Choroba henocha-schönleina
Etiologia i przyczyny

Choroba Henocha-Schönleina (HSP), czyli zapalenie naczyń związane z IgA, to zapalenie drobnych naczyń krwionośnych w skórze, stawach, jelitach i nerkach, wywołane odkładaniem kompleksów immunologicznych zawierających IgA, głównie IgA1. Patogeneza opiera się na leukocytoklastycznym zapaleniu naczyń, prowadzącym do uszkodzenia ścian naczyń i zwiększonej przepuszczalności, co manifestuje się charakterystyczną plamicą skórną oraz objawami systemowymi. Etiologia jest wieloczynnikowa, z dominującą rolą infekcji górnych dróg oddechowych (30-75% przypadków), w tym patogenów bakteryjnych (np. Streptococcus grupy A) i wirusowych (m.in. Parvowirus B19, EBV, CMV, SARS-CoV-2). Czynniki wyzwalające obejmują także szczepienia (MMR, HBV, grypa, COVID-19), leki (inhibitory ACE, antybiotyki, NLPZ) oraz czynniki środowiskowe (ekspozycja na zimno, alergeny). W patogenezie istotne są również predyspozycje genetyczne związane z regionami HLA (DQA1, DQB1, DRB1) oraz zaburzenia immunologiczne, takie jak zmiany w populacjach komórek ILC.

Choroba Henocha-Schönleina (HSP) – Etiologia

Choroba Henocha-Schönleina (HSP), znana również jako zapalenie naczyń związane z IgA (IgA vasculitis), to schorzenie charakteryzujące się zapaleniem drobnych naczyń krwionośnych w skórze, stawach, jelitach i nerkach. Zapalenie to prowadzi do zwiększonej przepuszczalności naczyń i krwawienia, co objawia się charakterystyczną plamicą skórną i innymi objawami systemowymi.123

Patofizjologia choroby

Choroba Henocha-Schönleina jest spowodowana nieprawidłową odpowiedzią układu immunologicznego, w której dochodzi do patologicznej aktywacji i odkładania się kompleksów immunologicznych zawierających immunoglobulinę A (IgA) w ścianach drobnych naczyń krwionośnych. Te kompleksy immunologiczne wywołują zapalenie naczyń (vasculitis), co prowadzi do ich uszkodzenia i zwiększonej przepuszczalności.45

Dokładna przyczyna nieprawidłowego zachowania IgA nie jest w pełni wyjaśniona, ale wydaje się, że może być związana z subtelnym defektem molekuły IgA1, która wykazuje tendencję do tworzenia polimerów i odkładania się w ścianach naczyń krwionośnych. Może to być spowodowane nadmierną produkcją IgA (w przewodzie pokarmowym lub szpiku kostnym) lub zmniejszonym usuwaniem nieprawidłowych IgA z krwiobiegu.65

W patofizjologii HSP odkładanie się kompleksów immunologicznych IgA w drobnych naczyniach krwionośnych wywołuje reakcję zapalną typu leukocytoklastycznego zapalenia naczyń. Prowadzi to do uszkodzenia naczyń i objawów klinicznych choroby w różnych narządach.78

Czynniki wyzwalające

Chociaż dokładna etiologia HSP pozostaje nieznana, zidentyfikowano wiele czynników wyzwalających, które mogą inicjować nieprawidłową odpowiedź immunologiczną:

Infekcje górnych dróg oddechowych

Najczęstszym czynnikiem wyzwalającym HSP są infekcje, szczególnie górnych dróg oddechowych. Szacuje się, że około 30-75% pacjentów z HSP przeszło infekcję górnych dróg oddechowych przed wystąpieniem objawów choroby, zazwyczaj 1-3 tygodnie wcześniej.91011

Najczęstsze patogeny związane z HSP obejmują:

101312

Szczepienia

Niektóre szczepienia zostały powiązane z wystąpieniem HSP:

