Showing papers by "Thomas Daikeler published in 2005"

Autologous Hematopoietic Stem Cell Transplantation for Autoimmune Diseases

Abstract: Ten years have passed since the first published consensus statement on the use of hematopoietic stem cell transplantation (HSCT) in the treatment of severe autoimmune disease (AD) appeared. During tha

Autologous stem cell transplantation of treatment-resistant systemic vasculitis - : A single center experience and review of the literature

Abstract: Aims: Autologous peripheral blood stem cell transplantation (autoPBSCT) is increasingly and successfully applied to patients with treatment-resistant autoimmune diseases, mainly multiple sclerosis and systemic sclerosis, but also juvenile idiopathic arthritis and systemic lupus erythematosus. We intended to analyze the effects of autoPBSCT in patients with treatment-resistant systemic vasculitis by analyzing the outcome of 4 patients from our own hospital, and comparing them to cases reported in the literature. Methods: 4 patients with treatment-resistant vasculitis (Wegener granulomatosis, C'hurg Strauss syndrome, Takayasu arteritis and relapsing polychondritis) received an autologous PBSCT. Stem cell mobilization was performed with cyclophosphamide (CY) and G-CSF, stem cells were purged by positively selecting CD34 + stem cells over a CliniMacs device, and the conditioning was performed with high dose CY and anti-thymocyte globulin (ATG). Results: AutoPBSCT was well tolerated in all 4 patients. The patient with WG achieved complete remission although cANCA persisted, the other patients are in good partial remissions and respond to maintenance treatments which had been ineffective before PBSCT (CSA, azathioprin). Glucocorticosteroids (GC) could be reduced to a maximum of 10 ing in all patients. Shortly after the procedure, reactivation of viruses from the herpes family occurred in 3 of the patients and had to be treated. In the data base, 25 patients transplanted for severe systemic vasculitis are registered, in the literature, 6 additional vasculitis patients remitting after autoPBSCT are reported. Conclusions: Autologous PBSCT is feasible and effective in severe, treatment-resistant forms of systemic vasculitis. Data are sparse, further prospective studies are needed. These should also aim at evaluating more optimal regimens for conditioning and purging during PBSCT, as in most of the vasculitis patients reported until now, mostly good partial remissions, but less complete remissions were achieved.

Familial inflammatory Sneddon’s syndrome—case report and review of the literature

Abstract: Sneddon's syndrome (SNS) which originally was a clinical diagnosis, is now regarded as a common clinical manifestation of different disease entities. It has been divided into idiopathic, autoimmune and thromboembolic subsets or in systemic lupus erythematosus (SLE)-associated, antiphospholipid syndrome (APS)-associated and primary forms. Familial occurrence of Sneddon's syndrome is rare. We present a familial case of Sneddon's syndrome with inflammatory disease pattern, early disease onset and association with autoimmune thyroid disease and anticardiolipin antibodies. Although most authors reporting on adult cases of SNS consider it a non-inflammatory, thromboembolic process, the study of cases with early onset brings attention to the possible inflammatory origin of the syndrome.

Fever and increasing cANCA titre after kidney and autologous stem cell transplantation for Wegener’s granulomatosis

Abstract: Wegener’s granulomatosis is a systemic vasculitis mainly affecting the lungs, nasal sinuses, and kidneys. Treatment usually consists of cyclophosphamide (Cy) and steroids.1 High dose Cy with autologous stem cell support could be an alternative treatment for patients resistant to conventional treatment or requiring long term immunosuppression with the risk of secondary malignancy.2 We report on a 33 year old woman with chronic relapsing sinusitis, pulmonary granuloma, and proteinuria with progressive renal insufficiency since 1988. Renal biopsy showed necrotising glomerulonephritis, and biopsy of the nasal sinus showed granuloma with necrotising vasculitis. Proteinase-3-antineutrophil cytoplasmic antibodies (PR3-ANCA) were detectable with a titre of 1/280. Despite treatment with Cy (750 mg/m2 every 3 weeks, later 100 mg/day orally) and steroids renal function deteriorated, and she underwent dialysis from April 1995 to December 1998. Live kidney transplantation from the patient’s sister matched …

Autologous stem cell transplantation of treatment-resistant systemic vasculitis - : A single center experience and review of the literature

Abstract: Aims: Autologous peripheral blood stem cell transplantation (autoPBSCT) is increasingly and successfully applied to patients with treatment-resistant autoimmune diseases, mainly multiple sclerosis and systemic sclerosis, but also juvenile idiopathic arthritis and systemic lupus erythematosus. We intended to analyze the effects of autoPBSCT in patients with treatment-resistant systemic vasculitis by analyzing the outcome of 4 patients from our own hospital, and comparing them to cases reported in the literature. Methods: 4 patients with treatment-resistant vasculitis (Wegener granulomatosis, C'hurg Strauss syndrome, Takayasu arteritis and relapsing polychondritis) received an autologous PBSCT. Stem cell mobilization was performed with cyclophosphamide (CY) and G-CSF, stem cells were purged by positively selecting CD34 + stem cells over a CliniMacs device, and the conditioning was performed with high dose CY and anti-thymocyte globulin (ATG). Results: AutoPBSCT was well tolerated in all 4 patients. The patient with WG achieved complete remission although cANCA persisted, the other patients are in good partial remissions and respond to maintenance treatments which had been ineffective before PBSCT (CSA, azathioprin). Glucocorticosteroids (GC) could be reduced to a maximum of 10 ing in all patients. Shortly after the procedure, reactivation of viruses from the herpes family occurred in 3 of the patients and had to be treated. In the data base, 25 patients transplanted for severe systemic vasculitis are registered, in the literature, 6 additional vasculitis patients remitting after autoPBSCT are reported. Conclusions: Autologous PBSCT is feasible and effective in severe, treatment-resistant forms of systemic vasculitis. Data are sparse, further prospective studies are needed. These should also aim at evaluating more optimal regimens for conditioning and purging during PBSCT, as in most of the vasculitis patients reported until now, mostly good partial remissions, but less complete remissions were achieved.

Relapse of autoimmune diseases after autologous T cell depleted stem cell transplantation may be triggered by T cells recently emigrated from the thymus

Abstract: High dose immunosuppressive therapy with subsequent autologous stem cell transplantation (ASCT) has proved effective for treatment resistant autoimmune diseases.1 However, in a number of patients, relapses of autoimmune disease after initial improvement or even remission occur. Until recently, it was impossible to differentiate recent thymic emigrants from residual or peripherally expanded T cells. Douek and coworkers2 and later our group3 described a method for detection of T cell receptor excision circles, stable DNA episomes which are formed during T cell receptor rearrangement in the thymus which are not replicated during mitosis, but are diluted during cell divisions.4 This study aimed at investigating whether these flares are associated with de novo T cell development. Peripheral blood mononuclear cells were collected from six patients, mean age 32.5 years, range 24–42 (four with systemic sclerosis (SSc), one with Wegener’s granulomatosis …