|
|
|
Henoch–Schönlein purpura
Henoch–Schönlein purpura (HSP)also known as IgA vasculitis, anaphylactoid purpura, purpura rheumatica, and Schönlein–Henoch purpura, is a disease of the skin, mucous membranes, and sometimes other organs that most commonly affects children.
In the skin, the disease causes palpable purpura (small hemorrhages), often
with joint pain and abdominal pain.
HSP is a systemic vasculitis (inflammation of blood vessels) and is characterized
by deposition of immune complexes containing the antibody immunoglobulin
A (IgA); the exact cause for this phenomenon is unknown.
It usually resolves within several weeks and requires no treatment apart from symptom control, but may relapse in a third of cases and cause irreversible kidney damage in about one in a hundred cases.
Purpura, arthritis and abdominal pain are known as the "classic triad" of Henoch–Schönlein purpura. Purpura occur in all cases, joint pains and arthritis in 80%, and abdominal pain in 62%.
Problems in other organs, such as the central nervous system (brain and
spinal cord) and lungs may occur, but is much less common than in the skin,
bowel and kidneys.
Of the 40% of patients who develop kidney involvement, almost all have
evidence (visible or on urinalysis) of blood in the urine. More than half
also have proteinuria (protein in the urine), which in one eighth is severe
enough to cause nephrotic syndrome (generalised swelling due to low protein
content of the blood).
Henoch–Schönlein purpura is a small-vessel vasculitis in which complexes
of immunoglobulin A (IgA) and complement component 3 (C3) are deposited
on arterioles, capillaries, and venules. As with IgA nephropathy, serum
levels of IgA are high in HSP.
Blood tests may show raised IgA levels (in about 50%[8]), and raised C-reactive
protein (CRP) or erythrocyte sedimentation rate (ESR) .
The platelet count may be raised, and distinguishes it from diseases where low platelets are the cause of the purpura, such as idiopathic thrombocytopenic purpura and thrombotic thrombocytopenic purpura.
In adults, kidney involvement progresses to end-stage renal disease (ESRD) more often than in children.
About 20% of children that exhibit nephrotic or nephritic features experience long permanent renal impairment.
HSP occurs more often in children than in adults, and usually follows an upper respiratory tract infection. The incidence of HSP in children is about 20 per 100,000 children per
year, making it the most common vasculitis in children.
HSP can develop after infections with mycoplasma.
Henoch–Schönlein purpura and Mycoplasma Infectious Diseases (MID)
Henoch-Schönlein purpura associated with Mycoplasma pneumoniae infection.(2011)
http://www.ncbi.nlm.nih.gov/pubmed/21838081
Mycoplasma pneumoniae-associated Henoch-Schönlein purpura nephritis.(1999)
http://www.ncbi.nlm.nih.gov/pubmed/10671029
Mycoplasma pneumoniae infection associated with Henoch-Schonlein purpura.(1988)
http://www.ncbi.nlm.nih.gov/pubmed/3397582 |
|
|