Sunday, June 30, 2013

I Need Help, My Allergies are Back!


I walked out to go call my next patient back into an exam room and when he responded I was pretty sure of what was going on.  For you see, this patient’s facial appearance of ‘raccoon eyes’ gave it all away. 
“Why don’t you come back into the exam room and tell me what’s happened.”
“Okay,” he replied, with a very definite nasal twang to it. 
“How long has your nose been plugged up like it is now?”
Answering in his nasal twang voice, “For about a week.  It has just gotten worse with all of the cottonwood trees blooming and the ragweed out.  I took my Zyrtec, but it hasn’t done anything this time.  I can hardly breathe and I cough all night.  My sinus’ are just stuffed full of crap.”
“What about your ears, do they feel full?”
“Yeah, oh and my throat is sore.”  As he sat there his nose started to drip and he needed to use some Kleenex.
“Okay, what’s typically draining from your nose, what color in other words?”
“I think it’s a dark yellow to sometimes green.”
“Any fevers, or wheezing?”
“No.”
“Have you had any past surgeries or have any other medical problems?”
“No.”
“So, besides your problems with your sinus allergies, nothing else is going on?”
“Nope.”
“How long have you had problems with allergies?”
“I started having problems with all of this stuff that blooms in the spring about five years ago.  Since then it has just gotten worse every year.  Now I have problems with allergies from spring until late fall.  The only time I get any relief is when it’s cold outside,” he twanged, as he reached for another Kleenex.
“Alright, well let me take a quick look at you and then you can tell me what you want to try for your allergies.”
The patient’s ears showed a dark gray tymphanic membrane (which meant his ears were full of sinus congestion), his nasal membranes were bright red, swollen, he had peri-orbital swelling going on (‘raccoon eyes’, swelling around his eyes due to his swollen, congested sinus’), as well as his throat was pink and swollen.  The rest of his exam was normal.
“Ok, Drew, you go by Drew correct?  Not Andy or Andrew?”
“I go by Drew.”
“Ok, Drew, why don’t you tell me what you have tried for your sinus congestion, what has worked and what hasn’t.”
“I’ve tried Claritin, Allergra and Zyrtec, all of which I get over the counter.  Zyrtec worked the best for me until this year and now it doesn’t even work anymore.  My primary care physician gave me some nasal steroid spray which helped until two years ago and then it quit.  I took some Afrin yesterday, but it’s not helping.  Gad, I need these sinus’ open again, I almost can’t breathe because they’re so clogged up.”
“Have you tried nasal saline washes?”
“No, what are those?”
“Well, ENT physicians, ear, nose andthroat doctors love saline nasal washes for their patients who have sinus congestion.  You use a small blue rubber bulb with 1 cup of saline water.  You gently squirt ½ cup of the saline mixture up each nostril three times a day.  This gently washes out your sinus’ and keeps all of the allergens washed out so that you don’t have problems with all of the ragweed, cottonwood flowering, etc.  Do you want to try it?”
“I’ll try anything at this stage.”
“Okay, well let me give you the instructions for it and then you can go pick up the supplies for it at the drugstore or grocery store.  Because your allergies have gotten so bad so quickly, I also want to send you to an allergist.  I think it’s probably time for you to get started on allergy shots.  Is that okay?”
“Yeah, that sounds really good.”
“Okay, hang tight, let me go make a call for you to see how soon she can get you into be seen.  I’ll be right back.”
I left the exam room and went to go call Dr. Miller’s office.  After I explained to the receptionist that I had a patient who needed to be seen in the near future instead of weeks away, her receptionist advised me that Dr. Miller had a patient cancellation for 3 pm that afternoon and could my patient be there then?
I advised her that Drew would be there and after giving her the details of his insurance plan, I hung up the phone to go tell Drew his good luck.
I walked back into the exam room and told Drew of his appointment time after which his facial appearance changed into a smile and in his nasal twang voice replied, “Thanks.”
After Drew left the clinic, I didn’t expect to see him again.  But two weeks later he walked back into the clinic and asked to speak to me.   I walked out to the patient waiting room after the receptionist called and told me who was there waiting.
“Hi, Drew, what can I do for you?”
“Look, my face, I’m not swollen.  I can also talk like normal, I don’t have a frog in my voice anymore.”
“I’m impressed.  What all did Dr. Miller do for you?”
“Oh, she started off by telling me I was one of the worst cases of seasonal allergies she has seen in a while.  I spent the next two hours in her office getting all sorts of allergy testing done, she did who knows how many allergy tests on my backside.  Boy did I itch that night.  She also started me on allergy shots last week. She then told me to use Zyrtec-D, the stuff that she says has Sudafed in it, along with a different kind of nasal steroid spray and the saline washes you put me on.  What a difference it’s made.  At any rate, I just wanted to say thanks for sending me over to her, now I can go outside and not immediately close up from all the pollen in the air."
“Well, you’re quite welcome, Drew, I’m glad she helped you out.”
“So, am I.  Having those ‘raccoon eyes’ wasn’t fun.  Now no one at work is asking me what’s wrong.”

