Case Report: Vogt-Koyanagi-Harada Syndrome Mimicking Acute Angle-Closure Glaucoma in a Patient Infected With Human Immunodeficiency Virus

Vogt-Koyanagi-Harada disease (VKH) is a rare multisystemic inflammatory autoimmune disorder. Glaucoma secondary to VKH frequently occurs during the recurrent phase of anterior uveitis; however, acute angle-closure glaucoma (ACG) secondary to both VKH and human immunodeficiency virus (HIV) infection has rarely been reported. We describe a case of secondary acute ACG involving VKH, characterized by sudden vision loss, moderately elevated intraocular pressure (IOP), shallow anterior chamber, and fully or partially closed angle, in an HIV-infected patient. Both VKH and HIV infection contributed to the occurrence of ACG due to the leakage and forward rotation of the ciliary body, as well as choroidal effusion. The deterioration of IOP and serous macular detachment were observed after initial corticosteroid therapy. Visual acuity and IOP were improved with subretinal fluid absorption after continued corticosteroid therapy. Understanding the response of IOP and serous macular detachment after corticosteroid therapy is important for clinical practice.

Efficacy of Corticosteroid Therapy for HTLV-1-Associated Myelopathy: A Randomized Controlled Trial (HAMLET-P)

Corticosteroids are most commonly used to treat HTLV-1-associated myelopathy (HAM); however, their clinical efficacy has not been tested in randomized clinical trials. This randomized controlled trial included 8 and 30 HAM patients with rapidly and slowly progressing walking disabilities, respectively. Rapid progressors were assigned (1:1) to receive or not receive a 3-day course of intravenous methylprednisolone in addition to oral prednisolone therapy. Meanwhile, slow progressors were assigned (1:1) to receive oral prednisolone or placebo. The primary outcomes were a composite of ≥1-grade improvement in the Osame Motor Disability Score or ≥30% improvement in the 10 m walking time (10 mWT) at week 2 for rapid progressors and changes from baseline in 10 mWT at week 24 for slow progressors. In the rapid progressor trial, all four patients with but only one of four without intravenous methylprednisolone achieved the primary outcome (p = 0.14). In the slow progressor trial, the median changes in 10 mWT were −13.8% (95% CI: −20.1–−7.1; p < 0.001) and −6.0% (95% CI: −12.8–1.3; p = 0.10) with prednisolone and placebo, respectively (p for between-group difference = 0.12). Whereas statistical significance was not reached for the primary endpoints, the overall data indicated the benefit of corticosteroid therapy. (Registration number: UMIN000023798, UMIN000024085)

Practice patterns regarding regional corticosteroid treatment in noninfectious Uveitis: a survey study

Background Regional corticosteroid therapy for noninfectious uveitis is well-established but usage patterns have not been studied extensively. This study aims to assess practice patterns of retina and uveitis specialists regarding their preferences on the use of local corticosteroid therapy. Methods A 13-question survey was developed regarding the practice patterns of regional corticosteroid use in specific situations and populations. The survey was distributed to both the American Uveitis Society and Macula Society. Results Responses from 87 ophthalmologists were analyzed. The two most commonly used drugs were the dexamethasone intravitreal implant (Ozurdex®) and posterior sub-tenon’s triamcinolone (also known as posterior sub-Tenon’s Kenalog, or PSTK). Regional corticosteroids were used more frequently as first-line treatment in more than half of posterior uveitis cases when compared to anterior uveitis (39.1–46.0% vs 10.3%, respectively). Respondents were more willing to use regional corticosteroids in more than half of unilateral uveitis cases than in bilateral cases (54.7% vs 18.6%, respectively). A majority of respondents (67.1%) stated that they would avoid using regional corticosteroids in patients under 8 years old. Conclusions Our results demonstrate more frequent regional corticosteroid use in posterior segment uveitis, unilateral cases, and avoidance in younger pediatric patients. Overall, the variability in these responses highlights the need for guidelines regarding regional corticosteroid use.

A relapse of pemphigus vulgaris in pemphigus herpetiformis or a phenotypic “switch”

Sir, Pemphigus herpetiformis (PH) was originally described by Jablonska et al. in 1975. Clinically, PH presents itself as a herpetiform dermatitis with immunopathological characteristics of pemphigus [1,2]. We report an exceptional case of typical pemphigus vulgaris (PV) relapsing after 36 years in PH. A 65-year-old patient, followed for PV for 36 years and treated with corticosteroid therapy with a remission for more than thirty years, consulted for pruriginous lesions evolving for the previous eight months. A dermatological examination revealed urticariform pruriginous ring lesions surmounted by small peripheral vesicles spread throughout the body (Fig. 1), sparing the mucous membranes, and without Nikolsky’s sign. After two non-specific skin biopsies, the histological examination revealed an intraepidermal bubble with acantholytic cells and eosinophilic spongiosis (Figs. 2a and 2b). Direct immunofluorescence confirmed the diagnosis of pemphigus and indirect immunofluorescence was at the upper limit. The diagnosis of a PV relapse in PH was retained and a dapsone-based treatment was initiated at a dose of 150 mg/day and stopped seven days later when met with hemolytic anemia. Oral corticosteroid therapy involving prednisone at a dose of 1 mg/kg/day was initiated but, given the persistence of the pruritus, the decision was to combine methotrexate at a dose of 12.5 mg/week. A good evolution and a decline within eight months were observed. An improved pruritus and the disappearance of the skin lesions were achieved after one month of treatment. PV and PH are two different anatomical and clinical entities of the autoimmune disease pemphigus, with distinct clinical, histopathological, and immunopathological characteristics [1,2]. Our observation documents a complete phenotypic “switch” of pemphigus with a transition from PV to PH both clinically, histologically, and immunologically. Several rare cases of PV switching to superficial pemphigus (SP) (“phenotypic switch”) have, since 1991, been reported, with a higher frequency this direction than otherwise; the transition period varies from six months to twenty years [3]. To the best of our knowledge, no case has been described of a progression from PV to PH. Having observed one firsthand, we are first to describe the case of a complete phenotypic switch from PV to PH. The mechanism of such a transition remains poorly understood and is often observed during a relapse. Some authors suggest that the effect of immunosuppressants on the desmoglein DSG3 more marked than on DSG1 could explain the relapse of PS in PH [3,4]. Future studies on the immunological factors and predictors of PV relapses after the discontinuation of treatment would be useful to better understand the mechanisms of a relapse in pemphigus, with or without a phenotypic transition.

