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Immunoglobulin A vasculitis in Adult
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Immunoglobulin A vasculitis in Adult

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Contributors: Yun Xue MD, Susan Burgin MD
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Synopsis

Immunoglobulin A vasculitis (IgAV), formerly called Henoch-Schönlein purpura, is a necrotizing small-vessel vasculitis of unknown etiology that predominantly occurs in children but can also occur in adults. It is characterized by IgA-immune complex and C3 and fibrin deposition in small vessels: primarily capillaries, postcapillary venules, and occasionally, arterioles in affected organs. The annual incidence in childhood is 3-26 per 100 000 whereas in adults it is much lower at 0.1-1.8 per 100 000. It is seen more frequently in males, white individuals, and those of Asian descent.

IgAV is seasonal, with most childhood cases occurring during fall and winter. In adults, cases can also occur in summer months. Episodes can often be preceded by an upper respiratory infection.

IgAV is characterized clinically by the tetrad of palpable purpura, abdominal pain, arthritis, and hematuria. Adults are more likely to have joint symptoms and renal dysfunction. Children are more likely to have fevers and abdominal pain. There are prodromal symptoms of malaise, headache, and arthralgias. An individual episode may persist for 3-6 weeks, with recurrences in many patients.

Severe abdominal pain, vomiting, hematemesis, diarrhea, and hematochezia may indicate gastrointestinal involvement in 50%-75% of patients. An involved inflamed and edematous bowel may be accompanied by appendicitis, ileus, and infarction. Intussusception and bowel perforation in adults is rare.

Joint abnormalities may accompany the eruption with severe pain and sometimes swelling, warmth, and tenderness. Ankles and knees are most often involved; symptoms are often transient and migratory.

Renal involvement is common but is usually self-limited, with only a fraction of patients progressing to chronic renal failure or end-stage renal disease; these patients often have baseline renal dysfunction. The risk of renal failure is higher in adult patients and in those presenting with nephrotic or nephritic syndrome.

Codes

ICD10CM:
D69.0 – Allergic purpura

SNOMEDCT:
191306005 – Henoch-Schönlein purpura

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Other small vessel vasculitides – Causes include infections, drugs, connective tissue diseases, and malignancies.
  • Erythema elevatum diutinum – Initially presents with palpable purpura over extensor surfaces that later become fibrotic.
  • Capillaritis – Consists of nonblanchable macules with no associated vessel inflammation.
  • Erythema multiforme – Presents with erythematous, targetoid plaques most commonly acrally distributed.
  • Connective tissue diseases – Have associated photosensitivity, additional antibody abnormalities, and are unlikely to be self-limited. 
  • Meningococcemia – May result in purpura fulminans characterized by necrotic, nonblanchable palpable purpura on predominantly acral areas; patients are more ill.
  • Disseminated intravascular coagulation (DIC) – Patients are more systemically ill and present with petechiae (nonblanchable, nonpalpable macules).
  • Endocarditis – Can present with characteristic Osler nodes and Janeway lesions.
  • Thrombocytopenic purpura – Presents like DIC with petechiae.
  • Cryoglobulinemia – Has palpable purpura commonly in the setting of hepatitis C.
  • Rickettsial infections – Have characteristic purpura on the hands and feet, and patients are systemically ill.

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Reviewed: 09/28/2017
Last Updated: 10/09/2017
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Immunoglobulin A vasculitis in Adult
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Immunoglobulin A vasculitis : Abdominal pain, Diarrhea, Vomiting, Buttocks, Extensor distribution, Hand edema, Hematuria, Palpable purpura, Symmetric extremities, Arthralgia, Foot edema, ESR elevated, Legs
Clinical image of Immunoglobulin A vasculitis
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