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| '''For patient information click [[Henoch-Schönlein purpura (patient information)|here]]'''
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| {{Infobox_Disease | | {{Infobox_Disease |
| | Name = Henoch-Schönlein purpura | | | Name = Henoch-Schönlein purpura |
| | Image = purpura.jpg | | | Image = purpura.jpg |
| | Caption = Typical purpura on lower leg | | | Caption = Typical purpura on lower leg |
| | DiseasesDB = 5705
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| | ICD10 = {{ICD10|D|69|0|d|65}}<br>([[ILDS]] D69.010)
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| | ICD9 = {{ICD9|287.0}}
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| | ICDO =
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| | OMIM =
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| | MedlinePlus = 000425
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| | MeshID = D011695
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| }} | | }} |
| {{SI}} | | {{Henoch-Schönlein purpura}} |
| {{CMG}} | | {{CMG}} |
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| {{Editor Help}}
| | '''For patient information click [[Henoch-Schönlein purpura (patient information)|here]]''' |
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| == Overview ==
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| In [[medicine]] ([[rheumatology]] and [[pediatrics]]) '''Henoch-Schönlein purpura''' (HSP, also known as ''allergic purpura'') is a systemic [[vasculitis]] (inflammation of blood vessels) characterized by deposition of [[immune complex]]es containing the antibody [[IgA]], especially in the skin and kidney. It occurs mainly in children. Typical symptoms include palpable [[purpura]] (small hemorrhages in the skin), [[arthralgia|joint pains]] and [[abdominal pain]]. Most cases are self-limiting and require no treatment apart from symptom control, but the disease may relapse (in 33% of cases) and cause irreversible kidney damage (in 1%).<ref name=Saulsbury2001>{{cite journal |author=Saulsbury FT |title=Henoch-Schönlein purpura |journal=Curr Opin Rheumatol |volume=13 |issue=1 |pages=35–40 |year=2001 |pmid=11148713 |doi=}}</ref>
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| ==Epidemiology==
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| HSP occurs more often in children than in adults, and usually follows an [[upper respiratory tract infection]]. [[Median|Half of affected patients]] are below the age of six, and 90% under ten. It occurs more often in boys than in girls (about twice as often).<ref name=Saulsbury2001/>
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| The [[incidence (epidemiology)|incidence]] of HSP in children is about 20 per 100,000 children per year; this makes it the most common vasculitis in childhood.<ref name=Gardner-Medwin2002>{{cite journal |author=Gardner-Medwin JM, Dolezalova P, Cummins C, Southwood TR |title=Incidence of Henoch-Schönlein purpura, Kawasaki disease, and rare vasculitides in children of different ethnic origins |journal=Lancet |volume=360 |issue=9341 |pages=1197–202 |year=2002 |pmid=12401245 |doi=}}</ref>
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| == Pathophysiology & Etiology==
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| 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]]'',<ref name=Saulsbury2001/> [[measles]], [[mumps]], [[rubella]], [[mycoplasma]] and numerous others.<ref name=Rai1999/> Drugs linked to HSP, usually as an ideosyncratic 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.<ref name=Rai1999/>
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| 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.<ref name=Rai1999/> 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.<ref name=Rai1999/><ref name=Saulsbury2001/>
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| ==Diagnosis==
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| The diagnosis is based on the combination of the symptoms, as very few other diseases cause the same symptoms together. [[Blood test]]s may show elevated [[creatinine]] and [[urea]] levels (in kidney involvement), raised [[IgA]] levels (in about 50%<ref name="Rai1999"/>), and raised [[C-reactive protein]] (CRP) or [[erythrocyte sedimentation rate]] (ESR) results; none are specific for Henoch-Schönlein purpura. The [[platelet]] count may be raised, and distinguishes the purpura from diseases in which the low platelets are the cause of the purpura ([[idiopathic thrombocytopenic purpura]], [[thrombotic thrombocytopenic purpura]]).<ref name=Kraft1998/>
