Blue toe syndrome as a first sign of systemic sclerosis.

We describe an unusual case of blue toe syndrome as the primary and solitary manifestation of systemic sclerosis. The possible cause was long-term occupational exposure in construction work. Blue toe syndrome is a small vessel disease, characterised by the sudden development of painful, blue discolouration in one or more toes. The most common aetiology is atheroembolic disease; however, it can also appear in several conditions ranging from hypercoagulability disorders to underlying systemic diseases such as vasculitis or autoimmune diseases. Here, we describe the case of a 57-year-old man who presented with blue toe syndrome without underlying atheroembolic disease. He was found to have positive anticentromere antibodies, which indicated that systemic sclerosis was the likely primary underlying cause. An extensive systemic evaluation and a thorough physical examination revealed no other symptoms associated with systemic sclerosis. He was prescribed nifedipin and rosuvastatin, and showed complete resolution of symptoms after 3 months.

Hypophosphataemia due to FGF-23 producing B cell non-Hodgkin's lymphoma.

Oncogenic osteomalacia (or tumour-induced osteomalacia) is a rare paraneoplastic syndrome caused by overproduction of fibroblastic growth factor 23 (FGF-23) by tumours. Excessive production of FGF-23 can lead to severe, symptomatic hypophosphataemia. The majority of cases have been associated with benign tumours of bone or soft tissue, such as haemangiopericytomas or other neoplasms of mesenchymal origin. We present a case of a 68-year-old woman with an FGF-23 producing B cell non-Hodgkin's lymphoma. Treatment with immunochemotherapy resulted in normalisation of serum FGF-23 and phosphate levels.

High cut-off haemodialysis induces remission of recurrent idiopathic focal segmental glomerulosclerosis after renal transplantation but is no alternative to plasmapheresis.

A 26-year-old male experienced a recurrence of idiopathic focal segmental glomerulosclerosis (iFSGS) after his second renal transplant. Reduction of proteinuria was rapidly induced by plasmapheresis (PP) and the patient has remained in remission with a once-weekly PP regimen, which has now been continued for >3½ years. We were also able to induce remission of iFSGS in this patient by treatment with high cut-off haemodialysis using the Theralite™ dialyser. This observation lends support for the pathophysiological role of an as yet unknown, circulating glomerular filtration barrier permeability factor with an estimated weight of between 30 and 50 kDa.

Ten-year follow-up of recipients of a kidney or heart transplant who received induction therapy with a monoclonal antibody against the interleukin-2 receptor.

OBJECTIVES: Twelve years ago, we performed two randomized clinical trials to investigate safety and efficacy of induction therapy with BT563, a highly potent murine monoclonal antibody against the interleukin-2 receptor after kidney and heart transplantation. We analyzed the long-term safety and efficacy data from all 120 patients who participated in the two randomized trials after kidney and heart transplantation 10 years ago. MATERIALS AND METHODS: One of these two trials was a randomized, double-blind, placebo-controlled trial, with 60 primary and secondary kidney allograft recipients (cadaveric and living-related donors). The control group was treated with the standard regimen at that time, consisting of cyclosporin and prednisone. In the study group, BT563 was added for 10 days. The second trial was a randomized, double-blind trial, with 60 recipients of a primary heart transplant. In that study, we compared induction therapy with BT563 with the standard regimen at that time, consisting of cyclosporin, prednisone, and OKT3 (both induction agents were given for 7 days). RESULTS: Patient survival in the kidney trial was excellent: in the BT563 group, 24 patients were alive (80%), and in the placebo, group 21 (70%) 10 years after transplantation. Also, graft survival was good: in the BT563 group, 63.3% of the kidneys (19/30) were functioning, in the placebo group, 72.4% (21/29) were functioning (P = 0.455). Also, in the heart study, patient (and graft) survival was excellent: 18 patients were alive in the BT563 group (58%), and in the OKT3 group, 21 (72%) patients were alive (P = ns). No increase in the incidence of malignancies was observed between patients treated with BT563 compared with the control groups. Patients following heart transplantation more often suffered from a malignancy than did patients after kidney transplantation (20/60 vs 10/59). CONCLUSIONS: We report follow-up data on all patients participating in the two randomized trials, and our data reflect a total of 932 years of patient follow-up. Patient and graft survival appear to be excellent in both the BT563-treated patients and the control groups. BT563 treatment was not associated with an increased likelihood of developing infections or malignancies.

Tacrolimus dose requirement in renal transplant recipients is significantly higher when used in combination with corticosteroids.

AIMS: To evaluate the effect of corticosteroids on tacrolimus pharmacokinetics. METHODS: In a randomized trial, kidney transplant recipients were treated with tacrolimus and mycophenolate mofetil with either daclizumab (n = 31) or 3 months of prednisone (n = 34). Tacrolimus dose-adjusted predose concentrations (C0) at month 1-6 were compared between both groups and within the corticosteroid group before and after prednisone withdrawal. RESULTS: At month 1 the tacrolimus dose-adjusted C0 in the corticosteroid group was 83 +/- 8 vs 119 +/- 17 ng ml-1 mg-1 kg-1 in the daclizumab group. The tacrolimus dose-adjusted C0 within the corticosteroid group at month 1 and 2 was 42% and 29% lower compared with month 4 (P < 0.001). CONCLUSIONS: A higher tacrolimus dose is required to reach target concentrations when used in combination with corticosteroids.