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Images in rheumatology
More than meets the eye
  1. Elise Siegert1,
  2. Werner Stenzel2,
  3. David Sinan Koca3,
  4. Gerhard Krönke1,
  5. Robert Biesen1
  1. 1Department of Rheumatology and Clincal Immunology, Charite Universitatsmedizin Berlin, Berlin, Germany
  2. 2Department of Neuropathology, Charite Universitatsmedizin Berlin, Berlin, Germany
  3. 3Department of Ophthalmology, Charite Universitatsmedizin Berlin, Berlin, Germany
  1. Correspondence to Dr Robert Biesen; robert.biesen{at}charite.de

https://doi.org/10.1136/ard-2024-226168

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    A 28-year-old man presented with a 4-month history of a prednisolone-sensitive, painful and progressive right-sided exophthalmos and consecutive diplopia. On examination, the ophthalmologist suspected a pseudotumour of the right eye socket with lid closure defect, hypertropia and exotropia and protrusion bulbi with an intraocular pressure of 21 mm Hg (range; 10–21 mm Hg) and no signs of compressive optic neuropathy (cup-to-disc ratio of 0.4; ≤ 0.7) (figure 1a). Antiproteinase-3-antibodies were found both by positive immunofluorescence and by ELISA (165 U/mL; <10 U/mL). An MRI with a gadolinium-based contrast agent identified a destructive mass within the right eye socket (figure 1b). A subsequent debulking and biopsy confirmed small-vessel vasculitis (figure 1c) and ruled out IgG4-related disease, sarcoidosis, tuberculosis, orbital cellulitis, thyroid-associated orbitopathy or rhabdomyosarcoma as differential diagnoses of orbital pseudotumour.

    Figure 1
    Figure 1

    Clinical findings with (a) pseudotumour of the right orbit with lid closure defect, hypertropia and exotropia and protrusion bulbi, (b) corresponding MRI examination with a gadolinium-based contrast agent and (c) histologically proven small-vessel vasculitis.

    History and physical examination were otherwise unremarkable with no signs of sinusitis, nose bleeds or stuffiness. Creatinine and urine analysis showed no evidence of renal involvement, and chest CT and pulmonary function tests showed no evidence of pulmonary involvement. Hence, we confirmed this unilateral ocular manifestation to be an isolated initial manifestation of a granulomatosis with polyangiitis. A course of high-dose methylprednisone and rituximab infusions (500 mg every 6 months) resulted in a sustained reduction in the size of the pseudotumour and a clinical improvement of the patient’s diplopia, pain and lid closure defect over the follow-up of 12 months.

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    Footnotes

    • Handling editor Josef S Smolen

    • Collaborators Not applicable.

    • Contributors ES, GK and RB took over the clinical care. WS is the neuropathologist who contributed the histology and DSK is the cotreatment ophthalmologist. All authors were involved in the correction and improvement of the manuscript. RB is the guarantor.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Provenance and peer review Not commissioned; externally peer reviewed.