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The Raynaud Syndrome MARTIN BIRNSTINGL

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The Raynaud Syndrome MARTIN BIRNSTINGL Postgraduate Medical Journal (May 1971) 47, 297-310. Postgrad Med J: first published as 10.1136/pgmj.47.547.297 on 1 May 1971. Downloaded from The Raynaud syndrome MARTIN BIRNSTINGL M.S., F.R.C.S. St Bartholomew's Hospital, London, E.C.1 'Arteritis. Thank heaven I have not to express in a later paper (1874) he put forward 'the hypothesis any opinion either upon its frequency, or upon the of a contraction of the terminal vascular ramifica- share which belongs to it in the different arterial tions, varying from a simple diminution of calibre changes, or to the part which it plays in general.' up to complete narrowing of the lumen of the vessel. Maurice Raynaud, 1862. To the total closure of the arterial and venous vessels would correspond an exsanguine and Maurice Raynaud described in his thesis 'De cadaveric state of the extremities ... whilst the l'Aphyxie Locale et de la Gangrene Symetrique des arterioles only being closed and the venules open, Extremites' (1862) examples of both episodic digital one would see a venous stasis produced by failure asphyxia and frank local gangrene, which justifies of vis a tergo, whence the cyanosis and livid ap. the broad pathological field covered by the present pearance'. article. Unfortunately he failed to understand the widely Protected by copyright. varying pathology of the examples he described, or Definitions that necrosis or frank gangrene can only result from Raynaud's syndrome. The general syndrome of actual structural obliteration of the vessels and it arterial insufficiency of the fingers, regardless of was left to Jonathan Hutchison (1901) to point out cause, and whether presenting as episodic or con- the many different diseases which are liable to pre- tinuous ischaemia, digital necrosis or gangrene. sent with digital ischaemia. Hutchison also advo- cated the term 'Raynaud's phenomenon' to describe Raynaud's phenomenon. Episodic digital asphyxia, the intermittent episodes and showed that these due to arterial insufficiency. could be due either to vasospasm or organic obstruc- Raynaud's disease. A disorder, seen principally in tion, although even today this distinction remains young, healthy women, who show episodic digital difficult on clinical grounds alone. asphyxia due to an exaggerated reaction to cold in otherwise normal digital vessels. Physiology of digital circulation http://pmj.bmj.com/ The rate offlow through any vascular bed depends Introduction upon the pressure gradient across the bed and the Raynaud provides us with an accurate clinical resistance to flow through it. The gradient, in turn, description of the disease which now bears his name. depends upon vessel length, radius and the viscosity 'Madame X, aged 26 years, has never been ill; but of the blood. Variation in any of these five para- she has been the subject since childhood of an meters may change the rate of blood flow; however, infirmity which makes her an object of curiosity to the local circulatory effects of digital vascular her acquaintances. Under the influence of very pathology are largely due to variations in digital on September 27, 2021 by guest. moderate cold, and even at the height of summer, arterial and arteriolar radius. Digital blood flow is she sees her fingers become exsanguine, completely normally concerned with temperature regulation insensible, and of a whiteish yellow colour. This rather than with local metabolic needs, and is remark- phenomenon happens often without reason, lasts a able both in the degree to which it is responsive to variable time and terminates by a period of very the local temperature and in the great variation of painful reaction, during which the circulation is flow rates attained, which may change by as much re-established little by little and returns to the normal as two hundred times (Burton, 1939). The mechanism state. Madame X has no better remedy than shaking by which local blood flow is varied is mainly in the her hands hard, or soaking them in lukewarm arterioles and arteriovenous anastomoses, the latter water.... This state, which I dare hardly call a being especially numerous in those parts of the disease, is local syncope in its simplest form' (1862). fingers and toes-the nail beds and distal phalangeal Raynaud clearly recognized the vasospastic nature pulps-in which flow rates alter most. of this phenomenon in young, healthy women and Normal control of digital blood flow is complex, 298 Martin Birnstingl Postgrad Med J: first published as 10.1136/pgmj.47.547.297 on 1 May 1971. Downloaded from but appears to be governed by (1) central nervous hand, which again appears to be a property of the influence acting mainly through sympathetic vaso- digital arterioles themselves. constrictor fibres and (2) local responses in the smooth muscle in the wall of the blood vessels Aetiology of Raynaud's disease themselves. Maurice Raynaud attributed the local syncope to In true Raynaud's disease, or functional vaso- an exaggeration of the normal