Renal involvement in antiphospholipid syndrome

Area of Science:

• Nephrology
• Rheumatology
• Pathology

Background:

• Antiphospholipid syndrome (APS) is an autoimmune disorder associated with aPL.
• Renal involvement in APS, termed APS nephropathy, presents unique clinical and histopathological features.
• Distinguishing APS nephropathy from lupus nephritis is critical for effective treatment.

Purpose of the Study:

• To review scientific literature on renal involvement in APS.
• To focus on clinical and histopathological findings and treatment strategies for APS nephropathy.
• To highlight the importance of differentiating APS nephropathy from lupus nephritis.

Main Methods:

• Systematic review of English-language articles from 1980-2017.
• Databases searched: Medline/PubMed and Scopus.
• MeSH terms used: "antiphospholipid syndrome", "antiphospholipid antibodies", "glomerulonephritis", "thrombotic microangiopathy" (TMA).

Main Results:

• APS nephropathy results from thromboses in renal vasculature, characterized histologically by TMA.
• Common antibodies include anticardiolipin and lupus anticoagulant.
• Renal manifestations include thrombosis, TMA, allograft thrombosis, and malignant hypertension.
• Anticoagulation is the primary treatment for thrombotic events in APS nephropathy.
• SLE nephritis requires immunosuppression, while APS nephropathy is treated with anticoagulants; eculizumab and sirolimus are potential rescue therapies.

Conclusions:

• APS nephropathy is a distinct clinical entity characterized by TMA and aPL.
• Histopathological findings of TMA are strongly associated with aPL in APS nephropathy.
• Accurate diagnosis is essential for appropriate therapeutic management, differentiating it from lupus nephritis.