Henoch-Schönlein Purpura

Description

Henoch-Schonlein Purpura (HSP) or IgA Vasculitis, is the most common childhood vasculitis.

The underlying pathogenesis is uncertain however the structural end organ injury is caused by the deposition of IgA molecules due to a reduction in glycosylation. It is classified as a small vessel immune complex vasculitis.

There is a tetrad of the core clinical manifestations:

  • Purpura – Most often present on areas of pressure or gravity dependence
  • Arthritis or arthralgia
  • Abdominal Pain – Often due to small bowel involvement and can be complicated by intussusception
  • Renal involvement – It is possible for single organ deposition of IgA to occur, with IgA nephropathy not distinguishable histologically from a systemic IgA vasculitis. Renal failure is the most common long term sequelae of IgA vasculitis and can initially present with hypertension or evidence of nephrotic syndrome

IgA Vasculitis has a good prognosis and is most often self-limiting. The main stay of management is ensuring adequate hydration and analgesia, with any other complications investigated and addressed as indicated. There is often a history of a preceding infection affecting the gastrointestinal system or upper respiratory tract. Assessment should include monitoring of blood pressure and urinalysis for haematuria/proteinuria. If initially normal in an otherwise well child these are followed periodically with development of hypertension or haematuria/proteinuria prompting further investigation and management.


History

1802 – First described by William Heberden the elder, a London based physician who described a case of a 4 year old boy with all four hallmarks of HSP: gastrointestinal involvement, kidney involvement, arthritis, and purpura. Initially known as Heberden-Willan disease [Ch 78 De purpureis maculis; or ]

1837Johann Lukas Schönlein described an association between the presence of purpura and arthritis

1874 and 1899 – Eduard Heinrich Henoch, a student of Schönlein’s, added gastrointestinal and renal involvement respectively, to the description

1920 – Eduard Glanzmann reported on the allergic components of HSP as ‘Anaphylaktoide Purpura

1990 – American College of Rheumatology published diagnostic criteria for HSP including palpable purpura; age 20yrs or younger at onset; bowel angina; and wall granulocytes on biopsy. A patient requires at least 2 of these criteria for a diagnosis of HSP

2012 – The International Chapel Hill Consensus Conference Nomenclature of Vasculitides proposed replacing the term Henoch-Schonlein Purpura with the term IgA Vasculitis to better reflect the underlying pathophysiology


Associated Persons
Alternative names
  • Schönlein-Henoch Purpura
  • Schönlein–Henoch–Glanzmann Purpura
  • IgA Vasculitis
  • Heberden-Willan disease
References

eponymictionary CTA

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Emergency physician MA (Oxon) MBChB (Edin) FACEM FFSEM with a passion for rugby; medical history; medical education; and asynchronous learning #FOAMed evangelist. Co-founder and CTO of Life in the Fast lane | Eponyms | Books | Twitter |

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