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)

Outcomes of Patients With Acute Vogt–koyanagi–harada Disease Treated With Intravenous Corticosteroid Pulse Followed by the Slow Tapering of Oral Corticosteroid Therapy

Purpose We investigated the treatment outcomes of patients with acute Vogt–Koyanagi–Harada (VKH) disease and assessed the differences between patients with no inflammation worsening and those with persistent or worsening inflammation. Potential factors responsible for eyes with low visual outcomes were also investigated.Methods We retrospectively reviewed the clinical records of patients with acute VKH disease who first visited us between 2009 and 2018 and were followed up for >300 days. Clinical characteristics, treatments, and posttreatment conditions were assessed. Patients were classified into no inflammation worsening (acute–resolved [AR]) and inflammation worsening (chronic–recurrent [CR]) groups based on conditions after 6 months from disease onset.Results This study assessed 62 eyes from 31 patients (mean age: 52.8 years). One patient was treated with topical treatment alone and showed poor visual outcomes. In total, 30 patients were treated with methylprednisolone pulse followed by the slow tapering of oral prednisolone; 73% of them developed AR and 27% CR. Although the total prednisolone dose was higher in patients with CR disease, no significant difference was noted in the final best-corrected visual acuity (BCVA). Among the patients receiving systemic steroid, five eyes had a final BCVA of ≤0.5 due to anisometropic amblyopia, diabetic maculopathy, pre-existing macular hole, epiretinal membrane, and ellipsoid zone loss. Conclusions Patients with acute VKH disease treated with corticosteroid pulse followed by the slow tapering of prednisolone appear to demonstrate good visual outcomes, including patients with CR; most eyes with low visual outcomes have pre-existing conditions that explain low vision.

RENAL DISEASE IN SYSTEMIC LUPUS ERYTHEMATOUS: ALL THAT GLITTERS IS NOT GOLD

Patients with systemic lupus erythematosus may occasionally develop renal pathology from causes unrelated to the underlying disease. We describe one such case. This 22-year-old lady, with past history of arthritis, presented with dyspnea and fever. She had clinical signs consistent with systemic lupus erythematosus. Echocardiogram revealed valvular regurgitation. Antibiotics were started for probable infective endocarditis. She developed acute kidney injury. This was initially attributed to lupus nephritis, but subsequently renal histopathology revealed acute interstitial nephritis. Renal functions normalized with oral prednisolone. This case highlights the fact that a broader differential diagnosis must always be considered even if the diagnosis is clear.

Pulse Methylprednisolone with Oral Prednisolone versus Adrenocorticotropic Hormone in Children with West Syndrome: a Randomized Controlled Trial

Background and Purpose: West syndrome is an epileptic encephalopathy of infancy. According to guidelines, adrenocorticotrophic hormone (ACTH) is probably effective for the short-term management of infantile spasm, but there is little uniformity in treatment due to variable response. This study has been done to evaluate the efficacy of pulse methylprednisolone as compared to ACTH in children with West syndrome.Methods: Children between 3 months to 24 months with the diagnosis of West syndrome were included and ACTH and pulse methyl prednisolone followed by oral prednisolone were given after randomization. Total duration of treatment was 6 weeks in both groups.Results: Total 87 children were enrolled; 12 patients lost in follow up. Finally, 43 received ACTH and 32 received pulse methylprednisolone. In pulse methylprednisolone group, 28.13% showed 50-80% response, 28.13% showed 80-99% response and 21.87% patients showed 100% response. In ACTH group, 41.86% showed 50-80% response, 25.58% showed 80-99% response and only 3 (6.97%) patients showed 100% response. Methylprednisolone treatment regimen did not cause significant or persistent adverse effects.Conclusions: Pulse methylprednisolone followed by oral prednisolone for 6 weeks is as effective as ACTH. Thus, methylprednisolone therapy can be an important alternative to ACTH.

