Case Report: Teprotumumab treatment in an HIV-infected patient with refractory thyroid eye disease: clinical and quantitative MRI evidence.

BACKGROUND

People living with human immunodeficiency virus (HIV) may develop Graves' disease as a delayed autoimmune manifestation of immune reconstitution inflammatory syndrome following antiretroviral therapy. Thyroid eye disease (TED) is uncommon in this setting. Teprotumumab targets the insulin-like growth factor-1 receptor (IGF-1R) and inhibits orbital fibroblast activation; however, its use in patients with HIV-associated TED has not previously been reported.

CASE PRESENTATION: A 37-year-old man with HIV infection developed Graves' hyperthyroidism and active moderate-to-severe TED five years after HIV diagnosis. Disease activity recurred after intravenous glucocorticoids and tocilizumab and persisted despite bilateral orbital decompression. At initial presentation, the CD4+ T-cell count was 229 cells/μL, the CD4/CD8 ratio was 0.48, and the thyrotropin receptor antibody level was elevated.

RESULTS

After three teprotumumab infusions, the clinical activity score decreased from 3/7 to 1/7 in both eyes. Proptosis decreased from 21 mm in the right eye and 22 mm in the left eye to 20 mm bilaterally, and extraocular motility and diplopia improved. Quantitative magnetic resonance imaging (MRI) showed reductions in medial rectus diameter and apparent diffusion coefficient values. Systemic immune recovery remained incomplete, but no opportunistic infections, HIV-related clinical deterioration, infusion reactions, or clinically evident hearing impairment were observed.

CONCLUSION

This case suggests that teprotumumab may improve clinical and imaging outcomes in selected patients with refractory HIV-associated TED despite incomplete systemic immune recovery. The relative dissociation between orbital improvement and conventional systemic immune indices suggests that local IGF-1R-dependent mechanisms may contribute to treatment response. Further studies are required to determine long-term efficacy and safety.