Mansour Salesi
1 
, Narges Ansari
2* 1 Department of Rheumatology, Isfahan University of Medical Sciences, Isfahan, Iran
2 Department of Internal Medicine, Isfahan University of Medical Sciences, Isfahan, Iran
Abstract
Antiphospholipid antibody nephropathy (APLN) is a rare autoimmune disease that affects the kidneys. It is characterized by the deposition of antiphospholipid antibodies (aPL) in the walls of small blood vessels in the kidneys. These antibodies cause inflammation and damage to the blood vessels, leading to various symptoms, including proteinuria, hematuria, hypertension, and renal failure. Several pathological lesions have been identified in APLN, including thrombotic microangiopathy, glomerular endothelial swelling, and fibrinoid necrosis of glomerular capillaries. Thrombotic microangiopathy is the most common lesion and is characterized by the formation of small blood clots in the vessels of the kidney, which can lead to kidney damage and dysfunction. In addition to these lesions, APLN can also cause damage to the tubules and interstitium of the kidney, leading to tubular atrophy, interstitial fibrosis, and chronic kidney disease. These lesions are thought to be caused by the chronic inflammation and immune response associated with APLN
Implication for health policy/practice/research/medical education:
Antiphospholipid antibody nephropathy (APLN) is a potentially serious complication of antiphospholipid syndrome (APS), an autoimmune disorder characterized by the presence of antiphospholipid antibodies (aPL) and a predisposition to thrombosis and pregnancy complications. APLN can manifest as a wide range of renal pathologies, including glomerulonephritis, thrombotic microangiopathy, interstitial nephritis, and vasculitis. APLN may be asymptomatic or present with proteinuria, hematuria, hypertension, and/or renal insufficiency. Diagnosis of APLN requires the presence of aPL and renal pathology on biopsy, although the sensitivity and specificity of aPL testing and interpretation of renal biopsy findings can be challenging. Treatment of APLN typically involves anticoagulation with heparin or warfarin, along with immunosuppressive therapy (such as steroids, cyclophosphamide, or rituximab) in severe or refractory disease cases. The prognosis of APLN varies depending on the severity and extent of renal involvement, with some patients experiencing complete remission while others progress to end-stage renal disease.
Please cite this paper as: Salesi M, Ansari N. Antiphospholipid antibody nephropathy; an updated mini-review. J Nephropathol. 2024;x(x):e21529. DOI: 10.34172/jnp.2024.21529.