Henoch schonlein purpura as a cause of renal failure in an adult. Childhood iga vasculitis henoch schonlein purpuraadvances. Dec 10, 2018 henoch schonlein purpura hsp, also called immunoglobulin a vasculitis igav, is a vascular disease that primarily affects small blood vessels. Author jessica reidadam 1 affiliation 1 department of pediatrics, icahn school of medicine at mount sinai, new york, ny. Forty children with henoch schonlein purpura in acute phase admitted in our hospital from february 2012 to march 20 were enrolled in this study, forty healthy children were. New insights in the pathogenesis of immunoglobulin a vasculitis.
Aug 15, 2020 jauhola o, ronkainen j, koskimies o, et al. Threequarters of the patients were children or young people aged 20 years or younger n. Henoch schonlein purpura hsp schonlein henoch purpura, anaphylactoid purpura rhumatoid purpura diagnosis diagnosis of hsp is established when at least 2 of the following 4 criteria are present. New insights in the pathogenesis of immunoglobulin a. It frequently occurs following an infectious trigger and involves iga and c3 deposition in small vessel walls. Thirty children with diagnosis of hsp and thirty agematched healthy controls were enrolled in this study. Henoch schonlein purpura hsp is the most common systemic vasculitis of the childhood. Renal failure with progression to endstage disease is a longterm complication in up to 27% of adult patients with hsp and renal involvement 1. Oct 10, 2017 the objective of this study is to explore the value of fecal calprotectin fc for early screening of the abdominal type of henochschonlein purpura ahsp in children.
Henoch schonlein purpura hsp is an igamediated small vessel vasculitis with a predominant cutaneous involvement. To comprehensively evaluate the epidemiology, allergen and laboratory biomarkers reflecting hsp disease phases for the first time in this region. Predictive factors of relapse in adult with henochschonlein. By contrast, the glycolysis rate of activated nk cells in hsp group was not significantly. William heberden, a london physician, described the first cases of henoch schonlein purpura hsp in 1801.
However, its mechanisms and pathogenesis still need more exploration. Renal failure with progression to endstage disease is a longterm complication in up to 27% of. Henochschonlein purpura an overview sciencedirect topics. Renal biopsy reveals diffuse mesangial cell proliferation, crescent formation and mesangial iga staining on immunofluorescence, consistent with henoch schonlein nephritis. Henoch schonlein purpura hsp, also known as iga vasculitis, is a systemic vasculitis which is the most common vasculitis in children. Renal involvement is the principal cause of morbidity and mortality in children with hsp. During the 2012 international chapel hill conference the eponym. It gets its name from two german doctors, johann schonlein and eduard. Risk factors, renal damage, henoch schonlein purpura introduction henoch schonlein purpura hsp is a selflimited vasculitis occurring mostly in children 1. Recent developments in relation to henoch schonlein purpura hsp include. Henochschonlein purpura, children, retrospective studies.
This anaphylactoid purpura, is an immunoglobulin a iga mediated, smallvessel vasculitis that predominantly affects children 5. Although its exact pathophysiology remains unknown, henoch schonlein purpura has been reported in association with various medical conditions including hypersensitivity. Watson l1, richardson ar, holt rc, jones ca, beresford mw. Henoch schonlein purpura, purpura, nephritis, vasculitis. Patients with henoch schonlein purpura should be followed over the long term, since recurrent and chronic cases account for more than 20% of the total. Oct 18, 20 henochschonlein purpura is the most common systemic vasculitis of childhood.
Henoch schonlein purpura hsp is an igamediated disorder that most commonly occurs in children. Management of iga vasculitis nephritis henochschonlein purpura. The role of t cells in the development of henochschonlein. Hsp typically affects children and is selflimiting. In the majority of children, the outcome of henochschonlein purpura is excellent with spontaneous resolution of symptoms and signs. Hsp is a small vessel vasculitis that classically involves the clinic triad of inflammatory arthritis, ischemic abdominal pain, and purpura, although not all cases exhibit all three manifestations. We use your linkedin profile and activity data to personalize ads and to show you more relevant ads. Henoch schonlein purpura hsp is a small vessel vasculitis which is common in children and its etiology is unknown, but it is thought to be the result of immune complex reaction to various antigens. In the skin, the disease causes palpable purpura, often with joint pain and abdominal pain.