Low-dose corticosteroid therapy for cardiogenic shock in adults (COCCA): study protocol for a randomized controlled trial

Background Cardiogenic shock (CS) is a life-threatening condition characterized by circulatory insufficiency caused by an acute dysfunction of the heart pump. The pathophysiological approach to CS has recently been enriched by the tissue consequences of low flow, including inflammation, endothelial dysfunction, and alteration of the hypothalamic-pituitary-adrenal axis. The aim of the present trial is to evaluate the impact of early low-dose corticosteroid therapy on shock reversal in adults with CS. Method/design This is a multicentered randomized, double-blind, placebo-controlled trial with two parallel arms in adult patients with CS recruited from medical, cardiac, and polyvalent intensive care units (ICU) in France. Patients will be randomly allocated into the treatment or control group (1:1 ratio), and we will recruit 380 patients (190 per group). For the treatment group, hydrocortisone (50 mg intravenous bolus every 6 h) and fludrocortisone (50 μg once a day enterally) will be administered for 7 days or until discharge from the ICU. The primary endpoint is catecholamine-free days at day 7. Secondary endpoints include morbidity and all-cause mortality at 28 and 90 days post-randomization. Pre-defined subgroups analyses are planned, including: postcardiotomy, myocardial infarction, etomidate use, vasopressor use, and adrenal profiles according the short corticotropin stimulation test. Each patient will be followed for 90 days. All analyses will be conducted on an intention-to-treat basis. Discussion This trial will provide valuable evidence about the effectiveness of low dose of corticosteroid therapy for CS. If effective, this therapy might improve outcome and become a therapeutic adjunct for patients with CS. Trial registration ClinicalTrials.gov, NCT03773822. Registered on 12 December 2018

Lobomycosis post long-term corticosteroid therapy: a case report

Introdução: A lobomicose é uma das infecções fúngicas mais raramente relatadas em todo o mundo. A maioria dos casos relatados de lobomicose é endêmica na América Central e do Sul, especialmente em torno da Amazônia. As áreas do corpo afetadas geralmente são as orelhas, extremidades inferiores, extremidades superiores, rosto, tórax e costas. Objetivo: apresentar a evolução da lobomicose após corticoterapia de longa duração. Metodologia: Estudo descritivo do tipo relato de caso, cujos dados foram obtidos no prontuário do paciente. Apresentação do caso: Este artigo relata uma mulher de 23 anos, que sofreu feridas que cobriam quase toda a superfície do corpo sem qualquer febre. Ao exame físico, foram encontradas múltiplas úlceras com maceração das bordas da ferida e múltiplas saliências avermelhadas nas regiões anterior e posterior do tronco. Não há aparecimento de quelóides. O paciente tinha história de pênfigo vulgar e pênfigo vegetante e estava em uso de corticoterapia por longo prazo. O exame microbiológico não revelou crescimento de fungos. O exame histopatológico do tecido com coloração de Hematoxilina-Eosina e Ácido Periódico de Schiff (PAS) confirmou o diagnóstico de lobomicose. Conclusão: Com base nos achados clínicos e laboratoriais, o paciente foi diagnosticado com Lobomicose.

MAGNITUDE OF DIABETES MELLITUS IN PATIENTS OF SUDDEN SENSORINEURAL HEARING LOSS AND ITS EFFECT ON CORTICOSTEROID THERAPY

Objective: To determine the magnitude and effects of diabetes mellitus in patients of sudden sensorineural hearing loss and on the final outcome of therapy. Study Design: Cross sectional study. Place and Duration of Study: ENT unit of Medical Teaching Institution Abbottabad, from Jul 2018 to Jun 2020. Methodology: A total of 84 patients presented who had idiopathic sudden sensorineural hearing loss by using non probability consecutive sampling enrolled in the study. Basic parameters, age, diabetes mellitus and hearing loss were used for data collection. The data was analyzed by using SPSS version 23. Results: Out of total 52 (61.9%) were male and 32 (38.1%) were female, mean age was 45.62 ± 14.12 years from 14-85 years. A large number of patients presented within one week of onset of hearing loss 57 (67.86%). In our patient group 18 patients (21.4%) had diabetes mellitus and 66 patients (78.6%) did not have diabetes mellitus at presentation. Initial Hearing loss at presentation was significantly more in patients who had diabetes mellitus p=0.006 and there was statistically strong association between the final hearing improvement and diabetes mellitus p<0.001. Conclusion: Diabetes mellitus is associated with more severe hearing loss at initial presentation and poorer final outcome in patients with Idiopathic sudden sensorineural hearing loss. As glycemic control does not affect the result so corticosteroid therapy must be given to all patients of sudden sensorineural hearing loss with diabetes mellitus.