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DOI: 10.1055/a-2761-3270
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From Lymph Nodes to Orbit: a Time Travel in IgG4-related Disease

Vom Lymphknoten zur Orbita: eine Zeitreise in der IgG4-assoziierten Erkrankung

Authors

  • Sophia Näther

    1   Department of Ophthalmology, University Hospital Zurich, Switzerland

  • Armin Handzic

    2   Department of Ophthalmology, Hospital of the Canton of Lucerne, Switzerland

  • Karla Chaloupka

    1   Department of Ophthalmology, University Hospital Zurich, Switzerland
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Introduction

Immunglobulin G4-related disease (IgG4-RD) is a systemic, immune-mediated fibroinflammatory condition that has emerged over the past two decades as a distinct clinical entity [1], [2], [3]. It is characterized histopathologically by dense lymphoplasmatic infiltrates rich of IgG4-positive plasma cells, storiform fibrosis, and often elevated serum IgG4 levels [1], [2], [4]. The first time IgG4-RD was identified in 2001, when Hamano et al. reported about 20 patients in Japan with autoimmune sclerosing pancreatitis and high serum levels of IgG4 [5]. Since then, there have been many reports about patients with high IgG4-levels and diverse organ involvement. Many single diseases have long been viewed as conditions confined to single organs that are now seen as part of the spectrum of IgG4-RD, i.e. Mikuliczʼs syndrome, Küttnerʼs tumor, Riedelʼs thyroiditis and Ormondʼs disease [2], [6]. The disease can affect virtually every organ system, with most common involvement of the pancreas, kidneys, lacrimal and salivary glands, retroperitoneum, orbit and lymph nodes [3], [6]. Commonly, the presentation is subacute due to the enlargement of organs and few are found incidentally during diagnostic imaging or laboratory work [1], [2]. IgG4-related autoimmune pancreatitis, retroperitoneal fibrosis and IgG4-related tubulointerstitial nephritis seem to occur more often in males, whereas head and neck involvement is more common in females [7]. In general, etiology and epidemiology knowledge is still limited [1], [3]. Orbital manifestations include dacryoadenitis, orbital masses, and extraocular muscle enlargement, which can mimic thyroid eye disease, idiopathic orbital inflammation, lymphoma, granulomatosis with polyangiitis or sarcoidosis [8], [9]. Involvement of lacrimal glands and orbital involvement occur at the rate of 17 – 23% [10], [11]. In a study of 38 patients diagnosed with chronic dacryoadenitis or orbital pseudotumor, 15 (39%) met definite criteria of IgG4-RD [12]. While IgG4-associated orbitopathy generally responds well to glucocorticoid therapy, delayed or missed diagnosis may result in irreversible tissue fibrosis and functional impairment [10], [13]. Increasing awareness among ophthalmologists is therefore crucial for timely recognition and management.

We report a diagnostically challenging case of IgG4-associated orbitopathy in a female patient, illustrating the variable clinical presentation and emphasizing the importance of interdisciplinary evaluation and histological confirmation in establishing the diagnosis. Furthermore, this case highlights the importance of re-evaluating previously inconclusive diagnostic results and to specifically watch out for systemic involvement.



Publication History

Received: 21 October 2025

Accepted: 02 December 2025

Article published online:
29 January 2026

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