Purple Shonlein-Henoch - it most often meeting system vaskulit in child`s age, which strikes mainly shallow, not containing muscles blood vessels (capillaries, arterioly) in a skin, gastroenteric highway and buds, and also in joints.

Association of purpury with artralgiyami first in German-talking space it was described Schonlein in 1837 as «peliosis rheumatica». 37 years after him student Henoch did a report under the name «o to the special form of purpury» about 4 children with purpuroy, by abdominal`nymi colics, melena and pains in joints. The defeat of buds in form nephrite was first mentioned by him in 8th edition him «Lectures on child`s diseases» in 1895. Earliest description of boy with by the symptoms of defeats of all of the organ systems, such as a purple, stomach-ache, melena, pains in joints and gematuriey, behaves to 1801, done Britisher Heberten, which, however, did not get in continental Evropu. Why in anglo-govoryaschem space the name of disease is used upside-down last names of authors of «Henoch-schonenlein Purpura» remains not clear. Epidemiology While amount of patients with a post-streptococcus glomerulonephritis after last a year in Western Evrope goes down constantly, it does not belong to to purple Shonlein-Henoch (PSH) and to the Schoenlein-Henoch-to the nephrite (SHN). At restrospektivnom getting up of hospital charts in child`s clinics of Shtuttgarta and Berna amount annual receipts of children with (PSH) for all the time of supervision during 20 years remains permanent. Epidemiology information about PSH show easy predominance of sex of men and an obvious age-dependent peak being on age between 3 and by 9 years of life. A disease considerably more frequent develops in cold times of year, between October and by April, and almost at two third of patients in a few weeks after an infection overhead respiratory tracts. Ekstrarenal`nye of display

Skin Greater part symmetric up-diffused purpury is often limited to the feet, by feet and buttock, but also ears, nose, can be struck in rare cases, genitalii and body. Not very much widely known circumstance that almost at one fourth patients of purple can be saved during weeks and months or can to arise up relapses. Joints Almost at 75% all of children arthral symptoms are marked in form sickly edemata with limitation of motion. Foremost, the genicular are struck, elbow and skakatel`nye joints. Stomach-intestinal highway Symptoms give from the side of gastroenteric highway 66-75% patients. Except for pain in a stomach register, nausea, vomiting and melena. Size of heavy gastroenteric complications, such as an indigitation and enterobrosia in scopes PSH) makes to 5%. Even without the kidney loss of albumen at separate patients there can be gipoproteinemiya because of belok-teryayuschey enteropatii. Frequency and degree weight of intestinal symptoms considerably increased with age. Cns Neyrologicheskie complications, such as attacks of cramps, head pains or paresises rarely develop within the framework PSH. Whether there are they by investigation of vaskulita of brain vessels or it remains expression giperetenzivnoy encefalopatii not clear. All neurological displays are transient without recedivov. Urogenital`nyy highway Information about the defeat of testicles in literature hesitate between 2,4% to 38%. Obvious the sickly swelling up a little of testicles quite often is a testimony for an operative revision scrotums, where torsiya (perekrut) gidatid comes to light on occasion. Etiology and pathogeny Similarly as well as almost 200 years back, after the first description of patient with PSH, etiology of this disease remains not clear. Although in approximately at two third of patients an infection is preceded development of PSH overhead respiratory tracts, remains incomprehensible, whether it causes vaskulit. In flow of desyatiteletiy the offered suspicion on a post-streptococcus a disease to today did not get the confirmation. In literature reported about the cases of PSH after a number of infectious diseases caused by different viruses (measles, german measles, chicken-pox, by the parvovirus of V19, adenovirusom), to causal intercommunication however set it never was. After causal participation of exciters is talked by seasonal frequency and reports about local epidemics of PSH. Already W. Osler supposed allergic genesis of PSH in 1914. Comes into a question possibility as possible reason allergens of food products. Genetic factors, probably as well as at autoimmunnykh diseases, play certain role in etiology of PSH. For it especially frequent displays talk in To Asia, Europe and at the white population of North America, and also and rare cases among coloured in Africa and USA, although take a place considerably more frequent for them glomerulonefrity other etiologiy. Weak association with hla-fenotipom of Bw35 and Dr4, and the considerably conditioned deficit of factor of komplementa of 4v is also specified also on genetic predisposition to PSH. At appearance of new results of research of etiology of PSH fully possible as presented on a fig. a 1 model of flow of disease of SHN. After an antigen contact there is an increase of Th2-t-limfocitov on a mucous membrane. Whether has relation this antigen to to by viruses or unknown bacteria. At the troop landing of interleykina-4 and -5 limfocity is directed on obrzovanie IGG and IGA, at what to on by greater part speech goes about IGA Anti-FcIgG-bodies which also Iga-revmofaktor is named. Then appearing immune complexes are put aside in buds cause local infiltration monocitami and makrofagami. They produce citokin and also interleykin of 1b, tumor-nekrotiziruyuschiey factor and factor Willebrand, which cause inflammations in a glomerule reaction, proliferacii, and also formation of fibrin. Many aspects of flow of disease SHN remain not clear, as well as question, what value has IGE and exposed activating of eozinofilov. Further not clearly as takes a place education immune complex without that was found out activating of the system of komplementa at PSH. Defeat of buds Levels of frequency of defeat of buds are scope 20-100% . Considerable the breadth of vibrations is related to the different applied diagnostic criteria, and also by a circumstance that the defeat of buds often develops the last from all organ displays, quite often to in 4 weeks after remissii of ekstrarenal`nykh symptoms.

