Materiały źródłowe

• #1 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. It is characterized by a clinical triad of palpable purpura (without thrombocytopenia), abdominal pain, and arthritis. Glomerulonephritis and gastrointestinal bleeding are common complications. […] There is no definitive test to diagnose Henoch-Schönlein purpura. The clinical triad of purpura, abdominal pain, and arthritis should raise concern. Palpable purpura in the absence of thrombocytopenia is most suggestive and is present in all patients. Punch biopsy of the skin is useful to show the characteristic leukocytoclastic vasculitis. Renal biopsy will demonstrate a membranoproliferative glomerulonephritis similar to IgA nephropathy. […] In 1990, the American College of Rheumatology defined criteria for the diagnosis of Henoch-Schönlein purpura. The criteria required the presence of two out of four features, and yielded a diagnostic sensitivity of 87.1 percent and specificity of 87.7 percent. The criteria were: patient 20 years or younger at onset, palpable purpura (without thrombocytopenia), bowel angina (diffuse abdominal pain or diagnosis of bowel ischemia), and histologic changes showing granulocytes in small walls of arterioles and venules (leukocytoclastic vasculitis). In 2006, the criteria were revised to make palpable purpura a mandatory feature, remove the age criterion, add arthritis as a criterion, and replace granulocytes in biopsy specimens with IgA deposition. These criteria have been accepted by expert organizations but still await validation in prospective trials.

• #2 Henoch-Schonlein purpura – Symptoms & causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/henoch-schonlein-purpura/symptoms-causes/syc-20354040

  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. […] Henoch-Schonlein purpura can affect anyone, but it’s most common in children under 10. The condition usually improves on its own. Medical care is generally needed if the disorder affects the kidneys. […] See your doctor if you have Henoch-Schonlein purpura and it’s causing serious problems with your digestive tract. […] In Henoch-Schonlein purpura, some of the body’s small blood vessels become inflamed, which can cause bleeding in the skin, abdomen and kidneys. […] Factors that increase the risk of developing Henoch-Schonlein purpura include: Age. The disease mainly affects children younger than 10. […] Complications associated with Henoch-Schonlein purpura include: Kidney damage. The most serious complication of Henoch-Schonlein purpura is kidney damage.

• #3 IgA Vasculitis (Henoch-Schönlein Purpura) – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK537252/

  IgA vasculitis, formerly known as Henoch-Schnlein purpura, is a complex immune-mediated vasculitis characterized by the involvement of small blood vessels in various organ systems. […] This activity explores the immunological basis of IgA vasculitis, including the role of IgA immune complexes in small vessel inflammation; the clinical presentation and diagnostic criteria; assessment and monitoring recommendations; treatment strategies; and the crucial role of the interprofessional team in improving the care and outcomes of patients with this condition. […] The diagnosis of IgA vasculitis is made based on the presence of petechiae (without thrombocytopenia) or palpable purpura that predominantly affects the lower limbs plus at least one of the following four characteristics: abdominal pain, arthralgia or arthritis, renal involvement (proteinuria, red blood cell casts, or hematuria), proliferative glomerulonephritis or leukocytoclastic vasculitis with predominant deposition of IgA on histology.

• #4 Henoch-Schönlein Purpura (IgA Vasculitis): Rapid Evidence Review | AAFP

  https://www.aafp.org/pubs/afp/issues/2020/0815/p229.html

  Henoch-Schnlein purpura, now called immunoglobulin A (IgA) vasculitis, is a systemic, immune complexmediated, small-vessel leukocytoclastic vasculitis characterized by nonthrombocytopenic palpable purpura, arthritis, and abdominal pain. Diagnostic testing is required only to exclude other etiologies of purpura, to identify renal involvement, and, if indicated, to determine its extent with biopsy. […] IgA vasculitis should be suspected in patients presenting with palpable purpura who also develop arthralgias (75% of patients) and abdominal pain (50% to 65% of patients). […] The European League Against Rheumatism/Paediatric Rheumatology European Society diagnostic criteria have a 100% sensitivity and 87% specificity in children, and a 99% sensitivity and 86% specificity in adults. These criteria are more accurate than the 1990 American College of Rheumatology criteria.

• #5 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 classic tetrad is a palpable purpuric rash, joint pains, gastrointestinal symptoms and renal involvement. A number of diagnostic criteria exist, the most recent being that proposed by European League Against Rheumatism/Paediatric Rheumatology International Trials Organisation/Paediatric Rheumatology European Society (EULAR/PRINTO/PRES). This set of criteria states that for a diagnosis of HSP there must be palpable purpura, which is not thrombocytopenic/petechiae, and one or more of the following: diffuse abdominal pain, typical histopathology (leukocytoclastic vasculitis or proliferative glomerulonephritis with predominant IgA deposits), arthritis or arthralgia, renal involvement (demonstrated by quantified proteinuria or haematuria).

• #6 Henoch-Schönlein Purpura: A Literature Review | HTML | Acta Dermato-Venereologica

  https://www.medicaljournals.se/acta/content/html/10.2340/00015555-2733

  Henoch-Schnlein purpura is the most common childhood vasculitis, but may also affect adults. […] The diagnosis of HSP is criteria-based. The European League Against Rheumatism (EULAR), the Paediatric Rheumatology International Trials Organization (PRINTO) and the Paediatric Rheumatology European Society (PRES) published a revised set of criteria in 2010, with high sensitivity and specificity. […] HSP diagnosis is based on clinical criteria. The revised criteria developed by EULAR/PRINTO/PRES were published in 2010, and are the gold standard for the diagnosis of HSP. […] There are currently no specific biomarkers useful for diagnosis of HSP. Some biomarkers can show activity and prognosis of the disease, but none have proven clinically useful. […] Skin biopsies are the gold standard for diagnosing any cutaneous vasculitis. IgA-predominant vascular deposits are characteristic for HSP, but not sufficient for the diagnosis of HSP, as these deposits can be found in other vasculitic syndromes, erythema nodosum and venous stasis-related conditions.

