Study Raises Questions about Sjogren's and Plaquenil

Image Source

For many patients with Sjogren's, Plaquenil (hydroxychloroquine) has long been the go to drug that their doctors prescribe.  It is often the first line of attack before other more aggressive therapies are added such as methotrexate or rituximab that have bigger impacts on the immune system.

The other day one of my favorite bloggers, Julia from Reasonably Well, wrote about how she's been feeling since discontinuing her Plaquenil. 

I can tell that my meds have changed. I had almost (but not quite) forgotten what these aches and pains feel like, but they're back…I am re-acquainting myself with those symptoms that it tames: the overall flu-like and feverish feeling -- aka malaise -- and joint pain." (Click here to read more).

I had a similar experience when I stopped taking plaquenil.  It was one of those situations where I didn't realize the medication was having such an impact until I stopped it.  My joint and muscle pain went up and my energy levels went down.  Did the drug eradicate my fatigue and pain?  No, but it did alleviate some of the symptoms and improve my quality of life.  I have been on enough drugs to know when one helps.

So I was very interested when I saw on Twitter this study presented at both the American College of Rheumatology (ACR) and the European League Against Rheumatism's (EULAR) annual meetings:  Inefficacy of Hydroxychloroquine in Primary Sjogren's Syndrome: Results of the Joquer Randomized Placebo-Controlled Trial in Primary Sjogren's Syndrome.  Yes, that's a mouthful, but for those of us with Sjogren's and on plaquenil, it's a subject that's pretty relevant.

I haven't yet been able to find a published article with the study findings, but I was able to access the abstract.  The key findings cited:  At six months, 19.2% of patients receiving placebo and 19.6% of patients treated with HQ had a favorable response….No significant difference was observed in the evolution of systemic disease activity, dryness symptoms and quality of life. (Click here and go to L9 to read the abstract).

To summarize, the study found that at six months hydroxychlorquine (plaquenil) had no impact on a range of Sjogren's symptoms nor on disease progression.  While previous studies of the drug's effectiveness have been mixed, this study is considered important because it was placebo controlled, done with a larger population (still only 120 patients), and occurred over a 6 month period.  

So what does this mean for those of us who are having some success with plaquenil?  I'm really not sure. To start with, until the results are fully published it is hard to know what the complete findings were. For instance, I have since found other references (like this from the Journal of Musculosketal Medicine) which indicate that the complete results are less clear than the abstract--that patients who took the drug for one year did have significant improvements. I'm assuming full publication will help clarify some of these points. 

I am always very excited to hear about new Sjogren's studies, but given the limited information available I was a bit concerned to see some of the early reaction online. I have already seen a few doctors--on Twitter and in podcasts -- making reference to the same findings I have seen, with some indicating it may cause them to revise their treatments for Sjoggies. 

This gave me pause. I'm just one patient, but I would urge caution to anyone currently treating patients with the drug. I have first hand knowledge of the benefits of the drug and know from several other Sjogren's patients have seen them as well; in short the drug has helped at least some of us. Since there are so few treatments available, it seems important to have plaquenil as an option for others seeking relief for their symptoms until other treatments are available.  Taking one arrow out of an already thin quiver doesn't seem like progress.

While watching my doctors at work, I've observed that part of treating patients with autoimmune disease involves trial and error. I don't view this as an argument against evidence based medicine but rather an indication of the importance of clinical judgement when treating patients with the same disease diagnosis but divergent symptoms and disease trajectory. I don't see how 60 patients (number of study patients given plaquenil) can encompass all the variant manifestations of the disease. 

I hope doctors and scientists use this research as a motivation to do further studies on Sjogren's, as opposed to considering the issue of plaquenil settled. We just know so little about this whole disease that I hope each piece of knowledge we gain is seen for what it is--a little bit of a puzzle revealed, but one that will take years and many studies to become clear.  Hopefully, one day in the not to distance future, newer, more effective treatments will make this whole thread irrelevant. 

Have you taken plaquenil and what was your experience?  

Top Ten List from the Sjogren's Conference

Majestic DC Cherry Blossoms

I attended the Sjogren's Syndrome Foundation's National Conference this past weekend and I am so glad that I did.  It was a huge effort to travel and participate in the conference, but I can say with complete certainty that any fatigue I am feeling now was well worth it.  It was super amazing to be around other Sjoggie's and have the opportunity to compare notes about how we cope with the disease. Never could I have imagined that I would be sitting in a room with almost 400 Sjogren's patients listening to doctors talk solely about SS. What a relief it was to know that the person sitting next you on you left and right could relate to what it is like to have this disease.

The doctors who presented are not only Sjogren's experts, but advocates in their own right, trying to improve research, treatments, and clinical care for their patients and others around the country.  After this weekend, I am even more inspired and ready to keep working to spread awareness about Sjogren's.

I thought I would share my top ten list from the Sjogren's Conference ranging from the medical to the social.  Now this isn't a Letterman style comedic list as we all know Sjogren's is serious.  Of course, there was so much more beyond this list from the Conference so I promise there will be other tidbits in upcoming posts. 

Top Ten List

1)  One of the biggest obstacles to improving the lives of Sjogren's patients is convincing pharmaceutical companies to fund and invest in research and clinical drug trials for Sjogren's. 

2)  Myth Busting that Sjogren's is more than a "dry mouth and dry eye disease" is critical. I actually didn't meet anyone this weekend who only had these symptoms.

