It is the most common cause of nonthrombocytopenic purpura in children. The american college of rheumatology 1990 criteria for the. Henochschonlein purpura nephritis in children mcmaster. Clinical course of extrarenal symptoms in henochschonlein purpura. Henochschonlein purpura epidemiology bmj best practice. Peebles departments ofhaematology and pathology, stobhill general hospital, glasgow g21 3uw, uk. The clinical implications of adultonset henochschonelin. Dapsone 100 mgday may be effective in cases of hsp, perhaps through disrupting the abnormal immune response. Henochschonlein purpura american academy of pediatrics. Henochschonlein purpura nephritis is a rare kidney disease leading to.
Steroid therapy led to complete resolution of the symptoms. Renal involvement occurs in a variable proportion 2060% of children suffering from igavhsp 2, 7, 8. Hsp is mainly a disease of children that occurs predominantly between the ages of 3 and 15 years. While the disease is commonly seen in the pediatric age group, it is rarely seen in adults. Vast majority of children with igavhsp 97% develop features of renal involvement within 6 months of disease onset, but sometimes hspn may occur later 7, 9,10,11. With kidney involvement, there may be a loss of small amounts of blood and. Renal biopsy or immunosuppressive treatments is not recommended in. Hsp presents with purple spots on the skin purpura, arthralgia, digestive problems, and kidney injury. Although hsp is seen in infancy through adulthood, most documented cases affect children.
We report the case of a 93yearold caucasian lady who presented with nonthrombocytopenic purpuric rash and acute kidney injury after an episode of bronchitis. Hsp is the most common form of vasculitis in childhood and affects about 20 in 100,000 children. As with iga nephropathy, serum levels of iga are high in hsp and there are identical findings on renal biopsy. Typically, hsp is considered to be selflimiting, although renal involvement hsp purpura nephritis, hspn is the principal cause of morbidity from this disease. Henochschonlein purpura nephritis with renal interstitial. Henochschonlein purpura hsp is a small vessel vasculitis mediated by igaimmune complex deposition. There are iga and c3containing immune deposits in vessels and elsewhere, but the cause of these deposits is unknown. The disease is characterized by abnormal deposits of immunoglobulin a an antibody in the blood vessels, leading to their inflammation vasculitis.
Henoch schonlein purpura nephritis is a rare kidney disease leading to. Usually, the immune system makes antibodies, or proteins, to protect the body from foreign substances such as bacteria or viruses. For this reason, it is important to clarify the mechanism of. The nephritis of igavigavhsp will be abbreviated as hspn for this. Henochschonlein purpura and hodgkins disease jeanpierre ng, john murphy, e. Henochschonlein purpura hsp is an immunemediated vasculitis. The dilemma of spontaneous recovery even in patients with severe clinical and histologic. It is characterized by the clinical tetrad of nonthrombocytopenic palpable purpura, abdominal pain, arthritis and renal involvement. Schonlein identified the association of joint pain and purpura, and henoch identified the gi and renal involvement. Henochschonlein purpura childrens hospital of philadelphia. The clinicopathological characteristics of henochschonlein. Pulmonary involvement is a rare complication of hsp and diffuse alveolar hemorrhage dah is the most frequent clinical presentation. Henochschonlein purpura nephritis hspn and iga nephropathy igan are considered.
The renal disease spectrum ranges from urinary abnormalities including hematuria. A 65yearold man presented with a 1week history of abdominal pain and was noted to have acute kidney failure, with a serum creatinine level of 3. Henoch schonlein purpura is caused by an abnormal immune system response in which the bodys immune system attacks the bodys own cells and organs. Henoch schonlein purpura anaphylactoid purpura, hsp ncbi. Pathologically, it can be considered a form of leukocytoclastic vasculitis that can involve not only the skin but other tissues as well. Henochschonlein purpura hsp is the most common small vessel. Henochschonlein purpura in adulthood and childhood. We describe a case of an adolescent male with henochschonlein purpura hsp, presenting with cutaneous and gastrointestinal manifestations. Grading of acute and chronic renal lesions in henoch. A full recovery is typical for hsp patients, at least in children. This results in leakage of blood and proteins into. Henoch schonlein purpura as a cause of renal failure in an. A spanish retrospective study of hsp in adults suggested a higher frequency of kidney involvement than children, but the final outcome of hsp is equally good in patients of both age groups.
