Initial Corticosteroid Treatment Research Articles

Common Practice Patterns in the Diagnosis and Management of Acute Retinal Necrosis: A Survey Study of Uveitis Specialists

ABSTRACT Purpose Due to lack of large randomized clinical trials to determine best practices in treating acute retinal necrosis (ARN), there is not a clear consensus amongst ophthalmologists on how to best manage this potentially blinding condition. The aim of this study is to survey common practice patterns and analyze the factors that affect ophthalmologists’ management of ARN. Methods An anonymous survey was distributed to uveitis and retina specialists who are members of the American Uveitis Society (AUS) via email to query practice patterns regarding ARN. The survey included 22 questions with an additional 10 questions based on response. Survey question topics included demographic information, diagnostic testing, antiviral therapy, corticosteroids, and surgical procedures. Results 67 surveys were included for analysis. Most respondents (87%) always or frequently obtain intraocular aqueous fluid for diagnostic PCR testing. The majority of respondents would administer intravitreal antiviral injections to a unilateral immunocompetent ARN patient (67%), but would be even more likely to do so for a bilateral immunosuppressed ARN patient (87%). Respondents tend to treat ARN with systemic rather than local corticosteroids, with the majority (63%) of respondents initiating corticosteroid treatment 48 hours after treatment. Most respondents (79%) never perform a vitrectomy to manage ARN unless the patient has a retinal detachment or tear. The majority (63%) rarely or never perform prophylactic laser barricade, but may consider laser treatment if there is extensive retinal involvement. Conclusion Current practice patterns for diagnosis and management of ARN among AUS members generally align with the suggested practices outlined by the American Academy of Ophthalmology.

A primary amoebic meningoencephalitis case associated with swimming in seawater.

This case report describes a 62-year-old male fisherman who presented with persistent vomiting, headache, and behavior changes. Despite initial antibiotic and corticosteroid treatment, his condition worsened, leading to coma and subsequent death. Macro-genome sequencing of cerebrospinal fluid (CSF) revealed the presence of Naegleria fowleri infection, which had been missed during initial laboratory tests. The patient's exposure history included sea-swimming near Zhoushan Island.

Cost-effectiveness of treatment strategies for populations from strongyloidiasis high-risk areas globally who will initiate corticosteroid treatment in the United States.

The risk of developing strongyloidiasis hyperinfection syndrome appears to be elevated among individuals who initiate corticosteroid treatment. Presumptive treatment or treatment after screening for populations from Strongyloides stercoralis-endemic areas has been suggested before initiating corticosteroids. However, potential clinical and economic impacts of preventative strategies have not been evaluated. Using a decision tree model for a hypothetical cohort of 1000 individuals from S. stercoralis-endemic areas globally initiating corticosteroid treatment, we evaluated clinical and economic impacts of two interventions, 'Screen and Treat' (i.e. screening and ivermectin treatment after a positive test), and 'Presumptively Treat,' compared to current practice (i.e. 'No Intervention'). We evaluated the cost-effectiveness (net cost per death averted) of each strategy using broad ranges of pre-intervention prevalence and hospitalization rates for chronic strongyloidiasis patients initiating corticosteroid treatment. For the baseline parameter estimates, 'Presumptively Treat' was cost-effective (i.e. clinically superior with cost per death averted less than a threshold of $10.6 million per life) compared to 'No Intervention' ($532 000 per death averted) or 'Screen and Treat' ($39 000 per death averted). The two parameters contributing the most uncertainty to the analysis were the hospitalization rate for individuals with chronic strongyloidiasis who initiate corticosteroids (baseline 0.166%) and prevalence of chronic strongyloidiasis (baseline 17.3%) according to a series of one-way sensitivity analyses. For hospitalization rates greater than 0.022%, 'Presumptively Treat' would remain cost-effective. Similarly, 'Presumptively Treat' remained preferred at prevalence rates of 4% or above; 'Screen and Treat' was preferred for prevalence between 2% and 4%, and 'No Intervention' was preferred for prevalence less than 2%. The findings support decision-making for interventions for populations from S. stercoralis endemic areas before initiating corticosteroid treatment. Although some input parameters are highly uncertain and prevalence varies across endemic countries, 'Presumptively Treat' would likely be preferred across a range for many populations given plausible parameters.