• #7 IgA Vasculitis (Henoch-Schönlein Purpura) – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK537252/

  IgA vasculitis, formerly known as Henoch-Schnlein purpura, is a complex immune-mediated vasculitis characterized by the involvement of small blood vessels in various organ systems. […] This activity explores the immunological basis of IgA vasculitis, including the role of IgA immune complexes in small vessel inflammation; the clinical presentation and diagnostic criteria; assessment and monitoring recommendations; treatment strategies; and the crucial role of the interprofessional team in improving the care and outcomes of patients with this condition. […] The diagnosis of IgA vasculitis is made based on the presence of petechiae (without thrombocytopenia) or palpable purpura that predominantly affects the lower limbs plus at least one of the following four characteristics: abdominal pain, arthralgia or arthritis, renal involvement (proteinuria, red blood cell casts, or hematuria), proliferative glomerulonephritis or leukocytoclastic vasculitis with predominant deposition of IgA on histology.

• #8 Henoch-Schonlein Purpura – Zero To Finals

  https://zerotofinals.com/paediatrics/rheumatology/hsp/

  There are many different sets of criteria for diagnosing HSP, the most recent being the EULAR/PRINTO/PRES criteria from 2010. This requires the patient to have palpable purpura (not petichiae) + at least one of: Diffuse abdominal pain; Arthritis or arthralgia; IgA deposits on histology (biopsy); Proteinuria or haematuria.

• #9 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. It is characterized by a clinical triad of palpable purpura (without thrombocytopenia), abdominal pain, and arthritis. Glomerulonephritis and gastrointestinal bleeding are common complications. […] There is no definitive test to diagnose Henoch-Schönlein purpura. The clinical triad of purpura, abdominal pain, and arthritis should raise concern. Palpable purpura in the absence of thrombocytopenia is most suggestive and is present in all patients. Punch biopsy of the skin is useful to show the characteristic leukocytoclastic vasculitis. Renal biopsy will demonstrate a membranoproliferative glomerulonephritis similar to IgA nephropathy. […] In 1990, the American College of Rheumatology defined criteria for the diagnosis of Henoch-Schönlein purpura. The criteria required the presence of two out of four features, and yielded a diagnostic sensitivity of 87.1 percent and specificity of 87.7 percent. The criteria were: patient 20 years or younger at onset, palpable purpura (without thrombocytopenia), bowel angina (diffuse abdominal pain or diagnosis of bowel ischemia), and histologic changes showing granulocytes in small walls of arterioles and venules (leukocytoclastic vasculitis). In 2006, the criteria were revised to make palpable purpura a mandatory feature, remove the age criterion, add arthritis as a criterion, and replace granulocytes in biopsy specimens with IgA deposition. These criteria have been accepted by expert organizations but still await validation in prospective trials.

• #10 Delayed diagnosis of abdominal Henoch-Schonlein purpura in children: A case report

  https://www.wjgnet.com/2307-8960/full/v11/i26/6311.htm

  Henoch-Schonlein purpura (HSP) is one of the most common vasculitis in childhood. The main pathogenesis is leukocytoclastic vasculitis and deposition of immunoglobulin A (IgA) immune complexes in small blood vessels. HSP can affect the skin, joints, gastrointestinal tract, and kidney, with skin purpura as the initial symptom in most cases. Abdominal pain is a common symptom of HSP, mostly manifesting after rash. EULAR/PRINTO/PRES 2008 proposed the diagnostic criteria for HSP as palpable purpura or petechia containing at least one of the following four conditions: (1) Acute diffuse abdominal pain; (2) leukocytoclastic vasculitis with IgA deposition revealed by biopsy; (3) arthritis or joint pain; and (4) renal involvement, including proteinuria or hematuria. The rash usually appears on the compressed sites, especially the lower limbs and hip, but is not associated with thrombocytopenia.

• #11 IgA Vasculitis (Henoch-Schonlein Purpura) Workup: Approach Considerations, Laboratory Studies, Ultrasonography

  https://emedicine.medscape.com/article/984105-workup

  Diagnosis of IgA vasculitis (IgAV; Henoch-Schnlein purpura) is clinical and is not based on laboratory evaluation. Routine laboratory test results are usually within reference ranges. Some laboratory studies help in excluding other diagnoses and in evaluating renal function, including the following: […] Some experts consider that imaging studies are indicated only if the diagnosis is uncertain. Kidney biopsy may be helpful in selected cases. […] No specific diagnostic laboratory test is available to assess for markers of IgAV. General laboratory tests may reveal the following: […] Total IgA levels are not very helpful in confirming the diagnosis or providing prognostic information. Elevated serum levels of galactose-deficient IgA1 seem to distinguish IgAV patients with nephritis from those without nephritis, but this assay is not used presently in clinical practice.

• #12 Henoch-Schönlein Purpura (IgA Vasculitis): Rapid Evidence Review | AAFP

  https://www.aafp.org/pubs/afp/issues/2020/0815/p229.html

  Tests are not required to diagnose IgA vasculitis. The laboratory evaluation should exclude other diagnoses and identify disease complications. […] A skin biopsy is needed only in cases where the diagnosis is unclear. IgA vascular deposits are characteristic of the disease. A renal biopsy is required only in cases of progressive renal disease. […] Order urinalysis and measure creatinine and blood pressure at least monthly for patients with renal abnormalities at the time of diagnosis. The optimal frequency and length of follow-up is unclear; however, a six-month monitoring period is prudent.

• #13 Henoch-Schönlein Purpura | AAFP

  https://www.aafp.org/pubs/afp/issues/2009/1001/p697.html

  Because there are no definitive serologic tests for Henoch-Schönlein purpura, the purpose of laboratory evaluation is to identify complications or exclude other diseases. Useful baseline studies include renal function tests (e.g., electrolytes, blood urea nitrogen, creatinine, urinalysis), complete blood count with platelet count, coagulation profile (e.g., prothrombin time, partial thromboplastin time, fibrinogen, fibrin degradation products), and IgA levels. […] A blood pressure measurement and urinalysis should be performed at the time Henoch-Schönlein purpura is diagnosed and at each return physician office visit. Serum blood urea nitrogen and creatinine determination are needed if hematuria or proteinuria are identified. If the initial urinalysis is normal, or if there is isolated hematuria (without nephritic or nephrotic syndrome), a monthly urinalysis should be performed for the first six months after the diagnosis of Henoch-Schönlein purpura. This will detect the first signs of renal involvement, and facilitate early nephrology consultation and the initiation of steroids.