  • Szczepionka przeciw odrze, śwince i różyczce (MMR)14
  • Szczepionka przeciw wirusowemu zapaleniu wątroby typu B14
  • Szczepionka przeciw grypie14
  • Szczepionka przeciw żółtej febrze14
  • Szczepionka przeciw cholerze14
  • Szczepionka przeciw durowi brzusznemu i paradurowi A i B14
  • Szczepionka przeciw COVID-191314

14

Leki i czynniki środowiskowe

Różne leki i czynniki środowiskowe mogą poprzedzać rozwój HSP:

1461

Czynniki genetyczne

Istnieją dowody na genetyczne predyspozycje do rozwoju HSP, chociaż dokładne zależności genetyczne nie zostały jednoznacznie określone:

  • Badania genomowe wykazały związek z regionem międzygenowym HLA-DQA1 i DQB1, loci HLA-DRB1*01:11/B1*13 oraz haplotypem DQA1*01:01/DQB1*05:01/DRB1*01:0113
  • Opisano przypadki HSP występujące rodzinnie, w tym u rodzeństwa, co sugeruje komponent genetyczny1617
  • Opisano również przypadki HSP u bliźniąt15

131617

Inne powiązane czynniki

Odnotowano również inne czynniki, które mogą wpływać na rozwój HSP:

  • Glomerulocystyczna choroba nerek została uznana za czynnik ryzyka1418
  • Związek z nowotworami złośliwymi, częściej z guzami litymi niż z nowotworami hematologicznymi, szczególnie u dorosłych mężczyzn7
  • Zwiększone krążące komórki limfoidalne wrodzonej odporności (ILC)1 i zmniejszone krążące ILC3 są zaangażowane w patogenezę HSP19

14187

Sezonowość występowania

HSP wykazuje pewną sezonowość, co dodatkowo potwierdza rolę czynników infekcyjnych w jej etiologii:

  • Najwyższa częstość występowania dziecięcej postaci HSP przypada na jesień i zimę6
  • Ta sezonowość koreluje z okresami zwiększonej częstości infekcji dróg oddechowych7

67

Podsumowanie etiologii choroby Henocha-Schönleina

Choroba Henocha-Schönleina jest wieloczynnikowym zaburzeniem o złożonej etiologii. Dokładna przyczyna pozostaje nieznana, ale wydaje się, że jest wynikiem nieprawidłowej odpowiedzi immunologicznej, w której dochodzi do odkładania IgA w ścianach drobnych naczyń krwionośnych, co prowadzi do zapalenia naczyń i objawów klinicznych.204

Najważniejsze czynniki etiologiczne to infekcje (szczególnie górnych dróg oddechowych), czynniki genetyczne oraz różne czynniki środowiskowe (leki, alergie pokarmowe, ekspozycja na zimno). Mechanizm, w którym te czynniki wyzwalają nieprawidłową odpowiedź immunologiczną, nie jest w pełni wyjaśniony i wymaga dalszych badań.214

Istotne jest podkreślenie, że choroba Henocha-Schönleina nie jest zakaźna ani dziedziczona bezpośrednio, choć może występować rodzinnie. W większości przypadków choroba jest samoograniczająca się, choć u części pacjentów, szczególnie dorosłych, może prowadzić do przewlekłych powikłań, głównie nerkowych.3722

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  1. 09.04.2026
  2. www.leksykon.com.pl

