Dose Of Prednisolone Research Articles

E024 A pregnant woman with vasculitis and a new rash

Abstract Background/Aims A 41-year-old pregnant woman at 16 weeks of gestation was followed-up in the combined obstetric and rheumatology clinic as she had a history of eosinophilic granulomatosis with polyangiitis (EGPA) and two previous miscarriages at first trimester. Her vasculitis was well-controlled with a baseline daily dose of prednisolone 7.5mg. Methods During her pregnancy, she started on vaginal progesterone by the IVF (In Vitro Fertilization) team for pregnancy support following a vaginal bleed. During her second review in clinic, she complained about a widespread pruritic rash for the last 3 weeks. She had already been seen by her GP who prescribed her emollients without any improvement. On examination, the active lesions looked urticarial and were tender. There was also residual hyperpigmentation. The lesions persisted for more than 24 hours before spontaneous resolution. Cutaneous vasculitis was suspected based on the duration of the rash more than 24 hours, the hyperpigmentation, tenderness, and the history of EGPA. Otherwise, she was well in herself with no symptoms of active vasculitis and no recent infections. Therefore, prednisolone was increased to 15mg daily and loratadine was given for the pruritus. Her repeat blood tests were reassuring with negative ANCA, normal C3 and C4, CRP, and eGFR. Results During her early follow-up, the rash was the same despite the higher dose of steroids and she volunteered that the rash is worse in the morning and at night. Autoimmune progesterone dermatitis (AIPD) was considered then. AIPD is a rare autoimmune hypersensitivity reaction to endogenous or exogenous progesterone. AIPD varies greatly from patient to patient and can present as urticaria, eczema or vesiculobullous eruptions. Urticarial or EGPA vasculitis, and urticaria would have improved with steroids. Furthermore, she did not have any symptoms suggestive of systemic vasculitis and the relevant blood tests were also normal. Of note, the rash was worse in the morning and at night, which corresponded to the time of vaginal exposure to progesterone. The patient stopped the progesterone pessaries after discussion with the obstetrician. The rash improved gradually and resolved in five days, confirming the diagnosis of AIPD. Conclusion The rash could have been mistaken for urticaria or vasculitis. Consequently, the patient may have had unnecessarily antihistamines and steroids during pregnancy with all relevant side effects, such as gestational diabetes. In November 2021, NICE recommended vaginal progesterone in women who experience bleeding in early pregnancy and have a history of miscarriage as it slightly reduces the risk of a further miscarriage. The new guidelines suggest that more pregnant women will use topical progesterone during pregnancy who may develop AIPD. This means that we are going to see more pregnant women in the rheumatology clinic with AIPD and the rheumatology community should be aware of this entity. Disclosure A. Madenidou: Grants/research support; Janssen. H. Kither: None. I. Bruce: Consultancies; AstraZeneca, GSK, Eli Lilly, and UCB. Honoraria; AstraZeneca, GSK, and UCB. Grants/research support; AstraZeneca, GSK, and Janssen.

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.

OA16 Clinical and biochemical improvement following daratumumab therapy for anti-MDA5 antibody positive dermatomyositis with rapidly progressive interstitial lung disease: a case report

