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A. Čengić

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Tarik Jarkoč, A. Čengić, V. Selmanović, Amila Hadžimuratović, E. Vukas, S. Užičanin, Zinka Huseinbegovic, Mirna Sarajlic

Background: Benign acute childhood myositis (BACM) is a rare complication of viral URTIs, usually occurring in winter. It is characterized by acute onset of bilateral calf pain and difficulty/refusal to walk. A prodromal phase precedes these manifestations, consisting of catarrhal and constitutional symptoms. These are associated with increased muscle-specific enzymes, usually normal inflammatory parameters, and leukopenia. Objective: Our study aimed to define the demographic, clinical, and laboratory characteristics of BACM patients and determine the etiology leading to their development. Methods: Medical charts for patients diagnosed with BACM from October to April 2023/2024 at the Pediatric Clinic Clinical Center University of Sarajevo, Department of Allergology, Rheumatology, and Immunology were reviewed retrospectively. Relevant medical information was collected for 20 patients. Statistical analysis was done in Microsoft Excel 2013. Results: Demographic analysis demonstrated male predominance (75%), with primarily school-aged children affected (median age 8.36). Most cases occurred in winter (60%). The majority of patients presented with bilateral calf pain (100%), difficulty walking (90%), and fever (100%). All cases demonstrated increased CK levels, with median values of 3779 U/L; a notable number had leukopenia (70%). The most commonly isolated pathogen was Influenza B virus (75%). Conclusion: BACM is relatively rare but presents acutely and leads to plenty of distress for both patients and their parents. A child with coryzal symptoms complicated by bilateral calf pain or difficulty/refusal to walk and an increase in CK levels should raise suspicion of BACM. The condition is self-limiting and usually resolves without complications.

AIM To determine whether demographic data, clinical features, and laboratory variables at disease onset can predict the response to methotrexate in juvenile idiopathic arthritis (JIA) patients. METHODS A cohort of 143 newly diagnosed JIA patients initially treated with methotrexate was enrolled in this study. Demographic, clinical, and laboratory parameters were analysed using univariate and multivariate logistic regression to identify predictors of response to methotrexate. The variables included erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), platelets, IgA, IgG, the number of active joints and age at disease onset. Treatment response was assessed at six months, with patients classified as responders (those who achieved clinically inactive disease according to the American College of Rheumatology - ACR criteria) or non-responders. RESULTS Poor response to methotrexate was associated with the number of active joints (p=0.0001; OR=2.7), baseline levels of CRP (p=0.044; OR=1.138), IgA (p=0.004; OR=2.159), and platelet count (p=0.01; OR=1.05). IgG level (P=0.236) did not correlate with the treatment response. CONCLUSION We identified widely available and clinically acceptable biomarkers that can be utilized as predictive indicators of response to methotrexate in JIA patients.

Juvenile idiopathic arthritis (JIA) is the most common chronic inflammatory autoimmune disease in childhood, significantly contributing to both short- and long-term disability. While certain human leukocyte antigen (HLA) class II alleles are known to be associated with specific subgroups of JIA, emerging evidence suggests a strong correlation between these alleles and treatment response. This study involved 143 JIA patients diagnosed according to International League of Associations for Rheumatology criteria. Each patient underwent HLA class II typing, including HLA-B27, as well as tests for rheumatoid factor (RF) and antinuclear antibodies (ANA). Comprehensive rheumatological assessments were conducted at diagnosis, with follow-ups at three and six months post-onset. After six months of methotrexate (MTX) treatment, patients were categorized as responders or non-responders. Responders achieved clinically inactive disease based on the American College of Rheumatology Provisional Criteria for Defining Clinical Inactive Disease and Clinical Remission. Non-responders, who did not reach clinically inactive disease after six months of treatment, required the addition of another non-biological disease-modifying antirheumatic drug (DMARD) or a biological DMARD. Our analysis revealed that the HLA-DRB1*01 allele is a significant prognostic marker for therapeutic response, predicting therapeutic resistance (P ═ 0.01). The most prevalent HLA-DRB1 alleles in the treatment-resistant group were HLA-DRB1*08:11 (11.3%), HLA-DRB1*01:01 (8.5%), HLA-DRB1*01:13, HLA-DRB1*04:11 (7%), HLA-DRB1*08:13, and HLA-DRB1*08:15 (4.2%). These findings highlight the critical role of HLA class II alleles in pediatric rheumatology, particularly in relation to treatment response and disease prognosis. In the era of personalized medicine, understanding the genetic contributions to treatment response and outcomes in JIA patients is essential. A key limitation of this study was the lack of comparison of treatment responses across different JIA subtypes. Future studies should prioritize evaluating MTX efficacy within specific JIA subgroups to enable a more tailored understanding of its effectiveness.

