We report three patients of the same family with linear calcification of
the ascending aorta, severe calcific mixed aortic valve disease
associated with increased levels of globulins, lambda-chain gammopathy,
an increased T4/T8 lymphocyte ratio, and other immunologic
abnormalities. None of the patients had syphilis, atherosclerosis,
abnormalities of calcium or phosphorus metabolism, lymphadenopathy, or
other systemic diseases. It is postulated that these cases and some
previously reported in the literature as idiopathic represent a distinct
pathologic entity, familial or sporadic, in which localized vascular and
valvular calcific disease is associated with an underlying immunologic
disorder or autoimmune process
