Pediatrik Behçet Hastalığında Kas İskelet Sistemi Tutulumu: Tek Merkez Deneyimi

Amaç: Amaç: Behçet hastalığı (BH) tekrarlayan oral ülserler, genital ülserler, oküler bulgular ve vasküler tutulum ile karakterize inflamatuvar bir hastalıktır. Kas-iskelet sistemi semptomları hem başvuru sırasında hem de Behçet hastalığının seyri boyunca yaygındır. Bu çalışmanın amacı, kliniğimiz tarafından pediatrik Behçet hastalığı (PEDBH) tanısı ile takip edilen hastalarda kas-iskelet sistemi tutulumunun sıklığını ve özelliklerini bildirmektir. Gereç ve Yöntemler: Bu retrospektif tıbbi kayıt incelemesi, Ocak 2010 ile Aralık 2022 tarihleri arasında 16 yaşından önce PEDBH tanısı alan hastaları kapsamaktadır. Bulgular: Çalışmaya dahil edilen 90 hastanın 48’i (%53.3) kadındı. Ortalama tanı yaşı 12.4±3 yıldı. Tüm hastalarda (%100) tekrarlayan oral ülserler, 55’inde (%61.1) genital ülserler, 44’ünde (%48.9) kas-iskelet sistemi tutulumu, 37’sinde (%41.1) deri bulguları, 19’unda (%21.1) göz tutulumu, 17’sinde (%18.9) nörolojik tutulum ve 17’sinde (%18.9) vasküler tutulum vardı. Eklem tutulumu olan hastaların 27’sinde (%65.9) artrit, 41’inde (%100) artralji, 37’sinde (%90.1) oligoartiküler eklem tutulumu ve 29’unda (%70.7) asimetrik tutulum vardı. Hastalarda en sık etkilenen eklem diz (%63.4) olurken, bunu ayak bileği (%31.7), el bileği (%19.5), sakroiliak eklemler (%14.6), eller (%12.2) (Bir hastada metakarpofalangeal eklem ve dört hastada proksimal interfalangeal eklem tutulumu vardı), dirsek (%9.8) ve ayaklar (%4.9) (Hastalardan birinde metatarsofalangeal eklem tutulumu ve diğerinde proksimal interfalangeal eklem tutulumu vardı) takip etti. Sonuç: Kas-iskelet sistemi semptomları PEDBH’de yaygındır ve tanı anında hastalığın erken bir belirtisi olarak gözlemlenebilir. Bu nedenle, kas-iskelet sistemi semptomları olan çocuklarda olası BH’nin ayrıntılı bir şekilde araştırılması önemlidir.

Anahtar Kelimeler:

Artralji, artrit, Pediatrik Behçet hastalığı, Kas iskelet sistemi

Musculoskeletal Involvement in Pediatric Behçet's Disease: A Single Center Experience

Background: Behçet’s disease (BD) is an inflammatory disease characterized by recurrent oral ulcers, genital ulcers, ocular manifestations, and vascular involvement. Musculoskeletal symptoms are common both at the time of presentation and throughout the course of BD, and similar symptoms are also observed in pediatric Behçet’s disease (PEDBD). This study aims to report the frequency and role of musculoskeletal involvement in PEDBD followed by our clinic. Method: This retrospective medical record review included a total of 90 patients diagnosed with PEDBD before the age of 16 years between January 2010 and December 2022. Results: Of the patients, 48 (53.3%) were female and 42 (46.7%) were male. Their mean age at diagnosis was 12.4 years. All patients (100%) had recurrent oral ulcers, while 55 (61.1%) had genital ulcers, 44 (48.9%) had musculoskeletal involvement, 37 (41.1%) had skin manifestations, 19 (21.1%) had ocular involvement, 17 (18.9%) had neurological involvement, and 17 (18.9%) had vascular involvement. Among the patients with joint involvement, 27 (65.9%) had arthritis, 41 (100%) had arthralgia, 37 (90.1%) had oligoarticular joint involvement, and 29 (70.7%) had unilateral involvement. The most commonly affected joints in the patients was in the knee (63.4%), followed by the ankle (31.7%), the wrist (19.5%), sacroiliac joints (14.6%), the hands (12.2%), the elbow (9.8%), and the feet (4.9%). Conclusions: Musculoskeletal symptoms are common in PEDBD and can be observed as an early sign of the disease at the time of diagnosis. Therefore, it is important to thoroughly inquire about possible BD in children with musculoskeletal symptoms.

Keywords:

arthralgia, arthritis, pediatric Behçet’s disease, musculoskeletal system,

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