Signs/Symptoms of Allergic Rhinitis

Sneezing
Sinus congestion
Runny nose
Seasonal exposure to either indoor or outdoor allergens (pollen, ragweed, etc)
Pet exposure

Treatment

Saline nasal washes
Anti-histamines (Zyrtec, Claritin, Allegra) and/or with decongestant (Sudafed)
Intranasal steroid sprays (either prescription or over the counter
Oral leukotriene inhibitors (Xyzal or Singulair)
Allergy shots (for those patients with severe symptoms, patients state that these work after 1 year of injections and the effect can last up to 3 years after the last shot).

Friday, June 28, 2013

Colicy Back Pain

I was working in an urgent care clinic when I went into see a new patient.  His name was David, and he worked as a math teacher at the local high school.  He was slightly overweight, carrying his excess weight around his midline.  David was in his mid-forties, with a smile and welcoming appearance. 
“Hi, my name is Sharon, I’m filling in at this clinic as a physician assistant, until they can hire another permanent provider.  What brings you in?”
“Oh man, I’ve had this back pain that just won’t quit.  It’s on my left side and when it starts up I just want to scream, it hurts so bad.  I’ve tried changing positions, nothing helps.  It started last night.  I really need help!.”
“Alright, are you in pain right now?   You’re winching your face, is why I ask.”
“Yeah, the pain has started back up again.”
“So the pain comes and goes at any time?”
“Yeap, but now mostly it just stays, last night it did come and go somewhat.”
“Okay, have you taken anything for the pain?”
“I took some Motrin last night and this morning, it helped a little bit.”
“Are you having any blood in your urine, changes in your bowel habits, abdominal pain, nausea, vomiting or fevers?
“I started noticing my urine was turning pink last night and this morning it was just red.  I’m not throwing up, nor do I think I have a fever.”
“Okay, have you had any previous history with urinary tract infections?”
“No, I don’t recall one.”
“Have you had any history of having kidney stones?”
As David squirmed trying to find a comfortable position to sit in, he answered, “No.”
“What medications are you on?”
“I take a blood pressure medication, I think it’s called lisinopril??  I also take something for this troublesome gout I get, when it occurs, which only happens about once, maybe twice a year.”
“Ah-ah, that could explain the possible cause of your back pain.  I think you could have a renal stone, seeing that uric acid is the cause of your gout and it can also form renal stones.”
“Oh, no, are you serious?”
“ ‘fraid, so.”
I finished taking his history and then did his physical exam, which revealed he was quite tender to palpation over his posterior left side.  He also had a swollen, red joint over his first toe on his right foot. 
“David, how long has you toe joint been swollen?”
“Huh, oh yeah, It’s been like that since yesterday.  But I forgot about it because my back is hurting so much.”
“Have you taken your medicine for your gout because of your toe?”
“Yeah, I started taking it last night, but I’ve only taken one dose, I’ve been too distracted by my back pain to think about my toe.” 

