Survival in scieroderma · Lung(abnormal chest xray) 17 Subcutaneous calcinosis 11 Trunkinvolvement 22 Telangiectasia 26 mean duration before a definitive diagnosis of scleroderma

Ann. rheum. Dis. (1971), 30, 581

Survival in scieroderma

R. BENNETT,* R. BLUESTONE,t P. J. L. HOLT,+ AND E. G. L. BYWATERS§Department of Medicine, Royal Postgraduate Medical School, and Hammersmith Hospital, London

Scleroderma is one of the commoner connectivetissue diseases, with a wide range of clinical ex-pression and considerable variation in prognosis. Assuch its initial diagnosis comes within the ambit ofmany specialties: the dermatologist because ofmorphoea, sclerodactly, or telangiectasia; thevascular surgeon because of Raynaud's pheno-menon; the rheumatologist because of a synovitis;the gastroenterologist because of dysphagia ormalabsorption; and the cardiologist because ofdyspnoea. This propensity for visceral involvementis now well recognized; since the original descriptionby Ehrmann (1903) of oesophageal involvement,most of the major organs have been recorded asaffected in varying degrees by a sclerosis of theirsupporting tissues.

This has led to the adoption of the more rationalname of 'systemic sclerosis'. In a disease with sucha wide variation in manifestations and involvementof viscera, it is not surprising that estimations of itsprognosis have varied considerably and have oftenbeen hindered by the semantics of classification.Attempts have been made to differentiate between aform of the disease characterized by predominantsclerodactly and Raynaud's phenomenon (acro-sclerosis) and a generalized form (O'Leary andWaisman, 1943; Truelove and Whyte, 1951), theformer being said to have a good prognosis and thelatter a poor prognosis. However, it has becomeapparent that many patients initially presentingwith acrosclerosis already have systemic involvementand that most of those who do not will subsequentlydevelop visceral changes. At the opposite extremesof the disease spectrum there is a general agreementabout the outcome. There are those patients withlocalized patches of thickened indurated skin(morphoea) who have an excellent prognosis andthose with the rare form, characterized by a rapidlyprogressing cutaneous sclerosis with multivisceralinvolvement, who have a uniformly poor prognosis(Tuffanelli and Winkelmann, 1961). However, themajority of patients seen in clinical practice fall intoa large 'middle-group' where a universally acceptable

classification is lacking and the natural course of thedisease is varied and uncertain. The clinician, ondiagnosing scleroderma, has little data on its courseand the factors influencing it to which he can referfor guidance. The lack of such information is partlythe result of the problems inherent in the follow up ofany chronic disease. These can be largely overcomeby analysing the data by 'Life Table' methods. Usingthis approach, we have attempted to provide anestimate of the prognosis in scleroderma and alsoto evaluate those factors influencing survival whenthe patients are first seen.

Material and methodsBetween 1947 and 1970, 67 patients have been seen atHammersmith Hospital with a diagnosis of systemicsclerosis. The criteria for inclusion in this survey hasbeen either typical skin changes, or Raynaud's pheno-menon with a characteristic oesophageal abnormality,as seen radiologically in patients with a normal skin.Only two patients came into this latter category: one ofthese has now been followed up for 13 years and is justbeginning to develop cutaneous sclerosis around themouth; the other patient has been followed up for 18months. Those with morphoea were specifically excluded.Of the 67 patients in the survey, 26 are known to be

dead, 31 have been followed up to the present time, andten could not be traced (Fig. 1 overleaf ).The following data were extracted from their medical

records: date of definitive diagnosis; date last observed;whether alive or dead; features at time of initial diagnosis(usually during the first hospital admission): blood urea,electrocardiogram, oesophageal involvement, radiologicalpulmonary involvement, calcinosis, telangiectasia, anderythrocyte sedimentation rate (Westergren); durationof Raynaud's phenomenon before diagnosis; presence orabsence of sclerodermatous trunk involvement (notincluding the neck).The prognosis has been expressed in terms of the

percentage survival from the time of initial diagnosis.These survivorship figures have been calculated by themethod of Life Table analysis (Merrell and Shulman,1955). The standard error of survivorship was calculatedas described by Greenwood (1926).The features at the time of initial diagnosis which

Given at a meeting of the Heberden Society on March 19, 1971.Accepted for publication May 21, 1971.* Registrar and tutor in medicine.t Now Head of Rheumatology Division, Wadsworth Hospital, Los Angeles.$ Consultant and lecturer in medicine.§ Professor of Rheumatology, University of London.

