum 60 mg) oral prednisolone for 11 days (total treatment: 14 days). The control group received 1 mg/kg (maximum 60 mg) oral prednisolone for 14 days. Hearing change was evaluated by comparing initial hearing tests and the third-month hearing tests in three ways: 1) pure tone improvement in each individual tone (0.5, 1, 2, 3, and 4 kHz); 2) word-recognition score improvement; and 3) complete, partial, and no recovery of hearing calculated (as defined by American Academy of Otolaryngology—Head and Neck Surgery Clinical Practice Guidelines). Results Sixty of 67 patients, 29 of 34 patients in the study group and 31 of 33 patients in the control group, completed the study. Frequency-specific hearing improvement did not differ significantly among the groups. Word-recognition score improvement was 20.34% ± 27.35% for the study group and 13.41% ± 23.48% for the control group, which had no statistically significant difference. There was also no significant difference in hearing recovery rates for the two groups. Conclusion Pulse therapy with methylprednisolone and traditional oral prednisolone therapy resulted in similar hearing improvement. Level of Evidence 1b. Laryngoscope, 126:150–155, 2016

Objectives/Hypothesis To investigate the long-term outcomes of accompanying tinnitus after steroid therapy for patients with sudden sensorineural hearing loss (SSNHL). Study Design Retrospective chart review and survey. Methods Fifty patients diagnosed with SSNHL accompanied by tinnitus were enrolled and divided into two groups—satisfied and unsatisfied—according to the degree of improvement of tinnitus after SSNHL treatment. Subjective improvement of tinnitus and hearing status were investigated before and 6 months after SSNHL treatment. Hearing improvement was assessed using criteria from our previous study and Siegel's criteria. The change of tinnitus was assessed using a visual analogue scale for tinnitus intensity and frequency. Results Patients with more severe initial hearing loss had less chance of hearing recovery (P = .05). The satisfied group included significantly more cases with better hearing recovery after SSNHL treatment than the unsatisfied group (P = .049). Pure-tone threshold and speech discrimination scores were significantly better in the satisfied group than in the unsatisfied group after SSNHL treatment (P = .033 and P = .018, respectively), although the two groups showed no definitive differences before treatment. Conclusions Optimal and successful treatment of SSNHL may be an important factor in obtaining favorable long-term control of tinnitus accompanied by SSNHL. Level of Evidence 4. Laryngoscope, 125:1433–1437, 2015

Aspergillusspecies,whicharenormalinhabitantsof therespiratory tract, are commonly responsible for fungalspondylodiscitis. These fungi invade the spine when theimmune status of the host is compromised such as in organtransplant patients, patients on chronic steroid treatment orpatientsreceivingchemotherapy

A 76-year-old woman with sarcoidosis who had an implantable pacemaker for complete atrioventricular block was admitted with syncope. Electrocardiogram revealed ventricular pacing failure, and a marked rise in the ventricular pacing threshold. 18F-Fluorodeoxyglucose positron emission tomography (FDG-PET) indicated increased uptake of FDG in the ventricular septum. Three days after steroid therapy, the ventricular pacing threshold reverted to normal, and FDG-PET showed decreased FDG uptake in the ventricular septum. In this case report, we demonstrate that a sudden deterioration in the ventricular pacing threshold due to worsening cardiac sarcoidosis can be reversed with early steroid therapy.

Aim The underlying etiology of dropped head syndrome (DHS) in Parkinson's disease (PD) patients seems to be heterogeneous, that is neck dystonia, a drug-induced etiology or focal myopathy of the neck extensor muscles. Focal myopathy might be primary myositis or a stretched muscle injury. In Japan, several reports have argued that neck dystonia is the cause of DHS. There have been no reports to date that documented a focal myopathy of the neck extensor muscles. The aim of the present study was to investigate myopathic changes in the neck extensor muscles of PD patients with DHS by needle electromyography (EMG), and to evaluate the effect of steroid therapy. Methods We carried out needle EMG on 15 consecutive PD patients with DHS who visited our hospital between July 2009 and November 2011. The effects of prednisolone for the treatment of DHS were evaluated clinically and electrophysiologically. Results Needle EMG of the neck extensor muscles in 15 PD patients with DHS revealed fibrillation potentials, positive sharp waves and small amplitude, short duration motor unit potentials in 14 patients. EMG findings were indicative of focal myopathy in five patients, and were compatible with focal myopathy in nine patients. DHS improved in eight out of 11 patients given prednisolone 20 mg/day. Follow-up needle EMG after steroid therapy in four patients showed improvements in the myopathic changes. Conclusion We report that focal myopathy of the neck extensor muscles is common in Japanese PD patients with DHS. Our findings suggest that steroid therapy might improve DHS in some PD patients.

