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Lupus Risk Factors

Lupus is an autoimmune disease, which means that the immune system attacks the body’s own cells and tissues by mistake. So far, no cure has been discovered to combat lupus.

There are different types of lupus with very different characteristics:

Systemic Lupus Erythematosus

It is the most common type detected and can be severe or mild. The immune system attacks the cells and tissues of various parts of the body such as the joints, skin, kidneys, blood cells, brain, heart, and lungs. Symptoms vary but can include fatigue, joint pain, erythema, and fever. “Systemic lupus erythematosus (SLE) is a heterogeneous disease characterized by wide variations of clinical onset and phenotypes with disease in multiple organ systems, frequently affecting young adults at the peak of their economic productivity. Although improvements in the diagnosis and treatment of SLE have had a remarkable impact on survival and life expectancy,4,5 patients can still experience frequent disease flares and develop severe organ involvement and, consequently, incur substantial costs. We posit that by understanding published demographic, clinical, biomarker, genetic and environmental features associated with the onset of SLE, an approach may be developed to case finding of early or incomplete lupus erythematosus (ILE) followed by the implementation of evidence-based strategies to avert, delay or ameliorate pathogenic disease processes. The anticipated outcomes of this approach are to achieve an early diagnosis, to initiate appropriate, evidence-based efficacious interventions to improve outcomes and quality of life and decrease health care costs.”1

“Systemic lupus erythematosus is a potentially severe disease. Since it is a type of vasculitis of the medium-sized and small vessels, all organs are subject to damage, including those whose function is vital for survival, such as heart, lungs, kidneys, and central nervous system. Nevertheless, with new armamentarium for therapy and making early diagnosis, the acute flares of the disease can be better controlled; mortality due to vasculitis has diminished and the disease took on a more chronic nature.”2

“The etiology of SLE is unknown but multiple genetic, epigenetic, and environmental risk factors have been implicated. The inheritance of genes alone is not sufficient for developing SLE, suggesting the influence of environmental triggers on disease expression.

It is known that SLE develops through multiple steps with the loss of self-tolerance and development of autoantibodies occurring sometimes several years prior to the onset of clinically symptomatic autoimmune disease. Although first degree relatives of patients with SLE overall have a higher prevalence of autoantibodies and a higher risk of SLE and other autoimmune diseases, some develop SLE-specific autoantibodies but never develop clinical disease, implying that there are protective factors as well. The multifactorial nature of the genetic risk of SLE and the low disease penetrance emphasize the potential influence and complexity of environmental factors and gene-environment interactions on the etiology of SLE.”3

Discoid Lupus

This type has a clear target, the skin. It causes very annoying skin rashes that appear with itching and stinging.

“Discoid LE (DLE) is the most common variant of CLE. It typically (but not exclusively) evolves at light-exposed skin: face, ears, extensor aspects of the forearms, scalp, trunk, and, more rarely, the oral mucosa. Lesions are single or sparse in most cases; if numerous disseminated lesions are present, they are haphazardly distributed at the predilection sites without striking symmetry. Among multiple lesions, many are found in the butterfly area (see later herein), and some tend to appear in unusual places, for example, the “niches” of the external ear, the eyelids, the lips, or the vestible of the nose; there is a tendency not to transgress natural border lines, for example, the vermilion border of the lip or the areola mammae. Lesions evolve according to a characteristic time course. Fresh lesions first present as small, round, well-defined, slightly raised erythemas with dull surfaces that soon become rough to the touch and scaly. Scales are adherent and are often attached to the hair follicles (“carpet tack” phenomenon). Follicular orifices are first widened with keratotic plugs and may then disappear completely; there is a gradual loss of hair in the lesions, leading to irreversible scarring alopecia. Lesions spread slowly and regress at the centers, which become smooth and sunken. Intermediate lesions become elevated and indurated at variable degrees and develop atrophy and loss of normal skin texture in their centers. At the periphery, rests of the active lesion remain as ring-like, arcuate, or polycyclic scaly erythemas that continue to spread. Old (burnt-out) lesions may be disfiguring: they are large, with irregular borders, sharply demarcated, depigmented (porcelain white in dark skin), hairless, flat, thin, and with a scarring appearance. Pitted scars and crateriform indentations may occur. In acral location (e. g., nose and ears), there may be a loss of tissue (mutilation). It is important to note that the lesions differ in their individual ages; fresh lesions will thus be seen alongside intermediate and burnt-out ones. Activity of lesions may spontaneously cease at all stages; fresh lesions may heal with restitutio ad integrum, older ones result in atrophy.”4