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582 Annals of the Rheumatic Diseases

68-64-bo-56 -52 -48 -44 -40-3632282420l 61284

C] Dying at 'x' yrs_ Leaving survey at 'x' yrs

UntraceableComplete follow-up

I, 2 Number still in survey at 'x' yrs

0 _0 2 4 6 8 10 12 14 16 18 20 22 24Years after diagnosis (x)

FIG. 1 Basic data for life tables.

might influence prognosis were studied by comparingtheir incidence in two groups: those dying in 6 years fromdiagnosis and those surviving more than 6 years.* Thesignificance of the frequency distribution of these featureswas assessed by conventional statistical methods. Certainfeatures, namely hypertension, uraemia, heart disease,and radiological lung disease, are usually considered tobe associated with an increased morbidity irrespective ofthe primary disease. In these features the level of signifi-cance was expressed from a 'one-tailed' distribution. Inall other cases the level of significance was based on a'two-tailed' distribution.

ResultsThe mean age at diagnosis of our patients was 46 a 2years (S.D. 15'6). There were 56 females and 11males. Evidence of visceral involvement was foundin 86 per cent. of patients at the time of initialdiagnosis and its distribution by our criteria (whichin some cases will tend to underestimate its incidence)is seen in Table I. Raynaud's phenomenon was apresenting symptom in 70 per cent. of patients; its

Table I Organ involvemenit at diagnosis in 67 patients

Organ Number ofpatients

Oesophagus 35Small bowel 10Heart

(abnormal electrocardiogram) 28Kidney (abnormal blood urea) 9Lung (abnormal chest x ray) 17Subcutaneous calcinosis 11Trunk involvement 22Telangiectasia 26

mean duration before a definitive diagnosis ofscleroderma was made being 53 years (S.D. 7-5).

PROGNOSISOn the basis of the Life Table analysis, the overallprognosis (Fig. 2) in this series is a 73 per cent.5-year survival (S.E. 7 5) and a 50 per cent. 10-yearsurvival (S.E. 8 5). Beyond the 10-year point theconventional statistical limits of + 2 Standard Errors(S.E.) are seen to diverge markedly-a result of thediminishing numbers in our survey beyond this point.

100

90

80

' 70

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survivorship from diagnosisstandard error limits

5-yr survival 730/oa10-yr survival 50%/oI~~~~ ~I .II.. . . .I I . . . . a

2 4 6 8 10 12 14 16 18 20 22 24Years from diagnosis

FIG. 2 Survivorship.

When our patients are divided into two age groups(Fig. 3), those under 40 years at diagnosis and those40 years and over, it is seen that there is a markedand significant difference in survival. Those over40 years have a 50 per cent. 5-year survival (S.D. 10)and a 30 per cent. 10-year survival (S.E. 10), whilst

100

90

80

- 70-a

> 60

50~

e 40~

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20

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age (yrs) survivalunder 40 5-yr

10-yr"^ over 40 5-yr

10-yr

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2 4 6 8Years from diagnosis

percentage95705030

10 12 14 lb

* The explanation for a 6-year period is given tinder 'Results'.

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F I G. 3 Effect of age on survival.



