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.”


  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.

  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.

  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?

    • 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

  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.

  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!

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