High-dose Prednisolone Research Articles

Vitamin B12 responsive developmental and epileptic encephalopathy due to a novel mutation in the FUT2 gene: a case report

BackgroundVitamin B12 deficiency is a recognised cause of neurological manifestations, including peripheral neuropathy, behavioural changes, and seizures. However, developmental and epileptic encephalopathy due to vitamin B12 deficiency is very rare. Here, we report an infant with vitamin B12-responsive developmental and epileptic encephalopathy due to a novel mutation in the fucosyltransferase 2 (FUT2) gene responsible for vitamin B12 absorption.Case presentationAn 11-month-old girl of non-consanguineous parents presented with recurrent episodes of seizures since four months. Her seizures started as flexor epileptic spasms occurring in clusters resembling infantile epileptic spasms syndrome with hypsarrhythmia in the electroencephalogram. She was treated with multiple drugs, including high-dose prednisolone, vigabatrin, sodium valproate, levetiracetam and clobazam, without any response, and she continued to have seizures at 11 months. She had an early developmental delay with maximally achieving partial head control and responsive smile at four months. Her development regressed with the onset of seizure; at 11 months, her developmental age was below six weeks. On examination, she was pale and had generalised hypotonia with normal muscle power and reflexes.Her full blood count and blood picture revealed macrocytic anaemia with oval and round macrocytes. Bone marrow aspiration showed hypercellular marrow erythropoiesis with normoblastic and megaloblastic maturation. Due to the unusual association of refractory epilepsy and megaloblastic anaemia, a rare genetic disease of the vitamin B12 or folate pathways was suspected. The whole exome sequencing revealed a homozygous missense variant in exon 2 of the FUT2 gene associated with reduced vitamin B12 absorption and low plasma vitamin B12 levels, confirming the diagnosis of vitamin B12 deficiency related developmental and epileptic encephalopathy. She was started on intramuscular hydroxocobalamin, for which she showed a marked response with reduced seizure frequency.ConclusionWe report a novel variant in the FUT2 gene associated with vitamin B12-responsive developmental and epileptic encephalopathy and megaloblastic anaemia. This case report highlights the importance of timely genetic testing in children with refractory developmental and epileptic encephalopathy to identify treatable causes.

PFAPA syndrome: a case report

Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is a complex autoinflammatory disease characterized by recurrent episodes of fever and systemic inflammatory features. It is usually a disease of children but adult onset has also been reported. The common clinical features are aphthous stomatitis, cervical lymphadenitis, pharyngitis etc with exclusion of upper respiratory tract infection and cyclic neutropenia. The patient remains completely asymptomatic between the episodes of these clinical features with normal growth and development. Here we report a case of 20-year-old male who presented with features suggestive of PFAPA syndrome and well responded with a single dose of high dose prednisolone. BIRDEM Med J 2024; 14(3): 161-165

Debulking surgery after muscular paraffin oil injections: Effects on calcium homeostasis and patient satisfaction.

Cosmetic paraffin oil injections can lead to granuloma formation causing hypercalcemia and kidney failure. This study explores whether debulking surgery is an effective treatment for improving calcium homeostasis, inflammation and clinical symptoms. In a retrospective study, we reviewed 33 patients undergoing debulking surgery. Changes in calcium, inflammatory markers, and renal function from baseline up to twelve months post-surgery were assessed. Patients were interviewed post-surgery. The patients were 34.6 years (SD 6.9) and had 1,104 grams (SD 591) of granuloma tissue removed following injection of 1,329 mL (SD 803) paraffin oil 7.9 years (SD 3.2) earlier. Seventeen patients had hypercalcemia and experienced a significant decline in ionized calcium from 1.48 mmol/L (SD 0.16) at baseline to 1.33 mmol/L (SD 0.03) at twelve months (p<0.002), although only four men (23.5%) became normocalcemic. Serum ferritin was reduced by 50% after twelve months (p=0.048). Sixteen patients were normocalcemic and had no change in calcium homeostasis but experienced a 20% drop in serum ferritin levels (p=0.025) after surgery. Fifteen patients completed all their planned surgeries within the study period and experienced a decline in serum ionized calcium (p=0.031), ferritin (p=0.011), and interleukin 2-receptor (p=0.037). A patient satisfaction survey showed that 55% of patients reported post-operative satisfaction scores of 10/10, and 59% of the patients reported reduced pain. Surgery improved calcium homeostasis in a fraction of patients, reduced inflammation and subjective symptoms such as pain and mental well-being in a patient group left with few treatment options except high-dose prednisolone.

