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  • 1
    ISSN: 1437-160X
    Keywords: Sjögren's syndrome ; Castleman's disease ; Lymphoproliferative disorder ; Monoclonal gammopathy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A 38-year-old woman suffering from primary Sjögren's syndrome for 2 years developed angiofollicular hyperplasia (multicentric Castelman's disease). In Sjögren's syndrome (SS) a number of findings indicate the presence of a B-cell hyperactivity that may evolve to a lymphoproliferative disorder. This report adds another pathological event to the complex spectrum of lymphoproliferative diseases in SS.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1437-160X
    Keywords: Key words Polymyositis ; Malignant melanoma ; α-Interferon
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Polymyositis is a systemic autoimmune disorder characterised by proximal muscle weakness which most frequently involves the limbs, neck and trunk. Alpha interferon is an antiviral molecule with well-known immunomodulatory and antiproliferative effects, but its use is often associated with a variety of side effects, in particular autoimmune phenomena. We report the occurrence of polymyositis during treatment with alpha-interferon in a patient affected by malignant melanoma. Indirect evidence suggests that in this case the patient's myositis was not linked to her neoplasia, since the melanoma was confirmed to be in remission both at the time of the diagnosis of the muscle disease and again after more than one year of follow-up.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Calcified tissue international 25 (1978), S. 53-57 
    ISSN: 1432-0827
    Keywords: PGE2 ; Bone resorption ; Calcinosis circumscripta
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine , Physics
    Notes: Summary A patient showed evident osteolysis in phalanges and heavy periarticular calcium deposits of the fingers, wrists and toes which avidly took up47Ca. The dense, white, tooth-paste like fluid contained in the periarticular calcium deposits has been studied by two different X-ray diffraction methods, by Ubatuba's bioassay for prostaglandin, by thin layer chromatography and by mass spectrometry. The calcium deposits were hydroxyapatite and prostaglandin PGE2 was detected in them. The bone resorption stimulating activity of PGE2 would be expected to result in increased bone destruction with release of calcium salts and this could be a working hypothesis of the pathogenesis of calcinosis circumscripta.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1439-0973
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Bei einer nicht selektierten Gruppe von 45 Patienten mit gemischter Kryoglobulinämie wurde mit ELISA (Chiron ELISA HCV, Second Generation) nach Anti-HCV-Antikörpern gesucht. Die Anti-HCV-Spezifität wurde mit einem auf rekombinantem Antigen basierenden Immunoblot-Assay (Chiron RIBA HCV, Second Generation) geprüft. Die Serumproben wurden außerdem auf Marker für HBV und auf Anti-HIV-Antikörper untersucht. Zum Vergleich wurden 80 Patienten mit anderen Erkrankungen des Immunsystems auf anti-HCV getestet. Antikörper gegen HCV fanden sich bei 91% der Patienten mit gemischter Kryoglobulinämie, sie wurden in allen Fällen mit RIBA bestätigt. In der Kontrollgruppe war der HCV-Test nahezu immer negativ. Bei 49% der Patienten mit Kryoglobulinämie fanden sich auch HBV-Marker, aber in keinem Fall Antikörper gegen HIV. Die Ätiopathogenese der gemischten Kryoglobulinämie erscheint durch diese Daten in einem neuen Licht.
    Notes: Summary The prevalence of antibodies against hepatitis C virus (anti-HCV) in an unselected series of 45 mixed cryoglobulinemia patients was assessed by an enzyme linked immunosorbent assay (Chiron ELISA HCV, Second Generation). The anti-HCV specificity was evaluated by a recombinant based immunoblot assay (Chiron RIBA HCV, Second Generation Assay). HBV-related markers and HIVAb were detected in the same samples. The prevalence of anti- HCV observed in mixed cryoglobulinemia was compared with 80 patients with other immunological systemic diseases. Anti-HCV were found in 91% of mixed cryoglobulinemia patients, and confirmed by RIBA in all cases; on the other hand, anti-HCV were practically absent in other control diseases. HBV markers were recorded in 49% of mixed cryoglobulinemia subjects; while HIVAb were constantly absent. These data give us new insights into the etiopathogenesis of mixed cryoglobulinemia.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1434-9949
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Calcium channel blockers have been used in the treatment of primary and secondary Raynaud's phenomenon (RP), and a beneficial effect was often recorded. The efficacy of slow-releasing nicardipine was assessed in a clinically homogeneous series of RP without underlying diseases in a randomized, double blind, cross-over and placebo controlled trial. Out of twenty-one selected patients (18 women and 3 men, mean age 46±12 yrs) eighteen completed the study and three dropped out, one for inadequate compliance and two due to headache. After a threeweek period, slow-releasing nicardipine (20 mg two times daily) was significantly more useful than placebo: the number of RP episodes per week decreased (p〈0.02), severity of discomfort and hand disability scores, evaluated after single RP attack, clearly improved (p〈0.005 and p〈0.02, respectively). According to clinical improvement, time of peak flow after postischemic reactive hyperaemia test was significantly reduced only after nicardipine (p〈0.01). These results show that slow-releasing nicardipine is generally well tolerated and can provide effective improvement in RP patients without underlying diseases.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1434-9949
    Keywords: Sjögren's Syndrome ; Clinical Markers ; Extraglandular Features
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Sixty Israeli patients, 30 with primary Sjögren's syndrome (SS) and 30 with rheumatoid arthritis (RA) and secondary SS, were evaluated. The Schirmer-1 test and a positive labial salivary gland biopsy were found to be the most helpful tools in assessing the diagnosis of SS. Extraglandular features such as Raynaud's phenomenon, lymphadenopathy and CNS involvement as well as parotid gland enlargement (p〈0.05) were more common in primary SS. Antinuclear antibodies, especially anti-Ro (SSA) and anti-La (SSB) were also more common in primary SS (p〈0.05). Our results are in accord with those of many European centers, despite the different genetic background.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1434-9949
    Keywords: Lung Volume Measurement ; Airway Obstruction ; Pulmonary Diffusing Capacity ; Connective Tissue Diseases
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Lung volumes, forced expiratory flow-volume curves, diffusing capacity indexes, and arterial blood gases were measured in 72 non-smoking patients with various connective tissue diseases (13 with rheumatoid arthritis, 17 with systemic lupus erythematosus, 25 with progressive systemic sclerosis, 10 with primary Sjögren's syndrome, 4 with polymyositis, and 3 with mixed connective tissue disease). Small airways disease and a diffusion capacity impairment were the most frequent and marked functional abnormalities in the whole group, and were often present in asymptomatic patients. Different lung function defects seemed to be present in each disease group. In fact, large airway obstruction was prevalent in progressive systemic sclerosis, diffusion capacity impairment in systemic lupus erythematosus, and small airways disease in rheumatoid arthritis. In contrast, primary Sjögren's syndrome appeared to be the connective tissue disease in which lung function abnormalities were less frequent and less pronounced.
