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Saudi Journal of Kidney Diseases and Transplantation
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CASE REPORT Table of Contents   
Year : 2022  |  Volume : 33  |  Issue : 1  |  Page : 196-200
Pauci-immune Lupus Nephritis in Antiphospholipid Disease: A Diagnostic Challenge

1 Department of Nephrology, Department of Histopathology and Riyadh, Saudi Arabia
2 Department of Histopathology and Department of Rheumatology, Riyadh, Saudi Arabia
3 Department of Rheumatology, Prince Sultan Military Medical City, Riyadh, Saudi Arabia

Correspondence Address:
Salwa A Alhemyadi
Department of Nephrology, Prince Sultan Military Medical City, P. O. Box 7897, Postal code 11159, Riyadh
Saudi Arabia
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1319-2442.367816

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Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that leads to immune complex deposition in different organs, especially the kidneys. Pauci-immune lupus nephritis (LN) is a very rare condition. About 40% of SLE cases have a positive antiphospholipid antibody (aPL). So, diagnosing primary antiphospholipid syndrome (APS) in SLE patients is challenging because most symptoms of primary APS can be similar to SLE as described in the American College of Rheumatology/Systemic Lupus Erythematosus International Collaborating Clinics classification criteria for SLE. APS might present as thrombotic microangiopathy (TMA) involving arterioles and glomerular capillaries. We need an adequate renal biopsy to differentiate between lupus and APS nephropathy. A 38-year-old man was diagnosed with biopsy-proven primary APS and LN. Hewas commenced on anticoagulants, pulse steroids, rituximab, and with mycophenolatemofetil as a maintenance therapy in collaboration with the rheumatologist. Here we discuss the occurrence and implications of primary APS and Pauci-immune LN in adults.

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