Materiały źródłowe

  • #1 Henoch-Schonlein purpura // Middlesex Health
    https://middlesexhealth.org/learning-center/diseases-and-conditions/henoch-schonlein-purpura
    Henoch-Schonlein purpura (also known as IgA vasculitis) is a disorder that causes the small blood vessels in your skin, joints, intestines and kidneys to become inflamed and bleed. […] In Henoch-Schonlein purpura, some of the body’s small blood vessels become inflamed, which can cause bleeding in the skin, abdomen and kidneys. It’s not clear why this initial inflammation develops. It may be the result of the immune system responding inappropriately to certain triggers. […] Nearly half the people who have Henoch-Schonlein purpura developed it after an upper respiratory infection, such as a cold. Other triggers include chickenpox, strep throat, measles, hepatitis, certain medications, food, insect bites and exposure to cold weather.
  • #2 What Is Henoch-Schönlein Purpura? | AAFP
    https://www.aafp.org/pubs/afp/issues/1998/0801/p411.html
    Henoch-Schnlein purpura (say: hen-awk shern-line purr-purr-ah) causes blood vessels to get inflamed (irritated and swollen). This inflammation is called vasculitis. […] HSP is caused when a person’s immune system doesn’t fight an infection like it’s supposed to. It occurs most often in the spring, usually after an upper respiratory infection, like a cold. […] Its exact cause is unknown. It might be triggered by bacterial or viral infections, medicines, insect bites, vaccinations or exposure to chemicals or cold weather. […] You may catch an infection that caused someone’s immune system to respond with HSP, but HSP itself isn’t contagious.
  • #3 Henoch Schönlein Purpura (HSP) | Great Ormond Street Hospital
    https://www.gosh.nhs.uk/conditions-and-treatments/conditions-we-treat/henoch-sch-nlein-purpura-hsp/
    Henoch Schönlein purpura (HSP) is a disease where small blood vessels called capillaries become inflamed and damaged, producing a rash on the skin called ’purpura’. […] We do not know exactly what causes HSP, but it seems to follow on from some viral or bacterial infections such as a cold or respiratory tract infection. […] HSP is not an inherited disease and is not contagious.
  • #4 Henoch-Schönlein Purpura (HSP) (for Parents) | Nemours KidsHealth
    https://kidshealth.org/en/parents/hsp.html
    Henoch-Schönlein purpura (HSP) is a condition that makes small blood vessels get swollen and irritated. This inflammation is called vasculitis (vas-kyuh-LY-tis). […] Doctors don’t know for sure what causes HSP. But it’s thought to be triggered by infections (from bacteria, viruses, or parasites). Other possible causes include medicines and food allergies. Some people might have a genetic risk for the condition. Researchers continue to look for the exact cause of HSP. […] HSP happens when the immune system doesn’t work as it should. A protein called immunoglobulin A (IgA) that normally works to fight infections gets deposited in the blood vessels. This leads to the vasculitis.
  • #5 Henoch–Schönlein purpura – Wikipedia
    https://en.wikipedia.org/wiki/Henoch%E2%80%93Sch%C3%B6nlein_purpura
    IgA vasculitis (HSP), previously known as Henoch–Schönlein purpura, is an autoimmune disease that most commonly affects children. […] 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. […] The genetic basis remains unclear except for involvement of the human leukocyte antigen region of the genome. It is hypothesized to involve autoimmunity triggered by infections. Streptococcus strains and Parainfluenza virus are the most commonly associated pathogens, and in children Human Parvovirus B19 is a frequent viral trigger. […] 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 IgA1 in polymers; 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. […] It is suspected that abnormalities in the IgA1 molecule may provide an explanation for its abnormal behaviour in both HSP and the related condition IgA nephropathy.
  • #6 Henoch-Schonlein purpura (anaphylactoid purpura)
    https://dermnetnz.org/topics/henoch-schoenlein-purpura