Drug reaction with eosinophilia and systemic symptoms (DRESS): an unusual manifestation of multi-visceral abnormalities and long-term outcome.

Drug reaction with eosinophilia and systemic symptoms (DRESS), also known as drug induced hypersensitivity (DiHS) is a rare, however a severe hypersensitivity reaction with a mortality rate of up to 10%, accounting for 10 to 20% of all cutaneous drug reactions in hospitalized patients. The clinical features of DRESS/DiHS may be challenging to recognize and diagnose, since they are delayed, stepwise, and heterogeneous. The classic presentation of DRRSS/DiHS involves a combination of cutaneous, hematologic, and internal organ involvement with a 2 to 8 weeks latency between drug exposure and the onset of symptoms. Finding the culprit drug in our case was difficult as the patient was taking multiple antibiotics. Drugs such as vancomycin and cefepime used before the rash outbreak for post-reconstructive surgery for left toal knee arthroplasty (TKA) approximately four weeks before the onset of the rash are likely offending agents. This patient also had multi-visceral involvement with eosinophilia and systemic symptoms. The current treatment guidelines for DRESS/DiHS are primarily based on expert opinion, as no randomized control trials exist. After the prompt withdrawal of the offending drug, systemic corticosteroids seem to have shown the best outcome for patients. Delaying discontinuing offending medications and initiating corticosteroid treatment may lead to poor results. The present case emphasizes that the close observation of patients with drug eruption induced by antibiotics is imperative. Primary care team should be able to promptly diagnose patients with DRESS syndrome, detect causative drug, and play a crucial role in the timely evaluation and treatment to reduce mortality rate. The later phase disease relapse or autoimmune complications may occur up to 5 years following the initial presentation. Therefore, we advised the patient to have an outpatient follow up for appropriate testing, including but not limited to genetic susceptibility due to the high risk of relapse and emerging risk of autoimmune diseases.

Pitfalls of Early Systemic Corticosteroids Home Therapy in Older Patients with COVID-19 Pneumonia

Corticosteroids have been widely used for acute respiratory distress syndrome (ARDS), but their role in the early phase of SARS-CoV-2 infection is controversial. Our study aimed to determine the effectiveness of early corticosteroid therapy (ECT) in preventing the progression of disease, reducing the escalation of care and improving clinical outcome in older patients hospitalized for COVID-19 pneumonia. A total of 90 subjects (47.7% women; mean age = 82.3 ± 6.7 years) were enrolled. ECT was administered to 33 out of 90 patients before the hospitalization. At admission, no difference was detected in median SOFA score (2, IQR:2 vs. 2, IQR: 2). We found a significant difference in mean PaO2/FiO2 ratio during the first week of hospitalization between ECT patients and controls (F = 5.49, p = 0.002) and in mean PaO2/FiO2 ratio over time (F = 6.94, p < 0.0001). We detected no-significant differences in terms of in-hospital mortality and transfer to ICU between ECT patients and controls (27.1% vs. 22.8%, respectively, p = 0.63). ECT was associated with worse clinical outcomes, showing no benefit in attenuating the progression of the disease or reducing the escalation of care. These findings are crucial given the current pandemic, and further studies are needed to provide additional data on the optimal timing of initiating corticosteroid treatment.

High-dose methylprednisolone versus dexamethasone therapy for hospitalized patients with severe COVID-19: a retrospective analysis

