RheumNow Podcast Scleroderma And The Lung (3.1.19) Save

This is the RheumNow podcast. It's March 1 and I'm Doctor. Jack Cush, executive editor of roomnow.com. This week, new signs of dermatomyositis you may not have known about. And what's the new fibromyalgia hangout?

Pain clinics, you say? And, Oh my, ONJ and bisphosphonates. This and more, but first, great rheumatologists like us usually go to great meetings. And I'm telling you the next great meeting is RoomNow. Live.

Check it out. It's happening in three weeks. Artie Cavanaugh and I are co hosting it. The faculty is stellar. The discussions and interactions are going to be unrivaled.

Very cool meeting. Be there. You can also sign up and watch it all live from home. So at the top, we're going to talk about the Shingrix vaccine. It was a nice analysis of this in Annals of Internal Medicine that looked at the new recombinant not live virus, Shingrix vaccine that they abbreviate as RZV and compared it to the Zostavax live virus vaccine they called RZL.

And in this cost efficacy analysis, they showed clearly that the new live virus Shingrix vaccine is highly cost effective much more so than the older vaccine which we know would lose efficacy with increasing age. This one does not, it's ninety plus percent in all age groups. And they also showed that it was effective even if patients were only going to get one injection and not that required two. So if you're looking for a good reason to use the newer vaccine and you can use it in based on this cost efficacy. What we don't know though, of course, is how safe is it in our patients with rheumatoid arthritis and lupus and other conditions that need it.

There are no studies of this new virus in patients with our diseases and the manufacturer is not planning on doing such studies. If you talk to them, you should remind GSK it's a study they should do. On the other hand, my anecdotal experience is that we've given it to a lot of patients. A lot of patients have received it inadvertently. I've not seen any major flares of their autoimmune disease once they've received this, but again, that's anecdotalism.

I wouldn't take that as true data. An interesting study comes from Israel. It's a cross sexual analysis of RA patients, almost twelve thousand RA patients compared to almost fifty four, fifty eight thousand controls, looking at the incidence of subclinical or clinical thyroid disease in RA patients and controls and looking at both hypo and hyperthyroidism, there actually is more thyroid disease in RA patients, sixteen percent versus eleven point seven percent with an odds ratio of about one point four two. So a forty two percent increase odds of thyroid disease in your RA patients. This has been seen in other autoimmune diseases.

It's not a shocking idea. The question is, should you really focus on this and make, you know, all after thyroid disease in patients with RA? I can tell you I've done this for many years. I think it's obvious when it's obvious. I think most of the thyroid diagnosis are made by other people.

I think fishing for abnormal TFT's in patients with RA is probably not a useful maneuver, but just recognize it does happen. Still's disease, again, my favorite disorder, a nice meta analysis of a cohort looked at the predictive value of multiple features of Still's disease especially in distinguishing Still's disease in the adult from an FUO and an infectious FUO and what they said in their, report was that arthralgia rash, prodromal sore throat, neutrophilia, and a ferritin that's greater than five times normal and LDH as well were actually more predictive of Still's disease than infectious FUO. Also, a three times, daily peak of fever is not typical of Still's disease. You know, Still's disease has a quotidian, a one time peak, and or a double quotidian, a two time peak. It's either Q24 or Q12 hours.

Having multiple peaks at three or more per day is more predictive of an FUO than Still's disease. An interesting report came from a medical student site that listed skin findings with dermatomyositis. I posted it because I was surprised that the medical students did such a good job in what they listed and they just listed, you know, key clinical findings in dermatomyositis. Now, you know about the common ones, Gottrin's lesions, Gottrin's papules, V neck shawl, periungal erythema, calcinosis and the heliotrope. Everyone knows those five or six.

But did you know about the other ones? A holster sign. It's an erythematous lesion around where your holster is. Now if you lived in Texas, you would know where that is. If you don't, buy a gun.

Doesn't seem like a good idea. Sorry, acid form changes in the scalp, also another finding. Periungal erythema, facial erythema, generalized erythroderma. These are all other features of dermatomyositis. Check it out.

A meta analysis of 18 studies shows that patients with multi site pain have a higher risk of future fall. The odds ratio here is one point seven four, almost a doubling of the risk in patients who have multi site pain. I don't know about you, but a lot of my patients come in and falls are part of the story. The question is, why did they fall? Do they have a new neurologic disease?

Is this a new syncopal event? Is this a cardiovascular issue? Is this a drug side effect issue? Often it's in people who have chronic pain. And I've always postulated that pain is a good enough reason to lead to falls either by causing weakness and disuse atrophy leading to instability and falls or this pain itself making people fall down.

Well, interesting study or meta analysis of study shows that, multi site pain is a reasonable cause of falls. Speaking of falls, bad segue, the Nurses Health Study, was analyzed recently by Jeff Sparks and his group looking at the contribution of smoking on seropositive rheumatoid arthritis. Now we certainly know this risk, but specifically they looked at what happens if nurses in question actually stop smoking and would the risk be less over time. So in their very large cohort of eighteen million people, I don't know it's like a few hundred thousand, they had fifteen twenty eight incident cases of RA. Current smoking increased the risk of seropositive but not seronegative RA.

Fact already well known. What they did show was that smoking cessation of greater than ten years, greater than twenty years or greater than thirty years led to a progressive decline in the risk of RA. Again, even if you, and it was only in seropositive. However, even if you were still not smoking for more than thirty years, there still was an augmented increased risk of having RA had you been exposed. But the good news is that if you do stop, the longer you stop, the risk does go down.

Between groups there was no significance but certainly an important trend. It's a nice article by Jess Sparks. An interesting two studies about scleroderma and lung disease. So one study looks at ninety three patients with systemic sclerosis and interstitial lung disease and shows that, you know, most of these people had diffuse systemic sclerosis. About half of these patients develop pulmonary hypertension and did so within seven years of the onset of their scleroderma.

