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E Reinhold-Keller

Publications and source records attributed to E Reinhold-Keller.

At least 19 recordsLinked to original sources
PubMed2006

[Imaging procedures in rheumatology: imaging in vasculitis].

In small vessel vasculitides, imaging studies aid in determining disease extent and activity, localization for biopsy, and for disease monitoring. They do not directly delineate the vasculitic lesion. Imaging studies focus on the upper and lower respiratory tract. Cranial magnetic resonance imaging (MRI) shows upper respiratory and retrobulbar granuloma in Wegener's granulomatosis. Furthermore, MRI depicts both mastoiditis and mucosal inflammation of the ear, nose, and throat. It is sensitive but not specific for the detection of cerebral vasculitis. Computed tomography (CT) reliably detects osseous facial lesions. Chest radiography in two planes remains the standard method of investigation for the lower respiratory tract. High-resolution CT aids in detecting further interstitial pathologies. Medium-sized vasculitides frequently occur with aneurysms. The classification criteria for polyarteritis nodosa involve the angiographic detection of visceral aneurysms. Patients with Kawasaki disease may develop coronary aneurysms that may be described by echocardiography or angiography according to diagnostic criteria. In large-vessel vasculitides such as temporal arteritis (giant cell arteritis) and Takayasu arteritis, MRI, MR-angiography, CT, CT-angiography, and duplex sonography delineate characteristic homogenous wall thickening with or without stenoses in the aorta and other arteries. There is a high correlation with angiography and positron emission tomography. Duplex sonography of the temporal arteries has a high sensitivity and specificity for the diagnosis. Data on temporal artery MRI in giant cell arteritis have recently been published.

Aneurysm↗
PubMed2005

[Symmetric polyarthritis in a patient with Churg-Strauss syndrome].

HISTORY: A 63-year-old man developed a Churg-Strauss syndrome with predominantly motor-sensory polyneuropathy. Initial treatment with cyclophosphamide and steroids achieved complete remission. Subsequent relapse with marked retinal vasculitis at first was refractory to the standard treatment. Renewed remission was obtained with additional infliximab, and was maintained with azathioprine for 12 months before the patient again presented with symmetrical polyarthritis. INVESTIGATIONS: Clinical examination revealed a symmetrical polyarthritis involving the joints of the hand and fingers. The acute-phase parameters were raised, the rheumafactor was highly positive. Radiology showed early erosions in the bones of the hand. There were no indices of renewed activity of the Churg-Strauss syndrome. TREATMENT AND COURSE: These findings indicated sero-positive rheumatoid arthritis and methotrexate was started, later supplemented with sulfsalazine and hydroxychloroquine because of continuing signs of activity. Infliximab was again given because of further progression, but a severe infusion reaction developed during the second infusion. After changing to etanercept remission of the rheumatoid arthritis was achieved. CONCLUSION: The development of rheumatoid arthritis during remission achieved with azathioprine in Churg-Strauss syndrome of four-year duration is very rare. Repeated dosis of infliximab at long intervals greatly increases the risk of an intolerance.

Antibodies, Monoclonal↗
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↗
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↗
PubMed2004

[Inflammatory aortic arch syndrome: contrast-enhanced, three-dimensional MR angiography in stenotic lesions].

PURPOSE: To determine the value of contrast-enhanced, three-dimensional MR angiography for the evaluation of stenotic and occlusive vascular lesions in inflammatory aortic arch syndrome. MATERIALS AND METHODS: 14 patients with inflammatory aortic arch syndrome (giant cell arteritis: n = 8, Takayasu arteritis: n = 4, ankylosing spondylitis: n = 1 sarcoidosis: n = 1) underwent MR angiography of the aortic arch and the supra-aortic vessels (n = 15, 2 patients were examined twice) and of the abdominal aorta (n = 2). MRA was performed using a 3D-FLASH sequence (TR/TE 4.6/1.8 ms, flip angle 30 (3)) on a 1.5T system. MRA imaging was compared with the findings of DSA, which served as gold standard. RESULTS: In a total of 467 examined vascular territories, DSA revealed 50 stenoses and 35 occlusions. All lesions were detected by MRA. In 23 segments, the degree of stenosis was overestimated by MRA. Sensitivity and specificity of MRA were 100 % and 94.3 %, positive and negative predictive values were 73.6 and 100 %, and the accuracy was 95.1 %. CONCLUSIONS: Despite a tendency to overestimate stenoses, contrast-enhanced three-dimensional MR angiography is a valid, non-invasive technique in the assessment of inflammatory aortic arch syndrome.

