Understanding Igg4 Related Orbital Disease: A Rheumatologist's Perspective

Öz

Immunoglobulin G4-related disease (IgG4-RD) is also known as IgG4-related systemic disease, hyper-IgG4 disease, IgG4-related autoimmune disease. It is a multi-organ, fibro-inflammatory condition with tumefactive lesions of unknown etiology and characteristic histopathological features. It can affect all orbital tissues, including the lacrimal glands and extraocular muscles. A 52-year-old female patient, presented with complaints of swelling above her right eye. After undergoing a contrast-enhanced MRI, she had a biopsy. Consequently, the pathological findings were reported as consistent with IgG4-related sclerosing disease. The patient was initially prescribed corticosteroid therapy, followed by methotrexate and azathioprine treatment, which successfully stabilized her condition. Since the disease can affect almost all organs and systems, it is crucial for clinicians to have knowledge about early diagnosis and employ a multidisciplinary approach in patient management.

Anahtar Kelimeler

• IgG4 related disease
• IgG4 related orbital disease
• IgG4
• eye

Olgu Romatoloji Pratiğinde IgG4 ilişkili Orbital Hastalı

Öz

İmmünoglobulin G4 ile ilişkili hastalık (IgG4-RD), IgG4 ile ilişkili sistemik hastalık, hiper-IgG4 hastalığı, IgG4 ile ilişkili otoimmün hastalık olarak da bilinir. Etiyolojisi bilinmeyen ve karakteristik histopatolojik özelliklere sahip tümefaktif lezyonlarla karakterize, birden fazla organ tutulumu ile seyredebilen, fibroinflamatuar bir durumdur. Lakrimal bezler ve göz dışı kaslar da dahil olmak üzere tüm orbita dokularını etkileyebilir. 52 yaşında kadın hasta, sağ gözünün üstünde şişlik şikayetiyle başvurdu. Kontrastlı MR çektirdikten sonra biyopsi yapıldı. Sonuç olarak patolojik bulguların IgG4'e bağlı sklerozan hastalık ile uyumlu olduğu bildirildi. Hastaya başlangıçta kortikosteroid tedavisi verildi, ardından metotreksat ve azatiyoprin tedavisi uygulandı ve bu tedavi, durumunu başarılı bir şekilde stabilize etti. Klinik pratikte bu hastalık neredeyse tüm organ ve sistemleri etkileyebildiğinden, klinisyenlerin erken tanı konusunda bilgi sahibi olması ve hasta yönetiminde multidisipliner bir yaklaşım benimsemesi büyük önem taşımaktadır.

Anahtar Kelimeler

• Igg4 ilişkili hastalık
• göz
• ıgg4 ilişkili orbital hastalık
• igg4

Kaynakça

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