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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 the Basics." The Basics patient education pieces are written in plain language, at the 5 th to 6th grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are written at the 10 th to 12th grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon. 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.) Use of UpToDate is subject to the Subscription and License Agreement. 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. 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