Scleroderma Mortality Studies Underestimate Early Deaths, Research Shows

Scleroderma Mortality Studies Underestimate Early Deaths, Research Shows

Many scleroderma patients die early in the course of the disease, and studies that measure mortality without taking that into consideration tend to underestimate death rates in the disease, according to a new report.

Researchers compared a group of patients in the early stages of scleroderma to a group with participants at various stages. They reported in “Early mortality in a multinational systemic sclerosis inception cohort” that the groups differed in causes of death, indicating that patients who die early more often do so of scleroderma-related problems.

With that data in hand, the researchers urged the scientific community to establish large, international, prospective studies that follow patients from the start to better understand mortality in scleroderma.

Published in the journal Arthritis & Rheumatology, the study included 1,070 patients who were followed from within four years of disease onset. Such a study group is known as an inception cohort. The second group included 3,218 patients who were at various disease stages. All patients were recruited at centers in Australia, Canada, and Spain.

In the inception cohort, 140 patients, or about 13%, died during a median follow-up time of three years. Patients in the group were four times as likely to die than a similar group of people in the general population. Women in the group had a life expectancy 22.4 years shorter than the general population. For men, it was 26 years shorter.

People with diffuse scleroderma were more likely to die than those with limited disease, with 24.2% of those patients dying within eight years.

In the second, larger group, patients were 3.4 times more likely to die compared to the general population. Only 9.3% of the patients with diffuse disease died at eight years.

In the inception group, 62.1% of the primary causes of death were related to scleroderma, with pulmonary arterial hypertension (PAH), interstitial lung disease, or a combination of the two being the most common causes of death.

In the larger group, only 55.5% died of scleroderma-related causes. More patients in the group died from heart-lung disease than in the inception group — 70.9% versus 55.2%.

The most common non-scleroderma causes were cancer, sepsis, cerebrovascular disease, and ischemic heart disease. Regardless of the main cause of death, scleroderma-related organ problems contributed to death in 50.1% of the patients.

Men, those with diffuse disease and PAH, as well as renal crisis, were predictors of poor survival odds.

Researchers at St. Vincent’s Hospital Melbourne in Australia performed the study and noted that several others reported that survival differed substantially in their estimates. Some of those studies lacked measures of disease duration, which introduced two types of errors in their death rate analyses:

Since early deaths often are missed, the studies do not account for all deaths. Second, analyzing a group consisting of people who have lived with the disease for a long time introduces what is known as “survivor bias,” because those people likely have less severe disease and better outcomes than those who died early on.

“Our results suggest that prevalent cohorts underestimate mortality in SSc (systemic sclerosus) by failing to capture early deaths, particularly in men and those with diffuse disease,” the team wrote. “Collectively, these findings provide a compelling rationale for establishing a large prospective multinational inception cohort of patients with SSc to more accurately quantify early mortality in this disease.”

53 comments

  1. Tomisa Starr says:

    A study of mortality in scleroderma patients of African descent is sorely needed. These patients (including African-Americans) have more severe disease and worse outcomes than scleroderma patients of European descent. Although scleroderma affects more Blacks, the majority of studies about scleroderma patients have focused on Caucasians, however.

    • Cherry says:

      Hi Tomisa. I live in South Africa and a number of clinical trials are underway that focus only on Scleroderma patients of African descent. Hopefully, their findings will be made public sooner rather than later.

      • Nicky Stirzaker says:

        My brother died a year ago. He was a South African living in Spain. He was diagnosed with Crest about 7 years ago and really nobody knew anything about this and he got little help from the doctors over there. I am still battling to get to the bottom of his death as I believed that this was not a life threatening disease. The hospital told me that it was his lungs and organ failure.

        • Jim S says:

          CREST is not a diagnosis but rather a collection of symptoms that may lead to a diagnosis of scleroderma. Scleroderma replacementes collagen with fibrotic tissue especially in the lungs that may lead to death. For more info, try scleroderma dot org

  2. Asheran Rahim says:

    I have been diagnose with this disease. I feel as if I have been sentenced to death without committing a murder.

    • Nina says:

      We will all die one day. The most important thing is to know Jesus Christ as your savior and remember Jesus is alive and He still heals today. “Nothing is impossible to them that believe. ” I might have Scleroderma but Scleroderma does not have me, Jesus has me.” Need a friend and someone to pray for you . Contact me.

