Lupus anticoagulant in systemic lupus erythematosus: a clinical and renal pathological study.
Circulating lupus anticoagulant (LA) is associated with thrombosis in large and small vessels. To determine how often the presence of LA is associated with thrombosis within the renal microcirculation, 33 patients with systemic lupus erythematosus (SLE), renal dysfunction, and LA were identified over a 25-year period (LA group) and 32 patients with renal SLE but with normal gross coagulation screen were matched for age, sex, and biopsy timing (C group). Prevalences of serositis, neuropsychiatric illness, leukopenia, thrombocytopenia, hemolysis, anti-DS-DNA elevation, and complement reduction were similar. Arthritis was less and biologic false-positive (BFP) syphilis serology more common in LA. More LA patients had thrombotic events (LA 39% v C 13%; P = 0.014); bleeding episodes, including postbiopsy, were similar. At biopsy, hypertension (LA 55%, C 41%), serum creatinine (mean +/- SD: LA 186 +/- 168 mumol/L [2.1 +/- 1.9 mg/dL] v C 150 +/- 168 mumol/L [1.7 +/- 1.9 mg/dL]) and proteinuria (LA 2.6 +/- 3.1 g/24 h v C 3.1 +/- 2.7) were similar. Lesions by World Health Organization (WHO) class, activity, and chronicity indices, as well as immunofluorescence (IF) and electron microscopy (EM) findings, were not significantly different. Occlusive glomerular, arteriolar, and arterial fibrin thrombi, along with varying degrees of renal thrombotic microangiopathy, were seen in five of 33 patients with LA, but zero of 32 C patients (P = 0.053); three of these five patients died soon after biopsy. Overall, mortality was not different between LA and C. We conclude that the majority of patients with SLE, renal dysfunction, and LA exhibit renal morphologic findings indistinguishable from patients without LA. However, a significant minority of LA patients have thrombotic microangiopathy in their biopsy, which is accompanied by a worse prognosis.
Farrugia
E
E
Division of Nephrology and Internal Medicine, Mayo Clinic, Rochester, MN 55905.
Torres
V E
VE
Gastineau
D
D
Michet
C J
CJ
Holley
K E
KE
D000293:Adolescent; D000328:Adult; D001777:Blood Coagulation / Q000502:physiology; D002196:Capillaries / Q000473:pathology; D005260:Female; D005337:Fibrin; D006470:Hemorrhage / Q000503:physiopathology; D006801:Humans; D007668:Kidney / Q000098:blood supply; D007678:Kidney Glomerulus / Q000098:blood supply / Q000473:pathology; D016682:Lupus Coagulation Inhibitor / Q000097:blood*; D008180:Lupus Erythematosus, Systemic / Q000097:blood* / Q000473:pathology; D008181:Lupus Nephritis / Q000097:blood / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D013927:Thrombosis / Q000473:pathology*
D016682:Lupus Coagulation Inhibitor; D005337:Fibrin