• #14 IgA Vasculitis (Henoch-Schonlein Purpura): Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/984105-overview

  IgAV is a clinical diagnosis, although confirmation by histologic analysis from skin or kidney biopsy is sometimes helpful. The diagnosis of IgAV, as revised by the European League Against Rheumatism (EULAR), Paediatric Rheumatology International Trial Organisation (PRINTO), and Paediatric Rheumatology European Society (PRES), requires the presence of palpable purpura or petechiae with lower limb predominance and without thrombocytopenia or coagulopathy, usually in clusters along with at least one of the following: […] No specific diagnostic laboratory test is available to assess for markers of IgAV. The following general laboratory tests may be helpful for excluding other diagnoses and evaluating kidney function: […] Imaging modalities that may be considered include the following: […] Other studies that may be warranted are as follows:

• #15 Henoch-Schönlein purpura Information | Mount Sinai – New York

  https://www.mountsinai.org/health-library/diseases-conditions/henoch-sch-nlein-purpura

  IgA vasculitis is a disease that involves purple spots on the skin, joint pain, gastrointestinal problems, and glomerulonephritis (a type of kidney disorder). It is also known as Henoch-Schönlein purpura (HSP). […] The health care provider will look at your body and look at your skin. The physical exam will show skin sores (purpura, lesions) and joint tenderness. […] Tests may include: Urinalysis should be done in all cases. Complete blood count. The platelet should be normal. Coagulation tests: these should be normal. Skin biopsy, especially in adults. Blood tests to look for other causes of blood vessel inflammation, such as systemic lupus erythematosus, ANCA-associated vasculitis or hepatitis. In adults, a kidney biopsy should be done. Imaging tests of the abdomen if pain is present. […] Contact your provider if: You develop symptoms of IgA vasculitis, and they last for more than a few days. You have colored urine or low urine output after an episode.

• #16 The clinical implications of adult-onset henoch-schonelin purpura | Clinical and Molecular Allergy | Full Text

  https://clinicalmolecularallergy.biomedcentral.com/articles/10.1186/1476-7961-9-9

  Henoch-Schonlein Purpura (HSP) is a small vessel vasculitis mediated by IgA-immune complex deposition. It is characterized by the clinical tetrad of non-thrombocytopenic palpable purpura, abdominal pain, arthritis and renal involvement. […] The diagnosis is usually based on clinical presentation with tissue biopsy demonstrating leukocytoclastic vasculitis associated with IgA deposition (by immunofluorescence). Skin biopsy should be obtained from the lesion less than 24 hours old and typically shows the classical leukocytoclastic vasculitis in postcapillary venule with IgA deposition. Renal biopsy should be performed in case of uncertain diagnosis or severe renal impairment such as nephrotic syndrome. […] Elevated serum IgA levels have been associated with HSP in about 60% of cases. Urine analysis can vary from microscopic hematuria to nephritic-syndrome range proteinuria. Coagulation studies and the platelet count are usually normal. Inflammatory markers such as sedimentation rate (ESR) and C reactive protein (CRP) levels are often elevated.

• #17 IgA Vasculitis (Henoch-Schonlein Purpura) Workup: Approach Considerations, Laboratory Studies, Ultrasonography

  https://emedicine.medscape.com/article/984105-workup

  Diagnosis of IgA vasculitis (IgAV; Henoch-Schnlein purpura) is clinical and is not based on laboratory evaluation. Routine laboratory test results are usually within reference ranges. Some laboratory studies help in excluding other diagnoses and in evaluating renal function, including the following: […] Some experts consider that imaging studies are indicated only if the diagnosis is uncertain. Kidney biopsy may be helpful in selected cases. […] No specific diagnostic laboratory test is available to assess for markers of IgAV. General laboratory tests may reveal the following: […] Total IgA levels are not very helpful in confirming the diagnosis or providing prognostic information. Elevated serum levels of galactose-deficient IgA1 seem to distinguish IgAV patients with nephritis from those without nephritis, but this assay is not used presently in clinical practice.

• #18 Henoch-Schonlein purpura – Diagnosis & treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/henoch-schonlein-purpura/diagnosis-treatment/drc-20354045

  Your doctor will be able to diagnose the condition as Henoch-Schonlein purpura if the classic rash, joint pain and digestive tract symptoms are present. If one of these signs and symptoms is missing, your doctor may suggest one or more of the following tests. […] No single laboratory test can confirm Henoch-Schonlein purpura, but certain tests can help rule out other diseases and make a diagnosis of Henoch-Schonlein seem likely. They may include: […] People who have Henoch-Schonlein purpura often have deposits of a certain protein, IgA (immunoglobulin A), on the affected organ. Your doctor may take a small sample of skin so that it can be tested in a lab. In cases of severe kidney involvement, your doctor may suggest a kidney biopsy to help guide treatment decisions. […] Your doctor may recommend an ultrasound to rule out other causes of abdominal pain and to check for possible complications, such as a bowel obstruction.

• #19 Henoch-Schönlein Purpura: Symptoms, Diagnosis, and TreatmentsHealthline

  https://www.healthline.com/health/henoch-schonlein-purpura

  Henoch-Schönlein purpura (HSP) is a disease that causes small blood vessels to become inflamed and leak blood. […] The hallmark symptom of HSP is a raised purple-colored rash on the lower legs and buttocks. […] HSP is most common in young children. […] Your doctor will examine you or your child for HSP symptoms, including a rash and joint pain. […] Tests like these can help diagnose HSP and rule out other diseases with similar symptoms: Blood tests. These can evaluate white and red blood cell counts, inflammation, and kidney function. […] Urine test. The doctor may check for blood or protein in your urine, a sign that your kidneys have been damaged. […] Biopsy. Your doctor might remove a small piece of your skin and send it to a lab for testing. This test looks for an antibody called IgA, which is deposited in the skin and blood vessels of people with HSP. […] A kidney biopsy can test for kidney damage. […] Ultrasound. This test uses sound waves to create pictures from inside your abdomen. It can provide a closer look at the abdominal organs and kidneys. […] CT scan. This test can be used to evaluate abdominal pain and rule out other causes.