Henoch schonlein purpura hsp is an autoimmune, multisystem, acute vasculitis of childhood commonly involving the skin, gut, joints and the kidneys. Objectives to describe a group of patients treated at our service for henoch schonlein purpura, with emphasis on recurrent and chronic cases, and to compare clinical and demographic characteristics of patients with monocyclic and recurrent disease. An evaluation of 182 cases with henochschonlein purpura in china. We studied the association between adult henoch schonlein purpura hsp and malignant neoplasms. Henoch schonlein purpura hsp is a systemic disorder characterized by leukocytoclastic vasculitis involving the capillaries and the deposition of iga immune complexes. Henochschonlein purpura american academy of pediatrics. Following this treatment proposal, hspn patients with nephritic syndrome, nephrotic syndrome or cellular glomerular crescents will be placed on. This disease is mainly characterized by palpable purpura, arthritis arthralgia, abdominal pain, and kidney. Dec 21, 2011 helper t th cells comprising th1, th2, th17, and treg are involved in the pathogenesis of various vascular inflammations, and information about th cells in henoch schonlein purpura hsp is still controversial. Hsp is characterized by palpable purpura plus iga deposition on biopsy, arthritisarthralgia, renal involvement hematuria andor proteinuria, and.
Renal damage eventually leads to chronic kidney disease in up to 20% of children with hsp nephritis. Intestinal infarction and portal vein thrombosis in a patient. Henochschonlein purpura hsp is associated with iga1dominant. Oct 21, 2020 henoch schoenlein purpura hsp is an igamediated smallvessel vasculitis caused by deposition of immune complexes within vessel walls 75% of patients with hsp are less than 10 years old, with a peak between ages 4 and 6 years. The transferrin receptor is important for the import of iron in the cell by binding to. Therefore, the chcc 2012 replaced the eponym henochschonlein. Henochschonlein purpura litfl medical blog medical. In the skin, the disease causes palpable purpura small, raised areas of bleeding underneath the skin, often with joint pain and abdominal pain.
Henochschonlein 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. T cell cytokines determine the severity of experimental iga. The most striking feature of this form of vasculitis is a purplish rash, typically on the lower legs and buttocks. Of 417 patients, 240 were male and 177 female, with a median age at the time of disease diagnosis of 7. The characteristic pathological feature of this vasculitis is iga immune complex deposition in vascular walls of the affected organs. Hsp is most common in younger pts clinical red maculopapules on legs and buttocks. Henoch s purpura a type of henoch schonlein purpura in which abdominal symptoms predominate.
Cutaneous vasculitis in ulcerative colitis mimicking henoch schonlein purpura published online may 22, 2012. Headaches and mild behavioral changes suggest cns inv. Abstract henoch schonlein purpura hsp is the most common vasculitis in children. Renal manifestations of henoch schonlein purpura in a 6month prospective study of 223 children. Henochschonlein purpura with concurrent cytomegalovirus. Thus, we aimed to analyze the nk cells phenotype and.
Presence of red blood cells casts in the urinary sediment. A condition characterized by hemorrhages in the skin and mucous membranes that result in the appearance of purplish spots or patches. Analysis of 5 cases of pediatric henochschonlein purpura. Lund university, faculty of medicine doctoral dissertation series 2012. We assessed adult patients with hsp to identify the clinical and histopathological features and evaluate predictive factors of relapse. Henochschonlein purpura hsp the most common form of vasculitis in childh.
In the united states, the prevalence peaks in children 5 years old. Several triggers for hsp have been recognized, including infections, medications and malignancy. Apr 17, 20 the vast majority of people with henoch schonlein purpura hsp recover on their own within several weeks. Evolution of immunoglobulin a nephropathy into henoch. The disease is characterized by abnormal deposits of immunoglobulin a an antibody in the blood vessels, leading to their inflammation vasculitis. Pdf a case of henochschonlein purpura in the elderly. Original article risk factors for renal damage in henoch. Henochschonlein purpura, also known as iga vasculitis, is a disease of the skin, mucous membranes, and sometimes other organs that most commonly affects children. However, a small subset of patients will develop long. Relationship between immune parameters and organ involvement. Natural killer nk cells are innate lymphocytes, and there is a growing appreciation that cellular metabolism is important in determining the immune responsiveness of lymphocytes. The pathogenesis and treatment of pediatric henochschonlein. Henochschonlein purpura in children from the eastern. Henoch schonlein purpura hsp is a disease that causes small blood vessels to become inflamed and leak blood.
Henoch schonlein purpura is the most common form of small vessel vasculitis in childhood. Narchi h risk of long term renal impairment and duration of follow up recommended for henoch schonlein purpura with normal or minimal urinary findings. Sep 04, 2018 background henochschonlein purpura hsp is one of the most common vasculitides of childhood, with 1020 cases per 100,000 children. We present the case of a 20yearold male with hsp who presented with recurrent episodes of abdominal pain, followed by classical symptoms with an identified postinfectious aetiology. Henoch schonlein purpura hsp is an acute, systemic, vasculitis with igadominant immune deposits. Background henoch schonlein purpura hsp is the most common systemic vasculitis of the childhood. Methods data on 67 patients who had been treated since disease onset were analyzed. With the emphasis on educational value of hsp, which is the most common form of. Renal damage is the most common and severe complication of hsp 3. Severe henochschonlein purpura complicating infliximab. We report the case of a patient with vancomycininduced henoch schonlein purpura.
