I know that Sjogren's occurs primarily in older women, but can anyone link me to the epidemiology of non-Sjogren's Sicca syndrome?
I must have really been beaten by the odds, since I'm male and suspect I've been cursed with the latter (or simply an extreme, as yet unexplained dryness of the nasal mucous membranes, and to a lesser degree the eyes, mouth, and throat) with no rheumatic-type symptoms I can detect since I was a child - many (many) decades ago.
Or is the distribution of NonSj Sicca syndrome different from that of Sjogren's?
Philip Clements says they're two different things, but doesn't elaborate here on what I'm asking:
Dry Eyes, Dry Mouth (Sicca Syndrome versus Sjogren?s Syndrome) Philip Clements, MD, MPH- 2017
https://www.youtube.com/watch?v=z3pjlQEw3Tw&t=404s
I was told no.
I think the primary difference is that the diagnosis of Sjogren's Syndrome is based on attacks by autoantibodies on the organs/systems of the body. The autoantibodies can be found in blood samples of patients diagnosed with Sjogren's Syndrome.
Sicca, on the other hand is the diagnosis when the patients has dry eye/dry mouth, and no autoantibody activity is present in the blood, and the lip biopsy is negative (?) (I'm not sure about the lip biopsy part, actually).
The damage from Sjogren's Syndrome can often be limited to the dry eye, dry mouth symptoms, with the attacks from autoantibodies limited to only one system (the moisture producing system). With proper medication and self care, Sjogren's Syndrome can be a manageable condition.
Unfortunately, the damage from the attacks of autoantibodies in those with Sjogren's Syndrome can be far more extensive, involving just about every organ/system in the body. Because our system of diagnosis of autoimmune conditions focuses mainly on the organs/systems damaged, those who suffer from Sjogren's Syndrome may accumulate many other autoimmune diagnoses: RA, Hashimoto's, Lupus, and any of the other nearly 100 autoimmune conditions.
The diagnosis of Sjogren's has routinely been entered into my health history, and yet I have never had significant antibodies in my blood. My immunologist believes that the biochemicals that my Immune System uses to attack my organs/system are cytokines, one of the principal biochemicals that produce inflammation as part of our normal Immune Response. The list of organs/systems damaged by my Immune Disorder is very long, painful, and disabling.
One of the reasons that we discuss so many other medical conditions in this forum is that the activity of autoantibodies produces inflammation, the underlying damaging condition is an inflammatory disorder. The inflammation causes damage of so many kinds, and so, for example, we discuss carpal tunnel syndrome, which afflicts many of us, or torn meniscuses, another example of tissue damaged by chronic inflammation.
As always, my belief is that the most effective management of Immune Disorders is to treat the symptoms, until such time that the underlying cause(s) of the Disorders can be addressed. Biomedical research may produce solutions/treatments that are currently beyond our imagination.
Regards, Elaine
I have asked this same question to my rhumy and he told that when a person has dry eyes/mouth/nose and is ssa/ssb/ana negative, they call it sicca syndrome. If the patient develops joints pain and fatigue, then they call it sero negative sjogren syndrome.
One research paper says about 30% sjogren patients have only dry eyes/mouth symptoms only. So you could be part of this 30% group.
But i believe the fundamental cause of sicca or sjogren is still the same. Only the symptoms vary or sicca could be the stage 1 of sjogren disease.
Quote from: Carolina on May 03, 2018, 08:28:47 PM
I think the primary difference is that the diagnosis of Sjogren's Syndrome is based on attacks by autoantibodies on the organs/systems of the body. The autoantibodies can be found in blood samples of patients diagnosed with Sjogren's Syndrome.
Sicca, on the other hand is the diagnosis when the patients has dry eye/dry mouth, and no autoantibody activity is present in the blood, and the lip biopsy is negative (?) (I'm not sure about the lip biopsy part, actually).
The damage from Sjogren's Syndrome can often be limited to the dry eye, dry mouth symptoms, with the attacks from autoantibodies limited to only one system (the moisture producing system). With proper medication and self care, Sjogren's Syndrome can be a manageable condition.
Unfortunately, the damage from the attacks of autoantibodies in those with Sjogren's Syndrome can be far more extensive, involving just about every organ/system in the body. Because our system of diagnosis of autoimmune conditions focuses mainly on the organs/systems damaged, those who suffer from Sjogren's Syndrome may accumulate many other autoimmune diagnoses: RA, Hashimoto's, Lupus, and any of the other nearly 100 autoimmune conditions.