indira melezović, emela Čvorak, Amra mehmedagic, V. Selmanović, A. Čengić

PAPA syndrome (Pyogenic Arthritis, Pyoderma gangrenosum and Acne) is an autosomal dominant, hereditary autoinflammatory disease resulting from mutations in the PstPiP1/Cd2BP1 gene on chromosome 15q. The disease begins in childhood, most often from the age of 2 to 11, and it is characterised by a triad of symptoms: pyogenic (sterile) arthritis, pyoderma gangrenosum and acne. The disease usually begins with arthritis and is rarely recognised in the initial stage. The appearance of skin symptoms of the disease, either acne or pyoderma gangrenosum, along with the previously existing arthritis, should arouse suspicion of the existence of PAPA syndrome and direct doctors to perform further genetic testing. The triad of symptoms does not always have to be present, but the presence of two of the three symptoms with a confirmed gene mutation is a sufficient criterion for the diagnosis of the disease. Biological drugs have shown the greatest effectiveness in treatment, and il1 inhibitors or tnF alpha inhibitors are most often used medications. in later life, the joint manifestations gradually calm down, but the skin manifestations can last for many years with frequent relapses and remissions even with applied therapy, which makes this syndrome a great challenge for the treatment of this disease. Considering the small number of cases with PAPA syndrome described in the literature, we present to you an interesting case of a twenty-five-year-old patient with this disease and his challenging diagnostic and therapeutic path from childhood to adulthood.

Aim To analyse the association of human leukocyte antigen B27 with clinical and laboratory parameters in patients with juvenile idiopathic arthritis (JIA) at the disease onset. Methods A retrospective review of medical records of 25 HLAB27 positive and 25 HLA-B27 negative JIA patients was performed. The diagnosis of JIA was based on the 1997-2001 International League Against Rheumatism (ILAR) criteria. Collected data: age, sex, HLA- B27 antigen presence, C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), rheumatoid-factor (RF), antinuclear antibody (ANA), fever, rash, uveitis, enthesitis, inflamed joints and subtype of JIA. Results HLA- B27 positive study group had more boys (p=0.01), higher erythrocyte sedimentation rate (p=0.038), higher presence of fever (p= 0.025) and enthesitis (p=0.024). Any significant difference in age of the disease onset, CRP, ANA, RF, rash, uveitis, inflamed joint and dactylitis was not noticed. The most common subtype of JIA in the HLA-B27 positive patients was ERA (60%). Conclusion This study showed that the presence of HLA- B27 antigen plays a significant role in determining the presenting clinical and laboratory characteristics in JIA patients.

Nedim Strukar, V. Mišanović, A. Čengić, Aida Karačić, Alma Mujić, E. Ribic

R. Papa, A. Consolaro, F. Minoia, R. Caorsi, G. Magnano, M. Gattorno, A. Ravelli, R. Pillon, D. Marafon et al.

P381 Transient periosteal hyperostosis with dysproteinemia (Goldbloom syndrome): two cases report Riccardo Papa, Alessandro Consolaro, Francesca Minoia, Roberta Caorsi, Gianmichele Magnano, Marco Gattorno, Angelo Ravelli, Paolo Picco Pediatria II, Reumatologia, Istituto Giannina Gaslini, Genoa, Italy; Radiologia, Istituto Giannina Gaslini, Genoa, Italy Presenting author: Riccardo Papa Pediatric Rheumatology 2017, 15(Suppl 1):P381

Background PAPA syndrome (pyogenic sterile arthritis, pyoderma gangrenosum and acne) is a rare autosomal-dominant autoinflammatory disease caused by mutations in PSTPIP1gene. Typically presents with recurrent sterile, erosive arthritis in childhood, occurring spontaneously or after minor trauma, occasionally resulting in significant joint destruction. By puberty, joint symptoms tend to subside and cutaneous symptoms increase. Cutaneous manifestations include pathergy, frequently with abscesses at the sites of injections, severe cystic acne, and recurrent nonhealing sterile ulcers, often diagnosed as PG.

Objective: Although the incidence of acute rheumatic fever (ARF) has significantly decreased, individually reported outbreaks of the disease still occur in developed countries. The aim of this report is to present three patients with an initial attack of ARF, treated in the Department for Allergology, Rheumatology and Clinical Immunology during 2012 and 2013. Methods: The medical records of these three patients with ATF – who were treated in our department during the abovementioned period, and whose diagnoses were established according to the revised Jones criteria from 1992 – were reviewed. Results: Of the three patients, two were female (13 and 17 years old) and one was male (9 years old). Clinical and laboratory data were: migrating arthritis (3); carditis (1); fever (3); raised inflammatory markers – erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) (3) – and a prolonged PR interval on electrocardiogram; and first-degree heart block (3). All patients had elevated antistreprolysin titres and all were evaluated by echocardiography. One patient had mitral regurgitation. We introduced streptococcus eradication therapy with penicillin, therapeutic doses of aspirin, corticosteroids (for the patient with carditis) and secondary prevention of streptococcal infections with 4-weekly doses of benzathine penicillin G. Patients have been monitored as outpatients, and as yet there is no evidence of recurrence of the disease or its complications. Conclusion: ARF still occurs occasionally in developed countries. It is a disease that has not been eradicated, and this should not be forgotten in our efforts to reduce long-term morbidity and mortality.

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