Cardiac complications of Behcet's disease.

We describe two patients with Behcet's disease (BD) with cardiac complications and their response to treatment. This report adds to the evidence that cardiac involvement can be a feature of Behcet's disease and may be more common than previously thought.

Alveolar haemorrhage in eosinophilic granulomatosis and polyangiitis (Churg-Strauss).

We describe two patients of alveolar haemorrhage in patients with eosinophilic granulomatosis with polyangiitis (eGPA). This report adds to the evidence that pulmonary haemorrhage is a rare but severe manifestation of eGPA. It may not be associated with positive ANCA antibodies and requires aggressive treatment.

Rituximab in the treatment of resistant lupus nephritis: therapy failure in rapidly progressive crescentic lupus nephritis.

OBJECTIVE: The objective of this paper is to report the clinical outcome of B cell depletion therapy in 18 patients with refractory lupus nephritis (LN). METHODS: Eighteen patients received rituximab on an open-label basis with prospective evaluations. All patients had renal disease refractory to conventional immunosuppressive therapy, including intravenous cyclophosphamide (CyC). All patients fulfilled the revised ACR classification criteria for SLE. Rituximab was given as 2 × 1 g infusions with 500 mg iv CyC and 500 mg iv methylprednisolone, two weeks apart. Complete remission (CR) of nephritis at six months was defined as normal serum creatinine and serum albumin levels, inactive urine sediment, and proteinuria < 0.5 g/day; partial remission (PR) was defined as a ≥50% improvement in all renal parameters that were abnormal at baseline. Clinical response was assessed by the British Isles Lupus Assessment Group (BILAG) score pre- and post-rituximab treatment, and efficacy was recorded by extent and duration of B lymphocyte depletion (normal range 0.100-0.500 × 10(9)/l). Follow-up data were collected at six months, one year post-treatment and at the most recent clinic visit. RESULTS: At six months, 11/18 patients reached renal CR and two of 18 PR. The mean global BILAG scores for responders decreased from 15 (SD 10) to 5 (SD 3), and a total of ten A scores disappeared. Five patients failed to show complete or partial renal response despite peripheral B lymphocyte count depletion, and progressed to end-stage renal failure (ESRF) and dialysis. Four of these patients had severe proliferative, crescentic nephritis, of whom three had Class IV-G, one Class III and one late membranous glomerulonephritis. One patient died six years after rituximab therapy from overwhelming sepsis while on long-term haemodialysis. CONCLUSION: Rituximab therapy achieved a response in 13/18 patients with refractory LN. However, in patients with rapidly progressive crescentic LN, when there is already evidence of significant renal impairment, rituximab therapy may not prevent progression to ESRF and dialysis. Our data also suggest that severe Class IV-G LN may be associated with a poor response to therapy.

Weight loss and improvements in fatigue in systemic lupus erythematosus: a controlled trial of a low glycaemic index diet versus a calorie restricted diet in patients treated with corticosteroids.

BACKGROUND: Patients with systemic lupus erythematosus (SLE) may require prolonged periods of corticosteroid therapy which lead to excessive weight gain and increased cardiovascular risk. OBJECTIVE: To assess the utility of a low glycaemic index diet in patients with corticosteroid dependent SLE in achieving weight loss and improving glycaemic control. DESIGN: A total of 23 women were enrolled in a 6 week study. All had mild, stable SLE, were receiving corticosteroids and had a body mass index > 25 kg/m(2). Subjects were randomly assigned to a low glycaemic index (Low GI) diet or a calorie restricted (Low Cal) diet. The primary end point was weight loss. Secondary end points included tolerability of diet, bio-markers of cardiovascular risk, disease activity, fatigue and sleep quality. RESULTS: Weight loss in both treatment groups was significant (mean ± SD: Low GI diet 3.9 ± 0.9 kg; Low Cal diet 2.4 ± 2.2 kg, p < 0.01 from baseline in each group). There were also significant improvements in waist and hip measurements. However, the difference in weight loss and waist and hip measurements between the two diet groups was not statistically significant. There was a statistically significant reduction in Fatigue Severity Scale in both diet groups, (p < 0.03). Both Low GI and Low Cal diets were well tolerated, resulting in no serious adverse effects or increase in disease activity. CONCLUSION: Significant weight loss is achievable over 6 weeks in a diet-specific trial in subjects with stable SLE, who are on low dose prednisolone. Both diets were equally tolerable, and did not cause flares in disease activity. Our results suggest that dietary manipulation may significantly improve fatigue in subjects with SLE.