Diabetic ketoacidosis in a patient with type 2 diabetes induced by multiple triggers: a case report and implications for clinical practice

AbstractDiabetic ketoacidosis is a recognized side effect of SGLT‐2 inhibitors precipitated by several factors. A 58‐year‐old man with long‐standing, well‐controlled type 2 diabetes on dapagliflozin, metformin and gliclazide and subacute De Quervain thyroiditis treated with high dose prednisolone was evaluated in the emergency department for abdominal pain and tachypnea. Severe diabetic ketoacidosis related to dapagliflozin was diagnosed. De Quervain thyroiditis and treatment with high dose prednisolone were identified as potential triggering factors. Combined use of dapagliflozin and high dose prednisolone may increase the risk for SGLT‐2 inhibitors related diabetic ketoacidosis, especially in the course of acute illness.

Airway proteomics reveals broad residual anti-inflammatory effects of prednisolone in mepolizumab-treated asthma

BackgroundMepolizumab is an anti-interleukin-5 monoclonal antibody treatment for severe eosinophilic asthma (SEA) that reduces asthma exacerbations. Residual airway inflammation on mepolizumab may lead to persistent exacerbations. Oral corticosteroids remain the main treatment for these residual exacerbations. ObjectiveOur study aimed to explore the corticosteroid-responsiveness of airway inflammation after mepolizumab treatment to find potentially treatable inflammatory mechanisms beyond the IL-5 pathway. MethodThe MAPLE trial was a multi-centre, randomized, double-blind, placebo-controlled, crossover study of 2 weeks of high-dose oral prednisolone treatment at stable state in 27 patients treated with mepolizumab for SEA. We analysed paired sputum (n=16) and plasma (n=25) samples from the MAPLE trial using high-throughput Olink® proteomics. We also analysed additional sputum proteins using ELISA. ResultsIn patients receiving mepolizumab, prednisolone significantly downregulated sputum proteins related to type-2 inflammation and chemotaxis including IL-4, IL-5, IL-13, CCL24, CCL26, EDN, CCL17, CCL22, OX40 receptor, FCER2, and the ST2 receptor. Prednisolone also downregulated cell adhesion molecules, prostaglandin synthases, mast cell tryptases, MMP1, MMP12, and neuroimmune mediators. Neutrophilic pathways were upregulated.Type-2 proteins were also downregulated in plasma, combined with IL-12, IFN-γ, and IP-10. IL-10 and amphiregulin were upregulated. ConclusionAt stable state, prednisolone has broad anti-inflammatory effects on top of mepolizumab. These effects are heterogeneous and may be clinically relevant in residual exacerbations.

Characterization of Anti-GAD65-Associated Neurological Syndromes: Clinical Features and Antibody Titers

Introduction: Anti-GAD65 antibodies are associated with several neurological phenotypes. Antibody titers are increasingly recognized as useful in diagnosis and prognosis. Objective: To describe a Portuguese cohort of patients with anti-GAD65-associated neurological syndromes. Methods: Retrospective analysis of all patients with positive anti-GAD65 antibodies and associated neurological syndromes followed in a tertiary referral center. Results: Nineteen anti-GAD65 antibody-positive neurological patients were identified, 62.3% female, with a mean age of onset of 56.0 (SD = 13.3) years. Comorbid autoimmune disorders were present in seven patients. Six patients had limbic encephalitis (31.6%), four had epilepsy (21.1%), four had cerebellar ataxia (21.1%), and three had stiff-person syndrome (15.8%). Two patients presented with isolated cognitive dysfunction (executive and mnesic) in the absence of other neurological symptoms. The mean follow-up time was 24.0 (14.0–42.0) months, at the end of which the mean modified Rankin Scale (mRS) value was 2.0 (1.0–4.0). Screening for malignancies was negative in all patients. Serum quantitative analysis was carried out in 18 patients, 10 of whom showed titers above previously defined cut-off points (&gt;10,000 IU/L for ELISA and &gt;20 mmol/L for RIA). Quantitative CSF analysis was performed in nine patients, with four showing above-threshold titers. There was no association between anti-GAD65 levels and clinical phenotype or the final mRS values. High-dose intravenous methylprednisolone and oral prednisolone were the most common acute and chronic treatment regimens, respectively. Conclusion: Anti-GAD65 antibodies are associated with varied neurological syndromes, and antibody titers alone should not be used to exclude a disease.

