UPDATE: Rheumatologist Sjogren's Incomplete?

[DeadGirl75]

I went to the rheumatologist today and spoke with her. She said that with Sjogren's white blood cells are actually lower than with any other AI. My blood work came back negative for RA, Lupus and Sjogren's or and any other AI syndromes. But she did say that my symptoms could apply to any number of things and that even with a positive biopsy that doesn't mean I have Sjogren's. She called it Sjogren's Incomplete. She did put me on Plaquenil to treat my symptoms and I have to go back in six months. When I mentioned the seronegative aspect, she did a little eye roll. She said that that's not the official term or along those lines. I had people with me this time, so I don't know if that played a part in getting medication or not. She wants me to taper off the steroids for certain. But if I do start to swell again, to let her know. She also said that Plaquenil takes a long time to see any results so I shouldn't get discouraged.

Has anyone else heard of Sjogren's Incomplete?

[cccourt1942]

Hi...a new one for me....
      I did google and to me it seems as this is a name for sicca Syndrome.  It actually says "it can have the 'face' of Sjogren's."  Pretty broad implications there as there are so many symptoms.  I presume they are referring to eyes and mouth.

     Google and see.

It does sound like a run around.  As to Seronegative, she can roll her eyes all she wants to, but on this forum alone, we are littered with seronegative SjS members.  As to "Incomplete SjS"---you are the first I've seen mention it.  Weigh those amounts!!!
ccc

[DeadGirl75]

It was really weird. I told her that I belonged to a forum and was finding out about the syndrome because I had never heard of it. So, when I mentioned that people were seronegative, she was like that's not even the proper term. I don't know. I even brought the printout about seronegative that someone from here had given me. She seemed to get irritated. Also, saying that WBC are lower in Sjogren's? It doesn't make sense to me, but I'm no doctor. She did put me on Plaquenil, but I kinda feel she did that just to shut me up. I see my family doctor in Dec and I'm going to discuss all this with him. Something isn't right. She said that all my blood work was negative for any type of AI issue, but did say to get my thyroid checked due to the nodule. I'm losing hair, toenails, but no weight...sadly.  *Sigh* I don't know. Is there something other than Sjogren's that can cause this? I just feel really confused.

[Pete0211]

I did a quick search on google and Incomplete Sjogren's (ISS) seems to be used in a clinical / research environment somewhat often. /shrug. I think the doc is a dork complaining that a patient may not be using the "proper" terms, and I'd probably tell her such.

[Sooki]

American College of Rheumatology (ACR) gives the following criteria for SjS:

Case definition requires at least 2 out of the following 3:

Positive serum anti-SSA and/or anti-SSB or [positive rheumatoid factor and ANA ? 1:320];
Ocular staining score ? 3;
Presence of focal lymphocytic sialadenitis with focus score ? 1 focus/4mm2 in labial salivary gland biopsies.

So, antibodies in the blood not required if eye symptoms and lip biopsy are positive.

This article compares the cases diagnosed with these criteria to those diagnosed with American-European-Consesus-Group (AECG) and Latent Class Analysis (LCA). 
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3349440/

There is a lot that isn't known about autoimmune causation and effect.  It seems to me if a patient has symptoms and the recommended treatment for Sjs helps ameliorate the symptoms, it should be considered regardless of diagnosis.

Because this isn't a straightforward disease, sometimes it helps to find a new doctor with a different understanding of the disease manifestations.  Seronegative is a much used term in the literature.  Her comments on seronegative and incomplete Sjogrens suggests that her reading and experience with Sjs isn't very broad.

[Maria3667]

My rheumatologist said something along the same lines. He explained there's no standard checklist and no international concensus on what the syndrome implies exactly. He said most diagnosis are made on the basis of symptoms, rather than on bloodtest. Sometimes a positive lip biopsy might count, but it doesn't rule out other syndromes/diseases. He prefers to call it 'sicca syndrome'. That sums it up for me - not enough is known about this weird affliction - hence the vast difference in symptoms.

[Wal]

I'm sorry you had this response. I got a similar one at my rheumy appt. I won't be going back after he spent 5min telling me I'm sooo healthy and then charged me $350 for the consult!!! I did see where she mentioned you have thyroid checked. If you haven't, that's important because from what I read it can cause similar symptoms.

[MAT51]

My rheumatologist said something along the same lines. He explained there's no standard checklist and no international concensus on what the syndrome implies exactly. He said most diagnosis are made on the basis of symptoms, rather than on bloodtest. Sometimes a positive lip biopsy might count, but it doesn't rule out other syndromes/diseases. He prefers to call it 'sicca syndrome'. That sums it up for me - not enough is known about this weird affliction - hence the vast difference in symptoms.
[/quote

I'm unsure whether Deadgirl75 has had a lip biopsy with positive result but if so, and eyes are dry then I have always assumed this is fairly definitive of Sjogrens? But perhaps this is only as a secondary to other AI disease (S) . I don't think Sjogrens can be diagnosed as a stand alone/primary  disease without positive ANA or ENA? But then again, surely, if a lip biopsy has been done, nothing else would cause lymphocytes to cluster around salivary glands - although I suppose it depends if there are enough to meet the diagnostic requirements of the Cheesom's criteria for Sjogrens?

I'm guessing stricter adherence to SS criteria this only has relevance if the disease is affecting more than the eyes and mouth i.e organ involvement, nervous system or joints and tendons. Otherwise it's just a case of using local therapies to try and stay moist?