• #20 Diagnosis and treatment of adult mixed-type Henoch-Schönlein purpura

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6745548/

  The purpose of this study was to investigate the clinical manifestations and outcomes of patients with adult mixed-type Henoch-Schnlein purpura (HSP) and imaging characteristics of the disease, and to evaluate the efficacy of combined therapy in treating symptoms of HSP. […] The diagnosis of HSP was made according to the American College of Rheumatology (ACR) 1990 criteria. […] The combined examination of abdominal contrast-enhanced CT, small intestinal endoscopy and renal needle biopsy is a valuable method for the early diagnosis of adult mixed-type HSP. […] The contrast-enhanced CT examination of the abdomen showed the intestinal canal wall thickening and edema. […] Endoscopic examination demonstrated diffused hyperemia, dropsy and erosion in the inferior segment of the ileum. […] Twenty-one (91.3%) of our adult mixed-type HSP patients were positive urine protein according to urinalysis. […] The IgA deposits at a glomerular capillary and mesangial region can damage small vessels, cause extensive capillaritis and arteriolitis, and immunologic injury. […] Hence, this study also provided a therapy schedule for adult HSP.

• #21 HSP: Diagnosis and Management | Children’s Hospital Colorado

  https://www.childrenscolorado.org/health-professionals/professional-resources/charting-pediatrics-podcast/henoch-schonlein-purpura-diagnosis-management/

  Henoch-Schonlein purpura (HSP) is a disorder that leads to inflammation and bleeding in the small blood vessels of skin, joints, intestines and kidneys. HSP may be the cause when a parent or pediatrician notices a purplish rash, often on the lower legs and buttocks. HSP can also present with abdominal pain and aching joints. […] Experts don’t yet know the exact cause of the initial inflammation associated with HSP, but hypothesize that it could be due to a strong immune response in some children. […] Tests that are helpful for confirming a diagnosis of HSP and for understanding the risk of progression. […] As our two pediatric experts explain, HSP is most often diagnosed and treated in the pediatrician or family doctor’s office. If a pediatrician has questions or concerns about the degree to which the child’s kidneys are being affected they should feel empowered to seek advice from a pediatric nephrologist. […] Our pediatricians and pediatric specialists offer a wide range of services for the diagnosis, treatment and management of HSP.

• #22 Henoch-Schönlein Purpura (IgA Vasculitis): Rapid Evidence Review | AAFP

  https://www.aafp.org/pubs/afp/issues/2020/0815/p229.html

  Tests are not required to diagnose IgA vasculitis. The laboratory evaluation should exclude other diagnoses and identify disease complications. […] A skin biopsy is needed only in cases where the diagnosis is unclear. IgA vascular deposits are characteristic of the disease. A renal biopsy is required only in cases of progressive renal disease. […] Order urinalysis and measure creatinine and blood pressure at least monthly for patients with renal abnormalities at the time of diagnosis. The optimal frequency and length of follow-up is unclear; however, a six-month monitoring period is prudent.

• #23 Immunoglobulin A–Associated Vasculitis (IgAV) – Musculoskeletal and Connective Tissue Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/musculoskeletal-and-connective-tissue-disorders/vasculitis/immunoglobulin-a-associated-vasculitis-igav

  Immunoglobulin Aassociated vasculitis (IgAV; formerly called Henoch-Schnlein purpura) is vasculitis that affects primarily small vessels. Diagnosis is clinical in children but usually warrants biopsy in adults. The diagnosis of IgAV is suspected in patients, particularly children, with typical skin findings. It is confirmed by biopsy of skin lesions when leukocytoclastic vasculitis with IgA in the vessel walls is identified by immunofluorescence. In children, biopsy is unnecessary if clinical diagnosis is clear. […] When necessary to confirm the diagnosis, biopsy skin lesions, looking for IgA deposition.

• #24 IgA Vasculitis (Henoch-Schonlein Purpura) Workup: Approach Considerations, Laboratory Studies, Ultrasonography

  https://emedicine.medscape.com/article/984105-workup

  If serial urine samples are obtained in patients with IgAV, microscopic hematuria is usually found; it is probably present in 100% of cases. However, frank nephritis appears in only 20-30% of unselected children. The entire clinical spectrum of glomerulonephritis may occur in IgAV. The most common renal manifestations are hematuria with mild-to-moderate proteinuria. […] In some cases, kidney biopsy may be useful. It should be performed when nephrotic syndrome persists (though other manifestations may have subsided) and when kidney function deteriorates. […] The International Study of Kidney Disease in Children (ISKDC) classification is widely used for patients with IgAV nephritis. […] Histopathologic features of the skin lesions in infantile IgAV can range from a typical leukocytoclastic vasculitis with or without fibrinoid necrosis to the less specific findings of a lymphohistiocytic perivascular infiltrate with extravasation of erythrocytes.

• #25 IgA Vasculitis (Henoch-Schonlein Purpura) Workup: Approach Considerations, Laboratory Studies, Ultrasonography

  https://emedicine.medscape.com/article/984105-workup

  IgAV often involves the kidneys. Kidney histology in IgAV varies considerably. In some cases, most glomeruli appear unaffected on light microscopy; only a few show mesangial proliferation. In instances of moderate kidney involvement, focal and segmental intracapillary and extracapillary proliferation may be present with adhesions and small crescents. Severe cases are characterized by a diffuse proliferation with infiltration of neutrophils and circumferential crescents in most of the glomeruli. […] The specific kidney pathology of IgAV includes the following:

• #26 Henoch-Schönlein Purpura : Johns Hopkins Vasculitis CenterFacebookTwitterYouTube

  https://www.hopkinsvasculitis.org/types-vasculitis/henochschnlein-purpura/

  HSP is the most common form of vasculitis in children, with an annual incidence on the order of 140 cases/million persons. […] How is Henoch-Schönlein Purpura Diagnosed? […] Purpura not due to a low platelet count, caused by inflammation in blood vessels of the skin, is the hallmark of HSP. […] The microscopic hallmark of HSP is the deposition of IgA (an antibody found in blood, saliva, tears, etc.) in the walls of involved blood vessels. […] HSP can be mimicked by other forms of systemic vasculitis that are more often life-threatening. […] Therefore, it is very important to distinguish the difference by performing a careful evaluation including bloodwork, urinalysis, chest imaging, and possibly biopsies. […] Palpable purpura should be biopsied, and two fresh samples should always be sent for testing (an adequate biopsy should be large enough to divide; one for H&E (hematoxylin and eosin) staining, and one for DIF testing).

