In my family, we have turned the word Lupus (Systemic Lupus Erythematosus)
into an adjective known as 'luped.’ Why? Because for us, it is a brief word
that does justice to fully encompass describing my mothers’ behavior, mood,
memory, physical state, ease of movement and lack of appetite during a flare-up.
It’s a play on words because, she truly acts loopy during a flare-up… I mean looooopy
What’s a flare-up you ask? Well, because lupus is an autoimmune
disease, her body is essentially attacking itself intermittently. During a
flare-up, her immune system is revved up and wrongfully recognizes her good
cells as bad cells and attacks the healthy cells. This results in degradation of
joints, bones, problems with organs – particularly heart and central nervous
system. To try to put perspective on how she feels during a flare-up, you can
think of having a really bad throw-up flu: achy all over, extremely nauseas/throwing
up, exhausted and slightly delirious.
Although she wasn’t diagnosed until she was 29, my mother has told
me she first noticed signs of Lupus when she was 15 following her contracting mononucleosis
(mono). Interestingly, Systemic Lupus and the Epstein-Barr (mono) interact in
such a way that the Epstein-Barr triggers Lupus to manifest. In other words, some
people can go his/her entire life without getting Lupus even if their genes are
set up for it. Lupus often requires an environmental trigger in combination
with genetic predispositions in order to develop the disease (James, J &
Robertson, J, 2013).
I know most of us ‘get loopy’ with age, but my mom has gotten loopy
with the severity of her disease - as in her cognitive abilities, especially her
memory have drastically declined over the years. I guess this observation is
common - Larissa Lapteva and coauthors performed a study examining the cognitive
function of those with systemic lupus and found that there is indeed a significant
decline in memory and cognitive functioning in those with the disease versus without
(Lapteva, L et al., 2006).
Despite my recent realization of my mother’s decline from her
disease, the woman is a relentless spitfire that has the most positive outlook
on life I’ve encountered.
Carbotte, R. M., Denburg, S.
D., & Denburg, J. A. (1986). Prevalence of cognitive impairment in systemic
lupus erythematosus. Journal of Nervous
and Mental Disease, 174(6), 357-364.
James, J, Robertson, J. (2012). Lupus and Epstein-Barr. Curr
Opin Rheumatol, 4(24), 383–388. https://doi.org/doi: 10.1097/BOR.0b013e3283535801
Lapteva, L., et al. (2006). Anti–N‐methyl‐D‐aspartate
receptor antibodies, cognitive dysfunction, and depression in systemic lupus
erythematosus. Arthritis & Rheumatology, 54(8), 2505–2514.
I thought it was so interesting that her Lupus was triggered from mononucleosis at the age of 15 – I didn’t realize that Lupus often requires an environmental trigger and that other viruses interact with Lupus to trigger it. This made me curious as to what other viruses are known to trigger Lupus.
ReplyDeleteI found that human cytomegalovirus (Neo et al., 2019) and zoster virus (the virus that causes shingles) have been known to trigger Lupus as well due to molecular mimicry (Hirbod, Altamura, Arkoumani, Stefanato, & Verdolini, 2017). I also discovered that women are 9 times more likely to develop Lupus because higher estrogen levels result in an enhanced immune response (Johns Hopkins Lupus Center, 2019). This must also be why other autoimmune diseases such as rheumatoid arthritis are seen more commonly in women.
Cited Literature
Hirbod, T., Altamura, D., Arkoumani, E., Stefanato, C. M., & Verdolini, R. (2017). Discoid lupus erythematosus occurring in an area previously affected by herpes zoster virus: Wolf's isotopic reaction? Eur J Dermatol, 27(2), 186-187. doi:10.1684/ejd.2016.2940
Johns Hopkins Lupus Center. (2019). Causes of lupus. Retrieved from https://www.hopkinslupus.org/lupus-info/lupus/
Neo, J. Y. J., Wee, S. Y. K., Bonne, I., Tay, S. H., Raida, M., Jovanovic, V., . . . MacAry, P. A. (2019). Characterisation of a human antibody that potentially links cytomegalovirus infection with systemic lupus erythematosus. Sci Rep, 9(1), 9998. doi:10.1038/s41598-019-46329-y
Alex,
DeleteThanks for your response! That is really interesting about which specific viruses can trigger it. Your response prompted me to do some more research on the topic.
