Additional Respiratory Notes Flashcards
primary immunodeficiency disorder
Serious
Persistent
Unusual
Recurrent
primary antibody disorders
e.g. sinusitis, ottitis media selective IgA deficiency common variable immunodeficiencies specific antibody deficiency X-linked agammaglobulinaemia
complement system disorders
e.g. laryngeal angiodema
hereditary angiodema
PID manifestations
e.g. pneumonia primary antibody deficiency complement system disorders congenital phagocytosis deficiency combined immunodeficiencies
problems in the innate immune system
- defects in neutrophil development
- defects in neutrophil trans-endothelual migration
- defects in neutrophil killing
- defects in macrophage killing
defects in neutrophil development
e.g. severe congenital neutropenia - Kostmann Syndrome
genetic (autosomal recessive)
severe lack of neutrophils
accumulation of precursor cells in the bone marrow
defects in neutrophil trans-endothelual migration
e.g. leukocyte adhesion deficiency
genetic
failure of neutrophil adhesion and migration
defects in neutrophil killing
e.g. chronic granulomatous disease
deep bacterial infections
granuloma formations
defects in macrophage killing
mycobacteria inside immune cells - they hide
treatment of phagocyte deficiencies
immunoglobin replacement therapy
aggressive management of infections
definitive therapy such as haematopoietic stem cell transplantation, gamma interferon therapy, gene therapy
problems with the adaptive immune system
- defects in leukocyte development (defect in haematopoietic stem cells or SCID)
- defects in thromocyte development
- defects in B cell development
- defects in leukocyte effector functions
- defects in T cell effector functions
- defects in B cell efector functions
- defects in class-switch recombinations
defect in haematopoietic stem cells
e.g. reticular dysgenesis
failure to produce all lymphocytes
fatal unless stem cell transplant
Sever combined immunodeficiency
failure of production of lymphocytes
B (high conc) and T (low conc) cells recrippled due to a genetic defect
persistently unwell from birth
presence of different lymphocyte subsets
what is th most common form of SCID
X-linked mutation in the IL-2Ryc gene inability to respond to cytokines decreased T cells and increased B cells poorly developed lymphoid tissues and thymus
how is SCID treated
prophilactically
definitively with a transplant
defects in thromocyte development
e.g. DiGeorge Syndrome
failure to produce CD4+ and CD8+ T cells
features - lower set folded ears, hypocalcaemia, complex congenital heart disease
management - correct metabolic and cardiac abnormalities, prophylactic antibiotics, Ig replacement, aggressive treatment
defects in B cell development
e.g. X linked agammaglobulinaemia
failure to produce mature B cells
due to tyrosine kinase defect
presents with URTI and LRTI
defects in T cell effector functions
cytokine production
defects in class-switch recombinations
e.g. selective IgA deficiency
features of T cell deficiencies
recurrent infection
opportunistic infections
maligancy at young age
autoimmune disease
features of B cell deficiencies
recurrent infection
opportunistic infection
antibody-mediated autoimmune disease
Type I hypersensitivity
Immediate e.g. allergy
IgE mediated
overstimulation of cells by IgE
degranulation response
Type II hypersensitivity
Direct cell effects e.g. cell surface antigen (IgM or IgG)
Goodpastures syndrome - affects lungs and kidneys in smokers, drug takers and infections
treatment is corticosteriods, cyclophosphamide, plasmapheresis and stop smoking
Graves disease (kirit)
Type III hypersensitivity
immune complex mediates e.g. antibody soluble antigens
result in tissue damage
SLE
acute hypersensitivity pneumonitis (chronic is not type III)
Type IV hypersensitivity
T cell mediated
sarcoidosis
TB
chronic hypersensitivity pneumonitis
what is IPEX syndrome
monogenetic disorder of immune dysregulation
X linked
what can some HLA genes do
predispose someone to autoimmunity
asthma is characterised by what lyphocyte activation
TH2 –> CD4+ express to TH0 cells that mature to the TH2 and activate B cells that mature to IgE secreting P cells
COPD is characterised by what lymphocyte activation
TH1 and TH2
what is there an imbalance of in COPD
proteases and anti-proteases (a1-antitrypsin)
what does cigarette smoke stimulate in COPD
activates macrophages which release neutrophil chemotaxic factors
neutrophils then release proteases that break foen connective tissue in the lung parenchyma and stimulate mucus hypersecretion