      • Karol says:

        You sound as if you are a very strong women!!! Keep your head up!!! I’m just in pain and shock of the word… This is all new to me so I’m just scared at the moment…

      • nina ross says:

        I have good days and bad days just as you do. God helps me get through the day. With out Him i would mot make it. I thank Him for every day i have here on earth. our real home is with Him in heaven.

      • Charles says:

        My name is Charles Ellis i have the scleroderma disease and it painful some time had it for about 8 months now what is your answer i know god got us this is all new to me

      • Gene says:

        Good grief.. If you think a god will save you by praying ask yourself why he invented the disease in he first place. A kind and loving god would not do that…. The only thing you can achieve by praying is a positive attitude which is a great assist when battling a disease.

      • john mills says:

        Bless you my sister. You are on the winning side. Your writings are giving hope to those suffering with this disease. Its my belief you will reach many people through this and similar columns.

      • Jennifer Richardson says:

        Hi Nina. My name is Jennifer Richardson. I live in San Antonio, Texas. I was diagnosed with scleroderma 8 years ago, when my first son was about to be 4 yrs old.
        I had a rheumatologist tell me I didn’t have scleroderma, but recently have sprouted this rash made up of red dots. All over my legs. My arms aren’t so noticeable.
        I believe that it is scleroderma. As I don’t know any other disease with that trademark.
        Anyway. My husband and I aren’t doing well. I really could use his support but I don’t think I have it. I just read your post, would you mind praying for me? I could use the positivity right now.
        You’re a very sweet woman. Thank you for the post and just know you made a difference in my night.

        • Rachel says:

          Hi Jennifer I’m in San Antonio also, I wish u the best and I hope you don’t have scleroderma. I was diagnosed in 2008

      • Liza says:

        Pls help me pray.I am diagnosed with Scleroderma since 2011.This time it is getting worse.But I always believe in miracles.God is my only refuge.

    • Marites valencia says:

      I can feel you, I feel depressed since the day they found out, just last September of this year that I have this kind of disease, till now sometimes I can’t believe or I can’t still accept, knowing I’m so young… :'(

  3. Dawn says:

    My mother diagnosed at the age of 60 with diffuse systemic sclerosus in 2006 and is still battling on. She has many challenges, but remains mostly positive. I have to say I’m impressed.

    • Cindy says:

      No such thing as diffuse systemic sclerosis. It’s just called diffuse scleroderma, and it’s unfortunately the worst form attacking the organs. There is also limited systemic sclerosis which affects the organs at a slower rate like I have but I have PH as well. 12 year survivor of both

  4. Heather says:

    I was recently diagnosed. My lungs, kidneys and heart are so far unaffected. I estimate I had the disease for 2 years before skin biopsies and formal diagnosis. I am on a combination of conventional and alternative medical protocols. I’m not going to an early grave without doing my best. Terminal illness has a way of sharpening the mind and reassessing priorities.

    • Helen says:

      Hello Heather, my relative age 45 diagnosed with scleroderma, he is in Russia. Doctors had no suggestions of treatment. Can I ask what sort of alternative treatment are you on please? Is there any food that is recommended to have or any to avoid?

      • Tajul Islam says:

        My mother has been diagnosed with scleroderma.We are taking treatment from CMC Vellore.This disease is related to reheumatology department.This disease is not curable but treatable.You can take treatment from CMC Vellore,Reheumatology Department.

    • Cindy says:

      Yes! Get diagnosed and probably on immunosuppressants asap to slow the disease as your body is attacking itself at a high rate. Epoprostenal is a great stuff for both PH and scleroderma. It saved my life. Going on 12 years.

  5. Bob says:

    I am sick of this death sentence.last year l was string and fit and expecting to live to 80. It sickens me with the unfairness and scares me 24/7
    I am weary and can hardly function

    • Julie says:

      Me too. It’s like a sword hanging above your head. It’s unfair. I am strong, capable and brilliant. Never lazy, always productive. So unfair. I still want to live forever, and feel like I can. But then, I remember…

  6. Dan says:

    Fit healthy at 52 had a good body was a builder worked out after a shift.Now 54 had ulcers in fingers now gangrene.lost tips of three fingers have gangrene in another finger as I type you this message.God knows what’s next can only say your not the only one who thinks why me.