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Survival in scieroderma 583

those under 40 years have a 95 per cent. 5-yearsurvival (S.E. 7) and a 70 per cent. 10-year survival(S.E. 16).On examination of the survival curves in these

two age groups, it is seen that they have a similarslope except for the initial 6 years, when in the over40 age group there is a sharp fall in survival. Indeed,of all the recorded deaths, 73 per cent. died in thefirst 6-year period from diagnosis (Fig. 4). Thus itappears that there is a group of patients in the over40 age group who rapidly succumb to the disease.

Total number of deaths 26(380/o of total survey)

730/a died in first 6 yrsI 270/o died in remaining 18 yrs

24

20

12

8

4

I- I1 2 3 4 5 6 7 8 9 lo11 12 14151117 181920 22 24

Years from diagnosisFIG. 4 Distribution ofdeaths.

This finding is most easily explained by postulatingthat this group contained those patients in the moreelderly range and they would thus have been expectedto have a considerably reduced life expectancy.This, however, does not fully explain the age dis-tribution in the two groups (Fig. 5): the mean ageat diagnosis of those dying in 6 years from diagnosis

being 55 years (S.D. 12 2) and of those survivingmore than 6 years being 42 years (S.D. 14-7). Thisdistribution suggests other features, in addition tothe age related factor as contributing to a poorerprognosis in those aged 40 or over.

FACTORS AFFECTING SURVIVAL (Figs 6 and 7)Those features at initial diagnosis which may con-tribute towards a group with a poor prognosis in the

Pulmonary involvement P40m-./lOOm-. P07

Telangiectasis

Raynaud' s < 1 yr

20 1 MeSto

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Age at diagnosis (yrs)5 Age incidence and survival.

B. P > 150 mm. Hg90mm

Sex incidence inthosedying within 6yrs

M20

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_1Mle_ Dying in 6 yrs

Surviving 6yrs

0 10 20 30 40 50 60 70 80 90 100Percentage in each group

F IG. 7 Factors not apparently affecting prognosis.

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over 40 age group, have been evaluated by comparingtheir incidence in those patients dying in 6 yearsfrom diagnosis with those surviving more than 6years (Figs 6 and 7). A 6-year point was found moresuitable than a 5-year point in this particular study,as 73 per cent. of those dying did so in 6 years asagainst 50 per cent. in 5 years. It is seen that a bloodurea over 40 mg./100 ml. and trunk involvementboth have an adverse effect on survival (P < 0 005).An abnormal electrocardiogram is common in both

groups but significantly so in those dying in 6 years(P < 0 * 05). Lung involvement is not so common butis seen to be of significance (P < 0 05). Factors notindicative of a poor prognosis are an erythrocytesedimentation rate of over 25 mm./lst hr, oeso-phageal involvement, telangiectasia, Raynaud'sphenomenon of less than 1 year, blood pressure over150/90 mm. Hg, and the patient's sex. However,this is not to say that these factors will not playsome part in influencing the survival after the initial

Table II Causes of death and post mortem findings

Case No. Cause ofdeath

130128 Intestinalobstruction

121411 Myocardialinfarction

49716 Bilateral lobarpneumonia

105166 Pulmonaryoedema

41289 Congestive cardiacfailure. (Nopost mortem)

339257 Cancer lung




182737 Peritonitis

246648 Anaemia


291877 Aspirationpneumonia

161898 Peritonitis

Heart

LVH and RVH

Pericarditis

Perocardialeffusions

Myocardialsclerosis

Myocardialsclerosis

Rheumatic heartdisease-mitraland tricuspidvalves

Myocardial andendocardialfibrosis

Pericardial effusionand LVH

Pericardialeffusion

Lung Gut

Left Faecal impactionbronchopneumonia and oesophageal

involvement

Cancer stomach

Basal lobarpneumonia

Bronchitis andpulmonaryoedema

Cancer lung

Pulmonaryoedema

Other

Generalizedsclerodenna

Oesophagus tocolon involved

Oesophagus,stomach, smalland large bowel

Pulmonary Benign gastricoedema ulcer

Ulceration ofcaecum andduodenaldilatation

Oedema

Fibrosis rightlower lobe

Chronicbronchitis

Small thyroid

Kidneys enlargedwith superficialpunctatehaemorrhages

Duodenaldilatation

Cancer stomachwith perforations



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Survival in scleroderma 585

6-year period from diagnosis. In fact, some of themmay be of importance in determining the relativelysimilar slopes of the survival curves after the first 6years.

