RESUMO
The primary Sjögren's syndrome is a chronic inflamatory autoimmune disease of the exocrine glands with various glandular and extraglandular (systemic) manifestations. Changes in exocrine function of glands--including bronchial, should influence the frequency of pulmonary affecting, hence functional disorders as well. Examining some standard parameters of pulmonary function can often give valuable information about pulmonary alterations. The lack of tipical alterations in pulmonary function in patients with primary Sjögren's syndrome, found by us, gives good reason to express the supposition that pulmonary engagement with this disease is insignificant in the majority of cases. (Tab. 1, Ref. 7.).
Assuntos
Mecânica Respiratória , Síndrome de Sjogren/fisiopatologia , Humanos , Pessoa de Meia-Idade , Síndrome de Sjogren/patologiaRESUMO
Primary Sjögren's syndrome (PSS) is an autoimmune disease of the exocrine glands that is presented with progressive ocular and oral dryness, parotid enlargement and often with systemic (extraglandular) manifestations. In patients with PSS the risk of development of non-Hodgkin's lymphoma (NHL) is 44-fold compared to healthy population. The risk is associated with certain characteristics of the disease's course: recurrent changes in the parotid glands, lymph node enlargement, skin vasculitis, peripheral neuropathy, anemia and lymphopenia. According to the morphologic and phenotypic characteristics, B-cell low-grade lymphomas prevail. This communication presents a 65-year-old woman with PSS who developed a follicular type B-cell NHL 21 years after the autoimmune disease had been diagnosed. The analysis of our case and the literature review summarize the characteristics of the course of PSS, histologic variants, and evolution and prognosis of NHL in this kind of patients.
RESUMO
The primary Sjögren's syndrome (pSS) is an autoimmune exocrinopathy which is presenting with progressive dryness of the eyes and the mouth, changes in the parotid glands and not rarely with systemic (extraglandular) manifestations. The development of the immunogenetics proved that the course of the disease is genetically determined. HLA-antigens, associated with pSS differ in the separate races, populations and ethnic groups.