Intraarticular corticosteroid injections in pediatric rheumatology: insights from specialists.

Juvenile idiopathic arthritis (JIA) refers to various types of arthritis appearing before age 16, categorized into seven subtypes by ILAR. Treatments target disease control, growth support, and quality of life, utilizing NSAIDs, DMARDs, and intraarticular corticosteroid injections (IACIs). Despite IACIs' efficacy for oligoarticular JIA, their usage and techniques vary due to anecdotal evidence. This study compares IACI strategies among pediatric rheumatologists in Turkey and India as part of a PReS Sister Center activity. A cross-sectional survey via Google Forms gathered IACI practice data from pediatric rheumatologists in Turkey and India. The 33-item questionnaire covered demographics, JIA subtypes treated with IACIs, preferred agents/dosages, injection sites, follow-up, complications, anesthesia, and post-IACI treatments. Seventy clinicians' responses were analyzed, with ethical approval from Gazi University's Ethics Committee. Seventy participants, with a mean age of 39.75 (±8.80) years responded, mostly clinical fellows (38.6%) at university hospitals (58.6%). All utilized IACIs, primarily for oligoarticular JIA (100%), with 20% exclusively using them for this subtype. Triamcinolone hexacetonide (TH) was preferred (74.3%), mainly targeting knee joints (15.7%). Initial side effect follow-up was 1-2 weeks post-IACI (65.7%), with ultrasound guidance used by 17.1%. Common complications included cutaneous hypopigmentation (38.6%) and subcutaneous atrophy (38.6%). Ketamine was the favored anesthesia (44.2%). Post-IACI, 21.4% did not add treatment for new-onset oligoarticular JIA, while NSAIDs and methotrexate were common for polyarticular JIA (51.4%). CONCLUSION: IACIs are widely utilized in pediatric rheumatology for oligoarticular JIA, yet practice variability exists. Standardized protocols through randomized studies can enhance IACI efficacy and patient outcomes. WHAT IS KNOWN: • Intraarticular corticosteroid injections (IACIs) are a widely utilized and effective treatment modality in managing oligoarticular and polyarticular juvenile idiopathic arthritis (JIA), offering rapid symptom relief and the potential to prevent long-term joint deformities. • Despite their widespread use, there is significant variability in the indications, techniques, and anesthetic methods employed for IACI administration among pediatric rheumatologists, and much of the supporting evidence remains anecdotal. WHAT IS NEW: • This study highlights the diverse clinical practices and preferences regarding IACI use in pediatric rheumatology across two different countries, revealing considerable variations in the use of ultrasound guidance, anesthetic approaches, and corticosteroid formulations. • The findings underscore the need for standardized treatment protocols and further research to optimize IACI procedures, aiming to reduce variability and improve outcomes in the management of JIA.

Clinical Associations of E148Q Heterozygosity: What to Expect From E148Q?

OBJECTIVE: The exact effects of MEFV variants on inflammation are still under investigation, and reports on variants of unknown significance, particularly the E148Q variant, have been conflicting. Therefore, this study aims to investigate patients exhibiting E148Q heterozygosity, focusing on diagnoses and disease courses to assist physicians in interpreting the variant. METHODS: Data of pediatric patients presenting to the Pediatric Rheumatology clinic between November 2016 and September 2023, exhibiting only E148Q heterozygosity in MEFV gene analysis, were extracted. Patients who were lost before 9 months of follow-up have been excluded to ensure the completion of initial diagnostic tests and evaluations. RESULTS: Among the 119 patients with E148Q variant, the diagnoses were as follows: healthy, 51.3%; IgA vasculitis, 10.1%; Familial Mediterranean Fever (FMF), 7.6%; Periodic fever, Aphtous stomatitis, Pharyngitis, Adenitis (PFAPA), 6.7%; and other diagnoses, 19.3%. IgA vasculitis patients experienced articular, gastrointestinal, and renal involvement at rates of 91.7%, 58.3%, and 16.7%, respectively. Complete response, partial response, and no response to colchicine were 37.5%, 12.5%, and 50%, respectively, in PFAPA patients. All FMF patients responded to colchicine treatment resulting in reduced mean FMF episode counts in 6 months from 3.22 ± 0.92 to 0.56 ± 0.52. CONCLUSIONS: The E148Q variant may amplify inflammation and modify disease courses. Patients with the E148Q variant experiencing typical FMF episodes should receive colchicine, but clinicians should exercise caution regarding alternative diagnoses. Additionally, the E148Q variant may increase acute phase reactants and disease severity in IgA vasculitis. However, to reach definitive conclusions on its treatment-modifying role in PFAPA, universal diagnosis and treatment response criteria should be adopted.

