Antiphospholipid Nephropathy Research Articles

28-Year-Old Man With Recurrent Vertigo, Syncope, and Progressive Memory Impairment

28-Year-Old Man With Recurrent Vertigo, Syncope, and Progressive Memory Impairment

Renal histopathological changes and clinical characteristics of antiphospholipid nephropathy in lupus nephritis patients

Objective The aim of the present study was to evaluate the renal histopathological changes and clinical characteristics associated with antiphospholipid nephropathy (APSN) in lupus nephritis (LN) patients. Patients and methods This study included 50 LN patients referred for renal biopsy. Patients underwent clinical and laboratory assessments for disease activity and damage. The biopsy specimens were classified according to the International Society of Nephrology/Renal Pathology Society (ISN/RPS) classification, activity, and chronicity indices, and assessed for renal vascular lesions of APSN - acute (thrombotic microangiopathy) and chronic (fibrous intimal hyperplasia, fibrous arterial/arteriolar occlusion and focal cortical atrophy). Results APSN lesions were found in 17/50 patients (34%); furthermore, 7/50 patients (14%) had thrombotic microangiopathy lesions, whereas chronic APSN lesions were detected in 15/50 patients (30%). LN patients with APSN had significantly higher age and Systemic Lupus International Collaborating Clinics scores (P = 0.032 and 0.004, respectively), but there were no differences in renal and antiphospholipid syndrome manifestations. Lupus anticoagulant positivity was significantly more frequent in patients with APSN (P = 0.002). LN patients with APSN had significantly higher renal chronicity scores (P = 0.033) with more frequent interstitial fibrosis and tubular atrophy (P = 0.006 for each). There was no significant difference in the distribution of LN classes in patients with and without APSN. Conclusion APSN is frequently found in LN patients irrespective of the LN class and antiphospholipid syndrome manifestations. It is associated with lupus anticoagulant positivity, higher disease damage, and renal biopsy chronicity indices, particularly interstitial fibrosis and tubular atrophy. Only the identification of intrarenal vascular lesions could characterize these patients, thus is it not time to revisit the ISN/RPS classification of LN to include renal vascular lesions of APSN.

Renal histopathological changes and clinical characteristics of antiphospholipid nephropathy in lupus nephritis patients

ObjectiveThe aim of the present study was to evaluate the renal histopathological changes and clinical characteristics associated with antiphospholipid nephropathy (APSN) in lupus nephritis (LN) patients.Patients and methodsThis study included 50 LN patients referred for renal biopsy. Patients underwent clinical and laboratory assessments for disease activity and damage. The biopsy specimens were classified according to the International Society of Nephrology/Renal Pathology Society (ISN/RPS) classification, activity, and chronicity indices, and assessed for renal vascular lesions of APSN - acute (thrombotic microangiopathy) and chronic (fibrous intimal hyperplasia, fibrous arterial/arteriolar occlusion and focal cortical atrophy).ResultsAPSN lesions were found in 17/50 patients (34%); furthermore, 7/50 patients (14%) had thrombotic microangiopathy lesions, whereas chronic APSN lesions were detected in 15/50 patients (30%). LN patients with APSN had significantly higher age and Systemic Lupus International Collaborating Clinics scores (P = 0.032 and 0.004, respectively), but there were no differences in renal and antiphospholipid syndrome manifestations. Lupus anticoagulant positivity was significantly more frequent in patients with APSN (P = 0.002). LN patients with APSN had significantly higher renal chronicity scores (P = 0.033) with more frequent interstitial fibrosis and tubular atrophy (P = 0.006 for each). There was no significant difference in the distribution of LN classes in patients with and without APSN.ConclusionAPSN is frequently found in LN patients irrespective of the LN class and antiphospholipid syndrome manifestations. It is associated with lupus anticoagulant positivity, higher disease damage, and renal biopsy chronicity indices, particularly interstitial fibrosis and tubular atrophy. Only the identification of intrarenal vascular lesions could characterize these patients, thus is it not time to revisit the ISN/RPS classification of LN to include renal vascular lesions of APSN.

Endothelin-1 expression in scleroderma renal crisis

The objective of the study was to investigate the role of endothelin-1 in the pathogenesis of scleroderma renal crisis in patients with systemic sclerosis. We used immunohistochemical analysis with anti-endothelin-1 and anti-von Willebrand factor antibodies in comparing kidney biopsies from patients with systemic sclerosis and scleroderma renal crisis (n = 14); from normal kidneys (n = 5); and from patients with typical hemolytic uremic syndrome and thrombotic microangiopathy (n = 5), antiphospholipid syndrome (n = 6), diabetic nephropathy (n = 5), minimal change disease with cyclosporine toxicity (n = 5), or nephroangiosclerosis (n = 5). Kidney biopsies from all systemic sclerosis patients presented specific lesions: glomerular lesions with thickened capillary walls (n = 6, 42.8%), mesangiolysis (n = 3, 21.4%), fibrin thrombi (n = 3, 21.4%), hypertrophy of juxtaglomerular apparatus (n = 5, 35.7%), arteriolar lesions showing mucinous intimal thickening and lumen mucoid occlusions (n = 13, 92.8%), proliferation of intimal cells (ie, "onion-skin" lesions; n = 13, 92.8%), fibrinoid necrosis (n = 3, 21.4%), and fibrin thrombosis (n = 4, 28.6%). Chronic lesions in large arteries showed modifications such as fibrous intimal thickening (n = 13, 92.8%). The pattern of endothelial staining for endothelin-1 in both glomeruli and arteriolar lesions appears to be specific for scleroderma renal crisis. Glomerular endothelin-1 staining without arteriolar staining was seen in hemolytic uremic syndrome; and isolated arteriolar staining (without glomerular staining) was seen in a number of conditions including antiphospholipid nephropathy, cyclosporine toxicity, and diabetic nephropathy. Endothelin-1 is overexpressed in glomeruli and arterioles of patients with scleroderma renal crisis, which suggests that endothelin-1 might be a therapeutic target in this condition.

Primary antiphospholipid nephropathy beginning during pregnancy

We describe a 29-year-old pregnant woman at 16 weeks gestation and antiphospholipid antibodies who developed nephrotic syndrome with massive hematuria. Renal biopsy evidenced chronic glomerular lesions of ischemic nature without proliferative changes and immune deposits suggestive of lupus nephritis. Anticoagulation was initiated, along supportive measures, and the patient recovered completely. This case demonstrates that chronic renal lesions of antiphospholipid syndrome may present with marked clinical manifestation including hypertension, massive proteinuria and hematuria, resembling the course of acute thrombotic microangiopathy and/or lupus nephritis.

Renal manifestations of the antiphospholipid syndrome

Renal thrombotic manifestations have been reported since the earliest descriptions of the antiphospholipid (Hughes) syndrome (APS). The spectrum of clinical features associated with antiphospholipid nephropathy continues to widen. This review will highlight recent developments such as the prevalence of hypertension, livedo reticularis and renal artery stenosis as well as the ultrastructural changes seen in antiphospholipid nephropathy. The increasing risks of renal transplantation in antiphospholipid antibody positive patients is also discussed leading some authors to question whether these patients should undergo transplantation at all.