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Biomedical subjects

L Kater

Publications and source records attributed to L Kater.

At least 37 records · Page 2Linked to original sources

Anti-phospholipid antibodies and vascular pathology.

Over the past decade it has been recognized that there is an association between the presence of anti-phospholipid antibodies (lupus anticoagulant, anti-cardiolipin antibodies) and thromboembolic phenomena. This "anti-phospholipid syndrome", which was originally described in patients with lupus erythematosus also occurs in patients without an underlying systemic disease. An answer to the question whether anti-phospholipid antibodies cause thrombosis warrants further studies. Especially the recent findings on a role of plasma proteins in the binding of these antibodies to phospholipids will have to be taken into account. To obtain guidelines for optimal treatment of patients suffering from this syndrome, additional studies will have to be performed.

Antibodies, Antiphospholipid↗

Hydroxychloroquine treatment for primary Sjögren's syndrome: a two year double blind crossover trial.

OBJECTIVES: In 1985 and 1988 a positive effect of treatment of primary Sjögren's syndrome with hydroxychloroquine was reported in two small open studies. To investigate further the clinical and laboratory effects of hydroxychloroquine in primary Sjögren's syndrome a two year study was performed. METHODS: The design of the study included a prospective, placebo controlled, two year double blind crossover trial in 19 patients. RESULTS: A significant decrease in IgG and IgM and a tendency for a decrease in the erythrocyte sedimentation rate (ESR) during treatment with hydroxychloroquine compared with treatment with placebo were found. No beneficial clinical effect of the use of hydroxychloroquine as expressed in preference for treatment with hydroxychloroquine or placebo with regard to symptoms and signs of primary Sjögren's syndrome could be shown, however, nor any relevant change in tear gland activity and sequelae of peripheral tear function deficiency, nor salivary gland scintigraphy. CONCLUSIONS: The use of hydroxychloroquine at a dose of 400 mg daily taken over a 12 month period does not have a worthwhile clinical benefit in patients with primary Sjögren's syndrome despite an improvement of hyperglobulinaemia and slight changes in the ESR and IgM.

Adult↗

Patients with antiphospholipid antibodies and venous thrombosis should receive long term anticoagulant treatment.

OBJECTIVE: To determine whether the finding of antiphospholipid antibodies in patients with venous thromboembolic episodes should influence the duration of treatment with anticoagulant drugs by mouth. METHODS: A retrospective study was carried out in 19 patients with antiphospholipid antibodies and a history of venous thromboembolic episodes. The median follow up from the first venous thromboembolic episode was 93 months and the median age at this episode was 26 years. The patients had in total 34 venous thromboembolic episodes. The total follow up period comprised 32 periods with and 23 periods without anticoagulant drugs. RESULTS: The probability of being free of recurrent venous thromboembolic episodes, calculated by the Kaplan-Meier method, was significantly influenced by the use of anticoagulant drugs. Patients receiving oral anticoagulants had at eight years a 100% probability of survival without recurrence, whereas patients in whom anticoagulant drugs were stopped had a 50% probability of a recurrent venous thromboembolic episode at two years, and a 78% probability of recurrence at eight years. CONCLUSION: Patients with venous thromboembolic episodes and antiphospholipid antibodies have a high risk for recurrent venous thromboembolic episodes and long term treatment with anticoagulant drugs by mouth is an effective prophylaxis.

Adolescent↗

Monotypic plasma cells in labial salivary glands of patients with Sjögren's syndrome: prognosticator for systemic lymphoproliferative disease.

