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Epidemiology of hypocomplementaemic urticarial vasculitis (anti-C1q vasculitis)

Sjöwall, Christopher; Mandl, Thomas LU ; Skattum, Lillemor Melander LU ; Olsson, Martin LU and Mohammad, Aladdin LU (2018) In Rheumatology (Oxford, England) p.1400-1407
Abstract
Objectives

The aim was to describe the clinical characteristics and epidemiology of hypocomplementaemic urticarial vasculitis (HUV; anti-C1q vasculitis) in two geographically defined areas of Sweden.
Methods

In the health-care districts surrounding Skåne University Hospital (mean population 950 560) and Linköping University Hospital (mean population 428 503), all incident cases of HUV residing within the study areas at the onset of disease were identified during the years 2000–15. The diagnosis of HUV was confirmed by review of medical records. Only patients meeting the proposed diagnostic HUV criteria and/or the 2012 Chapel Hill consensus definitions in combination with an ever-positive anti-C1q antibody test were... (More)
Objectives

The aim was to describe the clinical characteristics and epidemiology of hypocomplementaemic urticarial vasculitis (HUV; anti-C1q vasculitis) in two geographically defined areas of Sweden.
Methods

In the health-care districts surrounding Skåne University Hospital (mean population 950 560) and Linköping University Hospital (mean population 428 503), all incident cases of HUV residing within the study areas at the onset of disease were identified during the years 2000–15. The diagnosis of HUV was confirmed by review of medical records. Only patients meeting the proposed diagnostic HUV criteria and/or the 2012 Chapel Hill consensus definitions in combination with an ever-positive anti-C1q antibody test were included.
Results

Sixteen patients (14 females) were identified during the study period. The median (interquartile range) age at diagnosis was 51 (40.7–56.7) years. Median (interquartile range) time of follow-up from diagnosis to 31 December 2015, or death, was 94 (46.5–136.2) months. The most frequent manifestations at diagnosis were urticaria (100%), arthritis (88%), followed by biopsy-proven glomerulonephritis (19%), episcleritis/scleritis (19%) and recurrent abdominal pain (13%). The annual incidence rate per million inhabitants was estimated as 0.7 (95% CI: 0.4, 1.1). Sixty-three per cent suffered from pulmonary disease at the last follow-up. Two patients died during the follow-up period. One patient underwent lung transplantation, and two patients proceeded to end-stage renal disease. The point prevalence on 31 December 2015 was 9.5/million (95% CI: 4.5, 14.5).
Conclusion

Hypocomplementaemic urticarial vasculitis constitutes a rare, but not always benign condition. Renal and lung manifestations were severe in some cases, highlighting the need for careful screening and monitoring of this potentially serious condition. (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
Rheumatology (Oxford, England)
pages
1400 - 1407
publisher
Oxford University Press
external identifiers
  • scopus:85051176083
ISSN
1462-0332
DOI
10.1093/rheumatology/key110
language
English
LU publication?
yes
id
c605a8f5-e9f6-437b-ae2e-a33976db5522
date added to LUP
2018-05-01 18:59:48
date last changed
2018-11-11 05:02:03
@article{c605a8f5-e9f6-437b-ae2e-a33976db5522,
  abstract     = {Objectives<br/><br/>The aim was to describe the clinical characteristics and epidemiology of hypocomplementaemic urticarial vasculitis (HUV; anti-C1q vasculitis) in two geographically defined areas of Sweden.<br/>Methods<br/><br/>In the health-care districts surrounding Skåne University Hospital (mean population 950 560) and Linköping University Hospital (mean population 428 503), all incident cases of HUV residing within the study areas at the onset of disease were identified during the years 2000–15. The diagnosis of HUV was confirmed by review of medical records. Only patients meeting the proposed diagnostic HUV criteria and/or the 2012 Chapel Hill consensus definitions in combination with an ever-positive anti-C1q antibody test were included.<br/>Results<br/><br/>Sixteen patients (14 females) were identified during the study period. The median (interquartile range) age at diagnosis was 51 (40.7–56.7) years. Median (interquartile range) time of follow-up from diagnosis to 31 December 2015, or death, was 94 (46.5–136.2) months. The most frequent manifestations at diagnosis were urticaria (100%), arthritis (88%), followed by biopsy-proven glomerulonephritis (19%), episcleritis/scleritis (19%) and recurrent abdominal pain (13%). The annual incidence rate per million inhabitants was estimated as 0.7 (95% CI: 0.4, 1.1). Sixty-three per cent suffered from pulmonary disease at the last follow-up. Two patients died during the follow-up period. One patient underwent lung transplantation, and two patients proceeded to end-stage renal disease. The point prevalence on 31 December 2015 was 9.5/million (95% CI: 4.5, 14.5).<br/>Conclusion<br/><br/>Hypocomplementaemic urticarial vasculitis constitutes a rare, but not always benign condition. Renal and lung manifestations were severe in some cases, highlighting the need for careful screening and monitoring of this potentially serious condition.},
  author       = {Sjöwall, Christopher and Mandl, Thomas and Skattum, Lillemor Melander and Olsson, Martin and Mohammad, Aladdin},
  issn         = {1462-0332},
  language     = {eng},
  month        = {04},
  pages        = {1400--1407},
  publisher    = {Oxford University Press},
  series       = {Rheumatology (Oxford, England)},
  title        = {Epidemiology of hypocomplementaemic urticarial vasculitis (anti-C1q vasculitis)},
  url          = {http://dx.doi.org/10.1093/rheumatology/key110},
  year         = {2018},
}