World-wide shortage of rheumatologists= getting worse. This is why it is getting harder to get an appointment with a rheumatologist.
This NASA night view of the US looks very similar to the recent US map showing where rheumatologists are concentrated in the US. Some states have none!
The worldwide shortage of rheumatologists is getting worse: hold on to your rheumatologist!
If you have difficulty getting in to see a rheumatologist, there are good reasons why.In 2015, there was a shortage of rheumatologists throughout the US. The American College of Rheumatology (ACR) counted 6,013 rheumatology care providers. 4,997 were full-time rheumatologists, 598 worked part-time, and the other 418 were nurse practitioners and physician assistants specializing in rheumatology. This was 13% fewer rheumatologists than needed by the population, according to the ACR. Since then, the numbers have continued to decrease. Many rheumatologists are retiring, and not enough new ones are entering the specialty. In 2020, 100 doctors who wanted to get into rheumatology training programs could not do so because there were not enough training slots.
By the year 2030, the total number of healthcare providers specializing in rheumatology in the US is predicted to drop from 6,013 to 4,133 nationwide. There will also be a greater need for rheumatologists. The number of older people with arthritis (the baby boomers) will be much higher. It is estimated that there will be only half the number of rheumatology healthcare providers that will be needed to care for rheumatologic patients, such as those with SLE.
Some Areas Have No Rheumatologists!
Another problem is that many areas of the US have few to no rheumatologists. Many patients have to travel hundreds of miles to see the closest one. 21% of rheumatologists are in the Northeast while only 4% are in the Southwest, and this imbalance is predicted to get worse. There are much fewer people in the population per rheumatologist in the Northeast compared to the Southwest.
A 2019 US study showed that more than 60% of US patients had to wait more than a month to get a new patient appointment with a rheumatologist. More than a quarter of patients had to wait more than 2 months.
It’s much worse for children with rheumatic diseases (such as pediatric SLE). Only 1 of 4 children with arthritis can see a pediatric rheumatologist. Those fortunate to see one have an average of an hour’s drive. Nine states have no pediatric rheumatologists, and six states have only one.
A 2019 United Kingdom study showed that patients waited for an average of over 6 months after the onset of rheumatologic symptoms before being able to see a rheumatologist. So this is not just a US problem.
Medical Marijuana (Cannabis, Cannabinoids, THC, CBD for Lupus)?
Should patients use CBD for lupus? With the increasing popularity and availability of cannabis and CBD, many lupus patients are asking, “How about CBD for lupus?” This blog post goes over the latest information regarding the research, use, dosing, effectiveness, and potential side effects of CBD, medical marijuana, and medical cannabis for lupus. This information will also be useful for the healthcare provider considering whether their patients should prescribe these products or not. Read on to learn more.
Marijuana (cannabis) and its active components (THC and CBD) has become more popular for medical treatments. As of this writing (April 2021), 42 states in the US allow the use of medical marijuana, and 11 states (and the District of Columbia) have fully legalized its use recreationally. Many of my patients ask about using it, so I think it is important to go over some important information about it.
My goal is to present the facts based on scientific evidence without bias.
Cannabinoids are the active compounds of the cannabis plant. There are over 140 different cannabis-derived cannabinoids known, and each acts differently in the body. The 2 most studied and well-known are cannabidiol (CBD) and tetrahydrocannabinol (THC). THC is the cannabinoid responsible for the “high,” intoxicating effects with recreational users. CBD does not make people “high.”
NOTE: Make sure to read and follow my Lupus Secrets in order to live a Longer and Better Life with Lupus: These are practical, useful tips.
Anifrolumab (Saphnelo) has the lupus community excited as the next FDA-approved drug for lupus. Pronounced saf-NEH’-low
Saphnelo–the 2021 FDA-approved drug to treat lupus (at least the next biologic for systemic lupus). I received this email on 4/18/21 announcing the brand name!, I have never seen a brand name announced before official FDA-approval. Someone must know something I do not know. I suspect that anifrolumab will be the next FDA-approved drug for lupus. Did they get wind of a pending nod from the FDA? This drug has impressive results from its research studies. Let me share a few so all SLE patients who have failed other medications realize that there is HOPE!
anifrolumab (Saphnelo) has impressive results. Many patients with cutaneous (skin) lupus had total to near total clearing of their skin inflammation as shown in this pic … NOTE… this picture comes directly from the following research study found on the internet. The photo is owned by the ACR’s Arthritis and Rheumatology journal: Furie R, Khamashta M, Merrill JT, Werth VP, et al; CD1013 Study Investigators. Anifrolumab, an Anti-Interferon-α Receptor Monoclonal Antibody, in Moderate-to-Severe Systemic Lupus Erythematosus. Arthritis Rheumatol. 2017 Feb;69(2):376-386. doi: 10.1002/art.39962. PMID: 28130918; PMCID: PMC5299497.
