RESUMO
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.
RESUMO
Rituximab (RTX) is a chimeric monoclonal antibody that targets the CD20 antigen on B cells and is used in various autoimmune disorders. In this study, we aimed to measure the awareness of pediatric rheumatologists about the use of RTX through a survey. Between February and March 2023, a 42-question survey was sent via email to pediatric rheumatology specialists in Turkey. The participants were questioned for which diagnoses and system involvement they preferred to use RTX, which routine tests they performed, vaccination policy, and adverse events that occurred during or after infusion. Forty-one pediatric rheumatologists answered the survey. They prescribed RTX most frequently for systemic lupus erythematosus (87.8%) and ANCA-associated vasculitis (9.8%). Prior to the administration of RTX, 95% of clinicians checked renal and liver function tests, as well as immunoglobulin levels. The most frequently tested hepatitis markers before treatment were HBsAg and anti-HBs antibody (97.6%), while 85.4% of rheumatologists checked for anti-HCV. Clinicians (31.4%) reported that they postpone RTX infusion 2 weeks following an inactivated vaccine. Sixty-one percent of rheumatologists reported starting RTX treatment 1 month after live vaccines, while 26.8% waited 6 months. The most frequent adverse events were an allergic reaction during RTX infusion (65.9%), hypogammaglobulinemia (46.3%), and rash (36.6%). In the event of hypogammaglobulinemia after RTX treatment, physicians reported that they frequently (58.5%) continued RTX after intravenous immunoglobulin administration. CONCLUSIONS: RTX has become a common treatment option in pediatric rheumatology in recent years. Treatment management may vary between clinician such as vaccination and routine tests. WHAT IS KNOWN: ⢠During the course of rituximab therapy, clinicians should be attentive to specific considerations in pre-treatment, during administration, and in post-treatment patient monitoring. WHAT IS NEW: ⢠There are differences in practice among clinicians in the management of RTX therapy. These practice disparities have the potential to impact the optimal course of treatment. ⢠This study highlights that standardized guidelines are needed for RTX treatment in pediatric rheumatology, particularly for vaccination policies and routine tests.
Assuntos
Antirreumáticos , Padrões de Prática Médica , Reumatologistas , Rituximab , Humanos , Rituximab/uso terapêutico , Rituximab/efeitos adversos , Padrões de Prática Médica/estatística & dados numéricos , Antirreumáticos/uso terapêutico , Antirreumáticos/efeitos adversos , Criança , Inquéritos e Questionários , Masculino , Turquia , Feminino , Reumatologia , Doenças Autoimunes/tratamento farmacológico , Pediatras/estatística & dados numéricos , PediatriaRESUMO
Thymic tumors are very rare neoplasms in children and account for less than 1% of mediastinal tumors in pediatric patients. One-third of the pediatric patients present with symptoms related to the compression of the tumor mass on the surrounding anatomic structures, and paraneoplastic syndromes such as myasthenia gravis, pure red cell aplasia, acquired hypogammaglobulinemia, and connective tissue disorders, which rarely occur in children with thymic tumors. Herein, we report a case of thymic carcinoma mimicking the symptoms of a connective tissue disease with symmetrical polyarthritis accompanying myositis, fever, weight loss, and malaise in a 15-year-old male patient. To our knowledge, this is the first case pediatric thymic carcinoma accompany with severe polyarthritis and myopathy, thus we have reviewed the current literature regarding the cases of thymic malignancies coexisting with paraneoplastic syndromes in children.
