Findings in Phase 2 trial of prednisolone seen as inconclusive due to pandemic by Patricia Inácio, PhD from Scleroderma News

Moderate doses of oral prednisolone, a corticosteroid, given for a short period may help to ease symptoms in people with early diffuse cutaneous scleroderma (dcSSc), a Phase 2 trial suggests.
The study, intended to assess whether moderate dose of corticosteroids are safe and effective for early dcSSc, was affected by the COVID-19 pandemic starting in March 2020 and failed to provide conclusive results, its researchers noted.
Given the disease’s “inflammatory basis,” however, its findings may provide “support for the view of many clinicians that it is not unreasonable to prescribe short-term moderate dose prednisolone for symptom control, always remembering the importance of careful monitoring of blood pressure and renal function,” the team wrote.
The study, “A Phase II randomised controlled trial of oral prednisolone in early diffuse cutaneous systemic sclerosis (PRedSS)” was published in the journal Rheumatology.

Prednisolone use ‘contentious’ due to risk of renal crisis with dcSSc
Scleroderma, also known as systemic sclerosis (SSc), is marked by progressive scarring in the skin and, possibly, internal organs that include the kidneys. The disease is caused by a wrongful immune response against the body’s own proteins.
Organ damage is a particular risk for people with dcSSc, and one that develops early in the disease’s course and can worsen quickly. Currently, no effective disease modifying treatments are available for these patients.
Although the inflammatory basis of dcSSC supports the use of corticosteroids, their use poses of risk of a renal, or kidney, crisis.
“Whether or not they should be prescribed is therefore highly contentious,” the researchers wrote.
Scientists in the U.K. designed the Phase 2 PRednisolone in early diffuse SSc (PRedSS) trial (NCT03708718) to assess the safety and effectiveness of prednisolone at a moderate dose compared to a placebo in adults with early dcSSc. The study was funded by Versus Arthritis.

Finding reinforces research that viral infections increase likelihood of autoimmune disorders by Patricia Inácio, PhD

People who were infected with SARS-CoV-2, the virus that causes COVID-19, are at greater risk for developing autoimmune diseases, including scleroderma (SSc), according to a large study.

The findings support recent evidence that viral infections may increase the likelihood of autoimmune conditions, although the mechanisms remain unknown.

“It is crucial for physicians to have relevant notions and to recognize these autoimmune manifestations in order to respond appropriately in the ongoing pandemic and long-term post-pandemic phase,” the researchers wrote.

The study, “Risk of autoimmune diseases in patients with COVID-19: A retrospective cohort study,” was published in the journal eClinical Medicine.

Viral infections, such as those triggered by SARS-CoV-2, are believed to serve as potential triggers of autoimmune disorders in people who have genetic predisposition for such conditions. In fact, a number of cases of autoimmune diseases — such as Guillain-Barré syndrome and systemic lupus erythematosus — after SARS-CoV-2 infection have been reported.

“However, obtaining conclusive evidence of a connection between viral infection and subsequent autoimmune diseases is challenging, not only because it is frequently impossible to extract the virus from diseased tissues, but also because the collection of sufficient amounts of epidemiological evidence is constrained by lengthy process and geographic distances,” the scientists wrote.

Since the COVID-19 pandemic began, millions of people experienced this acute infection around the same time, which enabled the collection of large amounts of data to better establish the link between SARS-CoV-2 and autoimmune diseases.

Researchers in Taiwan conducted a retrospective analysis of data from the U.S. Collaborative Network, from 48 global healthcare organizations part of the TriNetX Research Network. TriNetX holds the largest global COVID-19 dataset.
In total, the analysis included 3,814,479 participants from January 2020 to December 2021. They were divided into two groups after matching for age, sex, race, socioeconomic status, lifestyle-related variables, and coexisting diseases.

After matching, participants with a positive test for the SARS-CoV-2 virus were part of the COVID-19 group (mean age 45.2 years) and those with a negative test were included in the control group (45.1 years). Each group had 887,455 participants. Women represented 57.4% of the COVID-19 group, and the majority were White (64.4%).
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Patients who had received COVID-19 vaccinations were excluded from the study.

Life with scleroderma can be a struggle. Sometimes, when the pain seeps into my bones and fatigue keeps me in bed for hours, the battle seems insurmountable.

But even on those difficult days, I’ve learned to fake it until I make it. I can’t tell you how many times I’ve plastered a smile on my face and pretended everything was fine when, in reality, I felt far from OK.

