Doses Of Glucocorticoids Research Articles

Efficacy of anifrolumab on systemic lupus erythematosus patients with serological manifestations: A post hoc analysis of the Japan subgroup of the TULIP-2 Trial.

To describe the efficacy of anifrolumab vs. placebo in Japanese systemic lupus erythematosus (SLE) patients with low complement (C3 or C4) and/or who are positive for anti-double stranded DNA (anti-dsDNA) antibodies. This was a descriptive post hoc analysis of Japanese SLE patients with serological manifestations in the TULIP-2 trial who received either anifrolumab or placebo. Of the 43 patients enrolled, 79.2% (19/24) and 73.7% (14/19) had low C3, low C4, and/or were positive for anti-dsDNA antibodies at baseline in the anifrolumab and placebo groups, respectively. At week 52, 52.6% (10/19) and 7.1% (1/14) patients in the anifrolumab and placebo groups, respectively, achieved a British Isles Lupus Assessment Group-based Composite Lupus Assessment (BICLA) response. The proportion of patients who tapered their glucocorticoid (GC) dose throughout the study, without increasing their dose, or who sustained baseline GC doses of ≤7.5 mg/day was numerically higher in the anifrolumab group (78.9% [15/19]) than in the placebo group (50.0% [7/14]). In line with the clinical profile of anifrolumab in the TULIP-2 study, the efficacy of anifrolumab was shown in Japanese SLE patients with serological manifestations achieving a BICLA response, and with tapered GC dose or sustained GC doses of ≤7.5 mg/day.

Characteristics of patients with polymyalgia rheumatica based on glucocorticoid dose in Japan: A cohort study using routinely collected health data.

This study aimed to describe the characteristics, inflammatory markers as surrogates for disease activity, and treatment of patients with polymyalgia rheumatica (PMR) in Japan. This cohort study analysed the data of 373 patients with PMR retrieved from an electronic medical records database in Japan. Patients were classified into quartiles, based on the daily glucocorticoid dose over the initial 90 days of treatment (Q1-Q4). The low glucocorticoid dose group (Q1) had more patients aged ≥90 years (11.7%), and a higher prevalence of comorbidities. At 52 weeks, glucocorticoid-free remission was achieved in 10% of patients, and higher C-reactive protein levels were observed during the follow-up period. In contrast, the high glucocorticoid dose group (Q4) exhibited a slower decline in C-reactive protein levels and more events of increased glucocorticoid dose compared to Q1-Q3. The introduction of methotrexate was low, with 4.3% to 7.3% of patients. The incidence of osteoporosis and diabetes was higher in patients <75 years, whereas the incidence of hypertension was higher in patients ≥75 years. Patients refractory to high glucocorticoid doses were identified. Patients receiving inadequately low doses of glucocorticoids were older with more comorbidities. This study highlights the unmet medical needs for PMR.

Risk factors for steroid-induced osteonecrosis of the femoral head in children with immune kidney disease

Abstract Objective To investigate the risk factors for steroid-induced osteonecrosis of the femoral head (SONFH) after glucocorticoid (GC) therapy in children with immune kidney diseases. Methods This retrospective study included patients (1–18years) diagnosed with immune kidney disease treated with GCs from January 2012 to July 2022 in our hospital. Data, such as sex, age and body weight at the first GC treatment, and the GC dose, were collected. Patients were divided into SONFH and non-SONFH groups based on the occurrence of SONFH. According to the International Classification of Diseases (ICD-10), the disease diagnosis was searched on the clinical data webpage of our hospital. The descriptive statistics and binary logistic regression analyses were performed. A nomogram was constructed to predict the risk of SONFH in children with immune kidney disease after GC treatment. The predictive ability of the nomogram was evaluated using the concordance index and calibration plots. Results The SONFH and non-SONFH groups included 18 and 90 patients, respectively. Age at the first GC treatment, the six-month cumulative (oral [po.] plus intravenous [iv.]) GC dose, the total cumulative prednisone (po.) dose, total cumulative methylprednisolone (iv.) dose for pulse therapy, and total cumulative GC (po. plus iv.) dose significantly differed between the two groups. Age at the initial GC treatment, the total cumulative prednisone (po.) dose, and the total cumulative methylprednisolone (iv.) dose for pulse therapy were independent risk factors for SONFH in the multivariate analysis. Conclusions This study identified specific risk factors for developing SONFH during GC treatment. Thus, children with immune-related kidney disease undergoing GC treatment should be closely monitored based on these risk factors, which aims to avoid the irreversible damage to the femoral head.