3)  The cognitive symptoms associated with Sjogren's such as brain fog are not thought to be progressive like other diseases such as alzheimers. What a relief!

4)  Up to 50% of Sjogren's patients with neurological symptoms do not present with SSA or SSB antibodies.  This can cause delays diagnosis. 

5)  The average time to diagnosis of Sjogren's has decreased from an average of 7 to approximately 5 years in part thanks to the Sjogren's Syndrome Foundation's (SSF) 50% in 5 years Campaign.  But we still need to get it down to 3.5 years and even lower and lower.

6) Clinical guidelines for how to treat SS and its many manifestations will be coming out soon thanks to the SSF.  These will hopefully help more doctors help us.

7) Venus Williams and Shannon Boxx fellow Sjogren's patients are working with the SSF now to bring attention to the disease. Yeah! Having high profile folks involved, helps bring attention to the disease. (I have an important story to tell here for my next post so stay tuned) 

8)  Sjogren's is as prevalent as breast cancer.  This means that the number people diagnosed with Sjogren's is equivalent to the number of people currently with and who have survived breast cancer.  

9)   Sjogren's is the 2nd most prevalent autoimmune disease. Only 2nd to RA. Awareness and funding for Sjogren's should be on par with other high prevalence autoimmune disease. This is not the case today.

10)  Sjogren's will only become a household name if we stand up, get involved and talk about our disease.  We need to be an army of over 3 million.

Any other readers out there who attended the conference, please feel free to add and keep this list going!  Remember share this list or your own with others to keep spreading the word.

We Need More Research for Sjogren's Syndrome

Sjogren's Awareness Bracelet

I had a hunch for some time that there weren't enough studies on Sjogren's Syndrome (SS).  In reading articles about recent autoimmune disease research, it seemed there were more studies on other diseases.  Well, it turns out that my hunch might not have just been a hunch.  In a review published last month in Expert Opinion in Pharmocotherapy evaluating treatments for Sjogren's, the authors found that there are have been few large studies on how to effectively treat primary SS.  Click here for the abstract. 

Given the high prevalence of Sjogren's among autoimmune diseases this should not be the case. 

Most larger random controlled trials (RCT -- which are the gold standard in scientific research) have focused on evaluating how a treatment effects symptoms of dryness for SS patients. As I have written about before, Sjogren's is more than a dry mouth and dry disease and patients can also experience other debilitating symptoms such as fatigue, muscle and joint pain, neurologic, and organ involvement.  It's far from a fun combination of symptoms and more research clearly needs to be done on how to improve these serious complications of SS.

A few points this article really hit home and made me realize that Sjogren's patients should expect more from the scientific community and must continue to push for more comprehensive research whenever possible.

• A good deal of the treatments used to for symptoms of SS is based on research from other autoimmune diseases such as SLE and RA.  This is even true of the use of corticosteroids and methotrexate.

• Studies involving Sjogren's patients often involve a small number of patients and in larger studies the focus tends to be on symptoms of dryness or glandular aspects of the disease.  

• Biologic therapies targeting B cells seem to the most promising area of treatment including the use of rituximab and epratuxumab.  Surprisingly, there have been more studies on rituximab and greater evidence for its use in Sjogren's patients than there have been for immunosupressive treatments such as methotrexate.  However, rituximab is still considered an off-label treatment for Sjogren's.  I'm not a doctor or scientist, but this doesn't seem to make much sense.

• The good news is that authors see the treatment of Sjogren's improving as biologic therapies become more specific. Two trials are in thew works using Belimumab (Benlyista), which targets the activation and stimulation or over-stimulation of B cells.  It has already been approved in the treatment of lupus.

I agree with the authors that we need more and larger trials specifically designed for Sjogren's patients, and especially trials focusing on the systemic aspects of the disease.  Sjogren's patients should not just be treated based on outcomes from trials and research for other autoimmune diseases.  That would seem obvious, but it's not the case today. 

A Modest Proposal

Here's an idea. It might seem simple, but to me it would be a big improvement if there was a way for doctors to share and compare the way they treat their patients. This would be a Sjogren's Syndrome Treatment Registry, similar to the Sjogren's International Collaborative Alliance funded by the NIH. Specifically, this would mean there would be a database kept by each hospital about each patient treated with SS, including symptoms, treatments, and results. It would be anonymized, of course.  

From my discussions with physicians, this registry would be quite helpful to them. Most say they are working with little actual data, and are largely confined to the clinical patients and results they see. (This is a larger problem in medicine I realize, but for specialized diseases I believe it is even more acute.) This data could also help provide insight into what trials should be funded.  Furthermore, a registry could help doctors and scientists systematically match specific disease complications to which treatments are successful or unsuccessful.  As many of us with autoimmune disease know, what works for one patient does not always work for another and rheumatologists often try a number of treatments before finding one that works.  

So, all this said, why is Sjogren's ignored to date?  I don't know, my husband claims we have bad branding. Could it be the name? It is hard to say and spell and that makes it hard to raise awareness in some ways he claims.  Maybe there's something to it. I don't know, but there doesn't seem to enough action in the area at the moment. 

Of course, more studies and funding are not only needed for Sjogren's but also for autoimmune diseases generally.  And it is the perfect time for all of us to put on our lobbying and activist hats because March is Autoimmune Disease Awareness Month. There is lots of work to be done. Our collective voice can make a difference.

How do you think we can improve research and treatment for SS and other autoimmune diseases?