Updated: Jun 30, 2022
Maybe you've heard of it. Selena Gomez is a suffer of #Lupus and necessitated a kidney transplant because of nephritis. One of my favorite celebrities, Lady Gaga, has also tested borderline titers for Lupus but has yet to present overt symptoms. Kristen Johnston from 3rd Rock from the Sun also suffers from Lupus, specifically lupus myelitis which affects the spinal cord. She sought help when she had difficulty climbing the stairs and had to endure chemotherapy. Singer/songwriter, Seal, was diagnosed with discoid lupus erythematous which resulted in his facial scarring. Nick Cannon - rapper, actor, comedian, direct - suffered kidney damage and clots in his lung.
Imagine though the challenge in identifying this condition with just the five celebrities mentioned previously, and the diversity of their symptoms. Systemic Lupus Erythematosus is a chronic, recurrent, potentially fatal multi-system inflammatory disorder that is not only tough to diagnose, but also tough to manage. Having had nearly a decade's experience in high risk obstetrics, it became familiar to me when we'd have women present in our high risk obstetrical practices with a multitude of miscarriages that their previous primary care providers had overlooked since ultimately, these women did achieve pregnancy.
After running a battery of tests, we'd often discover an underlying cause, and one of those primary findings was in fact, Lupus. Complicating the matter even more, there is no single diagnostic marker for Lupus; rather, it is identified through a combination of clinical and laboratory criteria. Clearly, accurate diagnosis is important as treatment can reduce morbidity and mortality, particularly from lupus nephritis. Our goal of course, is optimal health, not simply absence of disease or dis-ease.
Estimates on Lupus prevalence is about 200 cases per every 100,000 women in England and the overall prevalence in the United States, to be about 40 to 50 cases per 100,000 persons. Like most all inflammatory diseases, Lupus is most common in women, particularly Women of Color. Lupus is diagnosed 23 times more often in black women than in white men. It does impact about 5,000 to 10,000 U.S. children. Familial predisposition to systemic lupus erythematous plays a role.
Often I hear, from clients and practitioners, that "she doesn't seem like someone with Lupus to me." When the dysfunction is multi-organ or multi-system the presentation can be quite diverse, not unlike thyroid disease which impacts the mitochondria in every cell of the body. Often the symptoms of Lupus go fairly ignored until significant dis-ease results.
Lupus is an autoimmune condition which like more than 80 other diseases is one that results from a breakdown in the immune system causing it to attack its own tissues. Lupus is a systemic autoimmune condition because unlike rheumatoid arthritis or inflammatory bowel disease, which affects one specific area of the body, Lupus can affect anything from your joints to your brain cells, heart and lungs to your kidneys and skin. This is the disease of kicking your own ass.
Symptoms can impact the skin, muscles, joints, cause fatigue, or really challenge diagnostic skills when the symptoms are a bit more covert, impacting your blood work, your kidneys, or heart. Neuropsychiatric symptoms are also symptoms which can go unrecognized as Lupus, and although significant, the literature doesn't often recognize the reproductive system as being impacted in Lupus.
Infections in the respiratory and urinary systems can be common in those with Lupus, as does coronary artery disease. Fever, rash, arthritis, new-onset Raynaud phenomenon, and alopecia (thanks to Jada Smith, not one of us is ignorant here) are potentials, as well as headache. Children can present with malar rash a bit more frequently, or #anemia, leukcytopenia, and have more severe neurologic and renal involvement. The real red flag though for Lupus, is painful or swollen joints, unexplained fevers, kidney problems, and extreme fatigue. Like many autoimmune diseases, lupus is characterized by flare-ups and periods of remission.
Organ damage progresses over time. One study found that in seven years, sixty-one percent of those studied had detectable organ damage, with neuropsychiatric (20.5 percent), musculoskeletal (18.5 percent), and renal 15.5 percent) organ systems being most commonly affected. Infections and diseases of the cardiovascular, renal, pulmonary, and central nervous systems are the most frequent causes of poor outcome.
Remission is not uncommon. Certainly I've seen it a number of times in my own practice. Since just the 1950s, the five-year survival rate with systemic lupus erythematosus has increased from 50 percent to a range of 91 to 97 percent. Is this because of better management or earlier diagnosis, we aren't quite sure.
We do know though that mortality is related to seizures, Lupus nephritis, and azotemia (elevated nitrogen) and that children diagnosed before the age of 15 years have a fairly ominous poor progression when hypertension or renal involvement is identified. My thought is, because individuals with Lupus are living longer, for whatever reason, let's focus care on really comprehensive wellness strategies so we can not simply prevent progression or flares of Lupus, but really optimize health for lifelong vitality. Again though, studies show us that often primary care physicians aren't paying attention for Lupus and functional providers may miss this for chronic fatigue or #Lyme or #fibromyalgia so this one goes undiagnosed or maybe early markers are low so Lupus is ruled out entirely, while wellness strategies fail to get integrated into very necessary lifestyle changes.