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| If there is doubt about the cause of the skin lesions, a [[biopsy]] of the skin may be performed to distinguish the purpura from other diseases that cause purpura (such as [[vasculitis]] due to [[cryoglobulinemia]]); on microscopy the appearances are of a [[hypersensitivity vasculitis]] and [[immunofluorescence]] demonstrates IgA and [[C3 (complement)|C3]] (a protein of the [[complement system]]) in the blood vessel wall.<ref name=Kraft1998/>
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| On the basis of symptoms, it is possible to distinguish HSP from [[hypersensitivity vasculitis]] (HV). In a series comparing 85 HSP patients with 93 HV patients, five symptoms were found to be indicative of HSP: palpable purpura, [[abdominal angina]], digestive tract hemorrhage (not due to intussussception), hematuria and age less than 20. The presence of three or more of these indicators has an 87% [[Sensitivity (tests)|sensitivity]] for predicting HSP.<ref name="pmid1613701">{{cite journal |author=Michel BA, Hunder GG, Bloch DA, Calabrese LH |title=Hypersensitivity vasculitis and Henoch-Schönlein purpura: a comparison between the 2 disorders |journal=J. Rheumatol. |volume=19 |issue=5 |pages=721–8 |year=1992 |pmid=1613701}}</ref>
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| Biopsy of the [[kidney]] may be performed both to establish the diagnosis or to assess the severity of already suspected kidney disease. The main findings on kidney biopsy are increased cells in the [[mesangium]] (part of the [[glomerulus]], where blood is filtered), [[white blood cell]]s, and the development of [[crescentic glomerulonephritis|crescents]]. The changes are indistinguishable from those observed [[IgA nephropathy]].<ref name="Rai1999">{{cite journal |author=Rai A, Nast C, Adler S |title=Henoch-Schönlein purpura nephritis |journal=J. Am. Soc. Nephrol. |volume=10 |issue=12 |pages=2637–44 |year=1999 |pmid=10589705 |url=http://jasn.asnjournals.org/cgi/content/full/10/12/2637}}</ref>
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| ===Signs and symptoms===
| | {{SK}} Anaphylactoid purpura; purpura rheumatica; Schönlein–Henoch purpura |
| ====Presentation====
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| [[Purpura]], [[arthritis]] and [[abdominal pain]] are known as the "classic triad" of Henoch-Schönlein purpura.<ref name=Kraft1998>{{cite journal |author=Kraft DM, Mckee D, Scott C |title=Henoch-Schönlein purpura: a review |journal=Am Fam Physician |volume=58 |issue=2 |pages=405–8, 411 |year=1998 |pmid=9713395 |url=http://www.aafp.org/afp/980800ap/kraft.html}}</ref> Purpura occur in all cases, joint pains and arthritis in 80%, and abdominal pain in 62%. Some include [[Gastrointestinal bleeding|gastrointestinal hemorrhage]] as a fourth criterion - this occurs in 33% of cases (sometimes but not necessarily due to [[Intussusception (medical disorder)|intussusception]]).<ref name=Saulsbury1999>{{cite journal |author=Saulsbury FT |title=Henoch-Schönlein purpura in children. Report of 100 patients and review of the literature |journal=Medicine (Baltimore) |volume=78 |issue=6 |pages=395–409 |year=1999 |pmid=10575422 |doi=}}</ref> The purpura typically appear on the legs and buttocks, but may also be seen on the arms, face and trunk. The abdominal pain is [[colic]]ky in character. The joints involved tend to be the [[ankle]]s, [[knee]]s, and [[elbow]]s but arthritis in the hands and feet is possible; the arthritis is non-erosive and hence causes no permanent deformity.<ref name=Kraft1998/> 40% have evidence of [[kidney]] involvement, mainly in the form of [[hematuria]] (blood in the urine), but only a quarter will have this in sufficient quantities to be noticeable without laboratory tests.<ref name=Saulsbury1999/> Problems in other organs, such as the [[central nervous system]] (brain and spinal cord) and [[lung]]s may occur, but much less commonly than the skin, bowel and kidneys.<ref name=Saulsbury2001/>
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| The disease tends to last about 4 weeks, and then resolves spontaneously.<ref name=Saulsbury2001/>
| | == [[Henoch-Schönlein purpura overview|Overview]] == |
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| ====Renal disease==== | | == [[Henoch-Schönlein purpura historical perspective| Historical perspective]] == |
