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Immune Complexes

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•immune complex disease = type III hypersensitivity
•(in some diseases, circulating immune complexes may be formed secondary to tissue damage that has occurred through cell-mediated or other mechanisms)

 

serum sickness

•prototype disease = SLE
•occurs 8 to 12 days after the subcutaneous injection of the horse diphtheria antitoxin
•fever, lymphadenopathy, arthralgias, leukopenia, urticaria, and albuminuria
•SLE/ serum sickness similar:  glomerulonephritis, arthritis, and vasculitis
•a possible explanation of the continued circulation of the immune complexes in patients with SLE has been the defective function of the RES reticuloendothelial system in these patients

 

arthus reaction

•prototype disease= LCV
•elicited by:  immunizing an animal with a foreign protein and then injecting the antigen into its skin
•reaches peak intensity between 4 and 10 hours later
•characterized by erythema, edema, hemorrhage, and necrosis
•clinically, histologically, and immunopathologically similar to LCV in humans

 

 

whenever immune complexes are deposited, they activate the complement system, neutrophils are attracted to the site (C5a) and inflammation and tissue injury occur:

•pustules
•urticarial plaques
•erythema marginatum (may be true urticaria vs. variant of the figurate erythemas)
•Still’s disease
•SLE  (histology = LCV)
•urticarial vasculitis
•serum sickness prodrome of Hepatitis B  (histology = LCV)
•purpura (+/- palpable)/ulcers
•immune complexes cause arthritis and glomerulonephritis

 

the link is the neutrophils though there are other causes of neutrophil activation (which is why some diseases are in one group and not all 3)

•pathogenic group = immune complex disease
•histologic group = neutrophilic dermatoses
•clinical group = pustular diseases

 

 

IMMUNE COMPLEX DISEASE (and neutrophil diseases) (by antigen)

•infectious:
•group A beta hemolytic strep
•PSGN post-streptococcal glomerulonephritis
•rheumatic fever
•HSP
•SBE subacute bacterial endocarditis and chronic bacterial endocarditis
•erythema nodosum (neutrophilic dermatosis)
•Sweet’s syndrome (neutrophilic dermatosis)
•acute guttate psoriasis (TH1,  neuts on path)
•hepatitis B
•serum sickness syndrome
•other:  bowel associated dermatosis-arthritis syndrome
•drugs
•chronic disease
•IBD inflammatory bowel disease (--> PG, e. nodosum)
•malignancy
•AML = neutrophilic leukemia (--> Sweet’s, neutrophilic eccrine hidradenitis)
•erythema elevatum diutinum
•pyoderma gangrenosum
•e. nodosum
•self-antigens/connective tissue disease
•IgG – rheumatoid factor = IgM vs. Fc portion of normal human IgG
•DNA – SLE immune complexes
•idiopathic