How do We Diagnose?
The key is finding a clinician who will listen to you. Miscarriages aren't uncommon. Fatigue in women is super common, even musculoskeletal pain or various rashes. Sensitivity to light, sores in your mouth, arthritis, pleural rub, seizures, or various lab findings which may be peculiar in and of themselves may be the primary indicators. An overall sense of unwell is a pretty big indicator that I would like to dig in a little bit more.
Diagnosis though is both clinical and laboratory findings. The American College of Rheumatology has provided al algorithm for diagnosis which is super helpful, but here's the key, leaning into Lupus doesn't mean we just wait and see. There is much to be done to optimize your health and wellness. Ultimately though, we start with antinuclear antibody testing (ANA) and a titer of 1:40 or higher is the most sensitive for diagnosis. While some can have an elevated count and not have Lupus, 99 percent of those who do, do have an elevated ANA above 1:40.
If this returns elevated, additional laboratory tests are done to narrow in on diagnosis as there may be other causes of an elevated ANA as well, such as scleroderma, Sjorgrens syndrome, rheumatoid arthritis, even fibromyalgia. If sufficient clinical symptoms along with an elevated ANA are present, this is sufficient for diagnosis. If clinical presentation is still somewhat ambiguous, then additional testing can be ordered, such as antibody for double-stranded DNA antigen, anti-RNP, anticardiolipins, antibody to Sm nuclear antigen, and more to help dial in diagnosis.
If autoantibodies for ANA is positive and signs and symptoms are present, then a Rheumatology referral certainly can be helpful.
What if I am already Diagnosed and Need Management?
Primary care providers can manage Lupus, depending on their interest. Some may refer entirely to Rheumatology or another PCP with more knowledge in this area. Functional medicine practitioners are great resources as well because we look for root cause and have a more strict criteria for dis-ease and wellness. Ultimately though, management comes down to the organ system involved. Unfortunately, there is no cure for Lupus. That said, it is possible due to the cyclical nature of Lupus, to achieve full remission of the disease.
Education is key. Partnering with your practitioner is vital. Steroids are the cornerstone of treatment which not all clients are uber comfortable with, alas integrative providers can really be resourceful in this respect. Skin rashes are quite common and kidney disease is present to some degree in most all individuals with Lupus, with about half developing clinical disease. Screening for nephritis is recommended every three to six months. There are a number of presentations so it can be hard to really discuss within a simple blog post all the various ways we may approach Lupus, but certainly, maintaining a healthy lifestyle is vital. Our clients are offered a very comprehensive wellness program that works through environmental health, epigenetics, somatic healing, self-study and implementing lifestyle behaviors that honor our individual needs.
Understanding the various types of Lupus is helpful for clients and creating plans for potential management, most especially understanding epigenetic tendencies to optimize detoxification in both phase one and phase two so that your body burden of toxins can be reduced as much as possible, minimizing the work your body must tackle, reducing overall inflammation.
Simple approaches are implementing healthy fats such as fatty fish, avocado, and olive oil (room temperature) as these work against inflammation can may help reduce that underlying cause. Of course, identifying these triggers would be one of our first recommendations. Meditate. Stress is related to Lupus flares so try at least fifteen minutes a day. Move out of overwhelm and the triage mode, and find yourself in a relaxed and repair state of being. Probiotics are key. Take vitamin D3.
Am I at Risk?
We really don't know the underlying cause of Lupus quite yet, but experts do believe there is some level of genetic, environmental, and even hormonal aspect to Lupus as more than 90 percent of those with the condition are women. You are more likely to have Lupus if you are African, Asian, or Native American descent. Contributors to the disease are also sunlight, stress, medications, infections, and alterations in the gut microbiome. A study published in 2019 for example, showed that women with Lupus had five times more gut bacteria, called Ruminococcus gnavus, than women of similar age and racial backgrounds who did not have the disease. According to authors, it is also possible that a lack of a gut bacteria called Bacteroids uniformis - which hinders the growth of R. gnavus - may also contribute to the development and severity of the disease.
Azzouz, D., Omarbekova, A., Heguy, A., et al. (2019). Lupus nephritis is linked to disease-activity associated expansions and immunity to a gut commensal. Annals of the Rheumatic Diseases, 78, 947-956.
Gill, J. M., Quisel, A. M., Rocca, P. V., & Walters, D. T. (2003). Diagnosis of systemic lupus erythematosus. American Family Physicians, 68, 2179-2186.
Lam, N-C, V., Ghetu, M. V., & Bieniek, M. L. (2016). Systemic lupus erythematosus: Primary care approach to diagnosis and management. American Family Physician.