Clinical Characteristics and Treatment Management of Patients with Dermatomyositis and Polymyositis: A Single Center Experience

Öz

Dermatomyositis (DM) and polymyositis (PM) are systemic diseases featuring striated muscle inflammation as well as other autoimmune diseases or malignancy. Here, we aimed to investigate the demographic characteristics, organ involvement, clinical course of patients who were followed up in our center with the diagnosis of DM and PM. The records of 69 patients who were followed up in our center between January 1990 and December 2022 with the diagnosis of DM and PM according to the criteria of Bohan and Peter, were retrospectively reviewed. 85% of patients with DM and 70% of patients with PM were women. The mean age at presentation was 41.5 ± 13.5 years in patients with DM and 48.2 ± 13.8 years in patients with PM. Gottron papules were the most common skin manifestation. While some patients had lung involvement and dysphagia, there was no cardiac involvement in any patient.A greater proportion (73.9%) of DM patients were anti-nuclear antibody (ANA) positive (p<0,01) and anti Jo-1 antibody was associated with ILD (p<0,01). Malignancy-related myopathy was seen in 13% of patients. Age at diagnosis, duration of follow-up and malignancy were found to be associated with mortality in univariate and multivariate Cox regression analysis. The course of disease in our patients showed many similarities with other studies, but also striking differences. This may be due to genetic and environmental factors and screening conditions as well. Prospective multicenter studies are needed to clarify the associations of DM/PM prognosis with these possible factors and to confirm the current results.

Anahtar Kelimeler

• Keywords: Autoantibody
• Dermatomyositis
• Malignity
• Mortality
• Polymyositis.

Dermatomiyozit ve Polimiyozit Tanılarıyla İzlediğimiz Hastaların Klinik Özellikleri ve Tedavi Yönetimi: Tek Merkez Deneyimi

Öz

Dermatomiyozit (DM) ve polimiyozit (PM) çizgili kas enflamasyonunun yanısıra diğer otoimmün hastalıklar veya malignitenin eşlik edebildiği sistemik hastalıklardır. Biz de merkezimizde takipli DM ve PM tanılı hastaların demografik özelliklerini, organ tutulumlarını, klinik seyirlerini incelemeyi amaçladık. Ocak 1990-Aralık 2022 arasında merkezimizde takipli Bohan ve Peter kriterlerini karşılayan 69 DM ve PM tanılı hastanın kayıtları retrospektif olarak incelendi. DM tanılı hastaların %85’i, PM tanılı hastaların %70’i kadındı. Ortalama başvuru yaşı DM tanılı hastalarda 41,5 ± 13,5, PM tanılı hastalardaysa 48,2 ± 13,8 idi. Gottron papülleri görülen en sık cilt bulgusuydu. Bazı hastalarda akciğer tutulumu ve disfaji gözlenmişken kardiyak tutulum hiçbir hastada görülmemişti. DM hastalarında daha yüksek oranda (%73,9) anti nükleer antikor (ANA) pozitifliği mevcuttu (p<0,01) ve anti Jo-1 antikoru İAH ile ilişkiliydi (p<0,01). Hastaların %13’ünde maligniteyle ilişkili miyopati görülmüştü. Tek ve çok değişkenli Cox regresyon analizinde tanı yaşı, takip süresi ve malignite mortaliteyle ilişkili bulundu. Hastalarımızın hastalık seyrinin diğer çalışmalarla birçok benzerlik yanında çarpıcı farklılıklar da göstermektedir. Bu durum genetik ve çevresel faktörlere ve tarama şartlarına bağlı olabilir. DM/PM prognozunun bu olası faktörlerle ilişkisini ortaya koymak ve mevcut sonuçları doğrulamak için çok merkezli prospektif çalışmalara ihtiyaç vardır.

Anahtar Kelimeler

• Dermatomiyozit
• Malignite
• Mortalite
• Otoantikor
• Polimiyozit.

Kaynakça

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