Dealing with a rare disease such as relapsing polychondritis: “food for thought”…

Working as a rheumatologist in an average peripheral hospital means occasionally being confronted with conditions that are quite rare. One of these conditions is Relapsing Polychondritis (RPC), a quite typical condition in terms of how it can be recognised just based on the clinical presentation. However it is a condition not seen that foten at all in a regular rheumatology outpatient clinic.

The weird thing in medicine is that rare conditions happen to be encountered clustered in time in two or three cases shortly after one another, followed by a quite long period in which one does not see the same condition.

That’s also what happened to me for Relapsing Polychondritis. As a trainee in rheumatology I was actually lucky to meet two patients with Relapsing Polychondritis within a month’ s time. Ever since I have not seen a patient with Relapsing Polychondritis untill last month, and guess what…the case was followed by another one in the same month, so again “two-in-a-row” after not having seen RPC in about 4 years.

My selection of ACR2012 Sjögren’s Syndrome Abstracts (2)

This year’s annual scientific meeting of the American College of Rheumatology (ACR) included 76 accepted abstracts on Sjögren’s Syndrome. In this blogpost I name a selection of them that may have clinical relevance for rheumatologists in daily practice together with a few more basic research oriented abstracts that I personally consider of interest.

My selection of ACR2012 Sjögren’s Syndrome Abstracts (1)

It is early November, and that is usually the time of the year for the annual meeting of the American College of Rheumatology (ACR). Indeed the ACR 2012 convention has just ended while adding this blogpost. It took place in Washington, D.C., from November 9-14. I did not physically attend it, but -and that’s new- I would almost say it has been the first scientific meeting I could almost attend virtually (live video- and aaudiostrems from the meeting would provide a strikethrough for the word “almost” in this sentence). Thousands of tweets from all kinds of authors attending the ACR 2012 convention passed in my Twitter timeline. Whilst on a previous occasion one could make a nice survey of topics discussed at the ACR or (european counterpart) EULAR meetings by downloading a transcript of all tweets with the meeting’s hashtag via Symplur, on this occasion one could speak of a twitter tsunami. The tweets containing the hashtag #ACR2012 were so numerous that the previously mentioned transcript downloads via Symplur no longer satisfied my objective to obtain a nice survey of the meeting (this might change when Symplur decides to add more query features then only filtering based on a specified time-interval, i.e. if one could filter all tweets containing the #ACR2012 hashtag including the word “gout” it could still deliver a nice transcript of what came by via Twitter on the topic gout).

Dutch study suggests that COX-2 inhibiting NSAIDs should also always be prescribed with gastroprotective drugs

In clinical practice conventional (classic) NSAIDs are usually prescribed together with proton-pump-inhibtors (PPI) as concomitant gastroprotective agent (GPA). In patients not at risk for gstro-intestinal problems the COX-2 inhibtors group within the NSAIDs are not necessarily prescribed with concomitant GPA. I.e. when the COX-2 blocking agents were introduced, the reduced risk for gastro-intestinal bleedings was an important factor for drug registration by the authorities as well as in marketing for the pharmaceutical companies. However, from the classic NSAIDs the risk of gastrointestinal bleeding is well known as side-effect, especially when not (continuously) taking PPI. For the COX-2 inhibitors data on gastrointestinal complications when not or not continuously taking PPI were relatively lacking. A Dutch study was performed (and published today in Arthritis and Rheumatism) on the effect of adherence to a gastroprotective drug regimen with COX-2 blocker use. The investigatrs concluded that BOTH for classic NSAIDs as well as for COX-2 blockers the risk of developing gastrointetinal complications rises remarkably when PPIs are not taken as concomitant medication on a daily base.

Further analysis found that for every 10% decrease in GPA adherence, there was a 9% increase in the risk of gastrointestinal complications. “Our findings show that with every three day reduction of GPA coverage per 30 days of COX-2 inhibitor use, the risk of upper GI events increases 9%,” concludes Dr. Valkhoff.

Clinical research on Sjögren’s Syndrome continues, also in the Netherlands

Fatigue remains a major symptom encountered in Sjögren’s Syndrome, and this phenomenon has already been documented in quite a number of studies. A recent Dutch study (published in this month’s Annals of the Rheumatic Diseases) adds to that series and suggests that fatigue in patients with Sjögren’s syndrome might be reduced by targeting both physical activity and physical activity cognitions.

Find a link to the abstract here.

Basic research on Sjögren’s Syndrome continues, also in the Netherlands

A recently published thesis by Nienke Roescher from the University of Amsterdam further contributes to the difficult and ongoing search on how to tackle the disease Sjögrenś sydnrome. Although as a rheumatologist I am more into clinical research, the evolving knowledge obtained from basic (laboratory) research is of invaluable importance in guiding the way to find a cure for this disease.

Nienke Roescher concluded her thesis as follows:

The research presented herein summarizes the current knowledge on cytokines and their presence in SS, it identifies immunological targets in mice with an SS-like disease and shows that local gene therapy can be successful for the treatment of the inflammatory salivary gland component of a SS-like disorder in mice. The chance for success of this approach depends on proper timing and should be aimed at the right target.

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)