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Biomedical subjects

E Röther

Publications and source records attributed to E Röther.

At least 19 recordsLinked to original sources
PubMed1998

Diagnosis of idiopathic myositis: value of 99mtechnetium pyrophosphate muscle scintigraphy and magnetic resonance imaging in targeted muscle biopsy.

Our objective was to study the value of 99mtechnetium-pyrophosphate (99mTc-PYP) muscle scintigraphy and magnetic resonance imaging (MRI) in detecting areas of likely muscle inflammation and in increasing the rate of positive muscle biopsies in patients with suspected myositis. The results showed that in 13 out of 13 patients with clinical and/or signs of inflammatory muscle disease, increased 99mTc-PYP uptake was demonstrated at different muscle sites 3 h after isotope injection. Subsequent MRI of symmetric muscle areas with enhanced 99mTc-PYP uptake revealed signal patterns suggesting inflammation in all cases. Biopsy of these targeted muscles demonstrated characteristic histopathologic signs of muscle inflammation in 9 out of 13 patients. Four of these 9 patients had clinically atypical disease or did not show elevated creatine phosphokinase levels. Seven of these 9 patients had not been pretreated with corticosteroids. In 4 patients only muscle fiber atrophy and/or necrosis without cellular infiltrations was seen. These 4 patients had received either high doses of corticosteroids or low doses over longer periods of time before muscle biopsy. In conclusion, the combination of 99mTc-PYP muscle scintigraphy and MRI demonstrated muscle areas with maximum inflammatory signal patterns. Targeting of muscles by MRI only will probably yield reliable results of muscle biopsy in cases of clinically and serologically characteristic myositis. 99mTc-PYP muscle scintigraphy may provide useful initial information about localization of inflamed muscle tissue, especially in atypical disease. Treatment with corticosteroids prior to histologic diagnosis may abolish inflammatory infiltrations in affected muscle tissue.

Adolescent↗
PubMed1997

Elevated anticardiolipin antibodies in autoimmune haemolytic anaemia irrespective of underlying systemic lupus erythematosus.

In patients with systemic lupus erythematosus (SLE) and concomitant Coombs positive autoimmune haemolytic anaemia (AIHA) anticardiolipin antibodies (aCL) are found more frequently and at higher titres than in SLE patients without AIHA. In order to assess if aCL elevation is primarily associated with the underlying SLE, or with AIHA itself, we examined AIHA patients with and without underlying SLE for the presence of aCL. aCL (IgG and IgM) were determined by ELISA in 74 SLE patients without AIHA, 22 SLE patients with AIHA, 50 patients with idiopathic AIHA (warm-reactive autoantibodies), 52 patients with idiopathic AIHA (cold-reactive autoantibodies) and 50 healthy controls. The mean IgG and IgM aCL titres in SLE patients without AIHA (IgG 37.0 U/ml, IgM 8.9 U/ml) were significantly elevated compared with the values in healthy controls (IgG 9.1 U/ml, IgM 3.2 U/ml; P < 0.005). The mean aCL levels in SLE patients with AIHA (IgG 53.2 U/ml, IgM 28.2 U/ml) were higher than in SLE patients without AIHA (P = 0.09 for IgG, P < 0.005 for IgM). Interestingly, the mean aCL levels of patients with idiopathic AIHA (warm-reactive autoantibody type: IgG 29.2 U/ml, IgM 19.3 U/ml; cold-reactive autoantibody type: IgG 27.4 U/ml, IgM 18.9 U/ml) were also significantly elevated compared with healthy controls P < 0.001). As aCL are elevated not only in SLE (with and without concomitant AIHA) but also in idiopathic AIHA it can be speculated that aCL are involved in the pathomechanism of autoantibody-induced erythrocyte destruction per se irrespective of an underlying SLE.

Anemia, Hemolytic, Autoimmune↗
PubMed1996

Antineutrophil cytoplasmic antibodies (ANCA) in rheumatoid arthritis: a prospective study.