The diagnosis of Sjogren's has routinely been entered into my health history, and yet I have never had significant antibodies in my blood. My immunologist believes that the biochemicals that my Immune System uses to attack my organs/system are cytokines, one of the principal biochemicals that produce inflammation as part of our normal Immune Response. The list of organs/systems damaged by my Immune Disorder is very long, painful, and disabling.
One of the reasons that we discuss so many other medical conditions in this forum is that the activity of autoantibodies produces inflammation, the underlying damaging condition is an inflammatory disorder. The inflammation causes damage of so many kinds, and so, for example, we discuss carpal tunnel syndrome, which afflicts many of us, or torn meniscuses, another example of tissue damaged by chronic inflammation.
As always, my belief is that the most effective management of Immune Disorders is to treat the symptoms, until such time that the underlying cause(s) of the Disorders can be addressed. Biomedical research may produce solutions/treatments that are currently beyond our imagination.
Regards, Elaine
What an excellent summary Elaine - thanks so much for this. I find it so difficult to grasp how I can have the same disease as those who only suffer from Sicca Syndrome - which I find quite manageable most of the time myself. But I think we forget too readily that it's called a Syndrome rather than a disease for a good reason.
Regarding the epidemiology question: a UK Sjögren's expert told me that a large international trial showed that most of the damage done by Sjögren's (Sicca) is self limiting and, if well managed, it doesn't progress. So I assume it's the systemic complications that cause degeneration along with the ageing process.
The prognosis and epidemiology of lip biopsy positive/ seronegative Sjögren's is very under studied and probably very underestimated still because, certainly in the UK, we don't get to participate in any clinical trials. But it seems that the systemic complications are quite different rather than less severe. Many seronegative (ie lip biopsy positive) sufferers start with neurological symptoms - particularly SFN. This can present years before the Sicca symptoms apparently. https://academic.oup.com/rheumatology/article/55/suppl_1/i179/1795516
In my case I've been an autoimmuner since I was a child but the Sicca and alopecia areata and allergies and interstitial cystitis, slow transit bowel and UTIs and butterfly rashes weren't recognised as belonging to an underlying condition with me until my late 30s, when Hashimoto's was diagnosed. I think this is more typical of autoimmunity than is acknowledged by epidemiologists and I believe the reason that we are told that Sjögren's mainly affects older women is simply because that's when we finally get diagnosed.
Thanks everyone for your responses so far. At this point I have no diagnosis and am still trying to determine the likelihood of my Sicca symptoms having something to do with Sjogrens (and of course, what they could most likely be attributed to if they don't.)
Just before the 4-minute mark in Clements' talk, he points out that Sicca symptoms can point to Sjogren's, but apparently do so only 14% of the time. His audience is Scleroderma patients.
I understand that if Sicca symptoms point to Sjogren's, their epidemiology will be identical to that of Sjogren's, but my main question is:
What is the epidemiology of Sicca symptoms in general?
More to the point: what would a pediatrician be checking for if a male 4-year old presented with Sicca symptoms?
It could be related to Sjogren's, but I just don't fit the profile. 10% may be male, but how many male children can there be?!
I've been this way ever since I can remember. The inside of my nose has always been about as sensitive as an open blister to allergens or irritants. I had assumed that every allergy sufferer went through this, but I found out that this isn't normal at all and finally attributed it to the allergy-sensitive areas of my nose being so dry that they can't wash away what hits them.
I'm at my best only when I'm in the shower or within about 30 minutes of when I flush it all out with saline.
I produce tears and saliva, but not enough. Although I have no tooth decay or trouble swallowing, I do have a dry mouth and now see why I was always unable to wear contact lenses.
What's so baffling is that the predominant problem doesn't so much seem to be these glands, but that the nasal mucosa aren't producing - until they get irritated by any and all impurities in the air, and then they seem to do just fine...
Maybe there's a non-producing, sensitive mucosal area which triggers nearby mucosa into producing more when this happens, who knows.
Whatever I have also hasn't progressed through the decades. I've always been pretty much the same mess.
But I don't want to distract from my main question:
Male 4-year old with Sicca symptoms. If anyone can figure that one out, I think I'll be pointed in the right direction.