Efficacy of adalimumab in pediatric non-infectious uveitis with and without anterior segment inflammation

BackgroundThis study aimed to identify the clinical characteristics of cases that is related to the response rate of adalimumab (ADA) treatment.MethodsA retrospective review of medical records was conducted for pediatric patients with non-infectious uveitis undergoing ADA treatment for a minimum of six months. The patients were stratified into two groups: those with anterior segment inflammation (ASI+) and those without anterior segment inflammation (ASI-). The primary outcome was treatment success rate. Secondary outcomes including best-corrected visual acuity (BCVA), inflammation parameters [anterior chamber cell (ACC), vitreous haze (VH)], retinal nerve fiber layer (RNFL) thickness, fluorescein angiography (FA) scores, as well as systemic immunosuppression therapy (IMT) and glucocorticoid load, were assessed.ResultsThe study included 59 patients (111 eyes), with 44 patients (83 eyes, 74.58%) falling into the ASI + group and 15 patients (28 eyes, 25.42%) in the ASI- group. The treatment success rate in the ASI + group was significantly higher than in ASI- patients (93.18% vs. 20%, p < 0.001). Following 6-month of ADA treatment in the ASI + group, there was a significant improvement in BCVA (p < 0.001), inflammation parameters (p < 0.001), reduced RNFL thickness and reduced FA scores (p < 0.001). Conversely, no significant differences were observed in BCVA, inflammation parameters, RNFL thickness and FA scores in the ASI- group. There was also a significant decrease in systemic IMT and glucocorticoid dosing, comparing baseline to the 6-month follow-up visit in both the ASI+ (p < 0.001) and ASI- groups (p < 0.05). Adverse events observed during the study period included abdominal pain, skin erythema, articular symptoms and respiratory infections.ConclusionADA demonstrates superior efficacy in the treating pediatric non-infectious uveitis with ASI + compared to ASI-.

Treatment and Prevention of Adrenal Crisis and Family Education

Adrenal crisis is a life threatening complication of adrenal insufficiency (AI). Its treatment is urgent and parenteral hydrocortisone (HC) should be given at 10-15 times physiological doses in this situation. If HC is not available, alternatively prednisolone or methyl prednisolone may be used. In cases where peripheral venous access cannot be achieved quickly, intramuscular (IM) administration should be performed without delay. Fluid deficit, hypoglycemia, hyponatremia and hyperkalemia should be evaluated and corrected. Stressful conditions, such as physical stress, accidents, injuries, surgical interventions and anesthesia increase the need for cortisol and may lead the development of adrenal crisis. In order to prevent adrenal crisis, glucocorticoid dose should be increased according to the magnitude and severity of the stress situation as described in this review. Patients’ and/or their families’ education may improve the management of AI and reduce the frequency of adrenal crisis and/or mortality. They should be trained about conditions leading to adrenal crisis, how to increase the glucocorticoid dose in stress situations, recognizing signs of adrenal crisis and using IM HC if it is needed. All patients should be encouraged to carry a card/information sheet/medical alert bracelet or necklace indicating the diagnosis of AI and need for HC administration. It is useful for patients and parents to have an emergency glucocorticoid injection kit and to receive self-injection training.

Long-term Complications and Testicular Adrenal Rest Tumors in Congenital Adrenal Hyperplasia

Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency accounts for approximately 95% of all CAH cases and is one of the most common inborn errors of metabolism. While glucocorticoid therapy has significantly improved patient outcomes, the focus has shifted towards managing the long-term effects. Numerous adverse outcomes have been associated with CAH, including those resulting from supraphysiological doses of glucocorticoid and mineralocorticoid replacement, excessive adrenal androgen secretion, and elevated levels of steroid precursors and adrenocorticotropic hormone. Despite advances in treatment, long-term complications persist due to the inability to replicate physiological hormone secretion fully. In this review, we explore critical aspects of managing CAH, focusing on cardiometabolic health, bone integrity, fertility, and other significant long-term consequences, informed by the latest literature.

