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IgG4-Related Disease: A Prototypical Fibroinflammatory Disease. Overview on Clinical and Therapeutic Aspects

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Systemic Fibroinflammatory Disorders

Abstract

IgG4-related disease (IgG4-RD) is a recently recognized disease covering different fibroinflammatory disorders with common histopathological findings. Virtually all organs can be involved, but the most frequently affected are the pancreas, biliary tree, salivary and lacrimal glands, retroperitoneum and aorta, kidneys, and lymph nodes. Several organ manifestations can develop simultaneously or metachronously in the same patient. Clinical and radiological presentation is usually pseudotumoral with mass-forming lesions, requiring rigorous clinicopathological examination and exclusion of alternative diagnoses, especially cancer. Histopathological abnormalities include polyclonal lymphoplasmacytic infiltration, storiform fibrosis, and obliterative phlebitis. Immunohistology shows tissue infiltration by numerous IgG4-positive plasma cells. Steroids are usually dramatically effective in this condition, but relapses are frequent and second-line treatments are often necessary. An overview on main clinical and therapeutic aspects of the disease is proposed in this chapter.

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Ebbo, M., Grados, A., Schleinitz, N. (2017). IgG4-Related Disease: A Prototypical Fibroinflammatory Disease. Overview on Clinical and Therapeutic Aspects. In: Vaglio, A. (eds) Systemic Fibroinflammatory Disorders. Rare Diseases of the Immune System. Springer, Cham. https://doi.org/10.1007/978-3-319-41349-5_4

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