Ali Rastegar-Kashkouli
1 
, Mohsen Jafari
2 , Seyedeh Ghazal Shahrokh
3* , Amir Mohammad Taravati
3 , Pourya Yousefi
1 , Mohammadreza Jafari
3 1 Kowsar Hospital, Fars Heart Foundation, Research Committee, Shiraz, Iran
2 Abu-Ali Sina Organ Transplant Hospital, Research Committee, Shiraz, Iran
3 Nickan Research Institute, Isfahan, Iran
Abstract
The current narrative review study aims to provide an overview of thrombotic microangiopathy (TMA) in immunoglobulin A nephropathy (IgAN), with a particular emphasis on its pathophysiology, histopathology, and treatment options. The prevalence and clinical significance of TMA in IgAN may vary across different populations. Estimates suggest that TMA events occur in 2-50% of patients with IgAN. Endothelial injury is a key factor in TMA development in IgAN, triggered by immune complex deposition, complement activation, and potentially hypertension. TMA in IgAN correlates with vascular lesions, including arterial intimal sclerosis, arteriolar lumen reduction, and smooth muscle hypertrophy. Notably, patients with TMA show more intense deposition of C4, C3d, and C5b-9 complements. Treatment involves blood pressure management, immunosuppression, and targeted therapies such as eculizumab.
Implication for health policy/practice/research/medical education:
Healthcare providers and educators should emphasize early detection and intervention targeting endothelial injury, complement activation, and genetic factors to optimize treatment outcomes and reduce the burden of TMA-related complications in IgAN.
Please cite this paper as: Rastegar-Kashkouli A, Jafari M, Shahrokh SG, Taravati AM, Yousefi P, Jafari M. Thrombotic microangiopathy in IgA nephropathy: pathophysiology, histopathology, and treatment perspectives. J Nephropathol. 2024;13(3):e24547. DOI: 10.34172/ jnp.2024.24547.