Assuntos
Artrite , Miosite , Síndromes Paraneoplásicas , Timoma , Neoplasias do Timo , Humanos , Masculino , Miosite/diagnóstico , Miosite/complicações , Síndromes Paraneoplásicas/diagnóstico , Síndromes Paraneoplásicas/etiologia , Neoplasias do Timo/complicações , Neoplasias do Timo/diagnóstico , Adolescente , Artrite/diagnóstico , Artrite/etiologia , Timoma/complicações , Timoma/diagnóstico , Resultado do Tratamento , Timectomia , BiópsiaRESUMO
OBJECTIVES: The aim of this study is to investigate the effect of anti-interleukin (IL)-1/-6 biologics on systemic juvenile idiopathic arthritis (sJIA)-associated macrophage activation syndrome (MAS). METHODS: Demographic, clinical, and laboratory data of patients followed up with a diagnosis of sJIA-associated MAS assessed from sixteen pediatric rheumatology centers across the country. The clinical and laboratory features of MAS developing while on biological drugs were compared with those without this treatment. RESULTS: One hundred and sixty-two patients were included in the study. 45 of the MAS events were detected under the effect of anti-IL-1/-6 biologics, while the patients experiencing the remaining 155 events have not received biological treatment in the last three months. Platelet count [128 (72-232) vs 199 (130-371) 109/l], ferritin level on admission [1107 (676-2050) vs 2863 (1193-9562) ng/ml], C-reactive protein level [15.4 (2.9-56) vs 90 (32-160) mg/l], erythrocyte sedimentation rate [13 (3-36) vs 43.5 (13-77) mm/h] and fever duration [5 (4-7.5) vs 10 (7-14.3) days] were found lower in the group under the impact of anti-IL-1/-6 biologics. Among patients treated with biologics, 26.6% did not meet the published 2016 MAS classification criteria at presentation. The rates of hepatomegaly and splenomegaly were relatively lower in the canakinumab-treated group when compared with those receiving other biologicals or to patients, not on biologicals. CONCLUSION: Anti-IL-1/-6 therapies can mask the clinical and laboratory features of MAS, and proposed guidelines for MAS classification criteria may not be met.
RESUMO
OBJECTIVE: Biologic therapy has changed the prognosis of patients with rheumatologic disease. Despite all benefits of the biological agents, adverse events may occur due to their long-term use. The aim of this study is to analyze the adverse events observed in pediatric patients who received biological treatment. MATERIALS AND METHODS: This retrospective observational cohort study was conducted between January 2010 and January 2022. File records of 139 patients used biological agents for rheumatologic diseases in a pediatric rheumatology clinic were evaluated. Diagnosis, received treatment, the rationale for stopping treatment, requirement of tuberculosis prophylaxis, presence of an adverse event, and results were recorded. RESULTS: The most used biological therapy was etanercept (41.7%). Anakinra, adalimumab, canakinumab were used in 30.9%, 27.3%, 23.7% of patients, and the others in less than 10%. Totally 491 adverse events (97.9/100 patient-years) were encountered during the duration of biological treatment. The most often adverse event was recurrent upper respiratory tract infection in the patients (31.9/100 patient-years). Elevated aminotransferase levels (10.4/100 patient-years), abdominal pain (7/100 patient-years), and headache (5.2/100 patient-years) were among the other common side effects. Isoniazid (INH) prophylaxis was needed before biological treatment in 20.9% of the patients. Tuberculosis developed in none of the patients followed-up for latent tuberculosis, however, it developed in a patient while receiving etanercept due to noncompliance with his scheduled outpatient visits during etanercept treatment. CONCLUSION: The most commonly used biological treatments were TNFi and IL-antagonists, and the majority of side effects were infections and laboratory abnormalities. Although the rate of serious adverse events is quite low, close follow-up of patients receiving biological therapy is very important.
