This is a 4 part discussion. Each part could stand alone. We need a snappier title an I have a problem with tense and pronouns (if no one has noticed.) Same as before cut an paste make you change and we can up up with a final version:
Life expectancy and Adverse (serious) Events Prognosis
The first question everybody has when diagnosed with a disease like Sjögren’s, is how is it going to turn out? There is a simple answer that is 100% correct - no one knows. We can predict however with reasonable certainty how it will turn out for a few among many. I am going to break this down into 4 separate articles. PLEASE read ALL of them, if you read just the “numbers” you will not have anymore knowledge than you did before. With every number there are certain conditions. Unless you meet those conditions, that number does not and cannot apply to you. If you do not meet those conditions, Your chance of adverse events including death are the same as if you did not have SS.
While many believe that SS is a benign disease, it is not. Rather it is characterized by a steady evolution of the predominant symptoms (sicca features and general manifestations.) Recent studies have prospectively analyzed the outcome of primary SS in large groups over a period of twenty years and the factors present at diagnosis that were prospectively associated with an adverse outcomes (development of vasculitis, B-cell lymphoma or death) While there are certainly many life changing aspects to the disease that vary from mild to severe only adverse events are considered to have an effect on life expectancey.
These outcomes are only for primary SS which is defined as SS where there is no other Autoimmune disease present. Roughly 50% of patients have primary SS. For those patients who have secondary SS, data at this point seems to indicate while they have sicca symptoms and other general manifestations; outcomes are determined by the primary Auto immune disease.
If you have Primary SS, meaning it is the only Autoimmune disease you have this is what we know:
Over a 20 year period 9% will develop Vasculitis. There are conditions at the time of diagnoses, if you do not meet them, you have a much lower chance of getting vasculitis very similar to that of a stranger on the street. IF you were to get vasculitis the condition would vary from mild (90% of cases) to serious and life threatening forms (10%) or in short only 9 in a thousand will develop serious vasculitis
Over the same period 3% will develop B-cell lymphoma in a 20 year period. Again certain conditions at the time of diagnoses must be met, if not your chance of B-cell lymphoma are the same as the population as a whole. There is a 17% mortality rate with B-cell lymphoma. In short 5 in a thousand SS patients will die from this cancer.
Now the tough one Death. (9%) patients died during the 20 year study with a mean age at death of 72 yrs. The main causes of death were infections in 32% of the cases. cardiopulmonary diseases in 24%, vasculitis was 12% non-haematological neoplasia was 12% lymphoma in two and other causes in 3%. The death rate for the entire population for the same period of study is 5% . This means the SS when accompanied by adverse events shortens life expectancy by roughly six years.
In the follow-up articles we will define the circumstances and conditions at diagnoses in which these probabilities occur.
Life expectancy and Adverse (serious) Events Prognosis
The first question everybody has when diagnosed with a disease like Sjögren’s, is how is it going to turn out? The truth is, no one knows. We can predict, however, with reasonable certainty how it will turn out for a few among the many.
This will be discussed in four separate articles. Please read ALL of them. If you read just the “numbers,” you will not have any more knowledge than you did before. Every number is associated with a certain condition. Unless you have the condition, that number does not and cannot apply to you. If you do not meet any of the conditions, your chance of adverse events (including a shortened lifespan) is just the same as if you did not have SS.
While many believe that SS is a benign disease, it isn't. SS is characterized by a steady evolution of the predominant symptoms (sicca features and general manifestations). Recent studies have prospectively analyzed the outcome of primary SS in large groups over a period of twenty years. Certain factors present at diagnosis were associated with adverse outcomes later on (such as the development of vasculitis, B-cell lymphoma, or a shortened lifespan). While there are certainly many life-changing aspects to this disease, varying from mild to severe, adverse events are considered to have an effect on life expectancy.
These outcomes are only for primary SS which is defined as SS where there is no other autoimmune disease. Roughly 50% of patients have primary SS. For patients with secondary SS, data at this point seem to indicate while these patients have sicca symptoms and other general manifestations; outcomes are determined by the primary autoimmune disease (lupus, fibromyalgia, psoriatic arthritis, etc.).
If you have Primary SS, meaning that SS is your only autoimmune disease, this is what we know:
Over a twenty year period, 9% of patients will develop Vasculitis. There are conditions at the time of diagnoses. If you do not meet them, you have a much lower chance of getting vasculitis, about the same as the general population. If you should get vasculitis without the conditions, your vasculitis would vary from mild (90% of cases) to serious and life-threatening forms (10%). Only 9 in 1,000 will develop serious vasculitis -- quite a low number.
Over the same twenty year period, 3% of patients will develop B-cell lymphoma. Again, certain conditions at the time of diagnoses must be met. If you don't have them, your chance of B-cell lymphoma is the same as the general population's. B-cell lymphoma has a 17% mortality rate. In other words, 5 in 1,000 SS patients will die from this cancer -- again, a very low number.
Now the tough one -- shortened lifespan. During the twenty year study, 9% of patients died, at a mean age of 72 years. The main causes of death were infections (32% of cases). cardiopulmonary diseases (24%), vasculitis (12%), non-haematological neoplasia (12%), lymphoma (2%), and other causes (3%). The death rate for the entire population during the twenty year period was 5%. This means that SS, when accompanied by adverse events, may shorten life expectancy by roughly six years.
In the follow-up articles, we will define the circumstances and conditions at diagnosis in which these probabilities occur.
Tj, I switched "death" to "shortened lifespan" in some places, as our more frightened members may take one look at the word "death" and panic, not absorbing the rest.
Tj, I switched "death" to "shortened lifespan" in some places, as our more frightened members may take one look at the word "death" and panic, not absorbing the rest.
Maybe just “Sjogren’s in the Long Term” or “Long Term Prognosis.” Mind set is everything. I’m sure we have a bunch of folks nearing 72 here on the site. We want to make sure they don’t decide that their number is up, so to speak. I’ll take another pass through and see what might need softening.