Download Henoch-Schönlein purpura (immunoglobulin A vasculitis

Henoch-Schönlein purpura (immunoglobulin A vasculitis): Management
Authors:
Fatma Dedeoglu, MD
Susan Kim, MD, MMSc
Section Editor:
Robert Sundel, MD
Deputy Editor:
Elizabeth TePas, MD, MS
Contributor Disclosures
All topics are updated as new evidence becomes available and our peer review process is complete.
Literature review current through: Sep 2016. | This topic last updated: Dec 07, 2015.
INTRODUCTION — Henoch-Schönlein purpura (HSP), also called immunoglobulin A vasculitis (IgAV) [1], is the most
common systemic vasculitis of childhood. Ninety percent of cases occur in the pediatric age group. In contrast to other forms
of systemic vasculitis, HSP (IgAV) is usually self-limited and is characterized by a tetrad of clinical manifestations that vary in
their presence and order of presentation:
●Palpable purpura in patients with neither thrombocytopenia nor coagulopathy
●Arthralgia and/or arthritis
●Abdominal pain
●Renal disease
Management of HSP (IgAV) includes supportive care, symptomatic therapy, and targeted treatment to decrease the risk of
complications. The management and prognosis of HSP (IgAV) are presented here. The pathogenesis, clinical
manifestations, diagnosis, and differential diagnosis of HSP (IgAV) are discussed separately. (See "Henoch-Schönlein
purpura (immunoglobulin A vasculitis): Clinical manifestations and diagnosis".)
MANAGEMENT — The vast majority of patients with Henoch-Schönlein purpura (HSP), also called immunoglobulin A
vasculitis (IgAV), recover spontaneously. Thus, care is primarily supportive and includes adequate hydration, rest, and
symptomatic relief of pain.
Most patients may be cared for in the ambulatory setting. In these patients, therapy is directed toward adequate oral
hydration and symptomatic relief. Edema of the lower extremities, buttocks, and genital area is improved with bed
rest and/or elevating the affected area.
Treatment of pain — Symptomatic treatment for abdominal and joint pain in patients with HSP (IgAV) includes the use
of acetaminophen or nonsteroidal antiinflammatory drugs (NSAIDs). Clinical experience and limited observational data
suggest that the antiinflammatory effects of glucocorticoids can relieve many of the manifestations of HSP (IgAV), including
the rash, arthritis/arthralgias, scrotal pain/orchitis, and gastrointestinal pain [2]. However, glucocorticoids are not usually
indicated for these manifestations, unless symptoms are severe, given the limited data and the potential side effects of the
therapy. Patients with HSP (IgAV) who have gastrointestinal involvement, including bowel wall edema, may have disrupted
absorption of enteral medications. Thus, failure of NSAIDs or oral glucocorticoids to benefit certain patients may be due to
poor absorption. (See "Major side effects of systemic glucocorticoids".)
Mild-to-moderate pain — There are no randomized, controlled studies of the use of NSAIDs for symptomatic pain relief in
patients with HSP (IgAV). However, there is no suggestion that the risk of gastrointestinal hemorrhage in a child with bowel
vasculitis is increased by the use of cyclooxygenase inhibitors. Thus, we do not hesitate to use NSAIDs to control joint and
abdominal pain in patients with HSP (IgAV). However, NSAIDs may be contraindicated in patients with active
gastrointestinal bleeding or glomerulonephritis, because of their effects on platelets and renal perfusion.
For symptomatic relief of pain in patients with HSP (IgAV), we use naproxen, 10 to 20 mg/kg divided into two doses per day,
because of its ease of dosing. In adolescents and adults, a maximum total daily dose of 1500 mg may be used for a few
days. For longer-term use, a maximum dose of 1000 mg/day is suggested. Ibuprofenand other NSAIDs are equally effective
and may be better tolerated in some patients, although ibuprofen requires more frequent dosing.
Severe pain — Most studies show that glucocorticoid therapy shortens the duration of abdominal pain in patients with HSP
(IgAV) [3-7]. However, glucocorticoids do not appear to otherwise impact the clinical course [8,9]. Thus, we suggest
using prednisone (1 to 2 mg/kg per day, maximum dose of 60 to 80 mg per day) only in patients with symptoms significant
enough to affect their oral intake, interfere with their ability to ambulate and perform activities of daily living, and/or require
hospitalization. In patients who cannot tolerate oral medications, we administer equivalent doses of
parenteral methylprednisolone (0.8 to 1.6 mg/kg per day, maximum dose of 64 mg per day). Intravenous methylprednisolone
may be more beneficial early in the disease course when patients have active gastrointestinal disease, due to submucosal
edema and hemorrhage altering absorption of oral medications. (See 'Prevention of complications' below
and 'Hospitalization' below.)