CAUSES OF DEATHThe causes of death (Table I) are known in fourteenof the patients who died, and of these eleven hadautopsies.* In seven patients the cause of death wasdirectly attributable to cardiac causes and in nineof the eleven patients coming to autopsy the heartwas abnormal. In four of these there was evidence ofpericarditis, a feature seldom diagnosed during lifein patients with scleroderma. Two patients hadcancer of the stomach and one carcinoma of thebronchus. The high incidence of electrocardiogramabnormalities is analysed (Table III) and it is seenthat the changes are mainly of a non-specific nature.

Tablem Electrocardiogram abnormalitiesAbnormality Number ofpatiensAtrial fibrillation 4Flat 'T' waves 12ST depression 5Low voltage 5RBBB 3LBBB 1LVH 5RVH 4

EFFECT OF TREATMENTThe only drugs used often and long enough to assesstheir effect on survival were the corticosteroids.During the course of the 23 years covered by thissurvey, eighteen patients were given corticosteroidsfor varying lengths of time; all took them for atleast 1 year and our longest survivor for 23 years.They had no apparent effect on survival, eitherfavourable or unfavourable. Other forms of clinicallyineffective treatment used in a few patients included,epsilon-aminocaproic acid, relaxin, potassium para-aminobenzoate, penicillamine, and sympathectomy.

DiscussionScleroderma is a chronic disease in which progressionwill be variable in different patients and also in thecourse of the individual patient. Thus the prognosiswill be related to the hazards of ageing, the presenceof other diseases, host resistance, environmentalchanges, and in some cases the efficacy and side-effects of treatment. With so many variables, anyestimate of prognosis must be viewed critically,especially as regards selection of patients. Somepatients will have had their disease for varyinglengths of time before consulting a doctor, othersmay not have considered themselves ill enough to* Several patients (all Caucasians) came from overseas and later diedwithout an autopsy being performed.

need medical help, and some will have died of othercauses. However, similar selective processes operatein all hospital populations and although ourestimates of prognosis will tend to err on the sideof a reduced survival, they are ofvalue in the contextof patients in one's own clinic, in comparing resultsfrom different clinics, and as a possible guide forlarge-scale prospective studies.The usually estimated 5-year survival rate,

obtained by dividing the number of patients knownto be alive at 5 years by the number originallydiagnosed, inevitably tends to underestimate thesurvival as it does not take into account thosepatients lost to follow-up in the 5-year period, thosewho have died, and those still alive but not havingcompleted a 5-year follow-up. The analysis ofsurvival data by 'Life Table' methods makesappropriate adjustments for these possible sources oferror.Our survival figures for scleroderma are the first

to be based on Life Table methods. The majorityof studies point to a poor prognosis in most patientswhile conceding that some individuals can survivefor many years (Farmer, Gifford, and Hines, 1960;Orabona and Albano, 1958; Masi and D'Angelo,1967). However, our overall survival figures agreeclosely with those of Tuffanelli and Winkelmann(1961), but their paper did not analyse the markedeffect of age on survival demonstrated by ourfindings.The features found at initial diagnosis which

suggest an adverse prognosis (Fig. 6) are thoserepresenting involvement of important viscera, withthe exception of trunk involvement. This latterassociation with poor prognosis is probably due toan association with more extensive and rapid visceralinvolvement. Farmer and others (1961) found thatcardiac involvement, renal involvement, oesophagealinvolvement, and an erythrocyte sedimentation rateover 50 mm./lst hr were associated with poorerprognosis, but that pulmonary involvement was oflittle prognostic significance. As in our study, theyfound that sex, the duration of Raynaud's pheno-menon, calcinosis, and telangiectasia were of noprognostic import. However, theymadeno allowancefor variations in the course of the disease in that theycompared features in two groups: those living andthose dead. This probably accounts for differencesbetween our two studies.The absence of any significant relationship

between a raised blood pressure (> 150/90 mm. Hg)and a poor prognosis is rather surprising. Of thepatients with a much higher BP at diagnosis, one(200/120 mm. Hg) died within one year but another(210/120 mm. Hg) died after 13 years and 3 more(175/105, 180/100, and 210/100 mm. Hg) are stillliving at 16, 2, and 4 years respectively after diagnosis.



