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156 JOURNAL OF THE FACULTY OF RADIOLOGISTS Ankylosing Spondylitis" Results of Treatment, and their Presentation DANIEL O'CONNELL, M.D., F.F.R. Charing Cross Hospital, London, W.C.2 THIS paper presents the results of treatment of a series of 8 7 cases of ankylosing spondylitis at the Radio- therapy Department of Charing Cross Hospital. Because ankylosing spondylitis is a slowly-developing condition, medical advice is not generally sought, nor is the diagnosis made, until it has been present for some years (average 3½ years). The morbid condition in the affected joints or attachments, pannus, bone erosion, calcification of ligaments and loss of move- ment, have been established in varying degree, and hence it is not surprising that the more usual palliative and supportive measures are ineffective. It is in the natural history of the disease to 'burn itself out', but not usually until an advanced stage when a large part of the spine and possibly other joints have become ankylosed. In the treatment of the condition three types of relief are possible: First, the early mild case may be aborted completely. Secondly , in the severe type of case, the time of 'burning out' may be brought -forward and the disease halted at the state it has reached--one cannot expect decalcification of calcified ligaments or freeing of ankylosed joints, but all symptoms may be abolished. Thirdly, temporary r~lief or diminution of symptoms may be achieved. In a small number of cases the course of the disease is unaffected by the treatment, 6 out of 8 7 in this series. Whilst radiotherapy does institute an immediate remission in the great majority of cases, unless supportive measures are applied the maximum length of remission may not be obtained. Though a complete cure may be achieved by radiotherapy alone, these are difficult to select beforehand, and it is of importance, therefore, that ancillary treatments be employed. It is difficult to interpret the response to treatment of ankylosing spondylitis, as this is expressed mainly by the subjective opinion of the patient. Measurement of increases of moment are of limited value where there is bony ankylosis. The clinical appearance is of confirmatory value in the severe forms of the disease, but X-ray evidence is of little value except over long periods and even then it is only indicative of gross changes. The single objective test of real value is the Erythrocyte Sedimentation Rate. It reflects the severity of disease and the response to treatment to a reasonable degree of accuracy, especially when expressed in graph form. The presentation of results in the great majority of papers gives no standard by which one can compare response to treatment in different hospitals. It is hoped that the following method may appeal, if only because of its simplicity. Improvement in the series has been assessed by first grading each patient into one of three categories of severity of diseases, then registering the type of response in one of four groups. Following are the three degrees of severity of the disease (see Table 1):-- A. Active disease present: There is no evidence of systemic disease; the symptoms are mild and confined to the lower spine and pelvis. B. Severe active disease present: There is evi- dence of mild systemic disease, and the symptoms are severe and widespread. Immediate treatment" is necessary. C. Acute systemic disease present : There is evidence of severe systemic disease and admission as an in-patient is necessary. The results of treatment are based on the degree of improvement as classified below (see Table II) :~ i. Complete recovery of reasonable range of move- ment and loss of symptoms: 'Reasonable range of movement' is interpreted as indicating movements not limited by pain or tenderness "but by bony ankylosis. For instance, a patient may not be able to touch his toes, but this will be due only to bony ankylosis of the lurnbodorsal spine and not to muscle spasm. There will be no pain on flexing the spine to the limits of its movements. 2. Disease inactive : This indicates that the disease is quiescent but allows of occasional mild' rheumatics' which last not more than one day. The essential point is that the attacks are infrequent, transient, and mild. The patient does not require further treatment and the disease is not progressing. The patient may remain in the group indefinitely. 3. Further treatment necessary: The disease has recurred and further treatment is necessary. 4. Failure to halt the disease: The treatment has not affected the course of the disease, which may, indeed, have become worse. In a certain proportion of cases involvement of peripheral joints and muscle attachments appeared during or shortly after the spinal treatment. These were mild in all cases and the symptoms were abolished with a single treatment with X-rays. It was not considered justifiable in any case to call this an interruption of a remission. The Short-term Results.-- I. The 87 patients given treatment fell into three categories of severity shown in Table I. 2. Table 1I indicates the different results in each grade one year after treatment. 3. Table 11I combines Tables I and H and shows how cases of varying degrees of severity fared with treatment. Table I.--SEv~RIT¥ oF DISEASE -4. Active disease present ~o B. Severeactive disease present 55 C. Acute systemic disease present 12 Total 877

Ankylosing spondylitis: Results of treatment, and their presentation

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156 J O U R N A L OF T H E F A C U L T Y OF R A D I O L O G I S T S

Ankylosing Spondylitis" Results of Treatment, and their Presentation

D A N I E L O 'CONNELL, M.D., F.F.R.