When glucocorticoids are used for treating HSP (IgAV), it is important to remember that inflammation may be ameliorated,
but the pathophysiology of the disease does not appear to be affected. Accordingly, the glucocorticoid dose must be lowered
slowly, typically over four to eight weeks, to minimize the chance of precipitating a disease flare by overly aggressive
medication tapering. In addition, patients who are treated with glucocorticoids for severe abdominal pain require particular
vigilance, since these medications can obscure the signs and symptoms of abdominal catastrophes associated with HSP
(IgAV). (See "Henoch-Schönlein purpura (immunoglobulin A vasculitis): Clinical manifestations and diagnosis", section on
'Gastrointestinal symptoms'and 'Prevention of complications' below.)
Treatment of renal disease — Treatment of active renal disease in HSP (IgAV) and renal transplantation for end-stage
renal disease are discussed in detail separately. (See "Renal manifestations of Henoch-Schönlein purpura (IgA vasculitis)",
section on 'Treatment'.)
Hospitalization — Hospitalization is warranted for the following indications in patients with HSP (IgAV):
●Inability to maintain adequate hydration with oral intake
●Severe abdominal pain
●Significant gastrointestinal bleeding
●Changes in mental status
●Severe joint involvement limiting ambulation and/or self-care
●Renal insufficiency (elevated creatinine), hypertension, and/or nephrotic syndrome
Intravenous hydration is provided in the hospital setting if the patient cannot maintain adequate oral intake of fluids. In
addition, parenteral nutrition may be required in patients who have a severe and prolonged course of abdominal symptoms
that precludes enteral nutrition.
Hospitalized patients also are monitored for potential complications from their disease. Frequent assessment of vital signs,
urine output, and hematocrit, as well as serial abdominal examinations and stool examination for blood are required.
(See "Henoch-Schönlein purpura (immunoglobulin A vasculitis): Clinical manifestations and diagnosis", section on
'Laboratory findings' and "Henoch-Schönlein purpura (immunoglobulin A vasculitis): Clinical manifestations and diagnosis",
section on 'Additional evaluation'.)
●Patients with significant anemia may require red blood cell transfusion, as indicated by tachycardia and hypotension
in a patient with a low hematocrit level. Blood loss from hemorrhage into the skin, gastrointestinal tract, or urine rarely
depresses red blood cell levels enough to necessitate a transfusion, although superimposed decreased production
due to inflammation may result in symptomatic anemia. (See"Red blood cell transfusion in infants and children:
Indications".)
●Patients with an abdominal examination consistent with either signs of obstruction or peritonitis ("surgical abdomen")
require prompt evaluation, including surgical consultation and/or intervention for possible intussusception, bowel
infarction, or perforation. Bowel vasculitis often cannot be diagnosed by examination alone. In such cases, diagnostic
imaging is necessary. Abdominal ultrasonography is typically used because it can detect changes in bowel wall
thickness, hematomas, peritoneal fluid, and intussusception [10-12]. Contrast enema studies are not useful because
they may not detect small bowel intussusceptions, which are commonly seen in patients with HSP (IgAV) [11,12].
(See "Henoch-Schönlein purpura (immunoglobulin A vasculitis): Clinical manifestations and diagnosis", section on
'Imaging studies'.)
●Patients with acute changes in behavior or mental status require evaluation for a potential intracranial complication,
such as hemorrhage. These are rare events in HSP (IgAV). Most central nervous system findings are transient and do
not need further intervention. (See "Henoch-Schönlein purpura (immunoglobulin A vasculitis): Clinical manifestations
and diagnosis", section on 'Other organ involvement'.)
●Patients with renal disease may be hypertensive and require antihypertensive therapy. Close monitoring of fluid and
electrolyte management is imperative in patients with decreased renal function. Patients who present with nephrotic
syndrome, hypertension, or renal insufficiency are at increased risk for long-term renal morbidity. (See "Prevention
and management of acute kidney injury (acute renal failure) in children"and "Renal manifestations of HenochSchönlein purpura (IgA vasculitis)", section on 'Prognosis'.)
●Patients with severe arthritis usually improve with the use of NSAIDs. The risk of gastrointestinal hemorrhage
resulting from bowel vasculitis has not been shown to increase when inhibitors of cyclooxygenase are used, and
therefore these agents are not contraindicated in HSP (IgAV). When these medications fail, administration of
glucocorticoids will generally result in a rapid resolution of the arthritis. Rarely, a brief hospitalization may be required
in order to provide the medications needed to relieve the arthritis. (See 'Treatment of pain' above.)