Abstract Background/Aims Anti-melanoma differentiation-associated 5 gene autoantibody (anti-MDA5) positive dermatomyositis (DM) with rapidly progressive interstitial lung disease (RP-ILD) represents the most severe and difficult-to-treat form of autoimmune myositis with unfavourable 6-month survival rates despite intensive treatment strategies of combination therapy with glucocorticoids, cyclophosphamide, calcineurin inhibitors, mycophenolate mofetil and rituximab. We describe a case of anti-MDA5 positive DM with RP-ILD that was refractory to intensive combination therapy, requiring courses of rescue plasmapheresis, before eventually responding to intravenous daratumumab. Methods A 30-year-old non-smoking Chinese man who was previously well was hospitalised after 3 weeks of intermittent pyrexia, non-productive cough, lethargy, rashes and myalgia. Examination revealed typical cutaneous changes of dermatomyositis, bilateral mid-to-lower zone inspiratory crepitations in his chest with no proximal muscle weakness or synovitis. Initial investigations demonstrated elevations in creatine kinase, C-reactive protein, erythrocyte sedimentation rate, lactate dehydrogenase, ferritin and neutrophil-to-lymphocyte ratio. Although an initial chest radiograph was normal, CT thorax 4 days later showed non-specific ground-glass opacities with consolidation in both lower lobes. Anti-MDA5 antibodies were strongly positive on EUROLINE Inflammatory Myopathies 16 Ag (IgG) commercial line immunoblot. He was transferred to our centre for further care one week after hospitalisation and was intubated on arrival for type I respiratory failure. He was initiated on combination therapy of intravenous methylprednisolone, intravenous rituximab, hydroxychloroquine, tofacitinib and was also treated with plasmapheresis to remove pathogenic anti-MDA5 antibodies. Although his condition improved initially with successful extubation within one week, the disease remained active in the following months with several admissions for infection-related issues and progression of RP-ILD. Despite various combinations of immunosuppressives and further courses of plasmapheresis, at 6 months post-diagnosis he continued to require a minimum daily prednisolone dose of 45mg (0.625mg/kg/day), remaining positive for anti-MDA5 antibodies on the immunoblot assay, with persistently elevated serum ferritin and transaminases. As such, he was treated with four doses of intravenous daratumumab in an attempt to deplete the long-lasting plasma cells that may be driving the disease. Results Following daratumumab treatment, the anti-MDA5 antibody turned negative on the immunoblot assay with normalisation of ferritin and transaminases, with corresponding improvement in lung function tests and chest radiograph findings. 3 months post-daratumumab, he remains clinically well on hydroxychloroquine, tofacitinib and nintedanib, with successful continuing taper of prednisolone. Conclusion Daratumumab is an anti-CD38 monoclonal antibody licensed for the treatment of multiple myeloma which has been used off-label for other refractory autoimmune conditions such as SLE. It is postulated that besides CD19/20 positive B cells, CD38 positive long-lived autoantibody-secreting plasma cells are essential drivers of chronic inflammation in these autoimmune diseases. In our patient with anti-MDA5 DM and RP-ILD, the use of daratumumab was well-tolerated and led to definite clinical and biochemical improvement. Disclosure L. Koh: None. G. Chai: None. M. Manghani: None. X. Lim: None. B.P.L. Leung: None. C. Chua: None.

E030 Osteonecrosis of the spine in antiphospholipid antibody syndrome

Abstract Background/Aims Osteonecrosis (ON) is a well-known complication of several rheumatic diseases, affecting the hip, knee, shoulder, or ankle. The involvement of spine and other sites with ON has been reported with catastrophic APS, although the nature of this association is not completely clear. In antiphospholipid syndrome, thrombosis of blood vessels is considered a possible mechanism, along with other risk factors, such as active disease, thrombocytopenia, glucocorticoid use and associated autoimmune diseases, making the pathophysiology multifactorial. We report a case of a 66-year-old male with APS who developed bone infarcts/osteonecrosis after withdrawal of anticoagulation for APS, which was necessitated by a bleeding complication and introduction of steroids for immune thrombocytopenia. Methods A 66-year-old male came to us with history of unprovoked deep vein thrombosis (DVT) for which he was on antiplatelet and anticoagulation for over 20 years. He was diagnosed to have high titre anti cardiolipin antibody (ACA)(186 U/ml) without other autoimmune conditions. He had an episode of bladder hematoma in August 2022, needing his anticoagulation to be suspended temporarily. He was also found to have profound thrombocytopenia with raised inflammatory markers. Considering it to be immune mediated he was started on high dose oral corticosteroids and was later maintained with azathioprine while the oral prednisolone dose was gradually brought down to 10 mg daily in about 3 months’ time. His thrombocytopenia improved progressively however he developed an unprovoked DVT in October 2022 and was restarted on anticoagulation. He continued to have low dose steroids for about 6 months. Meanwhile, he started having a new onset of low back pain in December 2022. An MRI of the whole spine showed multilevel osteonecrosis with haemorrhagic cavities in a few vertebral bodies. Results This case highlights three interesting points. The first is the atypical presentation of the osteonecrosis involving vertebral bodies. Osteonecrotic lesions in the spine of a patient with catastrophic primary antiphospholipid syndrome has been reported. Our case signifies that although vertebral bodies are an uncommon site for avascular necrosis in patients with APS, it is not rare. Secondly, the pathogenesis of ON is complex. Whether ACA are directly responsible for this condition or whether they are the bystanders remains controversial. ACA have shown to be an independent risk factor for developing ON in many studies. Thirdly, steroids are known to be an important risk factor in patients presenting with osteonecrosis. The vascular compromise could be attributed to a steroid induced hypercoagulative state, abnormal lipid metabolism, or fat embolism. Conclusion ON in APS is unusual and its underlying pathophysiology remains multifactorial. However, when complicated by the high ACA levels, active disease, difficult anticoagulation (bleeding tendencies), thrombocytopenia or prolonged use of steroids, it can lead to hypercoagulable state causing micro thrombosis and avascularisation. Disclosure M. Shah: None. K. Putchakayala: None.