Treatment and Follow-up of Non-stress Adrenal Insufficiency

Adrenal insufficiency (AI) is defined as the inability of the adrenal cortex to produce adequate amounts of glucocorticoids and/or mineralocorticoids. As these hormones have important roles in water-salt balance and energy homeostasis, AI is a serious and potentially life-threatening condition. Glucocorticoid replacement therapy is vital in all cases of AI. In children with primary AI (PAI), it is recommended to start glucocorticoid replacement therapy with three or four doses of hydrocortisone and adjust according to individual need. Long-acting glucocorticoids such as prednisolone and dexamethasone are not recommended in children with AI. Mineralocorticoid and salt replacement therapy is also necessary in PAI with aldosterone deficiency. In childhood, it is recommended that patients are monitored at least every three to four months with clinical evaluation including weight gain, growth rate, blood pressure and general well-being of the patient. To prevent adrenal crisis in patients with PAI, glucocorticoid dose adjustment is recommended to patients and/or their families according to the magnitude and severity of the stress situation. This education should include recognition of conditions leading to adrenal crisis, signs of adrenal crisis and how to respond to an impending adrenal crisis. With long-term use of glucocorticoids, the lowest possible dose should be maintained to control the disease to avoid possible side effects. Here, members of the ‘Adrenal Working Group’ of ‘The Turkish Society for Pediatric Endocrinology and Diabetes’ present an evidence-based review with good practice points and recommendations for the diagnosis and follow-up of non-stress AI.

Baricitinib in early polymyalgia rheumatica (BACHELOR): a randomised, double-blind, placebo-controlled, parallel-group trial.

Moderate doses of glucocorticoids result in improvements in nearly all patients with polymyalgia rheumatica, but related adverse events are common in older individuals. We aimed to evaluate whether treatment with baricitinib (a Janus kinase 1/2 inhibitor) results in disease control without the use of oral glucocorticoids in people with recent-onset polymyalgia rheumatica. We conducted a randomised, double-blind, placebo-controlled, parallel-group trial at six expert centres in France. Participants with recent (<6 months) polymyalgia rheumatica naive to glucocorticoids and a C-reactive protein polymyalgia rheumatica activity score (CRP PMR-AS) of more than 17 were randomly assigned (1:1), with stratification by hospital, to receive either 4 mg baricitinib orally or placebo (with oral glucocorticoids as rescue treatment in the event of high disease activity) for 12 weeks, followed by 2 mg baricitinib or placebo for another 12 weeks. Subdeltoid glucocorticoid injections at week 1 and week 4 were permitted. Participants, investigators, outcome assessors, and sponsor personnel were masked to group assignments. The primary outcome was a CRP PMR-AS of 10 or less at week 12 without oral glucocorticoid use from week 1 to week 12, analysed in all randomly assigned participants who did not withdraw before first treatment administration. Participants were followed up for 36 weeks. An individual with lived experience of polymyalgia rheumatica was involved in the study design. The trial was registered on ClinicalTrials.gov, NCT04027101, and is complete. We assessed 39 individuals for eligibility between Dec 1, 2020, and Aug 30, 2023. 34 participants (22 women and 12 men) were randomly assigned; 18 participants were assigned to the baricitinib group and 16 participants were assigned to the placebo group. One person allocated to placebo withdrew before the first infusion and was not included in analyses. The primary endpoint was reached at week 12 by 14 (78%) of 18 participants in the baricitinib group and two (13%) of 15 participants in the placebo group (relative risk 5·8, 95% CI 3·2-10·6; crude p=0·0004; adjusted p<0·0001). The most common adverse events were musculoskeletal and connective tissue disorders (13 [72%] of 18 participants in the baricitinib group and four [25%] of 16 in the placebo group. There were no deaths and no major adverse cardiovascular events in either study group. This study suggests that, compared with placebo, individuals with polymyalgia rheumatica receiving 4 mg baricitinib are less likely to need oral glucocorticoids to have low disease activity at week 12 of treatment without any new safety signals. CHU Brest and Eli Lilly.