    HSP results from inflammation of the small blood vessels in the skin and various other tissues within the body. […] Although the precise cause of HSP is unknown, clinical evidence points to an infectious aetiology. The peak incidence of paediatric HSP is during the autumn and winter months. It often preceded by an upper respiratory tract infection and is found to cluster within families. Beta-haemolytic streptococci are often found on throat swab or there is positive streptococcal serology on blood testing. […] A multitude of other associations has been reported, including medications. These include ACE inhibitors, angiotensin II receptor antagonists, clarithromycin and nonsteroidal anti-inflammatories. […] The underlying mechanism of small blood vessel inflammation is the deposition of IgA immunoglobulin within the blood vessel walls. This leads to leukocytoclastic vasculitis. A subtle defect of IgA may predispose people to develop HSP.
  • #7 Henoch-Schönlein Purpura (HSP) | Doctor
    https://patient.info/doctor/henoch-schonlein-purpura-pro
    Henoch-Schnlein purpura (HSP) is an IgA-mediated, autoimmune hypersensitivity vasculitis of childhood. The aetiology remains unknown. […] The cause is unknown, but a mix of genetic, immune and environmental factors appear to be involved. The condition tends to be seasonal and there is often a history of recent infection. […] Infections preceding HSP include those involving group A streptococci, mycoplasma, Epstein-Barr virus, Coxsackievirus, hepatitis A and B, parvovirus B19, campylobacter, varicella and adenoviruses. Vaccination has also been described as a trigger. For example, HSP has been recorded as presenting following COVID-19 vaccination. […] There is also an association with malignancy; usually solid tumours rather than haematological malignancies, and more common in adult males. […] IgA immune complexes are involved in the pathophysiology of HSP, depositing in the small blood vessels of the skin, joints, kidneys and gastrointestinal tract, causing an inflammatory reaction.
  • #8 Henoch-Schoenlein Purpura – Core EM
    https://coreem.net/core/henoch-schoenlein-purpura/
    Henoch-Schoenlein purpura (HSP) is an IgA-mediated small-vessel vasculitis caused by deposition of immune complexes within vessel walls […] The etiology is often unknown; however, many cases are preceded by viral or bacterial upper respiratory infections.
  • #9 Henoch-Schönlein Purpura : Johns Hopkins Vasculitis CenterFacebookTwitterYouTube
    https://www.hopkinsvasculitis.org/types-vasculitis/henochschnlein-purpura/
    HSP is usually self-limited. Therefore, treatment is not indicated in all cases, and full recovery is the rule. […] In two-thirds of the cases, the disease follows an upper respiratory tract infection, with onset an average of ten days after the start of respiratory symptoms. Despite this association, no single microorganism or environmental exposure has been confirmed as an important cause of HSP.
  • #10 IgA Vasculitis (Henoch-Schonlein Purpura): Practice Essentials, Background, Pathophysiology
    https://emedicine.medscape.com/article/984105-overview
    The etiology of IgAV remains to be clearly defined but is thought to be multifactorial, with genetic, environmental, and antigenic components. More than 75% of patients report antecedent upper respiratory tract or GI infection. Multiple bacterial and viral infectious agents have been associated with the development of IgAV, and cases also have been reported in association with medication use and vaccinations. […] Infections that may precede the development of IgAV include the following: Group A streptococcal infection (most common), infectious mononucleosis, subacute bacterial endocarditis, hepatitis, mycoplasma infection, campylobacter enteritis, Helicobacter pylori infection (especially noted in China), Yersinia infection, Shigella infection, Salmonella infection, brucellosis, Legionella infection, parvovirus infection, adenovirus infection, Epstein-Barr virus (EBV) infection, varicella-zoster virus (VZV) infection, rotavirus infection, COVID-19.
  • #11 Pulsenotes | Henoch-Schönlein purpura notes
    https://app.pulsenotes.com/specialities/paediatrics/notes/henoch-schonlein-purpura
    Henoch-Schnlein purpura is an IgA vasculitis, which commonly presents in childhood. […] HSP is an immune-mediated disorder due to deposition of the IgA immunoglobulin in blood vessels. […] The exact cause for the deposition of IgA and subsequent development of HSP is unknown. It is estimated that up to 50% of children have a preceding upper respiratory tract infection, particularly following Streptococcal infections. […] The association with infections suggests the autoreactive IgA antibodies (i.e. antibodies that attack our bodies own antigens), may have originally been created as part of the normal immune response.
