Systemic onset juvenile idiopathic arthritis: a single center experience

Camptodactyly-arthropathy-coxa vara-pericarditis (CACP) syndrome, caused by biallelic pathogenic mutations in the <i>PRG4</i> gene, is characterized by early-onset camptodactyly, noninflammatory arthropathy, coxa vara deformity, and rarely, pericardial effusion. Herein, we report 3 patients with CACP syndrome from 2 unrelated families. All patients are female, born to consanguineous parents, and had camptodactyly since the first years of their lives. Two patients had a prior diagnosis of juvenile idiopathic arthritis. Hip changes were present in 2 patients, and 2 of 3 patients had undergone surgery for camptodactyly. Routine echocardiographic evaluations were normal during the 2-year follow-up. This paper represents the third study including CACP patients from Turkey. Clinically, all 3 patients resembled juvenile idiopathic arthritis cases and received unnecessary medication. There is also an ongoing need for improving awareness of CACP and an effective treatment focusing on the lubrication of the joint space in CACP patients.

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Anakinra in Systemic Juvenile Idiopathic Arthritis: A Single-center Experience

The Journal of Rheumatology ◽

10.3899/jrheum.141567 ◽

2015 ◽

Vol 42 (8) ◽

pp. 1523-1527 ◽

Cited By ~ 25

Author(s):

Manuela Pardeo ◽

Denise Pires Marafon ◽

Antonella Insalaco ◽

Claudia Bracaglia ◽

Rebecca Nicolai ◽

...

Keyword(s):

Juvenile Idiopathic Arthritis ◽

Primary Outcome ◽

Late Onset ◽

Disease Onset ◽

Systemic Juvenile Idiopathic Arthritis ◽

Inactive Disease ◽

Single Center ◽

Single Center Experience ◽

Number Of Patients ◽

The Ideal

Objective.To assess anakinra as a therapy for systemic juvenile idiopathic arthritis (sJIA) in a single-center series.Methods.We reviewed 25 patients with sJIA treated with anakinra for at least 6 months. The primary outcome was the number of patients who achieved clinically inactive disease at 6 months, according to preliminary criteria for inactive disease and clinical remission of JIA.Results.Among 25 patients evaluated, 14 (56%) met the criteria for inactive disease at 6 months and were classified as responders. For each individual patient, we compared the dose administered with the ideal dose of anakinra and we found that there was no relation with response. We also compared demographic characteristics and clinical and laboratory features at baseline in responders and non-responders: no differences were observed in relation with the number of active joints before starting anakinra or concomitant glucocorticoids treatment. The only variable significantly associated with response was the time from disease onset to receiving anakinra, with earlier treatment being associated with a better outcome.Conclusion.Anakinra is associated with rapid attainment of inactive disease in a significant portion of patients. We found that only the earlier treatment is associated with better outcome. However, formal studies on early treatment and on the pathophysiology and response to treatments, including anakinra, of early- and late-onset sJIA are needed to optimize the management of this challenging disease.

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Association of Vitamin D Status and Activity of Juvenile Idiopathic Arthritis: A Single Center Experience

The Central European Journal of Paediatrics ◽

10.5457/p2005-114.280 ◽

2021 ◽

Vol 17 (1) ◽

pp. 1-7

Author(s):

Almira Ćosićkić

Keyword(s):

Vitamin D ◽

Juvenile Idiopathic Arthritis ◽

Vitamin D Status ◽

Single Center ◽

Single Center Experience

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Juvenile idiopathic arthritis in Jordan: Single center experience

10.21203/rs.3.rs-103495/v2 ◽

2021 ◽

Author(s):

Raed M. Alzyoud ◽

Motasem O. Alsuweiti ◽

Heba Q. Almaaitah ◽

Bushra N. Aladaileh ◽

Mohammad K. Alnoubani ◽

...

Keyword(s):

Juvenile Idiopathic Arthritis ◽

Treatment Options ◽

Age At Onset ◽

Laboratory Data ◽

Population Based ◽

Detailed Knowledge ◽

Single Center ◽

Rheumatology Clinic ◽

Single Center Experience ◽

Single Center Study

Abstract Background: Juvenile idiopathic arthritis (JIA) is a heterogeneous group of disorders, including all forms of arthritis, which develops in children who are less than 16 years old. This study aimed to evaluate the clinical and laboratory features of JIA in a single center in Jordan.Methods: A retrospective analysis of the electronic medical records of pediatric patients diagnosed with JIA based on the International League of Associations for Rheumatology (ILAR) criteria during the period from 2015 to 2019 at the Pediatric Rheumatology Clinic in the Queen Rania Children’s Hospital. All patients were below the age of 14 years at the time of diagnosis and followed for at least six months. Collected data consisted of age, gender, age at initial presentation and diagnosis, JIA subtype, laboratory data, treatment options, and outcome.Results: A total of 210 patients were included in this cohort (94 males and 116 females) with the mean age at diagnosis and mean age at onset of 5.33 ± 3.40 years and 5.08 ± 3.40 years (range: 7 months – 14 years), respectively. Oligoarticular JIA was the commonest subtype (54.7%), followed by systemic arthritis (17.1%) and polyarticular arthritis (12.3%). ANA was positive in 70 patients (33.6%). Uveitis occurred in thirty (14.2%) patients.Conclusion: To the best of our knowledge, this study on this cohort is the first report on JIA in Jordan, in comparison with other regionally and internationally published reports. Oligoarticular JIA was found to be the most common subtype. For detailed knowledge on JIA characteristics and patterns, a population-based, rather than a single center study, should be conducted in Jordan.

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