Sibel BALCI, Rabia Miray KIŞLA EKİNCİ, Dilek DOĞRUEL, Mahir SERBES, Derya Ufuk ALTINTAŞ, Mustafa YILMAZ

IgA eksikliğinin juvenil dermatomiyozit prognozuna olumlu etkisi var mıdır? olgu sunumu.

Juvenil dermatomiyozit nedeni tam olarak bilinmeyen, birçok sistemi etkileyebilen otoimmün bir hastalıktır. Doğuştan ve kazanılmış immünitenin hastalık patogenezinde rolü bulunmaktadır. Selektif immünglobulin A eksikliği en sık görülen primer immün yetmezliktir. İmmünglobulin A eksikliği ile otoimmün hastalıkların birlikteliği daha önce birkaç juvenil dermatomiyozit tanılı olguda bildirilmiştir. Öncesinde sağlıklı olan 15 yaşında kız hasta Bohan ve Peter kriterlerine göre juvenil dermatomiyozit ve serum immunglobulin A değeri <6 mg/dl saptandığı için selektif immünglobulin A tanısı aldı. Üç ay immünsupresif tedavi aldıktan sonra fizik muayenede raş saptanmadı, kas gücü normaldi, ayrıca kas enzimleri normal düzeylerdeydi. Düşük doz metilprednizolon ve subkutan metotreksat tedavileri alan hastanın kas gücü muayenesi ve kas enzimleri 12 aylık izlem sonrasında halen normal sınırlarda seyretmiştir. İmmün yetmezlik ve otoimmün hastalık birlikteliği hakkındaki bilgilerimiz, otoimmün hastalık veya immün yetmezlik tanılı hastaların yine bu hastalıklar açısından incelenmesini gerekli kılmaktadır.

Does selective IgA deficiency have a good prognostic role on juvenile dermatomyositis? a case report.

Juvenile dermatomyositis is a multisystemic autoimmune disease with uncertain etiology. Both innate immunity and adaptive immunity play a role on the pathogenesis of the disease. Selective immunoglobulin A deficiency is the most common primary immunodeficiency. Association between immunoglobulin A deficiency and autoimmune diseases including few juvenile dermatomyositis patients have been reported. A previously healthy 15-year old girl was diagnosed with juvenile dermatomyositis according to Bohan and Peter criteria and selective immunoglobulin A deficiency due to the low level of immunoglobulin A (<6 mg/dl). After 3 months of immunosuppressive treatment, her physical examination revealed no muscle weakness, no rashes, and normal muscle enzyme levels. While she has been treated with low dose methylprednisolone and subcutaneous methotrexate, muscle strength and muscle enzymes remained in normal levels at 12 months follow-up. Our knowledge about the co-occurrence of immunodeficiency and autoimmunity emerge that patients diagnosed with autoimmunity should have investigations for immunodeficiency or vice versa.

Keywords:

Autoimmunity, IgA deficiency, juvenile dermatomyositis,

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