IgG4 İlişkili Hastalık
Özet
IgG4 ilişkili hastalık, 2000 yılından sonra yeni bir klinik durum olarak tartışılmaya başlanan, tumor benzeri lezyonlar, storiform fibrozis, obliteratif flebit ve IgG4-pozitif plazma hücrelerinden zengin lenfoplazmositik infiltrasyon ile karakterize sistemik, fibroinflamatuar bir hastalıktır. Safra yolları, tükrük bezleri, akciğer, böbrek ve aorta gibi neredeyse tüm organları etkileyebilen bu hastalık, yakın döneme kadar otoimmün pankreatitin bir dış tutulumu olarak değerlendirilmekteydi. Mikulicz sendromu, Küttner tümörü ve Riedel tiroiditi gibi geçmişte bağımsız kabul edilen birçok sendromun aslında bu spektrumun bir parçası olduğu anlaşılmıştır. Genellikle 50 yaş üstü erkeklerde subakut başlangıç gösteren hastalık, tedavi edilmediğinde hızlıca karaciğer ve böbrek yetmezliği ile aort diseksiyonu gibi ciddi organ hasarlarına yol açabilir. Tanıda en önemli yöntem doku biyopsisi ve histopatolojik korelasyondur; serum IgG4 seviyeleri hastaların çoğunda artsa da yüzde 30'unda normal kalabilir. Tedavide ilk tercih glukokortikoidler (prednizolon) olup, idame döneminde azatioprin, mikofenolat mofetil veya metotreksat gibi ajanlar tercih edilmektedir. Dirençli veya tekrarlayan vakalarda ise B hücresini azaltarak serum IgG4 seviyelerini hızla düşüren Rituximab tedavisi ile haftalar içinde klinik düzelme sağlanabilmektedir.
IgG4-related disease is a systemic fibroinflammatory condition conceptualized after 2000, characterized by tumor-like lesions, storiform fibrosis, obliterative phlebitis, and lymphoplasmacytic infiltration rich in IgG4-positive plasma cells. Capable of affecting nearly any organ, including the biliary tract, salivary glands, lungs, kidneys, and aorta, it was previously considered an extrapancreatic manifestation of autoimmune pancreatitis. Historical entities such as Mikulicz syndrome, Küttner's tumor, and Riedel's thyroiditis are now recognized as manifestations within this disease spectrum. Predominantly affecting men over 50 with a subacute onset, the disease can lead to rapid organ failure, including liver or renal insufficiency and aortic dissection, if left untreated. Tissue biopsy combined with histopathological correlation remains the cornerstone of diagnosis; although serum IgG4 levels are elevated in most patients, they remain normal in approximately 30% of cases. Glucocorticoids (prednisolone) serve as the first-line treatment, while azathioprine, mycophenolate mofetil, or methotrexate are utilized for maintenance. For recurrent or refractory cases, Rituximab therapy achieves prompt clinical improvement within weeks by depleting B cells and rapidly lowering serum IgG4 levels.
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