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During the period from Jan 1959 to Sept. 1967, 15 cases with primary arteritis or pulseless disease were observed in the Department of Medicine of National Medical Center. The diagnosis was made-on the basis of autopsy findings in 2 cases, of aortographic findings in 11 cases and of both it 2 cases. Female predominated in ratio to male to about 2.8:1, numbering 11 cases(73%) and the most predominant age- incidence was in the 2nd and 3rd decades of life, numbering 11 cases with youngest 15 years and the eldest 50 years of age. (During the period from January 1962 to December 1966, 7 cases with primary arteritis was observed in the pediatric department of the National Medical Center, with predilection for female numbering 4 cases and , age variation ranged from 7 years old to 11 years old )29) The clinical manifestations varied considerably, depending on the vessels involved the degreeof obstructionand extent of development of collateral circulation. The most frequent clinical complaints were headache in 6 cases, dyspneas dizziness and edema in b cases respectively. The prinicipal clinical signs were absent or weak arterial pulsation in 10 cases. hypertension } in 8 escapes, bruit audible in 6 cases and cardiomegaly in 7 cases The pathogenesis of hypertension in this series seemed to be renal vascular origin on the ground that among 8 cases with hypertension, a complete or partial obliteration of renal artery orifice w observed in 6 cases and involvement of abdominal aorta neighboring renal artery orifice in 2 case . The laboratory findings were, generally unremarkable except for the elevated erythrocyte sedim - ntation rate in 9 caseas, proteinuria in 7 cases and increase in serum gamma globulin fraction in 3 cases of 5 tested. The subclavian artery was the most commonly involved(10 cases); next were the abdominal aorta (6 cases), renal artery(6 cases), carotid artery(4 cases), terminal aorta(3 cases) and thoracic aort (2 cases). In general, all cases seemed to pursue a gradual down-hill course in spite of extensive medical therapies such as predisone, dicumarol, digitalis or anti-hypertensive agents, and 4 cases including case No. 4 receiving teflon bypass graft surgery, expired of hypertensive heart het failure. The histopathologic findings in this series were those of chronic panarteritis with marked lumina narrowing resulting from intimal fibrous thickening associated with thrombosis organized or party recanalized, vasular proliferation´ in intima; and scanty perivascular small round cell infiltrates. In the media there are striking fragmentation and condensation of elastic fiber associated wit focal necrosis and predominant lymphocytic infiltrates including a few of plasma cells and multinucleated foreignbody giant cells in one of cases. In adventitia, perivascular cuffing of lymphocytic infiltration notably around the vasa vasorum with pattern of endarteritis and fibrous hyperplasia are identified.
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