1,721,128 research outputs found
Severe alopecia complicating systemic sclerosis
No full textAims: To describe a case of systemic sclerosis (SSc) associated with severe alopecia areata (AA) responsive to topical and systemic treatments, including vasoactive and immunosuppressive drugs (mycophenolate mofetil).
Presentation of the Case: A 56 year old woman, affected by SSc as from 5 years back, developed a rapid hair loss that progressively involved a large area of the scalp. AA was diagnosed, after the exclusion of an overlapping systemic lupus erythematosus or fungal infection. Treatment with topical steroids and minoxidil, plus mycophenolate mofetil that was introduced for interstitial lung disease, led to progressive improvement of alopecia up to a complete resolution
within 4 months.
Discussion: This is an interesting observation of SSc complicated by severe AA, which is often observed in patients affected by various autoimmune disorders. A possible common pathogenesis of AA and SSc is also discussed
Impegno cardiaco nella sclerosi sistemica
No full textL'impegno cardiaco in corso di sclerosi sistemia consta di uno spettro variabile di manifestazioni cliniche conseguenti ad alterazioni di natura microangiopatica, elettrica e/o emodinamica. In danno ischemico e/o flogistico autoimmune del miocardio producono, in ultima analisi, il quadro istopatologico caratteristico della 'necrosi a bande' con deposizione di tessuto fibroso, responsabile della riduzione di elasticità e della capacità contrattile muscolare. ...
Renal Manifestations of Hepatitis C Virus
Full text linkHepatitis C virus (HCV) is a hepatotropic and lymphotropic virus responsible for hepatic and extrahepatic autoimmune and neoplastic disorders, including renal involvement, which is the consequence of immune-mediated organ damage due to glomerular deposition of immune-complex and/or anti-HCV IgG antibodies and complement. It can appear at any time during the natural history of HCV infection, more often as membranoproliferative glomerulonephritis, alone or in association with other HCV-related disorders. The presence of renal involvement should be investigated in HCV-infected individuals at the first referral and during clinical follow-up
Haematological Malignancies in Systemic Sclerosis Patients: Case Reports and Review of the World Literature
Full text linkBackground.The association of systemic sclerosis (SSc) and haematological cancers was reported in a large number of case reports
and cohort studies, describing SSc patients with highly heterogeneous clinical pictures. Objective. We reviewed the literature to
better describe SSc patients with haematological malignancies. Methods. SSc cases complicated by haematological malignancies
described in the world literature were collected; other 2 cases referred to our centre were reported. Results. One hundred-thirty SSc
subjects were collected from 1954 up to date. The mean age of patients at cancer diagnosis was 56.1 ± 16.7 years; 72% of patients
were females. In 60% of cases, the diagnosis of haematological malignancy was described within 5 years of SSc diagnosis. In 7.8% of
cases, coexistence of Sj ̈ogren’s syndrome or other autoimmune disorders was cited. Sixty-six cases with lymphoma (in the majority
of cases B-cell neoplasms), 28 with leukaemia (chronic lymphocytic form in 9), 14 with multiple myeloma plus one solitary IgM
plasmocytoma, and 16 with myeloproliferative disorders were found. No specific SSc subsets seem to be related to haematological
malignancies. Conclusions. We remarked the importance of clinical work-up in SSc, in order to early diagnose and treat eventual
occult haematological malignancies, especially during the first years of the disease
Prevalence and evolution of scleroderma pattern at nailfold videocapillaroscopy in systemic sclerosis patients: Clinical and prognostic implications
No full textBackground: Microvascular involvement plays a decisive role in systemic sclerosis (SSc) pathogenesis occurring early in the course of the disease. Microangiopathy is responsible of important clinical manifestations, such as Raynaud's phenomenon, digital ulceration, and pulmonary arterial hypertension. Typical microvascular alterations, called scleroderma pattern, are detectable at nailfold capillaroscopy in a significant percentage of SSc patients: however its prevalence is highly variable in published studies.
Aim: The aims of this study are to evaluate the prevalence and the evolution of scleroderma pattern in SSc patients and analyze their demographic, clinical and prognostic characteristics according to capillaroscopic features.
Methods: Two hundred and seventy-five SSc patients, underwent at least two nailfold videocapillaroscopy during follow-up, were retrospectively enrolled.
Results: A scleroderma pattern was observed in 80% of patients at baseline and 87.1% during follow-up, and it was significantly associated to digital ulcers, interstitial lung disease, reduction of diffusion lung of carbon monoxide <75%, teleangectasias and melanodermia, while sicca syndrome and arthralgias were associated to normal/nonspecific pattern. Digital ulcers, teleangectasias, sicca syndrome, and arthralgias remained independently associated with scleroderma pattern on multivariate analysis.