Nonspecific inflammatory pseudotumor of the maxillary and temporal fossa: a study of seven cases

ObjectiveInflammatory pseudotumor (IPT) is a rare, locally aggressive, benign neoplasm of unknown etiology. Because of its aggressive clinical behavior and locally destructive or infiltrative features, it may be mistaken for a malignant tumor. Approximately 5–44% of extrapulmonary IPT occur in the head and neck, primarily affecting the orbit. Study DesignBetween 2008 and 2021, our hospital received referrals for seven patients (three men and four women, aged 42–73 years) with pain, swelling, mass, and trismus. Computed tomography, magnetic resonance imaging, and biopsy were performed on all patients to diagnose IPT. ResultsOf the seven patients, four received low-dose prednisolone (PSL), one underwent surgery, and two were left untreated. The IPT disappeared in one of the two untreated cases, whereas it improved and later deteriorated in the other. The surgical patient had no recurrence. Low-dose PSL was effective in two patients; however, high-dose PSL and immunosuppressants were required in the remaining two cases owing to infiltration into each orbit or brain region. ConclusionsLow-dose PSL treatment was applicable in IPT cases affecting the maxillary to temporal fossa region, wherein symptoms did not improve without treatment. However, when low-dose prednisolone was ineffective, high-dose prednisolone and immunosuppressants were required.

Deciphering the association between biopsy-confirmed systemic small vessel vasculitis and Epstein-Barr virus-positive polymorphic B-cell lymphoproliferation.

The Epstein-Barr virus (EBV) is associated with various lymphoproliferative disorders (LPD). Additionally, EBV infection has correlated with diverse autoimmune diseases. However, the association between EBV and systemic small vessel vasculitis (SVV) remains controversial. Here, we report a case of SVV with pauci-immune glomerulonephritis accompanied by an EBV-positive polymorphic B-cell LPD, not otherwise specified. The intricate distinction between EBV-positive B-cell LPD and SVV was difficult, as both diseases demonstrated similar clinical presentations. Lymph node and kidney biopsies facilitated the accurate diagnosis of these two conditions. The administration of high-dose prednisolone, combined with rituximab, proved efficacious, with no instances of relapse over the subsequent 2-year period. This case indicates an association between EBV-positive B-cell LPD and SVV. The diligent execution of biopsies is a crucial diagnostic and interpretive strategy, generating precise comprehension of this condition and guiding its appropriate therapeutic management.

Split Dose of Prednisolone in the Treatment for Erythema Nodosum Leprosum: A Case Series.

Background Erythema nodosum leprosum (ENL) is an immune complex-mediated reaction that clinically presents as tender erythematous evanescent nodules, mostly associated with systemic symptoms. Oral prednisolone is the drug of choice, with doses ranging from 0.5 to 1 mg/kg. Some cases may develop new lesions and systemic symptoms despite 1 mg/kg prednisolone, and in ideal practice, physicians escalate the prednisolone dose for immediate arrest of inflammation to prevent complications. However, a high dose of prednisolone has more side effects in the long term and causes more immunosuppression. Methods In cases of ENL, those not responding to a conventional once-daily regimen were given a split dose of oral prednisolone instead of increasing the dose. They were followed up for response, and serum cortisol was measured to see for hypothalamic-pituitary-adrenal (HPA) axis suppression. Results Eight cases of ENL (three nodular, three necrotic, one pustular, and one nodulcerative) had a dramatic response to split-dose therapy without any relapse and HPA axis suppression. Conclusion A split-dosing regimen can be a good treatment option in ENL with better control, less steroid dependency, and a lower relapse rate.