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Repeated minor episodes of inhalation pneumoniadue to oesophageal involvement can lead to lunginvolvement. Thirteen of our seventeen patients withradiological lung involvement also had oesophagealchanges, suggesting an association. But, on theother hand, of 35 patients with oesophageal in-volvement, only thirteen had radiological pulmonaryinvolvement. Of the seventeen patients with anabnormal chest x ray, eleven had defects in 'pul-monary diffusion' as measured by the single-breathcarbon monoxide method. However, ten of thesepatients also had oesophageal involvement. As thetransfer factor for carbon monoxide can also below in ventilation/perfusion disturbances as well asin 'alveolar capillary block', these changes could becompatible with secondary lung involvement ratherthan a primary interstitial sclerosis. Further studiesare indicated to elucidate this point. The remainingsix patients did not have pulmonary function testsperformed. But two patients with normal radio-graphs had abnormalities of carbon monoxidetransfer, a finding consistent with early parenchymallung involvement and indicative of a probableunderestimation of total lung involvement if onlypatients with abnormal radiographs are included(Godfrey, Bluestone, and Higgs, 1968). UnlikeFarmer and others (1961) we found that pulmonaryinvolvement at initial diagnosis had some bearingon prognosis.As one of the commonest causes of death in

scleroderma is cardiac failure (Table II), it is notsurprising that electrocardiogram abnormalities areassociated with a poor prognosis. Our overallincidence of abnormal electrocardiograms at initialdiagnosis is 51 per cent. On analysis (Table III) nocharacteristic changes particularly diagnostic ofscleroderma have emerged. Most of the commonfindings (low voltage, 'T' wave flattening, and 'ST'segment changes) are fairly non-specific and probablyrepresent sclerotic involvement of the myocardiumor of the pericardium as seen in some of the postmortem material (Table II). More specifically, theoccurrence of left and right ventricular hypertrophywas associated with hypertension and pulmonaryinvolvement respectively. Some of the patientsdying in cardiac failure had predominantly leftventricular disease caused by hypertension, possiblyexacerbated by myocardial sclerosis. Similarly,pulmonary involvement is associated with an adverseprognosis, and it is logical to assume that its presencehelps to tip the balance in some patients on the vergeof right ventricular failure caused in some instancesby myocardial sclerosis.

It is interesting to note that, although the presenceof renal involvement was of high prognostic signifi-cance, none of our patients for whom the cause ofdeath is known died of renal failure. It may be that

renal involvement, like trunk involvement, is onlypresent when there is already extensive visceralinvolvement elsewhere. It is well recognized thatsome patients die of a rapidly progressive malignanthypertension and that this is usually associated withrenal involvement.

Of the ten patients known to have small bowelinvolvement radiologically (mainly duodenal dila-tation), four died in 6 years. However, only a smallproportion of those surveyed had specific reports onsmall bowel radiographs and thus this feature hasnot been included in our analysis of significantprognostic factors. Some patients rapidly becomeemaciated and do badly, partly through fear ofeating too much, because of oesophageal involve-ment, and partly as a result of malabsorption dueto small bowel involvement. However, Bluestone,MacMahon, and Dawson (1968) found that smallbowel involvement was relatively common and notusually associated with malabsorption. Obviouslya long-term prospective study is needed to clarifythis point.