This study aimed to evaluate the clinical effects of high-dose methylprednisolone therapy in hospitalized patients with severe coronavirus disease (COVID-19) who required oxygen therapy, but not noninvasive/invasive mechanical ventilation or extracorporeal membrane oxygenation therapy. This retrospective observational study that was conducted from April 2021 to October 2021 at a secondary hospital in Japan enrolled patients who were administered 6 mg/day dexamethasone as an initial corticosteroid treatment on admission (dexamethasone group) and those who were administered ≥ 250 mg/day methylprednisolone (methylprednisolone group). Of the 42 participants, 40.5%(17/42) were included in the methylprednisolone group. The incidence of transfer to a tertiary hospital did not differ significantly between the methylprednisolone and dexamethasone groups (5.9% vs. 20%, p = 0.37), and in-hospital mortality was similar in both the groups (0% vs. 4%, p = 1.00). Participants in the methylprednisolone group had a significantly longer duration of oxygen therapy than the dexamethasone group (median (interquartile range) 8.5 (5.5–11.2) days vs. 4 (2.0–7.5) days, p &lt; 0.05). Compared to dexamethasone, high-dose methylprednisolone therapy did not provide any added benefits for patients with severe COVID-19 who did not require respiratory mechanical support.

Case Series of Steroid-Resistant Immune Checkpoint Inhibitor Associated Myocarditis: A Comparative Analysis of Corticosteroid and Tofacitinib Treatment.

Background: Immune checkpoint inhibitor (ICI)–associated myocarditis is an uncommon and potentially fatal immune-related adverse event (irAE). Although corticosteroids are recommended as the first-line treatment by current guidelines, patients still have variable responses to it, and the guidelines vary significantly in terms of treatment strategies. Objectives: In this study, we performed a retrospective analysis of ICI-associated myocarditis in our hospital to propose a new comparative analysis to aid individualized treatment. Methods: We reviewed detailed records of 24 patients with confirmed ICI-associated myocarditis in our hospital from July 1, 2019, to April 1, 2021. Although all the cases in our study received recommended initial corticosteroid treatment according to the guidelines, different responses to corticosteroid were observed during the process of subsequent corticosteroid tapering. Basing on troponin cardiac troponin T rebound during corticosteroid tapering, we propose a new classification analysis of ICI-associated myocarditis that included two subgroups: corticosteroid-sensitive (n = 8) and corticosteroid-resistant group (n = 16). Results: Compared with corticosteroid-sensitive patients, larger doses of corticosteroid, longer period of treatment, and higher mortality rate were found in corticosteroid-resistant patients. Corticosteroid-resistant patients were characterized by more prominent ptosis, muscle weakness, elevated cardiac biomarkers, creatine kinase, and hepatic enzymes levels than that in the corticosteroid-sensitive patients. Tofacitinib (5 mg twice a day) was used in 11 corticosteroid-resistant patients, with seven patients recovered from ICI-associated myocarditis, showing a promising therapeutic effect. Conclusion: Our group analysis of corticosteroid responsiveness in patients with ICI-associated myocarditis may help clinicians to apply individualized treatment in this high-risk cohort. In addition, tofacitinib could provide clinical benefits when used early in the corticosteroid-resistant patients and may provide a new option for the treatment of ICI-associated myocarditis.

Diagnostics and Therapy of Idiopathic Facial Palsy (Bell's Palsy)

The purpose of this review is to report the knowledge for otolaryngologists on standard of care, latest advances, interesting new findings and controversies about the treatment of Bell's palsy. This review is focusing on the acute phase of the disease. The chronic phase, with incomplete, incorrect or no recovery of the palsy, is described briefly. Treatment with prednisolone alone within 72 hours after onset still is the cornerstone of the treatment. The role of antivirals still is unclear. Since 2009 no new and breakthrough clinical trials with influence on the treatment standards have been performed. A study to clarify the role of prednisolone treatment in children is ongoing. Patient-related outcome measures like the Facial Clinimetric Evaluation Scale and the Facial Disability Index are important tools to assess the subjective severity of the disease and psychosocial impact of Bell's palsy next to the motor deficits. Simplified subjective electronic grading systems like the eFACE and first automated image analysis systems have been introduced. Studies clarifying the role of antivirals for severe cases are urgently needed as well as studies on the role of salvage second line therapy after insufficient response to initial corticosteroid treatment. An international consensus on the outcome measures in diagnostics and follow-up is also needed.

