A Wolf in Sheep's Clothing - A Case Report and Literature Review of PAH Emergence at Complete Remission of SLE-PIG

We present an interesting case where severe pulmonary arterial hypertension (PAH) developed in a 30-year-old male systemic lupus erythematosus (SLE) patient after achieving complete remission of class V lupus nephritis (LN) complicated with overlooked podocytic infolding glomerulopathy (PIG). Despite rapid renal response to conventional therapy, emergent PAH failed to improve with SLE immunosuppression, suggesting the predominance of pulmonary vascular remodeling over immunological activity in PAH persistence. Notably, glucocorticoid resulted in avascular necrosis without PAH remission. However, belimumab combined with pulmonary vasculature-targeting agents effectively lowered pulmonary pressure. This case highlights that PAH requires ongoing surveillance even after apparent LN/SLE resolution, and directly rectifying vascular changes, rather than immunosuppression alone, is key to optimal PAH control. It also provides clues to potent treatment strategies for this refractory SLE complication. The association between PIG and PAH warrants further research.