IgA vasculitis (formally known as Henoch-Schonlein purpura (HSP)) is a type of non-thrombocytopaenic immune-mediated small vessel acute leukocytoclastic vasculitis.
It tends to occur in the paediatric population (peak incidence 3-10 years) 3.
In order to differentiate from other types of vasculitides, the four commonly adopted diagnostic criteria by the American College of Rheumatology are:
- age of onset less than 20 years (although some case series show disease involvement in older individuals)
- palpable skin purpura
- gastrointestinal bleeding
- Intussusception typically ileoileal
- biopsy showing granulocytes around small arteriolar/venular walls.
There is deposition of IgA immune complexes in small vessels.
Commonest organ systems which get involved are:
- skin: 95-100%, usually the first organ to get involved
- gastrointestinal tract: 50-75%
- joints: ~80% 6
- central nervous system 5,7
May show evidence of bowel wall thickening due to haemorrhage and/or evidence of bowel obstruction. plain film features are however non-specific.
Fluoroscopy: small bowel follow through
May show evidence of separation of bowel loops, "thumbprinting" and/or mucosal thickening.
May show multifocal bowel thickening, lymphadenopathy, mesenteric oedema and vascular engorgement. Although non-specific, CT findings may aid in the diagnosis of HSP in a young patient with acute gastrointestinal illness along with other, e.g. cutaneous features of HSP. Intussusception typically ileoileal can also be identified.
Echogenic kidneys, target or doughnut sign in case of intussusception. HSP has also been shown to cause massive scrotal oedema, and can be a source of severe pain in young boys 9.
The disease often tends to be self-limiting in most instances.
- small bowel intussusception
- small bowel obstruction
- small bowel infarction: 3-5% 3
- bowel perforation
History and etymology
It is named after Eduard Heinrich Henoch, German paediatrician (1820-1910) 8 and Johann Lukas Schönlein, German physician (1793-1864).
On a CT/contrast study for the bowel consider:
- 1. Johnson PT, Horton KM, Fishman EK. Case 127: Henoch-Schonlein purpura. Radiology. 2007;245 (3): 909-13. doi:10.1148/radiol.2453040999 - Pubmed citation
- 2. Jeong YK, Ha HK, Yoon CH et-al. Gastrointestinal involvement in Henoch-Schönlein syndrome: CT findings. AJR Am J Roentgenol. 1997;168 (4): 965-8. AJR Am J Roentgenol (abstract) - Pubmed citation
- 3. Ha HK, Lee SH, Rha SE et-al. Radiologic features of vasculitis involving the gastrointestinal tract. Radiographics. 20 (3): 779-94. Radiographics (full text) - Pubmed citation
- 4. Tizard EJ, Hamilton-ayres MJ. Henoch Schonlein purpura. Arch Dis Child Educ Pract Ed. 2008;93 (1): 1-8. doi:10.1136/adc.2004.066035 - Pubmed citation
- 5. Perez C, Maravi E, Olier J et-al. MR imaging of encephalopathy in adult Henoch-Shönlein purpura. AJR Am J Roentgenol. 2000;175 (3): 922-3. AJR Am J Roentgenol (full text) - Pubmed citation
- 6. Saulsbury FT. Henoch-Schönlein purpura in children. Report of 100 patients and review of the literature. Medicine (Baltimore). 1999;78 (6): 395-409. Medicine (Baltimore) (link) - Pubmed citation
- 7. Bartynski WS. Posterior reversible encephalopathy syndrome, part 1: fundamental imaging and clinical features. AJNR Am J Neuroradiol. 2008;29 (6): 1036-42. doi:10.3174/ajnr.A0928 - Pubmed citation
- 8. E. Henoch: Über den Zusammenhang von Purpura und Intestinalstörungen. Berliner Klinische Wochenschrift, 1868; 5: 517-519.
- 9. Ben-Sira L, Laor T. Severe scrotal pain in boys with Henoch-Schönlein purpura: incidence and sonography. Pediatr Radiol. 2000;30 (2): 125-8. Pubmed citation.