Risk Factors
Obesity
Hypertension
High calcium level in the urine
10-12% incidence rate (i.e. 10-12% of all patients will have at least 1 renal stone episode in their lives)
Males > females
Caucasian
Dehydration
Diet: high intake of chocolate, spinach, green/black tea which can all increase the amount of oxalate in the system which then has to be excreted and can lead to stones
Genetic disorder (autosomal recessive genes)

Signs/Symptoms
Nausea /vomiting
Back or abdominal colicy pain
Blood in the urine
Urgency/increased frequency
Possible crystals in the urine

“Okay, David I need you to give us a urine sample, get some blood drawn to assess your kidney function and then I’m going to send you over to the x-ray department for a CT scan of your abdomen and pelvis.  I’ll have the nurse come in and give you something for your pain, so that you’re not squirming while you’re having your CT scan done.”
“Okay, thanks for the pain med.”
I left to find one of the clinic nurses and gave her the written orders to give David sixty mgs. of Toradol IM, draw the blood work and give him the specimen cup for his urine sample.  After that she sent him over for his stat CT scan.
David was back in the clinic waiting room waiting for me to see him an hour later.  So with the news that he was waiting, I walked over to the radiology department to check on his CT scan results.  Thankfully the after-hours radiologist was still there and was willing to review the scan with me.
Dr. Earl, the radiologist asked, “you say, left sided pain?”
“That’s right.”
“Well here’s the reason for your patient’s pain.  See this right here?  He’s got a nice sized stone caught in his left ureter.  I’d say it looks to be about 7 mm in size.  Boy, does that have to hurt.”
“Well, the patient will agree with you there.  Thanks for reading the CT scan for me.”
“No problem, that’s why we’re here.
I went back to the urgent care clinic and looked up his lab results.  His kidney function was normal, as was his calcium and phosphorus levels.  He had gross blood in his urine sample, but nothing else was out of whack except his specific gravity which showed his urine was concentrated. 

Diagnostic Work-up
Urine sample to assess for blood
CT scan (non-contrast) to look for the presence of a renal stone
Blood work to assess for kidney function, phosphorus, calcium, uric acid levels
Analysis of stone (if captured upon passage)
24 hour urine collection to assess for kidney function, urea, calcium, phosphate, citrate, sodium, oxalate, uric acid (usually done by the patient’s primary care physician at their follow-up appointment)

General Treatment Guidelines
Treatment of renal stones is based on what type of stone the patient has: stone composition can be either uric acid (10% of stones), cystine, or struvite (composed of magnesium and calcium, 10% of stones), or calcium oxalate (80% of stones).  
All patients need fluid hydration, fluid hydration, fluid hydration
Pain medication
Tamsulosin or Nifedipine (either of which will help decrease muscular spastic pain from the ureters)
Most stones <7 mm can be passed with supportive help, if stone is not passed then patient has to be seen by urology who can then decide on shock therapy lithrotripsy, ureteroscopy (usage of a scope to retrieve stone), or open surgical procedure (nephrostomy).
Based on the type of stone patients can also be given potassium, sodium, or calcium supplements to help dissolve the stone.   

