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IgA vasculitis (Henoch-Schönlein purpura): 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: Jul 2020. | This topic last updated: May 20, 2019 INTRODUCTION Immunoglobulin A vasculitis (IgA vasculitis [IgAV]), formerly called HenochSchönlein purpura (HSP) [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, IgAV (HSP) 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 IgAV (HSP) includes supportive care, symptomatic therapy, and targeted treatment to decrease the risk of complications The management and prognosis of IgAV (HSP) are presented here The pathogenesis, clinical manifestations, diagnosis, and differential diagnosis of IgAV (HSP) are discussed separately (See "IgA vasculitis (Henoch-Schönlein purpura): Clinical manifestations and diagnosis".) MANAGEMENT The vast majority of patients with IgAV (HSP) recover spontaneously, and most patients may be cared for in the ambulatory setting Therapy is primarily supportive and includes adequate hydration, rest, and symptomatic relief of pain 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 IgAV (HSP) includes the use of acetaminophen or nonsteroidal anti-inflammatory drugs (NSAIDs) Clinical experience and observational data suggest that the anti-inflammatory effects of glucocorticoids can relieve many of the manifestations of IgAV (HSP), 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 IgAV (HSP) 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 — Though there are no randomized, controlled studies of the use of NSAIDs for symptomatic pain relief in patients with IgAV (HSP), it is commonly used in clinical practice We typically prescribe NSAIDs to control significant joint and abdominal symptoms in patients with IgAV (HSP) Of note, there is no evidence to suggest that the risk of gastrointestinal hemorrhage in a child with bowel vasculitis is increased by the use of cyclooxygenase inhibitors 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 IgAV (HSP), 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 Ibuprofen and other NSAIDs may be similarly 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 IgAV (HSP) [3-7] However, glucocorticoids not appear to otherwise impact the clinical course [8,9] Thus, we suggest using prednisone (1 to mg/kg per day, maximum dose of 60 to 80 mg per day) only in patients with symptoms significant enough to substantially limit 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 IgAV (HSP), it is important to remember that, although the inflammation may be ameliorated, the pathophysiology of the disease does not appear to be altered Accordingly, we advise lowering the glucocorticoid dose slowly, typically over four to eight weeks, to minimize the chance of triggering disease rebound from rapid 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 IgAV (HSP) (See "IgA vasculitis (Henoch-Schönlein purpura): Clinical manifestations and diagnosis", section on 'Gastrointestinal symptoms' and 'Prevention of complications' below.) Treatment of renal disease — Treatment of active renal disease in IgAV (HSP) and renal transplantation for end-stage kidney disease are discussed in detail separately (See "IgA vasculitis (Henoch-Schönlein purpura): Renal manifestations", section on 'Treatment'.) Hospitalization — Hospitalization is warranted for the following indications in patients with IgAV (HSP): ● 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 "IgA vasculitis (Henoch-Schönlein purpura): Clinical manifestations and diagnosis", section on 'Laboratory findings' and "IgA vasculitis (Henoch-Schönlein purpura): 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 IgAV (HSP) [11,12] (See "IgA vasculitis (Henoch-Schönlein purpura): 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 IgAV (HSP) Most central nervous system findings are transient and not need further intervention (See "IgA vasculitis (Henoch-Schönlein purpura): 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 "IgA vasculitis (Henoch-Schönlein purpura): Renal manifestations", 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 IgAV (HSP) When these medications fail, administration of oral or parenteral glucocorticoids will generally result in a rapid resolution of the arthritis Rarely, a brief hospitalization may be required to address the pain of arthritis (See 'Treatment of pain' above.) Prevention of complications — The use of glucocorticoids in patients with IgAV (HSP) is controversial Although many clinicians describe a rapid symptomatic improvement in patients who receive glucocorticoids, it is questionable as to whether such treatment alters long-term clinical outcomes In addition to the common side effects of glucocorticoid therapy (eg, hyperglycemia, hypertension, weight gain, acne, immunosuppression), there are potential side effects of glucocorticoid therapy more specific to IgAV (HSP), including the risk of obscuring the signs of compromised bowel viability if treatment is started after an intussusception has occurred, as well as masking signs of fever and pain As a result, we not recommend routine empiric glucocorticoid therapy for patients with IgAV (HSP) 