In new retrospective researches retests were conducted in which urines on squirrel and blood for patients with PSH, at 40-60% children it was and defeat is exposed buds. The risk of defeat of buds is increased with age, with duration of dermic symptoms, at development of abdominal`nykh pains and finding out blood in a chair. Clinical symptoms of SHN very variabel`ny (table. 2). Mikrogematuriya with can appear the exposure of dismorfichnykh red corpuscles the unique pointing on defeat of buds and, vice versa, transiently to show up during a few days or persistirovat` during a few months or years.

Quite often patients are mark makrogematuriya which arises up only at the beginning and can recidivirovat` at infections. In rare cases isolated glomerular proteinurya is the unique symptoms of defeat of buds, and, vice versa, nephrite at PSH often shows up in form classic nefriticheskogo syndrome with protein- and gematuriey, gipertenziey and sometimes a few by the limited function buds. Nefroticheskiy syndrome with considerable proteinuriey also is rather рby a causticity.

The most heavy form of defeat of buds shows up nefriticheski-nefroticheskim by a syndrome in form rapid making progress glomerulonephritis (RPGN).  

Laboratory diagnostics On the basis of typical clinical symptoms of skin, joints and gastroenteric a highway rarely can be not recognized diagnosis of PSH. Because prognosis of disease depends only on the defeat of buds, necessary laboratory diagnostics in first turn directed on that, to estimate its degree of weight. Tests of chair must be repeatedly probed on blood, because the defeat of intestine is more frequent associates with a nephrite.

The tests of urine undertake through certain intervals, at least to disappearances of skin symptoms, for research on squirrel and red corpuscles. Because the defeat of buds often arises up only in 1-4 weeks after other organ displays, urine can be estimated and at home for patients or their parents with by the help of test strips. At a positive test on squirrel proteinuriya must to be certain in number. The function of buds must be checked up at each patient with the defeat of buds by means of method of determination of kreatin in syvorotkе and to repeat oneself at the heavy flow of disease. General maintenance of albumen in to the whey or albumen also must be determined, because along with a loss a squirrel through buds can take a place and exudative enteropatiya. Whey IgA approximately at the half of patients appears enhanceable. Immunological the conservative methods of research, for example such as komplementa, do not have diagnostic value.

Testimonies to the leadthrough of biopsy of buds Testimonies to the leadthrough of biopsy of buds (table 3) set very variously. It hardly ever does not serve for confirmation of diagnosis, but for estimations of degree of weight of glomerulonephritis. A testimony to the biopsy of buds is present at nefroticheski-nefrticheskom syndrome with RPGN. Other testimony is proceeding more than 3 months of persistruyuschaya considerable proteinuriya (> 1 g/1,73 m2 in a day). Histology If for the estimation of degree of weight of clinical simptomatiki there are no single criteria, histological changes are estimated concordantly to international classification, conducted in 70th of international researches of disease of buds for children (ISKDC, table 4). A pathologist distinguishes 6 degrees of weight, at what psevdomembrano-proliferativnyy glomerulonephritis in child`s age develops very rarely.