• #27 IgA Vasculitis (Henoch-Schonlein Purpura) Workup: Approach Considerations, Laboratory Studies, Ultrasonography

  https://emedicine.medscape.com/article/984105-workup

  If serial urine samples are obtained in patients with IgAV, microscopic hematuria is usually found; it is probably present in 100% of cases. However, frank nephritis appears in only 20-30% of unselected children. The entire clinical spectrum of glomerulonephritis may occur in IgAV. The most common renal manifestations are hematuria with mild-to-moderate proteinuria. […] In some cases, kidney biopsy may be useful. It should be performed when nephrotic syndrome persists (though other manifestations may have subsided) and when kidney function deteriorates. […] The International Study of Kidney Disease in Children (ISKDC) classification is widely used for patients with IgAV nephritis. […] Histopathologic features of the skin lesions in infantile IgAV can range from a typical leukocytoclastic vasculitis with or without fibrinoid necrosis to the less specific findings of a lymphohistiocytic perivascular infiltrate with extravasation of erythrocytes.

• #28 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. […] Diagnosis is made based on rash and at least one of the following: (1) abdominal pain, (2) joint involvement, (3) renal involvement, or (4) histological features. […] Renal involvement is seen in up to half of cases, and ranges from microscopic hematuria and mild proteinuria to end-stage kidney disease caused by crescentic glomerulonephritis. […] Labs generally show a normal CBC and BMP unless there is significant renal impairment; microscopic hematuria and/or proteinuria are common. […] Differential diagnosis includes ITP, drug reaction, Rocky Mountain spotted fever, gonococcemia, and other vasculitides.

• #29 Henoch-Schönlein Purpura : Johns Hopkins Vasculitis CenterFacebookTwitterYouTube

  https://www.hopkinsvasculitis.org/types-vasculitis/henochschnlein-purpura/

  HSP is the most common form of vasculitis in children, with an annual incidence on the order of 140 cases/million persons. […] How is Henoch-Schönlein Purpura Diagnosed? […] Purpura not due to a low platelet count, caused by inflammation in blood vessels of the skin, is the hallmark of HSP. […] The microscopic hallmark of HSP is the deposition of IgA (an antibody found in blood, saliva, tears, etc.) in the walls of involved blood vessels. […] HSP can be mimicked by other forms of systemic vasculitis that are more often life-threatening. […] Therefore, it is very important to distinguish the difference by performing a careful evaluation including bloodwork, urinalysis, chest imaging, and possibly biopsies. […] Palpable purpura should be biopsied, and two fresh samples should always be sent for testing (an adequate biopsy should be large enough to divide; one for H&E (hematoxylin and eosin) staining, and one for DIF testing).

• #30 Henoch-Schönlein purpura Information | Mount Sinai – New York

  https://www.mountsinai.org/health-library/diseases-conditions/henoch-sch-nlein-purpura

  IgA vasculitis is a disease that involves purple spots on the skin, joint pain, gastrointestinal problems, and glomerulonephritis (a type of kidney disorder). It is also known as Henoch-Schönlein purpura (HSP). […] The health care provider will look at your body and look at your skin. The physical exam will show skin sores (purpura, lesions) and joint tenderness. […] Tests may include: Urinalysis should be done in all cases. Complete blood count. The platelet should be normal. Coagulation tests: these should be normal. Skin biopsy, especially in adults. Blood tests to look for other causes of blood vessel inflammation, such as systemic lupus erythematosus, ANCA-associated vasculitis or hepatitis. In adults, a kidney biopsy should be done. Imaging tests of the abdomen if pain is present. […] Contact your provider if: You develop symptoms of IgA vasculitis, and they last for more than a few days. You have colored urine or low urine output after an episode.

• #31 Henoch-Schönlein Purpura | AAFP

  https://www.aafp.org/pubs/afp/issues/2009/1001/p697.html

  Because there are no definitive serologic tests for Henoch-Schönlein purpura, the purpose of laboratory evaluation is to identify complications or exclude other diseases. Useful baseline studies include renal function tests (e.g., electrolytes, blood urea nitrogen, creatinine, urinalysis), complete blood count with platelet count, coagulation profile (e.g., prothrombin time, partial thromboplastin time, fibrinogen, fibrin degradation products), and IgA levels. […] A blood pressure measurement and urinalysis should be performed at the time Henoch-Schönlein purpura is diagnosed and at each return physician office visit. Serum blood urea nitrogen and creatinine determination are needed if hematuria or proteinuria are identified. If the initial urinalysis is normal, or if there is isolated hematuria (without nephritic or nephrotic syndrome), a monthly urinalysis should be performed for the first six months after the diagnosis of Henoch-Schönlein purpura. This will detect the first signs of renal involvement, and facilitate early nephrology consultation and the initiation of steroids.

• #32 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. […] If symptoms are severe or additional expertise is needed, a rheumatologist can help evaluate and treat a child for HSP. One of the most important parts of HSP management is monitoring for kidney disease. […] Urine screening and monitoring of the blood pressure is recommended for at least 6 months after the initial diagnosis, so it is important to follow the screening instructions provided by your doctor.