DRESS as a rare differential diagnosis in eosinophilia, skin rash and acute hepatitis

A 21-year-old female patient presented with fever, pharyngitis, lymphadenopathy and generalized exanthema that had started 2 weeks prior. Allergies were not known, the family and travel history were negative. Due to depression, Duloxetine had been taken for 1.5 years, and due to bipolar disorder, a treatment with Lamotrigine was started four weeks prior but was stopped because of increased transaminase levels. Laboratory findings on admission showed eosinophilia (1.327 /nl), lymphocytosis and acute hepatitis (GOT 428 U/l, GPT 438 U/l) with deranged coagulation. Inflammatory parameters were increased. Ultrasound revealed hepatosplenomegaly with ascites. Acute viral or parasitic infection was excluded serologically. A skin biopsy showed a perivascular inflammatory infiltrate, compatible with a drug reaction. An inflammatory infiltrate was found in the liver biopsy, consistent with drug-induced hepatitis. Cough, dyspnea and pleural effusion occurred. In summary of the findings and with the help of the RegiSCAR-Score, the diagnosis of drug reaction with eosinophilia and systemic symptoms (DRESS) could be made. Under high-dose prednisolone therapy, a gradual decrease of transaminases and reconstitution of liver synthesis could be observed.In patients with eosinophilia, lymphadenopathy, acute hepatitis and generalized exanthema, DRESS is a rare but-due to its potentially life-threatening consequences-important differential diagnosis. The most important measure is to stop the suspected inducing medication immediately. Severe cases should be treated with high-dose systemic corticosteroids.

Spontaneous Hypoglycaemia due to Insulin Autoimmune Syndrome in Six Cases, Response to Steroid Therapy and Rituximab.

Dr. Hirata of Japan first described insulin autoimmune syndrome (IAS) in 1970. Seven hundred ninety-five cases of this rare syndrome have been reported from Japan and China and 29 from India. IAS has the following characteristic features 1) severe spontaneous attacks of hyperinsulinemic hypoglycaemia, 2) high total immunoreactive insulin levels, 3) elevated insulin autoantibody (IAA) titres, 4) no prior exposure to exogenous insulin, and 5) no pathological abnormalities of the pancreatic islet cells. We treated six cases of IAS with high doses of prednisolone for 4-6 weeks and then gradually reduced the doses. Diagnosis of IAS was established by documenting Whipple's triad of symptoms and signs of hypoglycaemia, blood sugar <55 mg/dl, improvement of symptoms with dextrose infusion, inappropriately increased insulin levels >3 uU/ml, C-peptide levels >0.6 ng/ml, and increased titres of anti-insulin autoantibodies. Insulinoma and non-pancreatic tumours were ruled out by CECT (contrast-enhanced computerised tomography) or MRI (magnetic resonance imaging) of the abdomen and if necessary endoscopic ultrasonography and gallium 68 Dotanoc PET (positron enhanced tomography). Autoimmune screening and serum electrophoresis were done to rule out multiple myeloma. Monitoring of the patient's blood sugars was done by the laboratory, glucometer readings, and a freestyle libre glucose monitoring system. Remission of hypoglycaemic episodes, hyperglycaemic episodes, and marked reduction of serum insulin and insulin autoantibodies in four out of six patients with diet therapy and steroids. Two patients resistant to steroids were treated with rituximab successfully. Patient 6 developed serious complications of cytomegalovirus and Pneumocystis carnii after rituximab, which were treated successfully. A careful history including recent infections, medications, and vaccinations provides vital clues in the evaluation. An increased awareness of IAS will prevent unnecessary and costly investigations and surgery. Although it is often self-remitting, steroids are contributory in severe cases. Immunosuppressives are used successfully in cases refractory to steroids. Continuous glucose monitoring system (CGMS), by freestyle libre glucose monitoring system, provided real-time blood sugar values, total time in hypoglycaemia, and total time in the range (TIR), which proved very valuable in managing IAS patients. Low CGMS values should be corroborated clinically and with laboratory or glucometer values.

OA15 A case series of idiopathic granulomatous mastitis patients and their immunosuppressive management