To investigate a possible relationship between the presence of antineutrophil cytoplasmic antibodies (ANCA), rheumatoid factor (RF), antinuclear antibodies (ANA), complement, disease activity and disease severity, 111 clinically well-documented RA patients were studied prospectively for ANCA, RF, ANA, C-reactive protein (CRP), total haemolytic complement (CH50) and complement split product C3d. Disease activity and severity were also assessed clinically, as well as anamnestically, using the Hannover Activity of Daily Living Questionnaire, the functional Steinbrocker grades, and numeric and verbal rating scales. At a serum dilution of 1:50, 20% of the 111 sera showed predominantly an atypical perinuclear staining pattern. There was no correlation between ANCA positivity and serological markers, disease activity and disease severity. Regarding previous therapies with disease-modifying antirheumatic drugs, ANCA+ patients took sulphasalazine significantly more often than ANCA- patients.

Activities of Daily Living↗
PubMed1996

Multicentric reticulohistiocytosis. A report of 3 cases and review of literature.

Multicentric reticulohistiocytosis (MR) is a rare systemic disease of unknown cause. The disease is characterized by tissue infiltration of lipid-laden histiocytes and multinucleated giant cells. Destructive polyarthritis and skin lesions are the most common findings. The disease becomes apparent in adult life. We report on 3 patients and discuss the specific radiographic characteristics of bone and joint involvement. The radiologic feature is a bilateral, symmetric joint involvement with predilection for the interphalangeal and metacarpophalangeal joints. In all of our cases we found an early involvement of shoulder joints. Erosive arthritis begins at the margins of the joints, progressing to osseous defects and to severe joint destruction. In contrast to rheumatoid arthritis, periarticular osteoporosis and early joint space loss are absent in MR. In addition, significant erosions of distal interphalangeal joints are not common in rheumatoid arthritis.

Adult↗
PubMed1995

Clinical course of patients with antineutrophil cytoplasm antibody positive vasculitis after kidney transplantation.

Four patients with antineutrophil cytoplasm antibody (ANCA)-positive vasculitis were followed over a period from 27 to 54 months after successful kidney transplantation under immunosuppressive treatment with ciclosporine. ANCA titer prior to transplantation was markedly elevated and decreased in 1 of 4 patients under therapy with ciclosporine. Despite adequate ciclosporine blood trough levels, a relapse occurred in 2 patients 18 and 24 months after transplantation but therapy with cyclophosphamide induced remission and no patient lost his graft due to relapse of vasculitis. This observation demonstrates that kidney transplantation may be offered to ANCA-positive patients with end-stage renal disease due to vasculitis. However, patients under ciclosporine therapy are still at risk for the development of relapsing disease and cyclophosphamide has to be added to or substituted in part for standard immunosuppressive therapy in order to reverse clinical symptoms and prevent rapid deterioration of kidney function.

Adolescent↗
PubMed1994

Anti-neutrophil cytoplasm antibodies (ANCA) in rheumatoid arthritis: relationship to HLA-DR phenotypes, rheumatoid factor, anti-nuclear antibodies and disease severity.

To investigate a possible relationship between the presence of anti-neutrophil cytoplasm antibodies (ANCA), rheumatoid factors (RF), anti-nuclear antibodies (ANA), disease severity and HLA-DR phenotypes, 46 consecutive ANCA+ and 48 ANCA-, clinically well-documented RA patients were studied for RF, ANA and HLA-DR phenotypes. The 46 ANCA+ patients showed predominantly an atypical perinuclear staining pattern (89%). ANCA positivity was associated with higher RF titres (P < 0.005) and advanced functional Steinbrocker grades III/IV (P < 0.015). ANCA+ patients were also more often positive for ANA than ANCA- patients (P < 0.008). There was no correlation between ANCA positivity and certain HLA-DR phenotypes although the frequency of DR4+ (67% vs 52%) and, in particular, of DR4+ blanks (phenotypically homozygous) was increased in ANCA+ as compared to ANCA- patients (20% vs 8%). DR4-DR1-RA patients were twice as frequent in the ANCA- than in the ANCA+ group (22.9% vs 8.7%). Correspondingly, the DR4+DR1- phenotype was increased among ANCA+ RA patients. Regarding functional Steinbrocker grades, the DR4+ phenotypes were slightly but not significantly increased in grades III and IV whereas ANCA positivity was significantly associated with severe functional Steinbrocker grades III/IV (66% ANCA+ vs 39% ANCA-, P < 0.015). ANCA positivity identified a population of RA patients with a long-standing and severe clinical course of the disease. There was no correlation between ANCA positivity and certain HLA-DR phenotypes.