Subacute Cutaneous Lupus

Like discoid lupus, it also affects the skin, but only if it is exposed to the sun. The blisters that come out are very painful. “Subacute cutaneous lupus erythematosus (SCLE) lies clinically and histologically between the more aggressive form of DLE with a cicatricial tendency, and the short-lived non-destructive malar erythema of acute lupus erythematosus (ALE). Epidemiologic data suggest that environmental factors may be responsible for some cases of SLE, SCLE and lupus-like syndrome. Among exogenous agents with a presumed role in triggering SLE and SCLE one may cite: ultraviolet light, pesticides and insecticides, heavy metals and other elements, tobacco, foodstuffs, medications (hydrochlorothiazide, anti-histamines, calcium channel blockers, naproxen, oral contraceptives, estrogens) and infections. Reports showing the appearance of SCLE or exacerbation of systemic lupus in patients using terbinafine have drawn attention to the possibility of this drug helping trigger or perpetuate the clinical picture thematous macules or papules. Papules and scabby plaques, in addition to petechiae, mimicking Langerhans’ cell histiocytosis have been reported in a four-week-old newborn that also presented hepatosplenomegaly and thrombocytopenia. The cutaneous lesions regress spontaneously, in most cases by 12 months of age, when maternal antibodies transmitted to the child transplacentally have been metabolized. On regression the cutaneous lesions do not present scarring, but telangiectasias may sometimes persist for several years. Complete heart block is present in approximately 50% of affected newborns, death from heart failure occurring in 10% of newborns”5

Drug- Induced Lupus

Certain drugs can cause our immune system to turn on us. As soon as we stop taking them, in principle, the problem should disappear. Some symptoms are muscle pain and joint pain.

“Drug-induced lupus erythematosus (DILE) is defined as an entity characterized by clinical manifestations and immunopathological serum findings similar to those of idiopathic lupus but which is temporally related to continuous drug exposure and resolves after discontinuation of the offending drug. Similar to idiopathic lupus, DILE can be divided into systemic lupus erythematosus (SLE), subacute cutaneous lupus erythematosus (SCLE) and chronic cutaneous lupus erythematosus (CCLE). Based on the literature review and retrospective analysis of our case series, we focused on the dermatological aspects of DILE. The cutaneous features of drug-induced SLE are protean, including particularly purpura, erythema nodosum and photosensitivity as well as the skin lesions characterizing both urticarial and necrotizing vasculitis.”6

“Drug-induced lupus is a reversible lupus-like condition due to exposure to over 80 prescribed drugs to date. Its symptomatology is generally mild to moderate with resolution of both clinical and serologic features over time following drug cessation. The overall prognosis remains favorable although occasional life-threatening cases have been reported in the literature. In these severe cases, transient therapy with corticosteroids may be required. Hence, constant pharmacovigilance for prompt diagnosis as well as cessation of offending therapy offers the best outcome.”7

Neonatal Lupus

It is the least common and occurs only in newborns. Its appearance may be linked to certain antibodies of the mother.

“Neonatal lupus erythematosus (NLE) is another auto-immune disease related to SLE. Unlike SLE it is not a spontaneous syndrome but rather an acquired one. In NLE the most common disease manifestations are a transient cutaneous lesion and cardiac conduction disturbances. The cutaneous lesions and other non-cardiac manifestations of NLE are transient and disappear about six months after birth, at the time when maternal antibodies disappear from the neonatal circulation. This fact suggests that maternal antibodies may cross the placenta leading to an inflammatory reaction in the fetal tissues. The factors determining whether babies will develop heart block, skin disease, or both, are not yet known.

NLE is the principal cause of total atria-ventricular block, when it is not associated with congenital birth defects. Some children may develop a partial or total atria-ventricular block during fetal life or in the neonatal period. In general this manifestation is irreversible, and may be lead to death.”8

 

Types of Cutaneous Lupus Erythematosus
Fig. 1. Types of Cutaneous Lupus Erythematosus. Reprinted from “Cutaneous lupus erythematosus.” JAMA dermatology, 150(3), 344-344. By Eastham, A. B., & Vleugels, R. A. (2014). https://jamanetwork.com/journals/jamadermatology/fullarticle/1843885

 

Epidemiology

“Prevalence rates in lupus are estimated to be as high as 51 per 100000 people in the USA. The incidence of lupus has nearly tripled in the last 40 years, mainly due to improved diagnosis of mild disease. Estimated incidence rates in North America, South America, and Europe range from 2 to 8 per 100000 per year. Women are affected nine times more frequently than men and African American and Latin American mestizos are affected much more frequently than Caucasians, and have higher disease morbidity. The disease appears to be more common in urban than rural areas. Sixty-five percent of patients with SLE have disease onset between the ages of 16 and 55 years, 20% present before age 16, and 15% after the age of 55. Men with lupus tend to have less photosensitivity, more serositis, an older age at diagnosis, and a higher 1 year mortality compared to women. SLE tends to be milder in the elderly with lower incidence of malar rash, photosensitivity, purpura, alopecia, Raynaud’s phenomenon, renal and central nervous system involvement, but greater prevalence of serositis, pulmonary involvement, sicca symptoms, and musculoskeletal manifestations.”9

Some of the main risk factors for lupus are

Gender

One of the risk factors of lupus is to be female. Women are more likely to develop lupus, the main reason being the action of female hormones. The amount of estrogen, much higher in women than in men, affects the immune system by potentiating lupus. For this reason, women who develop the disease should not take contraceptives, because birth control pills have a large amount of estrogen that could aggravate it.