RESUMO
OBJECTIVE: Chronic nonbacterial osteomyelitis (CNO) is a noninfectious autoinflammatory bone condition that frequently occurs alongside other inflammatory diseases, such as familial Mediterranean fever (FMF). We aimed to determine the demographic, clinical, laboratory, and radiological characteristics of patients diagnosed with both FMF and CNO. METHODS: We reviewed the medical records of pediatric patients with both CNO and FMF at 3 pediatric rheumatology centers in Turkey from December 2008 to 2022. Patients' demographics, laboratory features, imaging findings, and treatment were recorded. RESULTS: Twelve patients with FMF and CNO were included in the study. Half of them were female. The mean ages at onset for FMF and CNO symptoms were 80 and 116 months, whereas the ages at diagnosis for FMF and CNO were 100 and 125 months, respectively. Ten patients (83.3%) had M694V mutation on at least 1 allele of the Mediterranean fever (MEFV) gene. The most common sites of osteitis were the long bones (58.3%), pelvis (50%), and clavicles (25%). Ten patients (83%) received nonsteroidal anti-inflammatory drugs; 8 (66%) received disease-modifying antirheumatic drugs; biological therapy was administered to 5 patients (41%), who did not respond to these treatments; and all patients received colchicine. CONCLUSION: The increased frequency of FMF in patients with CNO is of interest. Because most patients with CNO and FMF carried a homozygous or combined heterozygous M694V mutation, we speculated that the M694V mutation may play a role in the development of osteitis. Further studies are needed to elucidate the link between FMF and CNO.
RESUMO
BACKGROUND: Juvenile idiopathic arthritis (JIA) is a prevalent childhood chronic arthritis, often persisting into adulthood. Effective transitional care becomes crucial as these patients transition from pediatric to adult healthcare systems. Despite the concept of transitional care being recognized, its real-world implementation remains inadequately explored. This study aims to evaluate the thoughts and practices of healthcare providers regarding transitional care for JIA patients. METHODS: A cross-sectional survey was conducted among pediatric and adult rheumatologists in Turkey. Based on the American Academy of Pediatrics' six core elements of transitional care, the survey included 86 questions. The respondents' demographic data, attitudes towards transitional care, and practical implementation were assessed. RESULTS: The survey included 48 rheumatologists, with 43.7% having a transition clinic. The main barriers to establishing transition programs were the absence of adult rheumatologists, lack of time, and financial constraints. Only 23.8% had a multidisciplinary team for transition care. Participants agreed on the importance of coordination and cooperation between pediatric and adult healthcare services. The timing of the transition process varied, with no consensus on when to initiate or complete it. Participants advocated for validated questionnaires adapted to local conditions to assess transition readiness. CONCLUSIONS: The study sheds light on the challenges and perspectives surrounding transitional care for JIA patients in Turkey. Despite recognized needs and intentions, practical implementation remains limited due to various barriers. Cultural factors and resource constraints affect the transition process. While acknowledging the existing shortcomings, the research serves as a ground for further efforts to improve transitional care and ensure better outcomes for JIA patients transitioning into adulthood.
Assuntos
Artrite Juvenil , Transição para Assistência do Adulto , Cuidado Transicional , Adolescente , Humanos , Artrite Juvenil/terapia , Estudos Transversais , Reumatologistas , TurquiaRESUMO
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.
Assuntos
Esclerite , Uveíte , Masculino , Humanos , Criança , Adolescente , Etanercepte/efeitos adversos , Esclerite/induzido quimicamente , Fator de Necrose Tumoral alfa , Uveíte/complicações , Inflamação/complicaçõesRESUMO
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.
