Search PubMed⌕ Search

Biomedical subjects

W L Gross

Publications and source records attributed to W L Gross.

At least 37 records · Page 2Linked to original sources
PubMed2005

[A new training program--a status report: vasculitis].

Referring to the literature, it is known that structured standardized patient education represents an effective additional treatment in patients with chronic diseases. Programs are based on cognitive behavioral interventions. Within the last years an interdisciplinary approach for providing information on disease, therapies, side effects, coping strategies, nutrition and physiotherapy has been developed for patients with primary systemic vasculitides (PSV) in the vasculitis center Medical University of Luebeck/Bad Bramstedt. The contents of the seminars were revised and condensed into five modules. To evaluate the new program a documentation system consisting of patient and physician-administered questionnaires assessing socioeconomic, knowledge and disease-related outcome-parameters has been designed. Patients completed the questionnaires at baseline, 4 weeks, 6 and 12 months after training. First results show statistically significant improvement of knowledge and health-related quality of life.

Cognitive Behavioral Therapy↗
PubMed2005

[Jaccoud arthritis in systemic lupus erythematosus].

Jaccoud arthritis is a manifestation of systemic lupus erythematosus and other rheumatic diseases. It is characterized by non-erosive subluxation and synovialitis ultimately leading to severe deformity of the hands. The clinical aspect may be misleading and suggestive of rheumatoid arthritis manifestations.

Adult↗
PubMed2005

[Glucocorticoids: importance in the treatment of vasculitis].

Only the modification of natural steroids in the middle of the last century gave insights into the structural requirements for the biological activity of the glucocorticoids (GC). While the delta-4,3-keto-11-beta, 17-alpha,21-trihydroxyl configuration is needed for the GC-activity, an artificial additional double binding in position 1 and 2 lead to a four fold increase of the GC-activity. Of the artificial GC, prednisolone is the most frequently used compound and essential in the therapy of vasculitis today. Dosage, duration and way of application depend on the diagnosis, disease stage, -extend as well as -activity. Considering the use and side-effects of the GC, experiences from cohort-studies of the late 80-ties help at clinical decision making. For giant cell arteritis (GCA) it was shown, that doses of less then 60 mg/day are needed for the induction of remission. Concerning the visual loss in GCA, time of initiating GC-therapy seems more important than the dosage. In the treatment of ANCA-associated vasculitis therapy with GC, later in combination with cyclophosphamide, lead to a significant reduction of mortality. Due to the fact of an increasing survival rate, therapy-related morbidity becomes a more and more important issue. There is a proven correlation between the dosage respectively duration of the GC-therapy and the risk of GC-associated side-effects, especially the incidence of severe infections. This article gives a short review of the present data of the role of GC in the treatment of vasculitis.

Anti-Inflammatory Agents↗
PubMed2005

[Early diagnosis of vasculitides].

The often unspecific symptoms like myalgias, fever and weight loss at the onset of vasculitides are a frequent cause for a delay in diagnosis. Organ-specific symptoms like hemoptysis, dyspnoea, epistaxis, edema and organ infarcts a present when organ dysfunction occurs as a result of vasculitis. Targeted serologic testing including antineutrophil cytoplasm antibodies (ANCA) and cryoglobulins allows early diagnosis of certain vasculitides. Modern imaging techniques like magnetic resonance imaging, computed tomography, positron-emission tomography and ultrasound are cornerstones for an early diagnosis as they allow the detection of subclinical disease and are helpful in the identification of a site for biopsy. Bioptic proof of vasculitis is still the gold standard for diagnosis. Functionally relevant damage caused by systemic inflammatory disorders can by reduced or sometimes avoided by early initiation of treatment. This requires a correct diagnosis is made as early as possible.

Diagnosis, Differential↗
PubMed2005

Difficult to diagnose manifestations of vasculitis: does an interdisciplinary approach help?