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1434-9949
    Keywords: Key words:HBV – HCV – Mixed cryoglobulinaemia – Polyarteritis nodosa
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract: HCV infection has been associated with a broad spectrum of extrahepatic manifestations. In some of these, such as mixed cryoglobulinaemia (MC), the association is firmly established, whereas in others, such as polyarteritis nodosa (PAN), it is anecdotal; in fact, in this disorder the importance of the association is controversial, since it seems to be related to the frequent coinfection of HBV and HCV. The pathogenesis of MC and PAN is far from clear, but recent developments have added a plethora of information on the mechanisms underlying these disorders. Although both could be induced by a viral infection, the pathophysiological processes underlying the two diseases are different. We describe the occurrence in the same patient of HBV-related PAN and HCV-related MC.
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1439-0973
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Zwischen der Hepatitis C Virus-Infektion (HCV) und gemischter Kryoglobulinämie wurden bemerkenswerte Beziehungen festgestellt. Bei 15 Patienten mit gemischter Kryoglobulinämie wurde daher die Wirkung von alpha- Interferon (α-IFN) auf die HCV-Virämie und die klinischen und serologischen Parameter der Kryoglobulinämie geprüft (zehn Frauen, fünf Männer, mittleres Alter (±SD) 53±7 Jahre). α-IFN wurde während eines Monats täglich in einer Dosis von 2×106 IU und dann fünf Monate lang jeden zweiten Tag verabreicht. Die Kortikosteroiddosen blieben dabei in 14/15 Fällen unverändert. Nach der α-IFN-Therapie war eine signifikante Besserung der Purpura (p〈0,001), der Serumtransaminasen (p〈0,001) und des Kryokrit (p〈0,01) festzustellen. Bei 13/15 Patienten wurde mittels Polymerase-Kettenreaktion (PCR) eine HCV-Virämie nachgewiesen. Bei 8/15 fanden sich anti-GOR-Antikörper, die als Zeichen der HCV-assoziierten Autoimmunreaktion zu werten sind. Die HCV-RNA-Spiegel waren nach Behandlung mit α-IFN in fünf Fällen deutlich zurückgegangen und in einem Fall verschwunden. Zu einer änderung der anti-HCV Antikörper (Chiron ELISA und RIBA II) kam es während der sechsmonatigen Behandlung mit α-IFN jedoch nicht. Die Ergebnisse unterstützen die Annahme einer ätiopathogenetischen Beziehung zwischen HCV-Infektion und gemischter Kryoglobulinämie. Möglicherweise stellt α-IFN eine gezielte Therapie bei dieser Krankheit dar.
    Notes: Summary Since a striking association between hepatitis C virus (HCV) infection and mixed cryoglobulinemia has been demonstrated, the aim of this study is to investigate the effect of alpha-interferon (α-IFN) on HCV viraemia and clinico-serological manifestations of 15 patients (ten female and five male, mean [±SD] age 53±7 years). In 14/15 patients pre-study steroid dosage remained unchanged during treatment. α-IFN was administered at a dose of 2×106 IU daily for a month, then every other day for five months. On the whole, a statistically significant improvement of purpura (p〈0.001), serum transaminases (p〈0.001), and cryocrit (p〈0.01) was observed after α-IFN treatment. HCV viraemia was detected by polymerase chain reaction technique in 13/15 patients with mixed cryoglobulinemia and anti-GOR antibodies, expression of HCV-related autoimmunity, were present in 8/15. After α-IFN treatment, HCV RNA levels showed a clear-cut reduction in five persons and disappeared in another, while anti-HCV antibodies (Chiron ELISA and RIBA II) did not change after the six-month period of therapy. These data further support the possible etiopathogenetic role of HCV in patients with mixed cryoglobulinemia and suggest that α-IFN may be regarded as the elective treatment in this disease.
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 1434-9949
    Keywords: Behçet's disease ; Congenital panhypopituitarism ; Myelodysplastic syndromes
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Myelodysplastic syndromes (MDS) are hematologic disorders characterised by peripheral cytopenias and the hystologic features of hematologic dysplasia. Their association with autoimmune manifestations have been suggested by several authors: recently, the appearance of Behçet's disease during MDS has been reported. We describe the occurrence of MDS in a patient affected by Behçet's syndrome with gastrointestinal involvement and congenital panhypopituitarism.
    Type of Medium: Electronic Resource
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