Prevention of complications — The use of glucocorticoids in patients with HSP (IgAV) is controversial. Although many
clinicians describe a rapid symptomatic improvement in patients who receive glucocorticoids, data are insufficient to confirm
such an effect, and it is questionable as to whether such treatment alters clinical outcomes. In addition, there are potential
side effects of glucocorticoid therapy, including the risk of obscuring the signs of compromised bowel viability if treatment is
started after an intussusception has occurred. As a result, we do not recommend routine glucocorticoid therapy for patients
with HSP (IgAV) to treat symptoms or prevent renal or gastrointestinal complications. (See 'Treatment of pain' above.)
Initial reported benefits of glucocorticoid therapy in preventing complications from a systematic review that included 3
randomized trials and 12 retrospective studies included decreased risk of intussusception, renal involvement, and
recurrence of disease [3]. One subsequent retrospective study found a decreased risk of gastrointestinal procedures
(endoscopy, imaging, and surgery) [13].However, a prospective study of 223 children in Finland published after the
systematic review found no effect of prednisone on the timing of the appearance of nephritis [14] or on the clinical course
during six months of follow-up [15]. A subsequent meta-analysis incorporating the results of this trial reported no differences
in the risk of persistent kidney disease at 6 months (three studies) and 12 months (three studies) in children given
prednisone for 14 to 28 days at presentation compared with those who received placebo or supportive care [16]. This
analysis also found no benefit in using antiplatelet agents such as heparin to prevent persistent renal disease. Failure to
demonstrate benefit may be an artifact of the low prevalence rate of complications and the small number of patients studied
[4,17].
Additional therapies for the treatment of recalcitrant disease are not well-defined, but there are reports and case series that
suggest benefits from medications such as colchicine and dapsone [18,19]. There are also reports of benefit from additional
immunosuppression in cases of aggressive, recalcitrant disease, typically with renal involvement [20]. Management of renal
disease in HSP (IgAV) is discussed in detail separately. (See"Renal manifestations of Henoch-Schönlein purpura (IgA
vasculitis)", section on 'Treatment'.)
PROGNOSIS — The short- and long-term outcomes of children with Henoch-Schönlein purpura (HSP), also called
immunoglobulin A vasculitis (IgAV), are generally excellent. In the absence of significant renal disease, the initial episode of
HSP (IgAV) typically resolves within one month.
In two-thirds of children, there are no recurrent episodes [2,21]. In the remaining one-third of patients, HSP (IgAV) recurs at
least once, typically within four months of the initial presentation [2,22,23]. Each subsequent episode has similar clinical
findings, but is generally milder and/or shorter in duration than the preceding one. Recurrences are more common in
patients with nephritis, in those with evidence of acute inflammation (eg, elevated erythrocyte sedimentation rate [ESR]), and
in patients who received treatment with glucocorticoids [22]. These findings suggest that patients who have a more severe
course of HSP (IgAV) are at increased risk of recurrence.
One retrospective review from Israel reported a longer mean interval time of 13.5 months between the first and second
episode of HSP (IgAV) than was reported previously [24]. In addition, there was no difference in clinical and laboratory
findings between patients with recurrent disease and those without recurrence. The reasons for these differences between
study results are unclear.
Morbidity in the initial phase of HSP (IgAV) is primarily a result of gastrointestinal complications, including intussusception
and, less commonly, bowel ischemia, bowel perforation, or pancreatitis. (See "Henoch-Schönlein purpura (immunoglobulin A
vasculitis): Clinical manifestations and diagnosis", section on 'Gastrointestinal symptoms'.)
The long-term morbidity in patients with HSP (IgAV) is a result of renal disease. The risk of chronic renal disease is
increased in adults [25]. The severity of renal involvement correlates with the severity of the initial renal presentation and
histologic changes seen on renal biopsy. Risk factors for a worse renal prognosis include nephrotic range proteinuria,
elevated serum creatinine, hypertension, and certain histologic findings. The supportive data are discussed elsewhere.
(See "Renal manifestations of Henoch-Schönlein purpura (IgA vasculitis)", section on 'Prognosis'.)
