İmmünglobülin G4- ilişkili sistemik hastalık

Öz

İmmunglobulin-G4 (IgG4) ilişkili hastalık, esas olarak herhangi bir organı aynı anda veya farklı zamanlarda tutabilen fibro-inflamatuvar bir hastalıktır. Hastalık genellikle bir tümörü taklit eden ve gözyaşı bezlerini, orbitaları, büyük tükürük bezlerini, pankreası, safra kanallarını, retroperitoneal alanı, akciğerleri, böbrekleri, aortu, meninksleri ve tiroid bezini etkileyebilen organ büyümesi ile karşımıza çıkmaktadır. Bu yeni hastalığın arkasındaki immünopatogenez henüz açıklanamamıştır. Hastalığın histopatolojik ayırt edici özellikleri arasında IgG4 pozitif plazma hücrelerinin baskın olduğu yoğun lenfoplazmositik infiltrasyonlar, storiform fibrozis ve obliteratif flebit bulunmaktadır. IgG4-ilişkili hastalık tanısı önemli bir klinik sorundur ve basit bir tanısal test yoktur. Tanıdaki en önemli sorun, IgG4-ilişkili hastalığın sıklıkla hem klinik hem de radyolojik olarak maligniteyi taklit eden bulgularla ortaya çıkabilmesidir. Bu nedenle, yanlışlıkla malignite teşhisini önlemek için IgG4-ilişkili hastalığı, etkilenen organın malign bir tümöründen (kanser veya lenfoma) doğru ve zamanında ayırmak çok önemlidir. Doğru tedavinin verilebilmesi için IgG4-ilişkili hastalığını etkilenen organın benzer inflamatuvar hastalıklarından da ayırmak gereklidir. Pek çok tanı kriterleri önerilmiş ve nihayetinde IgG4-ilişkili hastalık için kapsamlı tanı kriterleri 2011 yılında oluşturulmuştur. Hastalık genellikle steroide iyi yanıt verir, ancak bazen özellikle yüksek risk faktörleri taşıyan hastalarda nüksler görülmektedir. Bu nedenle, aktif hastalığı olanlarda, idame tedavisi veya rituksimab gibi B hücre azaltıcı ajan gerekebilmektedir.

Anahtar Kelimeler

• İmmünglobülin G4-
• IgG4 ilişkili hastalık
• storiform fibrozis
• lenfoplazmositik inflamasyon

Immunoglobulın G4-related systemic disease

Öz

IgG4-related disease (IgG4-RD) is a fibro-inflammatory disease that can involve essentially any organ simultaneously or metachronously. The disease often presents with organ enlargement that mimics a tumour and can affect the lacrimal glands, orbits, major salivary glands, pancreas, bile ducts, retroperitoneum, lungs, kidneys, aorta, pachymeninges and thyroid gland. The immunopathogenesis behind this new disease has not yet been clarified. Histopathologic hallmarks of IgG4-RD include dense lymphoplasmacytic infiltrations with a predominance of IgG4-positive plasma cells, storiform fibrosis and obliterative phlebitis. The diagnosis of IgG4-RD is a significant clinical challenge, and there is no simple diagnostic test for IgG4-RD. One problem in its diagnosis is that IgG4-RD frequently presents both clinically and radiologically with findings that mimic malignancy. It is therefore critical to differentiate IgG4-RD from a malignant tumor (cancer or lymphoma) of the affected organ in an accurate and timely manner to avoid a misdiagnosis of malignancy. It is also necessary to differentiate IgG4-RD from similar inflammatory diseases of the affected organ for application of the appropriate therapy. Diagnostic criteria for several IgG4-RDs have been proposed, and comprehensive diagnostic criteria for IgG4-RD were established in 2011. The disease usually responds well to steroids, but sometimes relapses especially in patients carrying high risk factors. Highly active diseases may therefore require maintenance therapy or B-cell depletion agent such as rituximab.

Anahtar Kelimeler

• Immunoglobulin G4
• IgG4-Related disease
• storiform fibrosis
• lymphoplasmacytic inflammation

Kaynakça

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