Research results “in a nut shell” in easy-to-understand language:
The clinical trials for anifrolumab were called the TULIP trials TULIP comes from the full research study name: Treatment of Uncontrolled Lupus via the Interferon PathwayHow does it work?
Most (70%) of systemic lupus erythematosus (SLE) patients have high levels of interferons
Interferons cause inflammation of organs, leading to permanent organ damage
Anifrolumab competes with interferon by attaching to the attachments (receptors) on immune system cells
Normally, when interferons bind to those receptors, the immune system cells become more active
Then they alert more immune system cells to become more active and attack body organs
Anifrolumab decreases this cascade of events from happening
This leads to less inflammation
It is a biologic monoclonal antibody, so it will be expensive!
Anifrolumab (Saphnelo) works differently than any drug used in the past to treat lupus!
1st in its class! With it aimed at a major cause of lupus (high type-1 interferon levels), it should be the next FDA-approved drug for lupus.
Saphnelo, the next FDA-approved drug to treat lupus: How is it given?
Saphnelo is given intravenously (IV), 300 mg, every 4 weeks (I suspect it will be 300 mg)
What were the research results?
There was a 90% reduction of interferons at 6 months in the patients who started off with high levels
Other labs measuring lupus inflammation improved (anti-dsDNA, C3, C4, CH50)
Low white blood cell counts and low platelet counts improved
What other good things did it do for the SLE patients?
They had less disease activity
Even patients who started with normal interferon levels overall improved
In TULIP-2: 57% (46 placebo vs 72 anifrolumab patients) more patients who started with high interferon levels met the primary endpoint with anifrolumab
… 58% more patients! Impressive!
When looking at all patients, a 51% higher number of patients responded to anifrolumab, including those with normal interferon levels!
You do not have to have high interferon levels to respond!
Close to twice as many patients were able to decrease their steroids to goal (25 placebo vs 45 on anifrolumab)
Lower steroids is important because higher doses of steroids cause organ damage
Anifrolumab reduced lupus flares
It especially worked well for cutaneous lupus
Look at the picture above from one of the studies
This patient had an amazing improvement. Many others did just as well
They measured skin activity with a CLASI score and looked for more than a 50% improvement
CLASI stands for Cutaneous Lupus Erythematosus Disease Area and Severity Index
Twice as many anifrolumab patients responded: No wonder Saphnelo is the next FDA-approved drug to treat lupus
-The numbers were 25% of the placebo cutaneous lupus patients responded, while 49% on anifrolumab did
How fast does it work?
Reduced disease activity was seen as early as 2 months
What were the side effects of anifrolumab
When comparing side effects, it is important to know that the placebo patients were not truly “placebo”
They were receiving standard of care therapy
In other words, most were on immunosuppressants and steroids that cause side effects themselves
The patients on anifrolumab were also on immunosuppressants and steroids
Therefore sorting out what anifrolumab may do vs the other drugs is very difficult
But, here are the numbers (combination of three different anifrolumab research studies):
Around 87% of anifrolumab patients and 80% of placebo patients had side effects
This include nuisance, mild side effects
The most common were viral upper respiratory tract (URI) and throat infections (cold-like infections)
Infusion reactions were the other most common
For example, 10% of the placebo (standard of care) patients had URIs, 18% on anifrolumab did This is not surprising, interferon is important for fighting viral infections Shingles occurred in around 6% of anifrolumab patients and 1%-2% of placebo patients
Again, not surprising since shingles is due to chicken pox virus
(MAKE SURE TO GET A SHINGLES VACCINE, SUCH AS SHINGRIX, BEFORE TREATMENT!)
6% of anifrolumab patients had to stop treatment due to side effects; 3% of placebo patients had to stop
What patients were not studied?
Patients with severe kidney inflammation (lupus nephritis, LN), brain, and nerve lupus (neuropsychiatric) were not
Therefore, we do not know if it works in them
However, I will go out on a limb and predict it will help LN patients
We know that interferon plays a role in LN
Just because we do not have study results for severe LN patients, this does not mean it does not work
What could possibly keep the FDA from approving it?
