management of pulmonary sarcoidosis

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Page 1: Management of Pulmonary Sarcoidosis

890

technique is obviously important but several goldenrules of surgery can be ignored. The chosen drainagetrack does not have to be extraperitoneal, nor is

dependent drainage essential, although both are

desirable. IS For guidance of the needle to the abscesssome operators favour ultrasound, 18 others CT,20 andyet others fluoroscopy.12 Once pus is identified byaspiration, a drainage catheter is inserted either over aguide wire or through a trocar. The second method isusually easier for the larger and more superficialabscesses.21 The thick pus which is aspiratedimmediately after insertion of the drain is followed bythin serosanguineous fluid that flows easily along smallcatheters, so large drains are unnecessary. On the otherhand, drainage must be adequate. In one series 19 7

patients needed operations to complete the drainage ofan abdominal abscess; in another, 6 abscesses recurred(although 4 were caused by a pre-existing intestinalfistula); 17 and in a third 3 patients died from inadequatedrainage.16 Furthermore, it is possible to spread theinfection to the pleura or peritoneum, 18 to create anintestinal fistula, 17,18 or to damage blood vessels,17 socareful planning and accurate placement of the drainreally is essential. Irrigation of the catheters’is probablyunnecessary and some investigators prefer to monitorthe resolution of the abscess with ultrasound or CTrather than with sinograms. Antibiotic treatment isessential during and after percutaneous drainage and,of course, tubes fall out and have to be replaced(whether they were inserted by surgeons or

radiologists). Pancreatic abscesses are particularlytroublesome. 18 On CT, pus can be difficult to

distinguish from a phlegmon,8 and even when pus isdrained percutaneously the necrotic debris of a

pancreatic abscess will often block the drains. Surgicaldrainage may therefore be wiser. Multiple abscessesand those caused by an intestinal fistula are also besttreated by operation. Operative drainage of any intra-abdominal extrahepatic abscess carries a substantialmortality 22 and in this respect percutaneous drainageoffers a real advantage. However, the death rate

associated with undrained pus approaches 100%; so,when the percutaneous approach is contraindicated orfails, surgical drainage is essential.

Management of Pulmonary SarcoidosisWHEN can sarcoidosis be left untreated? Most chest

physicians will say, when the patient has hilar adeno-pathy without symptoms or biochemical disturbance;whereas hypercalcaemia, rapidly progressivepulmonary disease, and evidence of ocular, brain, or

20. Haaga JR, Alfidi RJ, Weinstem A. Percutaneous catheter drainage of abdominalabscesses N Engl J Med 1982; 306: 106-07.

21 Haaga JR, George C, Weinstein AJ, Cooperman AM. New interventional techniquesin the diagnosis and management of inflammatory disease within the abdomen.Radiol Clin North Am 1979; 17: 485-513

22 Bonfils-Roberts EA, Barone JE, Nealon TF Treatment of subphrenic abscess. SurgClin North Am 1975; 55;. 1361-66

myocardial involvement are indications for treatmentwith corticosteroids. Although the cause of sarcoidosisremains an enigma, it is now clear that T lymphocytesare concerned in pathogenesis of the pulmonarylesions. This opens the possibility that some measureof inflammatory activity, such as lymphocyte counts influid obtained by bronchoalveolar lavage, or

gallium-67 scanning of the lungs, might help in identi-fication of patients requiring treatment.2 For thispurpose some clinicians measure serum angiotensin-converting enzyme (ACE),3 but its value is question-able. It now emerges that serum ACE does not

accurately reflect activity of the pulmonary componentof the disease as assessed by the bronchoalveolar lavagelymphocyte count. 2,4 The place of gallium-67 scanninghas yet to be defined. It may be of use in distinguishingactive granulomatous pulmonary disease from burnt-out fibrosis, or in the assessment of response to

treatment. 5 On existing evidence, however, this

expensive investigation, with its small but distinctradiation hazard, should not be used routinely in theinvestigation of sarcoidosis. There is a rough correla-tion between gallium scanning and serum ACE, butnot between gallium scanning and lymphocyte countsin bronchoalveolar lavage fluid. Much has yet to belearned about the usefulness of these investigations inassessment and staging of the individual patient, but itis already clear that serum ACE measurements bythemselves are of limited value; so most clinicians willcontinue to use the crude guidelines which haveevolved from decades of clinical experience.