In conclusion, the main clinical manifestation correlated with scleroderma pattern is the occurrence of digital ulcers, and their appearance is strictly correlated with the variation of capillaroscopic feature during the time. Further studies should confirm the association between SSc pattern and lung fibrosis.Background: Microvascular involvement plays a decisive role in systemic sclerosis (SSc) pathogenesis occurring early in the course of the disease. Microangiopathy is responsible of important clinical manifestations, such as Raynaud's phenomenon, digital ulceration, and pulmonary arterial hypertension. Typical microvascular alterations, called scleroderma pattern, are detectable at nailfold capillaroscopy in a significant percentage of SSc patients; however its prevalence is highly variable in published studies. Aim: The aims of this study are to evaluate the prevalence and the evolution of scleroderma pattern in SSc patients and analyze their demographic, clinical and prognostic characteristics according to capillaroscopic features. Methods: Two hundred and seventy-five SSc patients, underwent at least two nailfold videocapillaroscopy during follow-up, were retrospectively enrolled. Results: A scleroderma pattern was observed in 80% of patients at baseline and 87.1% during follow-up, and it was significantly associated to digital ulcers, interstitial lung disease, reduction of diffusion lung of carbon monoxide <. 75%, teleangectasias and melanodermia, while sicca syndrome and arthralgias were associated to normal/nonspecific pattern. Digital ulcers, teleangectasias, sicca syndrome, and arthralgias remained independently associated with scleroderma pattern on multivariate analysis.In conclusion, the main clinical manifestation correlated with scleroderma pattern is the occurrence of digital ulcers, and their appearance is strictly correlated with the variation of capillaroscopic feature during the time. Further studies should confirm the association between SSc pattern and lung fibrosis
Rituximab in the treatment of patients with systemic sclerosis. Our experience and review of the literature
Full text linkThe treatment of systemic sclerosis (SSc) represents a great clinical challenge because of the complex disease pathogenesis including vascular, fibrotic, and immune T- and B-lymphocyte-mediated alterations. Therefore, SSc should be treated by combined or sequential therapies according to prevalent clinico-pathogenetic phenotypes. Some preliminary data suggest that rituximab (RTX) may downregulate the B-cell over expression and correlated immunological abnormalities
Role of CXCL10 in cryoglobulinemia
No full textInterferon (IFN)-γ-induced protein 10 (IP-10/CXCL10) and its receptor, C-X-C motif receptor 3, appear to contribute to the pathogenesis of hepatitis C virus (HCV)-related mixed cryoglobulinaemia (MC) (HCV+MC). The secretion of CXCL10 by cluster of differentiation (CD) CD4+, CD8+, and natural killer-T cells is dependent on IFN-γ, which is itself mediated by the interleukin-12 cytokine family. Under the influence of IFN-γ, CXCL10 is secreted by several cell types including lymphocytes, hepatocytes, endothelial cells, fibroblasts, etc. In tissues, recruited T helper 1 lymphocytes may be responsible for enhanced IFN-γ and tumour necrosis factor-α production, which in turn stimulates CXCL10 secretion from the cells, therefore creating an amplification feedback loop, and perpetuating the autoimmune process. High levels of CXCL10 in circulation have been found in HCV+MC, especially in patients with clinically active vasculitis. Furthermore, HCV+MC patients with autoimmune thyroiditis (AT) have higher levels than those without AT. Further studies are needed to investigate interactions between chemokines and cytokines in the pathogenesis, and to evaluate whether CXCL10 is a novel therapeutic target in HCV-related MC
Measuring microangiopathy abnormalities in systemic sclerosis patients: the role of capillaroscopy-based scoring models
No full textCapillaroscopy is a noninvasive imaging technique for the in vivo study of microcirculation. The role of a qualitative evaluation of capillaroscopy in the assessment of Raynaud's phenomenon secondary to scleroderma spectrum disorder, particularly systemic sclerosis (SSc), is well defined. The usefulness of capillaroscopy in the follow-up of SSc patients and the possible prognostic role for the appearance of typical SSc vascular and visceral involvement, namely, digital ulcers, pulmonary arterial hypertension, and mortality, is suggested by many authors but still under debate. In this regard, and for a reliable and repeatable longitudinal evaluation of SSc microangiopathy, a quantitative analysis should be required. In this review, we describe the current classifications proposed to define the SSc microvascular involvement and the scoring methods suggested for a semiquantitative and quantitative analysis of microangiopathy and its correlation with clinical manifestations of disease
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