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English Spanish Indonesian Henoch-Scho nlein purpura (HSP) adalah yang paling umum vaskulitis masa kanak-kanak.

. Sindrom ini mengambil nama dari 2 dokter Jerman. Pada 1837, Johan Schnlein pertama kali dijelaskan beberapa kasus peliosis rheumatica atau purpura terkait dengan arthritis. Tiga puluh tahun kemudian, Edouard Henoch menggambarkan manifestasi GI, termasuk muntah, sakit perut, dan melena. Henoch-Schnlein purpura juga telah disebut sebagai rheumatica purpura, vaskulitis leukocytoclastic, dan vasculitis.1 alergi English Spanish Indonesian Gambaran pertama gangguan ini adalah mungkin bahwa seorang anak muda dengan poin'' berdarah'' atas tulang kering kakinya, sakit perut, darah dalam tinja dan urin dan menyakitkan subkutan edema, dijelaskan oleh William Heberden pada tahun 1801. Pada 1837 Johann Scho nlein menggambarkan asosiasi purpura dan nyeri sendi sebagai'' Peliosis rheumatica''. Eduard Henoch, mantan mahasiswa Scho nlein, mencatat gastrointestinal keterlibatan dalam hubungan dengan purpura dan arthritis pada 1868 dan kemudian ia mencatat Keterlibatan ginjal juga. 5

DEFINITION
English Spanish Indonesian Henoch-Schnlein purpura (HSP) adalah gangguan inflamasi yang ditandai dengan vaskulitis umum yang melibatkan pembuluh kecil kulit, saluran pencernaan, ginjal, sendi, dan, jarang, paru-paru dan SSP. Ini adalah vaskulitis yang paling umum di children.1
Epidemiologi
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Studi epidemiologis telah menunjukkan HSP memiliki kejadian tahunan sekitar 000 13.518/100 children.1 2 Meskipun ini adalah suatu kondisi yang dapat terjadi dar

Etiology of Henoch-Schoaaaaaaaaaasdfenlein purpurahhgytghiikhuyytrerttyuio;lkjhgfffcxzzzzzzzzzzzzzzpura remains unknown. However, IgA clearly plays a critical role in the immunopathogenesis of Henoch-Schoenlein purpura, as evidenced by increased serum IgA concentrations, IgA-containing circulating immune complexes, and IgA deposition in vessel walls and renal mesangium. Henoch-

Schoenlein purpura is almost exclusively associated with abnormalities involving IgA1, rather than IgA2.
The predominance of IgA1 in Henoch-Schoenlein purpura may be a consequence of abnormal glycosylation of O-linked oligosaccharides unique to the hinge region of IgA1 molecules. Although several lines of evidence suggest a genetic susceptibility to HenochSchoenlein purpura, the fundamental basis for this abnormality remains unclear. IgA aggregates or IgA complexes with complement deposited in target organs, resulting in elaboration of inflammatory mediators, including vascular prostaglandins such as prostacyclin, may play a central role in the pathogenesis of Henoch-Schoenlein purpura vasculitis. A subpopulation of human lymphocytes bears surface Fc and/or C3 receptors (complement receptor lymphocytes), which can bind circulating immune complexes or C3 generated by activation of the alternative complement pathway. Such immune complexes appear in Henoch-Schoenlein purpura and may be part of the pathogenetic mechanism. Some have speculated that an antigen stimulates the production of IgA, which, in turn, causes the vasculitis. Allergens, such as foods, horse serum, insect bites, exposure to cold, and drugs (eg, ampicillin, erythromycin, penicillin, quinidine, quinine), may precipitate the illness. Infectious causes include bacteria (eg, Haemophilus, Parainfluenzae, Mycoplasma, Legionella, Yersinia, Shigella, or Salmonella species) and viruses (eg, adenoviruses, EpsteinBarr virus [EBV], parvoviruses, varicella). Vaccines such as those against cholera, measles, paratyphoid A and B, typhoid, and yellow fever have also been implicated. Evidence supporting a direct role of herpesvirus, retrovirus, or parvovirus infection in HenochSchoenlein purpura is lacking. 1

Immunopathology
The pathogenetic mechanisms underlying HSP are poorly understood. Widespread abnormalities in IgA have been described including raised serum IgA concentrations, IgA immune complexes, IgA class antibodies such as IgA rheumatoid factor (RF),10 IgA ANCA (antineutrophil cytoplasmic antibody),11 12 and IgA AECA (antiendothelial cell antibody).13 IgA deposits are also found in skin biopsies and deposited within glomeruli. There are reports of HSP developing in patients with IgA nephropathy14 as well as simultaneous development of IgA nephropathy and HSP within sibships.15 The strikingly similar histological abnormalities of mesangial IgA deposition suggest a common immunopathogenic process. IgA and IgG ANCA mirrored disease activity in a girl with frequently relapsing disease.12AECA has been detected in patients with vasculitic disorders such as Kawasaki disease and reported to be associated with renal involvement in HSP, although complement dependent cytotoxicity to glomerular endothelial cells has not been shown.13 Complement abnormalities have been described in association with HSP: C2 deficiency, homozygous null C4 phenotypes, and C4B deficiency. Other abnormalities including

glomerular C3 and properdin deposition, low CH50 and properdin, and raised C3d concentrations in the acute phase of the disease have suggested complement activation. However, a study of three multimolecular complement activation protein complexes has failed to support a role for complement activation in HSP.16
MANIFESTASI KLINIS Henoch-Schnlein purpura (HSP) is a vasculitis (inflammation of blood vessels) that affects small blood vessels mainly in the skin, intestines, and kidneys. Symptoms can begin in children, most commonly between the ages of 4 and 7 years, soon after an upper respiratory tract infection or a streptococcal pharyngitis (sore throat infection). Children may develop arthritis (inflammation of the joints), leading to pain. A rash may start as hive-like spots (urticaria ) or small raised red spots (erythematous maculopapules ) on the legs and buttocks. Eventually these spots blend to form bigger areas of bruising (purpura ) in the skin. Children may also develop abdominal pain that can be quite severe. Children younger than 2 years with HSP are more likely to develop edema (swelling of various areas of their bodies), which is a result of leaky small blood vessels in the skin. Kidney involvement can also cause edema, hematuria (visible or microscopic blood in the urine), or proteinuria (protein in the urine).3

Diagnostic criteria
The American College of Rheumatology 1990 criteria for the classification of Henoch-Schonlein purpura
The American College of Rheumatology has developed criteria for the diagnosis of HSP. The presence of 2 or more of the following criteria has a sensitivity of 87.1% and a specificity of 87.7%: 4

Palpable purpura Age at onset <20 years Abdominal pain

biopsy evidence of granulocytes in the walls of arterioles or venules.

EULAR/PRINTO/PRES criteria for Henoch-Schnlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria [14]
Ankara 2008 classification definition: Purpura or petechiae (mandatory) with lower limb predominance and at least 1 of the 4 following criteria:

Abdominal pain Histopathology Arthritis or arthralgia

Renal involvement .