Charing Cross Hospital, London, W.C.2

THIS paper presen ts the results of t r e a t m e n t of a series of 8 7 cases of ankylosing spondyl i t i s at the Radio- the rapy D e p a r t m e n t of Cha r i ng Cross Hospi ta l . Because ankylosing spondyl i t i s is a s lowly-developing condi t ion, medica l advice is no t general ly sought , no r is the diagnosis made, unt i l it has been presen t for some years (average 3½ years). T h e m o r b i d condi t ion in the affected jo in ts or a t tachments , pannus , bone erosion, calcification of l igaments and loss of move- ment , have been es tabl ished in vary ing degree, and h e n c e it is no t surpr i s ing tha t the more usual pall iat ive and suppor t ive measures are ineffective. I t is in the natural his to ry of the disease to ' b u r n itself ou t ' , b u t no t usually un t i l an advanced stage w h e n a large pa r t of the spine and possibly o ther jo in ts have become ankylosed.

In the t r e a t m e n t of the condi t ion three types of relief are poss ible : First , the early mi ld case may be abor ted completely. Secondly , in the severe type of case, the t ime of ' b u r n i n g o u t ' m ay be b r o u g h t

-forward and the disease ha l ted at the state i t has r e a c h e d - - o n e cannot expect decalcification of calcified l igaments or f reeing of ankylosed joints, bu t all symptoms m a y be abolished. Th i rd ly , t empora ry r~lief or d i m i n u t i o n of symptoms m a y be achieved. I n a small n u m b e r of cases the course of the disease is unaffected b y the t r ea tment , 6 ou t of 8 7 in this series.

Whi l s t r ad io the rapy does ins t i tu te an immedia te remiss ion in the great major i ty of cases, unless suppor t ive measures are appl ied the m a x i m u m length of remiss ion m a y n o t be obta ined. T h o u g h a complete cure m a y be achieved by rad io therapy alone, these are difficult to select be forehand , and it is of impor tance , therefore , tha t anci l lary t r ea tments be employed.

I t is difficult to in t e rp re t the response to t r e a t m e n t of ankylosing spondyli t is , as this is expressed ma in ly b y the subject ive op in ion of the pat ient . M e a s u r e m e n t of increases of m o m e n t are of l imi ted value where there is b o n y ankylosis. T h e clinical appearance is of conf i rmatory value in the severe forms of the disease, bu t X - r a y evidence is of li t t le value except over long periods and even t h e n it is only indicat ive of gross changes. T h e single object ive tes t of real value is the Ery throcy te Sed imen ta t ion Rate. I t reflects the severi ty of disease and the response to t r ea tmen t to a reasonable degree of accuracy, especially w h e n expressed in g raph form.

T h e p resen ta t ion of results i n the great major i ty of papers gives no s t andard by wh ich one can compare response to t r e a t m e n t in different hospitals. I t is hoped t ha t the fol lowing m e t h o d m a y appeal, if only because of its simplicity.

I m p r o v e m e n t in the series has been assessed by first g rad ing each pa t i en t in to one of th ree categories of severi ty of diseases, t h e n regis te r ing the type of response in one of four groups.

Fol lowing are the th ree degrees of severi ty of the disease (see Table 1 ) : - -

A . Act ive disease present: T h e r e is no evidence of systemic disease; the symptoms are mi ld and conf ined to the lower spine and pelvis.

B. Severe active disease present: T h e r e is evi- dence of mi ld sys temic disease, and the symptoms are severe and widespread. Immedia t e t rea tment" is necessary.

C. Acute systemic disease present : T h e r e is ev idence of severe sys temic disease and admiss ion as an in -pa t i en t is necessary.

