Autoimmune Conditions
Characterized by autoimmunity against systemic proteins and multi-system disease, often associated with constitutional symptoms.
idiopathic inflammatory myositis (polymyositis, dermatomyositis)
progressive systemic sclerosis (scleroderma)
Sjogren's syndrome
mixed connective tissue disease
can also have overlap of the above conditions.
associated with specific antibody - RNP
Immunodeficiencies
Serious primary immune deficiency diseases are not rare as a group, and there are over 140 known genetic causes.
An approach to recurrent infections is important.
Causes of immunodeficiency include:
- hypogammaglobulinemia
- B cell deficiency
- IgA deficiency
- CD-4 deficiency
- common variable immunodefiency
IgA deficiency
1:500
chronic infections at mucosal surfaces
clinical variability: may be based on function of IgM at mucosal surface
most people do not require treatment beyond antibiotics
no known cause
common variable immunodeficiency
can develop at any age; perhaps genetics
apparent defect in T cell/B cell communication
normal number of B cells, but no antibody response
also problems with viral clearance and occasionally autoimmunity
Immune system deregulation
fights against infection
tumour surveillance
autoimmune disease
Neutrophils are packed with hydrolytic enzymes that degrade the ECM to allow quick passage. This can lead to bronchiectasis.
B Cell Deficiency
CD4 Deficiency
Normal CD4 levels are 1.7-2.8 x 109/L .
With T cell deficiency, one sees viral, parasitic, and fungal infections that become serious.
T cells also respond to intracellular bacterial infections, and T cell deficiencies can lead to increased susceptibility to bacteria including salmonella and mycobacteria.
There is also an effect on the humoral response, leading to increased susceptibility to bacterial infections.
