Antiphospholipid antibodies and systemic scleroderma.

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Abstract

Antiphospholipid antibodies (APLs) could be associated with an increased risk of vascular pathologies in systemic scleroderma. The aim of our study was to search for APLs in patients affected by systemic scleroderma and to evaluate their involvement in the clinical manifestations of this disease.

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Epidemiology of systemic sclerosis: incidence, prevalence, survival, risk factors, malignancy, and environmental triggers.

Maureen D. Mayes, Peter Barnes (2012)

To identify the recent data regarding prevalence, incidence, survival, and risk factors for systemic sclerosis (SSc) and to compare these data to previously published findings. SSc disease occurrence data are now available for Argentina, Taiwan, and India and continue to show wide variation across geographic regions. The survival rate is negatively impacted by older age of onset, male sex, scleroderma renal crisis, pulmonary fibrosis, pulmonary arterial hypertension, cancer, and antitopoisomerase and anti-U1 antibodies. It appears that silica exposure confers an increased risk for developing scleroderma, but this exposure accounts for a very small proportion of male patients. Smoking is not associated with increased SSc susceptibility. Malignancies are reported in scleroderma at an increased rate, but the magnitude of this risk and the type of cancer vary among reports. Prevalence and incidence of SSc appears to be greater in populations of European ancestry and lower in Asian groups. Exposure to silica dust appears to be an environmental trigger, but this only accounts for a small proportion of male cases. Evidence for increased risk of neoplasia is suggestive, but the magnitude of the risk and the types of malignancies vary among reports.

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Invitation to a debate on the serological criteria that define the antiphospholipid syndrome.

M. Galli, G Reber, P de Moerloose … (2008)

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Antiphospholipid antibodies and kidney involvement in patients with systemic sclerosis.

E Tokarzewska-Wielosz, Maria Majdan, Magdalena Dryglewska (2009)

Antiphospholipid (aPL) antibodies are often detected in systemic autoimmune diseases. The aim of the study was to examine the correlation between the presence of aPL and certain markers of renal function in systemic sclerosis (SSc). Fifty patients (pts) with SSc were examined for the presence of antibodies to cardiolipin (aCL) and to anti-beta 2 glycoprotein I (a-B2GPI) in immunoglobulin M (IgM) and IgG class. Moreover, serum levels of creatinine, cystatin C, and glomerular filtration rate (GFR) were determined in all patients. In all studied pts together, three multiple-regression analyses were performed with one set cystatin C as a dependent variable, in the second GFR according to the Cockcroft-Gault formula and in the third creatinine clearance by Modification of Diet in Renal Disease (MDRD) formula. As independent variables, aPL of either type were inserted in addition to disease duration and age. IgG aCL was significantly positively associated with serum cystatin C (p = 0.002), significantly negatively associated with creatinine clearance according to the Cockcroft-Gault and MDRD formula (p = 0.01 and 0.02, respectively). IgG a-B2GPI was significantly negatively associated with creatinine clearance according to the Cockcroft-Gault (p = 0.03) and MDRD (p = 0.01) formula. IgM aCL and IgM a-B2GPI were not associated with any markers of the renal function. Our study suggests the relationship between kidney involvement and the positivity for some aPL in patients with SSc. Positivity for IgG aCL and IgG a-B2GPI in patients with SSc without secondary antiphospholipid syndrome seems to be connected with decrease of glomerular filtration.