Henoch-Schönlein purpura pathophysiology: Difference between revisions

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==Pathophysiology==
==Pathophysiology==
HSP can develop after infections with [[streptococcus|streptococci]] ([[Streptococcus pyogenes|β-haemolytic, Lancefield group A]]), [[hepatitis B]], [[herpes simplex virus]], [[parvovirus B19]], [[Coxsackievirus]], [[adenovirus]], ''[[Helicobacter pylori]]'', [[measles]], [[mumps]], [[rubella]], [[mycoplasma]] and numerous others. Drugs linked to HSP, usually as an idiosyncratic reaction, include [[vancomycin]], [[ranitidine]], [[streptokinase]], [[cefuroxime]], [[diclofenac]], [[enalapril]] and [[captopril]]. Several diseases have been reported to be associated with HSP, often without a causative link. Only in about 35% of cases can HSP be traced to any of these causes.
The exact cause of HSP is unknown, but most of its features are due to the deposition of abnormal antibodies in the wall of blood vessels, leading to [[vasculitis]]. These antibodies are of the subclass IgA<sub>1</sub> in [[polymer]]s; it is uncertain whether the main cause is overproduction (in the digestive tract or the [[bone marrow]]) or decreased removal of abnormal IgA from the circulation. It is suspected that abnormalities in the IgA<sub>1</sub> molecule may provide an explanation for its abnormal behaviour in both HSP and the related condition [[IgA nephropathy]]. One of the  characteristics of IgA<sub>1</sub> (and [[IgD]]) is the presence of an 18 [[amino acid]]-long ''hinge region'' between [[complement system|complement]]-fixating region 1 and 2. Of the amino acids, half is [[proline]], while the other ones are mainly [[serine]] and [[threonine]]. The majority of the serines and the threonines have elaborate sugar chains, connected through [[oxygen]] atoms ([[glycosylation#O-linked glycosylation|O-glycosylation]]). This process is thought to stabilise the IgA molecule and make it less prone to [[proteolysis]]. The first sugar is always [[N-acetyl-galactosamine]] (GalNAc), followed by other [[galactose]]s and [[sialic acid]]. In HSP and IgAN, it appears that these sugar chains are deficient. The exact reason for these abnormalities are not known.


==References==
==References==


{{reflist|2}}
{{reflist|2}}


[[Category:Disease]]
[[Category:Disease]]

Revision as of 01:54, 6 February 2018