Assuntos
Febre Familiar do Mediterrâneo , Obstrução Intestinal , Humanos , Criança , Febre Familiar do Mediterrâneo/complicações , Febre Familiar do Mediterrâneo/diagnóstico , Febre Familiar do Mediterrâneo/tratamento farmacológico , Colchicina , Obstrução Intestinal/diagnóstico , Obstrução Intestinal/etiologia , Obstrução Intestinal/cirurgia , Dor Abdominal/etiologia , HomozigotoRESUMO
OBJECTIVES: We aimed to outline the demographic data, clinical spectrum, and treatment approach of sarcoidosis in a large group of patients and sought to figure out the variations of early-onset (EOS) and late-onset paediatric sarcoidosis (LOS). METHODS: The study followed a retrospective-descriptive design, with the analysis of medical records of cases diagnosed as paediatric sarcoidosis. RESULTS: Fifty-two patients were included in the study. The median age at disease onset and follow-up duration were 83 (28.2-119) and 24 (6-48) months, respectively. Ten (19.2%) cases had EOS (before 5th birthday) and 42 (80.7%) cases had LOS. The most common clinical findings at the time of the disease onset were ocular symptoms (40.4%) followed by joint manifestation (25%), dermatological symptoms (13.5%), and features related to multi-organ involvement (11.5%). Anterior uveitis was the most common (55%) one among ocular manifestations. Patients with EOS displayed joint, eye, and dermatological findings more commonly than patients with LOS. The recurrence rate of disease in patients with EOS (5.7%) and LOS (21.1%) were not statistically different (P = .7). CONCLUSIONS: Patients with EOS and LOS may present with variable clinical features and studies addressing paediatric sarcoidosis cases in collaboration between disciplines will enhance the awareness of this rare disease among physicians and assist early diagnosis with lesser complications.
Assuntos
Sarcoidose , Uveíte , Humanos , Criança , Uveíte/diagnóstico , Uveíte/etiologia , Estudos Retrospectivos , Turquia , Sarcoidose/diagnóstico , Sarcoidose/terapia , Sarcoidose/complicaçõesRESUMO
BACKGROUND: The aim of this study was to evaluate the efficacy and safety of anti-interleukin-1 (IL-1) therapies in colchicine-resistant pediatric patients with familial Mediterranean fever (FMF). METHODS: In this study, we retrospectively evaluated 656 children with FMF and 27 patients who had been treated with anti-IL-1 therapies (anakinra/canakinumab) . Clinical and laboratory features, MEFV gene mutations, treatment responses were investigated. RESULTS: Twenty of the patients were treated with anakinra (the treatment of 6 patients who initially used anakinra was switched to canakinumab in the follow-up period), and 13 patients were treated with canakinumab. Clinical symptom and severity scores decreased in all patients A decrease in acute phase reactants was also observed in patients. A total of 18 (66%) patients had a M694V homozygous mutation, while 24 (89%) patients had a M694V mutation, at least in one allele. CONCLUSIONS: FMF patients with colchicine resistance may progress to amyloidosis. IL-1 antagonist treatment could be used safely with a favorable outcome in pediatric patients with FMF resistance to colchicine therapy and/or who have renal amyloidosis.
Assuntos
Amiloidose , Febre Familiar do Mediterrâneo , Humanos , Criança , Febre Familiar do Mediterrâneo/tratamento farmacológico , Febre Familiar do Mediterrâneo/genética , Febre Familiar do Mediterrâneo/diagnóstico , Colchicina/efeitos adversos , Proteína Antagonista do Receptor de Interleucina 1/uso terapêutico , Proteína Antagonista do Receptor de Interleucina 1/efeitos adversos , Interleucina-1/uso terapêutico , Estudos Retrospectivos , Amiloidose/induzido quimicamente , Amiloidose/tratamento farmacológico , Pirina/genéticaRESUMO
AIM: The aim of this study was to compare the clinical and laboratory features, treatment choices and responses, and outcomes between patients with clinically amyopathic juvenile dermatomyositis (CAJDM) and classical juvenile dermatomyositis (JDM). METHODS: We retrospectively reviewed the medical records of patients with CAJDM and JDM, and compared the 2 groups' clinical and laboratory data, treatment agents and responses, and outcomes. RESULTS: There were 38 JDM and 12 CAJDM patients, with female dominance. There was a higher delay time in diagnosis for CAJDM (P = 0.000). Compared to other clinical symptoms of JDM, muscle weakness and myalgia were more prominent in JDM than in CAJDM (P = 0.000). The absolute lymphocyte count was lower (P = 0.034) in patients with JDM than in those with CAJDM. Anti-p155/140 (TIF-1) antibody positivity was significantly more common in the CAJDM group (P = 0.000), while anti-NXP2 antibody was more common in the JDM group (P = 0.046). In terms of treatment, pulse corticosteroid usage was more common in patients with JDM than in those with CAJDM (P = 0.000). CONCLUSION: Close clinical follow-ups with effective treatments are important to prevent complications, such as calcinosis and skin ulcers, that may develop in patients with poorly controlled CAJDM. Anti-p155/140 antibodies may be a useful indicator for detecting amyopathic forms of dermatomyositis in children.