The most insignificant histological changes give only minimum defeats. At more high degrees of weight proliferaciya of mesangium is marked, which is or by a hearth or diffuse. By a classic find at SHN there is a mezangio-proliferativnyy glomerulonephritis. Stages II and V belong to to to formation of poluluniy, which proliferaciya of glomerular is at integumentary epithelium which is filled capsule of Bowmann and can squeeze it. Immuno-flyuorescentnaya and electronic microscopy expose a mezangial`nye depot, which mainly consist of IGA and often from the two-bits of IGG and С3. The histological picture of SHN is identical to such at an IgA-nephrite. It is not known whether there is PSH by the system form of IgA-nephrite.

Therapy SHN RPGN within the framework PSH presents a case, requiring exigent medical to the help, which is characterized a nefriticheskim syndrome and quickly - often in flow of a few days - by the worsened function of buds. Timely direction in a pediatric nefroticheskiy center for the leadthrough of biopsy of buds and has a decision value immunosupressivnoy therapy. Immunosupressivnaya therapy It was succeeded to rotin in many prospektvinykh researches, that leadthrough to intravenous steroidnoy shock therapy, by the accompanied oral summer residence prednizolona and ciklofosfamida at to 90% patients with quickly progresiruyuschim leads a glomerulonephritis the protracted renewal of function of buds, if to on therapy begins possibility early (table. 5). In a difference about RPGN for the easy forms of flow of SHN there is no promising success of medicinal treatment (table. 6). To the patients with isolated gematuriey i/ili small proteinuriey is require no therapy. Persistiruyuschaya considerable proteinuriya (>1 g/1,73 m2/in a day) presents along with by a hyperpiesis factor of risk of development of terminal kidney to insufficiency. By different immunosupressivnymi preparations, such as steroidy, khlorambucil, ciklofosfamid, azatioprin separately or in combination, in the pas arrived at no more considerable successes of treatment.  

In 1995 the French workinggroup reported about 13 adults with SHN, to which during 9 months at 14 daily intervals into muscularly entered immunoglolin-g preparation. For 12 patients they were able to look after considerable decline of proteinurii and improvement of histological displays at control to the biopsy. This form of therapy by the repeated intramuscular injections for children to this pores system not conducted certainly, that it hardly can be accepted at children. Inhibitirs АСЕ Inhibitirs of angiotenzin-konvertiruyuschego enzyme (АСЕ) along with it an antigipertenzivnym action is possessed additionally anti-proteinuricheskim and at it and by a nefroprotektivnym action. For adults with different glomerulonefritami with large proteinuriey it was meantime well-proven that they slow progress to kidney insufficiency. Whether it belongs also to the children - presently passes verification in European and American mul`tcentricheskikh researches IgA-nephrite. The same belongs to Omega-3-zhirnym acids (cod-liver oil), which in short-term researches can accelerate a nefroprotektivnoe action at kidney immune diseases, as well as for new immunosupressivnogo preparation of mikofenolatmofetil. Therapy of arterial high blood pressures Treatment of arterial high blood pressure, being a decision factor matters risk of development of terminal kidney insufficiency. By a mean choice, nesmotrya on absence of permission of application for children, there are inhibitirs АСЕ, after which diuretiki follow and antogonisty calcium. Transplanataciya buds In the case of terminal kidney insufficiency transplantation of buds is by therapy of choice. Especially within the framework of family donorship, however, in 75% cases it is necessary to count on the relapse of basic disease in transplanted organ, without that and so there is a frequent loss in child`s age transplantanta. Steroidnaya therapy for warning of nephrite It is accepted in general, that application of kortikosteroidov diminishes attacks considerably expressed abdominal`nykh pains. Whether steroidnaya therapy can at PSH remains to prevent a nephrite debatable. Saulsburry published in 1993 retrospective research on looking after 50 children with PSH, which at entering hospital in the beginning gave no signs of defeat of buds. Twenty patients on an occasion abdominal`nykh pains got steroidy, 30 without abdominal`nykh symptoms were not got. In both groups for 20% patients in further flow of disease a nephrite developed. Mollica et al. in 1992 published prospektivnom research got opposite results. From 168 children of entering hospital with PSH half at once after a receipt in 14 days got a flow steroidy. In a group where therapy was conducted not at one a nephrite did not develop from patients, in the group of not poluchavshe treatment defeat of buds it was exposed for 12 children (p<0,001). There were methodical in both researches failings, so that a question whether can primordial leadthrough of steroidnoy therapy at PSH to hinder development of nephrite, can be decided only in buduschi mul`ticentricheskikh researches.

