Case Study – Systemic Lupus Erythematosus and Immune Dysfunction

Case Study – Systemic Lupus Erythematosus and Immune Dysfunction

Jenny is a 28-year-old graphic designer who is presenting symptoms of systemic lupus erythematosus (SLE), which is an autoimmune condition. It can cause such symptoms as fatigue, joint pain, and a persistent rash. The goal of this case study is to identify those immunological mechanisms at play and to design a multifaceted approach to management.

Positive and Negative Selection

T lymphocytes that will be mature and capable of producing self-tolerance go through a process called positive and negative selection in the thymus. Positive selection enables T cells to bind to self-MHC molecules, while negative selection disposes of T cells that give a strong reaction to self-Ag. This two-step procedure avoids the onset of autoimmune reactions because only non-reactive T cells are allowed into circulation (Justiz Vaillant et al., 2023).

Cardinal Signs of Inflammation

The four cardinal signs of inflammation are:

1. Redness (rubor): Contrary to the cases with sulfatide salts, its occurrence is attributed to vasodilation and, consequently, enhanced blood circulation.
2. Heat (calor): Arising as a consequence of elevated blood flow rate and metabolic rate.
3. Swelling (tumor): Because of the collection of fluid and immune modulators, more immune cells are found.
4. Pain (dolor): A result of the liberation of inflammatory mediators and the pressure on nerves.

Types of T Lymphocytes

1. Helper T cells (CD4+): Recruit other immune cells into the area of inflammation.
2. Cytotoxic T cells (CD8+): Spread the virus and kill infected cells.
3. Regulatory T cells (Tregs): Reduce immunity to avoid alterations of immunity tolerance.
4. Memory T cells: Give long-term protection to the subjects against the diseases the vaccine was administered for.

Cell-Mediated vs. Humoral Immunity

In cell-mediated immunity, the T cells themselves attack and kill the infected cells, while in humoral immunity, B cells release antibodies to counteract the pathogen. These two arms of the immune system integrate to ensure the body is well protected against disease-causing pathogens (Henderson, 2022).

Role of MHC Proteins in Autoimmune Diseases

MHC proteins present self-antigens to T cells, helping the immune system distinguish between self and non-self. As suggested by Pan et al. (2019), in autoimmune diseases like SLE, dysregulation of MHC presentation leads to inappropriate immune responses against self-tissues.

References

Henderson, E. (2022, January 15). Humoral vs cell-mediated immunity. News-Medical.net. https://www.news-medical.net/health/Humoral-vs-Cell-mediated-Immunity.aspx

Justiz Vaillant, A. A., Goyal, A., Bansal, P., & Varacallo, M. (2023). Systemic lupus erythematosus. PubMed; StatPearls Publishing. https://www.ncbi.nlm.nih.gov/books/NBK535405/

Pan, L., Lu, M.-P., Wang, J.-H., Xu, M., & Yang, S.-R. (2019). Immunological pathogenesis and treatment of systemic lupus erythematosus. World Journal of Pediatrics, 16(1), 19–30. https://doi.org/10.1007/s12519-019-00229-3

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Cellular immunity involves the action of T lymphocytes that will directly attack and destroy abnormal host cells. During these immune responses, the body will get rid of pathogens that reside inside human cells as they are inaccessible to antibodies.

Systemic Lupus Erythematosus and Immune Dysfunction

Like all other formed elements, T cells are produced in red bone marrow by the hematopoietic stem cells. Once produced, these new cells will enter the bloodstream and travel to the thymus. Here, these lymphocytes will mature into fully functional T cells. T lymphocytes must pass two tests before they are permitted to leave the thymus:​ positive selection and negative selection. These tests ensure self-tolerance or restraint from attacking one’s own tissues. Failure of self-tolerance can result in autoimmune reactions or disorders, where the immune system will produce antibodies that will attack the body’s own tissues. ​

Systemic lupus erythematosus (SLE), commonly referred to as lupus, is a chronic autoimmune disease characterized by inflammation and tissue damage in multiple organs and tissues throughout the body. SLE can affect various systems, including the skin, joints, kidneys, heart, lungs, and central nervous system. The exact cause of lupus is not fully understood, but it is believed to involve a combination of genetic predisposition, environmental triggers, hormonal influences, and dysregulation of the immune system.

Client Background:
Jenny, a 28-year-old graphic designer, arrives at the immunology clinic with a weariness that belies her age. Once a vibrant and energetic woman, Jenny now feels overwhelmed by a myriad of symptoms that have plagued her for months. Her days are filled with unrelenting fatigue, joint pain that makes even simple tasks a challenge, and a persistent rash that mars her once-clear complexion. The rash across Jenny’s cheeks and nose has become a source of embarrassment and discomfort. No amount of makeup can conceal the angry red patches that seem to flare up unpredictably.

Jenny’s journey into the world of autoimmune disorders began gradually, with symptoms that initially seemed insignificant. She brushed off her fatigue as the consequence of long work hours and dismissed her joint pain as the natural result of stress and overexertion. However, as time passed, Jenny’s symptoms intensified, gradually eroding her quality of life, and leaving her feeling helpless and defeated.

Desired Outcomes:

1. Identify the underlying cause of Jenny’s recurrent infections and immune dysfunction.
2. Develop a comprehensive treatment plan to boost Jenny’s immune function and reduce her susceptibility to infections.
3. Educate Jenny about lifestyle modifications and preventive measures to support her immune health and overall well-being.

Findings:
Upon examination, Jenny exhibits signs of inflammation, including swollen and tender joints, along with a rash that appears to be consistent with a photosensitive skin reaction. Laboratory tests reveal elevated levels of antinuclear antibodies (ANA) and anti-dsDNA antibodies, suggestive of systemic lupus erythematosus (SLE), an autoimmune disorder characterized by widespread inflammation and organ involvement.

1. Describe the processes of positive and negative selection, and how they contribute to self-tolerance.
2. What are the four cardinal signs of inflammation, and what are their causes?
3. List the different types of T lymphocytes and describe their functions.
4. Differentiate between cell-mediated immunity and humoral-mediated immunity.
5. What is the role of major histocompatibility complex (MHC) proteins in autoimmune diseases?
   1. MHC proteins prevent autoimmune reactions by suppressing the immune system.
   2. MHC proteins present self-antigens to immune cells.
   3. MHC proteins regulate the production of antibodies against foreign pathogens, preventing autoimmune responses.
   4. MHC proteins promote the differentiation of regulatory T cells, which suppress autoimmune reactions.