Ankle-brachial pressure index: a simple tool for assessing cardiovascular risk in patients with systemic vasculitis.

OBJECTIVE: Cardiovascular disease may be increased in patients with systemic vasculitides (SV). The Ankle-Brachial Pressure Index (ABPI) is a non-invasive tool for the assessment of cardiovascular risk (CV). Our aim was to determine the prevalence of an abnormal ABPI in patients with SV and healthy controls and to correlate with clinical and serological parameters. METHODS: We studied 54 consecutive vasculitis patients (20 males) attending the vasculitis clinic and 49 healthy subjects. Patients were classified according to the ACR 1990 criteria and the Chapel Hill Consensus definitions. There were 18 patients with Wegener's granulomatosis, eight with Behcet's disease, seven with Churg-Strauss Syndrome, three with Henoch-Schonlein purpura, three with polyarteritis nodosa, three with Takayasu's disease, three with p-ANCA associated vasculitis, three with urticarial vasculitis, two with cutaneous leucocytoclastic angiitis, one with microscopic polyangiitis, one with primary central nervous system angiitis, one giant cell arteritis and one with cutaneous vasculitis secondary to Sjogren's syndrome. Traditional risk factors as well as glucose, lipid profile, CRP, hsCRP, ANCA and aPL were assessed. ABPI was measured according to a consensus statement on the methodology. RESULTS: The ABPI was abnormal in 11/54 (20.4%) of SV patients and 2/49 (4%) of the control group (chi(2) with Yates correction = 4.8, P

[Orthopedic manifestations of the antiphospholipid syndrome]. / Manifestations osseuses du syndrome des antiphospholipides.

PURPOSE: The antiphospholipid syndrome (APS) is characterized by arterial and/or venous thrombosis, and pregnancy morbidity in association with antiphospholipid antibodies. Since its classical description 22 years ago, the clinical spectrum of APS has embraced the realms of obstetrics, nephrology, cardiology, neurology, gastroenterology, angiology and now, possibly orthopaedics. This is not surprising given that this disease can affect virtually any organ system and blood vessel of any size and nature. Just as venous thrombosis may affect limbs and internal organs, arterial thrombosis has been shown to affect organs such as the brain, eye, heart, kidney, liver and may also involve the skeleton. CURRENT KNOWLEDGE AND KEY POINTS: In this review, we describe the orthopedic aspects of APS recently reported, bone metatarsal fractures, osteonecrosis and more exceptional complications, ie algodystrophy and bone marrow necrosis. We briefly discuss postulated pathogenesis and possible implications of anticoagulation. FUTURE PROSPECTS AND PROJECTS: This data need further confirmation. They may suggest complementary physiopathologic and therapeutic implications.

Renal artery stenosis in hypertensive patients with antiphospholipid (Hughes) syndrome: outcome following anticoagulation.

OBJECTIVE: We have demonstrated a point prevalence of 26% renal artery stenosis in patients with antiphospholipid syndrome (APS) and uncontrolled hypertension. We describe the effect of anticoagulation on blood pressure control and renal function. METHODS: We studied 23 patients retrospectively with renal artery stenosis (RAS). Fourteen received oral anticoagulation for more than 1 yr (target International Normalized Ratio (INR) of 3.0-4.5). Five patients had primary APS. Patients were divided into two groups based on their INR (< 3.0 and > or = 3.0). Nine patients had repeat magnetic resonance angiography (MRA) or an angiogram of the renal arteries after 2 yr. RESULTS: Only 8/14 patients managed to maintain their INR > or = 3.0 (median INR 3.1, range 2.8-3.7) while six had a INR < 3.0 (median INR 1.9, range 1.2-2.4). Patients with a median INR < 3.0 had poorly controlled blood pressure and there was significant deterioration in mean serum creatinine values (Wilcoxon's test, P < 0.03). Nine patients underwent follow-up renal artery imaging. Three of nine patients with an INR < 3.0 (median INR 1.9) had re-stenosis and a fourth developed bilateral renal artery stenosis. Five patients with INR > or = 3.0 (median INR 3.1) did not show re-stenosis of the renal arteries; their renal function was stable and blood pressure was well controlled. One other patient with secondary APS (mixed connective tissue disorder) with INR > 3.0 showed recanalization of the stenosed renal artery. CONCLUSION: Anticoagulation with INR maintained > or = 3.0 helped to control the blood pressure and prevent the progression of renal disease.

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 (