Why do I do that? Why do I feel the need to put on a brave face and ignore what’s happening in my body and mind? Why is it more comfortable to lie instead of owning my truth — the painful, the stressful, and the miserable?

Lying to doctors
I discovered early on in my diagnosis that no medication or treatment could alleviate my symptoms completely. On good days, some of the pain and discomfort subsided, but on the worst days, the treatments would barely touch the physical discomfort traveling up and down my body.
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I’d leave appointments so disappointed not to have a permanent solution to my ulcerated sores or itchy skin. Over time, I built up armor to shield me from the letdowns until I eventually became emotionally numb to the physical pain.
It seemed pointless to be honest with my doctors. Looking them in the eye and telling a bald-faced lie about how I felt quickly became second nature.

Scleroderma News ​columnist, Amy Gietzen, shares her thoughts on chronic illness and relationships

I’m a lot of work, even on a good day. Living with scleroderma requires me to spend a lot of time and energy on maintaining my health. Because of this, I often wonder if a potential life partner would think I am worth the emotional and physical effort a relationship would require of them.

Sometimes I feel lonely. I’ll wonder “what if” — what if someone loved me for me? I’m not talking about a parent’s love for a child, or even that of a friend. I’m talking about mind-numbing, all-consuming, once-in-a-lifetime romantic love. I’m envious of those who’ve found their person in life.

I know it sounds like I live in a fairy tale. But sometimes I get stuck in the thought that I’ll miss out on romantic love for the rest of my life.

After living with scleroderma for over 20 years, I have a good idea about what it would take to be a partner to someone who has it. When I imagine my own partner, there are some traits I think are important.

A romantic relationship with a chronically ill person is marked by ups and downs. If you’re starting a relationship with someone who has scleroderma, you should be prepared for the fact that it won’t be easy. It requires patience, communication, and understanding by both partners.

 ​When I'm frustrated with myself and my body, I see my biases at work - by Lisa Weber from Scleroderma News

I sat on the shower floor in complete exhaustion, a scrubbing brush in one hand and a natural disinfectant in the other. Physically, I was at my breaking point. But the shower was only half-done.
It’s tough throwing in the towel, but my body sometimes just doesn’t cooperate long enough to complete certain tasks. And if I push past the pain and exhaustion, I risk a major flare-up with unrelenting inflammation and fatigue.
So I did what was best for my body. I tapped out and crawled out of the bathroom. I managed enough strength to plop myself on my bed to rest and brainstorm a solution. Failure only happens if I quit!
​Finding tools to adapt to my limitations
​Did you know the internet has a wealth of results for the search “disabled cleaning tools”? Accepting disabilities caused by my scleroderma doesn’t make me weak. Strategically planning ways to stay independent gives me power over my shortcomings. It takes emotional strength to be unstoppable, and that’s what I strive for each day.
One search turned up a power scrubbing brush, a hand-held pole with a spinning brush to make scrubbing surfaces easier. I was so excited about this new purchase and couldn’t wait to clean the rest of my shower with no effort!
And when that box showed up on my doorstep, I acted like a kid on Christmas morning. I tore open my package, gave it a full charge, and dashed straight to the bathroom to test it.
It started out fantastic! Cleaning bubbles coated each tile, and the brush spun around like helicopter blades. I could clean all day with this nifty tool! I even imagined my future self sipping a glass of wine while nonchalantly moving the pole around.
But there was one problem: The motor died before the last wall was finished. Perhaps I received a defective product? Perhaps my definition of “clean” is too exhausting for any tool or person in this world? I’ll definitely try another brand before giving up, but this experience taught me an important lesson.

How to cope when even a well-meaning phrase can cut daggers by Amy Gietzen from Scleroderma News

“Wow, you were just discharged from the hospital? You look healthy to me.”
I can’t remember a time when words have hurt me more.

According to the U.S. Centers for Disease Control and Prevention, more than 53% of Americans are living with a chronic medical condition. As startling as that statistic may be, chronic illness, especially scleroderma, tends to create an extremely isolating way of life.

Scleroderma has a way of singling you out, maybe because no case of scleroderma is like another. This uniqueness can include symptoms both usual and unusual, and some of them are invisible to an onlooker. This leads to misconceptions about our physical complaints, one of the more upsetting aspects of living with scleroderma.
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These misconceptions are often packaged in scornful condemnations and accusatory statements of laziness, forgetfulness, or well-meaning ignorance. The reality is that just because we look OK, it doesn’t mean we are OK — physically or emotionally.