Treatment of class II lupus nephritis with combination therapy of mycophenolic acid and corticosteroid; a case report

Lupus nephritis (LN) is one of the most serious manifestations of systemic lupus erythematosus (SLE). Despite available guidelines recommendations on appropriate therapeutic agents, up to one-third of LN patients still do not meet expected response to initial corticosteroid or immunosuppressive treatment. We report a 17-year-old Indonesian female who was diagnosed LN with persistent proteinuria manifestations. Renal biopsy was suggestive of class II LN. Corticosteroid was given for a month without therapeutic response, and the patient was given combination of moderate dose methylprednisolone and mycophenolic acid resulted in complete remission after nine months therapy. Despite the existing guidelines, choices of LN treatment might be individual depends on disease severity (clinical, laboratory and histopathological findings) and demographic factors. The combination of mycophenolic acid and corticosteroid might be better option than high dose corticosteroid to treat class II LN for minimizing the adverse event of corticosteroid.

Timing of corticosteroids impacts mortality in hospitalized COVID-19 patients.

The optimal timing of initiating corticosteroid treatment in hospitalized patients is unknown. We aimed to assess the relationship between timing of initial corticosteroid treatment and in-hospital mortality in COVID-19 patients. In this observational study through medical record analysis, we quantified the mortality benefit of corticosteroids in two equally matched groups of hospitalized COVID-19 patients. We subsequently evaluated the timing of initiating corticosteroids and its effect on mortality in all patients receiving corticosteroids. Demographic, clinical, and laboratory variables were collected and employed for multivariable regression analyses. 1461 hospitalized patients with confirmed COVID-19 were analyzed. Of these, 760 were also matched into two equal groups based on having received corticosteroid therapy. Patients receiving corticosteroids had a lower risk of death than those who did not (HR 0.67, 95% CI 0.67–0.90; p = 0.01). Timing of corticosteroids was assessed for all 615 patients receiving corticosteroids during admission. Patients receiving first dose of corticosteroids > 72 h into hospitalization had a lower risk of death compared to patients with first dose at earlier time intervals (HR 0.56, 95% CI 0.38–0.82; p = 0.003). There was a mortality benefit in patients with > 7 days of symptom onset to initiation of corticosteroids (HR 0.56, 95% CI 0.33–0.95; p = 0.03). In patients receiving oxygen therapy, corticosteroids reduced risk of death in mechanically ventilated patients (HR 0.38, 95% CI 0.24–0.60; p < 0.001) but not in patients on high-flow or other oxygen therapy (HR 0.46, 95% CI 0.20–1.07; p = 0.07) and (HR 0.84, 95% CI 0.35–2.00; p = 0.69), respectively. Timing of corticosteroids initiation was related to in-hospital mortality for COVID-19 patients. Time from symptom onset > 7 days should trigger initiation of corticosteroids. In the absence of invasive mechanical ventilation, corticosteroids should be initiated if the patient remains hospitalized at 72 h. Hypoxia requiring supplemental oxygen therapy should not be a trigger for initiation of corticosteroids unless the timing is appropriate.

Efficacy of Intratympanic Corticosteroid as a Salvage Treatment in Idiopathic Sudden Sensorineural Hearing Loss

IntroductionIdiopathic sudden sensorineural hearing loss (ISSHL) is defined as an abrupt hearing loss of at least 30dB of unknown cause. The hearing response obtained after intratympanic steroid injection as a salvage treatment after a prior failure of initial systemic steroid treatment was analysed. Material and methodAn observational study was performed on 125 cases of ISSHL who were diagnosed from 2006 to 2014. Sixteen achieved complete recovery after one week according to Siegel's criteria. The remaining 109 cases were analysed in two groups: one that received intratympanic corticosteroid salvage therapy (treatment group) and one that did not (control group). The recovery was analysed after 6 months and 2 years of follow-up. ResultsThe difference between each group at baseline were not statistically significant. After systemic treatment for 7 days, PTA in the control group was 53.13dB and 66.11dB in the treatment group (P<.01). After 6 months, the mean PTA improvement was 10.84dB in the treatment group, and 1.13dB in the control group, a significant difference (P<.0001). Only 10 cases achieved full hearing recovery after intratympanic corticosteroid salvage therapy, none of the patients did so in the control group. ConclusionIntratympanic corticosteroid rescue for ISSHL achieved hearing improvement for the cases with failure of initial systemic corticosteroid treatment. However, this treatment did not provide complete hearing recovery according to Siegel's criteria in most cases.