Adult↗
PubMed2003

Percutaneous management of occlusive arterial disease associated with vasculitis: a single center experience.

The purpose of this study was to evaluate the safety and effectiveness of percutaneous transluminal angioplasty for occlusive arterial disease associated with vasculitis. Eleven patients (10 women, 1 man; ages 35-82 years) with the diagnosis of vasculitis of the large vessels underwent interventional treatment during intraarterial angiography. The causes included giant cell arteritis (n = 8) and Takayasu arteritis (n = 3). Thirty-three occlusive lesions (including brachiocephalic and renal arteries, and arteries of upper and lower extremities) were treated with balloon angioplasty and/or stent placement. Follow-up included clinical examination, angiography, and color duplex ultrasound. Technical success was 100% (25/25) for stenoses and 50% (4/8) for occlusive lesions, representing all lesions combined from different anatomic locations. Dissection (n = 3) and arterial rupture with retroperitoneal hematoma (n = 1) was found in three patients. During follow-up (mean 12 months), restenoses (n = 8) and re-restenoses (n = 1) occurred in 8 vascular areas. Three of these lesions were treated with repeated PTA (n = 4). The cumulative primary clinical success rate was 67.6%, cumulative secondary success rate 74.4%, and cumulative tertiary success rate 75.9%. Interventional therapy in systemic vasculitis provides promising results in technical success rates and followup. Angioplasty may result in arterial injury, but the rate of complications is low.

Adult↗
PubMed2003

[A new patient education program: vasculitis].

Referring to the literature it is known that structured standardized patient education represents an effective additional treatment in patients with chronic diseases. Programs base 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.

Adaptation, Psychological↗
PubMed2003

[Radiology of the primary systemic vasculitides].

Determination of disease extension and disease activity are in the foreground of diagnostic imaging in vasculitides. There are several radiologic modalities available each having specific indications. Magnetic resonance imaging (MRI) readily depicts granulomas and mucosal inflammations in the paranasal sinuses, nasal cavity and orbits. Computed tomography detects osseous lesions of the skull. Due to its superb sensitivity MRI is an established screening modality for CNS vasculitides, although there are limitations with regard to specificity. In spite of its limited accuracy in most institutions angiography is still required for radiological confirmation of CNS vasculitis. Perfusion and diffusion MR-imaging may combine the advantages of "conventional" MRI and angiography. By now the method is not fully validated for vasculitides, however. Vascular disease in Takayasu's arteritis and in giant cell arteritis involving predominantly large and medium sized vessels is readily diagnosed by non invasive magnetic resonance angiography. Percutaneous transluminal angioplasty has proven to be an effective and save therapeutic modality for the cure of vascular stenoses and occlusions. Plain film radiography in two planes is the established modality for pulmonary imaging. In pulmonary vasculitides a more thorough analysis of lung disease is provided by high resolution computed tomography. Diagnostic imaging does substantially assist in the interdisciplinary management of patients suffering from vasculitides.

Adolescent↗
PubMed2002

[Scientifically based patient education exemplified by patients with primary systemic vasculitis. The vasculitis patient education program--development, contents and initial results of evaluation].

Referring to the literature, it is known that structured standardized patient education represents an effective additional treatment in patients with chronic diseases. Most programs are based on cognitive behavioral interventions. Within the last few 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 of the Medical University of Lübeck/Bad Bramstedt. The contents of the seminars were revised and condensed into five modules. New slides and handouts for patients were developed. To evaluate the new form of the program, a documentation system consisting of patient and physician-administered questionnaires assessing socioeconomic, knowledge and disease-related outcome-parameters was designed. Patients are trained in closed groups (n = 10-15) and asked to complete questionnaires at baseline, 4 weeks and 6 months after training. First results show statistically significant improvement of knowledge and health-related quality of life.

Antibodies, Antineutrophil Cytoplasmic↗
PubMed2002

No difference in the incidences of vasculitides between north and south Germany: first results of the German vasculitis register.