In the early stages of disease, primary systemic vasculitides often present with non-specific symptoms that make early diagnosis a challenge. The variety of clinical manifestations found in systemic vasculitis is huge, and some manifestations are frequently not clinically overt at first presentation. A logical implication of the often non-specific and sometimes subclinical presentation of vasculitis is that a systematic diagnostic work-up is necessary. This requires a multidisciplinary approach involving the expert opinion of specialists from many disciplines, such as neurology, radiology, respiratory medicine, pathology and microbiology. There are no generally accepted diagnostic criteria for primary systemic vasculitides, and the application of classification criteria as diagnostic criteria is not feasible and may even be misleading. The demonstration of vasculitis on biopsy is still the gold standard for the diagnosis of vasculitis. In cases where biopsies cannot be obtained, surrogate parameters of vasculitis (e.g. glomerular hematuria or mononeuritis multiplex), along with serology and imaging, can support a clinical diagnosis of vasculitis. This review discusses the approach to the diagnosis of central nervous system and pulmonary manifestations of primary systemic vasculitis. These two examples of difficult to diagnose manifestations of vasculitis illustrate the necessity of an interdisciplinary approach to the diagnostic work-up.

Diagnosis, Differential↗
PubMed2005

Association study of Wegener granulomatosis and the functionally relevant A645G polymorphism in the bactericidal/permeability increasing protein (BPI) gene.

In antineutrophil cytoplasmatic antibody (ANCA)-associated vasculitides (AAV), bactericidal/permeability increasing protein (BPI) ANCAs are detected. Recent observations suggest that BPI-ANCAs can potentially contribute to a proinflammatory setting in the absence of proteinase 3 (PRTN3) ANCAs during the development of a pulmonary relapse by impeding the elimination of Gram-negative bacteria (GNB). However, it is as yet not clear whether the genetic background contributes to the generation of BPI-ANCAs in Wegener granulomatosis (WG) or if BPI polymorphisms are associated with WG. In this study we genotyped the functionally relevant single nucleotide polymorphism (SNP) A645 (Glu216Lys) of the BPI gene in 201 WG patients and 608 healthy controls. To investigate whether further SNPs might be associated with WG, we also examined an intragenic microsatellite marker. No significant differences were found between patients and controls. Thus BPI polymorphisms do not appear to contribute to genetic predisposition to WG. Moreover, our data do not suggest a genetic background for the generation of BPI-ANCAs in WG.

Antibodies, Antineutrophil Cytoplasmic↗
PubMed2005

Proinflammatory cytokines and autoimmunity in Churg-Strauss syndrome.

Churg-Strauss syndrome (CSS) belongs to the antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides and is further characterized by severe eosinophilia and, often, granulomatous inflammation. The therapeutic efficacy of recombinant interferon-alpha (IFN-alpha) and tumor necrosis factor-alpha (TNF-alpha) blockade point toward a central role of cytokines in the pathogenesis of CSS. Recent data show that, in contrast to other primary systemic vasculitides, peripheral blood mononuclear cells (PBMCs) secrete not only large amounts of T helper type 1 (Th1) cytokines, particularly IFN-gamma, but also release T helper type 2 (Th2) cytokines such as interleukin-4 (IL-4) and interleukin-13 (IL-13). Interleukin-5 is the most potent stimulator of eosinophil production and functional activation of mature eosinophils, the key effector cells in CSS. Data are presented showing that PBMCs from patients with CSS cultured with T cell-specific stimuli secrete significantly increased amounts of IL-5 compared with healthy controls, suggesting that IL-5 contributes substantially to the development of eosinophilia in CSS. As recombinant IFN-alpha downregulates IL-5 production of CD4(+) T cells in vitro, the increased secretion of IL-5 in patients with CSS may provide the clue for the therapeutic efficacy of recombinant IFN-alpha in the disease. Variations in the balance between Th1 and Th2 cytokines at different disease stages could contribute to the distinct clinical courses seen in patients with CSS, which can range from prominent Th1-mediated generalized vasculitis and granulomatous inflammation on one end of the spectrum to Th2-mediated systemic hypereosinophilia on the other. Although the association of ANCAs with CSS point toward an autoimmune origin of the disease, there is no direct evidence as yet for a direct pathogenic role of ANCAs in CSS.