I went to call David out of the waiting room, to have him come back into an empty exam room. 
“Come in here, David, I have your test results back and I need to explain them.”
David walked gingerly into the exam room and sat down.  “Thanks,”  he said.
“I reviewed your CT scan you had done with the radiologist.  Your CT scan shows you do indeed have a renal stone caught in your ureter, in other words the draining tube from your kidney to your bladder on the left side.  It’s a sizable stone, 7 mm.  So it may or may not pass on its own.  Your uric acid level is quite high, it came back at 11, when normal is around 6.  Your calcium and phosphorus levels are normal.  So I do believe you have a uric acid stone.  The stone has rough edges and this is why it hurts so much as your system is trying to pass it, every time it moves down your ureter, it goes into spasms and you end up in pain.”
“Wow, a little thing like a stone that small can cause this amount of pain?  Oh, geez.  What can I do about it?”
“Well, first let me ask a question.  Your urine sample showed your urine was really concentrated.  What quantity of fluids do you drink per day?”
“I thought I was keeping up with my fluids, but maybe not.  It’s been really hot outside, seeing that it is summer.  I’ve been working out 4 times a week to lose this extra weight I have put on.  As a part of my trying to lose weight, I’ve been on a high protein diet.”
“That answers the reason you ended up with a renal stone.  Your system is dehydrated, you’ve overloaded your system with purines, or protein to you, and you are somewhat overweight.  So we need to push IV fluids on you and continue your pain medication.  We’ll give you 6-8 hours of IV fluids here in the urgent care clinic as well as a medication called Tamsulosin which will help with the ureter spasms.  I’ll also start you on allopurinol which you will have to take every day.  The nurse will give you a cheesecloth to strain your urine with, in hopes of catching the stone.  We need to send it in for analysis if you are able to pass it.”
“Okay, so you’re saying this stone I have will pass in the next few hours.”
“No, I’m hoping you pass it in the next few hours, but you may not.  If you don’t then I’ll have to send you home with instructions to chug fluids at home, use the cheesecloth and continue taking the two meds I’m going to start you on.  If you don’t pass the stone within the next 24 hours or so I’ll have to send you to a urologist who will have to schedule you for a procedure called, lithotripsy.  Lithotripsy is where the urologist sends shock waves through your skin and into the stone to break it up so you can pass it.  But let’s cross that road after you’ve had lots of IV fluids and see what happens.”
I walked with him down the hallway to our observation unit and gave the nurse his orders for intravenous fluids which would have potassium bicarbonate in it.  Hopefully he would be able to pass the stone, but only time would tell.  He was also given pain medications.
I went back to the clinic and started seeing other patients.  At the end of my shift, I walked over to the observation unit and checked in on David.  I found out that he still hadn’t passed the stone, but he did think it had moved a little bit, due to his continuing to have spastic pain, masked by the pain meds he was on. 
“Okay, David it looks as though the IV fluids haven’t been successful in getting the stone to pass.  I’m going to go ahead and discharge you home.  You have to promise me that you will continue to chug fluids at home, take the prescription meds I gave you, i.e. the allopurinol, the pain medications and the tamsulosin.  You need to come back tomorrow afternoon if you still haven’t passed the stone.  You’ll know you will have passed it because the pain will be gone but right before you pass it you’ll have symptoms of needing to urinate frequently.  This will be your bladder’s response to the presence of the stone in it.  If you pass the stone, please make sure to bring it back into us so that we can test it for what sort of stone it is.  Most likely it is a uric acid stone, but only the lab will be able to tell us with 100% certainty that it is.
“Okay, well I hope that I don’t spent the night in the bathroom, but I promise I’ll just continue to drink water until I can’t stand to look at it again.”
“Alright, I hope you have progress tonight.”
:So do I.”
The following afternoon, David checked back into the urgent care clinic.  I saw him shortly after he arrived.
“Hi, David what’s happened?”
“I’m still having spastic pain and I’ve drank more water than I can stand.  I don’t even want to look at it again.”
“Okay, am I to assume you still have back pain on the left side?”
“Yeap.”
“Alright, then let me go call the urologist who’s on call.  He’ll come down and see you and then decide on how to proceed.  I’ll be back shortly.”
I went out to the nurse’s station and paged the urologist.  When he called back I told him what was going on with David, he said he’d be down to see him shortly.  Thirty minutes later the urologist showed up and went into see David.
He came out a little while later and advised me that he was taking David up to the procedure room to do shock therapy lithotripsy.  He wanted to get him up to the procedure room before the daytime urology nurses left for the day.
David was brought back down by one of the urology nurses to the urgent care clinic about 4 hours later.  I could tell by the look on his face that the stone was gone. 
“Hey, David you’re back.  How did it go?”
“I’m free of that stone, at last!  They put me in a big tub of water and then used some machine to break up the stone.  I passed the stone in pieces about an hour after they put me in the tub.  Boy am I glad it’s gone.”
“Well, I’m glad to hear it!  Let me get that IV line discontinued for you and then you need your discharge orders.  So hang tight, let me get the clinic nurse to take care of your IV line for you.”
After the clinic nurse removed his IV line, I went over his discharge meds.  I told him he had to stay on the allopurinol every day, his lisinopril, and take the colchicine whenever  he had another attack of gout.  I also advised him to increase his daily fluid intake and to stop his high protein diet.  I asked him to see his primary care physician in a few weeks for follow-up. 
I checked his labs 3 days later and found out that the urologist had indeed sent the stone fragments in for analysis to the lab.  The fragments were tested and they were indeed formed from concentrated uric acid.  Hopefully with David on allopurinol and drinking lots of fluids he wouldn’t have another episode of renal stones.   

