Cardiac involvement in mixed connective tissue disease. Pathomorphologic changes.
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Biomedical subjects
Publications and source records attributed to B Rozman.
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A patient in whom mixed connective tissue disease in association of Sjögren's syndrome had previously been diagnosed, experienced a syncopal attack. Electrocardiographic monitoring revealed periods of profound sinus bradycardia, sinus arrest with slow junctional escape rhythm, and first degree atrioventricular block during several episodes of dizziness. Complete right bundle branch block was a constant finding in this patient. Sinoatrial conduction time and sinus node recovery time were prolonged. Coronary heart disease was excluded by normal coronary arteriographic findings. This patient represents a rare case of cardiac involvement in mixed connective tissue disease.
OBJECTIVE: To determine whether the titers of anti-Ro/SS-A (Ro) antibodies fluctuate during the course of SLE and Sjögren's syndrome (SS) in parallel with disease activity, and if such fluctuations could be used to predict disease flares. We also evaluated whether the anti-Ro profile (anti-Ro 52, anti-Ro 60) changes over time, since such information could provide new insights into the induction and regulation of anti-Ro autoimmunity. METHODS: Sixteen patients with SLE and 15 patients with SS, all anti-Ro/SS-A antibody positive, were followed up for two years at three-month intervals. Clinical and laboratory parameters of disease activity were examined. Determination of the anti-Ro/SS-A titer was performed by counterimmunoelectrophoresis and the fine anti-Ro antibody specificity was determined by immunoblotting. RESULTS: The titers of anti-Ro antibodies fluctuated during the course of the illness in both SLE and SS patients. In SLE patients these changes were not (except in one case) associated with disease activity nor were they predictive of disease flares. The same was true for the SS patients, with the exception of two patients with skin vasculitis in whom anti-Ro antibody titers fluctuated in parallel with the disease activity. The anti-Ro antibody (anti-Ro 60 kD, anti-Ro 52 kD) specificity did not change in any of the patients during the follow-up period. CONCLUSION: Anti-Ro antibodies could represent a valuable indicator of disease activity in SS patients with cutaneous disorders. They do not, on the other hand, reflect disease activity in patients with SLE. The stable antibody profile in both SLE and SS patients supports the hypothesis that autoantibody production is predominantly genetically regulated.
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OBJECTIVE: To examine histomorphological and immunohistological changes in an autopsy series of systemic lupus erythematosus (SLE) patients with or without anticardiolipin antibodies (aCL). METHODS: Fourteen SLE patients who died at our department from 1988 to 1996 were included. The patients' medical files were reviewed for the clinical history and the presence of IgG and IgM aCL. Autopsy samples of various organs, including regularly the kidneys, heart, brain and skin, were studied by standard histological methods and the direct immunofluorescence technique. RESULTS: Thirteen of 14 (93%) autopsied SLE patients were persistently positive for IgG aCL and had common overt thrombotic complications and/or other clinical features related to the antiphospholipid syndrome. Their autopsy tissue samples showed frequent occlusive vascular changes such as bland thromboses, thrombotic microangiopathy (TMA) related changes and arterial intimalfibrous hyperplasia. The immune complex related vascular changes were mostly unremarkable and present mainly in low aCL positive patients, who also had more aggressive types of lupus glomerulonephritis (GN). CONCLUSION: Increased IgG aCL were found in 13 out of 14 autopsied SLE patients who had predominant occlusive vascular histopathologic changes. The coincidence of bland thromboses with a characteristic TMA histopathology suggested two pathogenetic mechanisms associated with the presence of aCL, one related to abnormal coagulation and the other to endothelial cell injury. The extent of granular vascular immune deposits, typical of SLE, and the severity of lupus GN were inversely related to the aCL level.
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In 22 patients with systemic sclerosis (SS) i.e. 16 patients with diffuse scleroderma and 6 patients with CREST syndrome the presence of pulmonary hypertension (PH) was investigated by microcatheter. Manifest PH was found in 3 patients with diffuse scleroderma (19%) and in 3 patients with CREST syndrome (50%). Latent PH was found in 8 patients with diffuse scleroderma (50%) and in 2 patients with CREST syndrome (33%). In our patients the occurrence of PH in CREST syndrome was more frequent than in diffuse scleroderma which is in accordance with the data from the literature. Isolated PH (without simultaneous involvement of pulmonary interstitium) was found in most patients with CREST syndrome (but in 1 case) and in 3 out of 11 patients with diffuse scleroderma.
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This paper describes a 47-years old patient with the history of lupus valvulitis for six and a half years, who had implantation of a Starr-Edwards prosthesis for a mitral insufficiency four years ago. This case represents the longest survival reported so far after mitral valve replacement for lupus valvulitis.
Our findings refer to a female patient with Felty's and Sjögren's syndromes and hypertrophic obstructive cardiomyopathy. The rheumatoid granuloma found in the left ventricle at autopsy and the absence of other conditions which may be manifested by hypertrophic obstructive cardiomyopathy suggest that the case studied, probably represents an extraarticular manifestation of Felty's and Sjögren's syndromes.
OBJECTIVE: The aim of this prospective 24-month follow-up study was to compare clinical features with radiological and magnetic resonance imaging (MRI) findings in evaluating synovial proliferation in the hand joints of 31 patients with rheumatoid arthritis (RA). A single joint was used for the follow-up of each patient. METHODS: Thirty-one small hand joints were examined by conventional radiography and MRI before and after 24 months of treatment. MRI assessment of disease progression (volume and/or signal intensity of the synovial proliferation on T1 weighted precontrast, T1 weighted postcontrast and T2 weighted images) was compared with a clinical assessment of the chosen joints, and with a plain x-ray film evaluation (Larsen's score). RESULTS: Of 26 joints which clinically improved (14 markedly and 14 slightly) during the study, on MRI 16 showed improvement, 8 showed no change, and 2 showed deterioration. Four clinically unchanged joints appeared improved on MRI. One joint deteriorated clinically and on MRI. Overall, there was a 58% congruence between clinical and MRI findings. On x-ray 23 joints showed no change; nine of these were also unchanged on MRI, while 13 showed improvement and one deterioration. Only in 2 out of 8 joints showing deterioration on x-ray were the MRI findings in accordance. In the remaining six joints MRI showed improvement. The congruence between x-ray and MRI was therefore 36%. CONCLUSION: The long-term follow-up of rheumatoid synovial proliferation of the small joints in the hand using contrast enhanced MRI is feasible and may provide additional information regarding disease activity. Important advantages over conventional radiography methods are its ability to demonstrate qualitative differences of synovial proliferation within bone erosions, and demonstrate not only deterioration, but also the improvement of inflammatory disease.