Anticorpo anti-colágeno tipo V em pacientes portadores de esclerose sistêmica: correlação com outros autoanticorpos específicos, critérios de gravidade e de atividade da doença

Abstract

Introduction: Systemic sclerosis (SSc) is an autoimmune disease of the connective tissue characterized by the triad of vascular injury, autoimmunity (cellular and humoral) and tissue fibrosis. The observation that type V collagen (COLV) is a hidden molecule between heterotypic fibers and deposition occurs with abnormal morphology of this collagen in the skin of SSc patients, strongly suggests that the COLV is an important character in triggering autoimmunity, since could elicit a specific response (COLV and anti COLV) with immune complex formation and deposition in the vascular endothelium, leading to vascular damage which would allow the influx of cells from innate immunity, causing release of proteolytic enzymes that degrade heterotypical collagen fibers with exposure of more COLV and perpetuation of the disease Objectives: To evaluate the socio demographic, clinical and laboratory characteristics of 60 SSc patients from de Department of Rheumatology of the University Hospital Maria Aparecida Pedrossian of FMUFMS and correlate the presence of anti COL V with clinical and laboratory aspects of the disease. Methods: We selected 60 patients with ES who agreed to participate in the study during the period from January to December 2013. During the initial interview were collected socio demographic data and clinical examination, in addition to serum anti COL V and specific antibodies ES, correlating the type of autoantibody with the clinical and laboratory manifestations found. Results: All patients evaluated ES met the new criteria of the 2013 ACR/ EULAR diagnostic pathology, were mostly female (98.3%) and the limited form of the disease (43.3%) with a mean age of 51 years-old white followed by brown, with initial manifestation of Raynaud's phenomenon before diagnosis, the mean disease duration of 9 years and skin score modified Rodnan average of 13.08. Regarding personal history, most stated that being a smoker (76.7%), diabetes mellitus was observed in 15% and cancer in 1.7% of patients with SSc. The main clinical manifestations observed in each organ system of the patients were: skin thickening in the hands (78.3%), Raynaud's phenomenon (100%), arthritis (33.3%), esophageal involvement (71.7%), interstitial lung disease (45%), pulmonary hypertension (19.4%) and scleroderma renal crisis (1.7%). The most significant laboratory abnormalities were: elevation of inflammatory markers in 41.7% of patients (ESR and CRP), elevation of CPK (15%), decrease in complement factors C3 and C4 (3.3%), antinuclear antibody (95%) anti centromere (41.7%), antibody against DNA topoisomerase 1 (26.7%), anti RNA polymerase III (11.7%), anti Ul RNP (16.7%) and anti Ro (SSA) in 11.7% of patients. The anti COLV positivity for the same. was detected in 8.3% of patients with SSc and showed a significant statistical correlation with disease activity and with scleroderma renal crisis, and tendency to association with pulmonary arterial hypertension; however, did not correlate with the severity index of disease or any other clinical manifestations, nor with any of the three specific antibodies ES studied. Conclusions: The anti COLV correlated significantly with disease activity and is probably related to the early injury of endothelium-basal membrane complex, being a good indicator of need for treatment in patients with