• #33 Henoch-Schönlein Purpura (HSP) | Doctor

  https://patient.info/doctor/henoch-schonlein-purpura-pro

  Diagnosis of HSP is clinical and not based on laboratory investigations, and there is no definitive test. The following tests may be relevant: urinalysis (should always be performed): haematuria and/or proteinuria are present in 20-40% of patients. FBC: there may be raised white cell count with eosinophilia; normal or increased platelets. Helps in excluding other diagnoses such as thrombocytopenia. […] GPs are likely to be involved in monitoring for renal involvement, usually under written guidance from the secondary care specialist. Typical monitoring advice would be: for those with no proteinuria, blood pressure checking and urinalysis at days 7 and 14 and at 1, 3, 6 and 12 months. For those with proteinuria, follow-up at days 7 and 14, monthly from 1-6 months and then at 12 months.

• #34 Henoch-Schönlein Purpura (HSP) – Alder Hey Children’s Hospital Trust

  https://www.alderhey.nhs.uk/conditions/patient-information-leaflets/henoch-schonlein-purpura-hsp/

  HSP is an illness, which causes skin rash, painful / swollen joints, tummy pains and may also affect your kidneys. It is caused by inflammation of blood vessels, which in medical language is called vasculitis. […] There are usually no special blood tests or scans needed in HSP, as the problems you report and the rash, are generally enough for accurate diagnosis. […] Everyone with HSP will be asked to return to MDU for a urine test (& blood pressure check) around 1 week after diagnosis. If these checks are normal the test will be repeated at 1 month, 3 months and 6 months after the episode of HSP. […] If the urine stays normal, you will be discharged after the 6 month check, without the need for further follow-up. […] If the nurses find blood or protein in the urine it does not mean that there are long term problems with the kidneys, but it does mean more tests may be needed.

• #35 Henoch-Schönlein Purpura (HSP) – Alder Hey Children’s Hospital Trust

  https://www.alderhey.nhs.uk/conditions/patient-information-leaflets/henoch-schonlein-purpura-hsp/

  The family held record is designed to record the monitoring of Blood Pressure and Urine following Henoch Schönlein Purpura (HSP). […] Most children will only need to attend for routine blood pressure and urine tests. Normally, children will be discharged after 6 months with no further follow-up required. […] If any signs of Hypertension (high blood pressure), Haematuria (blood in the urine) or Proteinuria (protein in the urine) are detected, additional rests will need to be performed.

• #36 HSP: Diagnosis and Management | Children’s Hospital Colorado

  https://www.childrenscolorado.org/health-professionals/professional-resources/charting-pediatrics-podcast/henoch-schonlein-purpura-diagnosis-management/

  Henoch-Schonlein purpura (HSP) is a disorder that leads to inflammation and bleeding in the small blood vessels of skin, joints, intestines and kidneys. HSP may be the cause when a parent or pediatrician notices a purplish rash, often on the lower legs and buttocks. HSP can also present with abdominal pain and aching joints. […] Experts don’t yet know the exact cause of the initial inflammation associated with HSP, but hypothesize that it could be due to a strong immune response in some children. […] Tests that are helpful for confirming a diagnosis of HSP and for understanding the risk of progression. […] As our two pediatric experts explain, HSP is most often diagnosed and treated in the pediatrician or family doctor’s office. If a pediatrician has questions or concerns about the degree to which the child’s kidneys are being affected they should feel empowered to seek advice from a pediatric nephrologist. […] Our pediatricians and pediatric specialists offer a wide range of services for the diagnosis, treatment and management of HSP.

• #37 Henoch-Schonlein Purpura: Vasculitis | HSS Rheumatology

  https://www.hss.edu/condition-list_henoch-schonlein-purpura.asp

  Henoch-Schnlein purpura (HSP) is a form of vasculitis, a condition in which some of the small blood vessels in the body become inflamed. […] HSP typically affects the blood vessels in the skin, gastrointestinal (GI) tract and kidneys. […] Doctors may suspect HSP in a child who has the characteristic rash, as well as joint pain and GI issues. […] Though there is no single lab test that confirms the diagnosis, blood work may be helpful to rule out other causes and to look for signs of infection. […] All children with HSP should have urine testing for blood and protein to see if there is any kidney involvement. […] Children with severe abdominal pain may require stool studies and imaging such as an ultrasound, X-ray or CT scan.

• #38 Henoch-Schönlein purpura Information | Mount Sinai – New York

  https://www.mountsinai.org/health-library/diseases-conditions/henoch-sch-nlein-purpura

  IgA vasculitis is a disease that involves purple spots on the skin, joint pain, gastrointestinal problems, and glomerulonephritis (a type of kidney disorder). It is also known as Henoch-Schönlein purpura (HSP). […] The health care provider will look at your body and look at your skin. The physical exam will show skin sores (purpura, lesions) and joint tenderness. […] Tests may include: Urinalysis should be done in all cases. Complete blood count. The platelet should be normal. Coagulation tests: these should be normal. Skin biopsy, especially in adults. Blood tests to look for other causes of blood vessel inflammation, such as systemic lupus erythematosus, ANCA-associated vasculitis or hepatitis. In adults, a kidney biopsy should be done. Imaging tests of the abdomen if pain is present. […] Contact your provider if: You develop symptoms of IgA vasculitis, and they last for more than a few days. You have colored urine or low urine output after an episode.

• #39 Diagnosis and treatment of adult mixed-type Henoch-Schönlein purpura

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6745548/

  The purpose of this study was to investigate the clinical manifestations and outcomes of patients with adult mixed-type Henoch-Schnlein purpura (HSP) and imaging characteristics of the disease, and to evaluate the efficacy of combined therapy in treating symptoms of HSP. […] The diagnosis of HSP was made according to the American College of Rheumatology (ACR) 1990 criteria. […] The combined examination of abdominal contrast-enhanced CT, small intestinal endoscopy and renal needle biopsy is a valuable method for the early diagnosis of adult mixed-type HSP. […] The contrast-enhanced CT examination of the abdomen showed the intestinal canal wall thickening and edema. […] Endoscopic examination demonstrated diffused hyperemia, dropsy and erosion in the inferior segment of the ileum. […] Twenty-one (91.3%) of our adult mixed-type HSP patients were positive urine protein according to urinalysis. […] The IgA deposits at a glomerular capillary and mesangial region can damage small vessels, cause extensive capillaritis and arteriolitis, and immunologic injury. […] Hence, this study also provided a therapy schedule for adult HSP.