It seems that, excuse me for that, that a lot of these patients went on to receive therapy for their ILD but also eighty percent received therapy for their pulmonary hypertension. Again, seems that you know, we often think that those who have interstitial lung disease is part and parcel of the systemic sclerosis, the diffuse form of the disease, but even in these patients, pulmonary hypertension can occur and seems to occur at a reasonably high rate. Another interesting study showed that amongst systemic sclerosis patients who were ANCA positive, what happens with those individuals? Well, a fairly large cohort looked for these people and found that almost nine percent of their cohort were ANCA positive and it's different kinds of ANCA, it's C ANCAs, P ANCAs, PR3s, NPOs, but mostly just ANCAs, let's just say, It turns out that this cohort who's ANCA positive is at a higher risk of developing ILD. So those who are ANCA positive, a forty five percent risk versus those who are not a twenty two percent risk.

So this is kind of interesting. They also had higher risk of developing pulmonary emboli, almost eight point eight percent is the number here versus three percent in those who were not ANCA positive. So ANCA might be a staging test that you can even do as scleroderma patients to see about their risk of lung disease and co associations. There's another, study recently about rheumatoid factor I believe, or CCP in systemic sclerosis patients also with a higher risk of ILD. Almost five hundred patients followed an academic pain center, in a single center, and these people are mainly being treated for back pain, shows that, forty two percent of those patients met criteria for fibromyalgia.

Those who were had fibromyalgia in this cohort more likely be younger, unemployed, have greater severity of pain, pain scores, and have neuropathic pain, depression, anxiety, etc. So it's, again, fibromyalgia shows up in a lot of places, not surprisingly in pain clinics, where they may be treated for other things. So again, they show up in GI clinics as IBS, in neuro clinics as all kinds of strange neurologic complaints, etc. Artie Cavanaugh called these multi organ dysesthesias, so again pain can show up in a lot of different places. And a Taiwanese insurance claims database looked at Sjogren's syndrome and the risk of osteonecrosis of the jaw, and they compared almost thirteen thousand patients with 54,000 controls and found the rate to be higher in Sjogren's zero point zero eight percent versus zero point zero three percent.

So while that sounds like it's significant and there's almost a doubling, it actually isn't that great. I mean, the idea here is it's zero point zero eight percent. It's eight per ten thousand. These are really, really rare events. There's a little bit higher in Sjogren's patients.

Turns out the patients were also on bisphosphonates had an eightfold higher risk. So eightfold higher than zero point zero eight percent is pretty close to nothing as well. You think about the numbers. Interesting report in the news this week about Peter Frampton, the rock star. I'm a big fan of Peter Frampton's.

He I was actually doing a rock and roll business back when he was touring as Peter Frampton Comes Alive, probably one of the more famous albums of all time, 1976. Peter Frampton, known for a lot of his music, he was the music advisor to the movie Almost Famous and Peter Frampton was diagnosed recently and came out both in on CBS Saturday Morning News and in Rolling Stone saying that he has inclusion body myositis. It turns out that about four years ago he started noticing weakness especially in his arms and then ultimately was diagnosed. He set up a center, the Peter Frampton Myositis Research Center that's gonna be run out of the Johns Hopkins Myositis Center and he's doing fundraising. He's touring The US doing one last farewell tour.

You should go out and support him. You should support this Myositis Center. He's in good hands with those docs. They're major league leaders in myositis and we should do some fundraising for them. Two more reports.

One, early psoriatic arthritis patients can do better with early TNF inhibition. This is a report that we reported yesterday. It's a small study about fifty one patients who have about six years, seven years of skin disease but only six months of arthritis who are DMARD and biologic naive get put on either methotrexate with a placebo or methotrexate plus, galimumab. And after twenty two months, remission defined as DAS CRP less than 1.6 was seen in eighty one percent in those on galimumab and only forty two percent in those on methotrexate alone. So early aggressive therapy works in psoriatic arthritis.

This is an ideal population. We should be very aggressive with them. Heck, we know they're coming. Thirty percent of people with chronic psoriasis are at risk to develop psoriatic arthritis. We should be doing studies on this cohort, instituting therapy early, doing preclinical psoriatic arthritis interventions and see what it does.

And the big news of the week was the, FDA coming out with a box warning, regarding the drug febuxostat. We've had reports, recently I was on the FDA advisory committee back in January that looked at this data largely based on the care study, and now the FDA is coming out with come out with its decision. Decision is one a box warning. That's the most serious warning the FDA can do warning, those who want to use fevuxostat that there is an significantly increased risk of cardiovascular death and all cause death in individuals taking febuxostat. Also in the label, there was a change to say that you don't use febuxostat first line, that you should only use febuxostat after having used other forms of urate lowering therapy including allopurinol.

Again, the backstory on this is pretty simple. When the drug was being developed, there were questions about cardiovascular safety and it was not initially approved. They made the company go back because it looked like there was more cardiovascular events in those onfobuxostat compared to allopurinol comparator patients. They made the company go back and do a safety study called the confirm study and now the safety events were flipped. There were more cardiovascular events with allopurinol, a little bit more than with the fluxazep, the drug was approved.

It's now on the market. Now there's this big post marketing commitment for what's called the CARE study, c a r e s, That actually shows again increased risk of all cause mortality and also cardiovascular death. Turns out this study was powered to do a composite measure of a cardiovascular endpoint and that was not significant, but these other two were and the panel looked at this and said, that's enough. Let's just do, we need the drug, but we need some guidelines and the FDA has accommodated, us with new guidelines. That's it.

Go to the website to look at these and more. We'll talk to you next week.