The immunological cardiovascular diseases are a very diverse group of
clinical entities that generally are of either unknown aetiology or of
unproven pathophysiology. Most of the conditions with a proven, or st
rongly suspected, aetiology are caused by infections, with the best ex
amples being acute rheumatic fever and Lyme disease. However, even wit
h these diseases, the primary pathophysiological mechanisms have not b
een irrefutably established. In addition to the importance of infectio
us agents in the immunological cardiovascular diseases, other factors
have been identified that are associated with or modify these diseases
. These factors include age, genetic background and coexisting inflamm
atory diseases. The proposed immunological mechanisms important in the
immunological cardiovascular diseases include: (a) immune mimicry, in
which antigens of an infectious agent crossreact with self antigens;
(b) modification of self antigens by infections or other inflammatory
processes; (c) introduction of self antigens to the immune system foll
owing a traumatic or inflammatory event; and (d) dysregulation of an a
utoimmune response. The immunological effector mechanisms include: (a)
passive deposition of immunoglobulin or immune complexes in cardiovas
cular tissues with resulting inflammation; (b) autoantibodies that dam
age the cardiovascular system directly or indirectly; and (c) cell-med
iated immune responses to antigens within the cardiovascular system. T
he clinical diagnosis of the immunological cardiovascular diseases is
facilitated by clinical criteria and by selective laboratory tests in
certain diseases. Laboratory tests, other than histology, do not usual
ly provide definitive answers but serve to confirm suspected diagnoses
. The vague, often systemic, symptoms associated with many of the diso
rders add to the clinical confusion of diagnosis. Despite the lack of
clearcut aetiologies, the classification of these diseases does facili
tate therapeutic decision making. This is particularly important since
the prognosis of some of these conditions, such as acute rheumatic fe
ver, Lyme disease, Wegener's granulomatosis, systemic necrotising vasc
ulitis and temporal arteritis, is significantly improved by treatment.
Classification schemes for vasculitis remain primarily descriptive, b
ut are useful for dividing the entities into categories with similar r
esponse to treatment. Significant progress and improvement in the trea
tment of the immunological cardiovascular disorders await better defin
ition of the aetiologies and primary pathophysiological mechanisms inv
olved.