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Immune Dysfunction

Type 1 Diabetes

Type 1 diabetes (T1D) is a chronic T-cell mediated disease that leads to the destruction of the insulin-secreting islet β-cells (Figure 1) resulting in absolute insulin deficiency and hyperglycaemia.

Systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is a severe, relapsing, remitting multisystem autoimmune disease. The name systemic lupus implies that almost any organ or system within the body might be affected and lupus is perhaps the classical multi-symptom illness. Onset can occur at any age however it most typically presents in young adult females at a female to male ratio of 9:1.

Multiple Sclerosis

Multiple sclerosis (MS) is a human disease that affects the nervous system. The most common pattern of disease is intermittent attacks affecting sight, mobility or sensation followed by gradual recovery. Over time the attacks can lead to permanent disability. The most widely accepted explanation for MS is that the immune system orchestrates repeated episodes of inflammation within the brain, because its normal ability to distinguish healthy tissue from infected tissue has failed.

Juvenile Idiopathic Arthritis

Juvenile idiopathic arthritis (JIA) is chronic swelling in the joints of children and affects 1 in 1000 children in the UK. JIA is defined as joint inflammation commencing before the age of 16, of duration greater than 6 weeks and of unknown origin. Complex genetic and environmental influences contribute to JIA onset. A number of subtypes of JIA exist based on the ILAR (International League of Associations for Rheumatology) criteria. It should be noted that not all these childhood subtypes occur in adult inflammatory arthritis.

Inflammatory Bowel Disease

Inflammatory bowel disease (IBD), comprising Crohns disease (CD) and ulcerative colitis (UC), is a chronic, relapsing-remitting inflammatory disorder of the gastrointestinal (GI) tract. CD and UC vary in the region of the GI tract they affect; the extent of inflammation and resulting tissue damage; and their associated symptoms.

Immunodeficiency Antibody

There are several mechanisms by which a person may fail to produce adequate levels of antibodies (Ab), resulting in a clinical antibody deficiency and an inability to eliminate microbial pathogens effectively from the body. Without early recognition and adequate replacement of Ab, structural damage to organs may occur due to recurrent infections. Fortunately, these conditions are rare – however this means that it can be difficult to diagnose them quickly.

Causes

Ab deficiencies may be primary, or secondary to other conditions.

Hereditary angioedema

Hereditary angioedema (HAE) affects approximately 1 in 50,000 of the population and does not show ethnic variation in frequency. HAE is inherited in an autosomal dominant manner and results in unpredictable episodic swellings which can affect the face, peripheries, genitals, abdomen and airway. Laryngeal swellings can result in death. As with many rare conditions, there is unfortunately often a delay to diagnosis during which time patients do not receive appropriate treatment.

Exosomes in cancer immunology

Exosomes are nanometer sized vesicles, typically 30-100nm in diameter. They are manufactured within the endosomal system, as multivesicular bodies (MVB), which are ultimately secreted into the extracellular space. They exhibit a typical bi-layer lipid membrane, which is high in cholesterol, ceramide and has phosphatidyl serine (PS) residues evenly distributed between outer and inner membranes; as such they may appear to the immune system as apoptosis-related particles.

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