FDA Approves Drug to Treat Rare Autoimmune Disease

The U.S. Federal Drug Administration (FDA) has approved a drug manufactured by ChemoCentryx to treat a rare group of autoimmune diseases, called anti-neutrophil cytoplasmic autoantibody-associated vasculitis, or ANCA-AAV for short. According to ANCA Vasculitis News, ANCA-AAV causes inflammation and damage to the body’s small blood vessels. This inflammation is the result of antibodies that bind to certain cells of the immune system, called neutrophils, and overly activate them.

Since small blood vessels are found throughout the body, ANCA-AAV causes a variety of symptoms, including:

  • Poor kidney function, leading to kidney failure
  • Severe respiratory problems, including shortness of breath, hoarse voice, cough with blood or mucus, and chest pain
  • Neurological symptoms, including tingling, burning, numbness, and weakness
  • Central nervous system (CNS) involvement, such as headaches, cognitive impairment, and memory deficits. In severe cases, seizures, paralysis or loss of consciousness may also result.
  • Ear, nose, and throat (ENT) symptoms, like sinusitis, nasal discharge, rhinitis, and tinnitus (ringing in the ears)
  • Eye symptoms, including eye pain, vision impairment, and vision loss
  • Joint pain, muscle pain, and muscle loss
  • Skin lesions, including rashes, sores, ulcers, bumps, and bleeding underneath the skin
  • Digestive problems, like vomiting, diarrhea, nausea, abdominal pain, and blood in one’s stool
  • And more…

Due to the numerous symptoms ANCA-AAV causes, it can often be fatal for patients; in fact, ChemoCentryx states that the first year mortality rate for patients living with the disease is between 11 and 18 percent. Current ANCA-AAV treatments on the market include steroids and immunosuppressant medications which compromise the body’s ability to fight off infections. That’s why having a new treatment on the market that works via a different mechanism may be a saving grace for many ANCA-AAV patients.

The new drug, called avacopan, will be sold by ChemoCentryx under the brand name Tavneos. It works by blocking a protein called C5a receptor that is responsible for causing numerous inflammatory diseases. The drug’s wholesale price will be an astronomical $150,000-$200,000 per patient per year. However, it could be the life-saving treatment that the 40,000 ANCA-AAV patients in the US need.

The drug is currently being tested for use on other conditions as well, including the autoimmune skin disease Hidradenitis Suppurativa (HS). The drug has already received approval for commercialization in Japan, and is expected to be approved in Europe by the end of 2021.

Martina, a 29-year-old mother-of-two from Stuttgart, Germany, describes what it was like being diagnosed with this challenging autoimmune condition:

“It began rather unspectacularly, with flu-like symptoms, fever, headaches, and joint pain. So anyone would think, ‘It’s just a normal flu.’ Then it got worse over the course of a week and I went to the hospital.”

She was eventually diagnosed with ANCA-AAV after undergoing a series of blood tests. Unfortunately, she had to give up her career as an educator in the process, since she didn’t have a strong enough immune system to be interacting with children while taking steroids and immunosuppressants to control the disease. Patients like Martina are the ones that could potentially stand to benefit from new drug therapies like avacopan.

Martina, 29, appears in an ad to raise awareness for vasculitis conditions.

To find resources for those living with ANCA-AAV and their caretakers, visit: https://www.myancavasculitis.com/living-with-aav/

Laser Hair Removal for Hidradenitis Suppurativa (HS)

Can laser hair removal be an effective treatment for patients with Hidradenitis Suppurativa (HS)? Photo via Good Housekeeping.

For those of you who are new to the Autoimmune Warrior blog, I have two autoimmune conditions – Sjogren’s Syndrome and Hidradenitis Suppurativa (HS). Over the years, my HS has taken a backseat while I’ve dealt with my Sjogren’s symptoms.

In the past year, however, I’ve been more active about working with my dermatologist to manage this chronic autoimmune skin condition. By actively managing my HS symptoms, my hope is that I won’t move past Hurley Stage I of the disease, or even go into remission.

As I noted in my last blog post about my new Hidradenitis Suppurativa treatment plan, I’ve been working with a new dermatologist who has prescribed me a topical cream called resorcinol, in addition to the clindamycin and Hibiclens that I routinely use. Besides topical treatments, however, I’ve also started getting laser hair removal in my groin and underarms, which are the main areas where Hidradenitis Suppurativa affects me.

What does the science say?

For those who are unaware, laser hair removal has been cited as a way to reduce HS symptoms like boils and abscesses in the groin, underarms, and elsewhere in the body’s axillary regions. The logic is that, by using a laser to destroy your hair follicles, the follicle cannot get clogged; this is important, since, as my dermatologist explained, follicular occlusion is one of the main parts of the disease.

In fact, studies have shown that patients with Hidradenitis have seen improvement in their HS symptoms after receiving laser hair removal treatments. A 2011 study found that when 18 patients were treated in a single area affected by Hidradenitis twice a week with intense pulsed light over four weeks, they experienced ‘significant improvement’ in the mean examination score of their lesions. The patients also reported being ‘highly satisfied’ with their treatment.

Laser hair removal in HS patients

There are different types of lasers that can be used for laser hair removal. Some of the more effective ones have been found to be the long-pulsed lasers such as the IPL and Nd:YAG laser. The Nd:YAG laser in particular has found to be more effective on darker skin tones; this is because the laser needs to distinguish between your hair and skin color in order to work. Some clinical trials using the CO2 laser have also shown promise in the treatment of HS, but larger study samples are needed.

