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Cardiovascular, thromboembolic and renal outcomes in IgA vasculitis (Henoch-Schönlein purpura): a retrospective cohort study using routinely collected primary care data
  1. Alexander Tracy1,
  2. Anuradhaa Subramanian2,
  3. Nicola J Adderley2,
  4. Paul Cockwell3,
  5. Charles Ferro3,
  6. Simon Ball3,
  7. Lorraine Harper1,
  8. Krishnarajah Nirantharakumar2
  1. 1 Centre for Translational Inflammation Research, Institute of Clinical Sciences, University of Birmingham, Birmingham, UK
  2. 2 Institute of Applied Health Research, University of Birmingham, Birmingham, UK
  3. 3 Department of Renal Medicine, University Hospitals Birmingham NHS Trust, Birmingham, UK
  1. Correspondence to Dr Lorraine Harper, Centre for Translational Inflammation Research, Institute of Clinical Sciences, University of Birmingham, Birmingham B15 2WB, UK; l.harper{at}


Background IgA vasculitis (IgAV, Henoch-Schönlein purpura) is a small-vessel vasculitis most common in children but also occurring in adults. Case series have suggested that IgAV may be associated with cardiovascular disease and venous thromboembolism, but this has not been evaluated in population-based studies. Renal disease and hypertension are possible complications of the disease with unknown incidence.

Methods Using a large UK primary care database, we conducted an open retrospective matched cohort study of cardiovascular, venous thrombotic and renal outcomes in adult-onset and childhood-onset IgAV. Control participants were selected at a 2:1 ratio, matched for age and sex. Adjusted HRs (aHRs) were calculated using Cox proportional hazards models.

Results 2828 patients with adult-onset IgAV and 10 405 patients with childhood-onset IgAV were compared with age-matched and sex-matched controls. There was significantly increased risk of hypertension (adult-onset aHR 1.42, 95% CI 1.19 to 1.70, p < 0.001; childhood-onset aHR 1.52, 95% CI 1.22 to 1.89, p < 0.001) and stage G3–G5 chronic kidney disease (adult-onset aHR 1.54, 95% CI 1.23 to 1.93, p < 0.001; childhood-onset aHR 1.89, 95% CI 1.16 to 3.07, p=0.010). There was no evidence of association with ischaemic heart disease, cerebrovascular disease or venous thromboembolism. All-cause mortality was increased in the adult-onset IgAV cohort compared with controls (aHR 1.27, 95% CI 1.07 to 1.50, p=0.006).

Conclusions Patients with IgAV are at increased risk of hypertension and chronic kidney disease (CKD) compared with individuals without IgAV; analysis restricted to adult-onset IgAV patients showed increased mortality. Appropriate surveillance and risk factor modification could improve long-term outcomes in these patients.

  • inflammation
  • cardiovascular disease
  • outcomes research
  • arterial hypertension
  • systemic vasculitis
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  • LH and KN contributed equally.

  • Handling editor Josef S Smolen

  • Contributors AT, LH and KN conceived the work. AT contributed to the analysis and interpretation of data, drafting the manuscript and revising for intellectual content. AS and KN contributed to the acquisition, analysis and interpretation of data, and drafting the manuscript. NJA contributed to data analysis and interpretation, and writing the manuscript. LH contributed to drafting the manuscript and revising for intellectual content. PC, CF and SB contributed to revising the manuscript for intellectual content. All authors approved the final version to be submitted for review.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent Not required.

  • Ethics approval The THIN data collection scheme and research carried out using THIN data were approved by the NHS South-East Multicentre Research Ethics Committee in 2003; under the terms of this approval, studies must undergo independent scientific review. Approval for this analysis was obtained from the Scientific Review Committee (for the use of THIN data) in April 2018 (SRC reference number 18THIN016).

  • Provenance and peer review Not commissioned; externally peer reviewed.

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