Abstract

Purpose: To report a biopsy-proven case of granulomatosis with polyangiitis (GPA) who visited for red eye and discomfort. We detected papillary thyroid cancer by a whole-body scan.Case summary: A 56-year-old female presented to our clinic with hyperemia and discomfort in the right eye for the past 1 month. The visual acuity was 0.9 in the right eye. Congestion and chemosis of the right conjunctiva were observed by slit-lamp microscopy. There was a firm, erythematous, tender mass near the right nasolacrimal duct. Impairment of adduction and infraduction and proptosis were observed. Radiologically, a 3.6 × 2.6 × 1.2 cm right orbital mass was seen in the intraorbital extraconal space, invading the right nasolacrimal duct and nasal cavity. Pathologic and supplementary investigations showed granulomatous, c-anti-neutrophil cytoplasmic antibodies (ANCA)-positive inflammation in the mass. The patient was diagnosed with GPA. Whole-body computed tomography and positron emission tomography-computed tomography revealed multiple lesions in the right orbit, nasal cavity, mediastinum, retropubic area, and left thyroid gland. Thyroid ultrasonography and biopsy led to a diagnosis of papillary carcinoma with lymph node metastasis. Topical and systemic steroids and rituximab injection were administered, and total thyroidectomy with bilateral central lymph node dissection was performed. This led to improvements in proptosis and limitation of extraocular movement.Conclusions: GPA is a rare disease that may initially present with ocular symptoms. It can be associated with malignancies even in the absence of immunosuppression. Patients with ocular problems suspected of having GPA should undergo autoimmune antibody testing, including ANCA and tissue biopsy.

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