In the first TULIP-1 trial, it did not meet its primary endpoint (research goal called the SRI-4)
This could get some voting members to vote “no” for approval
However, lupus experts realize how hard it is to prove that lupus drugs work
They evaluated how the drug performed in the 1st trial
Patients did have significant improvements
They were able to lower their steroids and there was marked improvements in cutaneous lupus
One criticism is that some patients changed their NSAID (like ibuprofen) during the study
These patients were automatically labeled as a drug failure, though many of them had great responses
So they changed the primary endpoint for TULIP-2 based on their analysis of TULIP-1 The TULIP-2 trial did meet its primary end point
Bottom lines- Saphnelo, the next FDA-approved drug to treat lupus:
– Impressive results, especially for skin lupus
– Low rate of side effects (colds, sore throats, infusion reactions, shingles are the most important)
– Get your Shingrix shot before treatment
– I have a list of SLE patients who have failed everything who need to try this drug if it is FDA-approved!
Good luck to AstraZeneca who is the manufacturer of Saphnelo
Update: August 2021
2020 and 2021 were monumental years for people who have lupus. Belimumab (Benlysta)became the first FDA-approved drug for lupus nephritis December 17, 2020. Voclosporin (Lupkynis) became the second FDA-approved drug for lupus nephritis on January 22, 2021 with the advantage of its being a pill rather than an injection. Then, anifrolumab (Saphnelo) was FDA-approved to treat lupus (the systemic form) on August 2, 2021!
Furie R, Khamashta M, Merrill JT, et al.; for the CD1013 Study Investigators. Anifrolumab, an anti-interferon-alpha receptor monoclonal antibody,in moderate-to-severe systemic lupus erythematosus.Arthritis Rheumatol. 2017;69(2):376–86.Furie R, Morand EF, Bruce IN, Manzi S, Kalunian K, Vital EM, et al. Type I interferon inhibitor anifrolumab in active systemic lupus erythematosus (TULIP-1): a randomised, controlled, phase 3 trial. Lancet Rheumatol. 2019;1(4):e208–19.
Morand EF, Furie R, Tanaka Y, Bruce IN, Askanase AD, Richez C, et al.; TULIP-2 Trial Investigators. Trial of anifrolumab in active systemic lupus erythematosus. N Engl J Med. 2020;382(3): 211–21.
Tanaka Y, Takeuchi T, Okada M, Ishii T, Nakajima H, Kawai S, et al. Safety and tolerability of anifrolumab, a monoclonal antibody targeting type I interferon receptor, in Japanese patients with systemic lupus erythematosus: a multicenter, phase 2, open label study. Mod Rheumatol. 2020;30(1):101–8.
Tanaka Y, Tummala R. Anifrolumab, a monoclonal antibody to the type I interferon receptor subunit 1, for the treatment of systemic lupus erythematosus: an overview from clinical trials. Mod Rheumatol. 2021 Jan;31(1):1-12. doi: 10.1080/14397595.2020.1812201. Epub 2020 Sep 17. PMID: 32814461.
We also need healthy volunteers and patients who have other autoimmune diseases as well. Share this information with your friends, family, and loved ones. You must live close enough to the NIH in Bethesda, Maryland in order to participate as you need to travel there for the study.
Note that they will reimburse you for your participation. You will also find out what your SARS spike protein antibody result is (how well you responded to the vaccine).
“Can discoid lupus turn into systemic lupus erythematosus?”
Discoid lupus: note the round “disc” shape, the outer active inflamed border is pink, the central area has scar tissue
“Can discoid lupus turn into systemic lupus erythematosus?”
– The above photo is a classic example of discoid lupus – Around 15% of people with systemic lupus erythematosus (SLE) will also have discoid lupus erythematosus (DLE) – If someone develops DLE by itself, it can potentially evolve to SLE over time – SLE will develop in 25% to 30% of children who have DLE – In adults, it depends upon how extensive the DLE is: – if the DLE is just above the neck (localized DLE), that person has approximately a 10% chance of SLE – If the DLE is above and below the neck (generalized DLE), there is a 1 out of 4 chance of evolving to SLE
A ray of hope
– People who have DLE that evolves to SLE tend to have a milder form of SLE. They are less likely to have severe organ involvement, such as lupus nephritis (kidney inflammation) compared to SLE patients who do not have DLE
– However, this rule is not 100%. There are some DLE patients who will develop severe SLE. This more commonly occurs in those with severe DLE that is generalized. – Make sure to follow all the advice in the “Lupus Secrets” to ensure you have the greatest chance of not developing severe SLE
If you have discoid lupus… what is your experience and advice for others. COMMENT above
Pons-Estel, G. J., Aspey, L. D., Bao, G., Pons-Estel, B. A., Wojdyla, D., Saurit, V., … Drenkard, C. (2017). Early discoid lupus erythematosus protects against renal disease in patients with systemic lupus erythematosus: longitudinal data from a large Latin American cohort. Lupus, 26(1), 73–83. https://doi.org/10.1177/0961203316651740
A drug made from up to 100,000 healthy volunteers!
