Sjogren’s syndrome (SS) is a chronic, slowly progressive, inflammatory,
autoimmune disease characterized by (1) lymphocytic infiltration of the
exocrine glands leading to diminished or absent glandular secretion, and
(2) marked B-lymphocytic cell hyperreactivity manifested initially by a
variety of serum autoantibodies, including those against the Ro(SSA) and
La(SSB) ribonucleoproteins, ending in the development of B cell
non-Hodgkin’s lymphoma in a substantial number of patients. Most
patients with SS present only with keratoconjunctivitis sicca and
xerostomia. However, similar to 40% develop extraglandular
manifestations that present in two ways: (1) the development of
lymphoepithelial lesions in several extra-exocrine gland tissues (i.e.,
bronchi, renal tubules, or biliary ducts), and (2) vasculitis related to
the deposition of immune complexes due to B cell hyper-reactivity.
Pulmonary manifestations develop in some patients and may present as (1)
bronchitis sicca; (2) a wide spectrum of lymphoproliferative diseases,
ranging from bronchus-associated lymphoid tissue (BALT) hyperplasia,
lymphoid interstitial pneumonia, and B cell non-Hodgkin’s lymphoma
mainly of the extranodal marginal zone B-cell lymphoma of BALT-type or
rarely of higher-grade malignancy; and (3) other interstitial
pneumonias. Pleuritis can be seen in SS patients with associated
systemic lupus erythematosus or rheumatoid arthritis