Oral corticosteroid prescribing practice for chronic rhinosinusitis with nasal polyps among otorhinolaryngologists in Scotland: a nationwide survey.

Oral corticosteroids are used to treat exacerbations of chronic rhinosinusitis with nasal polyps. Oral corticosteroid prescribing practices vary as reported from national surveys in Italy, China, Canada and the USA. A nationwide online survey of ENT doctors practicing in Scotland was conducted using Microsoft Forms. There was a 31 per cent response rate. The most common daily doses of oral corticosteroid courses were 25 mg and 40 mg with the lengths being 14 and 7 days, respectively. Seventy-seven per cent of respondents prescribed the same daily dose throughout the course. Rhinologists prescribed longer courses with a smaller daily dose of prednisolone. Only one respondent fully agreed that there were clear guidelines regarding the daily dose and the length of oral corticosteroid course in the treatment of chronic rhinosinusitis with nasal polyps. The heterogeneity of oral corticosteroid prescribing practice in different countries, including Scotland, reveals the need for clear guidelines with a specific oral corticosteroid daily dose and length of the course.

Defining remission in childhood-onset lupus: PReS-endorsed consensus definitions by an international task force

ObjectiveTo derive childhood-onset SLE (cSLE) specific remission definitions for future treat-to-target (T2T) trials, observational studies, and clinical practice. MethodsThe cSLE International T2T Task Force conducted Delphi surveys exploring paediatric perspectives on adult-onset SLE remission targets. A modified nominal group technique was used to discuss, refine, and agree on the cSLE remission target criteria. ResultsThe Task Force proposed two definitions of remission: ‘cSLE clinical remission on steroids (cCR)’ and ‘cSLE clinical remission off steroids (cCR-0)’. The common criteria are: (1) Clinical-SLEDAI-2 K = 0; (2) PGA score < 0.5 (0–3 scale); (4) stable antimalarials, immunosuppressive, and biologic therapy (changes due to side-effects, adherence, weight, or when building up to target dose allowed). Criterion (3) in cCR is the prednisolone dose ≤0.1 mg/kg/day (maximum 5 mg/day), whereas in cCR-0 it is zero. ConclusionscSLE definitions of remission have been proposed, maintaining sufficient alignment with the adult-SLE definition to facilitate life-course research.

Suspected multiple autoimmune syndrome in a Kelpie with hypoadrenocorticism

AbstractAn 8‐year‐old, female, spayed Kelpie cross presented with hyporexia, pica, vomiting, collapse and seizures. It was diagnosed with eunatraemic, eukalaemic hypoadrenocorticism, immune‐mediated anaemia and immune‐mediated thrombocytopenia, possibly representing an example of multiple autoimmune syndrome type 3. All abnormalities resolved and the patient was alive nearly 3 years later, maintained on physiological doses of prednisolone. This case serves as a reminder of the variable presentations of eunatraemic, eukalaemic hypoadrenocorticism and the potential for concurrent immune‐mediated disease.

An observational study to identify causative factors for not using hydroxychloroquine in systemic lupus erythematosus

Hydroxychloroquine (HCQ) use is indicated for patients with systemic lupus erythematosus (SLE). Nevertheless, reports discussing the reasons for not prescribing HCQ are limited. We identified the factors that interfere with HCQ use in patients with SLE. This observational, single-center study included data from 265 patients with SLE in 2019. The patients were categorized into groups with and without a history of HCQ use. Between these groups, clinical characteristics were compared using univariate analysis and logistic regression models. Among the 265 patients, 133 (50.2%) had a history of HCQ use. Univariate analysis identified older age; longer disease duration; lower prednisolone dose, clinical SLE disease activity index 2000, and estimated glomerular filtration rate; higher C3 level; and lower anti-double-stranded DNA antibody concentration as HCQ non-use-related variables. Logistic regression models identified a positive association between HCQ non-use and longer disease duration (odds ratio [OR] 1.08), prednisolone dose ≤ 7.5 mg/day (OR 4.03), C3 level ≥ 73 mg/dL (OR 2.15), and attending physician having graduated > 10 years prior (OR 3.19). In conclusion, a longer disease duration, lower prednisolone dose, higher C3 level, and longer time since attending physicians’ graduation correlated with HCQ non-use. Physicians and patients should be educated to facilitate HCQ use despite these factors.