[DeadGirl75]

My rheumatologist said something along the same lines. He explained there's no standard checklist and no international concensus on what the syndrome implies exactly. He said most diagnosis are made on the basis of symptoms, rather than on bloodtest. Sometimes a positive lip biopsy might count, but it doesn't rule out other syndromes/diseases. He prefers to call it 'sicca syndrome'. That sums it up for me - not enough is known about this weird affliction - hence the vast difference in symptoms.
[/quote

I'm unsure whether Deadgirl75 has had a lip biopsy with positive result but if so, and eyes are dry then I have always assumed this is fairly definitive of Sjogrens? But perhaps this is only as a secondary to other AI disease (S) . I don't think Sjogrens can be diagnosed as a stand alone/primary  disease without positive ANA or ENA? But then again, surely, if a lip biopsy has been done, nothing else would cause lymphocytes to cluster around salivary glands - although I suppose it depends if there are enough to meet the diagnostic requirements of the Cheesom's criteria for Sjogrens?

I'm guessing stricter adherence to SS criteria this only has relevance if the disease is affecting more than the eyes and mouth i.e organ involvement, nervous system or joints and tendons. Otherwise it's just a case of using local therapies to try and stay moist?

Yes, I've had a positive lip biopsy for Sjogren's. I also have a 9mm nodule on my theroid. I see my family practitioner in December and I'm going to have him refer me to and endocrinologist.  I have a ton of Sjogren's symptoms, but I have no clue what it does apart from regulating weight.

I'm on Plaquenil now and she wants me to ease off the steroids, so I'm only taking every other day for now and then taper off. She said if my parotid swells again to let her know.

[MAT51]

Thanks for explaining. I guess that it must depend on the way in which the lip biopsy is positive or the extent to which it is then because mine was 100% definitive for Sjogren's -even if I also have other things it is absolutely clear that I have SJS and no one can dispute it now apparently  -even though my symptoms are more neurological than Exocrine.

[Jasper]

If I were you, I would find a new Rheumatologist, preferably one at a large teaching/university medical center. The doctors at these facilities are usually more current on diagnosis and treatment.

There are many people who have Sjogren's Disease  who do not have positive SS-A or SS-B. Only about 50-60 percent of people test positive for SS-A and the percentage is even lower for positive SS-B.

Some people with Sjogren's Disease have altered lab results, including altered CBC results, such as WBCs. All people with Sjogren's Disease do NOT have abnormal lab work or abnormal WBC.

You have a positive salivary gland biopsy, which is indicative of Sjogren's Disease. It is one of the recognized criteria. I am not sure why your current Rheumatologist is ignoring this positive biopsy.

As stated above, I would suggest seeing another Rheumatologist.

[MAT51]

My rheumatologist said something along the same lines. He explained there's no standard checklist and no international concensus on what the syndrome implies exactly. He said most diagnosis are made on the basis of symptoms, rather than on bloodtest. Sometimes a positive lip biopsy might count, but it doesn't rule out other syndromes/diseases. He prefers to call it 'sicca syndrome'. That sums it up for me - not enough is known about this weird affliction - hence the vast difference in symptoms.

I think Sjogrens is actually a disease for some, rather than Sicca Syndrome. I am not severely affected in my eyes or mouth, but my small nerve fibres everywhere, my autonomic nervous system and joints are very problematic, as is my GI tract. So for me personally I wouldn't feel well served by  a rheumatologist who just called it Sicca Syndrome, as my first one did. According to Dr Julius Birnbaum, many who present with a neurological manifestation first, only present later with full blown Sicca Syndrome.

[DeadGirl75]

If I were you, I would find a new Rheumatologist, preferably one at a large teaching/university medical center. The doctors at these facilities are usually more current on diagnosis and treatment.

There are many people who have Sjogren's Disease  who do not have positive SS-A or SS-B. Only about 50-60 percent of people test positive for SS-A and the percentage is even lower for positive SS-B.

Some people with Sjogren's Disease have altered lab results, including altered CBC results, such as WBCs. All people with Sjogren's Disease do NOT have abnormal lab work or abnormal WBC.

You have a positive salivary gland biopsy, which is indicative of Sjogren's Disease. It is one of the recognized criteria. I am not sure why your current Rheumatologist is ignoring this positive biopsy.

As stated above, I would suggest seeing another Rheumatologist.

I'll be talking to my family doctor. I was positive for stage two.

This is what the biopsy read:
Clinical History
M35.00 SICCA syndrome. Rule out Sjogren's disease.

Gross Description
A. Lower lip: The specimen consists of a mucosal biopsy with underlying soft
tissue measuring 10 x 7 x 5 mm. The specimen is trisected and entirely
submitted in A1.

kl/7/20/2016

Microscopic Description
A. Lower lip: Sections show unremarkable squamous mucosal tissue and
underlying skeletal muscle tissue. Intervening the stroma around the skeletal
muscle tissue are numerous lobes of labial salivary gland tissue. There are
very focal atrophic changes. Overall the focus score is a moderate infiltrate
or less than one focus of lymphocytes (defined as 50 or more lymphocytes or
plasma cells per focus) per 4 mm2. Overall this is consistent with a Grade 2
for evaluation of evidence of Sjogren syndrome.

I'm not exactly sure what grade two is, but to me, this says I have Sjogren's. I'll be speaking to him and relaying this information that you guys gave me. I just don't get it, why do med professions have to be so resistant to new terms and different studies...

[Jasper]

You might find this article on Seronegative Sjogren's Disease interesting and informative:

https://issuu.com/mppub/docs/mn_physician_june_2016

This article is in MN Physician, June 2016, and was written by Dr. Paratoo Fazeli, Director of Lupus Clinic at the U of Minnesota.  Her clinical focus and her expertise is on patients who have Sjogren's Disease and patients who have Lupus.

Go to pages 14 and 15 and you will see the article. You can zoom in or expand the article in order to read it.