T h e resul ts of t r e a t m e n t are based on the degree of i m p r o v e m e n t as classified below (see Table I I ) : ~

i . Complete recovery of reasonable range of move- ment and loss of symptoms: 'Reasonab le range of m o v e m e n t ' is i n t e rp re t ed as ind ica t ing m o v e m e n t s no t l imi ted by pa in or tenderness "but by b o n y ankylosis. Fo r instance, a pa t i en t may no t be able to touch his toes, b u t this will be due only to bony ankylosis of the lurnbodorsa l spine and no t to musc le spasm. T h e r e will be no pa in on flexing the spine to the l imi ts of its movemen t s .

2. Disease inactive : T h i s indicates t ha t the disease is quiescent bu t allows of occasional m i l d ' r h e u m a t i c s ' wh ich last no t more t han one day. T h e essential po in t is t ha t the attacks are in f requent , t rans ient , and mild. T h e pa t i en t does no t requi re fu r the r t r e a t m e n t and the disease is no t progressing. T h e pa t ien t m a y remain in the g roup indefinitely.

3. Further treatment necessary: T h e disease has recur red and f u r t h e r t r e a tmen t is necessary.

4. Failure to halt the disease: T h e t r ea tmen t has no t affected the course of the disease, wh ich may, indeed, have become worse.

In a cer ta in p ropor t i on of cases invo lvemen t of per iphera l jo in t s and muscle a t t achments appeared du r ing or shor t ly af ter the spinal t rea tment . T h e s e were m i l d in all cases and the symptoms were abol ished wi th a single t r e a tmen t wi th X-rays . I t was no t cons idered just i f iable in any case to call this an i n t e r rup t i on of a remission.

T h e S h o r t - t e r m R e s u l t s . - - I . T h e 87 pa t ien ts g iven t r ea tmen t fell in to th ree

categories of sever i ty s h o w n in Table I. 2. Table 1 I indicates the different results in each

grade one year after t r ea tment . 3. Table 11I combines Tables I and H and shows

how cases of vary ing degrees of severi ty fared w i t h t r ea tment .

Table I.--SEv~RIT¥ oF DISEASE -4. Active disease present ~o B. Severe active disease present 55 C. Acute systemic disease present 12

Total 877

A N I ( Y L O S I N G

Table / / . - - T Y P E OF RESPONSE TO TREATMENT AFTER 3 M O N T H S ' INTERVAL

I. Complete recovery 44 2. Disease inactive 31 3- Fur the r t reatment necessary 6 4- Fai lure to haIt the disease 6

T o t a l 87

T a b l e I l L - - R E s P O N S E TO TREATMENT IN EACH DEGREE OF SEVERITY OF DISEASE

Degree of Severity A B C Total

I, CompIete recovery 17 2,4 3 44 2. Disease inactive 2 z4 5 31 3- Fur ther t reatment necessary o 6 o 6 4. Fai lure to halt the disease • I 4 6

To ta l zo 55 I2 87

The Long-term R e s u l t s . - - T h e s e are expressed in length of remission. I have def ined a remiss ion as the per iod fol lowing t r e a t m e n t d u r i n g w h i c h the disease is at bes t non-ex i s t en t or at least inactive. I n o ther words, as long as the pa t ien ts of Grade I or 2 r emain in those grades and do not d rop to Grade 3, they are said to be in remission. Once they reach a stage r equ i r ing f u r t he r t r e a t m e n t for the i r disease, t hen the i r remiss ion is said to be at an end. As the pat ients of this series have b e e n fol lowed u p at th ree - or s i x -mon th ly intervals, any significant s y m p t o m or sign was not iced w i t hou t delay.

F r o m Tables 1-111 i t will be seen t ha t the mi ldes t cases (Category A) general ly r e spond well to radio- the rapy and have an excellent prognosis . Of 18 cases, 15 appeared to be still s y m p t o m - f r e e 5 years af ter- wards. Of the severer cases (Category B) 3 ° out of 50 were apparen t ly cured, b u t the r e m a i n d e r recur red in the in t e rven ing years. In Category C, 12 very severely affected pat ients all h ad shor t r e m i s s i o n s - - the longest be ing 2{ years.

