IgG4-Related Disease
Özet
IgG4-related disease (IgG4-RD) is a systemic inflammatory disorder that affects multiple organs, including the pancreas, bile ducts, salivary and lacrimal glands, kidneys, lungs, and cardiovascular system. The disease is characterized by fibroinflammatory lesions and lymphoplasmacytic infiltration. Clinical manifestations include obstructive jaundice, interstitial nephritis, dacryoadenitis, sclerosing cholangitis, and retroperitoneal fibrosis. Cardiac involvement may present as pericarditis, aortitis, pseudotumors in the coronary arteries, and inflammatory aneurysms, which can lead to myocardial ischemia and heart failure. Diagnosis is based on clinical evaluation, imaging (CT, MRI, PET), and histopathological examination. Serum IgG4 levels can support the diagnosis but are not specific. Glucocorticoids are the first-line treatment and are effective in most cases. Biologic agents, such as rituximab, targeting B cells may be used in refractory or recurrent cases. The disease can lead to progressive fibrosis, making early diagnosis and treatment crucial for preventing organ damage.
IgG4 ilişkili hastalık (IgG4-RD), sistemik inflamatuar bir hastalık olup pankreas, safra yolları, tükrük ve lakrimal bezler, böbrekler, akciğerler ve kardiyovasküler sistem dahil birçok organı etkileyebilir. Hastalık, fibroinflamatuar lezyonlar ve lenfoplazmositik infiltrasyon ile karakterizedir. Klinik bulgular arasında obstrüktif sarılık, interstisyel nefrit, dakriyoadenit, sklerozan kolanjit ve retroperitoneal fibrozis bulunur. Kardiyak tutulumda perikardit, aortit, koroner arterlerde psödötümörler ve inflamatuar anevrizmalar görülebilir. Bu lezyonlar miyokard iskemisine ve kalp yetmezliğine yol açabilir. Tanı, klinik değerlendirme, görüntüleme yöntemleri (CT, MRI, PET) ve histopatolojik inceleme ile konur. Serum IgG4 seviyeleri tanıyı destekleyebilir ancak spesifik değildir. Glukokortikoidler temel tedavi seçeneğidir ve çoğu hastada iyileşme sağlar. Rituksimab gibi B hücrelerini hedefleyen biyolojik ajanlar, dirençli veya tekrarlayan olgularda kullanılabilir. Hastalık ilerleyici fibrozise neden olabilir ve erken tanı ile tedavi, organ hasarını önlemede kritik öneme sahiptir.
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