      • Mom (aka Nancy) says:

        My daughter was diagnosed with systemic scleroderma at 40, Just as she was preparing to get married. It has been brutal on her body. Within a year she lost peristalsis in her esophagus and is only able to get down food marginally. Being able to have a good meal would provide some comfort. She has an identical twin sister and people now say that they can see a big difference between the two of them. You all are experiencing something that most people don’t understand. My daughter is working and so far still able to play the drums even though her fingers look like Wax She receives infusions monthly which is really seem to have made a big difference on her health.Having children is out. I Thank God that her husband is so supportive. My heart felt prayers go out to all of you. God bless you all!

  7. Pam secord says:

    This is a very difficult defined disease that effects each of its victims at different rates. Maybe because we all have different DNA make-up and live in different environments with different eating, working, stress and sleeping habits. Do your best in taking care of yourself. Read your bodies needs.God bless!

  8. Pam says:

    This is a very difficult defined disease that effects each of its victims at different rates. Maybe because we all have different DNA make-up and live in different environments with different eating, working, stress and sleeping habits. Do your best in taking care of yourself. Read your bodies needs.God bless!

    • Bonnie Lambert says:

      I think why not me.
      I have more family on the other side than here.
      I’m in no rush by any means but go a day at a time, I’m just working with it, but won’t let it take over a piece of my mind.
      Good Luck new research don’t give up hope.
      Stay warm, eat food you can have stay away from what you can’t have.
      Take your Vitamin D 2000
      It helps a lot and just count your Blessings.
      Thank God keep him close. Family support has been great, love you all.
      I like the listen to your body it lets you know what not to eat and what you need to do.
      Stay strong

  9. Chris Nickerson says:

    I have had systemic sclerosis dermitomyositis overlap for 44 years. I was saved by Methotrexate. I am under Professor Denton at the Royal Free Hospital, Hampstead, London, England. You have to stick it out and do what the docs say.

  10. Julie says:

    I was diagnosed two years ago with systemic scleroderma. My arms were like wood, I could hardly stand up and my hands were bent up with no strength at all. I was referred to USC Hospital, LA and have never looked back. They run a photophoresis program which I was started on straight away. It is not yet approved by the FDA for Scleroderma, but with treatments two days every three weeks I have been given a second chance. My arms are almost back to normal and I am able to do everything again. I work full time and enjoy life. Wanted to share this as not sure how many people are aware of how this treatment works so well. I owe my life to USC Hospital as I believe without them I would probably not be here today.

  11. Gen Deltieure says:

    My mother was diagnosed with Scleroderma CREST syndrome at 56 y/o. She is now going on 89 y/o. Yes, she has all 5 symptoms of the disease, but she goes about a normal life with modifications. She has to eat moist foods and eat slowly. She wears warm clothing especially hand & foot ware. She has PH and lung changes so has to modify her exercise endurance. But she is living her life. Modern medications, especially for PH have improved survival. My suggestion is to live life to the fullest without always looking at the “clock” wondering how long you will live. No one is promised tomorrow. See your doctors faithfully and follow their directions. Most university hospitals have PH clinics with the most up to date treatments.

  12. Bonnie says:

    My experience with scleroderma:
    I am a female living in Canada. At 23 years old I was diagnosed with Raynauds phenomenon – I was a land surveyor, working outside in all types of weather (we laugh at -20 degrees Celsius), and experienced the symptoms of Raynauds including infections along my nail beds. Treatment? I changed occupation. In my 20s, under the care of a cardiologist, I tried various medications that did not provide relief. In my 40s I experienced a progression of symptoms to the rest of CREST, sans E, and had removal of calcium deposits from a knee and a thumb. About 10 years ago, I was referred to a great rheumatologist. This year, at the age of 56, I was diagnosed with Barrett’s esophagus, thus realizng the E.
    Systemic scleroderma. I’ve had a progression of the disease over the course of more than 30 years. I take no medication. I am under the care of a rheumatologist who I see once a year. With the help of other health care professionals like a gastroenterologist, cardiologist and my family physician, changes in my health are monitored. Eat right, get a good night’s sleep, dress for comfort (I avoid cold winds like air conditioning and blizzards), exercise, and keep stress levels down are things we can all do.
    Death sentence? No! Chronic condition? Yes. I agree with what Gen Deltieure (Aug 22, 2018) says. Celebrate life, have fun!

    • Vanessa says:

      Hello Bonie, I hope you are fine. My father has the same disease and almost the same evolution than you. What kind of treatment are you receiving? And what kind of food are you eating? Something to avoid? Thanks in advance!