Effectiveness and safety of TNF-α inhibitors and il-6 pathway antagonists for refractory Takayasu arteritis

Standard therapy for Takayasu arteritis (TA) usually comprises glucocorticoids in combination with cytotoxic agents. However, despite ongoing therapy disease activity remains high in some patients. In these cases, biological therapy is prescribed.The aim – to evaluate the efficacy and safety of tumor necrosis factor α inhibitors (TNFi) and interleukin 6 (IL-6) pathway antagonists (anti-IL-6) therapy in TA patients refractory to conventional therapy.Subjects and methods. In this single center retrospective cohort study, we included 36 female adult patients aged from 20 to 57 years (54 courses of biological therapy) with TA, diagnosed in accordance with the ACR classification criteria (1990 and/or 2022) and/or Chapel Hill Consensus Conference (2012) definition. All patients were on biologics due to resistance to corticosteroids and cytotoxic agents.Results. A total of 54 courses of TNFi (n=29) and anti-IL-6 (n=25) inhibitors were analyzed. The median duration of biological therapy was 14 (9; 32) months, with a median duration of 14 (8; 32) months for TNFi and 13 (7; 32) months for anti-IL-6. The remission rate showed no significant difference between TNFi (79%) and anti-IL-6 (84%; p=0.736) treatment groups. The incidence of relapses was higher with TNFi, although the difference between groups did not reach statistical significance (36% and 14%, respectively; p=0.291). Relapse was established after 15 (10; 22) weeks for TNFi and after 22 (13; 31) weeks for anti-IL-6 (p=0.919). TNFi and anti-IL-6 had a comparable steroid-sparing effect: the median dose of GC in terms of prednisolone decreased from 13 to 8 mg (p=0.009), and from 15 to 8 mg (p=0.026), respectively. Factors associated with achieving TA remission were assessed using a logistic regression model. None of the parameters (age at the start of biological therapy, duration of disease before the start of biological therapy, class of biological therapy) were associated with achieving remission of TA. Adverse events (AEs) occurred in 22% of cases resulting in therapy discontinuation in 13% of patients.Conclusion. Our study demonstrated the high effectiveness of TNFi and anti-IL-6 in patients with refractory to conventional immunosuppressive therapy TA: remission was achieved in 80% of cases, allowing for a reduction in glucocorticoid dosage. The safety profile of TNFi and antiIL-6 was acceptable. However, AEs occurred slightly more frequently in the TNFi group.

Evaluation of the effectiveness of combined therapy with intravenous immunoglobulin and plasmapheresis in patients with steroid-resistant form of pemphigus based on the cytokine and chemokine profiles assessment

BACKGROUND: Pemphigus is a serious life-threatening disease characterized by the formation of IgG autoantibodies against the cell membranes, leading to the formation of intraepidermal blisters. AIM: To evaluate the effectiveness of combined therapy with intravenous immunoglobulin and plasma exchange for steroid-resistant patients with pemphigus based on the cytokine, chemokine and granulysin profiles investigation. MATERIALS AND METHODS: The group of patients receiving systemic glucocorticoid monotherapy (Group 1; control group) consisted of 26 patients with pemphigus vulgaris. The group of steroid-resistant patients (Group 2; main group) who received combined therapy with systemic glucocorticoid, intravenous immunoglobulin (IVIg), and plasmapheresis included 15 people. The presence of steroid resistance was assessed by Murrell consensus (2008). All pemphigus patients received the initial dose of systemic glucocorticoids of 80–100 mg/day with subsequent slow tapering, according to guidelines. The treatment protocol for combined therapy included four sessions of discrete plasma exchange per week every other day. Immediately after completion of the plasma exchange cycle, IVIg was added to the ongoing treatment, with a total dose of 2 g/kg per cycle. The levels of IL-4, IL-10, IL-15, TNF-α, chemokines CXCL8, CCL11, and granulysin were assessed via ELISA method. RESULTS: We observed some discrepancies in cytokine profiles in both groups of patients. In patients who received combined therapy, there was a statistically significant decrease in the levels of IL-4, IL-15, TNF-α compared to those in patients on systemic glucocorticoid monotherapy ― IL-4, IL-15 TNF-α (p 0.01). Notably, that the level of CCL11 in serum of steroid-resistant patients before the IVIg therapy was significantly higher (Me=51 pg/ml) compared to systemic glucocorticoid monotherapy group (Me=10 pg/ml; p 0.01). The level of granulysin after the treatment with IVIg and plasma exchange in group 2 was also significantly lower (Me=0 ng/ml) compared to the group of control (Me=2700 ng/ml respectively; p 0.01). CONCLUSION: We found a trend towards higher serum levels of IL-4, IL-15, and CCL11 in steroid-resistant pemphigus patients who received combined therapy with IVIg and plasma exchange compared to the control group. Moreover, these cytokines can be considered as the potential biomarkers for refractory disease course, and might be used as therapeutic targets in the future. It should be also noted that the prolonged remission of patients receiving combined therapy with systemic glucocorticoid, IVIg, and plasma exchange, was on average two years.