Eficacia del corticoide intratimpánico como tratamiento de rescate en sordera súbita neurosensorial idiopática

IntroductionIdiopathic sudden sensorineural hearing loss (ISSHL) is defined as an abrupt hearing loss of at least 30dB of unknown cause. The hearing response obtained after intratympanic steroid injection as a salvage treatment after a prior failure of initial systemic steroid treatment was analysed. Material and methodAn observational study was performed on 125 cases of ISSHL who were diagnosed from 2006 to 2014. Sixteen achieved complete recovery after one week according to Siegel's criteria. The remaining 109 cases were analysed in two groups: one that received intratympanic corticosteroid salvage therapy (treatment group) and one that did not (control group). The recovery was analysed after 6 months and 2 years of follow-up. ResultsThe difference between each group at baseline were not statistically significant. After systemic treatment for 7 days, PTA in the control group was 53.13dB and 66.11dB in the treatment group (P<.01). After 6 months, the mean PTA improvement was 10.84dB in the treatment group, and 1.13dB in the control group, a significant difference (P<.0001). Only 10 cases achieved full hearing recovery after intratympanic corticosteroid salvage therapy, none of the patients did so in the control group. ConclusionIntratympanic corticosteroid rescue for ISSHL acheived hearing improvement for the cases with failure of initial systemic corticosteroid treatment. However, this treatment did not provide complete hearing recovery according to Siegel's criteria in most cases.

Inaugural tumor-like multiple sclerosis: clinical presentation and medium-term outcome in 87 patients

Tumefactive demyelinating lesions of the central nervous system can be the initial presentation in various pathological entities [multiple sclerosis (the most common), Balo's concentric sclerosis, Schilder's disease and acute disseminated encephalomyelitis] with overlapping clinical presentation. The aim of our study was to better characterize these patients. Eighty-seven patients (62 women and 25 men) from different MS centers in France were studied retrospectively. Inclusion criteria were (1) a first clinical event (2) MRI showing one or more large demyelinating lesions (20mm or more in diameter) with mass-like features. Patients with a previous demyelinating event (i.e. confirmed multiple sclerosis) were excluded. Mean age at onset was 26years. The most common initial symptoms (67% of the patients) were hemiparesis or hemiplegia. Aphasia, headache and cognitive disturbances (i.e. atypical symptoms for demyelinating diseases) were observed in 15, 18 and 15% of patients, respectively. The mean largest diameter of the tumefactive lesions was 26.9mm, with gadolinium enhancement in 66 patients (81%). Twenty-one patients (24%) had a single tumefactive lesion. During follow-up (median time 5.7years) 4 patients died, 70 patients improved or remained stable and 12 worsened. 86% of patients received initial corticosteroid treatment, and 73% received disease-modifying therapy subsequently. EDSS at the end of the follow-up was 2.4 ± 2.6 (mean ± SD). This study provides further evidence that the clinical course of MS presenting with large focal tumor-like lesions does not differ from that of classical relapsing-remitting MS, once the noisy first relapsing occurred.

Clinical features and visual outcomes of 111 patients with new-onset acute Vogt-Koyanagi-Harada disease treated with pulse intravenous corticosteroids

PurposeTo describe the clinical features, treatment and visual outcomes of Japanese patients with new-onset acute Vogt-Koyanagi-Harada (VKH) disease.MethodsClinical records of 111 patients who presented between 1999 and 2015 to the...