FOLLOW-UP — Ninety percent of children who develop renal involvement do so within two months of onset, and 97 percent
within six months [26]. Accordingly, all patients with Henoch-Schönlein purpura (HSP), also called immunoglobulin A
vasculitis (IgAV), should be followed with urinalysis and blood pressure monitoring weekly or biweekly for the first one to two
months after presentation. Results from one study suggested that home urine dipstick testing was adequate for detecting
development of nephritis [14]. Once the disease appears to be subsiding, additional follow-up for urine and blood pressure
monitoring should be scheduled monthly at first, then every other month until one year after the initial presentation. To
identify patients who may develop late renal disease, continued screening (eg, urinalysis and blood pressure
measurements) should be performed by the primary care clinician during subsequent well-child visits. (See "HenochSchönlein purpura (immunoglobulin A vasculitis): Clinical manifestations and diagnosis", section on 'Renal studies'.)
A serum creatinine should be obtained to assess renal function in any patient with significant or persistent urinary
abnormalities or elevated blood pressure.
Patients with persistent proteinuria, hypertension, or renal insufficiency should be referred to a pediatric nephrologist for
further evaluation and treatment. In addition, pregnant women with a history of HSP (IgAV) should be monitored closely, as
they have a higher risk of hypertension [27].
INFORMATION FOR PATIENTS — UpToDate offers two types of patient education materials, "The Basics" and "Beyond
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Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail these topics to
your patients. (You can also locate patient education articles on a variety of subjects by searching on "patient info" and the
keyword(s) of interest.)
●Basics topic (see "Patient education: Henoch-Schönlein purpura (IgA vasculitis) (The Basics)")
●Beyond the Basics topic (see "Patient education: Vasculitis (Beyond the Basics)")
SUMMARY AND RECOMMENDATIONS
●Most patients with Henoch-Schönlein purpura (HSP), also called IgA vasculitis (IgAV), may be cared for in the
ambulatory setting with therapy directed toward adequate oral hydration, bed rest, and symptomatic relief of joint and
abdominal pain.
●In patients with joint and/or abdominal pain, we suggest the use of a nonsteroidal antiinflammatory agent (Grade
2C). We typically use naproxen (10 to 20 mg/kg divided into two doses per day). A maximum dose of naproxen of
1500 mg per day may be used for a few days provided that adequate hydration is maintained. If more than a week of
nonsteroidal antiinflammatory drug (NSAID) treatment is necessary, the dose of naproxen should not exceed 1000 mg
per day. (See 'Mild-to-moderate pain' above.)
●In patients with severe abdominal pain that interferes with their oral intake and who fail to respond to a nonsteroidal
antiinflammatory agent, we suggest the use of systemic glucocorticoids (Grade 2C). We use oral prednisone (1 to
2 mg/kg per day) for children or adults, with a maximum dose of 60 to 80 mg per day, or equivalent doses of
intravenous methylprednisolone (0.8 to 1.6 mg/kg per day, maximum dose of 64 mg per day). Patients who are
treated with glucocorticoids for severe abdominal pain require particular vigilance, since these medications can
obscure the signs and symptoms of abdominal catastrophes associated with HSP (IgAV). Glucocorticoids should be
tapered slowly, by no more than 25 percent per week, lest symptoms relapse. (See 'Severe pain' above.)
●Hospitalization is indicated in patients who fail to maintain oral hydration and require the administration of
intravenous fluids. Inpatient management may also be necessary to manage patients who have significant
gastrointestinal bleeding, severe abdominal pain, changes in mental status, severe joint involvement limiting
ambulation and/or self-care, or evidence of significant renal disease (elevated creatinine, hypertension, or
proteinuria). (See 'Hospitalization' above.)
●We do not recommend glucocorticoid administration to prevent renal or gastrointestinal complications (Grade 1B).
(See 'Prevention of complications' above.)
●Although prognosis is excellent in children with HSP (IgAV), a small minority of patients (<1 percent) develops longterm complications, primarily renal disease. In adults, the risk of significant renal disease is increased. At present,
there is no known therapeutic regimen to forestall development of renal involvement. Therapy for optimal control of
HSP (IgAV)-related nephritis or nephrosis is discussed elsewhere. (See 'Prognosis' above and "Renal manifestations
of Henoch-Schönlein purpura (IgA vasculitis)", section on 'Treatment'.)
●Patients who have developed HSP (IgAV) should be seen in follow-up with screening for urinary abnormalities and
elevated blood pressure to identify patients with significant and potentially progressive renal involvement.
(See 'Follow-up' above.)
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REFERENCES
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
14.
15.
16.
Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference
Nomenclature of Vasculitides. Arthritis Rheum 2013; 65:1.
Saulsbury FT. Henoch-Schönlein purpura in children. Report of 100 patients and review of the literature. Medicine
(Baltimore) 1999; 78:395.
Weiss PF, Feinstein JA, Luan X, et al. Effects of corticosteroid on Henoch-Schönlein purpura: a systematic review.