AIMS: To determine the prevalence of plasma cell monotypia in labial salivary gland tissue of patients with and without Sjögren's syndrome, and to evaluate its relation to the development of systemic monoclonal lymphoproliferative disorders. METHODS: A quantitative immunohistological study was performed on labial salivary gland tissue of 45 patients with Sjögren's syndrome, 18 with rheumatoid arthritis without Sjögren's syndrome, and 80 healthy controls. In none of the patients with Sjögren's syndrome was there evidence of systemic monoclonal lymphoproliferative disease at the time of biopsy. RESULTS: Monotypic plasma cell populations, defined by a kappa:lambda ratio of > or = 3, were only observed in older patients (above 43 years) with Sjögren's syndrome. In almost all these patients monotypic plasma cell populations were present in multiple labial salivary gland tissues and the IgM/kappa monotypia was observed most frequently. The prevalence of monotypic plasma cell populations in the group with Sjögren's syndrome was 22% (10/45) and there was no significant predilection for primary Sjögren's syndrome. Of special clinical interest was the observation that progression to systemic monoclonal lymphoproliferative disease had occurred exclusively in this subgroup of patients with Sjögren's syndrome, with a prevalence of 30% (3/10). CONCLUSION: Quantitative immunohistological examination of labial salivary gland tissues provides pathologists with a simple method to select those patients with Sjögren's syndrome who have an increased relative risk at the time of biopsy to develop benign or malignant lymphoproliferative disorders.

Adolescent↗

Polyarteritis nodosa in a patient with AIDS.

A 33-year-old male homosexual with the acquired immunodeficiency syndrome (AIDS) presented with pain, blue colouring and ulceration of fingers and toes. Angiography of the superior mesenteric artery and renal arteries showed vessel wall irregularities and aneurysms, characteristic of polyarteritis nodosa (PAN). We describe this patient because of the possible association between systemic vasculitis and AIDS.

Acquired Immunodeficiency Syndrome↗

Quantitative immunohistologic criteria are superior to the lymphocytic focus score criterion for the diagnosis of Sjögren's syndrome.

OBJECTIVE: The aim of the present study was to evaluate the diagnostic value of quantitative immunohistologic criteria for Sjögren's syndrome (SS) in labial salivary gland biopsies. METHODS: Quantitative immunohistologic examination was performed on labial salivary gland biopsy samples from 80 healthy controls, 32 patients with primary SS, 14 patients with secondary SS, 5 with "probable" SS, 36 with keratoconjunctivitis sicca (KCS) with a lymphocytic focus score less than 1 on the lip biopsy, and 18 with rheumatoid arthritis (RA) without clinical evidence of SS. RESULTS: This is the first study to show that immunohistologic criteria for SS, based on the percentages of IgA-containing and IgG-containing plasma cells, are able to 1) confirm the diagnosis of SS in labial salivary glands of KCS patients in the absence of grade IV lymphocytic adenitis; and 2) distinguish between a grade IV focal lymphocytic adenitis in the labial salivary glands of SS patients and of RA patients without SS. CONCLUSION: Quantitative immunohistologic criteria were shown to be much more sensitive and disease specific than the widely accepted grade IV lymphocytic adenitis criterion, which corresponds to a lymphocytic focus score greater than 1, and these criteria should be included in the international diagnostic criteria for Sjögren's syndrome.

Adolescent↗

Quantitative immunohistologic study of lip biopsies. Evaluation of diagnostic and prognostic value in Sjögren's syndrome.

In a group of 45 patients with Sjögren's syndrome (SS) and 80 controls the high specificity (95%) and sensitivity (100%) of a recently proposed bivariate quantitative immunohistologic (QIH) criterion for SS, based on percentages of IgA and IgG-containing plasma cells in labial salivary gland (LSG) tissue, was confirmed. The best univariate QIH criterion for discrimination between LSG biopsies of SS patients and controls appeared to be based on the percentage of IgA containing plasma cells, and had a specificity of 99% and a sensitivity of 96%. A criterion based only on the percentages of IgM-containing plasma cells, proposed in another recent study, resulted in a high number (31%) of false negatives. Interobserver reproducibility of QIH diagnoses was excellent. Moreover it was demonstrated that accuracy, precision and the interobserver reproducibility of plasma cell counting depends on the choice of tissue fixation and immunohistologic staining procedure. The combination of formol sublimate fixation and peroxidase anti-peroxidase procedure appeared to be the best combination for QIH examination. Furthermore, in 2 SS patients systemic monoclonal IgM/kappa gammopathy was preceded by high predominance of IgM and kappa containing plasma cells in the plasma cellular infiltrate of the LSG tissue.

Biopsy↗

Immunofluorescence microscopy of healthy skin from patients with systemic lupus erythematosus: more than just the lupus band.