Another point I can add: No matter how bone dry my mucosa may be, and no matter how toxic the air I breathe, antihistamines have always magically taken away both the horrendous stinging and the allergic reaction - even though I'm told they contribute to the dryness even more, and that they're "the opposite of what you should be trying to do."
I recently became aware of the link between anticholinergics and Alzheimer's/dementia, so now I'm suddenly stuck, having to look for real answers instead.
I don't know this unfortunate man, but his symptoms are somewhat like mine. Dry eyes, constant sniffling:
https://www.youtube.com/watch?v=Gxjeummb_aU
SWF: When physicians and researchers offer varying responses to your question, I commend you for coming to the source: US! We aren't scientists or doctors. But WE have the symptoms and the disease!
You have WONDERFUL answers. I'm going to tell you what I think. Because most of us are Rxed with Plaquenil (almost immediately) to slow down/cease progression. Whichever your doc tells you. There is no "scale". No one knows if it retards the progression. We live long lives, so ...maybe so.
BUT..............if they SAY it's progressive AFTER diagnosis, ergo it's progressive BEFORE diagnosis. As has already been explained, the dry mouth/eyes can be shown to come from different sources. However, few of us have the opportunity (or need) to go further than initial dx by blood or biopsy. There are different levels of dry mouth (and eyes). Me? Mine progressed for decades. There are MANY ( M A N Y) on this board who search for SjS dx for decades. I didn't. I just thought I had dry mouth and eyes. I drank water and used eye drops. Had I asked about the dryness, a GP would have said "sicca." I am continuously surprised to hear people discuss various OTC products for sicca. There is dry mouth (caused by age, illness, etc) which can be soothed by such drugs. For SjS not so much. Why bother with such products? We need products for our disease...not a baby aspirin for a migraine so to speak.
Because of the progression, your sicca will make you more uncomfortable with each day, year, decade. As stated, because I never asked, I has atrophy of lacrimals and salivary glands by the time I asked. btw: the rheumy reluctantly offered the blood test after saying I had sicca. I really didn't understand any of it so it made little difference what "it" was. I just wanted something to help me. Needless to say, I was positive, positive, positive. Nearly five years later, I am as comfortable as I think I used to be.
All this to suggest, you may progress before you get the diagnosis you believe you need. BUT---good doctors can still Rx the meds which make your oral cavity comfortable and your eyes open and close more comfortably. The symptoms are the same. The meds are the same.
Good luck, and welcome. As to winning the "male" prize: I have been surprised over the years (on this forum) the number of men who show up. You are not alone...your numbers are smaller. Welcome, stay tuned...and remember to sip water, not guzzle!
Courtenay
Thanks for your response. I'm not sure what you're referring to by progression. I was saying I've always been this way, and that it has not progressed/gotten worse, as far as I can tell. For more years than I care to admit.
Also, because of the 14% stat Clements mentioned (as well as the statistically extreme unlikelihood of my Sicca being actual Sjogrens) I was looking for what else Sicca can mean. I.e., what do the other 86% have that might be a match for these symptoms?
You're right that I've found a good place to ask. You're all living with some of the same symptoms I have. I've wasted countless hours in trying to figure it all out myself and hope I can return the favor here with some advice of my own.
cccourt1942 I think I misunderstood part of what you said.
You may be right in speculating that my situation had progressed over the years without my knowing it, as has apparently happened to you, since you and I have learned to do what we can to deal with the symptoms, but I'm in my 50s now and so far have never had the thought cross my mind that I was in better shape with this when I was younger. I'm sure that age-related problems with dryness will be adding to my afflictions soon enough.
But if 14% of those tested (if I understand Clements right) with Sicca symptoms are diagnosed with Sjogren's (maybe 30% actually have it per happylife, as may also be the case with you) how else are the other 86%/70% categorized?
Are a large percentage attributed to "Sicca of unspecified origin" and just left with that?
My personal belief is that the number of men diagnosed with Sjogren's Syndrome in the literature is much lower than the actual number. I have several reasons for this belief:
- Doctors believe that SS is an 'old women's disease' and refuse to diagnose men, especially young males. As they are responsible for diagnosis, it becomes a self-fulfilling prophecy. My first rheumatologist fell into this camp; she told me that 'young men don't get Sjogren's Syndrome' and recommended a psychologist. It's one of a smaller number diseases where men struggle to get a diagnosis more than women (the 'hysterical female patient' bias is still alive and well). In addition, many doctors, including rheumatolgists, are woefully under-educated when it comes to SS.