Adult↗
PubMed1994

[Cold labile serum and plasma proteins: clinical and diagnostic significance of cryoglobulins, cryofibrinogen and cold agglutinins].

Cold labile serum and plasma proteins can cause a variety of clinicopathological symptoms. Due to altered physicochemical properties, cryoglobulins and cryofibrinogens may cause increased serum viscosity, cold dependent protein precipitation or, in rare cases, serum gelification. Cold agglutinins, on the other hand, cause temperature dependent agglutination of erythrocytes and eventually hemolysis. All pathological cold dependent serum and plasma phenomena are associated with either neoplasma, autoimmune disorders, various infections or are considered as "essential". While the diagnosis of these conditions remained largely unchanged during the last 10 years, new aspects regarding etiology, pathogenesis, and therapy have arisen.

Agglutinins↗
PubMed1993

C-ANCA and p-ANCA positive vasculitis with crescentic glomerulonephritis: the same clinical feature but different immunological pathways?

Patients with cytoplasmatic (c)-ANCA positive vasculitis and crescentic glomerulonephritis (n = 25) had similar clinical presentation, age, sex distribution, organ involvement, therapy requirements, and outcome as patients with perinuclear (p)-ANCA positive vasculitis and crescentic glomerulonephritis (n = 7). Therefore, we conclude that these two entities are very closely related or even the same disease.

Adult↗
PubMed1993

Complement split product C3d as an indicator of disease activity in systemic lupus erythematosus.

In order to investigate, if complement levels can be used as an indicator of clinical activity in systemic lupus erythematosus (SLE), levels of C3, C4, CH50, and C3d were measured in 79 patients, 41 with inactive, 31 with moderately active and 7 with severely active disease. Our study shows that C3d, and particularly the C3d/C3 ratio, provide sensitive markers for disease activity in SLE. Since C3d is a direct measurement of complement turnover, it reflects complement activation better than C3, C4 and CH50.

Adolescent↗
PubMed1993

[Anti-neutrophil-cytoplasmic antibodies in rheumatoid arthritis: specificity and clinical relevance?].

Sera of 90 patients fulfilling the criteria of the American Rheumatism Association for the rheumatoid arthritis (RA) were screened for antibodies against neutrophil cytoplasmic antigens (ANCA) by an indirect immunofluorescence test. All sera were also tested for rheumatoid factor (Waaler-Rose-Test, WRT), C-reactive protein (CRP), antinuclear autoantibodies (ANA) on HEp-2 cells. 40% of the 90 sera showed ANCA-positive reactions. c-ANCA and p-ANCA types were both observed but the latter was by far more common (91%). The peculiarity of the p-ANCA pattern was a fine granular perinuclear staining. Antibodies against myeloperoxidase (MPO) were negative in all RA sera tested. So far we found a significant relationship between positive ANCA staining, functional Steinbrocker grades, and rheumatoid factor. No significant correlation was observed with extraarticular manifestation, CRP, therapeutic modalities (gold) or MHC antigens. Currently the nature of the neutrophil antigen involved is under investigation.

Arthritis, Rheumatoid↗
PubMed1993

[Unilateral blindness in neurosyphilis].

A 55 year-old patient developed loss of vision in his right eye over a period of a few weeks with the clinical finding of uveitis and papillaedema. Subsequently, also the left eye was affected by a marked deterioration of vision. The neurological findings were normal. The laboratory findings showed a marked elevation of the ESR (> 100 mm within the first hour) and protein electrophoreses showed changes suggestive of chronic inflammation. Analysis of the cerebrospinal fluid revealed a lymphocytic meningitis with 180/3 cells; the positive serological and liquor findings for TPHA and FTA led to the definite diagnosis of neurosyphilis. Treatment intravenously with 3 x 4 million units penicillin G daily for a fortnight proved curative. Vision of the left eye recovered fully, but the ophthalmological changes in the right eye were irreversible. This case shows that even today the differential diagnosis of syphilis must be considered with loss of vision of doubtful aetiology and uveitis.

Blindness↗
PubMed1993

[Secondary Raynaud's phenomenon].

