Margaret Wisłowska
Szpital Kliniczny MSW, Poland
Title: Sjogren or sicca syndrome and IgG4 positive multiorgan lymphoproliferative syndrome
Biography
Biography: Margaret Wisłowska
Abstract
Sjogren syndrome [SS] is an inflammatory autoimmune disease affecting primarily the exocrine glands. Lymphocytic infiltrates replace functional epithelium leading to decreased exocrine secretions (exocrinopathy). Characteristic autoantibodies, anti-Ro (SS-A) and anti-La (SS-B) are produced. Mucosal dryness manifests as xerophthalmia (keratoconjunctivitis sicca), xerostomia, xerotrachea and vaginal dryness. The periepithelial extraglandular manifestations are the results of lymphocytic invasion in epithelial tissues of the lungs, kidneys and the liver. The extraepithelial manifestations, such as skin vasculitis, peripheral neuropathy and glomerulonephritis with low C4 levels, are associated with increased morbidity and high risk for lymphoma. Clinical manifestations include glandular involvement such as ocular involvement and oropharyngeal involvement; and extraglandular manifestations such as arthritis, skin involvement (purpura, annular erythema and Raynaud’s phenomenon); pulmonary involvement (bronchial abnormalities and parenchymal changes), gastrointestinal and hepatobiliary features; neuromuscular involvement (mononeuritis multiplex, polyneuropathy); and renal involvement (renal tubular acidosis, glomerulonephritis). Minor salivary gland biopsy from the inferior lip is a cornerstone for the diagnosis of SS. In microscopic examination, the focal score in an area of 4 mm2 of focal aggregates of at least 50 lymphocytes is sufficient for diagnosis. Another possibility to recognize SS is with an ocular staining score of 3 or greater. IgG4 positive multiorgan lymphoproliferative syndrome like Mikulicz disease (MD) is characterized by high serum levels of IgG4 and tissue biopsies showing an infiltration of IgG4+ plasma cells coupled with fibrosis or sclerosis. Compared with SS, MD does not show the same female predominance and is associated with a lower frequency of dry eyes and dry mouth, arthralgia, and serum antinuclear antibody (ANA) test positivity.