To distinguish IgG4-related disease from seronegative granulomatosis with polyangiitis

Rheumatology key messageIgG4-related disease might be an alternative diagnosis in limited granulomatosis with polyangiitis. Sir, IgG4-related disease (IgG4-RD) is a fibro-inflammatory disease that can affect almost every organ [1]. Indeed, previous unexplained conditions have now been reclassified as primarily IgG4-RD and may imitate many inflammatory, infectious and malignant disorders often leading to a delay in diagnosis or incorrect diagnosis. Nasal manifestation of IgG4-RD, mostly of the paranasal sinuses, has previously been described in case reports, but it can also manifest as a primary or secondary nasal disease such as chronic sinusitis and paranasal sinusitis with dacryoadenitis [2]. Since IgG4-RD localized in the nasal or orbital region remarkably resembles limited granulomatosis with polyangiitis (GPA) [3], IgG4-RD could be an alternative diagnosis in ANCA negative limited GPA. After revising the diagnosis to IgG4-RD in patient 1, we re-evaluated two other ANCA negative patients from a funded database with similar clinical features and could alter the diagnosis into IgG4-RD (Table 1). The histomorphological features matching IgG4-RD and absence of evident features of GPA were the reasons for immunohistochemical analysis in these cases leading to the diagnosis of IgG4-RD.Table 1Patient characteristicsCase 1Case 2Case 3GenderMaleMaleFemaleAge53 years73 years50 yearsMedical historyChronic spontaneous urticarial disease Unilateral orbital swelling without a definite ...
Source: Rheumatology - Category: Rheumatology Source Type: research

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