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ABSTRACT: Fever of unknown origin (FUO) is a common medical diagnosis by exclusion. In these cases, fever is the predominant symptom of an underlying disease. We describe the case of a 60-year old patient with FUO. Intensive search for the causative disease was carried out. Unfortunately all the investigations remained fruitless. Eventually, the patient was discharged with the diagnosis of common variable immunodeficiency, based on hypogammaglobulinemia and Cytomegalovirus replication. Two weeks after discharge, the patient presented in the outpatient clinic with the typical symptoms of giant cell arteriitis (GCA). The diagnosis was confirmed by a repeated ultrasound imaging and biopsy findings. The clinical condition of the patient improved rapidly after beginning of treatment with steroids. This case illustrates the importance of a longitudinal observation of patients presenting with FUO if the diagnosis remains unclear after intensive investigations.
Therapeutische Umschau 12/2012; 69(12):697-701.
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ABSTRACT: Purpose: In patients with suspected giant cell arteritis (GCA), a search for the perivascular halo sign, a sophisticated color duplex ultrasound (CDU) finding, at experienced centers reliably identifies inflamed temporal arteries (TA). We tested whether TA compression in patients with GCA, a simple, largely operator-independent maneuver, elicits contrasting echogenicity between the diseased artery wall and the surrounding tissue (compression sign). Materials and Methods: 80 individuals with suspected GCA were prospectively enrolled in this single-center study. In all study participants, bilateral ultrasound examination of the TA established the presence/absence of the halo and compression sign. A positive compression sign was defined as visibility of the TA upon transducer-imposed compression of the artery. Based on ACR criteria, a team of specialized physicians independently grouped patients as GCA versus non-GCA. Results: 43/80 study participants were grouped as GCA. Both the halo sign and the compression sign were positive in 34/43 patients in the GCA group, and negative in all 37/37 of the non-GCA group, resulting in a sensitivity of 79 % and a specificity of 100 % for both the halo and the compression sign. Conclusion: In this cohort of individuals with suspected GCA, the halo sign and the compression sign were equal in their diagnostic performance. The simplicity of the compression sign suggests a level of reliability warranting further evaluation.
Ultraschall in der Medizin 06/2012; · 2.40 Impact Factor
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ABSTRACT: Over 1,500 patients world wide have received a hematopoietic stem cell transplant (HSCT) as treatment for a severe autoimmune disease. Most of these have been autologous and mostly have occurred in the past 15 years. Over 1,000 of these have been registered in the European Group for Bone Marrow Transplantation (EBMT) and European League Against Rheumatism (EULAR) combined data base. A recent retrospective analysis of 900 patients (1) showed that the majority had multiple sclerosis (n = 345) followed by systemic sclerosis (n = 175), systemic lupus erythematosus (n = 85), rheumatoid arthritis (n = 89), juvenile idiopathic arthritis (n = 65) and idiopathic cytopenic purpura (n = 37). An overall 85 % 5-year-survival and 43 % progression-free survival was seen, with 100-day-transplant-related-mortality (TRM) ranging between 1 % (rheumatoid arthritis) and 11 % (systemic lupus erythematosus and juvenile idiopathic arthritis). Around 30 % of patients in all disease subgroups had a complete response, often durable despite full immune reconstitution. In many, e. g. systemic sclerosis, morphological improvement such as reduction of skin collagen and normalisation of microvasculature was documented, beyond any predicted known effects of intense immunosuppression alone. The high TRM was in part related to conditioning intensity, comorbidity and age, and the final risk/benefit assessment will be made after the results of the three randomised propective clinical trials are known. [nl]Recently, multipotent mesenchymal stromal cells have been tested in various autoimmune diseases, exploiting their immune modulating properties and apparent low acute toxicity. Despite encouraging small phase I/II studies, no positive data from randomised, prospective studies are as yet available in the peer reviewed literature.
DMW - Deutsche Medizinische Wochenschrift 08/2011; 136(33):1684-6. · 0.53 Impact Factor
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ABSTRACT: Giant cell arteritis (GCA) is the most common of the vasculitis syndromes and, being a disease of the elderly, its incidence is increasing with the general ageing of the population. GCA is most feared for its early complications, namely blindness and stroke, resulting from inflammation and subsequent occlusion of ocular and extra cranial arteries, respectively. More recently, however, GCA has been recognised to also affect limb arteries and the aorta with a high prevalence. These newly recognised features of GCA pose diagnostic, therapeutic and prognostic challenges to treating physicians. Here, recent developments in the field of GCA are summarised and discussed.