Other aspect which also must be studied is warning indigitations by setting of steroidov at PSH. Two retrospektvinykh researches patients with PSH from Bernа (n=139, without setting of steroidov, number of indigitations = 6) and Shtuttgarta (n=119, 12% patients were got by steroidy, amount of indigitations n=1) show that number of patients with indigitations during the leadthrough of steroidnoy therapies considerably below at abdominal`nykh pains. Prognosis of nephrite Schonlein- Henoch Researches of 70th testify to the good prognosis of SHN. Chronic kidney insufficiency developed for all of 2% children with PSH. Coming from an amount patients with the defeat of buds kidney remote morbidnost` made 4-7% . It agrees given new remote supervisions falls short of more to truth.

In the recently published Italian research 57 children with SHN on the average in 4,8 years at 24,5% pre- developed or terminal kidney insufficiency. For 22,7% patients with SHN prolechennykh during 15 years in a child nephrological separation of Medical higher school of Gannovera of razvilas terminal kidney insufficiency during this interval of time. International the register of dialysis and transplantation is shown, that 1-2% children and teenagers, which conducted transplantation of buds as basic diseases had SHN. In Italian research it was further studied whether determine a prognosis SHN primary clinical symptoms or to the biopsy of buds. Coming from present or otsutstvuyuschikh of poluluniy in histological preparations of buds patients were part on 2 groups. Children with poluluniem though showed on the average through 4,8 years a few worst prognosis, however much distinction statistically was not reliable. On the basis of degree of weight of histological changes in initial biopsiyakh buds can not be given sure prognosis in relation to the further state of function buds. The same belonged and to the size of proteinurii in this research. Only in beginning hypertension associated with more by an unfavorable prognosis. Surprisings results were rotined by long research, which it was Goldstein et al is published. in 1992. 78 children at which between 1962 and 1969 by years in two English clinics the biopsy of buds was conducted on an occasion SHN, were again inspected in 1971, 1976 and 1990 years. At 52 (67%) after almost 20 years no signs of yet present nephrite were more marked. At the end of time supervisions for 22 patients (28%) pre- or terminal nedostatochnost was marked buds among which 7 had no pointing on the defeat of buds at the first control research in 1971. From 7 patients with RPGN (D group) not at one was not improvements of function of buds. On occasion, even almost after 10 years of bessimptomnogo flow after raising of diagnosis of SHN, through subsequent 10 years, already becoming adults transplantation of buds was required patients. SHN and pregnancy Goldstein et al. further looked after that at 16 (36%) from 44 during time supervisions of successfully worn-out pregnancies regardless of degree of weight SHN the symptoms of EPH-gestosis developed. (proteinuriya, gipertenziya). For pregnant with by healthy buds a risk during the first pregnancy was 5%, at subsequent pregnancies less than 0,5%. Therefore for girls with SHN in child`s age in the case of future pregnancy there is an enhanceable risk of development of ERN-GESTOZA. Resume

• The defeat of buds at PSH takes a place in 40-60% cases.
• Etiology of SHN is unknown.
• The generally accepted therapy of SHN does not exist except for RPGN.
• SHN has the indefinite separated prognosis, at 10-15% patients can to develop limitation of function of buds up to terminal kidney to insufficiency during 20 years.
• The prognosis of SHN is not predictable, neither on the basis of clinical nor histological parameters.
• For girls in the case of future pregnancy there is an enhanceable risk of development ERN-GESTOZA, regardless of clinical degree of weight of SHN.
• Children with SHN require long-term pediatricheskogo/nefrologicheskogo protracted supervision with the regular leadthroughs of researches of urine on squirrel and red corpuscles, and also whey on a kreatinine and control of piesis.

Source: M. Kirschstein, J.H. Ehrich. Schoenlein-Henoch-Nephritis. Monatsschr Kinderheilkd. 146: 1208-1217

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