12.1 Clinical immunology Flashcards
Describe the innate vs adaptive immune systems.
Innate: non-specific, general, immediate, no memory
- Humoural: pattern receptors, complement, enzymes, cytokines
- Cell mediated: phagocytes, NKcells
Adaptive: specific, lag time from exposure to response, memory
- Humoural: antibodies and cytokines
- Cellular: T cells and B cells
What are the primary and secondary organs of the immune system?
Primary:
- Bone marrow for production of all haematopoietic cells
- Thymus for T cell maturation
Secondary:
- MALT, tonsils, adenoids
- Lymph nodes and lymphatic system for tissue fluid surveillance
- Spleen for blood surveillance
What are the lymphoid organs?
Sites of generation, maturation and initiation of adaptive immune response.
- Central lymphoid organs: thymus and bone marrow
- Peripheral lymhpoid organs: lymph nodes, spleen, mucosal/gut-associated lymphoid tissue
Outline the functions of the immune system.
- Searches out and destroys pathogens
- Searches out and destroys cancerous self-cells
- Clears debris from sites of inflammation
- Promotes healing of wounds
All of this while not harming normal self-cells.
What is immunodeficiency?
Defects or deficiencies in the immune system (congenital or acquired).
What is autoimmunity?
Abnormal activation of the immune system.
Immune response directed at self-antigens due to breakdown of normal regulatory mechanisms.
Caused by failure of central tolerance: failure of deletion of self reactive T-cells and B-cells.
What is an autoimmune disease?
Damage or disturbed physiological function due to autoimmunity.
Women are more commonly affected than males.
How are autoimmune disease classified?
Organ specific:
- Thyroid disorders e.g. Grave’s
- Tybe 1 diabetes
- Autoimmune haemolytic anaemia
Non-organ specific:
- Systemic lupus erythematosus
- Connective tissue diseases (e.g. rheumatoid)
What are the risk factors and triggers of autoimmune disease?
Genetics:
- Multigeneic
- Complement classical pathway deficiency
Environment:
- Hormones: oestrogen
- Infection
- Drugs e.g. penicillamine
- Others: stress, diet, UV
How are autoimmune diseases treated?
- Endocrine: replace deficient hormone/remove oversecreting gland
- Immunosuppression: steroids, cyclosporin, methotrexate, azathioprine
- Newer treatments: monoclonal antibodies e.g. anti-B cell, anti-TNF-alpha
What autoimmune condition may present with diffuse parotid gland enlargement?
Sjogren’s syndrome
Describe the main features of Sjogren’s.
- Autoimmune disorder affecting the exocrine glands (esp salivary and lacrimal glands)
- Complaints of dry mouth and dry eyes
- Affects females more than males (9:1)
- Clinical diagnosis from history and signs
- Schirmer’s test: tear production
- Lab diagnosis: biopsy; positive ANA,
anti-Ro and anti-La antibodies
What are the oral features of Sjogren’s, how can these be managed?
Zerostomia can lead to:
- Dental caries
- Gum and tongue atrophy
- Difficulty swallowing
- Associated with other autoimmune diseases (RA, SLE)
Management:
- Mouth washes and good dental care
- Salivary stimulants
- Dental check-ups
- Artificial tears
What are autoinflammatory disorders?
Lack of control of inflammation.
Not autoimmune, as there is not an immune response targeted at self antigens in autoinflammatory disorders.
Give an example of an autoinflammatory disorder.
Periodic fever with apthous stomatitis, pharyngitis and cervical adenitis (PFAPA).
Hereditary.