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Anti-dsDNA, anti-Sm antibodies, and the lupus anticoagulant: significant factors associated with lupus nephritis

Background: Lupus nephritis (LN) is a common manifestation in patients with systemic lupus erythematosus (SLE). Autoantibodies and ethnicity have been associated with LN, but the results are controversial. Objective: To study the immunological and demographic factors associated with the development of LN. Patients and methods: A retrospective case-control study of 127 patients with biopsy-proven LN, and 206 randomly selected patients with SLE without nephritis as controls was designed. All patients had attended our lupus unit during the past 12 years. Standard methods were used for laboratory testing. Results: Patients with LN were significantly younger than the controls at the time of SLE diagnosis (mean (SD) 25.6 (8.8) years v 33.7 (12.5) years; p<0.0001). The proportion of patients of black ethnic origin was significantly higher in the group with nephritis (p=0.02). There were no differences in sex distribution or duration of follow up. A higher proportion of anti-dsDNA, anti-RNP, anti-Sm, and lupus anticoagulant (LA) was seen in the group with nephritis (p=0.002; p=0.005; p=0.0001; p=0.01, respectively). In univariate, but not in multivariate, analysis male sex and absence of anti-dsDNA were associated with earlier onset of renal disease (p=0.03; p=0.008). In multivariate analysis the only factors associated with nephritis were younger age at diagnosis of SLE, black race, presence of anti-dsDNA, anti-Sm, and LA. No demographic or immunological associations were seen with WHO histological classes. Conclusions: Young, black patients with anti-dsDNA, anti-Sm antibodies, and positive LA, appear to have a higher risk of renal involvement. These patients should be carefully monitored for the development of LN.

Renal artery stenosis in the antiphospholipid (Hughes) syndrome and hypertension

Background: Hypertension is common in the antiphospholipid (Hughes) syndrome (APS) and its cause is poorly understood. Anecdotal evidence suggests that renal artery stenosis (RAS) may be a relevant and treatable cause of hypertension. Objective: To investigate the prevalence of RAS in patients with APS and hypertension. Patients and methods: Three groups of patients were evaluated: (1) 77 patients with positive antiphospholipid antibodies (aPL) (60 secondary APS, 11 primary APS, and 6 with aPL only) and uncontrolled hypertension who were receiving two or more antihypertensive drugs; (2) 91 patients (⩽50 years) attending hypertension clinics;(3) 92 normotensive healthy, potential renal transplant donors. Magnetic resonance renal angiography was used to image the renal arteries in all three groups. Results: Group 1: 20/77 (26%) patients had evidence of RAS (16 unilateral and 4 bilateral). Sixteen patients (80%) had smooth well defined stenoses in the proximal third of the renal artery. Three further patients had irregular arteries without distinct stenosis. Group 2: 7/91 (8%) hypertensive patients had RAS (χ2=10.3, p<0.001 v group 1). Group 3: 3/92 (3%) healthy donors had RAS (χ2=18.2, p<0.0001 v group 1). Conclusion: A significantly increased prevalence of RAS (26%) was found in patients with APS and hypertension, compared with relatively young (⩽50 years) hypertensive controls and healthy potential donors.

Hypertension as the presenting feature of the antiphospholipid syndrome

The antiphospholipid or Hughes syndrome is the association between antiphospholipid antibodies (aPL), venous and arterial thromboses and pregnancy morbidity.1 Antiphospholipid syndrome (APS) commonly coexists with autoimmune diseases usually systemic lupus erythematosus (SLE), when it is known as secondary APS. When present in isolation it is known as primary APS (PAPS). Although the kidney may be affected in APS, its involvement is perhaps not as well described as that of other organs. Thrombotic microangiopathy (TMA) affecting the kidney has been reported as a manifestation in both primary and secondary APS. This report describes hypertension related to underlying renal TMA as a presenting symptom of APS.

Association of Thrombotic Microangiopathy and Intimal Hyperplasia With Bleeding Post–Renal Biopsy in Antiphospholipid Antibody–Positive Patients

Renal biopsy remains the gold standard investigation for both diagnostic and prognostic purposes in the clinical management of lupus nephritis. However, it is not without potentially significant complications. The objectives of this study were to determine the rate of significant bleeding post–renal biopsy in patients with lupus nephritis and to identify risk factors associated with hemorrhagic complications.

Goldblatt's kidney, Hughes syndrome and hypertension:

In 1934 Goldblatt et al. discovered that hypertension can be induced in animals by tying their renal arteries or removal of one kidney.1 Since then extensive clinical experience and new techniques have shown the link between renal artery stenosis and hypertension. We present two patients with antiphospholipid syndrome (APS, Hughes syndrome), who were found to have renal artery stenosis (RAS), one of whom also had a thrombotic renal microangiopathy.