| 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). While abnormalities on urinalysis may continue for a long time, only 1% of all HSP patients develop [[chronic kidney disease]].<ref name=Saulsbury2001/> [[Hypertension]] (high blood pressure) may occur. Protein loss and high blood pressure, as well as the features on [[biopsy]] of the kidney if performed, may predict progression to advanced kidney disease. Adults are more likely than children to develop advanced kidney disease.<ref name=Saulsbury2001/><ref name=Shrestha2006>{{cite journal |author=Shrestha S, Sumingan N, Tan J, ''et al'' |title=Henoch Schönlein purpura with nephritis in adults: adverse prognostic indicators in a UK population |journal=QJM |volume=99 |issue=4 |pages=253–65 |year=2006 |pmid=16565522 |doi=10.1093/qjmed/hcl034| url=http://qjmed.oxfordjournals.org/cgi/content/full/99/4/253}}</ref>
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| === Physical Examination === | | == [[Henoch-Schönlein purpura classification| Classification]] == |
| ====Skin====
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| [[Image:Henoch-Schonlein_Purpura.jpg|thumb|200px|left|Henoch-Schonlein Purpura | |
| <ref>http://picasaweb.google.com/mcmumbi/USMLEIIImages/photo#5089143238731017602</ref>]]
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| ==Criteria== | | == [[Henoch-Schönlein purpura pathophysiology|Pathophysiology]] == |
| Multiple standards exist for defining Henoch-Schönlein purpura. These include the 1990 American College of Rheumatology (ACR) classification<ref name="pmid2202310">{{cite journal |author=Mills JA, Michel BA, Bloch DA, ''et al'' |title=The American College of Rheumatology 1990 criteria for the classification of Henoch-Schönlein purpura |journal=Arthritis Rheum. |volume=33 |issue=8 |pages=1114–21 |year=1990 |pmid=2202310 |doi=}}</ref><ref name="ACRcriteria">{{cite web |url=http://www.rheumatology.org/publications/classification/hsp.asp |title=1990 criteria for the classification of Henoch-Schönlein purpura |accessdate=2007-12-15 |format= |work=}}</ref> and the 1994 Chapel Hill Consensus Conference (CHCC).<ref name="pmid8129773">{{cite journal |author=Jennette JC, Falk RJ, Andrassy K, ''et al'' |title=Nomenclature of systemic vasculitides. Proposal of an international consensus conference |journal=Arthritis Rheum. |volume=37 |issue=2 |pages=187–92 |year=1994 |pmid=8129773 |doi=}}</ref> Some have reported the ACR criteria to be more [[Sensitivity (tests)|sensitive]] than those of the CHCC.<ref name="pmid12139664">{{cite journal |author=Murali NS, George R, John GT, ''et al'' |title=Problems of classification of Henoch Schonlein purpura: an Indian perspective |journal=Clin. Exp. Dermatol. |volume=27 |issue=4 |pages=260–3 |year=2002 |pmid=12139664}}</ref>
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| ==Treatment== | | == [[Henoch-Schönlein purpura causes|Causes]] == |
| Most patients do not receive therapy because of the high spontaneous recovery rate. [[Glucocorticoid|Steroids]] are generally avoided.<ref name=Saulsbury2001/> However, if they are given early in the disease episode, the duration of symptoms may be shortened, although abdominal pain does not improve significantly. Moreover, the changes of severe kidney problems are reduced.<ref name="pmid17974746">{{cite journal |author=Weiss PF, Feinstein JA, Luan X, Burnham JM, Feudtner C |title=Effects of corticosteroid on Henoch-Schönlein purpura: a systematic review |journal=Pediatrics |volume=120 |issue=5 |pages=1079–87 |year=2007 |pmid=17974746 |doi=10.1542/peds.2007-0667}}</ref>
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| Evidence of worsening kidney damage would normally prompt a kidney biopsy. Treatment may be indicated on the basis of the appearance of the biopsy sample; various treatments may be used, ranging from oral steroids to a combination of intravenous [[methylprednisolone]] (a potent steroid), [[cyclophosphamide]] and [[dipyridamole]] followed by prednisone. Other regimens include steroids/[[azathioprine]], and steroids/cyclophosphamide (with or without [[heparin]] and [[warfarin]]). [[Intravenous immunoglobulin]] (IVIG) is occasionally used.<ref name=Rai1999/>
| | == [[Differentiating Henoch-Schönlein purpura from other diseases|Differentiating Henoch-Schönlein purpura from other Diseases]] == |
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| ==Prognosis== | | == [[Henoch-Schönlein purpura epidemiology and demographics|Epidemiology and demographics]] == |
