Orbit (Amsterdam, Netherlands) · 2026
Exploring the intersection: adult orbital xanthogranulomatous disease and IgG4-related disease - report of 3 cases and literature review.
СтатьяКлинический случайОбзорНазвание и аннотация: Перевод в очереди
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Barros da Silva Patrícia, Duarte Ana, Almadhi Nada H, Alazzaz Rand, Borges Alexandra, Cabeçadas José, Nunes Albertina, Paridaens Dion
Аннотация
PURPOSE: To examine the clinical, radiologic, and histopathologic overlap between adult orbital xanthogranulomatous disease (AOXD) and IgG4-related disease (IgG4-RD)/IgG4-related ophthalmic disease (IgG4-ROD), illustrated by three representative cases and a literature review.
MATERIALS AND METHODS: We retrospectively characterized three patients with AOXD, documenting presentation, imaging, histopathology, serum IgG4 levels, systemic associations, and treatment responses. A targeted narrative review appraised shared features, proposed diagnostic criteria, and evidence of overlap between AOXD and IgG4-ROD. We applied the 2019 ACR/EULAR classification criteria for IgG4-RD and ophthalmic criteria for IgG4-ROD.
RESULTS: All three patients had progressive, painless periocular swelling with yellow-orange lesions and variable proptosis, consistent with AOXD subtypes. Imaging showed eyelid/anterior orbital and lacrimal gland/EOM involvement, patterns reported in AOXD and IgG4-RD. Histopathology showed xanthogranulomatous inflammation with foamy histiocytes/Touton giant cells; immunohistochemistry demonstrated increased, but variable, numbers of IgG4+ plasma cells; serum IgG4 ranged from normal to markedly elevated. Corticosteroids achieved partial responses, with relapses requiring steroid-sparing therapy or B-cell depletion (rituximab) in two cases. Literature synthesis suggests that IgG4-rich inflammation can accompany AOXD without fulfilling systemic IgG4-RD criteria; true coexistence of both conditions appears less common when modern criteria are applied.
CONCLUSION: AOXD and IgG4-ROD share overlapping clinical, imaging, and histopathologic features. Comprehensive assessment - including serum IgG4, tissue IgG4+ plasma cell counts, and systemic organ involvement - is recommended to avoid misclassification. The role of IgG4 in AOXD remains unresolved; evolving criteria and mechanistic studies are needed to distinguish a true pathologic overlap from an epiphenomenon. Corticosteroids remain first-line, with selected cases benefitting from steroid-sparing agents.
MATERIALS AND METHODS: We retrospectively characterized three patients with AOXD, documenting presentation, imaging, histopathology, serum IgG4 levels, systemic associations, and treatment responses. A targeted narrative review appraised shared features, proposed diagnostic criteria, and evidence of overlap between AOXD and IgG4-ROD. We applied the 2019 ACR/EULAR classification criteria for IgG4-RD and ophthalmic criteria for IgG4-ROD.
RESULTS: All three patients had progressive, painless periocular swelling with yellow-orange lesions and variable proptosis, consistent with AOXD subtypes. Imaging showed eyelid/anterior orbital and lacrimal gland/EOM involvement, patterns reported in AOXD and IgG4-RD. Histopathology showed xanthogranulomatous inflammation with foamy histiocytes/Touton giant cells; immunohistochemistry demonstrated increased, but variable, numbers of IgG4+ plasma cells; serum IgG4 ranged from normal to markedly elevated. Corticosteroids achieved partial responses, with relapses requiring steroid-sparing therapy or B-cell depletion (rituximab) in two cases. Literature synthesis suggests that IgG4-rich inflammation can accompany AOXD without fulfilling systemic IgG4-RD criteria; true coexistence of both conditions appears less common when modern criteria are applied.
CONCLUSION: AOXD and IgG4-ROD share overlapping clinical, imaging, and histopathologic features. Comprehensive assessment - including serum IgG4, tissue IgG4+ plasma cell counts, and systemic organ involvement - is recommended to avoid misclassification. The role of IgG4 in AOXD remains unresolved; evolving criteria and mechanistic studies are needed to distinguish a true pathologic overlap from an epiphenomenon. Corticosteroids remain first-line, with selected cases benefitting from steroid-sparing agents.