• #40 Delayed diagnosis of abdominal Henoch-Schonlein purpura in children: A case report

  https://www.wjgnet.com/2307-8960/full/v11/i26/6311.htm

  Henoch-Schonlein purpura (HSP) is one of the most common vasculitis in childhood. The main pathogenesis is leukocytoclastic vasculitis and deposition of immunoglobulin A (IgA) immune complexes in small blood vessels. HSP can affect the skin, joints, gastrointestinal tract, and kidney, with skin purpura as the initial symptom in most cases. Abdominal pain is a common symptom of HSP, mostly manifesting after rash. EULAR/PRINTO/PRES 2008 proposed the diagnostic criteria for HSP as palpable purpura or petechia containing at least one of the following four conditions: (1) Acute diffuse abdominal pain; (2) leukocytoclastic vasculitis with IgA deposition revealed by biopsy; (3) arthritis or joint pain; and (4) renal involvement, including proteinuria or hematuria. The rash usually appears on the compressed sites, especially the lower limbs and hip, but is not associated with thrombocytopenia.

• #41 Delayed diagnosis of abdominal Henoch-Schonlein purpura in children: A case report

  https://www.wjgnet.com/2307-8960/full/v11/i26/6311.htm

  Given the difficulty of making a clear diagnosis at the early stage when only abdominal pain without rash is present, a misdiagnosis of, for example, surgical acute abdomen or even a recommendation for surgery can result, bringing further pain and anxiety to children. To reduce the misdiagnosis rate of abdominal HSP and increase the clinical diagnostic accuracy, this study analyzed the clinical characteristics of this child in detail and summarized the characteristics of children with delayed abdominal HSP from reports that included a sample size of more than 50 cases published from 2000 to 2022. […] HSP, also called immunoglobulin A vasculitis, characterized by generalized aseptic inflammation of small blood vessels as a pathological basis, is the most common vasculitis in childhood. The main clinical manifestations include skin purpura, abdominal pain and gastrointestinal hemorrhage, joint swelling and pain, and renal involvement. Rash and abdominal pain are the most common symptoms. Gastrointestinal symptoms, reported in 50%-85% of HSP patients, are possibly attributed as secondary to edema and hemorrhage in the gastrointestinal wall and mesentery, and include nausea and vomiting, vomiting of blood, and bloody stools. Among these gastrointestinal symptoms, 14%-36% occur before the rash appears.

• #42 Delayed diagnosis of abdominal Henoch-Schonlein purpura in children: A case report

  https://www.wjgnet.com/2307-8960/full/v11/i26/6311.htm

  The child presented some specific clinical manifestations. First, gastrointestinal symptoms are reported in 50%-85% of HSP patients, commonly including nausea and vomiting, vomiting blood, bloody stool, and melena. Other rare gastrointestinal symptoms include intussusception, mesenteric ischemia, intestinal perforation, massive gastrointestinal bleeding, acute non-calculous cholecystitis, hemorrhagic ascites with peritonitis, pancreatitis, and biliary cirrhosis, but an association with chronic gastrointestinal diseases is rarely reported. The patient in our study presented with abdominal pain and peritonitis in the early stage, associated with underlying chronic non-atrophic gastritis and erosive duodenitis, a complicated condition reported here for the first time based on our literature review.

• #43 Delayed diagnosis of abdominal Henoch-Schonlein purpura in children: A case report

  https://www.wjgnet.com/2307-8960/full/v11/i26/6311.htm

  Treatment with glucocorticoids and antibiotics in another hospital failed to control the disease. After admission to our hospital, CRP (an inflammation indicator) increased progressively, and abdominal pain was significantly relieved after intensive anti-infection treatment. Thus, the poor effect of glucocorticoids might be related to the failure of infection control. In our case, it is possible that a gastrointestinal infection triggered HSP. In fact, HSP commonly occurs after bacterial or viral infections (usually upper respiratory tract or intestinal infections), especially during the autumn season. Therefore, infection may play a role in the etiology of IgA vasculitis. […] Currently, glucocorticoids are considered effective for HSP gastrointestinal and joint symptoms, and early initiation can effectively relieve abdominal and joint symptoms, significantly relieve abdominal pain, improve the relief rate within 24 h, as well as reducing the risk of intussusception and intestinal bleeding. There is no evidence to prove that glucocorticoid therapy is effective in the regression and recurrence of rash, but we guess that early use of glucocorticoids may be associated with delayed appearance of rash in HSP. The patient in the present study was treated out of hospital with hydrocortisone, 5 mg/kg/d for 6 d, which may be an important reason for the delayed rash. […] HSP is a relatively common childhood disorder that may be difficult to diagnose when the typical signs of rash, abdominal pain, arthralgias, and nephritis do not occur simultaneously. Our case serves as a reminder that abdominal pain can appear long before the rash.

• #44 Determination of Risk Factors in Children Diagnosed With Henoch-Schönlein Purpura | Volume 33 – Issue 4 – December 2018 | Archives of Rheumatology

  https://archivesofrheumatology.org/full-text/949

  Having skin lesions in the upper extremities is a risk factor for gastrointestinal system involvement. […] Female sex, soft tissue swelling, and low mean platelet volume levels are risk factors for joint involvement. […] Female sex, 10 years of age, and high C-reactive protein levels are risk factors for renal involvement. […] Renal involvement in Henoch-Schnlein purpura is independent of gastrointestinal system or joint involvement and very close follow-up is required in the acute period particularly in girls 10 years with high C-reactive protein levels.

• #45 Determination of Risk Factors in Children Diagnosed With Henoch-Schönlein Purpura | Volume 33 – Issue 4 – December 2018 | Archives of Rheumatology

  https://archivesofrheumatology.org/full-text/949

  Having skin lesions in the upper extremities is a risk factor for gastrointestinal system involvement. […] Female sex, soft tissue swelling, and low mean platelet volume levels are risk factors for joint involvement. […] Female sex, 10 years of age, and high C-reactive protein levels are risk factors for renal involvement. […] Renal involvement in Henoch-Schnlein purpura is independent of gastrointestinal system or joint involvement and very close follow-up is required in the acute period particularly in girls 10 years with high C-reactive protein levels.