Antibodies, Antineutrophil Cytoplasmic↗
PubMed2004

[Trypanosomiasis in a woman from Cameroon mimicking systemic lupus erythematosus].

HISTORY: A 27-year-old woman from Cameroon was admitted because of arthralgia, myalgia and severe thrombocytopenia (20,000/ micro l). She had been suffering from weakness, recurrent febrile episodes, generalized lymphadenopathy and pancytopenia for 2 years. Having a typical autoantibody constellation and fulfilling four (pleurisy, autoimmune-hemolytic anemia, antinuclear antibodies (ANA), anti-Sm antibodies) of the American College of Rheumatology (ACR) classification criteria, systemic lupus erythematosus (SLE) had been diagnosed at another hospital. Treatment with corticosteroids and azathioprine did not improve her condition. INVESTIGATIONS: Abnormal laboratory findings were pancytopenia, elevated markers of inflammation and extreme hypergammaglobulinemia (70 %) with polyclonal IgM (73 g/l). Antinuclear antibodies (ANA), anti-Sm-, anti-Scl 70-, anti-U1-RNP-, anti-histo-, anti-leukocyte- and IgM anticardiolipin antibodies were detected. A bone marrow biopsy showed polyclonal B-cell and plasma cell infiltrates. Examination of peripheral blood smears disclosed trypanosoma brucei infection. DIAGNOSIS, TREATMENT AND COURSE: After the diagnosis of stage 2 West African trypanosomiasis (sleeping sickness) specific treatment was initiated leading to subsequent remission of the disease. CONCLUSION: This case report underlines the importance of a thorough differential diagnosis in cases of suspected autoimmune disease. Induction of autoantibodies during infectious diseases may be misleading. The use of the ACR criteria for SLE must be restricted to the classification of proven connective tissue diseases.

Adult↗
PubMed2004

Maintenance of remission with leflunomide in Wegener's granulomatosis.

OBJECTIVE: To investigate the safety and efficacy of leflunomide plus low-dose prednisolone for the maintenance of remission in Wegener's granulomatosis (WG). METHODS: This was a Phase II, single-centre, open-label clinical investigation of patients with generalized WG treated with leflunomide after the induction of complete (n = 4) or partial (n = 16) remission by cyclophosphamide/prednisolone combination therapy. Leflunomide treatment was initiated at 20 mg/day and increased to 30 mg/day after 12 weeks and, in patients with partial remission, to 40 mg/day after 24 weeks. Concomitant low-dose prednisolone (< or =10 mg/day) was allowed during the study. In addition to the frequency of relapse, treatment efficacy was assessed by the standard measures of disease activity/extent. RESULTS: A total of 20 patients were enrolled in the trial. During a treatment period of up to 2.5 yr (median 1.75 yr, range 1-2.5 yr), one patient had a major relapse and required retreatment with cyclophosphamide/prednisolone. Eight patients had minor relapses that were successfully treated by dose increases to 40 mg/day leflunomide. Disease activity remained unchanged for the duration of the study. The most frequently reported adverse events were mild respiratory infection (40%), arthralgia (35%) and hypertension (35%); dry skin, nail disorder and diarrhoea were each reported by 30% of patients. Despite the aggressive pretreatment with cyclophosphamide, adverse events with leflunomide treatment at the higher dose (30-40 mg/day) were comparable with those seen with the standard dose (20 mg/day) for rheumatoid arthritis patients. CONCLUSION: Leflunomide appears to be safe and well tolerated for the maintenance of complete or partial remission of WG. The results of this pilot study encourage further controlled trials comparing leflunomide with alternative remission maintenance therapies.

Adult↗