Complicación hematológica con impacto pulmonar en una paciente con síndrome de Sjögren

Abstract

Sjögren's Syndrome (SS) is an autoimmune pathology with glandular and/or extraglandular compromise, secondary to the infiltration of lymphoid cells. The clinical course varies depending on genetic susceptibility, comorbidities, patient's age, and environmental risk factors. Lymphoid proliferation and differentiation are key factors in the progression of SS to haematological malignancies or amyloidosis. Amyloidosis is a secondary entity to the aberrant accumulation of soluble plasma proteins, derived from chronic infectious, inflammatory, neoplastic and haematolymphoid processes. The clinical manifestations vary and depend on the constitutive protein and the age of the patient; and may have glandular or extraglandular, local, or systemic compromise. Among the affected organs, pulmonary involvement poses a diagnostic and therapeutic challenge due to its variable course and clinical manifestation. The following is a case report of a woman over 70 years old, with SS and amyloidosis with glandular and extra glandular manifestations at pulmonary level.