| ===Recovery and recurrence===
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| Overall prognosis is good in most patients, with one study showing recovery occurring in 94% and 89% of children and adults, respectively (some having needed treatment).<ref name="pmid9153547">{{cite journal |author=Blanco R, Martínez-Taboada VM, Rodríguez-Valverde V, García-Fuentes M, González-Gay MA |title=Henoch-Schönlein purpura in adulthood and childhood: two different expressions of the same syndrome |journal=Arthritis Rheum. |volume=40 |issue=5 |pages=859–64 |year=1997 |pmid=9153547 |doi=10.1002/1529-0131(199705)40:5<859::AID-ART12>3.0.CO;2-J}}</ref>
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| In children, the condition recurs in about a third of all cases and usually within the first four months after the initial attack.<ref name="Saulsbury1999"/> Recurrence is more common in older children and adults.<ref name=Saulsbury2001/>
| | == [[Henoch-Schönlein purpura risk factors|Risk Factors]] == |
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| ===Kidney involvement=== | | == [[Henoch-Schönlein purpura natural history, complications and prognosis|Natural History, Complications and Prognosis]] == |
| In adults, kidney involvement progresses to ESRD more often; in a UK series of 37 patients, 10 (27%) progressed to advanced kidney disease; proteinuria, hypertension at presentation, and pathology features (crescentic changes, interstitial fibrosis and tubular atrophy) predicted progression.<ref name=Shrestha2006/>
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| The findings on renal biopsy correlates with the severity of symptoms: asymptomatic hematuria may only have focal mesangial proliferation while those with proteinuria may have marked cellular proliferation or even crescent formation. The number of crescentic glomeruli is an important prognostic factor in determining whether the patient will develop chronic renal disease or end-stage renal disease.<ref name=Saulsbury2001/>
| | == Diagnosis == |
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| In end-stage renal disease, some progress to [[hemodialysis]] or equivalent renal replacement therapy (RRT). If a [[kidney transplant]] is found for a patient on RRT, there is a risk of about 35% over 5 years that the disease will recur in the graft (transplanted kidney), and 11% that the graft will fail completely (requiring resumption of the RRT and a further transplant).<ref name=Rai1999/>
| | [[Henoch-Schönlein purpura history and symptoms|History and Symptoms]] | [[Henoch-Schönlein purpura physical examination|Physical Examination]] | [[Henoch-Schönlein purpura laboratory findings|Laboratory Findings]] | [[Henoch-Schönlein purpura electrocardiogram|Electrocardiogram]] | [[Henoch-Schönlein purpura chest x ray|Chest X Ray]] | [[Henoch-Schönlein purpura CT|CT]] | [[Henoch-Schönlein purpura MRI|MRI]] | [[Henoch-Schönlein purpura echocardiography or ultrasound|Echocardiography or Ultrasound]] | [[Henoch-Schönlein purpura other imaging findings|Other Imaging Findings]] | [[Henoch-Schönlein purpura other diagnostic studies|Other Diagnostic Studies]] |
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| ==History== | | == Treatment == |
| The disease carries the name of [[Eduard Heinrich Henoch]] (1820-1910), a German [[pediatrics|pediatrician]], and his teacher [[Johann Lukas Schönlein]] (1793-1864), who described it in the 1860s. The English [[internal medicine|physician]] [[William Heberden]] (1710-1801) and the [[dermatology|dermatologist]] Robert Willan (1757-1812) had already described the disease in 1802 and 1808, respectively, but the name ''Heberden-Willan disease'' has fallen into disuse. [[William Osler]] would be the first to see HSP as a form of [[allergy]].<ref>{{WhoNamedIt|synd|1022|Schönlein-Henoch purpura}}</ref>
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| ==References== | | [[Henoch-Schönlein purpura medical therapy|Medical Therapy]] |
| {{reflist|2}}
| | ==Case Studies== |
| | :[[Henoch-Schönlein purpura case study one|Case #1]] |
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| {{Hematology}} | | {{Hematology}} |
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| [[Category:Mature chapter]]
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| [[de:Purpura Schönlein-Henoch]] | | [[de:Purpura Schönlein-Henoch]] |
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