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Henoch-Schonlein Purpura Practice Essentials, Background, Pathophysiology
Henoch-Schonlein Purpura Practice Essentials, Background, Pathophysiology
com/article/984105-overview
Henoch-Schonlein Purpura
Updated: Nov 10, 2016
Author: Noah S Scheinfeld, JD, MD, FAAD; Chief Editor: Craig B Langman, MD more...
OVERVIEW
Practice Essentials
Henoch-Schönlein purpura (HSP) is an acute immunoglobulin A (IgA)–mediated disorder
characterized by a generalized vasculitis involving the small vessels of the skin, the
gastrointestinal (GI) tract, the kidneys, the joints, and, rarely, the lungs and the central
nervous system (CNS). See the image below.
Headache
Anorexia
Fever
Subsequently, symptoms develop, of which the following are the most common:
Rash (95-100% of cases), especially involving the legs; this is the hallmark of the
disease
Abdominal pain and vomiting (35-85%)
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Because HSP can affect all organ systems, a full physical examination is indicated. Physical
findings in HSP may include the following:
Skin findings (usually the first sign of HSP) - Erythematous macular or urticarial
lesions, progressing to blanching papules and later to palpable purpura; typically
symmetrical and tend to be distributed in dependent body areas, such as the ankles
and lower legs in older children and adults and the back, buttocks, upper extremities,
and upper thighs in young children; hives, angioedema, and target lesions can also
occur
Renal findings - Acute glomerular lesions, including mesangial hypercellularity,
endocapillary proliferation, necrosis, cellular crescents, and leukocyte infiltration
Gastrointestinal (GI) findings - Abdominal pain, melena, bloody diarrhea, hematemesis,
duodenal ulcers, and massive GI hemorrhage
Joint findings - Arthralgia and swelling
Arthralgia is the presenting feature in as many as 25% of cases. Joints may be
swollen, tender, and painful. Warmth, erythema, and effusions are not typically
associated with HSP. The knees and ankles are most commonly affected. On rare
occasions, symptoms involve the fingers and wrists. Findings are transient but can
occur again during active disease. The joints are not permanently deformed.
Other findings - Vasculitis involving the myocardium or lungs; stenosing ureteritis,
priapism, penile edema, or orchitis; vasculitis involving the central nervous system
(CNS) and intracranial hemorrhage; bilateral subperiosteal orbital hematomas; adrenal
hematomas; acute pancreatitis as the sole presenting feature (rare); cystic changes of
the ovaries
Diagnosis
No specific diagnostic laboratory test is available to assess for markers of HSP. The
following general laboratory tests may be helpful for excluding other diagnoses and
evaluating renal function:
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Endoscopy
Renal biopsy (particularly when nephrotic syndrome persists and when renal function
deteriorates)
Management
Joint and soft tissue discomfort may be reduced by giving analgesics, such as the following:
Acetaminophen
Ibuprofen
Flurbiprofen
Ketoprofen
Naproxen
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Other treatment regimens have included IV or oral steroids with or without any of the
following:
Azathioprine
Cyclophosphamide
Cyclosporine
Dipyridamole
High-dose IV immunoglobulin G (IVIg)
Danazol
Fish oil
Background
Henoch-Schönlein purpura (HSP; also referred to as Schönlein-Henoch purpura,
anaphylactoid purpura, or purpura rheumatica) is an acute immunoglobulin A (IgA)–
mediated disorder characterized by a generalized vasculitis involving the small vessels of the
skin, the gastrointestinal (GI) tract, the kidneys, the joints, and, rarely, the lungs and the
central nervous system (CNS). [1, 2, 3] It is a subset of necrotizing vasculitis characterized by
fibrinoid destruction of blood vessels and leukocytoclasis.
The prevalence of HSP peaks in children aged 3-10 years, but the condition is also seen in
adults. [4] In the Northern hemisphere, the disease occurs mostly between November and
January. The male-to-female ratio is 1.5-2:1.
The dominant clinical features of HSP include cutaneous purpura, arthritis, abdominal pain,
GI bleeding, orchitis, and nephritis. In one half to two thirds of children, an upper respiratory
tract infection (URTI) precedes the clinical onset of HSP by 1-3 weeks. In general, patients
with HSP appear mildly ill. They often have a fever, with a temperature that usually does not
exceed 38°C (100.4°F).