Abstract Background/Aims Rheumatologists are experts in managing immunosuppression and are asked to supervise immunosuppression for non-rheumatic conditions. Thus, it is important to be aware of these conditions and the regimes required. Idiopathic granulomatosis mastitis (IGM) is a rare inflammatory breast condition which can have systemic manifestations including arthritis. Methods Our index case presented with peripheral ankle synovitis/tenosynovitis, struggling to weight bear, whilst being investigated for a breast mass. Breast US, mammogram and biopsy (acute and chronic inflammatory cells including multi-nucleated giant cells) were completed and there was little response to 3 courses of antibiotics. No mycobacteria were seen on microscopy, ZN stain negative, CXR normal, T spot negative and serum ACE normal. There was no rash and no conditions associated with a seronegative spondyloarthropathy. RF, anti-CCP and ANA were negative. A moderate course of prednisolone immediately resolved the peripheral arthritis, but high-dose steroids were required to reduce the breast mass. The mass recurred upon weaning prednisolone requiring the addition of azathioprine as a steroid-sparing agent. Results Our case series includes the immunosuppressive management of 3 other IGM patients: 1) 43-year-old referred after an 8 year history of alternating breast ‘abscesses’ and sinuses treated with courses of antibiotics to little avail. High dose prednisolone and methotrexate resolved the breast mass within a month. 2) 37-year-old referred with a 14 year history of breast masses and fistulae previously treated with surgery and antibiotics. Currently asymptomatic so commenced on methotrexate. 3) 39-year-old referred with a 3 year history of recurrent sterile breast abscesses previously managed with antibiotics, incision and drainage. Recent small masses had settled with NSAIDs. Conclusion IGM is a rare benign inflammatory breast disease of unknown aetiology, commonest in Middle Eastern populations, median age 30s. The most common presentation is a unilateral painful mass, which can ulcerate, fistulate and form abscesses. Management of IGM varies from expectant/topical steroids/NSAIDs to high dose steroids and DMARDs. A literature review shows that the usual starting dose of prednisolone is 40-60mg; the addition of methotrexate and azathioprine increases likelihood of successful treatment and allows for faster steroid tapering. Surgery for specific types of IGM can also be useful; for example, where there is duct granuloma, surgical excision can be curative. IGM can associate with arthritis, erythema nodosum, and episcleritis. One case series found that 14% of patients with IGM had concomitant arthritis. Key points: In our local experience we have had four patients with IGM, all previously treated with antibiotics or surgical methods but subsequently diagnosed with IGM and referred for immunosuppressive therapy. Being a rare condition, we feel it’s important for clinicians to be aware of IGM and its link with inflammatory arthritis. Disclosure S. Islam: None. P. Stonelake: None. H. John: None.

P015 Nodular skin rash associated with relapsing polychondritis, a challenging diagnostic journey to VEXAS syndrome

Abstract Background/Aims VEXAS syndrome, first described in 2020, is a haemato-inflammatory disease caused by a mutation in the gene-UBA1.This syndrome can coexist with or without Myelodysplastic syndrome. What makes this case interesting is its long and challenging diagnostic journey and the involvement of different clinical specialties such as dermatology, ophthalmology, rheumatology, respiratory, haematology, and stroke teams. Ultimately, two years after the initial presentation, this appeared to be the first case of VEXAS with confirmed genetic mutation in the region. VEXAS being a relatively new entity, treating clinician/s were not aware of the possibility initially. Therefore, spreading awareness regarding the clinical picture of VEXAS is prudent. This case report highlights rheumatological and dermatological manifestations of the disease to aid in the timely diagnosis and management of future cases. Methods We report a 74-year-old previously healthy male presented in 2021 with a rash on his legs, arms, and torso since October 2019. It was progressive, painful, palpable, erythematous, non-pruritic, intermittent rash, cleared with hyperpigmentation. He also had oligoarticular arthritis, constitutional symptoms, recurrent episodes of polychondritis, uveitis, bibasal lung fibrosis, and an ESR and CRP above 100. He also had lymphadenopathy above the diaphragm, but the biopsy only showed necrotic material, and the bone marrow biopsy did not show any features of haematological malignancy. After excluding infections, he was started on high-dose oral prednisolone for relapsing polychondritis. The skin and inflammatory markers improved immediately following high doses of steroids. However, whenever steroids were weaned down, even with the help of Methotrexate, his skin was flaring. He also had unprovoked DVT and, while on anticoagulation, developed occipital infarction. He had extensive skin involvement when he had to stop Methotrexate following a chest infection. Results The skin biopsy was inconclusive. There was no significant cytopenia but persistent macrocytosis even after stopping Methotrexate. As a result, he underwent a repeat bone marrow biopsy, which showed Vacuoles and gene UBA1 mutation in 2023. Conclusion The heterogeneity and complexity of the disease are due to multi-organ involvement, including skin, joints, eyes, lungs, blood vessels, and cartilage, simultaneously or cumulatively. Skin involvement in VEXAS can also be vast, ranging from neutrophilic dermatosis, erythema nodosum-like lesions, leukocytoclastic, or urticarial vasculitis. Cutaneous manifestations develop in the majority with VEXAS syndrome and can be the first presenting symptom in this new rare syndrome. Disclosure M. Gunawardena: None. L. Yalakki: None. R. Moitra: None. E.L. Williams: None. H. Wong: None. D. D'Cruz: None.

