RESEARCH - APS nephropathy in catastrophic, primary, and SLE-related APS

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J Rheumatol. 2008 Aug 1. [Epub ahead of print]

Antiphospholipid Syndrome (APS) Nephropathy in Catastrophic, Primary,

and Systemic Lupus Erythematosus-related APS.

Tektonidou MG, Sotsiou F, Moutsopoulos HM.

From the Department of Pathophysiology, School of Medicine, National

University of Athens; Rheumatology Department, Euroclinic Hospital;

and Renal Pathology Department, Evagelismos Hospital, Athens, Greece.

OBJECTIVE: Renal involvement in antiphospholipid syndrome (APS) has

been poorly recognized. A renal small-vessel vasculopathy, defined as

APS nephropathy, has recently been observed in small series of

patients with primary APS (PAPS) and systemic lupus erythematosus

(SLE)-APS. We examined the renal histologic, clinical, and laboratory

characteristics of different groups of patients with APS including

catastrophic APS (CAPS).

METHODS: Our study included all CAPS (n = 6), PAPS (n = 8), and

SLE-APS (n = 23) patients with biopsy-proven renal involvement who

were referred to our departments. The kidney biopsy specimens were

retrospectively examined by the same renal pathologist. APS

nephropathy was diagnosed as previously described. Demographic,

clinical, and laboratory data were recorded.

RESULTS: All patients with CAPS had acute and chronic renal vascular

lesions compatible with diagnosis of APS nephropathy. Thrombotic

microangiopathy (TMA), the acute lesion, was observed in all CAPS

patients. Fibrous intimal hyperplasia of interlobular arteries (FIH)

and focal cortical atrophy (FCA) were the most common chronic vascular

lesions, occurring in 4 of 6 (66.7%) and 3 of 6 (50%) patients with

CAPS, respectively. TMA was detected in 3 of 8 (37.5%) patients with

PAPS and in 8 of 23 (35%) patients with SLE-APS, while FIH and FCA

were found with similar frequencies in all 3 groups. Hypertension,

proteinuria, hematuria, and renal insufficiency were the most common

renal manifestations of all APS groups.

CONCLUSION: Acute and chronic APS nephropathy lesions were detected in

all 3 APS groups. Acute lesions were more prominent in CAPS, while

chronic lesions were found with similar frequencies in all groups.

Hypertension, proteinuria, hematuria, and renal insufficiency were the

most common renal manifestations of all APS groups.

PMID: 18688914

http://www.ncbi.nlm.nih.gov/pubmed/18688914

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