Long-term prevention of renal stone re-occurrence
This is based on the type of stone the patient has. 
All patients have to be taking in a high amount of fluids every day
Uric acid formers are put on allopurinol (which prevents uric acid formation)
Calcium stone formers can be advised as to dietary changes, and given diuretics which prevent the formation of subsequent stones.
All stone formers are advised as to a low sodium and low protein diet.

Tuesday, June 11, 2013

A Patient with an Unusual Skin Tone


I was working in my hepatology clinic when I saw on my schedule a new patient consult.  She was a 47 year old Caucasian woman who needed to be seen due to elevated liver enzymes.  She ended up being a referral from the rheumatology clinic, which made me think she probably had elevated liver enzymes from her medications, such as  Methotrexate.  But that thought was to be proven wrong.

Later on that morning I knocked on the exam door and went into introduce myself to the this new patient.  “Hi, I’m Sharon, I’m a physician assistant and I run the hepatology clinic here.  What’s brings you into this clinic?”

“Hi, I’m Dorothy, Dr. Andrews, my rheumatologist referred me over to you.  I’ve been told I have elevated liver enzymes.”

“Yes, I saw your liver enzyme numbers.  Usually elevated numbers can be caused by medications, viral diseases, fatty deposits in the liver and the like.  What medications are you on?”

“Well, Dr. Andrews has me on Nexium for my acid reflux, Norvasc for my raynaud’s disease, and Lisinopril for my blood pressure.  I’ve been on some prednisone in the past, but not now.   I take some Tylenol for my painful joints, I’ve been told not to take any Motrin or Aleve for them due to their potentially causing problems with my kidneys.”

“Were you ever on Methotreaxate?”

“Umm, that doesn’t ring a bell with me.”

 “I’ve looked at your endoscopy reports, you’ve had two of them done in the past five years by one of my attendings.  Both of them showed you have some pretty significant inflammation at the tail end of your swallowing tube, or what we call your esophagus.  How’s the Nexium working, are you having any symptoms at all?”

“The Nexium is fine.  I take it with my dinner meal, and since switching to an evening dose, I haven’t had anymore reflux symptoms.”

“Good.  Okay, well I see that you are being treated for scleroderma, or what we call systemic sclerosis.  How long have you had it and how extensive is it?”

“I was diagnosed with it 10 years ago when I presented with skin tightening over my fingers.  Since then I’ve had skin involvement on my forearms and face.  It hasn’t gone anywhere else.  Then of course, I’ve had lots of problems with my stomach and bowels because of it.  I had an episode of diarrhea there for a while, which they treated with antibiotics.  Dr. Andrews also keeps my blood pressure on the low end of normal.  He doesn’t want me to have any problems with my kidneys, or at least that’s what he’s told me.  I’ve also been seen by the dermatology service here and they treated me with their light therapy.  I had to go into their special cylinder they have and stay there for a few minutes each day.  But the light therapy worked, my skin felt better after that.”

“Well, I’m glad to hear that.  The light therapy you had is called PUVA, by the way.  So based on what you’ve told me you would be classified as having limited systemic sclerosis.  Looking at your face I see that you have little, tiny visible blood vessels on your skin surface.  Did you have those when you were diagnosed?”

“Yes, now that you mention it, I did.”

“How long has your skin been this dark tan to copper color?”

“Umm, probably shortly after I was diagnosed.”

“Do you have any other significant medical illness?”

“No, just my scleroderma.”

“Any previous surgeries?”

“Nope.”

“How about allergies to medications?”

“Just penicillin.  I break out in hives with it.”

“Okay, well I think I got the rest of your medical information I need from reading through Dr. Andrews progress notes.  So let me explain what labs I’m going to be ordering, as well as the abdominal ultrasound, and then I’ll do your physical exam.  After that, I’ll schedule you to come back into clinic in about 10 days to 2 weeks for a follow up with me.”

“Umm, alright.”

