Category: research

Still’s Disease Onset After mRNA COVID-19 Vaccine

Posted on by Grayson

An 82-year-old woman was diagnosed with still’s disease following an mRNA COVID-19 vaccination. Onset after age 80 is really uncommon, making this an interesting study.

Now, it’s important to remember that these kinds of reactions are incredibly rare. Less than 30 patients have had this happen. Please still get your COVID-19 vaccines if you’re medically able to do so.

Check out the full case study:

Nishioka H, Shirota S (January 02, 2024) Adult-Onset Still’s Disease After an mRNA COVID-19 Vaccine in an Older Woman. Cureus 16(1): e51540. doi:10.7759/cureus.51540

JAK Inhibitors & ILD in Pediatric Rheumatology Patients

Posted on by Grayson

Interstitial Lung Disease (ILD) is a known complication of many rheumatic diseases. It can be hard to treat and get into remission.

One study published on December 21, 2023, digs deep into one possible treatment. Janus kinase inhibitors (JAKis) include drugs such as Tofacitinib, Baricitinib, Peficitinib, Upadacitinib, and Filgotinib.

After exploring JAKis, the study calls for more research.

Read the full article below.

Sapountzi E, Fotis L, Kotanidou E, et al. (December 21, 2023) Janus Kinase Inhibitors and Interstitial Lung Disease Associated With Pediatric Rheumatic Diseases: An Unexplored Field. Cureus 15(12): e50928. doi:10.7759/cureus.50928

New MAS Warning Sign Discovered

Posted on by Grayson

A journal article published on December 20, 2023, shows the discovery of a new MAS warning sign.

Intermediate monocytes produce TNF and IL-1B. Medications, such as TNF-inhibitors and IL-1B inhibitors, help suppress the ongoing inflammatory responses these IMs bring.

IMs are also known to increase inflammation within the cardiovascular system. This may be a part of why many rheumatic patients also have cardiovascular disease.

For people with adult-onset Still’s (AOSD) specifically, there was an uptick in the measured IMs leading up to and during MAS events. This could help explain why the IL-1B inhibitor Anakinra is a go-to treatment for MAS.

The study even went on to point out that this could become a biomarker, allowing us to catch MAS events earlier. This would hopefully lead to better outcomes, too.

Read the full article below.

Jia, J., Wang, M., Ma, Y. et al. Neutrophil extracellular trap-induced intermediate monocytes trigger macrophage activation syndrome in adult-onset Still’s disease. BMC Med 21, 507 (2023). https://doi.org/10.1186/s12916-023-03231-9 

Young Rheumatology Patients Not Counseled on Sexual Health

Young Rheumatology Patients Not Counseled on Sexual Health

Posted on by Grayson

Brittany M. Huynh, MD, MPH, presented an abstract at ACR Convergence of her paper, Adolescent and Young Adult Rheumatology Patient Reports of Reproductive Health Screening and Counseling in the Clinical Setting.

In this paper, Huynh and her colleagues found that only 38% of pediatric rheumatologists were screening patients between 14-23 years of age for sexual activity. Only 17% of patients surveyed had conversations with their rheumy about pregnancy prevention.

Thankfully, this number was higher for the 36% of surveyed patients that were on teratogenic drugs — those that would harm a fetus. 54% of these patients were screened for sexual activity and 44% were counseled on avoiding pregnancy. However, only 62% of these patients knew their medications would harm a fetus.

We already know that there’s a general lack of these conversations happening across healthcare. Many healthcare providers don’t feel like they know enough about this topic to talk to their patients. Others may struggle with getting their pediatric patient alone to have these conversations without a parent or caregiver present.

As a sex educator, I also wonder how many of these conversations were affected by the overturn of Roe v. Wade. Many clinic systems and professional organizations have struggled with how to highlight the importance of this issue. That’s especially true in states banning similar conversations or punishing healthcare providers for having them.

For any rheumatology professionals looking into how to have these conversations, consider reaching out! I’m always happy to talk shop, consult, or work with your clinic on how to best serve this population.

Photo from The Gender Spectrum // VICE

The Still’s disease Continuum

Posted on by Grayson

Earlier this year, rheumatologists at EULAR reached a consensus that Adult-Onset Still’s Disease and Systemic-Onset JIA are the same disease.

A new study using data from the International AutoInflammatory Disease Alliance Network registry has proved this decision correct. Despite some minor differences across age groups, this is in fact the same disease.

You can read the full journal article on the BMJ.

Survey on Rheumatoid Arthritis and Exercise

Survey on Rheumatoid Arthritis and Exercise

Posted on by Grayson

We are exploring the preferences and opinions of different types of exercise in adults with Rheumatoid arthritis. If you are older than 18 years and have been diagnosed with Rheumatoid Arthritis, you are eligible to participate.