Optimal Glucocorticoid Therapy in Lupus Nephritis.

This review provides an in-depth analysis of glucocorticoid therapy for lupus nephritis (LN), a severe manifestation of systemic lupus erythematosus that affects up to 51.7% of patients. LN significantly increases the risk of mortality and progression to end-stage kidney disease. Glucocorticoids have been central to LN treatment for decades due to their anti-inflammatory properties, but optimal dosing strategies remain uncertain. The review discusses the historical evolution of glucocorticoid use, highlighting the shift from high-dose regimens to combined approaches with immunosuppressants and lower glucocorticoid doses to minimize adverse effects. Mechanistically, glucocorticoids exert effects through genomic and non-genomic pathways, modulating immune responses and metabolism. Long-term use is associated with risks such as infection, osteoporosis, hyperglycemia, and cardiovascular disease. The review examines different dosing strategies, including intravenous pulse therapy and oral regimens, and presents evidence of their efficacy and safety. It also explores alternative approaches, such as low-dose and glucocorticoid-free regimens, which show promise but require further study. The review concludes by emphasizing the need for future research to optimize glucocorticoid regimens, refine tapering protocols, and identify safer therapeutic combinations, as glucocorticoids remain a cornerstone in LN management despite their challenges.

Glucocorticoid dosing and implications for vaccination: Evolution of global definitions.

Despite widespread adoption of "high-dose" glucocorticoid definitions across international immunisation guidelines (i.e., prednisone-equivalent dosing >20 mg/day, or >2 mg/kg/day in children), the rationale remains unclear. Literature searches were performed through academic databases for this narrative review to identify relevant evidence regarding glucocorticoid dosing on vaccine responses and safety. In people receiving prednisone <7 mg/day, vaccine responses are maintained. In people on 'high-dose' glucocorticoids (>20 mg/day), antibody titres and seropositivity are reduced, whereas the impact of low- to medium-dose glucocorticoids (7 to 20 mg/day) on vaccine efficacy remains inconclusive. Due to inconsistent paediatric dosing regimens, data is insufficient to support a unified "high-dose" glucocorticoid threshold. Non-live vaccines are well tolerated in patients receiving glucocorticoids with rheumatic/inflammatory disorders, but enhanced reactogenicity after live vaccination may occur in those with severe immunodeficiencies. Clinicians should consider individual risk-benefit profiles, rather than following strict dosing thresholds, when curating immunisation programs for patients prescribed glucocorticoids.

Practical Management of ANCA-associated Vasculitis – A Clinician’s Perspective

Background: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis can be a life-threatening condition, characterised by necrotising inflammation of small blood vessels. Major organ involvement, most commonly kidney and lung disease, is associated with significant morbidity and mortality. Intensive early immunosuppressive therapy is the cornerstone of management and has transformed ANCA-associated vasculitis (AAV) into a chronic relapsing condition. Remission induction with tapering glucocorticoids in combination with cyclophosphamide or rituximab is the standard of care for severe disease. Avacopan, an oral C5aR1 antagonist, has been approved for remission induction and helps minimise glucocorticoid exposure. Plasma exchange should be considered for severe kidney or life-threatening disease. Lower dose glucocorticoid induction regimens can be used without compromising remission rates. Remission maintenance therapy is recommended, and rituximab is usually first line over azathioprine. Mycophenolate mofetil (MMF) or methotrexate with low dose glucocorticoids are third line options. Immunosuppression associated infection risk remains a concern; both during acute presentations and in the long term; highlighted by the impact of rituximab on humoral immunity and vaccine response during the COVID-19 pandemic. There remains an ongoing need for therapies which induce rapid remission and optimise kidney recovery whilst minimising infection risk. Clinical trials are evaluating newer therapeutic options. Due to increasing treatment options, management should be individualised, balancing effective immunosuppression against co-morbidities and frailty. Summary: This review focuses on the treatment decision pathways for clinicians and patients in the management of severe AAV (granulomatosis with polyangiitis and microscopic polyangiitis). Key clinical trials, predictors of outcome, novel therapeutics and practical steps to mitigate infection risk are discussed. Key messages: Immunosuppression regimens have been refined due to emerging evidence from clinical trials. Rituximab, avacopan and reduced-dose glucocorticoid schedules have been the focus of recent studies. Infections and immunosuppression-induced immunodeficiency must be considered when determining individualised treatment.