T h e r e were 74 cases available for assessment 5 years after t r ea tment . Table I V shows the compar i son be tween the n u m b e r s of pat ients w i th remiss ion last ing one year and five years after t r ea tment .

T a b l e I V . - - S H o w s NUMBERS OF PATIENTS W I T H REMISSIONS OF 3 M O N T H S AND 5 YEARS AFTER TREATMENT

Total No. 2Vumbers in Remission after Treatment of Cases I and 5 years previously

3 months 5 years 74 63 (87 per cent) 36 (48 per cent)

Form of Treatment.--In our pract ice the ma jo r par t of the t r e a t m e n t is w i t h deep X- i r r ad ia t ion s u p p l e m e n t e d b y phys io the rapy and psychotherapy . In f r a - r ed and d i a the rmy i r radia t ion he tp to relieve spasm, and passive and active m o v e m e n t s and exercises ma in t a in muscle power. M a i n t e n a n c e of a p roper pos ture is of impor tance .

As some 3o per cent of all cases of ahkylosing spondyli t is suffer f rom a depressive neurosis , and often p resen t as such i n d e p e n d e n t l y before the onset of the disease, it is t h o u g h t to be of some impor t ance to ma in t a in a f r equen t wa tch on these patients.

S P O N D Y L I T I S 157

N u m b e r s show tha t these depressives figure largely among the r ecu r r en t cases; there were 26 depressive cases a l together in the series of 87, and it is cons idered essential for t h e m to do rout ine physical exercises n igh t and morn ing . T h e r e are two reasons for insist- ing on such a r6gime. First , it helps to keep the jo in ts supple; secondly, because it helps to p reven t the depress ing effects of t he i r neurosis b y fixing the i r minds on posit ive curat ive measures . I t is, therefore , of impor tance t ha t these cases should receive extra a t tent ion, and this is g iven by f r equen t a t tendances and ins is tence on the carrying out of regular daily periods of exercises. T h e impress ion received f rom a statistically inadequa te n u m b e r of pa t ien ts is t ha t the extra care prolongs the remission.

T h e detail of the m e t h o d of i r radia t ion will no t be discussed apar t f rom no t ing tha t the b e a m is appl ied t h r o u g h two series of oblique, paravert ical fields and measures are t aken to p reven t p r imary and scat tered i r radia t ion of the gonads. T h e dosage used in this hospi ta l has always been l imi ted to 6oo-7oo r to the bone -mar row, and an integral dose of 5 -7 '5 mg. / r . T h u s the l ikel ihood of p roduc ing a b lood dyscrasia due to i r rad ia t ion will be l imited, at the most , to be tween I and 4 pe r io ,ooo cases t rea ted ( C o u r t - B r o w n and Doll, 1957) ; far less risk t han tha t a t tached to the t r e a t m e n t of m a n y less serious diseases. Second courses of i r radia t ion are no t given.

O t h e r forms of t r e a t m e n t tha t were used w h e n the disease became react ivated include : - -

I. Steroid Therapy.--This has been used w i t h some success in 4 of I i cases which had worsened despite rad io therapy, and there seems to be a place for this f o r m of therapy.

2. Quinine Derivatives.--Chloroquine has been used in r e c u r r e n t cases and 4 of 8 cases have derived some benef i t such as lessening of pain.

3. Phenylbutozone.--This drug has been t r ied in 4 r ecu r ren t cases wi th advantage to each case_ I t is too early to say w h e t h e r this is palliative or curat ive.

A f u r t h e r s tudy is be ing made in the use of qu in ine and pheny lbu tozone . T h e la t ter is some- wha t toxic in large doses and it remains to be seen whe the r the re is an effect on the genetic system.

SUMMARY T h e results of t r e a t m e n t of a series of 87 cases o~

ankylosing spondyl i t i s are assessed. A s imple me thod , classifying the stage of the disease and the degree of improvemen t , is proffered so tha t a s t andard of efficiency of t r e a t m e n t may be apparent .

Acknowledgements.--I wish to acknowledge the advice and encouragemen t of Dr . R. Moore Pa t t e r son in comple t ing this paper ; also the help of Sister T o p e and he r staff.

REFERENCE COURT-BRowN, W. M., and DOLL, R. (I957), Med. Res.

Counc., Spec. Rep. Set., No. 295-