Raynaud's Syndrome

Raynaud's Syndrome

perforation. Any ulcers visible from the serosal surface but 9 Mandcl BK, Mani V. Colonic involvement in salmonellosis. Lan et 1976;i:887-8. 10 Pascall CR, Stearn EJ, Mosley JG. Salmonella hadar peritoniltis. BrMed.7 1980;280:26. unperforated should be oversewn. 11 Tauiner WA, Collins PG, Fah\ AM. Spontaneotus perforation of the coloti in salmonlella enterocolitis. IrJ Med Sci 1980;149:37. Gastroenteritis resulting from the various serotypes of 12 Gill KP, Feelev TM, Keanie FBV. T'oxic megacolon and perforationi caused by salmonclla. S enteritidis is becoming increasingly common and may Br Surg 1989;76:796. 13 Boyd JF. Colonic involvement in salmonellosis. Lancit 1976;i:1415. be extremely severe. The presentation varies but usually 14 Kurtx JB. Leg abscesses caused by Salmonella heidelberg. Lancet 1976;i:20(0-1. includes abdominal cramps, pain, and diarrhoea that may be 5 Christie AB. 'I'rcatment of typhoid carriers with ampicillin. Br Medj 1964;i: 1609. BMJ: first published as 10.1136/bmj.300.6724.553 on 3 March 1990. Downloaded from bloody. This picture may be confused with that of ulcerative colitis.9 Diagnosis may sometimes be difficult: salmonella enteritis and ulcerative colitis are both common and their sigmoidoscopic appearances are similar. If the colitis is treated with corticosteroids then simultaneous systemic anti- Raynaud's syndrome biotic cover is essential.' The micro-organisms invade focal areas of the small Thymoxamine, iloprost, and ACE inhibitors and large bowel,9 and occasionally the colitis progresses to perforation. This complication has been reported after are among the effective treatments now infection with many different species, including S hadar,'° available S typhimurium," and S newport.' We recently encountered perforation and faecal peritonitis in a patient infected in the Raynaud's phenomenon was described over 100 years ago, recent outbreak of S kegougou. A high index of suspicion is but we still do not understand the pathogenesis of the ab- necessary during an outbreak of salmonella enteritis; if normally prolonged (sometimes painful) episodic peripheral perforation of the colon does occur prompt colectomy is vasospasm in response to cold or emotional stimuli. The essential for a favourable outcome.'I controversy has yet to be settled between the hyperactivity of Extraintestinal focal lesions with salmonella are often the sympathetic nervous system described by Raynaud' and associated with other chronic disease. The focal lesions may Lewis's local fault.2 manifest themselves long after an episode of enteritis or the The clinical classification into primary and secondary original bowel infection may have been silent. S enteritidis Raynaud's syndrome and phenomenon is also unsatisfactory. has a predilection for blood vessels. Possibly, intravascular Patients who have Raynaud's phenomenon but are otherwise salmonellas localise at sites of atheromatous ulceration and fit may commonly be shown to have one or more autoanti- may cause thrombosis of major vessels or may colonise bodies present in low titre. Some of these patients will later vascular grafts.'9 In addition they may damage the venous develop one of the connective tissue diseases.34 system, causing septic thrombophlebitis,'4 iliac vein throm- So what do we know? Patients with Raynaud's syndrome bosis, or pulmonary embolism. These focal lesions have a may be shown to have increased sensitivity or density of the tendency to chronicity and may mimic tuberculosis, particu- peripheral c1 adrenoceptors, or both, in both the vessel walls3 larly in osteomyelitis of a vertebra or paravertebral abscess. and the platelet membranes.6 Other factors implicated in Circulating S typhi is trapped by the liver and excreted in the the disease are abnormal platelet adhesiveness,7 increased bile. Acute cholecystitis as a complication of typhoid has whole blood and reduced red cell deform- become rare since the advent ofantibiotics, but it may develop plasma viscosity,-'0 ability," reduced activity of the fibrinolytic system,'2 '3 an http://www.bmj.com/ in a normal gall bladder and may proceed to perforation and imbalance in the cyclo-oxygenase products of arachidonic biliary peritonitis. acid - thromboxane (TXA2) and prostacyclin (PGI2)-and Finally, the general surgeon may be asking for help in the hypersensitivity of serotonin (5-HT2) receptors.'4 Whether management of patients who are chronic typhoid carriers. any or all of these are crucial in the pathogenesis or are simply The typhoid carrier passes organisms in the faeces, presum- epiphenomena remains to be determined. Attempts to correct ably derived from a focus of infection in the gall bladder or these abnormalities have been reflected in numerous thera- biliary tract. Chronic typhoid cholecystitis is symptomless. peutic approaches to the disease. Despite the high proportion The most notorious carrier was Mary Mallon, who was the of women affected the contribution of oestrogen and other on 30 September 2021 by guest. Protected copyright. source of an outbreak affecting 1300 people in New York in female sex hormones remains vague and poorly defined.'