Assuntos
Dermatomiosite , Criança , Humanos , Feminino , Dermatomiosite/complicações , Dermatomiosite/diagnóstico , Dermatomiosite/tratamento farmacológico , Estudos Retrospectivos , Corticosteroides/uso terapêutico , Resultado do TratamentoRESUMO
BACKGROUND: Mevalonate kinase (MVK) plays a role in cholesterol and non-sterol isoprenoid biosynthesis and its deficiency-related diseases are caused by bi-allelic pathogenic mutations in the MVK gene, (MVK), which leads to rare hereditary autoinflammatory diseases. The disease may manifest different clinical phenotypes depending on the degree of the deficiency in the enzyme activity. The complete deficiency of the enzyme activity results in the severe metabolic disease called mevalonic aciduria, while a partial deficiency results in a broad spectrum of clinical presentations called hyper-immunoglobulin D syndrome (HIDS). Serum immunoglobulin (Ig) D and urine mevalonic acid levels may be increased during inflammatory attacks of HIDS. CASE PRESENTATION: Herein, for the first time in the literature, we present a 6-year-old male patient who suffered from recurrent episodes of fever, polyarthritis, skin rash, diarrhea, abdominal pain, and inflammatory bowel disease-like manifestations with elevated levels of serum IgD, and urine mevalonic acid. Eventually we detected compound heterozygous mutations in the phosphomevalonate kinase (PMVK) gene coding the second enzyme after mevalonate kinase in the mevalonate pathway. CONCLUSION: For patients presenting with HIDS-like findings, disease exacerbations and persistent chronic inflammation, and having high urinary mevalonic acid and serum IgD levels, raising suspicion in terms of MVK deficiency (MVKD), it is recommended to study all mevalonate pathway enzymes, even if there is no mutation in the MVK gene. It should be kept in mind that novel mutations might be seen such as PMVK gene.
Assuntos
Doenças Hereditárias Autoinflamatórias , Deficiência de Mevalonato Quinase , Humanos , Masculino , Doenças Hereditárias Autoinflamatórias/diagnóstico , Doenças Hereditárias Autoinflamatórias/genética , Imunoglobulina D , Deficiência de Mevalonato Quinase/diagnóstico , Deficiência de Mevalonato Quinase/genética , Ácido Mevalônico , Fosfotransferases (Aceptor do Grupo Álcool)/genética , Fosfotransferases (Aceptor do Grupo Álcool)/metabolismo , CriançaRESUMO
Hyperimmunoglobulin D syndrome (HIDS) is a hereditary autoinflammatory disease characterized by recurrent inflammatory attacks with fever, abdominal pain, lymphadenopathy, aphthous stomatitis, and skin lesions. There are few reports on HIDS patients complicated with macrophage activation syndrome (MAS); however, to our knowledge, there is no case of HIDS with recurrent MAS attacks. We report two pediatric patients initially diagnosed as Kawasaki disease and systemic juvenile idiopathic arthritis presented with recurrent MAS episodes with prolonged fever, skin rash, hepatosplenomegaly, cervical lymphadenopathy, aphthous stomatitis, headache, pancytopenia, hyperferritinemia, and hypofibrinogenemia, finally diagnosed as HIDS with a documented homozygous MVK gene mutation. This is the first report on recurrent MAS attacks due to HIDS in pediatric patients who were successful treated with corticosteroids and anti-IL-1 therapies. Thus, clinicians should be vigilantly investigated signs of autoinflammatory diseases in patients with recurrent MAS attacks during their disease course, and HIDS should be considered an underlying disease for triggering recurrent MAS attacks. We have also reviewed the current literature regarding HIDS cases complicated with a MAS attack and summarized their demographic, treatment, and outcome characteristics. Key points ⢠Hyperimmunoglobulin D syndrome should be considered in differential diagnosis in patients who experienced recurrent macrophage activation syndrome attacks.
