title: Hypergammaglobulinemic purpura in patients with Sjögren's syndrome: a report of nine cases and a review of the Japanese literature.
abstract: Nine patients with hypergammaglobulinemic purpura associated with primary Sjögren's syndrome were presented. All patients were female (mean age 45.6) and showed recurrent purpura on the lower extremities and typical findings of Sjögren's syndrome with high gammaglobulin and IgG levels, rheumatoid factors, anti-SSA/SSB antibodies (5/5) and anti-nuclear antibodies (6/9). Vasculitis was seen in 6 patients, 4 were of the mononuclear cell type and 2 of the neutrophilic cell type. Six patients had intermediate complexes between 7S and 19S shown by serum ultracentrifugation. Immunofluorescent staining showed immunoglobulin deposition along the blood vessel walls of the skin in these patients. These data suggest that hypergammaglobulinemic purpura in Sjögren's syndrome is vasculitis caused by immunologic processes.
authors.affiliation: Department of Internal Medicine, Kanazawa Medical University, Ishikawa, Japan.
authors.forename: S
authors.initials: S
authors.lastname: Sugai
authors.forename: S
authors.initials: S
authors.lastname: Shimizu
authors.forename: J
authors.initials: J
authors.lastname: Tachibana
authors.forename: M
authors.initials: M
authors.lastname: Sawada
authors.forename: R
authors.initials: R
authors.lastname: Yoshioka
authors.forename: Y
authors.initials: Y
authors.lastname: Hirose
authors.forename: T
authors.initials: T
authors.lastname: Takiguchi
authors.forename: S
authors.initials: S
authors.lastname: Konda
authors.forename: T
authors.initials: T
authors.lastname: Murayama
mesh_terms: D000328:Adult; D000368:Aged; D000974:Antibodies, Antinuclear / Q000032:analysis; D000936:Antigen-Antibody Complex / Q000032:analysis; D003176:Complement C3 / Q000032:analysis; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D008875:Middle Aged; D011694:Purpura, Hyperglobulinemic / Q000150:complications* / Q000276:immunology; D012217:Rheumatoid Factor / Q000032:analysis; D012859:Sjogren's Syndrome / Q000150:complications* / Q000276:immunology
chemical_list: D000974:Antibodies, Antinuclear; D000936:Antigen-Antibody Complex; D003176:Complement C3; D007074:Immunoglobulin G; D012217:Rheumatoid Factor