The Clinical Course of Minimal Change Nephrotic Syndrome With Onset in Adulthood or Late Adolescence: A Case Series

Few studies have examined the treatment and outcome of adult-onset minimal change nephrotic syndrome (MCNS). We retrospectively studied 125 patients who had MCNS with onset in either adulthood or late adolescence. Presenting characteristics, duration of initial treatment and response to treatment, relapse patterns, complications, and long-term outcome were studied. Case series. Patients with new-onset nephrotic syndrome 16 years or older and a histologic diagnosis of MCNS in 1985 to 2011 were identified from pathology records of 10 participating centers. Partial and complete remission, treatment resistance, relapse, complications, renal survival. Corticosteroids were given as initial treatment in 105 (84%) patients. After 16 weeks of corticosteroid treatment, 92 (88%) of these patients had reached remission. Median time to remission was 4 (IQR, 2-7) weeks. 7 (6%) patients initially received cyclophosphamide with or without corticosteroids, and all attained remission after a median of 4 (IQR, 3-11) weeks. 13 (10%) patients reached remission without immunosuppressive treatment. One or more relapses were observed in 57 (54%) patients who received initial corticosteroid treatment. Second-line cyclophosphamide resulted in stable remission in 57% of patients with relapsing MCNS. Acute kidney injury was observed in 50 (40%) patients. Recovery of kidney function occurred almost without exception. Arterial or venous thrombosis occurred in 11 (9%) patients. At the last follow-up, 113 (90%) patients were in remission and had preserved kidney function. 3 patients with steroid-resistant MCNS progressed to end-stage renal disease, which was associated with focal segmental glomerulosclerosis lesions on repeat biopsy. Retrospective design, variable treatment protocols. The large majority of patients who had MCNS with onset in adulthood or late adolescence were treated with corticosteroids and reached remission, but many had relapses. Cyclophosphamide resulted in stable remission in many patients with relapses. Significant morbidity was observed due to acute kidney injury and other complications. Progression to end-stage renal disease occurred in a few patients and was explained by focal segmental glomerulosclerosis.

Corticosteroid therapy in ulcerative colitis: Clinical response and predictors.

To evaluate clinical response to initial corticosteroid (CS) treatment in Chinese ulcerative colitis patients (UC) and identify predictors of clinical response. Four hundred and twenty-three UC patients who were initially treated with oral or intravenous CS from 2007 to 2011 were retrospectively reviewed at eight inflammatory bowel disease centers in China, and 101 consecutive cases with one-year follow-up were analyzed further for clinical response and predictors. Short-term outcomes within one month were classified as primary response and primary non-response. Long-term outcomes within one year were classified as prolonged CS response, CS dependence and secondary non-response. CS refractoriness included primary and secondary non-response. Multivariate analyses were performed to identify predictors associated with clinical response. Within one month, 95.0% and 5.0% of the cases were classified into primary response and non-response, respectively. Within one year, 41.6% of cases were assessed as prolonged CS response, while 49.5% as CS dependence and 4.0% as secondary non-response. The rate of CS refractoriness was 8.9%, while the cumulative rate of surgery was 6.9% within one year. After multivariate analysis of all the variables, tenesmus was found to be a negative predictor of CS dependence (OR = 0.336; 95%CI: 0.147-0.768; P = 0.013) and weight loss as a predictor of CS refractoriness (OR = 5.662; 95%CI: 1.111-28.857; P = 0.040). After one-month treatment, sustained high Sutherland score (≥ 6) also predicted CS dependence (OR = 2.347; 95%CI: 0.935-5.890; P = 0.014). Tenesmus was a negative predictor of CS dependence, while weight loss and sustained high Sutherland score were strongly associated with poor CS response.