reflex response to spasm, there is constriction of both arterioles and cooling, but dominated by Claude Bernard's recent venules, producing the characteristic waxy appear- description of the sympathetic nervous system he ance. Occasionally the venules and capillaries may thought that the abnormality was central or, in his dilate, probably through local anoxic paralysis, own words, due to 'increased irritability of the producing a bluish tinge. When the spasm passes central parts of the cord presiding over vascular off, a stage of reflex vasodilatation is produced due innervation' (1874). However, the fact that local to local accumulation of dilator substances in the syncope is usually unaccompanied by peripheral tissues. sweating and the frequency ofrelapse after apparently In organic obstruction, the block is usually at the complete sympathetic denervation are against this level of the digital arteries or arterioles. Quite apart explanation. from the overt reduction in digital blood flow, Sir Thomas Lewis (1930) felt that the fault lay in important passive factors contribute to the resulting abnormal susceptibility of the digital arteries them- ischaemia. The fall in transmural pressure in the selves to stimulation by local cooling. He showed artery beyond the block allows passive contraction that when reflex vasodilation had been produced of the elastic vessel wall, further decreasing flow. by warming the body, vasospasm could still Moreover, this reduction in vessel radius produces be produced by putting the hands in cold a four-fold decrease in flow, according to Poiseuille's water. Conversely, vasospasm could not Protected by copyright. be Law. Another passive factor is the apparent viscosity produced by body cooling if the hands were of the blood, which increases at low rates of blood kept warm. Whatever the explanation of Lewis's flow. The net result of these various influences is that 'local fault', his theory accords with the clinical flow becomes dependent upon a state of unstable picture and explains the poor results of sympathec- equilibrium, in which the intra-arterial pressure tomy in Raynaud's disease, since the digital vessels distal to the obstruction may fall beyond the 'critical retain their hypersensitivity to local cooling and closing pressure' of the vessel at this level, causing ultimately regain their intrinsic tone. However, sudden standstill (Roddie and Shepherd, 1957). This Raynaud was probably correct in maintaining that is particularly likely when vasomotor tone is high, emotional factors play an additional part in some of for instance during exposure to cold or when vessels these patients, since peripheral vasoconstrictor are compressed by gripping with the hand, and activity is undoubtedly influenced by the psyche explains the intermittency of the ischaemic episodes (Fox, 1968). (Raynaud's phenomenon) in the presence of per- Raynaud postulated that veins as well as arteries manent organic blockage. But since the structural and arterioles share in the constriction, accountinghttp://pmj.bmj.com/ changes usually involve the arteries of different for the waxy pallor, but direct evidence is lacking fingers to varying extents, it is uncommon for the on this point. The accompanying numbness of the ischaemia to occur symmetrically. fingers is presumably caused by temporary ischaemia of sensory nerves. Effect ofsympathetic denervation Peacock (1959) has shown by plethysmography The blood flow in the hand is first increased due to that blood flow in the hands of patients with Ray- removal of central vasoconstrictor activity. Peak naud's disease is reduced, compared with normal on September 27, 2021 by guest. flows are reached about 2 days after sympathectomy, controls, even under warm resting conditions. It is with increases of the order of five to twelve times difficult to exclude the presence of secondary organic resting flow. This is followed by gradual reduction narrowing in such subjects. Peacock also found that in blood flow until, after a few weeks, the resting the concentration of catecholamines in venous blood flow is close to the preoperative level (Barcroft, leaving the hands was higher in Raynaud's disease 1952). The decline of flow occurs irrespective of than in controls, particularly during reflex vaso- whether preganglionic or postganglionic section has constriction. He suggested that this was due to been performed and is probably due to recovery of increased production of adrenaline and noradrena- intrinsic tone in the muscle of the vessel wall. Pro- line during vasoconstriction, but unfortunately vided sympathectomy is complete, reflex body heat- failed to exclude a more obvious cause for the ing or body cooling has no effect upon flow rates in observed rise in concentration, namely the reduced the hand. However, after 1 or 2 years a vasoconstric- blood flow which he recorded. Furthermore, he did tor response usually returns to direct cooling of the not measure the levels in the arterial inflow to the The Raynaud syndrome 299 Postgrad Med J: first published as 10.1136/pgmj.47.547.297 on 1 May 1971.
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