What is the purpose of this study

This research aims to identify the exercise preferences of individuals with Rheumatoid Arthritis. The opinion on a new type of exercise, known as Blood Flow Restricted training, will also be explored.

What will I do

You will be asked to complete a ~15-minute online survey. The survey will contain questions regarding current pain scores and activity levels, exercise preferences, and your perception of blood flow restricted training.

Risks

While we do not believe there are any inherent risks associated with this research, you will be asked some questions pertaining to your perceived levels pain. Some of these questions could elicit feelings of distress.

The expected benefits of the research

We cannot guarantee or promise that you will receive any benefits from this research. However, the results of this research will be used to guide future exercise interventions tailored to individuals with RA, which may improve the quality and acceptability of research in this field.

What happens to information about me

All records containing personal information will be confidential and no information which could lead to your identification will be released unless required by law. Data will be kept for 5 years. Results will be reported in scientific publications and industry reports in a way in which you cannot be identified. Your data will remain anonymous.

Questions about the research, complaints, or problems

If you would like more information about the study, please download the Participant information sheet (participant information sheet) or email hunter.bennett -at- unisa.edu.au if you have any questions.

 

Click here to learn more

Continuous Anakinra IV for a Patient With Macrophage Activation Syndrome

Continuous Anakinra IV for a Patient with MAS

Posted on by Grayson

The following is from a research article published August 4, 2023.

Abstract

Macrophage activation syndrome (MAS) is a type of hemophagocytic lymphohistiocytosis (HLH), which occurs due to excessive stimulation of the immune system. Common precipitants of MAS include disseminated infection or underlying rheumatologic disorders such as adult-onset Still’s disease which is characterized as an inflammatory arthritis with daily fevers and a salmon-colored rash. We present a case of a patient with probable adult-onset Still’s disease and subsequent disseminated cytomegalovirus (CMV) infection, who met the criteria for MAS based on the presence of a fever, cytopenia in multiple cell lines, elevated ferritin, presence of hemophagocytosis on bone marrow, low fibrinogen, and mild splenomegaly on physical exam. The patient responded to treatment with continuous anakinra infusion and ganciclovir for treatment of CMV. Though cytotoxic medications such as etoposide have traditionally been considered first-line treatment for HLH/MAS, interleukin-1 inhibitors such as anakinra are emerging as a less cytotoxic alternative.

Key Quotes

MAS is often considered the most feared consequence of patients with AOSD and has been reported to occur in close to 15% of these patients. Treatment options for MAS currently include IV Igs, corticosteroids, etoposide, cyclosporine, tocilizumab, and anakinra. Though anakinra is generally administered via the subcutaneous route, the IV route may be preferred in critically ill patients due to increased absorption and a decreased risk of bleeding if an underlying coagulopathy is present.

Recent case series and reports have highlighted the role of IV anakinra infusion at doses of 1-2 mg/kg in the treatment of patients with macrophage activation syndrome.

What does this mean?

We have some new-ish options for treating MAS! Now, that isn’t new as in a new drug, but it is a new delivery method for Anakinra (Kineret) specifically.

This is important for a range of reasons, but especially considering that MAS — “the most severe form of cytokine storm” — has occurred in COVID-19 patients. Between happening during infection, we also know that COVID-19 causes major upset to the body’s systems, leading to the development of a host of conditions that may themselves cause MAS. And, in children, MIS-C may often appear — a condition somewhere between MAS and Kawasaki Syndrome.

In the rheumatology world, MAS is most common in AOSD and SJIA, happening in 10-15% and roughly 10% of patients respectively. It can occur in patients with other autoimmune and autoinflammatory arthritis types, most often Systemic Lupus Erythematosus (SLE). According to research, mortality in MAS cases is between 20-53%. It’s hard to know exact numbers when MAS may not be caught before a patient dies — if they even can access healthcare and try to do so.

This is also part of why it is imperative for AOSD, SJIA, and SLE patients to all know the early signs and symptoms of MAS — and to trust your gut if you feel something is really wrong.

Citation

Gullickson M, Nichols L, Scheibe M (August 04, 2023) A Novel Therapy for a Rare Condition: Continuous Anakinra Infusion for a Patient With Macrophage Activation Syndrome. Cureus 15(8): e42968. doi:10.7759/cureus.42968

disabled and pregnant? research opportunity

Are you disabled / chronically ill and pregnant?

Posted on by Grayson

The follow is a call for research participants to test a tool. I’m not involved, but it popped up on my radar, but I’m sharing here.

Brandeis University, the Cincinnati Children’s Hospital Medical Center, and others have worked together to develop a new tool – an Accessible Pregnancy Action Plan. This tool would help pregnant people with disabilities think about what they need during pregnancy, during birth, and after having their baby.

