Sjögren’s Syndrome Clinical and Immunological Aspects

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.