Many papers have been published on the lupus band in systemic lupus erythematosus (SLE), but little information exists on the possible diagnostic value of the lupus band and other microscopic immunofluorescence phenomena found in clinically normal skin of patients with SLE. In a study of 297 subjects (66 patients with SLE, 81 patients with other forms of LE, and 150 patients with other systemic connective tissue disorders) it was found that: (a) granular deposits of IgA, IgG, and IgM in the basal membrane zone and in the deeper blood vessels were more common in patients with SLE than in the other two groups; (b) depending on the clinical differential diagnosis, IgA and IgG deposits at the epidermal basal membrane can be specific for SLE; (c) using logistic regression analysis sets of variables can be selected with a high potential to discriminate between SLE and the other groups; and (d) immunofluorescence variables do not duplicate the information for the diagnosis of SLE given by the American Rheumatism Association (ARA) criteria or other laboratory methods. From these results, it is concluded that immunofluorescence microscopy of clinically normal skin is a valuable diagnostic method which should be reconsidered as a potential criterion for the diagnosis of SLE in the next evaluation of the ARA criteria.

Adolescent↗

The long-term clinical outcome of 56 patients with biopsy-proven lupus nephritis followed at a single center.

We retrospectively evaluated the clinical outcome of 45 female and 11 male patients with biopsy-proven lupus nephritis, followed at our hospital between February 1974 and February 1990. In the majority signs of nephritis were present at the time systemic lupus erythematosus was diagnosed (range: -42-156 months) and the median time from onset of nephritis to biopsy was 2 months. The median follow-up from the time of the biopsy was 53.5 months (range: 2-192), the median age at biopsy 25 years and the median serum creatinine level 1.2 mg/dl. Initial renal biopsies had the following histopathological classes according to the World Health Organization criteria (n): I (2); II (10); III (10); IV (28); V (5); VI (1). Over the study period active episodes were treated with high-dose oral prednisone alone or combined with intravenous nitrogen mustard and oral chlorambucil (1974-75), azathioprine (1978-86), cyclophosphamide (1986-90) and/or plasma-exchange (1976-84). These strategies were based on literature data or multicenter studies in which we participated. Eight patients developed end-stage renal disease (ESRD) (median: 47 months post-biopsy; range: 20-120). In these, initial biopsies showed class IV in seven, and class V in one. Confounded risk factors for ESRD were class IV biopsy, male gender and serum creatinine level above 1.4 mg/dl. The calculated proportion without ESRD 5 years post-biopsy was 87% (95% confidence limits: 98-76%), and at 10 years 70% (95% confidence limits: 90-49%). Five patients (11.2%) died; causes of death were cerebrovascular accident (n = 2), cerebral lupus (n = 2) and S. aureus sepsis (n = 1).(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

New developments in Sjögren's syndrome.

Sjögren's syndrome is an autoimmune exocrinopathy with characteristics of both organ-specific and generalized autoimmune disease. The main features are keratoconjunctivitis sicca and xerostomia, but patients may also show signs of extraglandular involvement. Sjögren's syndrome can occur in the absence (primary Sjögren's syndrome) or in the presence of other connective tissue disease (secondary Sjögren's syndrome). Sjögren's syndrome has often been considered a link in the spectrum between autoimmune disease and lymphoproliferative disorders. The cause of Sjögren's syndrome is still unknown, although the role of viruses in the pathogenesis is disputed. There is still no consensus about the diagnostic criteria, due to lack of specificity and sensitivity of the diagnostic procedures performed thus far. Recent studies using immunohistochemical and molecular biologic techniques highly suggest that disturbances in the target organs, such as salivary glands, are the consequence of local processes, rather than the reflection of systemic alterations. This underlines the importance of implementing these techniques for diagnostic purposes. Based on increasing understanding of the pathogenetic processes, new therapeutic possibilities will come into the picture. Over the past few years, interesting studies have contributed to our knowledge of the disease.

Antibodies, Antinuclear↗

Neuro-musculo-skeletal manifestations in primary Sjögren's syndrome.

