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-