* A 1903. Although prolonged administration of ampicillin in possible exception may be those patients whose symptoms high dosage has been successful in eliminating carriage of in S even in patients with gall stones and non-functioning begin at or near the menopause, whom hormone replace- typhi ment treatment may be beneficial. ` gall bladders,'5 cholecystectomy is regarded as the most In the clinical setting primary Raynaud's syndrome is effective way of permanently curing the carriers-provided common. 16 The average general practitioner may expect to see there is no associated infection in the biliary or urinary tract. one or two new cases a year. Fortunately it is also usually J G MOSLEY benign and compatible with an entirely normal lifespan Consultant General and Vascular Surgeon (unpublished data). The prognosis of secondary Raynaud's A K CHAUDHURI syndrome is that ofthe underlying disease and depends on the Consultant Microbiologist severity with which target organs are affected.3 Leigh Infirmarv, As the common stimulus is cold, either local or general, Leigh, advice not to smoke and to keep warm remains the corner- Lancashire WN7 I HS stone of management. Smoking a cigarette may produce a fall in temperature of2°C or 3°C in the fingertips ofnormal people 1 Keenani JP, Hadley SP. The surgical maniagemcnt of thyroid pcrforations in childreni. Br] Surg in a comfortable ambient temperature. Patients may be 1984;71:928-9. 2 Dunkerley GE. Perforation of'the ileum in enteric fever. BrMledj 1946;ii:454-7. helped to keep warm with appliances ranging from chemically 3 Dickson JAS, Cole GJ. Perforation of the terminal ileum. Br_7 Surg 1964;51:893-7. activated handwarmers to electrically heated gloves, foot 4 Gibney EJ. Typhoid perforation. Brj7Surg 1989;76:887-9. 5 Angorn IB, Pillarv SP, Hegarty MM, Baker LWd. I'yphoid perforationi of the ileum. S Afr Aledj warmers, and hand and ear muffs. Further information 1975;49:781-5 concerning these may be obtained from the Raynaud's 6 Chouhan MK, Pandu SK. Typhoid enteric perforation. Brj Surg 1982;69:173-5. 7 Archampong EO. Operative treatment of tvphoid perf'oration of the bowel. Br Med J 1969;iii: Association (112 Crewe Road, Alsagar, Cheshire ST7 2JA; tel 273-6. 0270 872 8 Dronfield MW, Fletcher J, Langman MJS. Coincident salmonella inf'ections anld ulcerative colitis. 776). BrMedj 1974;i:99-100. About one third of patients also have attacks in response to BAM1 VXiLrME 300 3 MARCII 1990 553 emotional stress, though the pattern of response in primary and cause generalised vasodilatation. These are acute effects and secondary disease may be different." Advice concerning and stop shortly after treatment is stopped. As circulatory avoidance of such stress may also be given. Patients in this (headache and dizziness) and gut (vomiting and diarrhoea) category may respond to behavioural therapy or may benefit side effects are frequent and may be severe their use has from conditioning (placing the affected hands and feet for a been confined to patients with severe secondary Raynaud's time in warm surroundings during general body cooling)- syndrome with much reduced resting perfusion complicated BMJ: first published as 10.1136/bmj.300.6724.553 on 3 March 1990. Downloaded from treatment requiring considerable time and motivation.'9 20 by digital ulceration and to patients with frank gangrene More simply, placing the hands in warm water for five who are undergoing digital surgery in the hope of enhancing minutes twice daily is said to increase resting peripheral postoperative wound healing. In patients with severe second- perfusion and reduce the reactivity to cold.2' ary syndrome with reduced perfusion and digital ulceration, The ineffectiveness ofcervical sympathectomy and plasma- regimens have varied with the prostanoid used. The stable pheresis has condemned these measures to interesting his- carbacycline analogue iloprost has been given for six to torical footnotes. The attacks may be so frequent, or so eight hours on three consecutive days at a rate starting with painful, or last so long that drug treatment may be offered in 0-5 ng/kg/min and increasing incrementally at 15 minute an attempt to enhance peripheral perfusion and reduce the intervals by 0 5 ng to a maximum of 2 ng/kg/min. Side effects response to cold, especially during the winter. Despite the may preclude this high dosage, but it has been shown to several

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