HSP is typically an acute, self-limited illness, and treatment is primarily supportive. However,
one third of patients have 1 or more recurrences.
Pathophysiology
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IgA aggregates or IgA complexes with complement deposited in target organs, resulting in
elaboration of inflammatory mediators, including vascular prostaglandins such as
prostacyclin, may play a central role in the pathogenesis of HSP vasculitis.
Some have speculated that an antigen stimulates the production of IgA, which, in turn,
causes the vasculitis. Allergens, such as foods, horse serum, insect bites, exposure to cold,
and drugs (eg, ampicillin, erythromycin, penicillin, quinidine, and quinine), may precipitate
the illness.
Alterations in the production of interleukins (ILs) and growth factors may also play a
pathogenetic role. Tumor necrosis factor (TNF), IL-1, and IL-6 may mediate the inflammatory
process present in HSP. Transforming growth factor (TGF)–β is a recognized stimulant of
IgA production. The elevated levels of hepatocyte growth factor present during the acute
phase of HSP may reflect endothelial-cell damage or dysfunction. Increased levels of
vascular endothelial growth factor (VEGF) may at least partly induce these changes.
Cytokines have been implicated in the pathogenesis of HSP, and endothelins (ETs), which
are vasoconstrictor hormones produced by endothelial cells, may also have a role. levels of
ET-1 are substantially higher during the acute phase of the disease than during remission or
in a control group of children. However, ET-1 levels do not appear to be correlated with
morbidity, severity of disease, or acute-phase reactant response.
Although several lines of evidence suggest a genetic susceptibility to HSP, the fundamental
basis for this abnormality remains unclear.
A functional correlation of the IL1RN-2 allele and IL-1ra production in patients with IgA
nephropathy and HSP nephritis (HSPN) has been described. Therefore, gene polymorphism
may contribute to the diversity of clinical responses to inflammatory stimulation.
The prevalence of the human parvovirus B19 component NS1 gene in patients with HSP
and hypersensitivity vasculitis is increased.
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Researchers are currently investigating the importance of nitric oxide (NO) production in
disease activity. Inducible NO synthase polymorphism has been associated with
susceptibility to HSP in northwestern Spain. Aliyazicioglu et al have suggested that leptin
and NO may play a role in the immunoinflammatory process of HSP, especially in the acute
phase. [5]
HSP that is likely due to montelukast has been noted in patients who present with subacute
intestinal obstruction.
Yilmaz et al examined 28 children with HSP and 79 healthy children to evaluate activities of
protein C, free-protein S, and antithrombin; resistance to activated protein C; and levels of
fibrinogen. [6] D-dimer, thrombin-antithrombin (TAT) complex, prothrombin fragment (PF)-1,
PF-2, and von Willebrand factor antigen (vWAg) and its activity (RiCof) were also
investigated.
The investigators found that in patients with HSP, fibrinogen, D-dimer, TAT complex, PF-1,
PF-2, vWAg, and RiCof levels were significantly higher during the acute phase than during
the recovery phase and were significantly higher than those of control subjects. [6] The
severity of disease was significantly correlated with TAT, PF-1, PF-2, vWAg, and D-dimer
levels.
Higher levels of matrix metalloproteinase (MMP)-9 levels in urine and serum appear to
increase nephrologic severity in children with HSP.
Use of TNF-α blockers such as adalimumab may increase the risk of developing HSP.