Intravenous (IV) means getting a medicine delivered into your vein
Intravenous Immune Globulin (IVIG)
Immune globulins (also called immunoglobulins) are antibodies. A large number of antibodies given in IVIG may help in some autoimmune disorders such as polymyositis and dermatomyositis. However, IVIG is not commonly used for SLE. It is useful in people who have a deficiency in IgG immune globulins (medically known as hypogammaglobulinemia) and have recurrent infections due to this. A person with hypogammaglobulinemia can benefit significantly from IVIG because it replaces the absent IgG globulins needed to fight infections.
Studies evaluating IVIG use in cutaneous lupus (chapter 8), lupus nephritis, muscle inflammation, low blood cell counts (to include myelofibrosis), lupus lung inflammation, myocarditis, arthritis, severe antiphospholipid syndrome, and neuropsychiatric lupus, have had encouraging results. A small 2012 study also suggested that it may help prevent congenital heart block in women with lupus who are SSA or SSB antibody positive. However, larger studies will be needed to see if IVIG should be used more often to treat lupus.
It can take up to 100,000 healthy blood donors to produce one dose of IVIG!
IVIG is produced from the antibodies of healthy blood donors. It takes anywhere between 1,000 and 100,000 donors for one dose. The preparation undergoes treatments to kill any potential germs and is thoroughly tested before using it for therapy.
Cartoon depiction of an immunoglobulin molecule. Photo: TimVickers at Wikipedia
How IVIG works: IVIG can decrease immune system overactivity.
What benefits to expect from IVIG:
IVIG can decrease infections in people with low levels of IgG globulins. It can help the conditions noted in the paragraph above. In some people, it works rapidly, even within days after the first treatment.
How IVIG is given:
Usually given as an intravenous (IV) infusion once or twice a month. A self-injectable form that can be given at home is available.
If you miss a dose of IVIG:
Reschedule as soon as you realize that you missed your dose, then reset your schedule from that point. Consult with your prescribing doctor to double-check these instructions, but these guidelines will be enough for most people.
What needs to be monitored while you get IVIG:
You will need to have periodic lab tests, including blood cell counts and kidney function tests.
Reasons not to take IVIG:
Many IVIG brands are unsafe to receive if you have a deficiency in IgA globulins. Your IgA globulin level can be measured using a blood test similar to the one that measures IgG globulin levels. Gammagard S/D and Polygam brands of IVIG are generally safe to use if you are IgA deficient. Pre-existing heart disease, kidney disease, and a history of blood clots may increase your risk of having these sorts of problems on IVIG. Discuss these possibilities with your doctor.
If you have severe IgA deficiency, you should receive IVIG that does not contain IgA, or it should be provided in a hospital setting. Patients with severe IgA deficiency can sometimes develop severe allergic reactions (anaphylaxis) to IVIG containing IgA immunoglobulins.
While taking IVIG therapy:
See your doctor regularly for appropriate blood tests. Seek medical attention immediately if you develop a red, painful, swollen leg; shortness of breath; chest pain; slurred speech; or arm or leg weakness.
Vaccines and IVIG:
No need to stop drug or time dosing with inactivated vaccines (such as for influenza, pneumonia, and COVID-19). IVIG may decrease the response to some live vaccines. Ask your doctor about timing with any live vaccines (such as Zostavax, yellow fever, MMR, and others).
Pregnancy and breastfeeding while on IVIG:
IVIG can be used for severe lupus flares during pregnancy. Some think it may help prevent congenital heart block progression due to anti-SSA antibodies.
It can be used during breast-feeding.
Older people and IVIG:
There may be an increased risk for side effects such as kidney problems, heart problems, and blood clots.
What to do at the time of surgery with IVIG:
IVIG can potentially increase blood clots and probably should not be used close to the time of surgery. Check with the prescribing rheumatologist and infusion doctor for more information.
High blood pressure = common, treated with blood pressure medicines Severe allergic reaction (anaphylaxis, low blood pressure, wheezing) = uncommon, treated by the nurses during the infusion Severe headache, aseptic meningitis = uncommon. May be prevented with antihistamines, steroids, and pain medicines. May need to stop medicine. Kidney failure = rare Increase in lupus flares = rare Blood clots = rare
Side effect incidence key (approximations, as side effects can vary widely study to study): rare < 1% occurrence; uncommon 1%–5% occurrence; common > 5% occurrence
If you have been treated with IVIG, what was your experience like? Please share in the Comments section above
The above is an excerpt from “The Lupus Encyclopedia” by Johns Hopkins University Press. This is a preliminary draft for the 2nd edition, before print