Lupus nephritis with vasculopathy
- Systemic lupus erythematous (SLE) is a systemic autoimmune disease, most commonly involving the skin, kidneys, joints, heart, and serosal surfaces.
- Women are affected more than men.
- Lupus nephritis (LN) is defined as an immune complex mediated renal disease that occurs in patients with SLE.
- Onset usually is from teenage to the third decade of life, but LN may manifest at any age.
- LN is a major cause of morbidity and the most common cause of death in SLE patients.
- A consensus report pertaining to the improved clarity of definitions and classification of glomerular lesions in lupus nephritis was provided by the international nephropathology working group in Leiden, Netherlands, in 2016.
- New definitions were provided for mesangial hypercellularity and for cellular, fibrocellular, and fibrous crescents. The term “endocapillary proliferation” was replaced by endocapillary hypercellularity.
- Also, the class IV-S and IV-G subdivisions of class IV lupus nephritis were eliminated.
- The active and chronic designations for class III/IV lesions were replaced by a proposal for activity and chronicity indices that were recommended to be applied to all classes.
- Lupus vasculopathy was defined as luminal narrowing of arterioles or terminal interlobular arteries by intramural immune deposits, typically admixed with fibrinoid changes, without inflammation of the vessel wall.
- Management: Varies according to disease severity and risk for progressive kidney damage. Clinical response criteria according to various guidelines have been proposed.
References
- Fogo AB, Lusco MA, Najafian B, Alpers CE. AJKD Atlas of Renal Pathology: Focal and Diffuse Lupus Nephritis (ISN/RPS Class III and IV). Am J Kidney Dis. 2017;70:e9-e11.
- Bajema IM, Wilhelmus S, Alpers CE, et al. Revision of the International Society of Nephrology/Renal Pathology Society classification for lupus nephritis: clarification of definitions, and modified National Institutes of Health activity and chronicity indices. Kidney Int. 2018;93:789-796.
- Parikh SV, Almaani S, Brodsky S, Rovin BH. Update on Lupus Nephritis: Core Curriculum 2020. Am J Kidney Dis. 2020;76:265-281.
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