PD-L1 expression in keratinocyte and infiltration of CD4 + T lymphocyte can predict a severe type of erythema multiforme major induced by the anti-PD-1 antibody, pembrolizumab

Skin toxicity is the most common adverse event of treatment with immune check point inhibitors. Among them, erythema multiforme is a rare occurrence with a frequency of 4%, with most of the cases developing grade 1/2 disease. We experienced high grade erythema multiforme major developing with pembrolizumab treatment for anal canal cancer with extensive skin metastases. Steroid ointment was ineffective, and the skin lesions with blisters expanded to > 45% of the body surface area. The patient was at risk for symptom aggravation, and a pulse therapy with methylprednisolone and increasing the dose of oral prednisolone (1 mg/kg) were started. The skin lesions improved in 1.8 months. Unless urgent and appropriate treatments such as high dose steroid administration were conducted, the skin toxicities could not be controlled. The presence of CD4+ T cells and PD-L1+ keratinocytes in the skin biopsy might be a predictive marker of erythema multiforme major resistant to standard steroid treatment.

Predictors of medications-free and long-term remission in anti-neutrophil cytoplasmic antibody-associated vasculitis: Real-world evidence.

In this study, we report the outcomes of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in daily practice based on Connective Tissue Diseases Research Center-Vasculitis Registry (CTDRC-VR) data. Patients were included if they were 18 years or older, had a diagnosis of the groups of AAV based on 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis and microscopic polyangiitis, and were followed for a period longer than 2 years or were died. Complete clinical remission was defined as granulomatosis with polyangiitis (BVAS/GPA) of 0. Sustained remission was defined as a complete clinical remission for at least six months and tapering prednisolone dose to ≤ 7.5 mg/d. Long-term remission was defined as complete clinical remission for ≥ 5 years and tapering prednisolone dose to ≤ 7.5 mg/d. Medications-free remission was defined as complete clinical remission and discontinuation of glucocorticoids, cytotoxic medications and biologics. Sixty patients with AAV were enrolled in this study. Sustained and long-term remission were developed in 91.7 and 72.1 percent of patients, respectively. Relapse was developed in 27 (45%) patients. Medications-free remission was developed in 23 (33.3%) patients. Vasculitis induced damage was developed in 40 (66.7%) patients. Patients with damage had significantly lower age and higher BVAS at the baseline. Upper airway and renal involvement, and non-adherence in patients with damage was significantly more common. Induction therapy leads to long-term and medications-free remission in 72% and 38% of patients with AAV, respectively.

Effective efgartigimod treatment for severe thymoma‐associated myasthenia gravis experiencing myasthenic crisis: A case report

AbstractWe report the long‐term treatment of a 38‐year‐old man with generalized thymoma‐associated myasthenia gravis who was treated with efgartigimod. He received chemotherapy and oral prednisolone before the thymectomy. However, after initial improvement in symptoms, he deteriorated and needed continuous noninvasive positive‐pressure ventilation, with the highest Myasthenia Gravis‐Activities of Daily Living score of 14. Immunosuppressive therapy and plasma exchange showed limited benefit, and the anti‐acetylcholine receptor antibody titer peaked to 130 nmol/L. Seven cycles of efgartigimod treatment were administered, which improved the symptoms, achieving a Myasthenia Gravis‐Actvities of Daily Living score of 0. The anti‐acetylcholine receptor antibody titer fluctuated until the end of the fifth cycle, thereafter, plateauing at 22 nmol/L. These findings allowed the prednisolone dose to be tapered and the interval between the treatment cycles to be increased.

Tocilizumab discontinuation after remission achievement in patients with adult-onset Still's disease.