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 randomized trials and 12 retrospective studies included decreased risk of intussusception, renal involvement, and recurrence of disease [3] One subsequent retrospective cohort study using an administrative database reported a decreased risk of gastrointestinal procedures (endoscopy, imaging, and surgery) in children with IgAV (HSP), who were hospitalized and treated early with glucocorticoids [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 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] Treatment of recalcitrant disease — 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 IgAV (HSP) is discussed in detail separately (See "IgA vasculitis (Henoch-Schönlein purpura): Renal manifestations", section on 'Treatment'.) PROGNOSIS The short- and long-term outcomes of children with IgAV (HSP) are generally excellent In the absence of significant renal disease, the initial episode of IgAV (HSP) typically resolves within one month In two-thirds of children, there are no recurrent episodes [2,21] In the remaining one-third of patients, IgAV (HSP) recurs at least once, typically within four months of the initial presentation [2,22,23] Each subsequent episode has similar clinical manifestations but is generally milder and/or shorter in duration than the preceding one In a single-center study involving 417 patients, approximately one-third of the patients had at least one relapse, with a median time to relapse of one month Presence of an infectious trigger was associated with a lower likelihood of relapse, while the risk of relapse was higher when joint and gastrointestinal symptom were present [24] 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 [22,25] One study found that persistent hematuria was a predictive factor for relapse [26] Another study found that development of nephritis was associated with persistent purpura and gastrointestinal bleed [27] These findings suggest that patients who have a more severe course of IgAV (HSP) 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 IgAV (HSP) than was reported previously [28] 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 IgAV (HSP) is primarily a result of gastrointestinal complications, including intussusception and, less commonly, bowel ischemia, bowel perforation, or pancreatitis (See "IgA vasculitis (HenochSchönlein purpura): Clinical manifestations and diagnosis", section on 'Gastrointestinal symptoms'.) The long-term morbidity in patients with IgAV (HSP) is a result of renal disease The risk of chronic renal disease is increased in adults [29] 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 "IgA vasculitis (Henoch-Schönlein purpura): Renal manifestations", section on 'Prognosis'.) FOLLOW-UP Ninety percent of children who develop renal involvement so within two months of onset, and 97 percent within six months [30] Adults are at higher risk for renal disease both early in the course of disease and longer term [31-33] Accordingly, all patients with IgAV (HSP) 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 annual well-child visits In the previously discussed study, an increasing percentage of children showed some degree of renal involvement over a 15 year follow-up period, although none developed severe kidney disease [14] (See "IgA vasculitis (Henoch-Schönlein purpura): 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 IgAV (HSP) should be monitored closely as they have a higher risk of hypertension [34] Close observation and frequent abdominal exams are recommended, in addition to follow-up imaging, for patients who presented with abdominal pain and required treatment with glucocorticoids, especially if the patient has hematochezia, persistent abdominal pain, guarding, or other significant change in the physical exam SOCIETY GUIDELINE LINKS Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately (See "Society guideline links: Vasculitis".) 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 5th 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-toread materials Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed These articles are written at the 10th 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: IgA vasculitis (Henoch-Schönlein purpura) (The Basics)") ● Beyond the Basics topic (see "Patient education: Vasculitis (Beyond the Basics)") SUMMARY AND RECOMMENDATIONS ● Most patients with immunoglobulin A vasculitis (IgA vasculitis [IgAV]), formerly called Henoch-Schönlein purpura (HSP), 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 anti-inflammatory 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 anti- inflammatory 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 anti-inflammatory agent, we suggest the use of systemic glucocorticoids (Grade 2C) We use oral prednisone (1 to 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 IgAV (HSP) 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 not recommend empiric glucocorticoid administration to prevent renal or gastrointestinal complications (Grade 1B) (See 'Prevention of complications' above.) ● Although prognosis is excellent in children with IgAV (HSP), a small minority of patients (