With kidney involvement, there may be a loss of small amounts of blood and protein in the urine, but this usually goes unnoticed. Reversible henoch schonlein purpura complicating adalimumab therapy. Hsp is a multisystem disease affecting skin, joints, gastrointestinal tract, and kidneys 2. In describing hsp, heberden wrote of a 5year old boy who was seized with pains and swellings in various partshe sometimes had pains in his belly with vomitingand the urine was tinged with blood. Henoch schonlein purpura is a smallvessel systemic vasculitis. The number of red blood cells and white blood cells in urine was. Henochschonlein purpura in children trnka 20 journal. Clinical significance of fecal calprotectin for the early. Limited information exists about the epidemiology, allergen and laboratory biomarkers reflecting hsp disease phases in northwestern china. Introduction henoch schonlein purpura hsp is the most common. Organ involvement, particularly in adults, and notably the kidneys and gastrointestinal tract may require therapeutic intervention and may have a less favorable outcome. Henochschonlein purpura is the most common childhood vasculitis, but may also affect adults.
Henoch schonlein purpura hsp is an iga mediated vasculitis predominantly affecting the pediatric population. Safe use of rituximab in a pregnant woman with henoch. In 1874, henoch described the purpura with abdominal pain and melena 4. Oct 30, 2019 henoch schonlein purpura hsp is the most widespread systemic smallvessel vasculitis of childhood. Henochschonlein purpura definition of henochschonlein.
Predictive factors of relapse in adult with henoch. The pathogenesis and treatment of pediatric henoch. Jan 20, 2012 my assignmnet of pediatric in year 5 iump. The estimated annual incidence of hsp in china is 14. Henoch schonlein purpura hsp is the most common type of vasculitis in childhood. Anaesthestic considerations in an obstetric patient with. Complement activation, cellular damaging and iga deposition suggest that hsp is an igamediated. Henoch schonlein purpura hsp is the most common type of connective. We present the case of a 20yearold male with hsp who presented with recurrent episodes of abdominal.
A retrospective analysis of children with henochschonlein. Henoch schonlein purpura is the most common childhood vasculitis, but may also affect adults. The study cohort included 40 children with ahsp treated at shengjing hospital of china medical university from november 2014 to november 2015, and 40 children hospitalized in the division of pediatric orthopedics in the. Henoch schonlein purpura a type of nonthrombocytopenic purpura, of unknown cause but thought to be due to a vasculitis.
Henochschonlein purpura synonyms, henochschonlein purpura pronunciation, henochschonlein purpura translation, english dictionary definition of henochschonlein purpura. Mar 31, 2016 henoch schonlein purpura hsp is the most common form of vasculitis in children. Dec 01, 2017 incidence of henoch schonlein purpura, kawasaki disease, and rare vasculitides in children of different ethnic origins lancet, 360 2002, pp. Henoch schonlein purpura hsp is a systemic vasculitis affecting the small blood vessels of the skin, the gastrointestinal tract and the kidneys.
Henoch schonlein purpura hsp is a multisystem, selflimiting, nongranulomatous, autoimmune immunoglobulin a iga mediated small vessel vasculitis primarily affecting children. So far the association of hsp and hepatitis a has been reported in only five cases1, 5. Henochschonlein purpura thefreedictionary medical dictionary. The interaction between leukocytes and vascular endothelial cells contributes to the pathogenesis of hsp.
Henochschonlein purpura symptoms and causes mayo clinic. With kidney involvement, there may be a loss of small amounts of blood and. This disease may leave a lifelong residual effect on renal system with a propensity for a relapse during pregnancy leading to hypertensive and hemorrhagic complications. A case of henochschonlein purpura with dilated coronary. Lymphoma presenting as henochschonlein purpura clinical. Author frank t saulsbury 1 affiliation 1 division of immunology and. Twelve patients were excluded because they failed to return. Iga vasculitis igav, also referred to as henochschonlein purpura. Schonlein defined the association of purpuric cutaneous lesions with arthralgia 3. Henochschonlein purpura hsp is a systemic vasculitis involving the small vessels with distinct clinical features. Its incidence is estimated to be 10 to 14 per 100,000, with boys affected twice as frequently as girls. In recent years, numerous studies have pointed to a dysfunction of t cells in the pathogenesis of hsp. Oct 01, 2009 renal disease is the most serious sequela of henoch schonlein purpura, occurring in 40 to 50 percent of patients.
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