4.1 Article

Hypocomplementemic urticarial vasculitis syndrome presenting with bilateral scleritis

Journal

BMJ CASE REPORTS
Volume 14, Issue 5, Pages -

Publisher

BMJ PUBLISHING GROUP
DOI: 10.1136/bcr-2020-240041

Keywords

ophthalmology; immunology; dermatology; vasculitis

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The case presented a 70-year-old male patient with a variety of symptoms and persistent hypocomplementemia and positive anti-C1q antibodies, eventually achieving disease control with mycophenolate and prednisolone.
Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare autoimmune disorder characterised by recurrent urticarial lesions and acquired hypocomplementemia with systemic manifestations. The authors present the case of a 70-year-old man who presented to the ophthalmology clinic with bilateral scleritis and ocular hypertension. He was diagnosed with HUVS after a 6-month period of bilateral scleritis, vestibulitis, significant weight loss, mononeuritis multiplex and recurrent urticarial vasculitis with pronounced persistent hypocomplementemia and the presence of anti-C1q antibodies. Disease control was eventually obtained with mycophenolate and prednisolone.

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