Session Type: ACR Poster Session C
Session Time: 9:00AM-11:00AM
Background/Purpose: Since first described by McDuffie et al. in 1973, hypocomplementemic urticarial vasculitis (HUV) syndrome has been recognized as a specific autoimmune disorder involving at least 6 months of urticaria with hypocomplementemia in the presence of systemic manifestations, such as arthritis/arthralgia, glomerulonephritis, uveitis and recurrent abdominal pain. Skin biopsy is essential in the diagnosis of HUV and the presence of antibodies against complement protein 1q (anti-C1q) has been judged ‘a rule without exception’. The objective of this study was to describe the clinical characteristics and epidemiology of HUV in two geographically areas of Sweden during a 16-year period.
Results: Sixteen patients (Skåne 7 + Östergötland 9) with a clear female predominance were identified during the study period. Clinical characteristics of the patients are given in Table 1. The pooled annual incidence rate per million inhabitants was estimated to 0.7 (95% CI 0.4-1.1), with a significant higher incidence in Östergötland compared to Skåne [0.5 (95% CI 0.1-0.8) and 1.3 (0.5-2.2), P=0.02]. The prevalence did not differ significantly between the regions, Table 2). Two patients died during the follow-up period. One patient underwent lung transplantation and two patients proceeded to end-stage renal disease.
| Table 1
* Ro/SSA 3; Ro/SSA+La/SSB 2
| Table 2
To cite this abstract in AMA style:Sjöwall C, Mandl T, Mohammad A. Hypocomplementemic Urticarial Vasculitis (HUV) Syndrome in Two Geographically Defined Populations of Sweden [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/hypocomplementemic-urticarial-vasculitis-huv-syndrome-in-two-geographically-defined-populations-of-sweden/. Accessed August 21, 2019.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/hypocomplementemic-urticarial-vasculitis-huv-syndrome-in-two-geographically-defined-populations-of-sweden/