Aim: Determine the frequency and predictors of sleep disorders in boys with Duchenne Muscular Dystrophy (DMD). Method: Cross-sectional study by postal questionnaire. Sleep disturbances were assessed using the Sleep Disturbance Scale for Children (validated on 1157 healthy children). A total sleep score and six sleep disturbance factors representing the most common sleep disorders were computed. Potential associations between pathological scores and personal, medical and environmental factors were assessed. Results: Sixteen of 63 boys (25.4%) had a pathological total sleep score compared with 3% in the general population. The most prevalent sleep disorders were disorders of initiating and maintaining sleep (DIMS) 29.7%, sleep-related breathing disorders 15.6% and sleep hyperhydrosis 14.3%. On multivariate analysis, pathological total sleep scores were associated with the need to be moved by a carer (OR = 9.4; 95%CI: 2.2–40.7; p = 0.003) and being the child of a single-parent family (OR = 7.2; 95%CI: 1.5–35.1; p = 0.015) and DIMS with the need to be moved by a carer (OR = 18.0; 95%CI: 2.9–110.6; p = 0.002), steroid treatment (OR = 7.7; 95%CI: 1.4–44.0; p = 0.021) and being the child of a single-parent family (OR = 7.0; 95%CI: 1.3–38.4; p = 0.025). Conclusion: Sleep disturbances are frequent in boys with DMD and are strongly associated with immobility. Sleep should be systematically assessed in DMD to implement appropriate interventions.

Background: Inflammatory bowel disease (IBD) is a chronic disease requiring long-term management. General practitioners (GPs) are often the first point of contact for initial symptoms and flares. Thus we assessed GPs' attitudes to and knowledge of IBD. Methods: A state-wide postal survey of GPs was performed collecting demographic details, practice and attitudes in IBD-specific management and knowledge. Results: Of 1800 GPs surveyed in South Australia, 409 responded; 58% were male, 80% Australian trained and 73% practised in metropolitan areas. Most GPs (92%) reported seeing zero to five IBD patients per month. Overall, 37% of the GPs reported being generally ‘uncomfortable’ with IBD management. Specifically, they were only somewhat comfortable in providing/using maintenance therapy, steroid therapy or unspecified therapy for an acute flare. They were uncomfortable with the use of immunomodulators and biologicals (71 and 91% respectively). No GP reported never referring, referring sometimes (12%), often (34%) or always (55%). Most (87%) GPs rated their communication with private specialists positively; while only 32% were satisfied with support from public hospitals. Of concern, most (70%) monitored patients on immunosuppression on a case-by-case basis rather than by protocol. In multivariable analyses, GPs' IBD-specific knowledge did not influence comfort with overall management, nor did knowledge influence GP comfort with any particular therapy. Conclusion: Individual GPs care for few IBD patients and have variable attitudes in their practice. Whether improvement can realistically be achieved given individual GP's paucity of patients is questionable. These data support the provision of better support and specific action plans for IBD patients.

Recently, the progress of endoscopy has made it possible to evaluate bile duct mucosa by peroral cholangioscopy. Herein, we report a case of immunoglobulin G4-related sclerosing cholangitis accompanying autoimmune pancreatitis in a patient who improved with treatment by steroid therapy and endoscopic biliary stenting, and observed the bile duct mucosa by peroral cholangioscopy before and after treatment.

We report a case of bilateral idiopathic segmental ureteritis in a 52-year-old woman. The diagnosis was established by imaging studies (magnetic resonance imaging/computed tomography scan), ureteroscopy and biopsies. The left side improved spontaneously, but the right ureteral stricture persisted after 3 months of ureteral stenting. Administration of prednisolone effectively relieved obstruction on the right side after 4 months. This is the first report of idiopathic segmental ureteritis successfully treated by steroid therapy, without invasive open procedures.

Aim: Phimosis is a common paediatric urological disorder and often necessitates circumcision. We prospectively evaluated local steroid therapy (LST) as the first choice therapy for such children. Methods: Two hundred and sixty symptomatic boys up to 15 years of age (mean 34 months) with phimosis were started on betamethasone dipropionate (0.05%) application on gently stretched prepuce twice a day. Follow-up visits were arranged at the end of weeks 1, 2 and 4 and 6 months. Grade of phimosis was objectively graded. Results: Ninety one percent of the boys showed a successful outcome at the end of 4 weeks; 72% responded in first week, further 16% responded in week 2, and only 2.6% achieved alleviation of phimosis on further application of LST beyond 2 weeks. Fourty two (17.8%) boys had a recurrence of phimosis on a long-term follow-up (mean – 25.4 months, range 6–48 months); thus, the long-term success rate was 77%, while 60 (23%) boys underwent surgery. Conclusion: Local steroid therapy is safe and successful in alleviating symptomatic tight foreskin in a large majority of children. The response can be seen as early as 1 week; most of the children respond by week 2 and continuing therapy further may not be very effective.

Neuromyelitis optica (NMO) is a severe inflammatory demyelinating disease with exacerbations involving recurrent or bilateral optic neuritis and longitudinally extensive transverse myelitis. Pulse steroid therapy is recommended as the initial, acute-phase treatment for NMO. If ineffective, treatment with plasma exchange (PE) should commence. However, no evidence exists to support the effectiveness of PE long after the acute phase. Immunoadsorption therapy (IA) eliminates pathogenic antibodies while sparing other plasma proteins. With IA, side effects of PE resulting from protein substitution can be avoided. However, whether IA is effective for NMO remains unclear. We describe a patient with anti-aquaporin-4-positive myelitis who responded to IA using a tryptophan polyvinyl alcohol gel column that was begun 52 days after disease onset following the acute phase. Even long after the acute phase when symptoms appear to be stable, IA may be effective and should not be excluded as a treatment choice.