  • #12 Henoch-Schönlein Purpura | AAFP
    https://www.aafp.org/pubs/afp/issues/2009/1001/p697.html
    Henoch-Schönlein purpura is an acute, systemic, immune complex-mediated, leukocytoclastic vasculitis. […] Exposure to an antigen from an infection, medication, or other environmental factor may trigger antibody and immune complex formation. Group A streptococcus has been found in cultures of more than 30 percent of children with Henoch-Schönlein nephritis, and serum antistreptolysin-O titers are more likely to be positive in patients with Henoch-Schönlein nephritis. […] Other postulated viral and bacterial triggers of Henoch-Schönlein purpura include parvovirus B19, Bartonella henselae, Helicobacter pylori, Haemophilus parainfluenza, Coxsackie virus, adenovirus, hepatitis A and B viruses, mycoplasma, Epstein-Barr virus, varicella, campylobacter, and methicillin-resistant Staphylococcus aureus.
  • #13 IgA Vasculitis (Henoch-Schönlein Purpura) – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK537252/
    Environmental, genetic, and antigenic factors appear to contribute to the etiology of IgA vasculitis. Genomic studies have found an association with HLA-DQA1 and DQB1 intergenic zone, the HLA-DRB1*01:11/B1*13 loci, and DQA1*01:01/DQB1*05:01/DRB1*01:01 haplotype. Many patients report a preceding infection. Upper respiratory tract infections are the most common; however, patients may also present with an antecedent gastrointestinal or pharyngeal infection. […] Other causative agents include. Coxsackie virus, Hepatitis A, hepatitis B (and hepatitis vaccines), Mycoplasma, Parvovirus B19, Infectious mononucleosis, Subacute bacterial endocarditis, Helicobacter pylori, Yersinia, Shigella, Salmonella, Brucellosis, Legionella, Campylobacter, Varicella, Parainfluenza virus, Influenza virus (and vaccine), Respiratory syncytial virus (RSV), rotavirus, Cytomegalovirus (CMV) reactivation, Adenoviruses. […] More recently, IgAV has also been found in association with COVID-19 infections. The virus is thought to damage blood vessels directly, leading to inflammation and immune complex formation. IgAV has also been seen with COVID-19 immunizations.
  • #14 IgA Vasculitis (Henoch-Schonlein Purpura): Practice Essentials, Background, Pathophysiology
    https://emedicine.medscape.com/article/984105-overview
    Vaccinations that may precede the development of IgAV include the following: measles, mumps, rubella (MMR), hepatitis B, influenza, yellow fever, cholera, typhoid and paratyphoid A and B, COVID-19. […] Environmental exposure to the following may precede the development of IgAV: drugs (eg, adalimumab, ampicillin, cytarabine, erythromycin, infliximab, losartan, penicillin, quinidine, quinine), foods, horse serum, cold temperatures, insect bites. […] Glomerulocystic kidney disease has also been noted as a risk factor.
  • #15 Henoch-Schönlein purpura: Causes, symptoms, and treatmentMedical News Today
    https://www.medicalnewstoday.com/articles/318948
    Henoch-Schönlein purpura is an inflammation of small blood vessels that causes them to leak, resulting in a rash. […] The exact cause of HSP is not clear. It may be an abnormal immune system response to infection or an extreme allergic reaction. Other people may be genetically predisposed to developing HSP. […] Possible causes of HSP include: Immune system: A healthy immune system produces antibodies to combat bacteria and viruses. In cases of HSP, the antibodies attack the blood vessels instead, which causes inflammation, leakage, and a subsequent rash. […] Allergic reaction: An extreme allergic reaction to certain foods may cause HSP. […] Genetics: Although rare, HSP occurs in people within the same family, including in twins. This suggests that some cases of HSP may have a genetic cause, although there is no research to prove this. […] Other associations: Certain drugs, bacteria, insect bites, viral infections, food, cold weather, and trauma may also cause HSP.
  • #16 Henoch-Schonlein Purpura (HSP) | Boston Children’s Hospital
    https://www.childrenshospital.org/conditions/henoch-schonlein-purpura-hsp
    Henoch-Schonlein purpura (HSP) is a form of vasculitis, a condition that involves inflammation of the blood vessels. […] As with the other forms of vasculitis, the cause of HSP is not known. HSP may be associated with an upper respiratory tract infection or possibly an allergic reaction. […] A family connection has been noted with HSP, where the disease has happened to two or more siblings of the same family, either at the same time, or one after another.
