Essay On Igg4-Related Disease

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IgG4-related disease (IgG4-RD) is a newly recognized, multiorgan, fibro-inflammatory disease characterized by mass forming lesion with dense lymphoplasmacytic infiltration with large numbers of IgG4+ plasma cells, storiform fibrosis and sometimes obliterative phlebitis. IgG4-RD was first described in 2001 by Japanese researchers in patients with autoimmune pancreatitis with high levels of serum IgG.[1, 2]
IgG4-RD usually affects individuals of middle to old age, with an onset at 50–70 years, although few paediatric cases have been described. Males are more commonly affected especially for IgG4-related pancreatitis with an M:F ratio of 3:7. However, IgG4-related dacryoadenitis and sialadenitis may occur more frequently among
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Enlargement of one or more of trigeminal nerve branches is another feature of IgG4-ROD. The most commonly involved branches are the infraorbital nerve (ION) and the frontal nerve. Other nerves may be involved such as perioptic nerves, paravertebral nerves and great auricular nerve. The disease involves mainly the epineurium and the affected nerves are infilterated by masses of lymphocytes, plasma cells, eosinophils and large numbers of IgG+ plasma cells.[20-23]
EOM enlargement is not rare in IgG4-ROD especially in cases with enlarged orbital nerves. Single or multiple muscles may be involved during the disease course in the following order of frequency: inferior rectus, followed by superior rectus-levator complex, lateral rectus, medial rectus, inferior oblique and superior oblique. Histopathologically, the muscle biopsy shows a mixed and dense infiltration with polyclonal B- and T-cells with some fibrosis.[24, 25]
IgG4-ROD may also involve the lacrimal drainage apparatus.[26] Furthermore, sclera and conjunctival affection and nongranulomatous anterior uveitis have also been reported. [27, 28]
There is a correlation between IgG4-RD and ocular adnexal xanthogranulomatous disease in which a high

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