Raynaud's phenomenon (RP) is an episodic digital ischemia provoked by internal and/or external stressors. While the primary RP occurs independently of other diseases and appears to be mediated by a vegetative imbalance of the peripheral vasomotor tone, the secondary RP is often part of an underlying disorder, especially of collagen-vascular diseases such as SLE or scleroderma. Vascular involvement in the pathogenesis of connective tissue diseases is well known but poorly understood. Endothelium injury by autoantibodies and circulating immune complexes may play a central but not sufficient role in the pathogenesis of secondary RP. In this review, epidemiology and diagnosis of RP secondary to connective tissues diseases are described and new pathogenic concepts are discussed in view of current knowledge on endothelial function.

Connective Tissue Diseases↗
PubMed1992

[Systematic lupus erythematosus and pregnancy].

Systemic lupus erythematosus (SLE) is an autoimmune disease that affects primarily women of childbearing age. 20-30 years ago it was common belief that SLE is exacerbated during pregnancy. Recent studies, however, could not support this notion. The likelihood of an increase in clinical activity of SLE is similar during pregnancy and in nonpregnant patients. It correlates directly with the activity of disease at the time of conception. The pregnancy outcome in patients with SLE has improved considerably in recent years. The frequency of fetal loss, however, still remains higher in SLE patients than in healthy women. In most of the cases fetal loss is due to habitual abortions, intrauterine death and premature labors. The main reasons are anti-phospholipid antibodies and the activity of SLE at the onset of pregnancy, respectively during the pregnancy. For monitoring disease activity and especially for distinguishing between the development of eclampsia and exacerbation of SLE, periodic determinations of complement proteins CH50, C3, C4 and C3d are helpful. A prophylactic therapy e.g. with prednisone in pregnant SLE patients is not justified. However, when SLE is exacerbating immunosuppressive therapy with prednisone and, if needed in combination with azathioprine, should be administered. In cases with high antiphospholipid antibody titers and prior fetal loss a treatment with low-dose acetylsalicylic acid can be considered.

Female↗
PubMed1992

[Neonatal lupus erythematosus as an example of passively acquired autoimmunity].

Neonatal lupus erythematosus is a typical example of passively acquired autoimmune disease. Congenital heart block is the main complication caused by transplacentally transferred maternal IgG antibodies directed against SS-A- or SS-B-antigen. While the transient lupus dermatitis occurs post partum and is often triggered by UV-light exposition, the irreversible cardial damage takes place between the 18. and 24, week of pregnancy. During this period 52-kD-SS-A- and 48-kD-SS-B-antigen are expressed in the fetal cardial tissue. The autoimmune reaction leads to an irreversible fibrotic destruction of the atrioventricular node. The resulting AV block and an antibody-induced perimyocarditis support the development of fetal bradyarrhythmia and hydrops. The very early diagnosis of a fetal heart block in bradycardiac fetus by echocardiography is essential for a consecutive therapy with steroids. By a reduction of the maternal antibodies an improvement of cardial symptoms in the fetus may be achieved in cases with fetal hydrops. Pregnant women at high risk with known connective tissue disease, previous children with congenital heart block, high titers of SS-A-/SS-B-antibodies and immunogenetic predisposition (HLA-DR3) have to be monitored intensively because of the possibility of a prophylaxis with dexamethasone and, in some selected cases, plasmapheresis.

Female↗
PubMed1991

Immunoglobulin allotypes are not associated with HLA-antigens, autoantibodies and clinical symptoms in systemic lupus erythematosus. Members of the SLE Study Group.

Immunoglobulin heavy chain (G1m, G2m, G3m, A2m) and kappa light chain (Km) allotype and phenotype frequencies of 323 central European Caucasian patients with systemic lupus erythematosus (SLE) were examined and correlated with various genetic, serologic and clinical markers of SLE. No significant associations were found between immunoglobulin allotypes or phenotypes and all 20 parameters tested (nephritis, vasculitis, arthralgias, photosensitivity, discoid lesions, central nervous system disease, Raynaud's phenomenon, sex, anti-Ro, anti-La, anti-nRNP, HLA-DR1-DR7, HLA phenotypes B8-DR3, B7-DR2). It could therefore be assumed that Gm, A2m and Km allotypes were not associated with HLA-antigens and had no influence on the serologic and clinical expression of SLE.

Autoantibodies↗