Schweizerische medizinische Wochenschrift 01/2011; 141:w13272. · 1.68 Impact Factor
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Scandinavian journal of rheumatology 04/2009; 38(5):393-4. · 2.51 Impact Factor
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ABSTRACT: Vision loss and ischaemic stroke are feared complications in GCA. We investigated how platelet count and size and platelet inhibition with ASA relate to ischaemic complications in patients with GCA.
Charts of patients with GCA were retrospectively analysed. Jaw claudication, amaurosis fugax, blurred vision, ischaemic stroke and permanent visual loss were classified as 'ischaemic events'; ischaemic stroke and permanent visual loss were sub-grouped as 'severe ischaemic events'. The incidence of ischaemia and the association to the pre-defined covariates age, fever, ESR, platelet count and size and ASA treatment were assessed.
Eighty-five patients (mean age 73 yrs, 60% women, 78% biopsy-proven) were included in the analysis. Of the 85 patients, 62 (73%) presented with ischaemic events, 29/85 patients (34%) with severe ischaemic events. At the time of diagnosis 22/85 patients (26%) were treated with ASA. Of these 22 patients, 15 (68%) presented with ischaemic events, 7/22 patients (32%) with severe ischaemic events. In multivariate analysis, neither platelet count nor size or ASA treatment were significantly associated with ischaemic or severe ischaemic events.
The incidence of severe ischaemic events in patients with GCA was high, irrespective of platelet count and size and established ASA treatment.
Rheumatology (Oxford, England) 02/2009; 48(3):258-61. · 4.24 Impact Factor
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Thomas Daikeler
Swiss medical weekly: official journal of the Swiss Society of Infectious Diseases, the Swiss Society of Internal Medicine, the Swiss Society of Pneumology 02/2009; 139(3-4):34. · 1.89 Impact Factor
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T Daikeler,
T Hügle,
D Farge,
M Andolina,
F Gualandi,
H Baldomero,
C Bocelli-Tyndall,
M Brune,
J H Dalle,
C Urban, [......],
B Lioure,
A Marmont,
S Matthes-Martin,
D Nachbaur,
P Schuetz,
A Tyndall,
J M van Laar,
P Veys,
R Saccardi,
A Gratwohl
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ABSTRACT: Allogeneic hematopoietic SCT (HSCT) has been used as treatment for single patients with autoimmune diseases (AD). To summarise currently available information, we analyzed all patients who underwent allogeneic HSCT for AD and who reported to the European Group for Blood and Marrow Transplantation (EBMT) database. Thirty-five patients receiving 38 allogeneic transplantations for various hematological and non-hematological AD were identified. Four patients had had an allogeneic HSCT for a conventional hematological indication in the past. Fifty-five per cent of the transplantation procedures led to a complete clinical response of the refractory AD and 23% to at least a partial response. The median duration of response at the last follow-up was 70.7 (15.2-130) months. Three patients relapsed at a median of 12.3 months after HSCT. Treatment-related mortality at 2 years was 22.1% (95% CI: 7.3-36.9%). Two deaths were caused by progression of AD. The probability of survival at 2 years was 70%. No single factor predicting the outcome could be identified. The retrospective nature of this study and the heterogeneous, partly incomplete data are its limitations. However, allogeneic HSCT can induce remission in patients suffering from refractory AD. These data provide the basis for carefully conducted prospective trials.
Bone marrow transplantation 02/2009; 44(1):27-33. · 3.00 Impact Factor
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Annals of the rheumatic diseases 08/2008; 67(7):1057-9. · 8.11 Impact Factor
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ABSTRACT: Many rheumatic diseases have been observed in HIV-infected persons. We, therefore, conducted a comprehensive literature search in order to review the prevalence, presentation and pathogenesis of rheumatic manifestations in HIV-infected subjects. Articular conditions (arthralgia, arthritis and SpAs) are either caused by the HIV infection itself, triggered by adaptive changes in the immune system, or secondary to microbial infections. Muscular symptoms may result from rhabdomyolysis, myositis or from side-effects of highly active anti-retroviral therapy (HAART). Osseous complications include osteonecrosis, osteoporosis and osteomyelitis. Some conditions such as the diffuse infiltrative lymphocytosis syndrome and sarcoidosis affect multiple organ systems. SLE may be observed but may be difficult to differentiate from HIV infection. Some anti-retroviral agents can precipitate hyperuricaemia and are associated with arthralgia. When indicated, immunosuppressants and even anti-TNF-alpha agents can be used in the carefully monitored HIV patient. Thus, rheumatic diseases and asymptomatic immune phenomena remain prevalent in HIV-infected persons even after the widespread implementation of highly active anti-retroviral therapy.