Henochschonlein purpura, kidney failure, pathologic examination, nephrotic syndrome, acute nephritis introduction henochschonlein purpura hsp, a nonthrombocytopenic purpura, is the most common systemic vasculitis in children. The incidence of this disease is between 30% and 60%. Henoch schonlein purpura hsp is a small vessel vasculitis that annually affects 10 to 20 children per 100,000 population. Nsaids may alleviate arthralgias but can aggravate gastrointestinal symptoms, and should be avoided in any patient with renal disease. Little is known about the real incidence of lung involvement during hsp in the pediatric age and about its diagnosis, management and outcome. About 50% of all cases occur at or before the age of 5 years.
Iga nephropathy and henoch schonlein purpura nephritis. Different histological classifications for henochschonlein purpura. The authors describe a case of hsp in a 14yearold adolescent girl who presented with atypical features of painful hemorrhagic bullae. A diagnosis of henochschonlein purpura is fairly easy to make if the. The renal outcome of 34 patients with henoch schonlein purpura nephritis was assessed clinically and by grading acute and chronic renal lesions using a system we applied to primary iga nephropathy. This wide range depends on criterion for performing a biopsy, but also on racial. What is the difference between iga nephropathy and henoch. Henoch schonlein purpura hsp, also known as iga vasculitis, is a disease of the skin, mucous membranes, and sometimes other organs that most commonly affects children. Henoch schonlein purpura hsp is an immune mediated disease associated immunoglobulin a iga deposition within the affected organs. Biopsies revealed a leukocytoclastic vasculitis in the skin and gastrointestinal tract.
Hsp is caused by an abnormal immune system response in which the bodys immune system attacks the bodys own cells and organs. Although retrospective studies suggest beneficial effects of some therapies, prospective randomized clinical trials proving treatment efficacy are still lacking. In the skin, the disease causes palpable purpura small, raised areas of bleeding underneath the skin, often with joint pain and abdominal pain. Renal damage eventually leads to chronic kidney disease in up to 20% of children with hsp. Henochschonlein purpura associated with celiac disease. Trapani s, micheli a, grisolia f, resti m, chiappini e, falcini f, et al. It is manifested by skin purpura, arthritis, abdominal pain, renal involvement, etc. Frequently, hsp leads to abdominal pain and intestinal bleeding, renal involvement and joint pain. Iga nephropathy and henochschonlein purpura propath. He described a fiveyearold boy presenting with generalized edema, macroscopic hematuria associated with a purpuric rash, colicky pain, bloody stools, and arthralgia. Henochschonlein purpura hsp is a systemic disease best characterized as a immunerelated, smallvessel vasculitis.
Pdf henochschonlein purpura hsp is the most common vasculitis of. Henochschonlein purpura hsp is a systemic vasculitis featured by pathological changes such as small vasculitis. The renal pathology of hsp closely resembles that of bergers nephritis, and some investigators have proposed that the two diseases are related 16. It more frequently affects children than adults and has a classic tetrad of rash palpable petechiae or purpura, arthralgias, abdominal pain, and kidney disease. It occurs most commonly in children ages 26, although it can occur at any age. The henochschonlein purpura hsp origa vasculitis is the most common vasculitis.
Risk of long term renal impairment and duration of follow up recommended for henochschonlein purpura with normal or minimal urinary findings. The small vessels of the skin, joints, kidneys, and digestive organs are particularly involved. Henochschonlein purpura is pathological condition characterized by inflammation and bleeding in the blood vessels of the various joints of the body, the intestines, and the renal system of the body. Outcome data from hsp nephritis in adults are from retrospective series. Henochschonlein purpura hsp, also called immunoglobulin a vasculitis igav, is a vascular disease that primarily affects small blood vessels.
Henoch schonlein purpura nephritis is a rare kidney disease leading to chronic kidney disease in a nonnegligible percentage of patients. Iga nephropathy and henochschonlein purpura nephritis. Clinicopathologic correlations in henochschonlein nephritis. Henochschonlein purpura national kidney federation. Significant clinicopathological differences with iga nephropathy zhongguo dang dai er ke za zhi 2012. Renal prognosis and related risk factors for henoch. Hspn is caused by the glomerular deposition of immunoglobulin a1 iga1containing immune complexes in the mesangium. Henochschonlein purpura is a small vessel vacuities in which complexes of immunoglobulin a iga and complement component 3 c3 are deposited on arterioles, capillaries, and venules. Glomerulonephritis hspn occurs in 3050 % of hsp patients, mostly in a mild form but a small percentage of patients present with nephrotic syndrome or renal failure.
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