    • Terry says:

      Bonnie, Thanks so much for your comment. I just came home from the doctor who informed me that my blood test results were positive for scleroderma. I don’t have any symptoms except some little red spots on my legs. He referred me to a rheumatologist so I guess that’s the next step. I’ve heard of scleroderma but never thought much about it. Not sure how concerned I should be.

  13. janette acevedo says:

    I tested positive for scleroderma 3 times and was told they were all false positive. I feel as if I do have it. My health is deteriorating. I don’t know what to believe or do!

  14. Julia Harrison says:

    Hello friends. I am a 39 year old female. I was diagnosed 2 years ago with Limited Systemic Scleroderma. Pursued tests because of swollen hands and Raynaud’s. I do not have any other symptoms. I feel great and try not to think about the diagnosis. I exercise regularly, eat well, sleep well, and enjoy life with my 2 young kids. My husband has just bought me THE BEST gift ever – electric mittens! Along with very heavy coats and scarfs to keep my body temperature warm, these mittens are letting me play outside again (I live in Barrie, ON where the winters are rough). My doctors are wonderful and keep very close watch over me. I just got a few strange blood results regarding liver – thus on this site for more info. However, I hope to stay relatively symptom free for as long as possible.

  15. Brittany says:

    I was diagnosed as a “juvenile” when I was 8years old. They didn’t know exactly what it was for a long time because its extremely rare for the disease in a child. Constant tests, surgeries to remove tumors, many trial medications that cause fatigue and headaches, trips to see the rheumatologist, dermatologist, kidney specialist, heart mammograms I was sick of the visits. The list goes on and I feel for all of you.. I’m 30 years old now and my disease is dormant. It went dormant soon as I moved to a warmer city at 18years old. Fortunately I am from Northern Arizona and only had to move 2hrs away down to Phoenix. The only symptom I have now is Raynaud’s when im traveling for a winter trip or im home for the holidays. I’ve come to learn my body very well and that things are triggered when the elevation is too high or when the weather changes quickly. Id recommend living in a climate with minimal weather change. I am also a runner and feel its good to condition my lungs and keep active as a preventative treatment for when im older. Im sure the disease will reappear with age but until then I just remain thankful the disease is dormant and Ive lived this long. They sure scare you as a child and I didn’t think I would live to 18. Know your body and try to keep to natural products. I hope for the best with everyone. This disease is scary and all we can do is monitor and cope.

    • Vanessa says:

      Hello Britanny, I hope you are fine. My father has this disease as well and we are battling with hope. May I ask you, can kind of food are you eating? Thanks in advance

  16. Ondrea says:

    i have Diffuse Scleroderma. 12 long years. The first 5 I thought I was going to die it was so bad and I was raising a little girl. Now I have good days and bad days. But I’m greatful. A lot of the medications give me other problems so I know my body and do things my own way. I’m not trying to speed up my illness or anything and I do talk to all my doctors. Advocate for yourself! Know your meds, your body and All your diagnosis’s!

  17. Anita says:

    I am 80 years old, just learned the diagnosis of the ongoing symptoms of down immune system is scleroderma, with showed up high in my blood work,
    Not sure which form yet tho I see scarring on my thighs and have had pain in my toes. I fight like tiger with red light therapy, foot soak baths, alot of natural supplements. Important is vitamin E, Evening primrose oil, grapefruit seed extract, a high grade daily multivitamin. This is according to my Integrative, Internal Medicine Dr. Prayer is always most important. Remember it is not over until God says so, fight and seek as much help as you can

  18. Mary Frances Moore says:

    My brother passed away 6 months ago with the localized.It came on him in Spring of 2016.they didn’t know what was wrong with him till that Dec.It was to late.He went down hill ,he was a healthy 56 yr old and then passed almost 2 yrs later.Horrible watching my brother die.He had feeding tube . passed away at Curis nursing home.

  19. Unknown says:

    My mom has systematic sclerosis

    She had symptoms of raynaud’s syndrome and suddenly her fingers begins to turn blue and then black the finger is begin to gangreene, she was admitted in icu for the treatment in which she was given an injection now symptoms are improving very slowly but i am living in fear of loosing her she is my everything. And i just want god to give me time to complete my studies and give her all the happiness she deserve.! 😭😭😭 I can’t type more, (i whish we could share the disease.!)

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