Abstract: Henoch-Schonlein purpura nephritis (HSPN) is the most common secondary glomerular disease in children, often caused by respiratory infections complicated by tonsillitis. Abnormal mucosal immunity leads to the production of large amounts of galactose-deficient IgA1, forming immune complexes that deposit in the kidneys, causing the disease to prolong and progress chronically. The lung, pharynx, and kidney are interconnected and functionally linked, forming the “lung-pharyngeal-kidney” axis, which plays a crucial role in the pathogenesis and progression of HSPN. This article reveals the theoretical connotation of the “lung-pharynx-kidney” axis in HSPN, explores the pathogenesis of HSPN from the perspective of the “lung-pharynx-kidney” axis, and discusses treatment in three stages. In the lung-pharynx stage, six exogenous pathogenic factors cause heat to accumulate in the throat; treatment focuses on dispelling wind and releasing the exterior, clearing heat and benefiting the throat. In the pharynx-kidney stage, heat enters the nutrient blood and travels along the meridians to the kidneys; treatment focuses on clearing the nutrient level and cooling the blood, resolving stasis and unblocking the meridians. In the kidney deficiency stage, there is kidney yin deficiency, upward scorching of deficient fire, or insufficient kidney yang and inadequate lung guard; treatment focuses on nourishing kidney yin, guiding fire back to its source, or warming and tonifying kidney yang and enhance lung guard.