Main description:

I have been a student of Sjogren's syndrome for virtually all of my professional life. My education in this disease began in 1962 when I arrived at the National Institutes of Health to begin a Clinical Asso- ciateship with Dr. Joseph J. Bunim. Bunim introduced me to a pat- ient with Sjogren's syndrome of 8 years duration who had devel- oped malignant lymphoma 6 months previously. He told me that there were other such patients. I obtained serum samples from these patients and studied them by the then new technique of immuno- electrophoresis. We observed that an initial hypergammaglobulin- emia could progressively decline to hypogammaglobulinemia with loss of autoantibodies. One patient in this initial series had macrog- lobulinemia. We published this report and suggested that the auto- immunity predisposed to the malignant transformation. Thus began my love affair with this disease. In those days many rheumatologists considered Sjogren's syn- drome simply a variant of rheumatoid arthritis. It's curious that two decades ago there was little confusion between Sjogren's syndrome and systemic lupus erythematosus, whereas today there is great con- fusion.
There is still a great need for internationally agreed upon di- agnostic criteria, which merely illustrates once again the difficulty of accurate diagnosis in our profession. The multidisciplinary aspects of Sjogren's syndrome require au- thorities in several areas of medicine. The various chapter contribu- tors are experts in their field and have often put aside other respon- sibilities to complete their contributions and not delay publication.

Contents:

I. Introduction.- 1. Historical Perspective: The Early Years (1930-1960).- 2. The Patient's Perspective.- II. Clinical Spectrum.- A. Glandular Findings.- 1. Oral Manifestations of Sjoegren's Syndrome.- 2. The Eye in Sjoegren's Syndrome.- 3. Chemistry of Saliva.- 4. Histopathology of Sjoegren's Syndrome.- B. Extraglandular Findings.- 1. Primary Sjoegren's Syndrome in the People's Republic of China.- 2. Neuromuscular Complications of Primary Sjoegren's Syndrome.- 3. The Respiratory System in Sjoegren's Syndrome.- 4. Gastrointestinal and Hepatobiliary Features in Sjoegren's Syndrome.- 5. Renal Disease with Sjoegren's Syndrome.- 6. Inflammatory Vascular Disease in Sjoegren's Syndrome.- C. Lymphoid Malignancy.- 1. Lymphoid Malignancy and Monoclonal Proteins.- 2. Detection of Lymphoma in Sjoegren's Syndrome by Analysis of Immunoglobulin Gene Rearrangements.- 3. Lymphoproliferative Disorders in Japanese Patients with Sjoegren's Syndrome.- D. Relationship to Other Autoimmune Diseases.- 1. Relationship of Sjoegren's Syndrome to Rheumatoid Arthritis.- 2. Sjoegren's Syndrome with Systemic Lupus Erythematosus/Mixed Connective Tissue Disease.- 3. Sjoegren's Syndrome and Systemic Sclerosis (Scleroderma).- E. Diagnosis and Differential Diagnosis.- 1. Diagnosis and Differential Diagnosis of Sjoegren's Syndrome.- III. Laboratory Abnormalities.- 1. Rheumatoid Factor in Sjoegren's Syndrome.- 2. Autoantibodies in Sjoegren's Syndrome.- 3. Immunogenetics of Sjoegren's Syndrome.- 4. The Relationship Between Anti-RO(SS-A) Precipitin Antibody Positive Sjoegren's Syndrome and Anti-RO(SS-A) Precipitin Antibody Positive Lupus Erythematosus.- 5. Immunologic Abnormalities in Sjoegren's Syndrome.- 6. Animal Models of Sjoegren's Syndrome.- IV. Treatment of Sjoegren's Syndrome.