Lower Cranial Nerve Palsy due to Prevertebral Tuberculosis Infection

Prevertebral tuberculosis is a rare infectious disease that often affects immunocompromised patients in developing countries. We present the case of a patient who complained of headache, dysarthria, and dysphagia. Neurological examination revealed multiple cranial nerve palsies, including the hypoglossal, glossopharyngeal, and vagus nerves. Brain magnetic resonance imaging demonstrated an infiltrative lesion in the prevertebral space, and the biopsy revealed chronic inflammation. On suspicion of immune-mediated inflammation, the patient was treated with intravenous dexamethasone and oral prednisolone, with minimal response. Eleven months after the initial diagnosis, the patient’s neurological symptoms were aggravated, and we detected newly developed pulmonary tuberculosis. After the treatment of pulmonary tuberculosis, his neurological symptoms improved, and the imaging study demonstrated improvements. Although we lacked positive laboratory or biopsy results for tuberculosis, we suspect that the lesions were distant tuberculosis infections. Tuberculosis should be considered in patients with unknown infiltrative mass-like lesions in the prevertebral spaces.

De Novo Minimal Change Disease following Vaccination with the Pfizer/BioNTech SARS-CoV-2 Vaccine in a Living Kidney Donor

Coronavirus disease 2019 has developed as a pandemic. Immunization with the introduction of vaccines against COVID-19 seems be the only way to end this pandemic. We report on a case of a kidney donor, who developed minimal change disease (MCD) within 4 days post-vaccination with the SARS-CoV-2 BNT162b2 mRNA vaccine (Pfizer/BioNTech). She donated her kidney to her husband 4 years ago. After receiving the 1st vaccine dose, she presented with nephrotic syndrome, with complete remission 5 days later. She proceeded with the second dose of the BNT162b2 vaccine at the appointed time. Two days later, she presented with a relapse of full-blown nephrotic syndrome with preserved renal function. We performed an ultrasound-guided percutaneous kidney biopsy and the final diagnosis was consistent with minimal change disease. Oral prednisolone was promptly initiated at a dosage of 1 mg/kg daily and complete remission was achieved 10 days later. More data about this rare appearance of de novo glomerular diseases after SARS-CoV-2 vaccination are emerging and should be interpreted rigorously.

Safety and Effectiveness of Oral Methylprednisolone Therapy in Comparison With Intramuscular Adrenocorticotropic Hormone and Oral Prednisolone in Children With Infantile Spasms

Background and Purpose: To assess the safety and effectiveness of oral methylprednisolone (oMP) in comparison with intramuscular adrenocorticotropic hormone (imACTH) and oral prednisolone (oP) therapies in children with infantile spasms (IS).Methods: In this prospective, open-label, non-blinded, uncontrolled observational study, children (aged 2–24 months) with newly diagnosed IS presenting with hypsarrhythmia or its variants on electroencephalogram (EEG) were included. It was followed by imACTH, oP, or oMP (32–48 mg/day for 2 weeks followed by tapering) treatments. Electroclinical remission/spasm control, relapse, and adverse effects were evaluated in the short-term (days 14 and 42) and intermediary-term (3, 6, and 12 months) intervals.Results: A total of 320 pediatric patients were enrolled: 108, 107, and 105 in the imACTH, oMP, and oP groups, respectively. The proportion of children achieving electroclinical remission on days 14 and 42 was similar among the three groups (day 14: 53.70 vs. 60.75 vs. 51.43%, p = 0.362; day 42: 57.55 vs. 63.46 vs. 55.34%, p = 0.470). The time to response was significantly faster in the oMP group (6.5 [3.00, 10.00] days vs. 8.00 [5.00, 11.00] days for imACTH and 8.00 [5.00, 13.00] days for oP, p = 0.025). Spasm control at 3, 6, and 12 months was also similar in the three groups (P = 0.775, 0.667, and 0.779). The relapse rate in the imACTH group (24.10%) was lower than oMP (30.77%) and oP groups (33.33%), and the time taken for relapse in the imACTH group (79.00 [56.50, 152.00] days) was longer than oMP (62.50 [38.00, 121.75] days) and oP groups (71.50 [40.00, 99.75] days), but the differences were not statistically significant (p = 0.539 and 0.530, respectively). The occurrence of adverse effects was similar among the three groups.Conclusions: The short and intermediary-term efficacy and recurrence rates of oMP are not inferior to those of imACTH and oP for the treatment of IS. Significantly, the time to achieve electroclinical remission with oMP was quicker than that with imACTH and oP. Considering its convenience, affordability, and the absence of irreversible side effects, oMP can serve as a form of first-line treatment for newly diagnosed IS.

