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Immunoglobulin A–Associated Vasculitis (IgAV)

(Henoch-Schönlein Purpura)

by Carmen E. Gota, MD

Immunoglobulin A–associated vasculitis (IgAV—formerly called Henoch-Schönlein purpura) is vasculitis that affects primarily small vessels. It occurs most often in children. Common manifestations include palpable purpura, arthralgias, GI symptoms and signs, and glomerulonephritis. Diagnosis is clinical in children but usually warrants biopsy in adults. Disease is usually self-limited. Corticosteroids can relieve arthralgias and GI symptoms but do not alter the course of the disease. Progressive glomerulonephritis may require high-dose corticosteroids and cyclophosphamide.

IgA-containing immune complexes are deposited in small vessels of the skin and other sites, with consequent activation of complement. Possible inciting antigens include viruses that cause URIs, streptococcal infection, drugs, foods, insect bites, and immunizations. Focal, segmental proliferative glomerulonephritis is typical but mild.

Symptoms and Signs

The disease begins with a sudden palpable purpuric rash typically occurring on the feet, legs, and, occasionally, the trunk and arms. The purpura may start as small areas of urticaria that become palpable and sometimes hemorrhagic and confluent. Crops of new lesions may appear over days to several weeks. Many patients also have fever and polyarthralgia with periarticular tenderness and swelling of the ankles, knees, hips, wrists, and elbows.

GI symptoms are common and include colicky abdominal pain, abdominal tenderness, and melena. Intussusception occasionally develops in children. Stool may test positive for occult blood.

Symptoms usually remit after about 4 wk but often recur at least once after a disease-free interval of several weeks. In most patients, the disorder subsides without serious sequelae; however, some patients develop chronic renal failure.

Diagnosis

  • Biopsy of skin lesions

The 2012 Chapel Hill Consensus Conference defined IgAV as vasculitis with IgA1-dominant immune deposits, affecting small vessels in the skin and GI tract and frequently causing arthritis. IgAV is also associated with glomerulonephritis indistinguishable from IgA nephropathy.

The diagnosis is suspected in patients, particularly children, with typical skin findings. It is confirmed by biopsy of skin lesions when leukocytoclastic vasculitis with IgA in the vessel walls is identified. Biopsy is unnecessary if clinical diagnosis is clear in children. Urinalysis is done; hematuria, proteinuria, and RBC casts indicate renal involvement.

CBC and renal function tests are done. If renal function is deteriorating, renal biopsy may help define the prognosis. Diffuse glomerular involvement or crescent formation in most glomeruli predicts progressive renal failure.

Treatment

  • Primarily corticosteroids and symptomatic measures

If the cause is a drug, it has to be stopped. Otherwise, treatment is primarily symptomatic. Corticosteroids (eg, prednisone 2 mg/kg up to a total of 50 mg po once/day) may help control abdominal pain and are occasionally needed to treat severe joint pain or renal disease. Pulse IV methylprednisolone followed by oral prednisone and cyclophosphamide can be given to attempt to control inflammation when the kidneys are severely affected. However, the effects of corticosteroids on renal manifestations are not clear.

Key Points

  • IgAV is vasculitis that affects primarily small vessels and occurs in children.

  • Manifestations can include purpuric rash, arthralgias, fever, abdominal pain, and melena.

  • Symptoms usually remit after about 4 wk.

  • Diagnose by biopsy.

  • Treat symptoms and consider corticosteroids.

Drugs Mentioned In This Article

  • Drug Name
    Select Trade
  • CYTOXAN (LYOPHILIZED)
  • MEDROL
  • RAYOS

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