HSP and IgA nephropathy appear to be related disorders. However, the precise relation
between them requires further definition. The question has been raised as to whether HSP
and IgA nephropathy are 2 aspects of a single disease entity or 2 distinct entities. The
following commonalities and differences have been noted:
IgA nephropathy almost exclusively involves young adults and typically affects only the
kidneys, whereas HSP affects mostly children and involves the skin and connective
tissues, GI tract, joints, and scrotum, as well as the kidneys [3, 7, 8]
The occurrence of extrarenal manifestations in IgA nephropathy is similar to that in
HSP
IgA nephropathy has developed in patients with a history of HSP, and HSP and IgA
nephropathy have occurred in the same families; in a survey of 40 families in which 2
or more members had IgA nephropathy, 5 presented with HSP [9]
Patients with HSP who undergo renal transplantation develop IgA deposits in the graft
The prevalence of both conditions is high in certain geographic areas
Similar changes in the IgA system (ie, high IgA, IgA-1C, IgA1-IC, IgA-fibronectin
aggregates, aberrantly glycosylated IgA in the circulation) occur in the 2 diseases [10,
11, 12]
Cystic changes in the ovaries of a prepubertal girl with HSP have been recorded
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Overall, the data tend to support the view that HSP and IgA nephropathy are distinct
diseases. [13] Zhou et al examined 31 children aged 3-15 years with IgA nephropathy and
120 children aged 4-15 years with HSP, noting their clinical manifestations, blood
biochemistries, serum immunology, and follow-up data. [14] Renal pathologic findings on light
microscopy, immunofluorescence study, and electron microscopy were analyzed and
compared between 31 children with IgA nephropathy and 32 children with HSP.
The age of onset was greater than 12 years in 25.8% of the children with IgA nephropathy
but in only 10% of those with HSP. [14] Clinical patterns of IgA nephropathy were similar to
those of HSP, but extrarenal manifestations were observed more often in patients with HSP.
All of the HSP patients had skin purpura, 59% had GI symptoms, and 47% had arthralgia.
Abdominal pain occurred in only 3.2% of children with IgA nephropathy. [14] In patients with
IgA nephrology and in patients with HSP, renal pathology revealed global sclerosis in 35.5%
and 3.1%, mesangial sclerosis in 41.9% and 6.3%, endothelial proliferation in 29% and
65.6%, and thin basement-membrane nephropathy in 6.5% and 0%, respectively.
In HSP, electronically dense deposits in HSP were sparse, loose, and widely spread in the
glomerular mesangium, in the subendothelial area, and even in the intrabasement
membranes, whereas in IgA nephropathy, the deposits were dense, lumpy, and mostly
limited to mesangium and paramesangium. [14]
Immunoglobulin G (IgG) was found in glomerular immune deposits in 71.9% of patients with
HSP but in only 19.4% of patients with IgA nephropathy. [14] No IgG deposit was observed in
81.6% of those with IgA nephropathy; most had IgA and immunoglobulin M (IgM) or C3
deposits. Predominant IgG deposits were found in 12.5% of HSP patients, with relatively
weak IgA deposits. Moreover, 6.3% of HSP patients had linear IgG deposits in the
glomerular capillary wall, a finding that was not noted in patients with IgA nephropathy.
The rate of complete remission was 72.5% in patients with HSP at an average of 20 months’
follow-up; the corresponding rate was 19.4% in those with IgA nephropathy after 34 months’
follow-up. [14] Moreover, 64.5% of patients with IgA nephropathy had consistent hematuria
and proteinuria, and 16.1% had active nephritides.
The important clinicopathologic differences Zhou et al found between HSP and IgA
nephropathy argue against the single-disease hypothesis.
Etiology
The etiology of HSP remains to be clearly defined but is thought to be multifactorial, with
genetic, environmental, and antigenic components. More than 75% of patients report
antecedent URTI, pharyngeal infection, or GI infection. Multiple bacterial and viral infectious
agents have been associated with the development of HSP, and cases also have been
reported after drug ingestions and vaccinations. [15]
Infections that may precede the development of HSP include the following:
Mononucleosis
Group A streptococcal infection (most common)
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Hepatitis
Mycoplasma infection
Campylobacter enteritis
Hepatitis C–related liver cirrhosis
Subacute bacterial endocarditis
Helicobacter pylori infection [16, 17, 18] (specifically noted in China [19] )
Yersinia infection
Shigella infection
Salmonella infection
Brucellosis
Legionella species
Parvovirus
Adenovirus
EBV infection
VZV infection
Vaccinations that may precede the development of HSP include the following:
Epidemiology
United States statistics
In the United States, the prevalence of HSP is approximately 14-15 cases per 100,000
population.