Some have considered that patients presentingwith acrosclerosis have a good prognosis (O'Learyand Waisman, 1943). Thus it might be expected thatthe duration of Raynaud's phenomenon beforeobvious cutaneous sclerosis would affect survival.However, this is not so and it is now clear thatacrosclerosis does not preclude progressive systemicinvolvement, rather it is a common mode ofpresenta-tion and the course is similar to that of other varietiesof the disease. Particularly interesting in our studywas that the mean duration of Raynaud's pheno-menon belfore a definite diagnosis of sclerodermacould be made was 5 * 3 years (S.D. 7 * 5). Hence, ifa patient presents with Raynaud's phenomenon,one will in some cases have to follow him up for20 years before one can say with 95 per cent.certainty that he will not develop scleroderma.

In the course of the 23 years covered by thissurvey, many different forms of treatment have beentried, including epsilon-aminocaproic acid, relaxin,potassium para-aminobenzoate, penicillamine, andsympathectomy. The only therapy used long enoughto warrant consideration as being of possible valuewas corticosteroids. The patients who receivedsteroids naturally comprised a highly selected groupand thus it is not possible to evaluate the preciseeffect of steroids in every type of patient. However,in our particular group, steroids did not seem toaffect survival. Indeed, when one is dealing with adisease with an overall 5-year survival of 73 per cent.and a 90 per cent. 5-year survival in the under40 age group, any therapy will have to exhibit adramatic effect before it can be regarded as fullysuccessful.



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Survival in scleroderma 587

SummaryWe have attempted to evaluate, for the first time,using 'Life Table' methods, the survival and factorsinfluencing prognosis in scleroderma. The prognosisis very good, with a 73 per cent. 5-year survivaloverall and a 90 per cent. 5-year survival in the under40 age group. Of those patients dying, 73 per cent.do so in the first 6 years. The features found atinitial diagnosis which predispose towards an earlydeath are trunk involvement, blood urea over 40mg./100 ml., electrocardiogram abnormalities, radio-logical pulmonary involvement, and age over 40years.

We should like to thank Prof. C. V. Harrison's Depart-ment of Pathology for performing the autopsies, Dr D. K.Peters for the generous use of an electronic calculator,and the many people who, over the years, have helped toinvestigate, document, and manage these patients.

DISCUSSIONDR. E. N. GLICK (London) Have you any informationon the influence of treatment, particularly steroids orACTH, on survival?

DR. BENNETT We did look at the effect of treatmentin our series. The only group of drugs used often andlong enough to assess their effect on survival werecorticosteroids. Eighteen patients took them for at leastone year and our longest survivor for 23 years. They werefound to have no apparent effect on survival, eitherfavourable or unfavourable. Other forms of treatmentused and found to be clinically ineffective were epsilon-aminocaproic acid, relaxin, potassium para-amino-benzoate, penicillamine, and sympathectomy. When oneis dealing with a disease with a 5-year survival of 73 percent. and a 90 per cent. 5-year survival under the age of40, any therapy producing a further significant effect onsurvival will be difficult to confirm.

DR. M. WILKINSON (Perth) Where does morphoea endand scleroderma begin? Is it the size, or the distributionof the lesion? How did you exclude morphoea?

DR. BENNETT Morphoea was taken as a sharplylocalized plaque or hard sclerotic skin which ended up asa depressed atrophic area. The important differentiatingfeatures was its obvious border and its nonprogressivenature.

DR. W. W. BUCHANAN (Glasgow) One of the fallaciesin cohort prospective studies, described by Neyman(1955), is that, if one starts the study after the disease has

been present for some time, one may miss patients whohave already died. Thus, if the study is started 5 yearsafter the onset of the disease and there is a high mortalityin the first 5 years, this observation will be missed. Didyou attempt to analyse your results in patients withdisease of only 1 or 2 years duration?