Clin. Otolaryngol. 2010, 35, 486–489 Objective: To evaluate the therapeutic efficacy of coenzyme Q10 added to systemic steroid in patients with idiopathic sudden sensorineural hearing loss. Design: A controlled prospective study. Setting: Asan medical center, University of Ulsan College of Medicine. Participants: Between August 2007 and October 2008, the first 60 patients diagnosed with sudden sensorineural hearing loss were managed with systemic steroid treatment for 2 weeks including 5-day hospitalisation. And the second 60 patients were managed with coenzyme Q10 for 2 weeks added to previous management. Main outcome measures: We evaluated auditory function by pure tone audiometry and speech discrimination score. Auditory evaluations were performed before and 3 months after treatment using Siegel’s criteria. The degree of improvement in four-tone average hearing and speech discrimination score was calculated from the results before and after treatment. Results: The total hearing improvement rate after the treatment was 75.0% (90/120 patients) in this study. Although 47 patients (78.3%) of coenzyme Q10 group showed better hearing improvement than 43 patients (71.7%) of control group, there was no significant difference. However, the coenzyme Q10 group showed significantly higher improvement in speech discrimination score. Conclusion: From this study, we suggest that coenzyme Q10 may have beneficial effects in the treatment of sudden sensorineural hearing loss.

Canine chronic hepatitis has also been suggested to be an autoimmune disease and one study suggested that steroid treatment improved outcome (Strombeck et al., 1988). However, it is likely that the condition actually represents a common end-point of any one of a number of immunological, infectious, toxic or metabolic insults (Watson, 2004). Consequently, steroids should be considered on a case-by-case basis and used only where there is biopsy evidence of ongoing inflammation (Fig. 1) and when infectious and metabolic causes of hepatitis have been ruled out.

A 45-year-old man who developed brain radionecrosis in the right frontal and left temporoparietal lobes after receiving whole brain radiotherapy and stereotactic radiosurgery for primary central nervous system lymphoma. Since high dose steroid treatment failed and he declined to undergo surgery, he was referred to hyperbaric oxygen (HBO) therapy. Both clinical and radiological findings improved after HBO therapy. Steroid requirements were also reduced. HBO therapy may have a potential value in treatment of brain radionecrosis. J. Surg. Oncol. 2009;100:732–735. © 2009 Wiley-Liss, Inc.

Both eyes of twenty patients were treated for myopia with refractive keratectomy using an excimer laser (193 nm), and followed for six months. The 40 eyes were divided into Group I (22 eyes) with an attempted refraction change of 5-8 diopters; and Group I (18 eyes) with 9-12 diopters of attempted refraction change. In Group I the corrected visual acuity improved or was unchanged in 16/22 eyes. Two eyes with high preoperative corrected visual acuity had experienced a significant loss in corrected visual acuity after 6 months. In Group II, the corrected visual acuity decreased in 10/18 eyes, in 4 eyes significantly. Among the 40 eyes, 39 had a refraction change less than intended after 6 months. Overcorrection was seen in only 1 eye. In Group I, 20/22 eyes were corrected up to 2.5 diopters less than attempted. In Group II, 9/18 eyes were more than 2.5 diopters from the attempted correction. All eyes developed subepithelial opacification ('haze'), which, in spite of steroid treatment, was still present after 6 months. The haze was more severe in eyes treated with 9-12 diopters of attempted refraction change. The achieved refraction change in the two eyes of the same patient was found to be correlated, possibly due to an individual factor in corneal wound healing.

The efficacy of combined acyclovir and steroid therapy in the treatment of herpetic stromal disease was evaluated by clinical evaluation of disease, the rebound of disease following termination of therapy, and the recovery of virus and viral DNA from corneas in a rabbit model. Therapy with acyclovir alone produced a significant reduction in corneal thickness in 10% of eyes. Addition of steroid to acyclovir therapy decreased the severity of stromal disease as measured by corneal thickness and increased the frequency of response to treatment to 63% of eyes. All eyes receiving acyclovir alone experienced rebound of disease following termination of therapy. Combined therapy increased the severity of rebound of corneal disease. Virus was recovered from cell cultures established after recovery from rebound in 50% of untreated and treated eyes. Viral DNA was detected by PCR in five of the nine corneal cultures which did not produce infectious virus.

A case is reported of hemolytic-uremic syndrome with irreversible renal failure in a young girl who had received a renal transplant 20 months previously. Splenectomy and high-dose steroid therapy were without effect on hemolysis and thrombocytopenia. Removal of the anuric kidney graft was followed by cessation of hemolysis and disappearance of thrombocytopenia. The microscopic lesions in the graft revealed microvascular disease, while histological signs of rejection were absent.