Society of Diabetic Nephropathy Prevention Journal of Nephropathology 2251-8363 13 3 2024 05 25 Thrombotic microangiopathy in IgA nephropathy: pathophysiology, histopathology, and treatment perspectives e24547 e24547 10.34172/jnp.2024.24547 EN Ali Rastegar-Kashkouli https://orcid.org/0000-0001-6714-3549 Mohsen Jafari https://orcid.org/0000-0001-8779-5149 Seyedeh Ghazal Shahrokh https://orcid.org/0009-0005-6496-1040 Amir Mohammad Taravati https://orcid.org/0009-0008-2039-4859 Pourya Yousefi https://orcid.org/0000-0002-8286-9548 Mohammadreza Jafari https://orcid.org/0009-0004-3949-2927 Journal Article 10.34172/jnp.2024.24547 2024 02 04 2024 05 04 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.