• #46 Henoch-Schönlein Purpura (IgA Vasculitis): Rapid Evidence Review | AAFP

  https://www.aafp.org/pubs/afp/issues/2020/0815/p229.html

  Henoch-Schnlein purpura, now called immunoglobulin A (IgA) vasculitis, is a systemic, immune complexmediated, small-vessel leukocytoclastic vasculitis characterized by nonthrombocytopenic palpable purpura, arthritis, and abdominal pain. Diagnostic testing is required only to exclude other etiologies of purpura, to identify renal involvement, and, if indicated, to determine its extent with biopsy. […] IgA vasculitis should be suspected in patients presenting with palpable purpura who also develop arthralgias (75% of patients) and abdominal pain (50% to 65% of patients). […] The European League Against Rheumatism/Paediatric Rheumatology European Society diagnostic criteria have a 100% sensitivity and 87% specificity in children, and a 99% sensitivity and 86% specificity in adults. These criteria are more accurate than the 1990 American College of Rheumatology criteria.

• #47 Henoch-Schönlein Purpura: A Literature Review | HTML | Acta Dermato-Venereologica

  https://www.medicaljournals.se/acta/content/html/10.2340/00015555-2733

  Henoch-Schnlein purpura is the most common childhood vasculitis, but may also affect adults. […] The diagnosis of HSP is criteria-based. The European League Against Rheumatism (EULAR), the Paediatric Rheumatology International Trials Organization (PRINTO) and the Paediatric Rheumatology European Society (PRES) published a revised set of criteria in 2010, with high sensitivity and specificity. […] HSP diagnosis is based on clinical criteria. The revised criteria developed by EULAR/PRINTO/PRES were published in 2010, and are the gold standard for the diagnosis of HSP. […] There are currently no specific biomarkers useful for diagnosis of HSP. Some biomarkers can show activity and prognosis of the disease, but none have proven clinically useful. […] Skin biopsies are the gold standard for diagnosing any cutaneous vasculitis. IgA-predominant vascular deposits are characteristic for HSP, but not sufficient for the diagnosis of HSP, as these deposits can be found in other vasculitic syndromes, erythema nodosum and venous stasis-related conditions.

• #48 Henoch-Schönlein purpura Information | Mount Sinai – New York

  https://www.mountsinai.org/health-library/diseases-conditions/henoch-sch-nlein-purpura

  IgA vasculitis is a disease that involves purple spots on the skin, joint pain, gastrointestinal problems, and glomerulonephritis (a type of kidney disorder). It is also known as Henoch-Schönlein purpura (HSP). […] The health care provider will look at your body and look at your skin. The physical exam will show skin sores (purpura, lesions) and joint tenderness. […] Tests may include: Urinalysis should be done in all cases. Complete blood count. The platelet should be normal. Coagulation tests: these should be normal. Skin biopsy, especially in adults. Blood tests to look for other causes of blood vessel inflammation, such as systemic lupus erythematosus, ANCA-associated vasculitis or hepatitis. In adults, a kidney biopsy should be done. Imaging tests of the abdomen if pain is present. […] Contact your provider if: You develop symptoms of IgA vasculitis, and they last for more than a few days. You have colored urine or low urine output after an episode.

• #49 The clinical implications of adult-onset henoch-schonelin purpura | Clinical and Molecular Allergy | Full Text

  https://clinicalmolecularallergy.biomedcentral.com/articles/10.1186/1476-7961-9-9

  Henoch-Schonlein Purpura (HSP) is a small vessel vasculitis mediated by IgA-immune complex deposition. It is characterized by the clinical tetrad of non-thrombocytopenic palpable purpura, abdominal pain, arthritis and renal involvement. […] The diagnosis is usually based on clinical presentation with tissue biopsy demonstrating leukocytoclastic vasculitis associated with IgA deposition (by immunofluorescence). Skin biopsy should be obtained from the lesion less than 24 hours old and typically shows the classical leukocytoclastic vasculitis in postcapillary venule with IgA deposition. Renal biopsy should be performed in case of uncertain diagnosis or severe renal impairment such as nephrotic syndrome. […] Elevated serum IgA levels have been associated with HSP in about 60% of cases. Urine analysis can vary from microscopic hematuria to nephritic-syndrome range proteinuria. Coagulation studies and the platelet count are usually normal. Inflammatory markers such as sedimentation rate (ESR) and C reactive protein (CRP) levels are often elevated.

• #50 Henoch-Schönlein Purpura | AAFP

  https://www.aafp.org/pubs/afp/issues/2009/1001/p697.html

  Because there are no definitive serologic tests for Henoch-Schönlein purpura, the purpose of laboratory evaluation is to identify complications or exclude other diseases. Useful baseline studies include renal function tests (e.g., electrolytes, blood urea nitrogen, creatinine, urinalysis), complete blood count with platelet count, coagulation profile (e.g., prothrombin time, partial thromboplastin time, fibrinogen, fibrin degradation products), and IgA levels. […] A blood pressure measurement and urinalysis should be performed at the time Henoch-Schönlein purpura is diagnosed and at each return physician office visit. Serum blood urea nitrogen and creatinine determination are needed if hematuria or proteinuria are identified. If the initial urinalysis is normal, or if there is isolated hematuria (without nephritic or nephrotic syndrome), a monthly urinalysis should be performed for the first six months after the diagnosis of Henoch-Schönlein purpura. This will detect the first signs of renal involvement, and facilitate early nephrology consultation and the initiation of steroids.

• #51 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. […] If symptoms are severe or additional expertise is needed, a rheumatologist can help evaluate and treat a child for HSP. One of the most important parts of HSP management is monitoring for kidney disease. […] Urine screening and monitoring of the blood pressure is recommended for at least 6 months after the initial diagnosis, so it is important to follow the screening instructions provided by your doctor.

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