1,721,220 research outputs found
Cryoglobulinemic Vasculitis
No full textCryoglobulinemic vasculitis, also termed mixed cryoglobulinemic syndrome, is a rare systemic small vessel vasculitis due to the vascular deposition of immune- complexes, mainly mixed IgG-IgM cryoglobulins. It is associated with hepatitis C virus infection, immunological, and neoplastic diseases. Cryoglobulinemic vasculitis is characterized by the classical triad of purpura, weakness and arthralgia, frequent multiple organ involvement, and with infrequent late lymphatic and hepatic malignancies. The etiopathogenesis of cryoglobulinemic vasculitis is not completely understood. However, hepatitis C viral infection and associated lymphotropism, genetic and environmental factors play important roles in cryoglobulin and immune-complex production that deposit in blood vessels, and in B-lymphocyte expansion. The diagnosis is suggested by clinical evidence of purpura, circulating mixed cryoglobulinemia and low C4 levels, and pathologically evident leukocytoclastic vasculitis in skin biopsy lesions. The prognosis is poor in patients with renal disease, liver failure, and malignancy. Treatment is directed toward eradicating hepatitis C viral infection employing combination PEGylated- interferon-alpha and ribavirin treatment, immunomodulatory and immunosuppressant medications as warranted by the level of clinical severity
Viral infections and systemic sclerosis.
No full textnon presente in quanto trattasi di Lettera all'editor
Successful treatment with leflunomide of arthritis in systemic sclerosis patients
No full textSystemic sclerosis (SSc) is a connective tissue disease clinically characterized by different degrees of skin fibrosis and visceral organ involvement Joint involvement with severe synovitis during SSc is relatively uncommon. About 11% of SSc patients present with arthritis at disease onset , usually characterized by mono-oligoarthritis, responsive to steroid therapy
Cryoglobulins and Cryoglobulins Secondary to Hepatitis C Virus Infection
No full textThe term cryoglobulinemia refers to the presence in the serum of one (monoclonal cryoimmunoglobulinemia) or more immunoglobulins (mixed cryoglobulinemia, MC), which precipitate at temperatures below 37 °C and redissolve on rewarming; this is an in vitro phenomenon. © 2014 Elsevier B.V. All rights reserved
Autonomic dysfunction in systemic sclerosis: time and frequency domain 24 hour heart rate variability analysis
No full textTo evaluate the autonomic nervous control of the heart in patients with systemic sclerosis (SSc), spontaneous heart rate variability was investigated by means of time-domain and spectrum analysis of 24 h ECG ambulatory recordings in 30 SSc patients (four males, aged 45.2 +/- 9 yr, mean +/- S.D., range 27-60) and 30 age-matched healthy subjects. A significantly higher heart rate (P < 0.01) and lower circadian and spectral indices of heart rate variability (P < 0.01) were observed in SSc patients, compared with controls. A predictive value of age (P = 0.002), tachycardia (P = 0.002), circadian heart rate variability (P = 0.0025) and spectral power values (P = 0.005) for patient mortality was found. Moreover, the relative risk of death was higher (P = 0.05) in older subjects with circulating anti-Scl70. These abnormalities, detectable by a feasible, non-invasive diagnostic approach, indicate the presence of autonomic cardiac neuropathy in SSc patient
Treatment of severe scleroderma skin ulcers with recombinant human erythropoietin
No full textSystemic sclerosis (SSc) is frequently complicated by skin ulcers, often unresponsive to traditional treatments. A preliminary evaluation of the effects of recombinant human erythropoietin (rHuEPO) was carried out in 14 patients with SSc with nonhealing, severe cutaneous ulcers. Patients received rHuEPO subcutaneously at a dosage of 150 IU/kg 3 times weekly for 2 weeks, twice weekly for the next 2 weeks, and then once weekly for 1 month. At follow-up 3-6 months from the beginning of the treatment, six patients showed complete resolution of the skin ulcers, while a significant reduction (> 60%) in lesional areas was obtained in the other eight patients (mean +/- SD ulcer area reduced from 27.6 +/- 28 to 5.3 +/- 7.8 cm(2); P<0.005). Moreover, patients' quality of life significantly improved (pain, as measured on visual analogue scale reduced from 96 +/- 5 to 46 +/- 17 points; P=0.0001; disability as measured by the Health Assessment Questionnaire-Disability Index reduced from 1.6 +/- 0.5 to 0.9 +/- 0.4 points; P=0.0001). The rHuEPO may represent a novel treatment of nonhealing scleroderma skin ulcers, suggesting some important aetiopathological implications
Systemic sclerosis following human cytomegalovirus infection
No full textSystemic sclerosis (SSc) is a connective tissue diseasecharacterised by skin and visceral organ involvement.The cause of SSc is still unknown; it has been suggestedthat one or more factors may be responsible for the diseasethrough a complex pathogenic mechanism. Immune systemdysregulation, collagen hyperproduction by altered fibroblasts,and vascular alterations can variably contribute to SScdevelopment. The presence of Raynaud’s phenomenon anddiffuse microangiopathy suggests that endothelial injury mayrepresent the first step in the pathogenesis of the disease.4Numerous genetic, environmental, and infectious agents havebeen proposed as possible triggering factors. Among these,human cytomegalovirus infection may play a part inthe pathogenesis of the SSc owing to its ability to infect bothendothelial and monocyte/macrophage cells
Trattamento con IgIV ad alte dosi in un caso di dermatomiosite con grave disfagia refrattaria alle terapie standard.
No full textdescrizione dell'efficacia di una terapia con immunoglobuline ad alte dosi per via endovenosa in un caso di dermatomiosite con impegno esofage
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