Sjogren's Syndrome
Sjogren's Syndrome and The Respiratory System
A Brief History of Sjögren's Syndrome by Doctor Ian D. Griffiths FRCR
Consultant Rheumatologist, Freeman Hospital, Newcastle-upon-Tyne
Knowing when and where a disease first started may help in understanding the factors that have caused the illness. If the disease affected the bones or joints, then clues may come from the excavation of ancient burial sites, but, more often than not, no lasting features of the disease remain and the evidence arises from the medical literature of the day.
Although we do not know when Sjogren's Syndrome first occurred, some features including dry mouth, dry eyes and enlargement of salivary and parotid glands were described in the 1880s, but at that stage the association of these different features was not recognised. In the 1920's two authors, one French and one Swedish independently drew attention to these features occurring together in some patients, but it was Henrik Sjogren who was responsible for the most clear description of the disorder, and whose name has subsequently been linked to the disease.
Henrik Sjogren (1899-1986) was a Swedish Ophthalmologist who in conjunction with his wife, also an Ophthalmologist, collected a series of patients with dry eyes and mouth which he described in his thesis written and presented in 1933. In it he describes 19 patients, all female, aged between 29 and 72 who had dry eyes and mouth and of whom 13 had a chronic arthritis. In a few patients he was also able to describe the microscopic features of chronic inflammation that occurred within the affected tissues. The factors that may have been causing the disease remained a mystery.The 1940's and 1950's saw the emergence of the concept of auto immune disease. It had been recognised that some local diseases (like thyroid disease) and some widespread disease (like Sjogren's and rheumatoid arthritis) were associated with changes of chronic inflammation in tissues in the absence of any obvious chronic infection. The 1940's and 1950's saw the identification of circulating proteins, called antibodies in the blood which were directed at normal cells in the body. These so-called "auto antibodies" seemed to correlate with some chronic diseases, for example thyroid antibody with thyroid disease, and rheumatoid factor with rheumatoid arthritis. Patients with Sjogren's Syndrome, both with and without arthritis, often had a variety of "auto-antibodies", but it was to be almost two decades before a strongly associated antibody was to be found. However, Sjogren's Syndrome seemed to be slotting in to this group of disorders collectively known as "Auto-lmmune Diseases".The National Institute of Health in the United States of America had, under the direction of Joseph Bunim, collected sizeable population of patients with Sjogren's Syndrome from across the USA. During the 1960's they described their findings. They reaffirmed that the majority of sufferers were female and recognised that most of their patients fell into one of the two groups - either associated with rheumatoid arthritis or occurring without arthritis, but often with severe generalised symptoms, for example malaise or rash.These two groups later became known as Primary Sjogren's Syndrome, when no other clearly identifiable associated disease is present and Secondary Sjogren's Syndrome when another disease, usually rheumatoid arthritis is present. In following up their patients, the group also became aware that lymphoma, a tumour of the lymph glands, was occurring more commonly than expected in the patients with Primary Sjogren's Syndrome. This association with lymphoma has now been described by several groups. The cells that undergo tumour change are the same cells that produce the auto-antibodies, but why this increased risk of lymphoma, which seems confined to Sjogren's occurs remains unexplained.The 1960's and 1970's also saw several other clinical developments. It became clear that dry eyes and mouth occurred commonly as part of the ageing process, that some drugs could mimic Sjogren's Syndrome and it could occur after organ transplantation. Moreover, not everyone with Primary Sjogren's Syndrome behaved the same. In some cases the disease remained confined to the eyes and mouth, whereas in others it was much more extensive with involvement of other organs and strikingly abnormal laboratory tests. The 1980's confirmed the validity of this sub division of Primary Sjogren's as it became clear that the patients with the more generalised systemic form of the disease had both different genetic and auto-antibody patterns.The possibility that familial factors may be relevant had been raised in the 1960's, but useful scientific support had to wait until the refinement of the so-called HLA tissue typing methodology in the 1970's which opened the door to organ transplantation. It became apparent that some of the inherited HLA antigens were associated with different forms of auto-immune disease. In Sjogren's the HLA antigen DR3, which is present in approximately a third of the normal population was present in over two thirds of patients with the Primary, generalised or systemic disease. The frequency of this HLA antigen is not increased in other groups. On the auto-antibody side, an antibody directed at part of a cell nucleus, usually called the anti-Ro antibody, had been detected and was shown to be strongly associated with the Primary Systemic Sjogren's Syndrome and certain forms of Systemic Lupus Erythematosus, a disease that shares some features with Sjogren's. It seemed, therefore, that even the Primary Sjogren's Syndrome could be sub-divided on clinical, laboratory and genetic grounds.While the last 30 years have revealed much about the clinical features of the differing forms of Sjögren's, along with the laboratory and genetic abnor-malities, the factors which irritate Sjögren's Syndrome remain elusive. An infectious cause, probably viral, remains the most likely contender. The revolutionary advances that have occurred in the science of molecular biology in the last few years, has provided investigators with the means to address the possible infectious causes in ways that were previously not possible.There is no reason to suspect that steady increase in our knowledge of Sjogren's Syndrome will not continue and with it bring advances in treatment which, to date, have been disappointing.
Sjogren's Syndrome : An Advisory Guide for Patient's and Doctors : £2.50
This booklet is produced by BSSA and is an invaluable source of information on Sjogren's syndrome both to newly diagnosed patients and also G.P.s, junior hospital staff, dental surgeons and nurses who will find the contents helpful in understanding the problems of paitients with the disorder.
Note:
WebHealth has additional articles about Sjogren's Syndrme at:
Systemic Features By:
Dr Ian Griffiths, Dr Elizabeth Price, Dr Clive Kelly and Dr Patrick Venables
and
The Problems of Sjogren's Syndrome By:
Dr CW Hutton, Consultant Rheumatologist, Demford Hospital, Plymouth
and
Sjogren's Syndrome and the Gastro-Intestinal Tract By
Doctor Richard Mount Ford MD, FRCP, FRCR.
Consultant Physician, Department of Gastroenterology, Bristol Royal Infirmary
and
Neurological problems in Sjogren's Syndrome by Doctor Annabel Coote and Doctor Michael Snaith, Department of Rheumatology, Royal Hallamshire Hospital, Sheffield.
and
Sjogren's Syndrome and the Respiratory System
A Brief History of Sjogren's Syndrome By
Doctor Ian D Griffiths FRCR, Consultant Rheumatologist,
Freeman Hospital, Newcastle-Upon-Tyne