International statistics
In the United Kingdom, the estimated annual incidence of HSP is 20.4 cases per 100,000
population. [21] In a Norwegian community hospital, the prevalence of Henoch-Schoenlein
purpura was 3.3 cases per 100,000 inhabitants. [22]
In a study that examined the renal biopsy results of 65 children younger than 18 years
obtained by the Clinical Hospital in the Croatian region of Dalmatia over a 10-year period
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Nong et al reviewed the records of 107 Taiwanese pediatric patients diagnosed with HSP
between 1991 and 2005 who had a mean age of 6.2 ± 2.5 years (range, 2-13 years); the
male-to-female ratio was 1:0.7. [24] The primary symptoms included the following:
Rashes (95.3%)
GI symptoms (72.0%)
Joint involvement (46.7%)
Kidney involvement (28.0%)
Rashes (56.1%)
GI symptoms (35.5%)
Joint involvement (12.1%)
From January 1983 to June 2004, Suehiro et al followed 4502 patients at the Pediatric
Rheumatology clinic in Brazil. [25] A diagnosis of HSP was made in 203 cases (4.5%); 5
patients (0.1%) had acute hemorrhagic edema of infancy (AHEI). All patients with AHEI were
male, and the mean age at onset was 18 months (range, 8-21 months).
Age-related demographics
HSP primarily affects children; it may be seen in adults, but much less frequently. [2, 8] In the
United States, the prevalence peaks in children aged 5 years. Approximately 75% of cases
occur in children aged 2-11 years; HSP is rare in infants and young children. Older age at
the onset of HSP is associated with the development of chronic renal disease. [26] AHEI, a
related but milder condition, occurs in infants younger than 2 years. [27]
Sex-related demographics
HSP occurs more often in boys than in girls. In children, the male-to-female ratio is 1.5-2:1.
In adults, the male-to-female ratio is approximately 1:1.
Race-related demographics
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In a study from Thailand, patients most commonly presented between the ages of 3 and 5
years. [29] Frequency peaked from December to February. Organs involved included the skin
(100%), GI tract (74.5%), and kidneys (46.8%). Joints were also affected (42.6%). Renal
involvement was detected within the first 2 months in 16 patients (72.7%); however, it was
delayed until 6 months after diagnosis in 6 patients.
No risk factors for renal involvement could be identified in this study. [29] Mean follow-up time
was 2.6 years (range, 1-5 years). Residual renal disease occurred in 6 (38%) of 16 patients,
but none had end-stage renal disease (ESRD).
In a study from China, a male predominance was observed in children but not in adults. [30]
Preceding infection was noted in 40.5% of children and 31.6% of adults; 8.3% of children
and 13.2% of adults were receiving medication at the onset of the disease.
The investigators found that abdominal pain was more common in children than adults
(70.2% vs 28.9%), but renal involvement was more common and severe in adults than in
children; this involvement manifested as frequent hypertension and heavy proteinuria. [30]
During acute attacks, leukocytosis, thrombocytosis, and elevated serum C-reactive protein
(CRP) levels were most frequently observed in children, whereas elevated serum IgA and
cryoglobulin levels were most common in adults.
A study of 450 cases from Turkey showed that girls, patients with atypical presentations, and
patients undergoing early corticosteroid treatment had an increased risk of developing
kidney disease; relapses occurred more often in children treated with corticosteroids. [31]
Familial kindreds with HSP have been noted in Taiwanese aboriginal people. [32]
Prognosis
HSP is generally a benign disease with an excellent prognosis. Spontaneous resolution is
usual: Most patients experience complete resolution of symptoms within 8 weeks, and
probably fewer than 5% experience chronic symptoms. Initial attacks of HSP can last several
months, and relapses are possible. HSP is fatal only in the rarest of cases.
A clinical course with complete resolution of the disease usually occurs in patients with the
following:
Children younger than 3 years usually have a shorter, milder course than older patients do,
as well as fewer recurrences.
Recurrences occur in as many as 50% of patients within 6 weeks but can happen as late as
7 years after the initial disease. A study by Calvo-Río et al indicated that in patients with
HSP, the chance of relapse is greater in those with GI and joint manifestations, with 72.3% of
patients in the study with abdominal pain relapsing, compared with 62.3% of those who did
not, and 27.8% of patients with joint manifestations relapsing, compared with 15.5% of those
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Although HSP generally resolves without permanent consequences, serious GI and renal
complications may occur, and the higher the number of recurrences, the higher the likelihood
of permanent renal damage. Potential GI complications include intussusception (usually
ileoileal), bowel infarction, bowel perforation, hydrops of the gallbladder, pancreatitis, and
massive GI bleeding.