  • #17
    https://www.nicklauschildrens.org/conditions/henoch-schonlein-purpura
    Henoch-Schonlein Purpura is caused by an abnormal immune response, in which the bodys immune system attacks its own blood vessels. […] 30%-50% of children have had a previous upper respiratory tract infection (like a cold) before HSP; it may also be associated with other viral infections (like chickenpox, measles, hepatitis etc.), certain medications, foods, insect bites, or trauma. […] There may be a genetic component as other family members in the same family may be affected.
  • #18 What Causes Henoch Schonlein Purpura?
    https://www.emedicinehealth.com/what_causes_henoch_schonlein_purpura/article_em.htm
    Henoch-Schnlein purpura, now called IgA vasculitis or IgAV, is a vascular condition that usually causes a rash that looks like bruises. […] Causes are not well understood, but probably more than one factor is involved. […] The cause of Henoch-Schnlein purpura (IgA vasculitis) is not completely understood but is believed to be caused by multiple factors, including: […] Genetics […] Environmental factors […] Certain drugs such as ampicillin, erythromycin, penicillin, quinidine, quinine, losartan, and cytarabine […] Foods […] Horse serum […] Cold exposure […] Insect bites […] Infections […] Upper respiratory tract […] Throat infections […] Gastrointestinal infections […] Certain vaccines […] Typhoid and paratyphoid A and B […] Measles […] Yellow fever […] Cholera. […] Glomerulocystic kidney disease has also been noted.
  • #19 Henoch-Schonlein purpura » Global Autoimmune Institute
    https://www.autoimmuneinstitute.org/autoimmune-resources/autoimmune-diseases-list/henoch-schonlein-purpura/
    HSP, also known as IgA vasculitis, is characterized by inflammation of the small blood vessels located in the skin, joints, intestines, and kidneys, sometimes accompanied by bleeding. […] Environmental triggers include colds, chickenpox, strep throat, measles, and hepatitis. Many recorded cases of HSP have followed upper respiratory infections. Other triggers include certain medications, food, insect bites, and cold weather. […] Increased circulating innate lymphoid cell (ILC)1 and decreased circulating ILC3 are involved in the pathogenesis of Henoch-Schonlein purpura.
  • #20 Henoch Schönlein Purpura (HSP)
    https://rheumatology.org/patients/henoch-schonlein-purpura-hsp
    Henoch Schönlein Purpura (HSP) is a type of vasculitis, which means inflammation of the blood vessels. The exact cause of HSP is unknown and there is no specific test to diagnose it. […] In HSP, the person’s own immune system attacks blood vessels in the skin, intestines, joints, and kidneys.
  • #21 Henoch-Schonlein Purpura | Children’s Hospital of Philadelphia
    https://www.chop.edu/conditions-diseases/henoch-schonlein-purpura
    Henoch-Schönlein purpura (HSP) is a form of vasculitis, a condition that involves inflammation of the blood vessels. It can affect any organ of the body. […] The cause of Henoch-Schönlein purpura is unknown. Environmental triggers, such as an infections, have been proposed as a trigger for initiating Henoch-Schönlein purpura, although such triggers are not always identified. […] A genetic component to the disease has also been proposed, although no clear link has been made between an individual gene and the development of Henoch-Schönlein purpura. […] More research is needed to understand the cause of this disease.
  • #22 Henoch-Schonlein purpura – WikEM
    https://wikem.org/wiki/Henoch-Schonlein_purpura
    Classical triad of non-thrombocytopenic palpable purpura, abdominal pain, arthritis […] Most common vasculitis in childhood (usually 2-11 yr) […] IgA mediated […] 5% of cases associated with intussusception (abdominal vasculitis) […] Most cases preceded by a URI […] Progression to renal insufficiency is feared complication (5-15% in children) […] Presentation in adults is often worse, with persisting renal insufficiency in 30-50% […] Adults may require more aggressive treatment with steroids +/- cyclophosphamide though evidence of benefit is lacking […] Henoch-Schonlein purpura (HSP) […] Henoch-Schonlein purpura […] Henoch-Schonlein purpura […] Henoch-Schonlein purpura.