What are the biliary and renal symptoms of Henoch-Schönlein purpura (IgA vasculitis)?

Updated: Oct 28, 2020
  • Author: Rajendra Bhimma, MBChB, MD, PhD, DCH (SA), FCP(Paeds)(SA), MMed(Natal); Chief Editor: Craig B Langman, MD  more...
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Answer

The liver and gallbladder can be involved in IgAV. [76] Hepatomegaly, hydrops of the gallbladder, and cholecystitis may ensue. These may contribute to a patient’s abdominal pain. Acute appendicitis has been noted in patients with IgAV.

Kidney pathology is the most important cause of morbidity and mortality in patients with IgAV. Kidney involvement may precede skin manifestations (1-4% of patients) but is usually evident during the acute phase of the disease, sometimes developing as long as 3 months after the initial presentation. [77] It may persist for as long as 6 months after the onset of the rash. In most cases, the severity of nephritis is not related to the extent of other IgAV manifestations.

Hemoptysis and hemarthroses can develop in patients who have bleeding abnormalities such as coagulopathy, factor VIII deficiency, vitamin K deficiency, or hypoprothrombinemia. Inherited thrombophilias (eg, factor V Leiden, protein C deficiency, and protein S deficiency) may contribute to necrotic cutaneous lesions in IgAV. [78]


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