PERIPHERAL VASCULAR DISEASES 



12 39 



Involvement of the gastrointestinal tract is common, 

 especially the esophagus, frequently the small bowel 

 and occasionally the colon. This may produce 

 obvious functional changes in these organs. Joint 

 involvement may mimic rheumatoid arthritis and 

 skeletal muscle involvement with atrophy and fibrosis 

 may resemble dermatomyositis. Pulmonary involve- 

 ment is very common with peribronchial and inter- 

 stitial fibrosis and destruction of alveolar walls. 



The vascular manifestations of the disease may be 

 widespread. There is thickening of vessel walls, peri- 

 vascular fibrosis, intimal proliferation, obliterative 

 vasculitis, thrombosis, fibrinoid necrosis, and cellular 

 infiltrations with polymorphonuclear and round 

 cells. 



Usually smaller vessels are predominantly affected, 

 but lesions may be encountered in any vessel of the 

 body. The coronary, pulmonary, dermal, and renal 

 vascular beds are notable participants in this vascular 

 disease. Primary changes in the myocardium, apart 

 from the involvement of mvocardial vessels, are 

 frequent. There is interstitial fibrosis, myocardial 

 degeneration, endocardial, epicardial and peri- 

 cardial fibrosis, and ventricular dilatation and hyper- 

 trophy. 



Obviously, clinical manifestations may be multiple 

 and varied. Renal involvement may cause albumi- 

 nuria, hematuria, and hypertension. Myocardial 

 disease may present any of the findings seen in 

 congestive heart failure. Pulmonary involvement 

 may be expressed as respiratory alveolar-capillary 

 block syndrome, fibrosis and emphysema, obstructive 

 and restrictive ventilatory dysfunction, hypoxia, 

 carbon dioxide retention, pulmonary hypertension, 

 polycythemia, cor pulmonale, and the like. Thoracic 

 involvement with the tight constricting skin may 

 produce hypoventilation and circulatory dysfunc- 

 tion. 



All the above and other changes in scleroderma 

 are obviously of importance to a discussion of the 

 peripheral circulation because, potentially, they may 

 all contribute to disordered peripheral vascular 

 physiology. To quantitate their effects, however, 

 would be a difficult or impossible task. In addition 

 to these general effects, more direct factors in sclero- 

 derma may influence vascular function. Comments 

 made in the preceding section on vasculitis and 

 collagen disease, in general, are applicable here. As 

 noted, obliterative vascular change is probably the 

 major factor in vascular dysfunction, but a super- 

 imposed functional vasospastic element has been 

 suggested. 



The frequent occurrence of Raynaud's phenome- 

 non in scleroderma is of interest. It may precede, 

 accompany, or follow the clinical disease onset. The 

 true relationship between the two is unknown. It 

 was long held by many that the vasomotor ab- 

 normality was of etiologic importance in the patho- 

 genesis of scleroderma. The majority of current 

 opinion, however, is that associated Raynaud's 

 phenomenon is a secondary manifestation of sclero- 

 derma due to the primary disturbance in blood 

 vessels and connective tissue. In this regard it is 

 analogous to secondary Raynaud's phenomenon 

 occurring in other collagen diseases. 



Of the collagen diseases, however, scleroderma 

 presents an additional unique factor in that the 

 vessels are entrapped in a fibrotic ever-contracting, 

 poorly distensible environment. Thus, in addition to 

 intravascular occlusion there may be, in effect, 

 extravascular constriction or strangulation (59, 81, 

 92). Greatly increased pressures have been found 

 in the subcutaneous tissue in patients with sclero- 

 derma. Studies have shown tissue pressure values 

 up to 320 mm of water in patients with scleroderma, 

 whereas normal persons do not exceed 54 mm (92). 

 These added factors may be of significance in ex- 

 plaining the altered peripheral vascular function in 

 scleroderma. In this disease, reduced skin tempera- 

 ture and decreased digital pulsations are common. 

 That these changes are largely structural or organic 

 in origin is suggested by failure of these parameters 

 to return to normal after use of sympatholytic drugs 

 or inhibition of sympathetic tone through nerve 

 block or sympathectomy. 



Vasoconstrictor mechanisms in acute thrombophlebitis. 

 The clinical manifestations of thrombophlebitis have 

 been adequately described in a number of publica- 

 tions (1, 3, 87, 104) and will not be repeated here. 

 Mechanisms of intravascular clotting are discussed 

 elsewhere in this volume. 



Perhaps a few words regarding terminology are in 

 order. It has been common practice in the past for 

 clinicians to use the terms "thrombophlebitis" and 

 "phlebothrombosis" to represent two different and 

 distinct clinical syndromes (69). Thrombophlebitis 

 was considered to represent a rather intense inflam- 

 matory reaction in the involved vein with a more 

 firmly attached thrombus. Although it produces a 

 more dramatic local reaction, it was considered to be 

 less dangerous, since there was less likelihood for 

 emboli to break from the thrombus. In contrast, 

 phlebothrombosis, although bland with respect to 

 local reactions and manifestations, was considered to 