Primary Sjögren's syndrome is a systemic autoimmune disorder whose main characteristics are dryness of the eyes and mouth, caused by lymphocytic infiltration of the exocrine glands. Patients may also show signs of extraglandular involvement of lung, liver, kidney and vessel walls, as well as of the central and peripheral nervous systems, muscles and joints. This article presents a review of the literature on extraglandular involvement of the peripheral and central nervous systems, muscles and joints. Several data support the hypothesis that vasculitis is the underlying mechanism. The need for an extended inventory of the extraglandular manifestations, preferentially linked to immunoserological and -histological investigations to gain more insight into the aetiology and pathogenesis is stressed. As far as the clinical picture is concerned, myalgia and arthralgia are often reported, but myositis and arthritis are rare. Data about the prevalence of peripheral and central nervous system involvement are conflicting: factors contributing to these differences are discussed. As insight into prognosis and therapy will strongly depend on the diagnostic criteria used, the need for international agreement on these is emphasized.

Humans↗

Labial salivary gland biopsy in Sjögren's syndrome.

Labial salivary gland histopathology has long been considered to be the most disease-specific single test for diagnosis of Sjögren's syndrome. However, the diagnostic yield of widely used grading systems of focal lymphocytic sialoadenitis is rather low. Determination of the percentages IgA-, IgG-, IgM-, kappa-, and lambda-containing plasma cells in minor salivary glands obtained from a lower lip biopsy has recently been shown to be extremely valuable in Sjögren's syndrome, not only for diagnosis, but also for prognosis with regard to the development of systemic monoclonal lymphoproliferative disease. This technique greatly improves the value of labial salivary gland biopsy in Sjögren's syndrome.

Biopsy↗

Treatment of systemic lupus erythematosus: which options do we have for therapy regimens?

The prognosis of systemic lupus erythematosus has improved markedly. This has been due to various factors: improved serological testing leading to better diagnosis, better understanding of secondary complications, and the possibility of treating these. How much has improved treatment of the primary disease process contributed to the improvement in prognosis? We have evaluated the clinical outcome of 56 patients with lupus nephritis proven by biopsy, followed at out hospital over the past 16 years. During this period various therapies were used during active periods of the disease, based on literature data or participation in trials. Prognostic risk factors for the development of end stage renal disease (ESRD) appeared to be: WHO-class IV histopathology of the renal biopsies, male sex and raised serum creatinine. Development of ESRD at 5 years was 13% and at 10 years was 30%. Overall survival was 95%. Based on data from well controlled trials performed at the National Institute of Health (US) and our observations the need for well conducted long-term prospective randomized trials is stressed again.

Humans↗

Influence of fixation and immunohistological technique on accuracy, precision and inter-observer reproducibility of plasma cell counting.

Recently two highly sensitive and specific diagnostic criteria for Sjögren's syndrome based on percentages of IgA-, IgG-, and IgM-containing plasma cells measured in immunohistologically stained labial salivary gland tissue have been described. The reliability of such a criterion is dependent on the accuracy, precision and inter-observer reproducibility in plasma cell counting. The present study evaluates the effect of tissue fixation and immunohistological procedures on the aforementioned factors. Immunoglobulin (Ig)-containing plasma cells in sections of lamellated submandibular salivary gland tissue, alternately fixed in a 4% buffered formol solution or formol-sublimate solution and stained with an indirect immunoperoxidase and unlabelled peroxidase anti-peroxidase (PAP) method respectively, were enumerated by three independent observers. Relative numbers of Ig-containing plasma cells appeared to be less sensitive for systematic errors due to tissue fixation and immunohistological procedure than absolute numbers of Ig-containing plasma cells. The best inter-observer reproducibility of plasma cell counts was obtained in sections from formol sublimate-fixed specimens stained according to the PAP procedure.

Analysis of Variance↗

Diagnostic and prognostic value of quantitative immunohistological examination of lip biopsy in Sjögren's syndrome.

Three patients are presented to illustrate the diagnostic and prognostic value of quantitative immunohistological examination of labial salivary gland biopsy in Sjögren's syndrome. In an obvious case of primary Sjögren's syndrome and in a case of rheumatoid arthritis without clinical and serological evidence of secondary Sjögren's syndrome, quantitative immunohistological examination of the labial salivary gland biopsy gave more appropriate information than the widely accepted lymphocytic focus score criterion for Sjögren's syndrome. In the third case quantitative immunohistological examination disclosed monoclonal B-cell expansion in the labial salivary gland biopsy six months before evidence of systemic monoclonal gammopathy appeared.

Adult↗