Scleroderma: Treatment Options for Various Symptoms

 More information related to this Podcast


Scleroderma: Treatment Options for Various Symptoms




Guest:  Dr. Edwin Smith – Rheumatology & Immunology

Host:  Dr. Linda Austin – Psychiatry


Dr. Linda Austin:  I’m Dr. Linda Austin.  I’m talking with Dr. Edwin Smith, who is Professor of Medicine in Rheumatology here at MUSC.  We’re talking, today, about scleroderma, a relatively common illness, affecting about 1 in 1000 people.  Dr. Smith, let’s talk, now, about the treatment for scleroderma.  Once you’ve made the diagnosis, what’s the next step for a patient?


Dr. Edwin Smith:  Dr. Austin, the next step is to listen to the symptoms, because some of the treatment is symptomatic, and to find out what organs may be involved.  So, we look very carefully at the function of the lungs, special x-rays of the lungs, maybe a CT scan of the lungs.  We look very carefully at blood pressure and kidney function to sort of stage to see what sort of treatment we might embark on.  Even though the skin is what the patients notice first, the skin thickening that is scleroderma, we do not have a treatment that we know of that makes any difference.  I can say that we do know something about the natural course, what happens with time, if the skin thickening is bad.  It actually, given enough time, starts to get better on its own. 


But the people with this illness are often left with difficulty with their hands because they have difficulty with opening and closing their hands, not because they hurt, but because the scarring has affected it so that the hands are tight.  And even if the skin will thin and get better on other parts of the body, the hands are almost always a difficulty later on. 


For the Raynaud’s phenomenon, that is the color changes that people get in their fingers, blue or white color changes, especially when they get cold, we use high blood pressure medicines, such as what are called calcium channel blockers.  They help the most because they open up blood vessels and help with flow.


Dr. Linda Austin:  And what are some examples of those?


Dr. Edwin Smith:  Common ones are nifedipine or amlodipine, which are brand names, or Procardia or Norvasc.  There are others that we might use that open up blood vessels.  We also, often, have people on medicines to stop the function of the platelets, the little blood particles that have so much to do with blood clotting.  So, a baby aspirin, we often have people take that to keep them from having little blood clots in their fingers. 


For Raynaud’s, obviously, the important thing is to bundle up.  They not only lose heat from their extremities, from their hands and feet, but, also, their whole body, because any time we lose heat anywhere, our blood vessels close up and decrease blood flow to our fingers.  So, we ask them to wear hats and scarves and mufflers.  And, if they’re going to be some place really cold, electric gloves, electric socks, keeping warm, keeping the temperature up, and avoiding places that, even in the summer time, we might come into contact here, in Charleston: going from 98 degrees outside into a grocery store.  Patients often have difficulty with that because of the rapid temperature change. 


For the esophagus problems, the trouble with heartburn that’s so common in scleroderma, the use of proton pump inhibitors, which decrease the acid production by the stomach, things like Prilosec that people can buy over the counter really made a big difference to people with scleroderma.  So, we use those kinds of medicines.  There are medicines to help the esophagus muscle work better.  We ask people not to eat late evening meals, the usual things that we would do for somebody with bad heartburn.


For the kidney problem, we ask that people closely monitor their blood pressure.  If there’s any elevation, we want to know about that, because we can start medications for that.  It’s a group of medicines called ACE inhibitors that have been very effective for stopping the progression of the kidney problem that can happen in scleroderma. 


For the lungs, we look at them in sort of two ways.  The scarring can occur in the lungs.  And the National Institutes of Health, a few years ago, sponsored a study that looked at the use of a chemotherapy drug, called cyclophosphamide, and compared that to a placebo, to sugar pills, to find out if cyclophosphamide would help to keep the lung disease from getting worse, and it turned out that it did work, to a certain degree.  I should say that that research was started based on information and research that had been started here at the medical university, but that was a nationwide study.  So, we do have some treatment for the scarring in the lungs.  It’s not 100 percent effective, but it is a medicine that can help.


There, also, is the possibility of high blood pressure in the lungs, in the artery, the blood vessels going to the lung.  And, for a long time, we had no treatment for that.  But, now, there are several medicines, different types of medicine, that are available for that.  The main one is something called an endothelin receptor antagonist.  And what that does is block the message that’s going from one kind of cell in the blood vessel to the blood cells that close of the blood vessels, and stops them from closing off.


The other thing that’s very helpful and is now approved for treating this high blood pressure in the lung is Sildenafil, which goes by the name Revatio, but people more commonly know that as Viagra.  So, that is very effective for treating what’s called pulmonary hypertension, the high blood pressure in the lungs.  And all of those things have just occurred in the last few years.  There was really nothing for that high blood pressure in the lungs for the longest time.  So, those are things that have been found out very recently and now approved by the FDA for those indications. 


Dr. Linda Austin:  So many rheumatologic illnesses seem to require or use prednisone.  I haven’t heard you mention that.  Does that not have a place in scleroderma?

Dr. Edwin Smith:  Small doses of prednisone can sometimes be helpful if there’s overlapping arthritis.  However, prednisone is something we like to avoid because there is a relationship of that inducing the kidney problem.  So, as opposed to rheumatoid arthritis or lupus where the arthritis or the illness gets, always, better when you use prednisone, there is some concern about using steroids, like prednisone, because it can make the disease worse. 


Dr. Linda Austin:  What’s on the horizon in the treatment of scleroderma, any clinical trials going on now at MUSC?


Dr. Edwin Smith:  There are clinical trials of medication.  And we’re involved with sponsored studies that are going on around the country that are being undertaken by several universities for the treatment of scleroderma.  If people are interested in that, they can call our study coordinator:  792-2014.


Dr. Linda Austin:  Dr. Smith, thank you so much.  


Dr. Edwin Smith:  You’re quite welcome. 


If you have any questions about the services or programs offered at the Medical University of South Carolina or if you would like to schedule an appointment with one of our physicians, please call MUSC Health Connection:  (843) 792-1414.


Close Window