P025 Routine Giant-cell arteritis turned Heidenhain variant of sporadic Creutzfeldt-Jakob disease: a mysterious case report!

Abstract Background/Aims Giant-cell arteritis (GCA) is a large vessel vasculitis predominantly affecting those above 50 years of age. Creutzfeldt-Jacob disease (CJD) is not a well-recognised differential diagnosis of GCA presentation. Prion diseases are progressive, currently untreatable and ultimately fatal. Methods We report the case of a 76-year-old female who had an eventful hospital stay of 37 days. She has a history of asthma, hypertension, ulcerative colitis and right-sided GCA 5 years back (normal inflammatory markers &amp; biopsy). Results The patient presents to Harlow ED with a right temporal headache with tenderness, jaw claudication symptoms, blurred vision, diplopia, dizziness and painful right eye movements. Her CRP was &amp;lt;1 and ESR was 5. The plan was to treat it as a recurrence of her GCA and to continue with the high dose Prednisolone started by the ED. CT Head was normal. Owing to poor response, IV Methyl Pred was given. On day six, patient was re-admitted with worsening headache and generalised decline. Ophthalmology review noted multiple visual field defects and treated as GCA. A neurology review noted the patient to be encephalopathic. Advice was for MRI head and CSF analysis - both NAD. On day 12 she had ongoing headache, diplopia, poor vision/memory, and reduced hearing in right ear, but alert and following instructions. On day 17, ENT suggestion was to treat as Meniere’s and start on Betahistine. On day 22, the patient had a TIA episode and discussed with Stroke team. Repeat CT Head was nil acute. By day 26, the patient was unresponsive intermittently and was treated as hypoactive delirium. On day 31 a seizure activity was noticed. Neurology review on day 33 noted rapid decline- the patient being mute, seizure/atypical encephalitis. MRI-Head with contrast showed the possibility of CJD. The very next day patient was reviewed by the team from National CJD Research and Surveillance team, Edinburgh. The formal diagnosis given was: probable Sporadic CJD (visual/Heidanhain variant), rapidly progressive cognitive decline with visual signs, myoclonus, and akinetic mutism, positive MRIs, bland CSF, no clear iatrogenic or variant risk factors, and no credible alternative diagnosis. The distressed family members were updated and the patient was put on EoL pathway. She died peacefully four days later (Day 37). Conclusion The importance of this case is the conflicting inputs from at least five specialities in a rapidly deteriorating patient. A background of previous right-sided GCA did prompt initial treatment in those lines. MRI Head DWI sequence did show the classic ‘cortical ribboning’ with right caudate and lentiform hyperintensity; which clinched the mysterious diagnosis of this rare disease which requires public health surveillance. CJD is to be suspected in any undiagnosed rapidly progressive mental deterioration or dementia, often with behavioural abnormalities and myoclonus. Disclosure S. Narikkoottungal: None. K. Gunasekera: None. K. Ahmed: None.

Vogt-Koyanagi-Harada disease developed during chemotherapy for Hodgkin lymphoma: a case report