I explained all of the blood work I was going to order (which would rule out the common forms of hepatitis: viral, iron overload, alcohol related, as well as auto-immune related (auto-immune related hepatitis, primary biliary cirrhosis {which is common with patients who have limited sclerosis} as well as primary sclerosing cholangitis.)   The ultrasound of her abdomen would show any fatty infiltrates in her liver, i.e. non-alcoholic steatohepatitis (inflammation in the liver due to fat). 

Besides Dorothy having telangiectasias on her face, she had darkened course skin on her forearms, wrists and hands.  I could also see some small healed digital pits on her fingers.  Other than this her physical exam was unremarkable.  Later that day as I was writing my progress note, I notated that she was a pleasant, well informed Caucasian woman who did indeed fit the profile for having limited systemic sclerosis with CREST syndrome being present.  It would be interesting to see what her test results showed.

Scleroderma (Systemic Sclerosis) Risk Factors:

Black race has a higher prevalence over other races
Peak incidence occurs in the third or fourth decade of life
Etiology is unknown.

Systemic sclerosis is a disease where patient produce an excess of collagen fibers which results in fibrosis of the skin and internal organs.  It can also cause internal organ injury to the heart, lungs, GI, brain and kidneys.  Most patients also have muscular involvement.

Skin symptoms can include: itching, swelling, telangiectasia (micro small broken blood vessels), ulcers, fingernails that are pitting, hardened finger digits (calcinosis cutis).

GI symptoms (90% of patients have involvement) can include any part from the mouth to the anus: such as acid reflux, diarrhea, abdominal pain, GI bleeding, colic, bloating, malabsorption. 

Muscularskeletal involvement  can include myopathy (weakness of the muscle group or due to collagen deposition in the skeletal muscle), arthralgias (joint pain due to fibrosis of the overlying skin or inflammation of the joints). 

Pulmonary involvement will be either in the lung parenchyma (end result is interstitial  lung disease) or in the vasculature (which ends up in pulmonary hypertension).

Cardiac (heart) involvement can include:  scarring of the heart muscle, congestive heart failure, irregular heart rhythms, fluid in the pericardial sac, and/or microvascular disease.

Renal (kidney) (60-80% of patients with diffuse systemic sclerosis) can include:  increased protein in the urine, blood in the urine, high blood pressure, renal failure.

Vasculature (blood vessels): hypertension, Raynaud’s phenomenon (small blood vessel spasms), digital ulcers.

Patients who have sclerosis will have one of two kinds of sclerosis:  1) limited cutaneous sclerosis which has skin involvement of the distal extremities and face, or 2) patients will have diffuse systemic sclerosis which will involve skin proximal to the distal extremities (which can include chest, abdomen, shoulders, knees).

 Patients who have sclerosis can also be classified as having the CREST syndrome (this is usually found in patients who have limited systemic sclerosis)

C: Calcinosis cutis (hardening of the skin in the finger digits)
R: Raynaud’s phenomenon (vascular spasms which affect the hands)
E: Esophageal (swallowing tube) dysmotility (problems with moving food down the      
esophagus)
S:  Sclerodactyly (skin thickening over the finger digits)
T: Telangiectasia (micro small blood vessels on the surface of the skin which are              
broken).

Patients who have sclerosis can have any of the following antibodies found in their blood work:

Anticentromere
Anti Scl-70
Anti U3-RNP
Anti PM Scl
Anti Th/To
ANA (95% of patients will be positive for this antibody)

The presence of a particular antibody will put them at risk for one type of organ disease over another.  For example, if the patient carries the Anti Scl 70 antibody this puts them at increased risk for lung involvement. 

Most patients who initially present with sclerosis will have the following symptoms:

Fatigue (76%)
Stiff joints (74%)
Loss of strength (68%)
Pain (67%)
Problems with sleeping (66%)
Problems with skin discoloration (47%)

Diagnosis of systemic sclerosis is done by the patient having tightness, thickening and/or swelling of the joints distal to the metacarpals.  Or they can have the typical skin changes seen in the hands: sclerodactyly, digital ulcers, or digital ulcers. 

Dorothy came back into the clinic towards the end of the following week.  Her lab results showed a positive ANA (which would be seen with her disease), but also a positive ASMA (anti-smooth muscle antibody) which could mean she had auto-immune hepatitis.  Her AMA titer was negative (and it would have been positive if  she had primary biliary cirrhosis).  Her AST and ALT were both elevated, above 200 each.  All of the rest of her blood work was negative. 