OBJECTIVE: To register all newly diagnosed patients with primary systemic vasculitis (PSV) in two large regions in north and south Germany. METHODS: Between 1 January 1998 and 31 December 1999, all newly diagnosed cases of PSV, as defined by the Chapel Hill Consensus Conference 1992, were identified in two large mixed rural/urban regions in north and south Germany with a combined population of 4,880,543, for a population-based prospective study. The following sources were used: (i) all departments of every hospital, including their out-patient clinics; (ii) all departments of pathology; and (iii) all reference immunological laboratories serving the catchment area. All cases were re-evaluated by the authors. RESULTS: Over the whole period, 473 individuals were registered as having a new PSV. The incidence rates for all PSV were 54 cases per 1,000,000 inhabitants in the north and 48 in the south in 1998, and 48 and 41 respectively in 1999. People 50 yr and older had a three- to five-fold higher risk of getting PSVs than those under 50 yr. The incidences of antineutrophil cytoplasmic antibody (ANCA)-associated PSVs [Wegener's granulomatosis (WG), microscopic polyangiitis (MPA) and Churg-Strauss syndrome (CSS)] remained stable in both regions, at about 9.5 per 1,000,000 annually. The incidence of WG was two to three times greater than those of MPA and CSS. There was no difference in incidence rates between north and south Germany. CONCLUSION: First results from a population-based vasculitis register serving nearly 5,000,000 inhabitants in north and south Germany revealed no regional differences in the incidence of all PSVs between north and south. The incidence rates of ANCA-associated PSVs, such as WG and MPA, were lower than those in the UK and Norway but higher than that in Spain.

Adolescent↗
PubMed2002

[Pathology of vasculitis: differential diagnosis and selected disorders].

The morphological diagnosis of vasculitis is a challenge for the pathologist for many reasons. Primary systemic vasculitides are rare diseases (ann. incidence 4-5/100,000 according to the Vaskulitis-Register Schleswig-Holstein), pathognomonic histopathological findings are hard to obtain in many patients even with multiple biopsies, and the nomenclature of vasculitis has long been confusing and unequivocal. The Chapel-Hill Consensus Conference (1992) is credited with the establishment of clinico-pathological definitions for primary systemic vasculitides. For the discrimination of primary systemic vasculitis from secondary vasculitis as well as pseudovasculitic syndromes, correlation with clinical data is essential; it has been facilitated by the American College of Rheumatology (ACR) classification criteria (1990). Differential diagnostic schemes for vasculitis are based on the predominant type and size of the blood vessel involved, as well as the histopathological type of inflammation and the presence or absence of immune complex deposits. However, differential diagnostic problems remain: even with the clarified nomenclature of Chapel-Hill, considerable overlap still occurs. A clinical diagnosis of vasculitis is now often established in an earlier disease stadium, in part of the cases facilitated by ANCA testing; the pathologist may therefore at first be confronted with a more subtle or abortive morphology, while in the course of disease, histopathology is modulated by immunosuppressive therapy. A number of immunopathogenic mechanisms prevailing in vasculitis can be verified with immunohistochemical and molecular biological methods, although it is frequently not known how they are initiated and perpetuated. ANCA-associated vasculitides serve as a good example to demonstrate the central role of morphology, its potentials but also its limitations in the differential diagnosis of vasculitis.

Diagnosis, Differential↗
PubMed2001

Standardized neurologic evaluations of 128 patients with Wegener granulomatosis.

OBJECTIVE: To assess the frequency and type of neurologic involvement in a cohort of patients with generalized Wegener granulomatosis (WG). PATIENTS AND METHODS: In a prospective analysis the clinical, electrophysiologic, radiological, and serologic data of 128 patients have been studied over a median observation period of 19 months (range, 1-60 months). RESULTS: Sixty-four patients (50%) revealed central or peripheral nervous system involvement. Peripheral neuropathy (PN) affected 56 patients, in 9 cases the central nervous system was involved, and in 6 cases the cranial nerves were involved. Thirty-one patients showed a distal symmetrical polyneuropathy, 25 a mononeuritis multiplex. Within the first 2 years of the disease course 47 of the 56 patients had developed their PN, sometimes as the initial symptom of WG. Patients with PN were significantly more often male (34 of 65 patients) than female (22 of 63 patients, P =.04), were significantly older at the onset of WG (median age, 53 vs 44 years; P =.001), had a significantly larger disease extent (P =.001), and had higher classic antineutrophil cytoplasmic antibody titers (P =.002) than neurologically unaffected patients. Response to immunosuppression was moderate concerning peripheral nervous system manifestations. CONCLUSIONS: Peripheral neuropathy is frequent in generalized WG, occurring early in the disease course. As PN can be the first and sole symptom of a beginning systemic vasculitis, it is important that in cases of PN of an unclear origin, interdisciplinary investigations are initiated to detect, treat, and closely follow-up a possible underlying WG, especially as these patients seem to have a more severe disease course.

