To the Editor,IgG4‐related disease(IgG4‐RD)is a multisystem auto‐immune disorder with propensity to involve the kidneys.The clinical presentation,1 diagnostic classification criteria 2 and grading of disease activi...To the Editor,IgG4‐related disease(IgG4‐RD)is a multisystem auto‐immune disorder with propensity to involve the kidneys.The clinical presentation,1 diagnostic classification criteria 2 and grading of disease activity(Responder Index)3 of IgG4‐RD have been well established,however there is scarce data on histological evolution of this disease entity ensuing immunosuppressive treatment.There is a concern that the dense fibrotic lesions of IgG4‐RD could remain obstinate,notwithstanding immunosuppressive therapy,4 thereby leaving the affected organ with permanent scarring.We present the case of a 57‐year‐old man with multi‐system IgG4‐RD in whom the dense lympho‐plasmocytic intersti-tial inflammation and the typical whorl‐like storiform fibrosis observed in initial kidney biopsy showed a near complete resolution following 4 months of immuno-suppressive therapy.展开更多
文摘To the Editor,IgG4‐related disease(IgG4‐RD)is a multisystem auto‐immune disorder with propensity to involve the kidneys.The clinical presentation,1 diagnostic classification criteria 2 and grading of disease activity(Responder Index)3 of IgG4‐RD have been well established,however there is scarce data on histological evolution of this disease entity ensuing immunosuppressive treatment.There is a concern that the dense fibrotic lesions of IgG4‐RD could remain obstinate,notwithstanding immunosuppressive therapy,4 thereby leaving the affected organ with permanent scarring.We present the case of a 57‐year‐old man with multi‐system IgG4‐RD in whom the dense lympho‐plasmocytic intersti-tial inflammation and the typical whorl‐like storiform fibrosis observed in initial kidney biopsy showed a near complete resolution following 4 months of immuno-suppressive therapy.