BackgroundOcular manifestations are known for non-Hodgkin lymphoma, but are rare for Hodgkin lymphoma. We report a case of Vogt-Koyanagi-Harada (VKH) disease presenting as serous retinal detachment and uveitis in both eyes in a child undergoing chemotherapy for Hodgkin lymphoma.Case presentationThe patient was a 7-year-old boy with stage IIB Hodgkin lymphoma (nodular lymphocyte predominant type) who was undergoing chemotherapy, including 2 cycles of the OEPA regimen and 1 cycle of the COPDAC regimen. Two days after the end of the COPDAC regimen, the patient complained of headache and of blurred and decreased vision in both eyes. On the basis of optic symptoms, such as uveitis and serous retinal detachment in both eyes, increased cell counts in cerebrospinal fluid, and positivity for human leukocyte antigen (HLA)-DR4 in peripheral blood cells, incomplete VKH disease was diagnosed. Intravenous treatment with high-dose prednisolone (60mg/m2/day) for 7 days improved both visual acuity and serous retinal detachment and enabled the remains of the COPDAC chemotherapy cycle to be administered. With prednisolone treatment, visual acuity improved from 20/500 to 20/20 in the right eye and from 20/63 to 20/25 in the left eye. Because multiple vitiligo lesions later appeared in the abdomen, complete VKH disease was finally diagnosed.ConclusionThe onset of VKH disease occurred during chemotherapy for Hodgkin lymphoma. The patient was HLA-DR4-positive and might have had a predisposition to develop autoimmune diseases, including VKH disease. However, the anticancer drugs administered to this patient have not been reported to cause uveitis. Whether Hodgkin lymphoma triggered the development of VKH remains unclear. Early diagnosis of VKH disease and prompt treatment with high-dose prednisone enabled the patient to maintain good visual function despite chemotherapy for Hodgkin lymphoma.

Efecto de la terapia de inducción con micofenolato o ciclofosfamida sobre los niveles séricos del factor activador de células B (BAFF) en pacientes con lupus eritematoso sistémico

IntroductionSystemic lupus erythematosus (SLE) is a complex autoimmune disease in which B cell hyperactivity plays a key role in its pathophysiology. In this disease, the B cell activating factor (BAFF) is implicated in the loss of immunological tolerance, and high concentrations of this factor have been linked to disease progression. It is unknown how induction therapies with cyclophosphamide (CFM) or mycophenolate (MMF) modulate BAFF levels. ObjectiveTo determine how induction therapies modulate BAFF concentrations. Materials and methodsAn analytical observational study was performed with 20 patients with SLE from two institutions between 2020-2022, clinical and laboratory information was obtained from medical records. Measurement of serum BAFF levels was performed using an ELISA kit and statistical analyses with GraphPad Prism version 9. Results20 patients with a diagnosis of SLE, 18 with CFM and 2 with MMF, were included, nine patients at baseline and eleven at 3-6 months. The median BAFF in SLE patients was 902.2 pg/mL and 379.7 pg/mL in healthy controls, statistically significant differences (p=.0003). BAFF levels were also found to be different among patients treated with antimalarials (p=.0465) and an inverse correlation with creatinine values and prednisolone doses was also observed. ConclusionsBAFF levels are affected during induction therapy, observing an initial reduction and subsequent recovery at 3-6 months, our results suggest that chloroquine and high doses of prednisolone maintain lower serum BAFF levels.

Post–COVID-19 autoimmune hypoglycemia: A case report

ABSTRACT Insulin autoimmune hypoglycemia is characterized by insulin autoantibodies causing initially hyperglycemia followed by hypoglycemia. The authors describe a case of a patient who presented with recurrent episodes of severe hypoglycemia 1 month after COVID-19 infection. Blood glucose monitoring during admission revealed postprandial hyperglycemia ranging from 15 to 18 mmol/L followed by severe hypoglycemic episodes. His insulin at the time of hypoglycemia (blood glucose level 1.5 mmol/L) was 255 mU/L (3–25 mU/L). C-peptide was 3.3 nmol/L (0.3–1.4 nmol/L), and β-hydroxybutyric acid was 0.06 mmol/L (&lt;0.27 mmol/L). The anti-insulin antibodies level elevated at 403 U/mL (&lt;0.5 U/mL). A diagnosis of post–COVID-19 autoimmune hypoglycemia was performed, and the patient was started on high-dose prednisolone 60 mg/day with gradual tapering over 3 months. Metformin and acarbose were also started to treat hyperglycemia. On follow-up after glucocorticoid cessation, blood glucose profile stabilized with no hypoglycemia or hyperglycemia. This is the fourth case report written in the literature.

