Renal Microangiopathy in the Primary Antiphospholipid Syndrome: A Case Report with Literature Review

Abstract

We report the case of a 52-year-old male patient with recurrent thrombosis from ‘primary antiphospholipid syndrome’ who developed renal microangiopathy. Despite anticoagulant therapy with coumadin, serum creatinine progressively increased from 398 to 592 μmol/l and platelets decreased to 43,000. The patient responded to high-dose methylprednisolone and aspirin and the renal function improved. A review of the literature disclosed 4 other cases of association between primary antiphospholipid syndrome and renal microangiopathy. The clinical characteristics of these cases are discussed.

Non-lupus nephropathy associated with antiphospholipid antibodies

“…Renal symptoms were found in two cases [9,10] in addition to our own [8].Kidney biopsy was performed in two out of three cases and they were classified as TMA [8,10] which were consistent with the major findings in adult patients with primary APS [11]. In the present case, the patient was asymptomatic except for persistent proteinuria.…”

Kidney Disease in Antiphospholipid Syndrome

“…Subsequent isolated cases or small series have confirmed our findings [32][33][34]. Recently, it has been emphasized that APS should be considered in the differential diagnosis of systemic hypertension and that APS-related TMA may cause isolated hypertension without significant renal impairment [35].…”

Kidney disease in primary anti-phospholipid antibody syndrome

“…Nochy and colleagues [65] studied kidney biopsy specimens from 16 patients with APS, in which a combination of glomerular basement membrane wrinkling and reduplication were seen ultra-structurally.…”

“…Proteinuria is often mild but can be within nephrotic range [34,73]. Histologically, focal or diffuse microangiopathic changes, affecting the whole intrarenal vascular tree and the glomerular tufts, have been observed [34,65].…”