Purpura, Schoenlein-Henoch

In medicine (rheumatology and pediatrics) Henoch-Schönlein purpura (HSP, also known as allergic purpura) is a form of vasculitis that mainly affects children. Its course is generally benign, although some serious complications can develop, mainly of the kidney and intestine. more...

Signs and symptoms

The purpura that are mentioned in the name of the disease are present in all patients. They are located on the legs and arms, and progress to the trunk. As purpura are small haemorrhages, they are non-blanching (i.e. they do not disappear on pressure), which can lead to confusion with the petechiae of meningococcal sepsis. Often, the symptoms are preceded by a viral upper respiratory tract infection.

Other symptoms are: arthritis (75%, non-erosive, often present before all other symptoms), abdominal pain (65%), intestinal bleeding (35%), nephritis and hematuria (40%) and proteinuria (25%). Many other symptoms have been reported, but their specificity for HSP is limited.

33% will develop recurrence of the syndrome. 1% will progress to chronic renal failure. Both recurrence and renal complications are more frequent in older children and adults.

Some patients can present with intussusception; this complication is the folding of a segment of the intestine into its own lumen, leading to hematochezia and severe abdominal pain. It requires urgent surgery, before the intestinal tissue becomes necrotic and dies.

Diagnosis

There is no definitive test for HSP, but blood tests are commonly performed. These include: full blood count, electrolytes, renal function, C-reactive protein and erythrocyte sedimentation rate. Immunoglobulin levels show elevated IgA in 50% of the cases. Complement levels are normal (in other vasculitis syndromes these can be consumed and therefore low).

Rarely, a renal biopsy has to be performed to arrive at a full diagnosis. This shows mesangial proliferation and is indistinguishable from IgA nephritis.

Pathophysiology

HSP is a form of vasculitis that is mediated by antibodies of the subclass IgA₁. These are found in the cutaneous lesions, as well as other inflammatory patches in the kidney (mesangium) and the intestine. IgA nephritis has many parallel symptoms, and is also often preceded by an infection, raising the suspicion that the nephritis may be a form of HSP limited to the kidney.

HSP can develop after infections with streptococci (Group A, β-haemolytic), hepatitis B, herpes simplex virus, parvovirus B19, Coxsackievirus, adenovirus and Helicobacter pylori. Certain medications have been reported to cause HSP as an idiosyncratic reaction.

In the 1990s, it was discovered that an abnormality of the IgA₁ may be the causative factor of this disease. IgA₁ and IgD differ from the other classes of antibody through an 18 amino acid-long hinge region between 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 (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 galactoses and sialic acid.

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