Kidney damage related to HSP is the primary cause of morbidity and mortality. As many as
15% of patients may have long-term renal insufficiency, but no more than 1-2% will have
ESRD. As many as 20% of children who have HSP and are treated in specialized centers
require hemodialysis. The renal prognosis appears to be worse in adults than in children (in
particular, those aged ≤6 years).
Bloody stools
Rash persistence
Hematuria and proteinuria (patients with only hematuria do not develop ESRD, but
about 15% of patients with hematuria and proteinuria do develop ESRD)
Signs of nephritis or nephrotic syndrome (50% of patients progress to ESRD within 10
years)
Renal biopsy with extensive glomerular crescents
Patients with a normal urinalysis at 6 months and without previous renal involvement have
not gone on to develop kidney problems. [34]
Pregnant women who had HSP during childhood appear to be at increased risk for
developing hypertension and proteinuria during pregnancy. [35]
Patient Education
Patients should be informed that the disease is most likely to resolve with few residual
adverse effects but that relapses are possible. The clinician should explain that severe
kidney involvement is rare but that if it does occur, aggressive treatment may be required.
Clinical Presentation
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Author
Noah S Scheinfeld, JD, MD, FAAD is a member of the following medical societies: American
Academy of Dermatology
Coauthor(s)
Chief Editor
Craig B Langman, MD The Isaac A Abt, MD, Professor of Kidney Diseases, Northwestern
University, The Feinberg School of Medicine; Division Head of Kidney Diseases, The Ann
and Robert H Lurie Children's Hospital of Chicago
Disclosure: Received income in an amount equal to or greater than $250 from: Alexion
Pharmaceuticals; Raptor Pharmaceuticals (now Horizon Pharmaceuticals); ; Dicerna, QLT
Pharmaceuticals.
Acknowledgements
Jeffrey L Arnold, MD, FACEP Chairman, Department of Emergency Medicine, Santa Clara
Valley Medical Center
Jeffrey L Arnold, MD, FACEP is a member of the following medical societies: American
Academy of Emergency Medicine and American College of Physicians
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Steven C Dronen, MD, FAAEM is a member of the following medical societies: American
Academy of Emergency Medicine and Society for Academic Emergency Medicine
Edmond A Hooker II, MD, DrPH, FAAEM Associate Professor, Department of Health
Services Administration, Xavier University, Cincinnati, Ohio; Assistant Professor, Department
of Emergency Medicine, University of Cincinnati College of Medicine
Edmond A Hooker II, MD, DrPH, FAAEM is a member of the following medical societies:
American Academy of Emergency Medicine, American Public Health Association, Society for
Academic Emergency Medicine, and Southern Medical Association
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Richard Neiberger, MD, PhD Director of Pediatric Renal Stone Disease Clinic, Associate
Professor, Department of Pediatrics, Division of Nephrology, University of Florida College of
Medicine and Shands Hospital
Richard Neiberger, MD, PhD is a member of the following medical societies: American
Academy of Pediatrics, American Federation for Medical Research, American Medical
Association, American Society of Nephrology, American Society of Pediatric Nephrology,
Christian Medical & Dental Society, Florida Medical Association, International Society for
Peritoneal Dialysis, International Society of Nephrology, National Kidney Foundation, New
York Academy of Sciences, Shock Society, Sigma Xi, Southern Medical Association,
Southern Society for Pediatric Research, and Southwest Pediatric Nephrology Study Group
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Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha,
American Academy of Dermatology, American Medical Association, and Texas Medical
Association
Robert J Willard, MD Dermatologist and Mohs Surgeon, Private Practice, Dermatology and
Mohs Surgery Center, PC
Wayne Wolfram, MD, MPH, is a member of the following medical societies: American
Academy of Emergency Medicine, American Academy of Pediatrics, and Society for
Academic Emergency Medicine
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