Assuntos
Linfadenopatia , Síndrome de Ativação Macrofágica , Deficiência de Mevalonato Quinase , Estomatite Aftosa , Criança , Humanos , Síndrome de Ativação Macrofágica/diagnóstico , Síndrome de Ativação Macrofágica/tratamento farmacológico , Síndrome de Ativação Macrofágica/etiologia , Deficiência de Mevalonato Quinase/complicações , Deficiência de Mevalonato Quinase/diagnóstico , Deficiência de Mevalonato Quinase/tratamento farmacológico , FebreRESUMO
OBJECTIVES: The aim of this study is to compare the demographic, clinical features, treatment results and outcomes in pediatric patients with idiopathic uveitis and uveitis due to juvenile idiopathic arthritis (JIA) and Behçet's disease (BD). METHODS: 97 pediatric uveitis patients were divided into three groups according to the etiology of uveitis: Group 1 comprised idiopathic uveitis patients, Group 2 uveitis patients who had JIA, and Group 3 uveitis patients with BD. RESULTS: Symptomatic presentation and intermediate uveitis were more common in Group 1 (p < 0.005). Asymptomatic presentation and anterior uveitis in Group 2 (p < 0.005), whereas symptomatic presentation and posterior uveitis in Group 3 (p < 0.005). Erythrocyte sedimentation rate (ESR) was higher in patients with BD or JIA uveitis than those with idiopathic uveitis (p < 0.005). Biologic therapy was more commonly used in JIA group compared to other groups (p < 0.005). Patients who had a complication related with uveitis were more common in females, asymptomatic disease course, and needed use of biologic treatment than in those without any complication (p < 0.005). CONCLUSION: Uveitis accompanying rheumatologic diseases may have asymptomatic and insidious course but have higher ESR as an important notice.
Assuntos
Artrite Juvenil , Síndrome de Behçet , Uveíte , Criança , Feminino , Humanos , Artrite Juvenil/complicações , Síndrome de Behçet/complicações , Resultado do Tratamento , Uveíte/complicaçõesRESUMO
OBJECTIVES: Persistent inflammation is an insidious feature of familial Mediterranean fever (FMF) that may cause chronic complications. This study aimed to investigate the predictors of persistent inflammation in children with FMF. METHODS: The medical charts of 1077 paediatric FMF patients were retrospectively collected. The patients were divided into two groups: with and without subclinical inflammation. RESULTS: A total of 133 (12%) patients had persistent inflammation. M694V homozygosity, colchicine resistance, positive family history for FMF, erysipelas-like erythema, leg pain, arthritis, chest pain, inflammatory comorbidities, early disease onset, high PRAS score, and long attack duration were established as independent predictors of persistent inflammation (P < .001, P < .001, P < .001, P < .001, P = 0.006, P < .001, P < .001, P = .014, P < .001, P < .001, and P < .001, respectively). However, gender, abdominal pain, fever, and attack frequency were not found to be independent risk factors for predicting persistent inflammation (P = .412, P = .531, P = .451, and P = .693, respectively). CONCLUSIONS: M694V homozygosity, colchicine resistance, positive family history, erysipelas-like erythema, leg pain, arthritis, chest pain, inflammatory comorbidities, early disease onset, high activity score, and long attack duration may be predictors of persistent inflammation in FMF. These predictors may help clinicians suspect the occurrence of subclinical inflammation and should aid in better disease management in FMF.