Abstract
Sjögren syndrome (SS) is a chronic autoimmune condition that mainly targets exocrine glands where it produces lymphocytic infiltrates. The clinical display varies in severity and can include malignancy association such as non-Hodgkin lymphoma. Its incidence ranges from 5 to 8%, while the risk of occurrence is 16 to 44 times higher. This article aims to describe the evolution of a patient with primary SS who developed pulmonary lymphoma for which she received Rituximab as part of the oncological therapeutic scheme. During the course of the disease she developed membranous glomerulonephritis, rarely described in these patients and low complement levels cited as predictive factor for lymphoproliferation.
Highlights
Sjögren syndrome (SS) is a chronic autoimmune disease characterized by lymphocytic infiltration of exocrine glands, predominantly at salivary and lacrimal sites
We present the case of a 61-year old female patient who was diagnosed with primary SS in 2004 based on the classical triad of xerostomia, xerophalmia and high levels of anti-Ro and anti-La antibodies together with bilateral parotid enlargement and arthralgia of the small joints
A Swedish study on a cohort of 175 patients with SS had a biopsy taken from the salivary tissue and identified lymphoid groups with a germinative center (GC) structure which proved to be highly predictive for further non-Hodgkin lymphoma
Summary
Sjögren syndrome (SS) is a chronic autoimmune disease characterized by lymphocytic infiltration of exocrine glands, predominantly at salivary and lacrimal sites This process can present itself alone or be related to other autoimmune disorders (secondary SS) [1]. Clinical presentation varies, going from exhibits of decrease in gland secretion to loss of acini and ducts to life-threatening events such as small vessel vasculitis, renal and pulmonary involvement or neoplastic occurrence, primarily malignant non-Hodgkin lymphoma [2]. The latter has a 5-8% incidence, with a 16 to 44-fold higher risk of development than the general population [3]
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