Is Henoch-Schönlein purpura nephritis hereditary?
Jun 07, 2022
Source: Cainiu Health
Dr. Wang Xiaoyan
Introduction
IgA vasculitis–associated nephritis (also known as Henoch–Schönlein purpura nephritis) is a particularly challenging form of IgA vasculitis to treat; however, it is not hereditary and does not exhibit a genetic predisposition. Initially, patients with IgA vasculitis–associated nephritis present with hemorrhagic, symmetric, erythematous maculopapular rashes—commonly located on the lower extremities, especially around the ankles and knees—and may also appear on the buttocks and upper limbs.
IgA vasculitis nephritis (also known as Henoch-Schönlein purpura nephritis) is primarily an inflammatory renal response triggered by IgA vasculitis (Henoch-Schönlein purpura), and is associated with the body’s own immune system. So, is IgA vasculitis nephritis hereditary? The specific details are as follows:
IgA vasculitis nephritis is one of the more refractory forms of IgA vasculitis; however, it is not hereditary and does not exhibit a genetic predisposition.
Firstly, the skin rash in patients with IgA vasculitis nephritis manifests as hemorrhagic, symmetrical, erythematous maculopapular lesions, commonly distributed on the lower extremities—especially around the ankles and knees—and may also appear on the buttocks and upper limbs. Gastrointestinal involvement typically presents with abdominal pain, nausea, vomiting, and hematochezia; hematemesis may occasionally occur.
In children, IgA vasculitis nephritis may be complicated by intussusception, intestinal obstruction, or intestinal perforation. Hypertension secondary to IgA vasculitis nephritis accounts for approximately 20%–40% of all hypertension cases in affected patients; blood pressure elevation is usually mild to moderate. Hypertension is often detected concurrently with urinary abnormalities; however, the majority of patients with IgA vasculitis nephritis recover relatively quickly.
Regarding medications: avoid drugs that reduce platelet count, such as cephalosporins, quinine, sodium aminosalicylate, rifampicin, aspirin, digoxin, quinidine, sulfonamides, and promethazine; also avoid drugs that inhibit platelet function, such as dipyridamole and dextran.
