İnflamatuvar Miyopatilerde Miyozit Spesifik Antikorlar ve Klinik Kullanımı: Tek Merkez Deneyimi

Öz

Miyozit spesifik antikorlar (MSA), inflamatuvar miyozitleri sınıflandırmada, klinik seyir ve prognozu öngörmede son yıllarda artan sıklıkla kullanılmaktadır. Bu çalışmada merkezimizde takipli inflamatuvar miyopatili hastalar içerisinde miyozit spesifik antikoru çalışılmış hastaların demografik özellikleri, tanıları, klinik bulguları, laboratuvar verileri, antikor profilleri, enfeksiyöz komplikasyon ve malignite gelişimi ve mortalite verileri analiz edildi. Çalışmaya toplam 33 hasta dahil edildi (29 kadın, 4 erkek). Medyan yaş 52 yıl (15-72) ve medyan takip süresi 27 ay (2-250) olarak hesaplandı. Hastaların 15 (%45.4)’i dermatomiyozit tanılı idi. Hastaların 26’sında MSA pozitif saptandı. En sık pozitif saptanan antikorlar Jo-1 (%26,9) ve TIF-1 gamma (%23,1) idi. MSA pozitif inflamatuvar myopatili hastalarda konstitüsyonel semptomlar daha sık görülmekteydi (p:0,011). MSA alt gruplarına (Jo-1 ve diğerleri/ TIF-1 gamma ve diğerleri/ MDA-5 ve diğerleri) göre klinik bulgular değerlendirildiğinde; pulmoner tutulum ile Jo-1 pozitifliği (p<0,001) ve MDA-5 pozitifliği ile kalsinozis (p:0,018) arasında istatistiksel olarak anlamlı bir ilişki bulundu. Toplam 13 hastada infeksiyöz komplikasyonlar ve 3 hastada malignite gelişmişti (serviks, over ve kolorektal karsinom). Çalışmamızda MSA alt gruplarının sıklığı literatür ile benzerlik taşısa da genelleme yapabilmek için geniş hasta popülasyonunda yapılacak çalışmalara ihtiyaç vardır.

Anahtar Kelimeler

• İnflamatuvar myopatiler
• miyozit spesifik antikorlar
• klinik bulgular
• prognoz

Myositis-Specific Autoantibodies in Patients with Inflammatory Myopathies and Clinical Practice: a Single Centre Experience

Öz

Myositis-specific autoantibodies (MSAs) have been increasingly utilized in recent years for the classification of inflammatory myopathies, and for predicting clinical course and prognosis. In this study, patients with idiopathic inflammatory myopathy diagnosis whose MSAs were present included in this study. were screened randomly for the presence of MSAs within the past year. Demographic characteristics, myositis type, clinical manifestations, laboratory findings, autoantibody profiles, infectious complications, malignancy development, and mortality data were analyzed. A total of 33 patients were included in the study (29 female, 4 male). The median age was 52 years (range: 15–72), and the median follow-up duration was 27 months (range: 2–250). Dermatomyositis was the most common myositis type (45.4%). 26 patients had at least one positive MSA. The most frequently detected antibodies were anti-Jo-1 (26.9%) and anti-TIF-1γ (23.1%). Constitutional symptoms were more frequently observed in the MSA-positive group (p = 0.011). When clinical features were evaluated according to MSA subgroups (Jo-1 vs. others; TIF-1γ vs. others; MDA-5 vs. others), a statistically significant association was found for Jo-1 positivity and pulmonary involvement (p < 0.001), and between MDA-5 positivity and calcinosis (p = 0.018). Infectious complications were observed in 13 patients, and malignancy developed in 3 patients (cervical, ovarian, and colorectal carcinoma). Although the frequency of MSA subgroups in our cohort was consistent with the literature, further studies with larger patient populations are needed to evaluate the relationship

Anahtar Kelimeler

• Inflammatory myopathies
• myositis specific antibodies
• clinical manifestation
• prognosis

Kaynakça

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