Scleroderma Patients with Lung Conditions Have Shorter Lifespans, Need Better Therapies, Study Reports

Scleroderma Patients with Lung Conditions Have Shorter Lifespans, Need Better Therapies, Study Reports

Scleroderma patients with lung diseases have shorter lifespans than those without pulmonary conditions, underscoring the need for better treatments for patients with lung conditions, a Colorado study reports.

Another finding was that scleroderma patients with a lung disorder have higher medical costs than those without the condition. That’s particularly true of patients with pulmonary arterial hypertension, or PAH, compared with those with an interstitial lung disease, or ILD.

The study, “All-cause Healthcare Costs and Mortality in Patients with Systemic Sclerosis with Lung Involvement,” was published in The Journal of Rheumatology.

Scleroderma is an autoimmune disease whose hallmarks are thickening and scarring of tissue. An autoimmune disorder is one in which the immune system attacks healthy tissue instead of invaders.

The tissue thickening and scarring in scleroderma can affect several organs, including the lungs. Thirty to 70 percent of patients also develop ILD, and 2 to 30 percent PAH.

To get a better grasp of patient outcomes and the economic burden of scleroderma with a lung condition, a University of Colorado Medical School team used two U.S. databases to identify patients with both disorders. They were the Truven Health MarketScan Commercial and Medicare Supplemental healthcare claims repositories.

They discovered 11,752 people whom doctors diagnosed with scleroderma between 2004 and 2013. In the same period, doctors diagnosed 1,808 scleroderma patients with ILD and 1,223 with PAH.

Researchers decided to use only patients whom doctors had followed for at least five years in their healthcare costs evaluation. They included 957 patients with scleroderma, 219 with scleroderma and ILD, and 108 with scleroderma and PAH.

Sixty-five percent of the PAH patients were hospitalized during the five years. The figures were 53 percent for ILD patients and 44 percent of scleroderma patients without a lung condition. Emergency room visits were also more frequent among ILD and PAH patients than scleroderma patients without a lung disease.

PAH patients’ costs were much higher than the costs of the other two groups during the five years. The range was $18,513 to $23,268 for patients with scleroderma only, $31,285 to $55,446 for ILD patients, and $44,454 to $63,320 for PAH patients.

Another important finding was that costs increased over time, with the highest expenses of all the patients occurring in the fifth year.

Most of the costs involved hospital admissions, outpatient services and medicine. PAH patients had the highest outpatient services and medicine expenses.

The cost that varied the most among the three patient groups was medicine. PAH patients’ cost were more than four times that of scleroderma patients without a lung condition and nearly double that of ILD patients.

Among those who were treated at least 10 years after their diagnosis, the team found that scleroderma patients with a lung condition had the worst death rates. The median survival time after diagnosis was 11.3 years for scleroderma patients with no lung disorder, 8.8 years for ILD patients and six years for PAH patients.

“In comparison to those with SSc [scleroderma] alone, the presence of ILD and/or PAH is associated with substantial increases in healthcare costs and a worse survival experience,” the team wrote.

“Given the effect of lung disease on survival with SSc, these data highlight that there remains an unmet need to identify more effective therapeutic strategies for both ILD and PAH in this patient population,” they concluded.

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  1. Tomisa Starr says:


    As an African American person with long standing Scleroderma, I have found very few articles about Scleroderma, its treatment and patient experiences which are useful to someone like me.

    While I find the coping with Scleroderma articles useful because the advice they offer can apply in general to any Scleroderma patient, regardless of race – the research articles are a different matter. Almost all the articles about research studies – while informative – hardly ever include patients of Black African descent, or information about complications which could be useful or applicable to treating that particular Scleroderma patient demographic.

    It is well known that, although while Scleroderma is more prevalent among African Americans – and Scleroderma patients of Black African descent experience more serious complications with a worse prognosis – very few Scleroderma studies even include us in their sample populations. At least 95 percent of all research done on Scleroderma and its complications concentrate solely on Caucasians.

    Scleroderma research populations are comprised of individuals of European descent, almost exclusively.

    This is very disheartening to me, as a Black Scleroderma patient. In a rare disease like Scleroderma, which few physicians seem to have experience in even recognizing – let alone diagnosising and treating – there is even more confusion when it comes to diagnosing and treating Scleroderma and its complications in Black patients.

    Most videos and studies about Scleroderma provide information on autoantibodies such as SCL-70, primarily found in Caucasian Diffuse Systemic Scleroderma patients. However, most videos do not address the fact that U3-RNP autoantibodies are common in Diffuse Systemic Scleroderma patients of Black African descent, not SCL-70. This oversight can add to the general lack of knowledge Scleroderma, and also how it is expressed in patients of various races and ethnicities – which can place Black Scleroderma at risk of disparate care.

    This is a very troubling situation – one which places Black Scleroderma patients at risk, and can result in difficulty in getting diagnosed and obtaining access to proper care for their disease and its life-threatening complications.

    I can only hope that this situation will soon change for the better, both for me, and Scleroderma patients like me.

    As a long standing Scleroderma patient (diagnosed with Diffuse Systemic Scleroderma in 1993, at age 30), I have personally experienced these challenges in my care. This has made my existence with Scleroderma a painful and rocky one.

  2. Tomisa Starr says:

    I meant to add to my comment above, is that some community Specialists concentrate only on SCL-70 as a significant risk factor for progressive diffuse ILD which requires treatment in Scleroderma patients. Also, community Specialists focus on Anti-Centromere Autobodies as being associated with both Limited Diffuse Scleroderma and PAH.

    However, I believe, research on Black African American Scleroderma patients shows that U3-RNP autoantibodies primarily found in this patient demographic indicates that the presence of U3-RNP means that we are at risk of developing BOTH ILD AND PAH – despite the likelihood of testing negative for SCL-70 and AutoCentromere Antibodies.

    It is *imperative* that – for the best outcome for Black Scleroderma patients – Community Specialists who potentially diagnose and treat Scleroderma patients be knowledgeable about the expression of Scleroderma in different racial groups.

    I have personally experienced this lack of awareness in my Physicians, and it has most definitely affected the quality of my care when it comes to my Scleroderma Lung complications.

    This is a situation which desperately needs to be addressed!

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