Tocilizumab, an IL-6 inhibitor, has been proven effective in patients with adult-onset Still's disease (AOSD). This study aimed to clarify whether tocilizumab can be discontinued after achieving remission and to identify factors relevant to its successful discontinuation. Consecutive patients with AOSD diagnosed according to Yamaguchi's criteria from April 2012 to July 2022, who were treated with tocilizumab, were retrospectively reviewed. Forty-eight patients with AOSD treated with intravenous tocilizumab, with sufficient information, were included. Thirty-eight patients (79.2%) achieved remission after 6 months of tocilizumab treatment, 12 of whom discontinued tocilizumab during remission. Within 1 year after tocilizumab discontinuation, six patients (50.0%) recurred at a mean of 5.5 months, while the other six (50.0%) remained in remission. Between the non-recurrence and recurrence groups, no difference was found in disease activity at tocilizumab discontinuation (systemic feature score, p = 0.24; ferritin, p = 0.46). While the duration of tocilizumab use was not different (p = 0.32), the interval of tocilizumab administration at tocilizumab discontinuation in the recurrence group was 21 (14-35) days, which tended to be shorter than 35 (28-53) days in the non-recurrence group (p = 0.08). Patients with prednisolone dose < 7 mg/day at last tocilizumab treatment had fewer recurrences than those without (p = 0.001). After recurrence, tocilizumab was resumed in half of the patients, resulting in successful disease control. The recurrence rate after tocilizumab discontinuation was 50% in 1 year. Patients who remained in remission with a longer interval of tocilizumab administration and lower prednisolone dose were likely to succeed in the withdrawal of tocilizumab.

Rituximab – An effective first line treatment in pemphigus vulgaris patients

: Rituximab is a humanized chimeric anti-CD20 monoclonal antibody that quickly lowers blood serum levels of anti-desmoglein autoantibodies, causing pemphigus lesions to heal. To evaluate the efficacy of Rituximab in treating pemphigus vulgaris. : We identified and included 14 patients (3 males and 11 females), in the age group of 14 years to 67 years, of severe pemphigus vulgaris from 2016 to 2022 treated at our institution with rituximab infusion in our retrospective case study. Baseline anti-desmoglein antibodies 1 and 3 levels were elevated in all the patients. 12 of these patients received rituximab infusion as first-line treatment. 11(out of 14) patients received two doses of rituximab (1 gram in each) as intravenous infusions, as per the rheumatoid arthritis protocol; whereas, 3 (out of 14) received a single dose of intravenous rituximab infusion (1 gram). These patients were kept on maintenance dose of prednisolone/ mycophenolate mofetil post rituximab infusion. : 14 patients of pemphigus vulgaris were enrolled in the study. The follow-up period ranged from 2 months to 6 years. All 14 patients responded well to therapy. No serious adverse events were observed. 10 (71.4%) of the 14 patients exhibited complete remission; whereas, 4 (28.6%) showed partial remission. Those with partial remission experienced relapse but the subsequent episode was less severe than the first episode. However, 2 patients who were non-responders to high doses of systemic steroids expired. : For treating patients with pemphigus vulgaris, rituximab infusion was found to be safe and effective. Hence, it should be considered as first-line for management of pemphigus vulgaris.

Outcomes of polymyalgia rheumatica in real-world practice: a longitudinal cohort study.

Background: Polymyalgia rheumatica (PMR) is an inflammatory condition closely linked with giant cell arteritis, which is a large vessel vasculitis. To provide real-world evidence on PMR outcomes and their determinants, we conducted a longitudinal study focusing on symptom relief and acute phase reactant normalization. Methods: We followed patients with PMR who were registered in Tabriz University of Medical Sciences Vasculitis Registry (TUOMS-VR) until February 2023. We measured sustained remission (primary outcome) and secondary outcomes including glucocorticoids (GCs)-free remission, medication-free remission, relapse rate and disease-induced damage. Results: We identified eighty-one patients with PMR and followed them for a median time of 57 months. In a median duration of 3 weeks, 98.8% of patients achieved symptom control, with 86.4% achieving sustained remission in a median duration of 9 weeks. Sustained remission was more common in non-smokers and adherent to therapy patients. Relapse occurred in 22.1% of patients, primarily due to non-adherence. Medication-free remission was observed in 30.9% of patients, especially among females and those with an initial prednisolone dose > 15 mg/d. Damage occurred in 42.0% of patients. Conclusion: Although sustained remission in PMR is not an unattainable goal in daily practice and most patients are in remission at the last visit, two-thirds of patients require long-term treatment.

Clinical and immunological predictors of post-rituximab paradoxical pemphigus flare: A prospective cohort study.