Fractures of vertebrae and peripheral bones in patients with rheumatoid arthritis (based on long-term observation)

Introduction. In rheumatoid arthritis (RA), fractures occur on average 2–3 times more often than in the general population. Data on the incidence of vertebral fractures in RA is controversial and depends on the detection method. Aim. To determine the incidence of vertebral and peripheral bone fractures in patients with RA during long-term prospective follow-up. Materials and methods. A prospective multi-year cohort non-interventional study included 120 women with RA (mean age at enrollment 54.3±8.9 years), with a follow-up of 9.5±1.9 years. Initially and then repeatedly, a clinical, laboratory and radiological examination was performed: X-ray morphometry of the spine according to the Genant method, X-ray densitometry of the lumbar spine (LI-LIV) and femoral neck. Results. During the follow-up period, there were 104 low-energy fractures in 64 (53%) patients: 69 (66%) vertebral fractures and 35 (34%) peripheral fractures. Two or more fractures occurred in 25 (39%) subjects. In 30 (25%) patients, 52 fractures occurred repeatedly. Among peripheral fractures, the most frequent localization was fractures of the distal forearm and lower leg bones. Patients with fractures during the follow-up period were also more likely to have fractures before enrollment in the study, had an initially longer duration of RA, a mean daily dose, cumulative dose, and duration of glucocorticoid administration, and a lower bone mineral density in the main parts of the skeleton, determined by densitometry. There was no effect of RA activity on DAS-28, rheumatoid factor positivity, or antibodies to cyclic citrullinated peptide on fractures. Conclusion. More than half of the patients had low-energy fractures during the observation period, the most common being fractures of the vertebrae, distal forearm, and lower leg bones; a high frequency of repeated fractures was reported. The analysis of risk factors showed that a long duration of RA, a mean daily dose, cumulative dose, and prolonged use of glucocorticoids, a history of low-energy fractures, and low bone mineral density were associated with the occurrence of fractures in patients with RA.

Characterizing Protracted Febrile Myalgia: Fasciitis and Vasculitis of the Fascia and Muscle as Novel Histopathological Features

Background: Protracted febrile myalgia (PFM) is a rare but severe form of myalgia mainly occurring in pediatric patients with familial Mediterranean fever (FMF). PFM imaging and histopathological data remain scarce. Objectives: A comprehensive clinical, imaging, and histopathological characterization of PFM was performed by retrospectively analyzing a reference center cohort of adult patients with FMF and myalgia, and by a PubMed search of well-described cases with PFM. Results: Among 56 adults with FMF from our center, 32 (57.1%) experienced myalgia, which was generalized in 21 (37.5%) and affected lower limbs in 11 (19.6%) subjects. One (1.8%) patient suffered PFM, mainly affecting calves and Achilles tendons. From our patient’s detailed information and the data from 123 PFM cases reported in the literature, PFM was characterized as usually presenting with fever and severe generalized myalgia, with occasional involvement of lower legs and calves. It is mainly associated (in &gt;90% of cases) with the pathogenic mutation M694V in the MEFV gene. Raised acute phase reactants and normal creatine kinase levels are constant. High glucocorticoid doses are useful in most patients, and sustained colchicine treatment protects from PFM recurrences. MRI may identify a variable degree of muscle inflammatory changes, especially subfascial and myofascial lesions with extension to tendinous structures. PFM histopathology is characterized by T-cell rich inflammatory infiltrates and vasculitis mainly involving the fasciae and myofascial areas, with a lower muscle extent. Conclusions: PFM can occur in children and adults and appears to be clinically manifested as fasciitis/tendinitis caused by a vasculitis of the fasciae rather than a major muscle vasculitis.

Glucocorticoid treatment for primary and secondary central nervous system vasculitis

Although the first-line treatment of central nervous system vasculitis (CNSV) is glucocorticoids (GC), the duration and dose of GC is still unknown. Although CNSVs seem to be a disease limited to a single area, they are a heterogeneous group of diseases clinically, in terms of involvement pattern and course. Due to the highly heterogeneous nature of the disease, patient-specific determination of the initial dose of GC is essential. Considering the disease courses, it may be more appropriate to consider 1mg/kg oral GC treatment if small/distal vessels are involved and intravenous bolus GC treatment if large/proximal vessels are involved in primary-CNSVs. In secondary-CNSVs (Anca-associated vasculitis, Behçet’s syndrome, Systemic Lupus Erythematosus) intravenous high dose GC treatment is recommended.

