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, gastrointestinal (GI) symptoms and signs, and glomerulonephritis. Diagnosis is clinical in children but usually warrants biopsy in adults. Disease is usually self-limited in children but may be chronic in adults. Corticosteroids can relieve arthralgias and GI symptoms but do not alter the course of the disease. Progressive glomerulonephritis may require high-dose corticosteroids and another immunosuppressant.
(See also Overview of Vasculitis.)
IgA-containing immune complexes are deposited in small vessels of the skin and other sites. Possible inciting antigens include viruses that cause upper respiratory infections, streptococcal infection, medications, foods, insect bites, and immunizations. Focal, segmental proliferative glomerulonephritis is typical but usually mild.
Symptoms and Signs of IgAV
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.
DR P. MARAZZI/SCIENCE PHOTO LIBRARY
Image courtesy of Karen McKoy, MD.
Gastrointestinal 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 of immunoglobulin A–associated vasculitis usually remit after approximately 4 weeks but often recur at least once after a disease-free interval of several weeks. In most patients, the disorder subsides without serious sequelae; however, although rare, some patients develop chronic kidney disease.
In adults, intussusception is rare and chronic kidney disease is more common than in children.
Diagnosis of IgAV
Biopsy of skin lesions with direct immunofluorescence
The 2012 Chapel Hill Consensus Conference (1) defined IgAV as vasculitis with IgA1-dominant immune deposits, affecting small vessels in the skin and gastrointestinal tract and frequently causing arthritis. IgAV is also associated with glomerulonephritis indistinguishable from IgA nephropathy.
The diagnosis of IgAV 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 by immunofluorescence. In children, biopsy is unnecessary if clinical diagnosis is clear.
Urinalysis is done; hematuria, proteinuria, and red blood cell casts indicate renal involvement.
Complete blood count 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.
Diagnosis reference
1. Jennette JC, Falk RJ, Bacon PA, et al: 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 65(1):1-11, 2013. doi: 10.1002/art.37715
Treatment of IgAV
For adults, primarily symptomatic measures and corticosteroids with or without another immunosuppressant
For children, symptomatic treatment for pain control as needed
If the cause is a medication, it has to be discontinued. Otherwise, treatment is primarily symptomatic.
For children, corticosteroids or other immunosuppressants are usually not necessary.
Key Points
Immunoglobulin A–associated vasculitis is vasculitis that affects primarily small vessels and occurs more frequently in children than adults.
Manifestations can include purpuric rash, arthralgias, fever, abdominal pain, melena, and glomerulonephritis.
Symptoms usually remit after approximately 4 weeks.
When necessary to confirm the diagnosis, biopsy skin lesions, looking for IgA deposition.
Treat symptoms and, for adults, consider corticosteroids with or without another immunosuppressant.