Background Paradoxical flare of pemphigus following rituximab infusion has been reported previously, however, its incidence or risk factors have not been studied in detail. Objectives To evaluate the clinical and immunological predictors associated with post-rituximab paradoxical pemphigus flare. Materials and Methods This was a prospective cohort study including adult patients with pemphigus vulgaris or foliaceus who were treated with rituximab. Patients were administered 1000 mg of intravenous rituximab on days 0 and 14 (Rheumatoid arthritis (RA) protocol), with or without oral prednisolone and/or conventional immunosuppressive agents. Baseline clinical and immunological predictors of post-rituximab pemphigus flares were assessed. Results Fifty patients (mean age 40.44 ± 12.36 years) with a mean pemphigus disease area index (PDAI) score of 27.8 ± 15.48 were administered rituximab. Post-rituximab flare occurred in 10 (20%) patients after a mean of 14.1 ± 4.33 days after the first rituximab infusion. The mean baseline PDAI score (36.4 ± 11.7 vs. 25.6 ± 15.7, P = 0.02) and serum anti-Dsg1 levels (1216.8 ± 850.1 vs. 592 ± 562.12 RU/mL, P = 0.03) were statistically significantly higher in patients experiencing a flare. Using ROC-curve analysis, a PDAI score of 328 (OR 8.3, 95% CI 1.5-44.7) was 80% sensitive and 67.5% specific in predicting post-rituximab flare, while serum anti-Dsg1 level of 31137.78 RU/ml had a sensitivity of 60% and specificity of 85%. There was no significant difference in terms of affected body surface area, type of pemphigus, starting prednisolone dose, oral immunosuppressive adjuvant, serum anti-Dsg3, serum anti-AchRM3, and peripheral CD19+ B cell population. Limitations Our study is limited by a relatively small sample size. Immunological factors were not evaluated at the time of pemphigus flare. Though these unexpected pemphigus flares are likely to be associated with rituximab infusion, the possibility of spontaneous disease exacerbation cannot be entirely excluded. Conclusions Patients with more severe pemphigus or high serum anti-Dsg1 are at risk of post-rituximab paradoxical flare, and may benefit from rituximab administration under close monitoring.

Risk factors for fragility fractures in patients with immunobullous diseases on long-term systemic glucocorticoids

Dear Editor, Systemic glucocorticoids remain a cornerstone of therapy in immunobullous diseases. Yet little is known about glucocorticoid induced osteoporosis (GIOP) in patients with immunobullous diseases. We performed a retrospective review of medical records at an immunodermatology clinic in a Singaporean tertiary centre. Inclusion criteria consisted of patients with a newly diagnosed immunobullous condition between January 2011 and October 2017, who were on long-term (&gt;= 3 months) systemic glucocorticoids at a minimum daily dose of prednisolone 15mg. [...]

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.

Impact of chronic oral glucocorticoid treatment on mortality in patients with COVID-19: analysis of a population-based cohort

ObjectivesWhile glucocorticoid (GC) treatment initiated for COVID-19 reduces mortality, it is unclear whether GC treatment prior to COVID-19 affects mortality. Long-term GC use raises infection and thromboembolic risks. We investigated...

Comparison of the effects of two different styles of orally prescribing prednisolone on postoperative sequelae of surgical extraction of an impacted mandibular third molar: a single-blind randomized study.

Surgical intervention for removal of an impacted third molar can lead to significant pain and swelling. Corticosteroids show promise for mitigating postoperative sequelae across various surgical contexts. The use of corticosteroids following minor oral surgery, though controversial, has already been proven effective. However, little research has explored peroral prescription of corticosteroids despite its convenience for outpatients and for non-surgeons like implantologists and periodontists and others who don't have access to needle injections. The aim of this study was to address a void in the literature by comparing the effects of two styles of preoral administration of prednisolone after surgical removal of the mandibular third molar and to determine which style minimizes postoperative sequelae. A randomized, split-mouth clinical study was conducted to investigate the efficacy of two different styles of preoral prednisolone in mitigating postoperative sequelae following surgical extraction of impacted mandibular third molars. Fifteen participants were enrolled in the study. Random selection was used to determine the prescription style for the right and left mandibular arch. Group A included those who received a single dose of prednisolone 25 mg, while group B received prednisolone 5 mg postoperatively for a period of three days (5 mg three times/day on the first postoperative day, 5 mg twice/day on the second postoperative day; 5 mg once/day on the third postoperative day). There was a significant difference in the distance between the corner of the mouth and tragus, which decreased with the time interval with respect to group B when compared to group A. The present study showed that a three-day tapered dose of prednisolone postoperatively was more effective in reducing post-extraction sequelae than a single-dose regimen.

Risk of flare and damage accrual after tapering glucocorticoids in modified serologically active clinically quiescent patients with systemic lupus erythematosus: a multinational observational cohort study

ObjectivesTo assess the risk of flare and damage accrual after tapering glucocorticoids (GCs) in modified serologically active clinically quiescent (mSACQ) patients with systemic lupus erythematosus (SLE).MethodsData from a 12-country longitudinal...