Although the prevalence of Lupus is much greater for women than men, men have a more severe presentation of the disease and higher rate of mortality, the reason behind this is unknown (Gullermo, J, Pons-Estel, G, Ugarte-Gil, M, & Alarcon, G, 2017). Child onset Lupus (very rare) is also associated with a more severe presentation of the disease (Gullermo, J, Pons-Estel, G, Ugarte-Gil, M, & Alarcon, G, 2017). Lupus is about 2-4 times more prevalent (and more severe) in African-American, Hispanic/Latino, Asians, and Native American populations than Caucasian populations (Somers, E et al., 2014). There are theories behind this finding but the definitive reason is unknown.
Lupus can also be 'triggered' by certain drugs, such as hydralazine (treats hypertension) and procainamide (treats heart arrhythmia) through the mechanism of DNA demethylation (certain genes get 'turned off' by the medication) (Jefferies, M & Sawalha, A, 2011). People with SLE have a harder time clearing dead cells from their immune systems, which is a positive feedback mechanism for further demethylation of DNA (Jefferies, M & Sawalha, A, 2011).
Just some more fun facts on Lupus for you all!
Guillermo J. Pons-Estel, Manuel F Ugarte-Gil & Graciela S. Alarcón (2017) Epidemiology of systemic lupus erythematosus, Expert Review of Clinical Immunology, 13:8, 799-814, DOI: 10.1080/1744666X.2017.1327352
Jeffries MA, Sawalha AH. Epigenetics in systemic lupus erythematosus: leading the way for specific therapeutic agents. Int J Clin Rheumtol. (2011) 6:423–39. 10.2217/ijr.11.32
Somers, E. C., Marder, W., Cagnoli, P., Lewis, E. E., DeGuire, P., Gordon, C., Helmick, C., Wang, L., Wing, J., Dhar, P., Leisen, J, Shaltis, D, & McCune, W. J. (2014). Population-based incidence and prevalence of systemic lupus erythematosus: the Michigan Lupus Epidemiology and Surveillance program. Arthritis & rheumatology (Hoboken, N.J.), 66(2), 369–378. doi:10.1002/art.38238
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ReplyDeleteHannah, first I am very sorry that your family struggles with this disease. However, you are not alone! Like you, my family is plagued by generations of lupus. My grandmother died from the disease and my aunts all suffer. I too have become very interested in this disease, constantly seeking out the newest and coolest research relating to the pathology, symptomology and overall outcome of this disease, because lupus is a very confusing disease and has a very strong heritability and famililial aggregation (Càrdenas Roldàn et al, 2013). One of my coolest finds, that you might be interested in as well, is the relationship between systemic autoimmune diseases such as system lupus erythematosus and the microbiome.
ReplyDeleteIn a study done by Hevia, Milani, Lopez et al, called "Intestinal Dysbiosis Associated with Systemic Lupus Erythenatosus" they compared the microbiome of individuals with SLE and those without in a well-defined geographical location with similar lifestyles. The researchers found a significant decrease in the microbacteria: firmicutes/bacteroidetes ratio in SLE individuals. Following this finding, the researchers mentioned that an increase in the ratio of firmicutes/bacteridetes was seen in individuals following a diet high in fiber and low in fat. This made me think that some microbiomes could be altered using diets. I don't know if that would lessen symptoms for those affected but it certainly would be an interesting place to start. This study also mentioned the possibility that different levels of different bacteria's somewhat aligned with differing symptoms of individuals with SLE. I would be incredibly interested for this to be studied further. Perhaps this could lead to treatment options that are non-invasive and relatively simple for those who suffer.
Citations:
Cárdenas-Roldán, J., Rojas-Villarraga, A., & Anaya, J.-M. (2013). How do autoimmune diseases cluster in families? A systematic review and meta-analysis. BMC Medicine, 11(1). doi: 10.1186/1741-7015-11-73
López, P., Sánchez, B., Margolles, A., & Suárez, A. (2016). Intestinal dysbiosis in systemic lupus erythematosus. Current Opinion in Rheumatology, 28(5), 515–522. doi: 10.1097/bor.0000000000000309