Immunopathology Flashcards
Hypersensitivity reactions can be easily remembered by the ABCD pneumonic, what do ABCD stand for?
"ABCD" Anaphylaxis AntiBody mediated Immune Complex mediated Delayed hypersensitivity
What are the features of SLE? What is needed for its diagnosis?
autoimmune disease characterized by loss of self-tolerance & production of autoantibodies. To be diagnosed with lupus, you must have 4 out of 11 common signs of the disease (acute cutaneous lupus, chronic cutaneous lupus, oral or nasal ulcers, non-scarring alopecia, arthritis, serositis, renal, neurologic, hemolytic anemia, leukopenia, and thrombocytopenia)
Which hypersensitivity reactions are involved in the pathogenesis of SLE?
Hematologic (type II HS)
-hemolytic anemia, thrombocytopenia, neutropenia, lymphopnia
Arthrities (type III HS)
-polyarthralgia & synovitis w/o joint deformity
What are ANA’s and why do they form?
ANA’s are a type of autoantibody - they are antinuclear antibodies. They are found in many disorders including autoimmunity, cancer and infection, with different prevalences of antibodies depending on the condition. This allows the use of ANAs in the diagnosis of some autoimmune disorders, including systemic lupus erythematosus, Sjögren’s syndrome, scleroderma, mixed connective tissue disease, polymyositis, dermatomyositis, autoimmune hepatitis and drug induced lupus.
Sjogren Syndrome causes what symptoms? Does it always occur as a single disease?
autoimmune Dx characterized by destruction of lacrimal and salivary glands. Clinical features: inability to produce saliva and tears, F>M, keratoconjunctivitis sicca (dry eyes) and corneal ulcers, xerostomia (dry mouth), mikulicz syndrome (enlargement of the salivary and lacrimal glands).
It is often associated with RA and other autoimmune dz
What is the pathogenesis of scleroderma, and which visceral organs can be involved?
autoimmune dz characterized by fibroblast stimulation and deposition of collagen in the skin and internal organs. Visceral organs involved: esophagus, GI tract, pulmonary fibrosis, cardia fibrosis, and kidney fibrosis
What is CREST syndrome?
localized scleroderma. Anti-centromere antibodies, skin involvement of the face and hands, late involvement of the face and hands, late involvement of visceral organs, relatively benign clinical course
How can Polymyositis be differentiated from fibromyalgia on physical exam?
Dermatomyositis has skin involvement and is due to anti-body mediated damage. It is also characterized by purple-red (heliotrope) rash on eyelids.
Polymyositis does not have skin involvement and is due to T cell-mediated damage
What is Bruton’s agammaglobulinemia? What does the mutation result in ?
inherited immunodeficiency characterized by a failure to produce mature B cells and plasma cells, resulting in agammaglobulinemia (very low levels of protective immune system proteins called immunoglobulins)
Is common variable immunodeficiency a single disease?
No! It’s a group of disorders characterized by B-cell maturation defect & hypogammaglobulinemia
What fails to develop in DiGeorge Syndrome, and what does it result in?
embryologic failure to develop the 3rd & 4th pharyngeal pouches. Results in the absence of the parathyroid glands and thymus.
Why do males usually get SCID? How does treatment differ now than from the past?
This condition is inherited in an X-linked recessive pattern. The gene associated with this condition is located on the X chromosome, which is one of the two sex chromosomes. In males (who have only one X chromosome), one altered copy of the gene in each cell is sufficient to cause the condition. In females (who have two X chromosomes), a mutation would have to occur in both copies of the gene to cause the disorder. Because it is unlikely that females will have two altered copies of this gene, males are affected by X-linked recessive disorders much more frequently than females. Tx: gene therapy (p) interference RNA therapy (c)
What are the common systemic diseases that cause a functional immunosuppression?
DM, collagen vascular diseases (e.g. SLE), and chronic alcoholism
What 3 type of rejection occur in renal transplantation?
hyperacute rejection (micro: neutrophilic vasculitis with thrombosis), acute rejection (abrupt onset of oliguria and azotemia), chronic rejection (poor response to treatment)