The existing treatment policies are not whollywithout rational foundation, and a review fromDenmark’ now provides reassuring data. 243 patientswere followed for up to 10 years and in 8707o the chest

X-ray abnormalities spontaneously improved within 2years. A clear X-ray for 2 years can be regarded as apermanent remission. This review also confirmed thathilar glandular sarcoidosis (stage I) carries the mostfavourable prognosis, 58% of patients in this groupimproving within 12 months and only 9% progressingto pulmonary sarcoidosis. Patients presenting withpulmonary infiltration and hilar adenopathy (stage II)

1. Crystal RG, Roberts WC, Hunninghake GW, et al. Pulmonary sarcoidosis a disease

characterised and perpetuated by activated lung T-lymphocytes. Ann Intern Med1981, 94: 73-94

2. Schoenberger CI, Line BR, Keogh BA, et al. Lung inflammation m sarcoidosiscomparison of serum angiotensin-converting enzyme levels with bronchoalveolar

lavage and gallium-67 scanning assessment of the T-lymphocyte alveolitis Thorax

1982, 37: 19-25.3. Leiberman J. Elevation of serum angiotensin-converting enzyme (ACE) level in

sarcoidosis. Am J Med 1975; 59: 365-724. Rossman MD, Dauber JH, Cardillo ME, Daniele RP Pulmonary sarcoidosis:

correlation of serum angiotensin-converting enzyme with blood andbronchoalveolar lymphocytes. Am Rev Resp Dis 1982; 125: 366-69.

5. Turton CWG, Grundy E, Firth G, et al Value of measuring serum angiotensin-converting enzyme and serum lysozyme in the management of sarcoidosis Thorax

1979, 34: 57-62.6 Beaumont D, Herry JY, Sapene M, et al. Gallium-67 in the evaluation of sarcoidosis

correlations with serum angiotensin-converting enzyme and bronchoalveolar

lavage Thorax 1982, 37: 11-187 Romer FK. Presentation of sarcoidosis and outcome of pulmonary changes Dan Med

Bull 1982; 29: 27-32

Page 2: Management of Pulmonary Sarcoidosis

891THE LANCET, APRIL 17,1982

did slightly less well: in the first year only a quarter hadX-ray clearing; 2907o showed improvement and 10%had disease progression. Of 11 patients with

pulmonary disease without associated hilar

adenopathy (stage III), only 1 improved spon-

taneously. Erythema nodosum was much more oftenseen in females than in males, as were uveitis andarticular complaints. Hypercalcaemia was detected in6.6% of patients, most of whom were male. Factorswhich pointed to a poorer prognosis were: age atdiagnosis more than 40; stage II and stage III disease;

unchanged chest X-ray at 1 year; extrathoracic lymphnode involvement; cutaneous lesions; and hyper-calcaemia.

The Danish review provides useful confirmationthat, in stage I sarcoidosis, specific treatment is seldomneeded. In stage II disease therapy may be required,but there is a case for postponing decisions about treat-ment for a year after diagnosis, unless there is rapidprogression of pulmonary disease. In stage III

sarcoidosis, treatment is nearly always indicated if thedisease is still active. It is in these patients with stageIII, and also in those with "stable" stage II sarcoidosis,that assessment of disease activity would be of greatestvalue. Time will tell whether bronchoalveolar lavage,and perhaps gallium-67 scanning, together with serumACE measurement, will fulfil their promise-for assess-ment and management.

THE RESPONSIBILITY TO PROTEST

A HEADLINE to an editorial in Nature1 a few weeks agoproclaimed: "Doves in false garb: the claim by the anti-nuclear movement of professional support is mostly a sham".Not all Nature’s readers agreed2-4 and the editor5 wasprovoked into renewing his strictures on those professionalgroups who are calling attention to the dangers of nuclearwar. There was no sham about the Second Congress ofInternational Physicians for the Prevention of Nuclear Warheld in Cambridge, England, on April 3-6, to which wereferred in a leader last week (p. 835). The First Congress ofI.P.P.N.W. was held a year earlier in Fairlie, Virginia, and 11countries were represented. In Cambridge the number ofcountries had nearly trebled and the movement is gainingforce, despite the reluctance of some doctors to enter intowhat they regard as a strictly political argument.Unfortunately, the doctors of China, France, and India, threeof the nuclear powers, did not send delegates to Cambridge tojoin in the protest against the continuation of the arms raceand against the acceptance of nuclear war as a survivableevent-when, in fact, medicine and its practitioners havevirtually no help to offer if the worst should happen. Wereproduce on p. 900 some of the messages and conclusionsfrom the I.P.P.N.W. Congress.