Rational use of glucocorticoids during pregnancy in patients with systemic vasculitis

Glucocorticoids (GC) are important in fetoplacental development and GC production increases in pregnant women.GCs can be used for various reasons during pregnancy, but there are some things to keep in mind.In patients with systemic vasculitis, GC exposure may be associated with complications such as low birth weight and preterm birth. When planning pregnancy in patients with systemic vasculitis, the aim should be to control disease activity with the lowest possible dose of GC. Pregnant women who are required to use GC should also be monitored more closely for gestational diabetes and hypertension.

Differences in glucocorticoid use in childhood vasculitis

This review aimed to explore the differences in glucocorticoid use across various subtypes of childhood vasculitis, focusing on their effectiveness, potential side effects, and tapering strategies to minimize toxicity. A comprehensive review was conducted to evaluate the clinical applications of glucocorticoids in pediatric vasculitis subtypes. Recommendations from recent studies and guidelines were assessed, focusing on glucocorticoid protocols for conditions such as IgA vasculitis, Kawasaki disease, polyarteritisnodosa, ANCA-associated vasculitis, and Takayasu arteritis. Glucocorticoid tapering strategies and toxicity indices, such as the Pediatric Glucocorticoid Toxicity Index (pGTI), were also analyzed. Glucocorticoid use varied across vasculitis subtypes. For IgA vasculitis, mild cases were managed with oral prednisolone, while severe nephritis required intravenous methylprednisolone and immunosuppressants. In Kawasaki disease, corticosteroids were used for refractory cases alongside IVIG and aspirin. Severe polyarteritis nodosa cases showed better outcomes with cyclophosphamide and high-dose glucocorticoids, whereas non-severe cases benefitted from low-dose glucocorticoids and NSAIDs. ANCA-associated vasculitis studies reported no significant correlation between glucocorticoid dose and outcomes, although side effects were dose-dependent. In Takayasu arteritis, children generally received lower doses than adults, based on adult treatment guidelines. The pGTI was highlighted as a valuable tool to monitor and assess glucocorticoid toxicity in pediatric patients. Glucocorticoids remain a cornerstone of treatment in pediatric vasculitis, but their use must be carefully tailored to balance efficacy and toxicity. Early tapering and transitioning to alternative therapies, when feasible, are critical to minimizing adverse effects.

Glucocorticoid doses according to tissue/organ involvement in ANCA-associated vasculitis

Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides (AAV) are a group of disorders that include granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), renal-limited vasculitis, and eosinophilic granulomatosis with polyangiitis (EGPA). Infections and cardiovascular diseases are the main causes of death in patients with AAV. New treatment regimens with low-dose glucocorticoidsare proposed to reduce the frequency of side effects. Studies related to the use of glucocorticoids in AAV patients were searched in the literature and results were summarized. Low-dose steroid protocols are not inferior to standard dose treatment and have fewer side effects. Although their effectiveness appears to be similar and their side effects appear to be less, the long-term results of low-dose regimens should also be evaluated.New studies are needed for alternative treatment regimens.

Management of glucocorticoid-induced diabetes

Glucocorticoid-induced diabetes (GID) is a frequent metabolic complication of glucocorticoid therapy. It results from both insulin resistance and impaired insulin secretion, exacerbated by glucocorticoid use. Despite its prevalence, consensus guidelines on screening and management remain limited. GID affects approximately one in five patients receiving long-term glucocorticoid therapy. Risk factors include older age, high BMI, prediabetes, ethnicity, and high-dose systemic glucocorticoids. All patients initiated on moderate to high doses of glucocorticoids should be assessed for GID risk factors and closely monitored for the development of hyperglycemia and diabetes. In addition, glucocorticoid therapy can significantly exacerbate hyperglycemia in individuals with pre-existing diabetes, and stringent glucose monitoring is crucial. Treatment should be tailored to individual patient. Oral anti-diabetics such as metformin and sulfonylureas might be used in selected patients with mild GID. However, insulin is the primary treatment for severe hyperglycemia. Early detection and individualized management strategies are critical to mitigate GID’s impact. Further research is needed to develop consensus guidelines and optimize treatment approaches.