They are looking for people between 12 and 36 weeks along in their pregnancies who have disabilities / chronic illnesses and also use English or American Sign Language (ASL) to communicate.

Participants will work on their Action Plan with a peer facilitator, who is also a parent with a disability. These meetings will take place over Zoom. There will be between 2 and 4 meetings.

Most participants will complete the intervention in two sessions. They will be paid $50 per session. Participants who complete the program will also be compensated an additional $50.

Learn more about the study and take the screener here.

screener poster (has all the same information as in the Brandeis link)

Tocilizumab-induced Hypofibrinogenemia in SJIA Patients

Posted on by Grayson

Tocilizumab (or Actemra) is an IL-6 inhibitor commonly used to treat SJIA and other types of autoimmune and autoinflammatory arthritis. As with all medications, we know that tocilizumab has a variety of side effects. These include lowering blood cell counts, making it easier to catch upper respiratory infections, and allergic reactions (in extreme cases).

Before we dig into the latest study on tocilizumab and hypofibrinogenemia, what in the world is hypofibrinogenemia?

Fibrinogen is a protein that is created in the liver. The body uses this, along with other proteins, to form blood clots and stop bleeding. Depending on how often you’ve run into issues with this protein, you may also know it as Coagulation Factor 1. Blood tests like Factor 1 and serum fibrinogen help monitor the level of fibrinogen in the body, either taking a look at how well the protein is working or what level of the protein is in your blood. Medscape has a really deep dive for those interested.

Generally speaking, hypofibrinogenemia is a rare, inherited blood disorder. They’ve found some responsible chromosomes for this. What hypofibrinogenemia does is present as an abnormality in the quality of fibrinogen, and so it causes issues with blood clotting. Folks with this condition might have things like GI bleeding, excess bleeding following surgeries or procedures, or even events like miscarriages. This can often present as bloody noses that take a long time or are difficult to stop. People may require plasma infusions all the way up to a liver transplant, depending on the severity of this condition. They also have to avoid medications such as NSAIDs that tend to alter bleeding and platelets.

Now, what does this have to do with tocilizumab?

According to a study in Nature, tocilizumab can cause hypofibrinogenemia in SJIA patients. They found that 76.47% of SJIA patients receiving tocilizumab had hypofibrinogenemia. The patients with this condition had a variety of disease activity, including inactive disease, and some were on both methotrexate and prednisone as well. However, those medications were determined to have negligible effects on developing hypofibrinogenemia.

It’s important to note that there was a pretty small number of patients retroactively studied. Around 60% of these patients had no specific symptoms, but some symptoms included bleeding gums and increased bruising. Only one patient studied struggled with nose bleeds.

Why is this happening?

IL-1, IL-6, and TNF all affect fibrinogen. IL-1 and TNF negatively affect it, meaning those of us on IL-1 or TNF inhibitors may receive a boost in our fibrinogen levels due to lower IL-1 and TNF levels. Makes sense, right? Well, IL-6 positively affects fibrinogen. That means IL-6 inhibitors like tocilizumab may cause issues such as hypofibrinogenemia.

Should I be worried?

Worried? No. Proactive? Yes.

First, some notes:

They did test these patients and found they did not have the chromosomes related to hypofibrinogenemia. That means this may not have been an inherited condition for them OR there may be a link between SJIA and this condition that we’re unaware of at the moment.

The researchers admit that this retroactive study with a small number of patients means not enough overall data was collected, so a lot more research needs to be done. They also wondered if there’s a difference for adults versus children when it comes to this, especially since hypofibrinogenemia set in after 1-4 doses of tocilizumab in pediatric patients.

Now, as far as being proactive:

If you or your child is on tocilizumab, it’s worth keeping track of symptoms that may be related – nose bleeds, increased bruising, etc. These could be explained by anemia, which needs to be treated, too. However, it’s worth sharing this study with your rheumatologist to see if they can test your fibrinogen levels if/when they test for anemia as well.

You can read the full study here.

Survey: Talking to HCPs and Access 2023

Survey: Talking to HCPs and Access

Posted on by Grayson

Back in 2016, I collected some initial information about how people felt they could talk to healthcare providers (HCPs) about sexual health and function, sexual orientation, and gender. Then, in the fall of 2019, I conducted a more extensive survey. I’m still working on the results of that survey.

With the changes occurring in the last few years, though, I wanted to collect information again. Allow me to introduce you to the new 2023 survey.

This survey asks the same questions as before, such as “Have any of your physicians or other healthcare providers talked to you about sex or sexual issues?” It also has a section about how recent anti-equity efforts, such as rampant transphobia, have affected these conversations.

This survey is open as of May 28, 2023, and will close at the end of the day on July 31, 2023. It is only open to US residents aged 18 and up.

Take the survey here.