Etanercept-associated episcleritis: a pediatric case report of a paradoxical adverse reaction and review of the literature.

Scleritis is an inflammation of the episcleral and scleral tissues, characterized by injection in both superficial and deep episcleral vessels. When only episcleral tissue is involved, it is referred to as episcleritis. Episcleritis is mainly idiopathic but may be secondary to an underlying rheumatologic disease. Despite being rare, drug-associated episcleritis and scleritis should also be included in the differential diagnosis. Tumor necrosis factor-alpha (TNF-α) inhibitors are generally well-tolerated, but etanercept, in particular, has the potential to cause paradoxical adverse reactions including ocular inflammations, such as uveitis, scleritis, and ocular myositis. Etanercept differs in its mechanism of action from other TNF-α inhibitors as it acts as a decoy receptor, and this may partly explain the more frequently reported etanercept-associated ocular inflammation. Etanercept may also be ineffective in preventing ocular inflammation. However, the dechallenge and rechallenge phenomena have proven there is a causative link between etanercept and new-onset ocular inflammation. We report a case of a 15-year-old boy with enthesitis-related arthritis and familial Mediterranean fever who presented with episcleritis and blepharitis while receiving etanercept treatment and subsequently showed dechallenge and rechallenge reactions. Therefore, physicians should also be aware that episcleritis should be considered a paradoxical adverse reaction to etanercept and can occur in pediatric patients. We also reviewed the English literature to provide an overview and evaluate intervention options.

A 7-year-old boy with scurvy owing to coeliac disease.

ABBREVIATIONS: ESR: erythrocyte sedimentation rate; Hb: haemoglobin; HSP: Henoch-Schönlein purpura; WCC: white-cell count.

Juvenile idiopathic arthritis management: insights into the utilization of intra-articular corticosteroid injections.

BACKGROUND: Juvenile idiopathic arthritis (JIA) is a common chronic rheumatic disease in children, requiring careful management to reduce both short- and long-term morbidity. In this study, our objective was to assess the clinical features of patients diagnosed with JIA who received intra-articular corticosteroid injections (IACI). METHODS: In this retrospective study, we evaluated the clinical and laboratory characteristics of 225 JIA patients monitored from January 2012 to October 2023 at a tertiary care center. We focused on patients who underwent intra-articular corticosteroid injections (IACI) as part of their treatment. Triamcinolone hexacetonide (TH) was used due to its demonstrated safety and efficacy. RESULTS: Our analysis revealed that IACI, particularly utilizing TH, was a widely employed and effective adjunct therapy, contributing to rapid symptom relief and local disease control. Patients receiving IACI exhibited earlier symptom onset, younger age at diagnosis, longer follow-up durations, and higher cumulative treatment burden (p < 0.001, p < 0.001, p < 0.01, p < 0.001 respectively). Despite inconclusive acute-phase reactants, a higher frequency of ANA positivity and elevated initial lymphocyte counts were associated with increased IACI use (p < 0.001, p < 0.001 respectively). Importantly, on a joint basis, a high percentage of arthritis remission following IACI underscores its efficacy and favorable safety profile. CONCLUSIONS: Notably, the high percentage of arthritis remission achieved with intra-articular corticosteroid injections (IACI) on a joint-specific basis highlights its efficacy and favorable safety profile. A lymphocyte count exceeding 5000/mm3 at the time of diagnosis may serve as an early indicator for considering intra-articular steroid administration. These findings emphasize the need for nuanced and individualized treatment strategies in JIA management to optimize outcomes for affected children.

Adhesive small-bowel obstruction as a challenging complication of familial Mediterranean fever: A case-based review.

Familial Mediterranean fever (FMF) is the most common inherited autoinflammatory disorder, characterized by recurrent and self-limiting episodes of fever and serosal inflammation. Recurrent serositis may rarely lead to the formation of adhesions in the peritoneum, which may result in mechanical bowel obstruction. The symptoms, such as abdominal pain and vomiting, may mimic typical FMF attacks, resulting in misdiagnosis and severe morbidity, including strangulation and intestinal necrosis. Physicians are generally aware of other complications associated with FMF but reports on peritoneal adhesions and intestinal obstruction in English-language literature are inadequate to increase clinicians' awareness. Therefore, it is crucial to meticulously evaluate FMF patients presenting with abdominal pain and ileus because these symptoms could be due to adhesive small-bowel obstruction (ASBO). Furthermore, patients presenting with ASBO without a history of abdominal surgery should also be thoroughly evaluated, especially as it could be an initial presentation for an autoinflammatory disease. Herein, we present a pediatric case of FMF with the M694V homozygous mutation, complicated by ASBO while under colchicine treatment. Additionally, we provide a comprehensive review of the available literature on ASBO in FMF.