Home > published mauscripts of interest > American College of Rheumatology classification criteria for Sjögren’s syndrome: A data-driven, expert consensus approach in the Sjögren’s International Collaborative Clinical Alliance Cohort – Shiboski – 2012 – Arthritis Care & Research – Wiley Online Library

American College of Rheumatology classification criteria for Sjögren’s syndrome: A data-driven, expert consensus approach in the Sjögren’s International Collaborative Clinical Alliance Cohort – Shiboski – 2012 – Arthritis Care & Research – Wiley Online Library

American College of Rheumatology classification criteria for Sjögren's syndrome: A data-driven, expert consensus approach in the Sjögren's International Collaborative Clinical Alliance Cohort – Shiboski – 2012 – Arthritis Care & Research – Wiley Online Library.

(thanks @AmiDauhoo for notifying me)

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  1. 28 March 2012 at 00:44

    Very interesting. Thanks for posting this!

    I was diagnosed with Sjogren’s on the basis of blood work (anti-Ro/SS-A > 8 and an ANA of 1:640) and symptoms. My rheumatologist has never felt it necessary to do a biopsy. With those blood test results and my symptoms–dry eye, joint pain, fatigue, etc.–it’s pretty clearly either SJS, SLE, both, or an overlap. These new proposed diagnostic guidelines strike me as unnecessarily invasive for people with positive blood tests. What’s your take on it?

    I understand the need to be careful with more aggressive medications (e.g., Rituximab), but so long as the treatment would be appropriate for any of the illnesses indicated by blood tests and symptoms (SLE, SJS, or RA seem especially fond of mimicking each other), I don’t understand requiring additional tests before using a treatment. Am I missing something?

    • 28 March 2012 at 08:06

      Thanks, for your comment.

      In answer to your questions in my personal opinion I would put forward two statements:

      1) As before and comparable to other rheumatic diseases one should always keep in mind that the main purpose of proposing classification criteria is to create a group of patients that has maximum simliarities in order to make study results more reliable and reproducable. Especially diseases like Sjögren’s syndrome and SLE can have a wide spectrum of signs and symptoms in daily clinical practice, for study purposes that should be a bit less heterogeneous, however in daily clinical practice the rheumatologist and the patient of course deal with the broad clinical spectrum. If one does not meet the classification criteria, can one still be diagnosed as a Sjögren’s sydnrome patient, that will be a question without a single answer and typically will be continuously debated. However based on the context and the broader picture one can at least argue that someone is more or less likely to have Sjögren’s sydnrome. I will explain this in statement 2). In daily clinical practice there are also quite a lot patients whose disease course is followed by the rheuamtologist because they are “likely” to have SLE (SLE-like if you want), but just do not (yet) meet the SLE classification criteria. That is quite similar to what I just stated about Sjögren’s syndrome. However, as you pointed out: Sjögren’s syndrome has some mimics that should be ruled out.

      2) I wrote a thesis on Sjögren’s syndrome 5 years ago (Classification and follow-up of Sjogren’s syndrome- Usefulness of objective parameters in clinical practice), and it is mainly focused on diagnostic aspects, if you are interested you can download or read it online. In daily clinical practice in my personal opinion salivary gland biopsy is not always required to diagnose Sjögren’s syndrome (but for study and trial purposes it is, I even let patients have one before and after treatment with Enbrel in a pilot study, also included in my thesis). However serological (anti-SS-A or preferably anti-SS-B antibodies) and histological (biopsy results, including immunohistochemistry, i.e. staining for percentage IgA containing plasmacells, should be less than 70%) are the hallmarks of diagnosis being the most objective parameters. Now when you have a patient with sicca symptoms that has either SS-A and/or SS_B antibodies, a positive IgM rheumatoid factor, hypergammaglobulinemia, an elevated ESR with in general low or normal CRP, in my opinion you have your diagnosis Sjögren’s syndrome and obtaining a biopsy is not necessary because of the typical blood results picture. A slight to moderately elevated CPK and/or a low normal or low vitamin B12 are blood results that are also more often seen in Sjögren’s syndrome and add up to the presumption of Sjögren’s sydnrome already made. However when you have “only” anti-SS-A (also seen in normal population, rheumatoid arthritis and SLE) or anti-SS-B (also seen in SLE, so a bit more specific then SS-A) but not the other characteristic blood results (no elevated ESR, no hyeprgammaglobulinemia etc), in my opinion you are mot there yet and it is too little to diagnose Sjögren’s syndrome. In daily clinical practice you have all the combinations of blood results in between of course. On the other hand when you do not have anti-SS-A or anti-SS-B (about 20% of Sjögren’s sydnrome patients) in my opinion a biopsy is required to make the diagnosis. Whether you do that is dependent on the degree of suspicion: when all other blood counts are also normal, the chance of finding a positive biopsy is quite low, so unless the patient insists on ruling Sjögren’s syndrome out, also in probably not-Sjögren’s syndrome patients most of the time a biopsy would not change your diagnostic conclusion. So I preserve having biopsies taken to patients with moderate to high likelihood of having Sjögren’s syndrome but no conclusive blood results, patients with a low suspicion (normal blood results) and sicca symptoms that are willing to have a biopsy taken to rule the diagnosis out, and patients with marked salivary glands enlargement (these should always have a biopsy which can confirm the diagnosis, but also lead to other diagnosis like a lymphoma or sarcoidosis). In the latter case (when enlarged salivary glands are present) you should “always go where the money is” so to say.

      I hope by sharing these considerations you can put your own picture in prespective, you’re welcome,

      Greetings from the Netheralnds,

      Michiel Zanabelt, MD, PhD, rheumatologist.

  2. 28 March 2012 at 11:31

    Thank you for the clarification! I appreciate it.

  3. 28 March 2012 at 17:48

    Thanks for your kind comment.

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