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Am Fam Physician. 2020;102(4):229-233

Patient information: A handout on this topic is available at https://familydoctor.org/condition/henoch-schonlein-purpura/.

This clinical content conforms to AAFP criteria for CME.

Author disclosure: No relevant financial affiliations.

Henoch-Schönlein purpura, now called immunoglobulin A (IgA) vasculitis, is a systemic, immune complex–mediated, small-vessel leukocytoclastic vasculitis characterized by nonthrombocytopenic palpable purpura, arthritis, and abdominal pain. It is the most common vasculitis in children but can also occur in adults. Diagnostic testing is required only to exclude other etiologies of purpura, to identify renal involvement, and, if indicated, to determine its extent with biopsy. Imaging or endoscopy may be needed to assess organ complications. IgA vasculitis spontaneously resolves in 94% of children and 89% of adults, making supportive treatment the primary management strategy. However, a subset of patients experience renal involvement that can persist and relapse years later. Additional complications can include gastrointestinal bleeding, orchitis, and central nervous system involvement. Systematic reviews have shown that steroids do not prevent complications and should not be used prophylactically. However, randomized trials have demonstrated success with high-dose steroids, cyclosporine, and mycophenolate in treating glomerulonephritis and other complications. Long-term prognosis depends on the extent of renal involvement. Six months of follow-up is prudent to assess for disease relapse or remission.

Immunoglobulin A (IgA) vasculitis, formerly known as Henoch-Schönlein purpura, is defined as a systemic, immune complex–mediated, small-vessel leukocytoclastic vasculitis characterized by nonthrombocytopenic palpable purpura, abdominal pain, and arthritis. It is the most common vasculitis in children. IgA vasculitis is typically self-limited, but a subset of patients experience a remitting-relapsing course. Glomerulonephritis and gastrointestinal bleeding are the most commonly associated complications.

Epidemiology

  • IgA vasculitis occurs in 3.0 to 26.7 out of 100,000 children and 0.8 to 1.8 out of 100,000 adults each year.1

  • The mean age of onset in children is six years; onset ranged from 32 to 50 years of age in several adult case series.14 More than 90% of cases occur in children younger than 10 years.3

  • There is a slight disease predominance in males.1,2

  • In children, onset is more common during the fall and winter, but no seasonal pattern has been consistently shown in adults.1,5

  • IgA vasculitis is milder in children younger than two years, but more severe in adults, with worse outcomes.3,4,6

Pathophysiology

  • IgA vasculitis is a small-vessel vasculitis caused by IgA immune deposits in the gastrointestinal system, joints, skin, and kidneys.

  • Multiple viral and bacterial infections are thought to trigger the disease, including Streptococcus, parainfluenza, and human parvovirus B19.7 Cytokines and chemokines are involved; however, the full pathophysiology is not understood.

  • Numerous studies have linked disease predisposition, severity, and long-term morbidity with genes on portions of the HLA alleles.8

Diagnosis

  • IgA vasculitis should be suspected in patients presenting with palpable purpura who also develop arthralgias (75% of patients) and abdominal pain (50% to 65% of patients).2,9,10

  • The differential diagnosis includes immune thrombocytopenic purpura, child abuse, bleeding disorders, medication reactions, senile purpura, meningococcal sepsis, familial Mediterranean fever, Rocky Mountain spotted fever, acute leukemia, bone marrow failure syndromes, and other vasculitides.11,12

  • The European League Against Rheumatism/Paediatric Rheumatology European Society diagnostic criteria (Table 113 ) have a 100% sensitivity and 87% specificity in children, and a 99% sensitivity and 86% specificity in adults. These criteria are more accurate than the 1990 American College of Rheumatology criteria.13,14

Mandatory criterion: purpura or petechiae with lower limb predominance
Minimum of one out of the four following criteria:
 Arthritis or arthralgia of acute onset
 Diffuse abdominal pain with acute onset
 Histopathology showing leukocytoclastic vasculitis or proliferative glomerulonephritis with IgA deposits
 Renal involvement (proteinuria or hematuria)

SIGNS AND SYMPTOMS

  • Signs and symptoms may develop over days to weeks in any sequence.11 The onset of IgA vasculitis typically follows an upper respiratory infection.15

  • Joint involvement and abdominal pain are more common in children, whereas adults are more likely to have lower-extremity edema and hypertension.9

  • The characteristic rash may start as erythematous papules that develop into crops of petechiae and palpable purpura.2,9,16 The purpura can enlarge into palpable ecchymoses that evolve from purple to rust-colored as they fade over approximately 10 days.1618 The purpura are predominantly on extensor surfaces and dependent areas that are subject to pressure, such as the legs and buttocks19 (Figure 1).

  • Arthralgias are more common in the knees and ankles than in small joints.11,19 Arthritis is transient and does not damage the joints.17,19

  • Abdominal pain is typically colicky and can be severe enough to mimic an acute abdomen.11 Emesis and gastrointestinal bleeding can occur in approximately one-third of patients.17,19 Intussusception can occur in rare cases.20

  • Renal disease occurs in 50% of patients and may cause long-term damage.21,22 The risk of renal disease is highest in adults and children older than 10 years.21,23 Males and patients with persisting purpura, abdominal pain, gastrointestinal bleeding, or relapsing episodes are also at increased risk.24 Renal disease typically develops within one to three months after the rash, but it may be delayed up to six months.21,22,24,25 Signs of renal disease include microscopic hematuria, red cell casts, proteinuria, and overt renal failure. Progressive glomerulonephritis may develop, and patients with persistent proteinuria are at highest risk of this complication.19,22

  • Low-grade fever and fatigue are common. Less common symptoms include orchitis, pulmonary hemorrhage, and central nervous system involvement with headaches, behavior changes, seizures, or hemorrhage.19,26,27

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