High-Dose Glucocorticoids for the Treatment of Sudden Hearing Loss

BackgroundSystemic glucocorticoids are commonly used for primary therapy of idiopathic sudden sensorineural hearing loss (ISSNHL). However, the comparative effectiveness and risk profiles of high-dose over lower-dose regimens remain unknown.MethodsWe randomly assigned patients with sudden hearing loss of greater than or equal to 50 dB within 7 days from onset to receive either 5 days of high-dose intravenous prednisolone at 250 mg/d (HD-Pred), 5 days of high-dose oral dexamethasone at 40 mg/d (HD-Dex), or, as a control, 5 days of oral prednisolone (Pred-Control) at 60 mg/d followed by 5 days of tapering doses. The primary outcome was the change in hearing threshold (pure tone average) in the three most affected contiguous frequencies from baseline to day 30. Secondary outcomes included speech understanding, tinnitus, communication competence, quality of life, hypertension, and insulin resistance.ResultsA total of 325 patients were randomly assigned. Mean change in 3PTAmost affected hearing threshold from baseline to 30 days was 34.2 dB (95% CI, 28.4 to 40.0) in the HD-Pred group, 41.4 dB (95% CI, 35.6 to 47.2) in the HD-Dex group, and 41.0 dB (95% CI, 35.2 to 46.8) in the Pred-Control group (P=0.09 for analysis of variance). There were more adverse events related to trial medication in the HD-Pred (n=73) and HD-Dex (n=76) groups than in the Pred-Control group (n=46).ConclusionsSystemic high-dose glucocorticoid therapy was not superior to a lower-dose regimen in patients with ISSNHL, and it was associated with a higher risk of side effects. (Funded by the Federal Ministry of Education and Research [BMBF]; EudraCT number, 2015‐002602‐36.)

De novo normotensive scleroderma renal crisis six years after living-donor renal transplantation in a patient with overlapping systemic sclerosis/systemic lupus erythematosus syndrome: a case report

BackgroundScleroderma renal crisis (SRC) is a critical kidney involvement of systemic sclerosis (SSc), often resulting in end-stage renal disease. Although the recurrence of SRC in the allograft has been reported, the development of de novo SRC after kidney transplantation has not been reported. Furthermore, normotensive SRC, which rarely occurs, makes prompt diagnosis more challenging. This fact should be recognized widely among nephrologists.Case presentationWe report a 37-year-old Japanese man with overlapping SSc/systemic lupus erythematous syndrome who developed normotensive SRC in the transplanted kidney shortly after glucocorticoid escalation. Six years prior to admission, he underwent an ABO-compatible living donor kidney transplantation because of lupus nephritis. He was admitted to our hospital for gradually worsening kidney dysfunction. A kidney biopsy showed idiopathic granulomatous interstitial nephritis and high-dose prednisolone was prescribed. Although renal function improved tentatively, it deteriorated again a week later. A secondary kidney biopsy revealed acute thrombotic microangiopathy, leading to the diagnosis of normotensive SRC because all other causes were excluded, and blood pressure was within normal range. Adding an angiotensin-converting enzyme inhibitor and tapering glucocorticoid slowed the speed of deterioration of his kidney function, but he finally required hemodialysis induction.ConclusionsSRC can newly develop even in the transplanted kidney, especially when high-dose glucocorticoid is administered. Normotensive SRC makes the diagnosis challenging, so nephrologists should carefully monitor patients with SSc and transplanted kidneys to treat SRC promptly.

Analysis of Risk Factors for Developing Tuberculosis in Patients Who Received Prophylactic Latent Tuberculosis Infection Treatment with Experience of Biologic Medications.

Biologic medications have dramatically improved the treatment outcomes of immunological inflammatory diseases, but their immunosuppressive effects put patients at risk for tuberculosis (TB). We investigated the risk factors for developing TB in patients treated for latent tuberculosis infection (LTBI) who also had experience of using biologic medications. At Keio University Hospital, we retrospectively investigated patients treated with anti-mycobacterial drugs before or concurrently with biologic medications from January 2012 to August 2020. Patients in the 'follow-on cases group' who had a positive TB screening test after initiating biologic medications and subsequently started LTBI treatment were excluded. We researched and compared the patient characteristics for TB and non-TB patient groups. Of the 146 eligible patients, 5 (3.4%) developed TB. The incidence rate was 600/100000 person-years. There were no significant differences between TB and non-TB patient groups in the history of TB, interferon-gamma release assay (IGRA), duration of biologic medication therapy, LTBI treatment periods, concomitant use of calcineurin inhibitors or anti-rheumatic drugs. The percentage of patients who received prednisolone at a dose of ≥15 mg for more than 1 month was higher in those who developed TB than in those who did not (40.0 vs. 7.1%, p = 0.054); however, this difference was not statistically significant. Regular monitoring of TB is necessary for long-term concomitant use of high prednisolone doses during and after the administration of biologic medications.