Treatment of MuSK-Associated Myasthenia Gravis

Approximately 5-8% of myasthenia gravis (MG) patients test positive for antibodies against muscle- specific tyrosine kinase (MuSK) receptors. Except in extremely rare reports, all are acetylcholine receptor (AChR) antibody-negative. While MuSK myasthenia gravis (MMG) patients have distinct clinical phenotypes and may differ from AChR-positive patients in diagnostic testing and response to treatment, goals for the treatment of MMG are similar to those in non-MMG. Priority of treatment should be directed toward reducing weakness as much and as quickly as possible. This is particularly true in patients with bulbar or respiratory weakness in order to avoid progression to respiratory failure. After this initial phase, medications should be slowly tapered to the minimum effective dose. Considering the natural history of MMG, a small proportion of patients can be completely taken off treatment at some point, but the vast majority will require treatment for life. Response to acetylcholinesterase inhibitors (ACEi) is usually poor, and the likelihood of side effects is relatively high. However, considering the benign nature of this line of treatment and the potential for rapid response, an initial trial of ACEi is reasonable. Unless clearly contraindicated by other medical conditions, we recommend initiating corticosteroid treatment for all MMG patients, starting at a dose of 1.5-2mg /kg/ day of prednisone, followed by gradual and slow taper to the minimum effective dose. A steroid-sparing agent such as azathioprine - and, less often, mycophenolate mofetil or cyclosporine - may be added. When prednisone is used in combination with another immunosuppressive agent, reducing and then tapering off prednisone may be tried after maximum improvement is achieved. It should be emphasized that response to immunosuppressive medications can be delayed for months, although most patients eventually show marked and sustained response. Cyclophosphamide may be used sparingly in select patients who do not respond to the above medications. Rituximab has shown promising results in MMG, and should be considered in severe and refractory cases or in situations where other options are contraindicated or not tolerated by patients. Acute exacerbations may be treated by plasma exchange, which most reports indicate is superior to IVIg, although IVIg may still be used. To date, there is no convincing evidence for the role of thymectomy in MMG.

Pemphigus vulgaris in a Welsh pony stallion: case report and demonstration of antidesmoglein autoantibodies

To describe the clinical, histological and immunological findings of an equine case of pemphigus vulgaris, including the demonstration of antidesmoglein (anti-Dsg) autoantibodies. The diagnosis of pemphigus vulgaris was confirmed in a 9-year-old Welsh pony stallion with both direct and indirect immunofluorescence and immunoprecipitation studies, the latter identifying circulating anti-Dsg3 IgG. Treatment with immunosuppressive medications was initiated. Lesions were seen in the perineal area, sheath, mane, tail, eyelids, coronary bands and mucosa of the mouth and oesophagus. Initial corticosteroid treatment improved the clinical signs, but the onset of laminitis necessitated a reduction in dosage, which was associated with a recurrence of lesions and development of oral ulcers. A corneal ulcer developed after 60days of treatment. Despite treatment with azathioprine, gold salts and dapsone, the disease progressed and the pony was euthanized. Postmortem examination showed additional lesions of the cardia of the stomach. Pemphigus vulgaris is rarely diagnosed in equids. We describe a case that was substantiated by the demonstration of anti-Dsg3 IgG. Response to treatment was poor, with the best response to high doses of prednisolone. Equine pemphigus vulgaris is likely to carry a poor prognosis and if there is no response to treatment, humane euthanasia is warranted.

Late but effective long-term response to splenectomy in the treatment of immune thrombocytopenia

Late but effective long-term response to splenectomy in the treatment of immune thrombocytopenia

Effects of early corticosteroid treatment on magnetic resonance imaging and ultrasonography findings in giant cell arteritis

To compare the impact of initial corticosteroid treatment on high-resolution MRI and colour-coded duplex sonography (CCDS) findings in patients with GCA (temporal). Sensitivity and specificity of CCDS and high-resolution contrast-enhanced MRI studies of 59 patients with suspected GCA were retrospectively analysed. Patients were grouped according to the duration of steroid treatment before imaging: 0-1 day, 2-4 days and >4 days. In 41 patients, imaging results were compared with findings of temporal artery biopsy (TAB). Sixty-one per cent (36/59) of patients were diagnosed with GCA. TAB findings were positive in 59% (24/41). The compared results of TAB sensitivity of CCDS and MRI under steroid treatment of 0-1 day were 92% and 90%, 2-4 days 80% and 78% and >4 days 50% and 80%, respectively. The compared results of the final clinical diagnosis sensitivity of CCDS and MRI under steroid treatment of 0-1 day was 88% and 85%, 2-4 days 50% and 64% and >4 days 50% and 56%, respectively. Sensitivity of a first-time CCDS or an MRI for detection of GCA rapidly decreases under corticosteroid treatment. Therefore imaging of patients with suspected GCA should be performed as soon as possible, preferably within the first days of treatment.