Age

Age is another of the risk factors for lupus. Between 15 and 45 years old, women are at their most fertile. The reason is, again, high levels of estrogen. Estrogens intervene in the fertility of women, protecting the body and preparing it for possible pregnancy. The first symptoms of lupus are usually detected before the age of 18.

Race

Anyone can suffer from lupus, regardless of race. However, it is more common among African-American, Hispanic-Latin, Asian or Native American people. When lupus develops in people of the above-mentioned races, it usually appears at an early age and its severity is greater.

Family background

The last of the risk factors for lupus is the family history. As with many other diseases, the fact that someone in the family has had it significantly increases the chances of getting it. A curiosity about this condition is that it is not usual to be inherited directly from mothers to children.

Since lupus has no cure, treatment must be aimed at preventing future outbreaks, treating the symptoms and reducing the damage that the disease may cause. Depending on the type of lupus we suffer, systemic symptoms like arthritis, anemia, kidney problems, depression, ulcers, cholesterol and others may appear. To try to reduce these symptoms and problems, specific medications containing hydroxychloroquine and chloroquine may be used. This way, you can reduce inflammation and pain, prevent future outbreaks, minimize and prevent joint damage and balance out hormones that were out of whack.

Lupus disease usually causes a lot of stress, anxiety and sadness because it will accompany us forever. This causes hopelessness and frustration. However, if you get the right treatment it can be kept under control, allowing you to enjoy a normal and happy life.

 

References:

(1) Choi, M. Y., Barber, M. R. W., Barber, C. E. H., Clarke, A. E., & Fritzler, M. J. (2016). Preventing the development of SLE: identifying risk factors and proposing pathways for clinical care. Lupus, 25(8), 838-849. Available online at https://journals.sagepub.com/doi/pdf/10.1177/0961203316640367

(2) Skare, T. L., Dagostini, J. S., Zanardi, P. I., & Nisihara, R. M. (2016). Infections and systemic lupus erythematosus. Einstein (São Paulo), 14(1), 47-51. Available online at http://www.scielo.br/pdf/eins/v14n1/1679-4508-eins-14-1-0047.pdf

(3) Kamen, D. L. (2014). Environmental influences on systemic lupus erythematosus expression. Rheumatic Disease Clinics, 40(3), 401-412. Available online at https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4198387/

(4) Weber, F., & Fritsch, P. (2005). Clinical differential diagnosis of cutaneous lupus erythematosus. In Cutaneous Lupus Erythematosus (pp. 147-160). Springer, Berlin, Heidelberg. Available online at http://eknygos.lsmuni.lt/springer/109/147-160.pdf

(5) Berbert, A. L. C. V., & Mantese, S. A. D. O. (2005). Cutaneous lupus erythematosus: clinical and laboratory aspects. Anais Brasileiros de Dermatologia, 80(2), 119-131. Available online at http://www.scielo.br/pdf/abd/v80n2/en_a02v80n02.pdf

(6) Marzano, A. V., Vezzoli, P., & Crosti, C. (2009). Drug-induced lupus: an update on its dermatologic aspects. Lupus, 18(11), 935-940. Available online at https://journals.sagepub.com/doi/10.1177/0961203309106176

(7) Vasoo, S. (2006). Drug-induced lupus: an update. Lupus, 15(11), 757-761. Available online at https://pdfs.semanticscholar.org/4302/9ed9405d7e229968a70aff12bd563c369fcf.pdf

(8) Garcia, S., & Campos-de-Carvalho, A. C. (2000). Neonatal lupus syndrome: the heart as a target of the immune system. Anais da Academia Brasileira de Ciencias, 72(1), 83-90. aVAILABLE ONLINE AT http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0001-37652000000100012

(9) Bertsias, G., Cervera, R., & Boumpas, D. T. (2012). Systemic lupus erythematosus: pathogenesis and clinical features. EULAR textbook on rheumatic diseases, Geneva, Switzerland: European League Against Rheumatism, 2012, 476-505. Available online at https://www.eular.org/myuploaddata/files/sample%20chapter20_mod%2017.pdf

 

Robert Velasquez
14 December, 2018

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Hello everyone, my name is Robert Velazquez. I am a content marketer currently focused on the medical supply industry. I studied Medicine for 5 years. I have interacted with many patients and learned a lot...read more:

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