Kimura Disease as a Rare Cause of Proptosis: A Case Report

Kimura disease (KD) is a rare chronic inflammatory disorder of unknown aetiology that primarily affects the head and neck region with lymph node involvement. Young to middle-aged adult Asian males are predominantly affected. The most common presentation is painless subcutaneous swelling in the head and neck region, while proptosis or orbital involvement is very rarely reported. KD shares some features with other inflammatory and neoplastic disorders, including lymphoma; thus, investigations to confirm the diagnosis should not be delayed. Systemic corticosteroids are commonly used to treat KD and show an excellent response; however, the optimal treatment is still uncertain, and KD has a high recurrence rate. We describe the case of a patient with KD who presented with proptosis and post-auricular swelling, which responded well to oral prednisolone treatment.

Tocilizumab for Juvenile Takayasu Arteritis Complicated with Acute Heart Failure at Onset

Chronic heart failure caused by aortic valve regurgitation is a common complication of Takayasu arteritis (TA). However, fewer patients develop acute heart failure (AHF), and no specific treatment for AHF in TA has been established. We encountered a 12-year-old girl with TA who developed AHF at onset. We successfully treated her with intravenous methylprednisolone and tocilizumab. She developed palpitations and shortness of breath three weeks before admission. Her symptoms exacerbated rapidly and she finally entered the intensive care unit due to respiratory distress and tachycardia. Blood pressure measurements on the left arm and bilateral legs were paradoxically lower than that on the right arm. Chest X-ray revealed a severely enlarged heart. Contrast computed tomography showed an expanded aorta, aortic aneurysm, meandering, and irregular diameter of the aorta. The left ventricular ejection fraction (LVEF) was 20% on cardiac ultrasound. Her medical condition was finally diagnosed as TA with AHF. Along with inotropes and diuretics, methylprednisolone pulse therapy was administered on hospital days 2-4 and hospital days 12-14, followed by oral prednisolone. However, cardiac function was not notably improved. As intravenous cyclophosphamide therapy requires hydration and may exacerbate AHF, we initiated weekly subcutaneous tocilizumab treatment (162 mg/week) from hospital day 20. Inotropes were discontinued on hospital day 51 and her LVEF had gradually improved to 37.5% at discharge (day 63). As AHF in TA is presumed to be due to inflammation of the myocardium, tocilizumab could be a treatment option for TA with AHF.

Scedosporium scleritis following pterygium excision with conjunctival autograft

A 67-year-old female presented 6 months following left pterygium surgery with autoconjunctival graft with presumed episcleritis. Following a trial of topical dexamethasone, she returned with pain, reduced vision, and a donor-site scleral nodule. MRI orbits demonstrated scleritis; oral prednisolone was commenced for presumed immune-mediated scleritis. Ten days later, vision reduced to light-perception with significant vitritis overlying a subretinal lesion associated with the donor site. Vitreous tap cultured Scedosporium aurantiacum. Treatment consisted of vitrectomy, scleral debridement with corneal patch graft, with both systemic and intravitreal voriconazole. Further scleral debridement was attempted but unable to be completed due to its posterior extent. As repeat MRI orbits showed persistent active scleritis in proximity to the optic nerve which posed a risk of meningitis, a decision was made for enucleation. This case highlights the difficulties in distinguishing between infectious and autoimmune scleritis, and the importance of excluding infection, particularly in eyes with prior surgery.