Adolescent↗
PubMed2001

Improved differentiation between Churg-Strauss syndrome and Wegener's granulomatosis by an artificial neural network.

OBJECTIVE: To examine the operating characteristics of the American College of Rheumatology (ACR) classification criteria for Churg-Strauss syndrome (CSS) and Wegener's granulomatosis (WG), and to develop and validate improved criteria for distinguishing CSS from WG. METHODS: The ACR classification criteria for WG and CSS were applied to 40 consecutive CSS patients age- and sex-matched with 40 patients with WG. Forty-three clinical, laboratory, and biopsy parameters were assessed. Artificial neural networks (ANNs) were trained and tested with all 43 parameters (set A) and with 15 solely clinical parameters documented at the initial manifestation of the disease (set B). The ANNs were trained with data from the first 27 CSS and 27 WG patients and validated with data from the next 13 consecutive CSS and 13 WG patients. To compare the ANNs with established methods, traditional format and classification tree criteria were generated using the same data sets. RESULTS: Fourteen of 40 CSS patients fulfilled the ACR criteria for WG, while 4 WG patients met the ACR criteria for CSS. The ANN, in contrast, reliably distinguished all CSS cases from WG cases (parameter set A, accuracy 100%). For parameter set B, the ANN achieved an accuracy of 100% in the training phase and 96% for validation. The newly formulated traditional format and classification tree criteria reached an accuracy of 81% and 88%, respectively. CONCLUSION: The ACR criteria for WG do not reliably differentiate between CSS and WG (specificity 65%). An ANN, however, could be trained to correctly allocate all but 1 patient on the basis of clinical data. Indeed, the ANN applied in this study proved superior to established methods of classification. We suggest that an ANN may be effectively applied in the classification of systemic vasculitides.

Churg-Strauss Syndrome↗
PubMed2001

[Interdisciplinary management of vasculitis patients: internist/rheumatologist].

Systemic vasculitides (SV) represent a heterogeneous group of different entities with varying clinical and pathological-anatomical characteristics that physicians of diverse disciplines are involved in the treatment of patients with SV. At the onset of disease organ manifestations often present as a single symptom without appearance of indirect signs of vasculitides as musculoskeletal complaints and constitutional symptoms indicating inflammatory systemic disease. Therefore early interdisciplinary care is extremely important to avoid major organ involvement with the development of fatal disease. Besides the multidisciplinary physical examination serological and immunological parameters, particularly in small vessel vasculitides are relevant in establishing the diagnosis. Regarding the interdisciplinary care we differentiate between primary diagnostic procedures and continuous follow-up to observe therapeutic and side effects of medications. Instruments for the assessment of disease extent (DEI), activity (BVAS) and irreversible damage (VDI) were developed in recent years to document prospectively the disease status and support activity-adjusted treatment. Because of the chronic relapsing character of systemic vasculitides, the measurement of health-related quality of life gained progressive interest in the longitudinal follow-up. In addition in these rare diseases early patient education with information on the disease, treatment, side effects and training in self management strategies will enable patients to actively participate in the management of their disease and bear responsibility.

Diagnosis, Differential↗
PubMed2001

Development and validation of a disease extent index for Wegener's granulomatosis.

AIMS: The quantitative assessment of disease extent (staging) and activity (grading) in patients with ANCA-associated vasculitides is one prerequisite for the comparison of outcomes in different patient cohorts. We present a validation study of the Disease Extent Index (DEI). METHODS: 66 patients with Wegener's granulomatosis were examined to assess the validity, reliability and sensitivity to change of the DEI. Correlation coefficients were calculated to estimate associations between DEI and BVAS (Birmingham Vasculitis Activity Score), a previously established activity score, and between DEI and serological markers. RESULTS: Among patients with active disease, DEI correlated significantly with cANCA titer (r = 0.46), leukocyte count (r = 0.38) and platelet count (r = 0.53). Among patients in remission, DEI correlated significantly with cANCA titer (r = 0.61), CRP (r = 0.47) and sIL2R (r = 0.47). Additionally, a high association of DEI and BVAS (r = 0.9) confirmed a rather high convergent validity. The effect size of DEI equals 2.37 standard deviation units and indicates a considerable change from active disease to remission. CONCLUSIONS: The DEI is valid, reliable, easy, quick to perform and highly reproducible. Although it correlates with some surrogate markers of disease activity and the BVAS, the DEI quantifies different domains of the disease than the BVAS and should therefore be used in conjunction with the BVAS.

Female↗