Her abdominal ultrasound did not show any fatty infiltrates, although it was mildly enlarged.  I explained all of this to Dorothy, and advised her that her lab results required that we acquire a fine needle biopsy of her liver tissue to see what was going on microscopically.  This would then tell us whether auto-immune hepatitis was indeed present.

Dorothy agreed, so I set her up with the interventional radiologists.  She came back to see me two weeks after her biopsy.

“Dorothy, your liver biopsy shows that you have auto-immune hepatitis going on.  You already have one auto-immune disease, in other words your sclerosis, so it’s not uncommon to eventually come down with a second auto-immune disease.  I was actually expecting your results to come back with primary biliary cirrhosis, but when your AMA titer was negative, this was ruled out.”

“I see. So what can I do about this?”

“Well for you, it should be rather simple.  I’ve already talked with Dr. Andrews and he has agreed that I can start you on a short course of low dose prednisone while I’m slowly increasing a medication called Imuran.  Imuran is a chronic long term medication we use to suppress a person’s immune system.  In your case we will be suppressing it so that it doesn’t continue its attack on your liver.  You’ll probably also find that it will help out with your sclerosis, seeing that it is a medication that is used there.”

“Oh, alright, that sounds simple.”

“Yes, we try to keep things as simple as possible.”

Treatment of Systemic Sclerosis is based on which organ system(s) are involved.  Patients who have this disease need to be seen by the various specialists (cardiologist, pulmonologist, nephrologist, etc). 

Skin:  Anti-histamines are used for pruritis (itchy) skin, UVA light therapy, calcium channel blockers (Raynaud’s).

GI tract: proton pump inhibitors for acid reflux (esophageal symptoms), endoscopy and laser treatment for those who have gastric antral venous ectasia, rotating extended use of antibiotics for those who have bacterial overgrowth, surgery for those who have small bowel bleeding from numerous small vessel disease. 

Renal:  angiotension converting enzyme medications for hypertension (high blood pressure) or impending renal failure, hemodialysis (acute renal failure)
 
Pulmonary: for those with interstitial lung disease (parenchymal disease) are treated with cyclophosphamide and/or systemic steroids.  They can be maintained on chronic long acting steroids such as imuran. 

For those who have pulmonary hypertension they can be treated with endothelin receptor antagonist (bosentan) along with a vasodilatory agent (sildenafil) and/or prostacyclin analogue (such as iloprost). 

Patients are also eventually on oxygen supplementation.

Cardiac:  patients are treated with congestive heart failure medications which include blood pressure medications (ACE inhibitors, ARBs, calcium channel blockers, etc), digoxin, coumadin/xarelto for irregular heart rhythms), pacemakers, etc.

Overall treatment of the systemic sclerosis is usually done with oral steroids (although patients have to be watched for renal disease), Imuran (chronic long term steroid), methotrexate (immune suppressive medication).

With the new field of monoclonal antibodies, a monoclonal is being looked at investigationally to see whether it has any effect in patients with systemic sclerosis.  The monoclonal is named TGF beta 1.  This monoclonal blocks endothelial growth factor.

Dorothy came back into the clinic several times over the next few months as I tapered her prednisone and increased her Imuran.  At her four month visit her liver enzymes were normal and she was on full dose Imuran without any side effects.  So I started seeing her every three months for a visit and she never had any problems to speak of.  Her liver enzymes stayed normal the whole time.  Her sclerosis was mildly better with her on the Imuran, so Dr. Andrews and I decided to leave her at the current dose schedule.
 
Long term complications from this disease includes:

Infections
Heart failure
Lung failure (from pulmonary hypertension or parenchymal involvement)
Kidney failure (sclerosis renal crisis)
Cancer (5 fold increase for patients to have lung cancer).

Mortality in increased in patients who have sclerosis, for any patient’s age matched group, patients with sclerosis have a 5-8 times higher mortality (if they have diffuse disease).  If they have limited disease their mortality is 2x  higher.  There is a 10% mortality at 5 years and 18% at 10 years for those patients with diffuse sclerosis.  Patients generally succumb to a pulmonary complication.