Pediatrics 2007; 120:1079.
Rosenblum ND, Winter HS. Steroid effects on the course of abdominal pain in children with Henoch-Schonlein
purpura. Pediatrics 1987; 79:1018.
Leung SP. Use of intravenous hydrocortisone in Henoch-Schonlein purpura. J Paediatr Child Health 2001; 37:309.
ALLEN DM, DIAMOND LK, HOWELL DA. Anaphylactoid purpura in children (Schonlein-Henoch syndrome):
review with a follow-up of the renal complications. AMA J Dis Child 1960; 99:833.
Szer IS. Gastrointestinal and renal involvement in vasculitis: management strategies in Henoch-Schönlein purpura.
Cleve Clin J Med 1999; 66:312.
Dudley J, Smith G, Llewelyn-Edwards A, et al. Randomised, double-blind, placebo-controlled trial to determine
whether steroids reduce the incidence and severity of nephropathy in Henoch-Schonlein Purpura (HSP). Arch Dis Child
2013; 98:756.
Davin JC, Coppo R. Pitfalls in recommending evidence-based guidelines for a protean disease like HenochSchönlein purpura nephritis. Pediatr Nephrol 2013; 28:1897.
Chang WL, Yang YH, Lin YT, Chiang BL. Gastrointestinal manifestations in Henoch-Schönlein purpura: a review of
261 patients. Acta Paediatr 2004; 93:1427.
Choong CK, Beasley SW. Intra-abdominal manifestations of Henoch-Schönlein purpura. J Paediatr Child Health
1998; 34:405.
Schwab J, Benya E, Lin R, Majd K. Contrast enema in children with Henoch-Schönlein purpura. J Pediatr Surg
2005; 40:1221.
Weiss PF, Klink AJ, Localio R, et al. Corticosteroids may improve clinical outcomes during hospitalization for
Henoch-Schönlein purpura. Pediatrics 2010; 126:674.
Jauhola O, Ronkainen J, Koskimies O, et al. Renal manifestations of Henoch-Schonlein purpura in a 6-month
prospective study of 223 children. Arch Dis Child 2010; 95:877.
Jauhola O, Ronkainen J, Koskimies O, et al. Clinical course of extrarenal symptoms in Henoch-Schonlein purpura:
a 6-month prospective study. Arch Dis Child 2010; 95:871.
Hahn D, Hodson EM, Willis NS, Craig JC. Interventions for preventing and treating kidney disease in HenochSchönlein Purpura (HSP). Cochrane Database Syst Rev 2015; :CD005128.
17.
18.
19.
20.
21.
22.
23.
24.
25.
26.
27.
Huber AM, King J, McLaine P, et al. A randomized, placebo-controlled trial of prednisone in early Henoch
Schönlein Purpura [ISRCTN85109383]. BMC Med 2004; 2:7.
Saulsbury FT. Successful treatment of prolonged Henoch-Schönlein purpura with colchicine. Clin Pediatr (Phila)
2009; 48:866.
Shin JI, Lee JS, Chung KS. Dapsone therapy for Henoch-Schonlein purpura. Arch Dis Child 2006; 91:714.
Chartapisak W, Opastirakul S, Hodson EM, et al. Interventions for preventing and treating kidney disease in
Henoch-Schönlein Purpura (HSP). Cochrane Database Syst Rev 2009; :CD005128.
Meadow SR. The prognosis of Henoch Schoenlein nephritis. Clin Nephrol 1978; 9:87.
Trapani S, Micheli A, Grisolia F, et al. Henoch Schonlein purpura in childhood: epidemiological and clinical analysis
of 150 cases over a 5-year period and review of literature. Semin Arthritis Rheum 2005; 35:143.
Haroon M. Should children with Henoch-Schonlein purpura and abdominal pain be treated with steroids? Arch Dis
Child 2005; 90:1196.
Prais D, Amir J, Nussinovitch M. Recurrent Henoch-Schönlein purpura in children. J Clin Rheumatol 2007; 13:25.
Lu S, Liu D, Xiao J, et al. Comparison between adults and children with Henoch-Schönlein purpura nephritis.
Pediatr Nephrol 2015; 30:791.
Narchi H. Risk of long term renal impairment and duration of follow up recommended for Henoch-Schonlein
purpura with normal or minimal urinary findings: a systematic review. Arch Dis Child 2005; 90:916.
Ronkainen J, Nuutinen M, Koskimies O. The adult kidney 24 years after childhood Henoch-Schönlein purpura: a
retrospective cohort study. Lancet 2002; 360:666.