1 Editorial Doves in false garb Nature 1982; 295: 542.2 Meredith C. Doves in false garb. Nature 1982; 296: 386.3 Hartog M, Baumer JH, Fleming PJ, Hall MJ. Doves in false garb. Nature 1982, 296:

386.4 Britten S. Doves in false garb Nature 1982; 296: 386.5 Editorial. Professional propaganda. Nature 1982; 296: 380.

FIBRILLATION, FAILURE, AND THE FOXGLOVE

SINCE William Withering observed that extracts of

foxglove helped his patients with dropsy, controversy has, surrounded the use of digitalis in clinical practice. Thetherapeutic role of digitalis after myocardial infarction is

perhaps the most contentious. Does digitalis help post-infarction arrhythmias or does it increase that risk? Does

digitalis have any effect in cardiogenic shock? How muchdoes digitalis improve the haemodynamic performance of thefailing heart? These questions continue to be asked 70 yearsafter Herrickl recommended that digitalis should be given toall patients after a myocardial infarction.

,

On both experimental and clinical evidence, digitalis is

clearly the drug of choice in atrial arrhythmias such asfibrillation, flutter, and supraventricular tachycardias.2 Itseems to work partly by a direct effect on the function of thecontracting, myocardium3 and partly by an increase inatrioventricular block which slows the ventricular rate.4Despite the arrhythmogenic properties detectable in thelaboratory, digitalis does not seem to increase the incidence ofarrhythmias when given to patients with myocardialinfarction.2,5

Should digitalis be given in cardiogenic shock? In those fewpatients who have been studied, little or no improvement inleft ventricular performance has been achieved; and, in

animals, cardiac output sometimes falls after digitalis. Thereseems therefore no place for digitalis in the management ofcardiogenic shock except when there is an atrial arrhythmia.In clinically apparent acute left ventricular failure

complicating myocardial infarction, some small benefits havebeen seen. Digitalis increases the blood flow to the ischaemicarea6 and decreases the amount of ST elevation’ in dogs,increases myocardial contractility (both in laboratoryanimals8 and in human beings 9), and increases left ventricularejection fraction. Most work in man has shown a modestincrease in cardiac output and a small decrease in leftventricular filling pressure; 11 so digitalis may be of somebenefit in acute failure. The evidence for benefit of digitalis inchronic heart failure remains uncertain. There are risks with

digitalis toxicity, especially in the elderly,12 and some of theinitial benefits’of digitalis may be lost with time.’3 In patients

1. Herrick JB. Clinical features of sudden obstruction of the coronary arteries. JAMA1912; 59: 2015-20.

2. Rahimtoola SH, Gunnar RM. Digitalis in acute myocardial infarction: help or hazard?Ann Intern Med 1975; 82: 234-40.

3. Smith TW, Haber E. Digitalis (second of four parts). N Engl J Med 1973; 289:1010-15.

4. MacKenzie J. Digitalis. Heart 1911; 2: 273-386.5. Reicansky I, Conradson T-B, Holmberg S, Ryden L, Waldenstrom A, Wennerblom B.

The effect of intravenous digoxin on the occurrence of ventricular tachyarrhythmiasin acute myocardial infarction in man. Am Heart J 1976, 91: 705-11.

6. Watanabe T, Covell JW, Maroko PR, Braunwald E, Ross J. Effects of increased arterialpressure and positive inotropic agents on the severity of myocardial ischaemia in theacutely depressed heart. Am J Cardiol 1972; 30: 371-77.

7. Vatner SF, Baig H, Manders WT, Murray PA. Effects of a cardiac glycoside on regionalfunction, blood flow and electrograms in conscious dogs with myocardial ischaemia.Circ Res 1978; 43: 413-23.

8. Kumar R, Hood WB, Joison J, Gilmour DP, Norman JC, Abelmann WH.Experimental myocardial infarction. VI Efficacy and toxicity of digitalis in acuteand healing phase in intact conscious dogs. J Clin Invest 1970, 49: 358-64.

9. Rahimtoola SH, Sinno MZ, Chuquimia R, Loeb HS, Rosen KM, Gunnar RM. Effectsof ouabain on impaired left ventricular function in acute myocardial infarction NEngl J Med 1972; 287: 527-31

10. Morrison J, Coromilas J, Robbins M, Ong L, Eisenberg S, Stechel R, Zema M, ReiserP, Scherr L. Digitalis and myocardial infarction in man. Circulation 1980; 60: 8-16.

11 Marcus FI. Use of digitalis in acute myocardial infarction. Circulation 1980; 62: 17-1912. Smith TW. Digoxin toxicity: epidemiology and clinical use of serum concentration

measurements. Am J Med 1975; 58: 470-76.13. Guz A, McHaffie D The use of digitalis glycosides in sinus rhythm. Clin Sci Mol Med

1978; 55: 417-21.