Rheumatology (Oxford, England) 08/2008; 47(7):952-9. · 4.24 Impact Factor
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Annals of the rheumatic diseases 06/2008; 67(5):723-4. · 8.11 Impact Factor
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J Larghero,
D Farge,
A Braccini,
S Lecourt,
A Scherberich,
E Foïs,
F Verrecchia, T Daikeler,
E Gluckman,
A Tyndall,
C Bocelli-Tyndall
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ABSTRACT: Mesenchymal stem cells (MSCs) have a potential immunomodulatory role in autoimmune disease; however, the qualitative properties and haematopoietic support capacity of MSCs derived from patients with autoimmune disease is unclear.
To further characterise phenotypically and functionally bone marrow (BM)-derived MSCs from patients with systemic sclerosis (SSc).
Key parameters of BM-derived MSC function and phenotype were assessed in 12 patients with SSc and compared with 13 healthy normal controls. The parameters included the ability to: form colony-forming unit fibroblasts (CFU-F), differentiate along the adipogenic and osteogenic lineages, express cell surface antigens defining the MSCs population, support normal haematopoiesis and suppress in vitro lymphocyte proliferation induced by either anti-CD3epsilon plus anti-CD28 monoclonal antibodies or the mixed lymphocyte reaction.
SSc MSCs were shown to have a similar characteristic phenotype, capacities to form CFU-F and to differentiate along adipogenic and osteogenic lineages as those of healthy donor MSCs. The ability of SSc MSCs to support long-term haematopoiesis was also identical to that of controls. Both healthy donor and SSc BM MSCs reduced the proliferation of autologous and allogeneic peripheral blood mononuclear cells in a cell number dependent fashion.
These results show that BM-derived MSCs from patients with SSc under the described culture conditions exhibit the same phenotypic, proliferative, differentiation potential and immunosuppressive properties as their healthy counterparts and could therefore be considered in an autologous setting. Further studies are needed to ensure the quality and safety of large-scale expansion of patient MSCs prior to their potential use in clinical trials.
Annals of the rheumatic diseases 05/2008; 67(4):443-9. · 8.11 Impact Factor
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ABSTRACT: We report on a twenty-three year old woman with shifting polyarthritis after an episode with gastroenteritis. Despite normalisation of stool we could detect campylobacter jejuni one month later. After therapy with macrolide antibiotics arthritis recovered.
Praxis 03/2008; 97(3):147-50.
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DMW - Deutsche Medizinische Wochenschrift 03/2008; 133(9):411-2. · 0.53 Impact Factor
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ABSTRACT: We present a 53 year old woman with a progressive violaceous erythema (heliotrop rash) of the face with periorbital oedema. A clinical diagnosis of dermatomyositis was proposed. Except for the cutaneous manifestation there was no further clinical evidence of systemic disease and specifically of muscle involvement. The lesions developed about six months earlier and an amyopathic dermatomyositis was diagnosed. The patient was treated with hydroxychloroquine. The risk of malignant disease is increased in primary idiopathic and amyopathic adult onset dermatomyositis. Therefore an adequate work-up is mandatory.
Praxis 08/2007; 96(27-28):1087-90.
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The Journal of the Association of Physicians of India 06/2007; 55:348.
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ABSTRACT: 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.
4 patients with treatment-resistant vasculitis (Wegener granulomatosis, Churg 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).
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 mg 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.
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.
Clinical nephrology 01/2006; 64(6):485-9. · 1.17 Impact Factor
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Annals of the Rheumatic Diseases 01/2006; 64(12):1787-9. · 8.73 Impact Factor
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Annals of the Rheumatic Diseases 05/2005; 64(4):646-7. · 8.73 Impact Factor
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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.
Clinical Rheumatology 03/2005; 24(1):79-82. · 2.00 Impact Factor