- Men are significantly less likely to seek medical help for their symptoms. This has been established by many studies.
- From anecdotes of men I've talked to, as well as my personal case, I believe that seronegative SS is more common in men, though I've been unable to find enough data on men to make a strong argument. Seronegative patients (no SS-A/SS-B) have a much more difficult time obtaining a diagnosis due to an over-reliance on bloodwork by many doctors. The current SS criteria require either positive anti-SSA or anti-SSB antibody tests or a positive lip biopsy, but they are meant for the rigors of clinical testing. Individual doctors have more agency to make their own diagnoses, but rarely take it. I've written some of my concerns with the current diagnostic criteria and our medical system; suffice to say that I believe they are not serving the patient population well.
Taken together, I believe these points lead to an under-diagnosis of SS in men, particularly young men.
At the end of the day, there aren't many treatments available for SS patients (yet). That being said, I believe that if a patient is diagnosed with 'sicca syndrome', but is helped by SS treatments like Plaquenil, they should be prescribed said treatment. Our HMO and government-driven medical system strives for 'efficiency', meaning short visits, lots of bloodwork, and prescriptions based on test results. It is not well suited for complex diseases like SS. 'Sicca Syndrome', being a broad diagnosis, is fairly easy to make, but often does little to help patients understand and treat their underlying issue.
There was a very interesting John Hopkins study (http://www.hopkinsrheumatology.org/2015/12/leap-winter-2016/) that found two distinct interferon pathways for SS patients that the authors hypothesized could contribute to some treatments working better for some patients than others. Unfortunately, I haven't seen any follow-up work since the article was published in 2015.
Whatyousjo
I could not agree more !!
Im male 42, seronegative. I do not want to do lipbiopsy as it is invasive and a diagnostic tool.
But my symptoms can only be explained by sjogren syndrome.
Inflammatory dry eyes
Dry mouth
Post nasal drip
Rhinitis
Atypical right side face pain
Upper right abdomen chronic discomfort
All these symptoms appeared in span of 4 weeks and have not gone away.
Regards
"...my symptoms can only be explained by sjogren syndrome."
This is where I'm still stuck. I don't yet know everything else Sicca symptoms may indicate, so I'm hoping to compile a list, and at least informally rule out the alternatives to Sjogren's, considering the statistical possibilities for each as well as my observations.
Might I test positive for something else while still only having Sicca symptoms? Maybe there's something that is more common in male children and likely to not get any worse over 50 or so years? I thought Sjogren's generally progresses, where whatever I have hasn't. I don't yet have a clue.
(I'm actually in much better shape than before, since I've figured out to target the dryness instead of just the allergies. It wasn't soon enough to have kept it from destroying most of my life, but I should be grateful for small victories.)
Another thing: I had a turbinectomy as a teenager which obviously didn't do me any favors in this respect. But this came from my having ignorantly, violently blown my nose for a few years prior - because of the same horrible stinging, which I take it came from the same dryness from before the operation.
So there's also Empty Nose Syndrome to consider, although the dryness is still about the only thing I have in common with ENS sufferers. No vertigo, etc.
If I do stumble onto something else, this would be what I should be looking to deal with. Even after having lived with this probably from birth I'm still surprised to discover remedies for making life easier that had never occurred to me so of course it would help to be looking in the right direction.
Then there's the added possibility of there being a long-term sinus infection causing or contributing to the postnasal drip, although I never have sinus headaches. I recently read about permanent, microscopic biofilms, which can apparently protect remnants of an infection, so that anything done to combat them never permanently wipes them out.
As I said, my situation is also compounded by severe nasal allergies, which are unavoidable several months out of the year. Sicca plus allergies, what a combination...
I think WhatYouSjo nailed it: "...short visits, lots of bloodwork, and prescriptions based on test results. It is not well suited for complex diseases like SS"
But I don't want to go in too many different directions here before I find a list of what else Sicca might point to and try to rule it all out.
I would encourage everyone to see Dr. Clements' presentation. I guess I could email him for such a list and hope he'll find the time to respond.