Researchers study high levels of the ADAR1 protein in macrophages by Margarida Maia, PhD

The protein ADAR1 occurs in great amounts in macrophages, a type of immune cell that appears in the early stages of systemic sclerosis (SSc), making the cells more active and stirring up a “turmoil” of inflammation, a mouse study found.

Researchers also observed that mice in which the disease was induced by injection of a chemical — bleomycin — developed fewer symptoms in their skin and lungs when engineered to have ADAR1 deficiency in macrophages. Their macrophages also made fewer certain inflammatory molecules.

The findings suggest that “targeting ADAR1 could be a potential novel therapeutic strategy for treating sclerosis,” the researchers wrote.
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The study, “ADAR1 promotes systemic sclerosis via modulating classic macrophage activation,” was published in the journal Frontiers in Immunology.

Case study highlights option of a second stem cell transplant for relapsed SS
by Patricia Inácio, PhD 

A 42-year-old man with scleroderma responded well to a second stem cell transplant after experiencing a disease relapse with lung involvement following his initial transplant, according to a recent report.
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Improvements in skin thickening and lung function were quickly seen, and the patient was still in remission two years after the procedure.

The study, “Case report: A successful second autologous hematopoietic stem cell transplantation in refractory systemic sclerosis, with positive effect on skin involvement, pulmonary function and microcirculation,” was published in the journal Frontiers in Immunology.

As 2022 comes to an end, I’m reflecting on the year and trying to look forward to all 2023 might offer.

Many adults are probably excited to ring in the new year in style by going out on the town or enjoying a few drinks with friends. Sometimes I envy people who can spend a fortune on lavish outfits and expensive bottles of Champagne, and eat filet mignon and lobster tail in celebration. I long for the days when my only problem was deciding what to wear when I went out at night.

The last time I went out for New Year’s Eve, I was 20 years old. At that point, I had recently been diagnosed with scleroderma and was in denial about my disease. So I behaved as if I were unaffected by symptoms, brushing aside the pain and discomfort and forcing my body to continue as normal.
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I made plans to go out with close friends to celebrate at a local bar. Sounds normal, right? Unfortunately, my evening was anything but normal. I ended up getting sick after one sip of my cocktail and had to leave immediately. I became sick several times on the drive home and had to pull over to vomit.
I couldn’t understand what was wrong with me. In my mind, I was fine, when in reality, I was having a serious scleroderma flare-up.

A dash of humor often helps me get the reaction I want from concerned others by Lisa Weber

Scleroderma knows how to be a Debbie Downer. I can be enjoying a moment and BAM! Some debilitating pain or body malfunction pops up to ruin it.
I can’t control when I’ll need to hit the pause button on our fun, but I can control how I approach the situation so I don’t become the equivalent of the lights going out at a party.
Having become a pro at living with pain and limitations, I’ve learned that if I share why I can’t do something, people understand. But they also show pity — which makes me feel even worse!
I discovered that if I use colorful, silly descriptions to share the why behind my limitation, I cushion the negative impact it has on the company I’m with.

Explanations require careful thought
My husband and kids were so excited to be at the Tampa Bay Lightning hockey game. We walked endlessly around Amalie Arena looking for an elevator to take my broken lungs to the top floor. With the game about to start, I could see their excitement shifting to frustration.
My youngest sighed and said, “Can’t we just take the stairs?” I know I should’ve taken a moment to explain how that could backfire, but I have a toxic trait: I think I can overcome any challenge.
Without hesitation, I turned toward the stairwell and braced myself mentally for the battle ahead. My husband sounded the alarms and did his best to discourage me. But stubbornness is my other toxic trait.
I made it up the first flight of 30 to 40 steps, but it didn’t feel good: burning lungs, pounding heart, shock waves of pain, as well as complete disorientation while my vision spun around and around.
It was no surprise that I needed to take a break. Without question, my family huddled around me while I leaned against the wall in full concentration, practicing mind control so I wouldn’t panic.
“Are you ready, Mom?” my teenage daughter asked, with anxiety in her voice.
Here’s where I could’ve said, “I just need another minute.” But if I know teen girls, I’d probably get a quick eye roll. And if I shared the truth, “I just need a minute or I’m going to pass out,” I’d get those uncomfortable, concerned looks of worry.