DR. BENNETT I think this criticism is perfectly valid.It can, of course, be levelled at any retrospective study ofhospital patients and we realize that our survival figuresstrictly apply only to our own selected group of patients.However, one hopes that these figures will be of use incomparing results from other centres and a guidelinefor any future long-term prospective study. As for thequestion whether the study is started from the time ofdiagnosis or a predated starting point; though thetemptation is great to attempt the latter, this is ofnecessity dependent upon usually vague early subjectivesymptoms, which inevitably lead to a fictitious result.

DR. F. DUDLEY HART (London) Although this is calledprogressive systemic sclerosis, did any remit, and for howlong?

DR. BENNETT The natural progression of the disease is,of course, one of intermittent relapses and remissions.Some of our patients had apparent remissions lastingseveral years, but none has shown a complete regressionof the disease.

DR. J. T. SCOTT (London) In a previous study in theU.S.A. a low haemoglobin was found to be related to badprognosis. What were your findings?

DR. BENNETT In a pilot study we looked at the initialhaemoglobin readings and found them to have nosignificant effect on subsequent survival. I think the studyyou are quoting (Farmer, Gifford, and Hines, 1961) didnot specifically look at this feature at the time of initialdiagnosis and this probably accounts for the discrepancy.

DR. D. PITKEATHLY (Manchester Region) One impor-tant feature in the prognosis may be the acuteness of onsetof the systemic sclerosis. One sees patients with veryswollen hands, who later develop tight shiny skin andclawed fingers.

DR. BENNETT We did not specifically look at theacuteness from the point of view of the skin involvement.But we did look at the effect of a raised ESR, whichFarmer and others (1961) found augered an adverseprognosis. However, we found that a high ESR at initialdiagnosis did not predispose towards a poor prognosis.ReferencesFARMER, R. G., GIFFORD, R. W., AND HiNEs, B. E. (1961) Circulation,

21, 1088.NEYMAN, J. (1955) Science, 122, 401 (Statistics-servant of all sciences).

ReferencesBLUESTONE, R., MACMAHON, M., AND DAWSON, J. M. (1969) Gut, 10, 185 (Systemic sclerosis and small bowel

involvement).CULLINAN, E. R., AND HARPER, R. A. K. (1907) Proc. roy. Soc. Med., 46, 507.EHRMANN, S. (1903) Wien. med. Wschr., 53, 1097 (Uber die Beziehung der Sklerodermie zu den Autoxischen).



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FARMmR, R. G., Giroiw, R. W., AND HINES, E. A. (1960) Circulation, 21,1088 (Prognostic significnce ofRaynaud's phenomenon and other clinical charcteristics of systemic scieroderma. A study of 271 cases).

GODEY, S., BLuSTONE, R., AND HIGGs, B. E. (1969) Thorax, 24, 427 (Lung function and the response to exercisein systemic sclerosis).

GREENWOOD, M. (1926) Reports on Public Health and Statistical Subjects, No. 33, Appendix I, p. 23. HMSO,London.

MAsI, A. T., AmD D'ANGELO, W. A. (1967) Ann. intern. Med., 66, 870 (Epidemiology in fatal systemic sclerosis).MERRELL, M., Am SHULAN, L. E. (1955) J. chron. Dis., 1, 12 (Determination of prognosis in chronic disease,

illustrated by systemic lupus erythematosus).O'LEARY, P. A., AND WAsmN, M. (1943) Arch. Derm. Syph. (Chicago), 47, 382 (Acrosclerosis).ORABONA, M. L., AND ALBANO, 0. (1958) Acta med. scand., Suppl. 333, p. 5. (Systemic progressive sclerosis (or

visceral scleroderma)).TRUELOVE, S. C., AND Wirvm, H. M. (1951) Brit. med. J., 2, 873 (Acrosclerosis).TUFFANELLI, D. L., AND WINKELMANN, R. K. (1961) Arch. Derm. (Chicago), 84, 359 (Systemic scleroderma).



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Documents

Survival in scieroderma · Lung(abnormal chest xray) 17 Subcutaneous calcinosis 11 Trunkinvolvement 22 Telangiectasia 26 mean duration before a definitive diagnosis of scleroderma