Serum Elastin-Derived Peptides and Anti-Elastin Antibody in Patients with Systemic Sclerosis

The elastin metabolism in systemic sclerosis (SSc) has been known to be abnormal. The authors investigated relationship between the clinical manifestations of systemic sclerosis (SSc) and serum levels of soluble elastin-derived peptide (S-EDP) and anti-elastin antibodies. Serum samples were obtained from 79 patients with SSc and 79 age- and sex-matched healthy controls. Concentrations of serum S-EDP and anti-elastin antibodies were measured by ELISA. The serum concentrations of S-EDP in SSc patients were significantly higher than in healthy controls (median, 144.44 ng/mL vs 79.59 ng/mL, P < 0.001). Serum EDP concentrations were found to be correlated with disease duration in SSc (P = 0.002) and particularly in diffuse cutaneous SSc (P = 0.005). Levels of anti-elastin antibodies were found to be more elevated in SSc patients than in healthy controls (median, 0.222 U vs 0.191 U, P = 0.049), more increased in diffuse cutaneous SSc than limited cutaneous SSc (median, 0.368 U vs 0.204 U, P = 0.031). In addition, levels of anti-elastin antibodies were also found to be negatively associated with presence of anti-centromere antibody (P = 0.023). The S-EDP levels were not found to be correlated with levels of anti-elastin antibodies. The increased S-EDP and anti-elastin antibody levels and association with clinical and laboratory characteristics may reflect the abnormal metabolism in SSc.

Especificidad molecular a proteínas CENP en sueros anti-centrómero / Molecular specificity to CENP proteins in anti-centromere sera

Se estudió la reactividad molecular a proteínas CENP, en sueros con especificidad anticentrómero, utilizando extractos de células HEp-2, por la técnica de Western blot, las bandas inmunorreactivas se revelaron por autorradiografía. En 1691 sueros en los que se determinaron anticuerpos, por inmunofluorescencia indirecta, 11 resultaron con patrón anti-centrómero, de éstos, siete reconocieron bandas de CENP en w. blot, los antígenos más frecuentes detectados fueron CENP-B y CENP-C. El estudio a nivel molecular constituye una alternativa fina para la determinación de la especificidad de los autoanticuerpos

The anticentromere antibody and carbon monoxide diffusing capacity in progressive systemic sclerosis

The authors studied the frequency and the clinical of ACA in 34 patients with PSS, 20 with inflammatory form and 14 with CREST variant. All of them were evaluated by ANA, ACA, pulmonary function study. Carbon monoxide diffusing capacity was studied in 19 patients. ACA occurred with a frequency of 50% in the group with non-inflammatory from and 20% in that with inflammatory form. However this difference wasn't statistically significant. Although patients with CREST syndrome and severe restrictive pulmonary disease were always ACA positive, there wasn't statistical significant correlation between ACA and pulmonary impairment evaluated by DLCO

Atividade biologica do anticorpo anticentrômero. Capacidade de fixaçäo de complemento / Biological activity of anticentromere antibody. Complement fixation capacity

Anticorpo anticentrômero (AAC) foi detectado em oito de nove pacientes com síndrome de CREST, num estudo de 105 pacientes com doenças difusas do tecido conectivo (DDTC) e 60 normais, por técnica de imunofluorescência indireta em células HEp-2. Quatro destes soros foram avaliados quanto a capacidade de fixaçäo de complemento, pela mesma técnica, porém revelando a reaçäo com anti-Ciq e anti C, marcados com fluoresceína. Todos os soros mostraram positividade da reaçäo apenas com anti-C3. Apesar do ensaio sugerir fixaçäo de complemento pela via alternativa, näo foram observadas alteraçöes do nível sérico do complemento. O estudo seriado de uma paciente tratada com D. penicilamina, demonstrou negativaçäo deste auto anticorpo após seis meses de tratamento. O estudo familiar, deste caso, revelou a presença de AAC em uma das filhas, que apresentava apenas fenômeno de Raynaud. Esses dados sugerem que AAC pode ter um papel de importância relevante no diagnóstico, e possivelmente na monitorizaçäo destes pacientes