In addition to these studies, anecdotal evidence from other HS patients is what motivated me to move forward with getting laser hair removal to treat my hidradenitis. Reading the experiences of other bloggers who are living with the condition and have found positive results after laser hair removal gave me hope that I could experience the same benefits.

Drawbacks of laser hair removal for Hidradenitis Suppurativa

There are, however, some drawbacks to consider when getting laser hair removal to treat your HS. If you are at Hurley Stage III of the disease, for example, laser hair removal may not be the best option for you, since your skin is highly sensitive, and the laser may exacerbate inflammation and cause undue pain to the area(s) affected. Also, the laser may not be able to penetrate scar tissue that has formed as a result of your HS. For patients at an advanced stage of the disease, wide-excision surgery or deroofing may be better options instead, in combination with antibiotics or even immunosuppressants like Humira. In summation, laser hair removal is a more practical option for those with Hurley Stage I or II of the disease.

Also, though laser hair removal technology continues to evolve, if you have a darker skin tone and dark hair, or a lighter skin tone and light-colored hair, you may not be a good candidate for laser hair removal, since the laser may not be able to distinguish between your hair and skin.

Another drawback is the expense. Laser hair removal can cost thousands of dollars, depending on the type of laser used, the number of treatments necessary to see results, and the size of the area. Getting your laser treatment done at a medical clinic by a doctor or nurse, or at a beauty salon by a certified technician, may impact the price you pay. Furthermore, many health insurance plans do not cover the cost of treatment, since, despite the research out there, laser hair removal is still not a universally recognized treatment for hidradenitis suppurativa, and is viewed as a cosmetic procedure.

Finally, laser hair removal can take a long time. At the clinic I am going to, laser hair treatments are usually delivered every 4-8 weeks, depending on the area being treated. I am getting treated every 6 weeks, and while I think it is worth it to see results, patients looking for a more immediate change may be disappointed with such slow progress.

Should I get laser hair removal to treat my HS?

In conclusion, whether or not you should move forward with getting laser hair removal to treat your hidradenitis suppurativa symptoms is really a decision that should be made between you and your dermatologist. While HS is not an easy condition to live with, as biotechnology and pharmaceutical treatments evolve, and as patients and medical professionals become more aware of alternative treatment methods, there is hope for those living with HS.

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My New Hidradenitis Suppurativa Treatment Plan

Last year, I switched health insurance plans, and as a result, the dermatologist that I was seeing to treat my hidradenitis suppurativa (HS) was no longer in-network. So, I had to find a new physician. I found a new dermatologist in my area that was covered under my health insurance plan, so I made an appointment and saw her for the first time last week.

My last physician – who diagnosed me with this chronic autoimmune skin condition – had been treating my hidradenitis by giving me cortisone (steroid) injections. While this was effective to control the inflammation and pain caused by my HS boils, it wasn’t really a long-term solution. I had also been prescribed clindamycin lotion to put on my hidradenitis lesions after showering, and over-the-counter Hibiclens, a wash that medical staff use to ‘scrub in’ in order to prevent any infections from occurring. However, neither of these topical solutions have gotten rid of my HS boils.

My new dermatologist was very caring, and didn’t rush me at all. She suggested that I use a new topical solution called resorcinol. Resorcinol is not covered by my health insurance, since it’s not made by a typical pharmacy, but rather, a compounding facility. While this is a downside, I did some research about the medication, and according to the Journal of the American Academy for Dermatology (JAAD), a 2010 study found that when patients with Hurley stage I or II HS used topical 15% resorcinol twice daily for 30 days, they saw a ‘marked decrease in pain and mean duration of the lesions’.

Below are the side-by-side results from the study. The first left-side image (A) is the patient’s baseline state, the second (B) is seven days into treatment, and the third image (C) is 30 days into the treatment. The right-side images (D, E, and F) are the respective lesions shown via ultrasonography.

Image courtesy of the JAAD (Journal of the American Academy for Dermatology).

Beyond the resorcinol, I spoke with my dermatologist about a medication called spironolactone for hidradenitis suppurativa treatment. Spironolactone is commonly used to treat acne and other similar skin disorders. Although HS is sometimes referred to as ‘acne inversa’, anyone with the condition knows that it’s not acne – not even close! That being said, HS may be similar to acne in the sense that it may be affected by hormones. Plus, a 2017 study of 46 women with HS found that acne and polycystic ovarian syndrome (PCOS) were the most commonly reported comorbidities with hidradenitis. The same study found that spironolactone was effective, even in small doses, for managing patients’ pain and inflammatory lesions.

Though I haven’t decided whether or not to move forward with spironolactone treatment yet, I thought it was interesting that there was a potential connection between HS and hormones, especially since I myself have adult acne and have had multiple ovarian cysts in the past. Plus, I have Sjogren’s Syndrome, an autoimmune disease that may also have a hormonal component. I do notice that my hidradenitis boils do worsen on a pre-menstrual basis.

I also discussed with my dermatologist getting laser hair removal in the areas most commonly affected by HS – the groin and underarms. She did say that laser hair removal does help with the follicular occlusion aspect of the disease. Obviously, there are drawbacks – for example, laser hair removal is almost impossible to do in patients with Hurley stage III of the disease, because it’s simply too painful. It’s also not usually covered by insurance, since it’s considered a cosmetic procedure, rather than a medical one. But, it’s another option that I want to consider since I’m still at stage I of the disease, and would rather have laser hair removal than wide-excision surgery.

Do you have hidradenitis suppurativa? If so, what treatments have been most effective for you? Let me know in the comments below!