Is Henoch-Schönlein purpura nephritis hereditary?
May 29, 2022
Source: Cainiu Health
Dr. Wang Xiaoyan
Introduction
IgA vasculitis–associated nephritis (previously 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 macular rashes—commonly localized to the lower extremities, especially around the ankles and knees—and may also involve the buttocks and upper limbs. Gastrointestinal manifestations include abdominal pain, nausea, vomiting, and hematochezia; hematemesis may occasionally occur.
Henoch-Schönlein purpura nephritis (HSPN) is primarily an inflammatory renal condition triggered by Henoch-Schönlein purpura (HSP), and is associated with the body’s own immune response. Does HSPN run in families? The details are as follows:
HSPN is one of the more refractory forms of HSP, yet it is not hereditary and shows no genetic predisposition.
Firstly, skin rashes in patients with HSPN are hemorrhagic and symmetrically distributed, appearing as red maculopapular lesions—most commonly on the lower extremities, especially around the ankles and knees—and may also occur on the buttocks and upper limbs. Gastrointestinal manifestations include abdominal pain, nausea, vomiting, and hematochezia; hematemesis may occasionally occur.
In children, HSPN may be complicated by intussusception, intestinal obstruction, or intestinal perforation. Hypertension secondary to HSPN accounts for approximately 20%–40% of all hypertension cases in affected individuals; blood pressure elevation is typically mild to moderate. Hypertension is often detected concurrently with urinary abnormalities; however, the majority of HSPN patients recover relatively quickly.
Regarding medications, avoid drugs that reduce platelet counts—such as cephalosporins, quinine, sodium aminosalicylate, rifampicin, aspirin, digoxin, quinidine, sulfonamides, and promethazine—as well as agents that inhibit platelet function—including dipyridamole and dextran.
