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THE RARER ARTHROPATHIESBy F. DUDLEY HART, M.D., F.R.C.P.

Westminster Hospital, London

Pains referable to muscle, bone or joint mayoccur in almost all diseases in the medical diction-ary; were one to list the causes of arthralgia veryfew conditions would be left out of the differentialdiagnosis. It is necessary, therefore, to confineourselves to those conditions which show definiteswelling of joint structures accompanied by somedegree of pain or discomfort and' some disability.Even so the list is long and only those more likelyto be encountered will be considered. The im-portance of recognizing these rare arthropathieslies not only in the fact that they may need specifictherapy but also that misdiagnosed as rheumatoidor osteoarthritis the true diagnosis may be delayedseveral weeks or months or even occasionally yearsto the detriment of the patient. It is all too easyin any speciality in medicine to think only in termsof a handful of disease processes common in thatspeciality and to forget that the whole field ofmedicine may contribute cases very similar inexternal features but of quite different aetiology.A list of such arthropathies might include:

(a) Those syndromes which are merely a variantof rheumatoid arthritis and do not need to beconsi(ered as an entity apart; Felty's syndrome,arthritis mutilans, Still's disease, Sj6gren's syn-drome.

(b) Those syndromes which, though due to avariety of cifferent causes, are commonly mani-festations of rheumatoid arthritis; e.g. Palindromicrheumatism, intermittent hydrarthrosis.

(c) Reiter's (Brodie's) syndrome.(d) Psoriatic arthropathy.(e) The disseminated ' collagen' or ' para-

rheumatic' disorders; disseminated lupus ery-thematosus, polyarteritis nodosa, scleroderma,dermatomyositis.

(f) Manifestations of a generalized granulo-matous disease; e.g. tuberculosis, syphilis, yaws,leprosy, sarcoidosis.

(g) Manifestations ofother bacterial infective dis-orders; undulant fever, enteric fever, meningo-coccal fever, gonorrhoea.

(h) The neuropathies; Charcot's tabetic joint,syringomyelia.

(i) Malignant or submalignant conditions; car-cinoma of bronchus, lymphadenoma.

(j) Primary haematological conditions; leu-kaemia, haemophilia and other bleeding conditions,thrombocytopenic purpura.

(k) Allergic conditions; anaphylactoid purpura(Osler-Sch6nlein), serum sickness.

(1) Vitamin deficiency; scurvy, rickets.(m) Endocrine disorders; acromegaly.(n) Drug induced arthropathy; e.g. sulpha

drugs, penicillin.This list is not intended to be comprehensive

and does not include bony metastastic disease,osteomyelitis, bursitis, Paget's disease, reflexdystrophy, herpes zoster, the osteochondroses or ahost of other disorders which may cause pain butrarely a true arthropathy. Only some of theconditions listed above will be considered.

Palindromic RheumatismThis disease was described by Hench and

Rosenberg as a specific entity, the essential featureof which was a recurrent swelling of the jointsunaccompanied by fever, the joint structuressuffering no degenerative or inflammatory changewith the passage of time. While Hench andRosenberg insisted that their cases were not avariant of rheumatoid arthritis, the majority ofsuchcases seen in this country are in the final analysisand after the passage of time shown to be examplesof this disorder. Rheumatoid arthritis is aremitting-relapsing disease and this palindromicsyndrome when we have seen it has merely beenrheumatoid arthritis turned, as it were, off andon, demonstrating the essential reversibility of thisdisease in its early stages. These patients have intheir tissues the ' reversibility factor' operatingperhaps every few days; a patient with swollenpainful joints on Monday may be completely freefrom signs and symptoms on Wednesday, only tobe affected again the next day. In time the com-plete restitution to normality fails to occur andrheumatoid arthritis of the established usual typeslowly develops. We have seen cases where thepalindromic phase has lasted 30 years only to

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merge gradually into the pattern of a classicalrheumatoid arthritis after this time. While otherdisorders, for example lymphadenoma and un-dulant fever, may give a similar picture and whilethere are reported cases of an apparently specificprimary palindromic syndrome those patients wehave seen with this disorder have in almost allcases in the course of time been revealed assufferers from rheumatoid arthritis.

Intermittent HydrarthrosisWhile many conditions may produce inter-

mittent effusion into joints, as with palindromicrheumatism, most cases in our experience eventu-ally demonstrate other signs of rheumatoid disease,either arthritis or spondylitis, and become classicalexamples of one or other of these disorders. Theknees are the most common joints affected,unilaterally or bilaterally.A typical history is that of a male patient I have

seen who had suffered recurrent swelling of one orboth knees for nine years. The swelling was notpainful and lasted only several days at a time, butconsiderable inconvenience was experienced. Skintests for allergic reactions revealed a reaction tocertain dusts, long courses of which given byinjection failed to affect the subsequent course ofevents though every natural remission was hope-fully attributed to the latest injection given.Spinal stiffness and pain gradually increased and hewas eventually correctly diagnosed as a case ofankylosing spondylitis. In both this disorder andin rheumatoid arthritis such intermittent swellingmay precede the classical features by severalmonths or years. Nevertheless, not all cases canbe accounted for by these two disorders and anumber of other underlying causes are occasionallyfound, for instance-trauma, tuberculosis orundulant fever. Some patients may suffer alsofrom an associated recurrent iridocyclitis; thisagain suggests a rheumatoid aetiology, ankylosingspondylitis being more common in this associationthan rheumatoid arthritis.

Reiter's (Brodie's) SyndromeBenjamin Brodie described six patients suffering

from polyarthritis, urethritis and conjunctivitisearly in the i9th century; Ioo years later, in I9I6,Reiter described a single similar case whichfollowed bacillary dysentery. This syndrome hasbeen exciting considerable interest lately as casescome to the fore in war time and the yearsimmediately following; nevertheless, singularlylittle is generally agreed on in this disorder and theaetiology is still obscure.

Certain features are apparent. The triad mayfollow infective disease of the lower colon andrectum; Paronen described many cases in the

Finnish Army some 13 days after the onset offlexner dysentery. Many cases but by no means allfollow exposure to venereal infection, though thegonococcus is not isolated; in some cases the dis-order commences two to four weeks after successfulpenicillin therapy of a known gonococcal infection.The L or pleuropneumonia-like organism has beenconsidered by some workers as the possible causa-tive agent but the bulk of evidence is against it.Though the male sex is predominantly affected,female cases do occur. The skin condition,keratoderma blenorrhagicum, commonly co-exists,as it does with gonorrhoeal infection. Indeed, inthese days of readily-available rapidly-effectiveantibiotic therapy one wonders if many of thesecases of Reiter's syndrome do not follow gonococcalinfection immediately treated. The latent intervaland the development after a variety of pelvicinfections makes it appear to be a non-specificarthropathy fired off by a variety of infections.Nevertheless, on occasion its almost epidermicform suggests a more specific aetiological agent.Ford, in following up a number of cases ofgonococcal and non-gonococcal urethritis, foundthat four developed changes in the sacro-iliacjoints after earlier arthritis of the ankles and feet.Such cases he considers a variant of ankylosingspondylitis, as did Buckley some years previously.It is clear that much work remains to be done inthis rather untidy corner in the field ofrheumatology.Psoriatic ArthropathyThere is still a difference of opinion as to this

condition, whether it is merely a variant of rheuma-toid arthritis in association with the skin conditionor whether it is a separate disease entity. In manycases it appears to be no different from any othercase of rheumatoid arthritis, in others it presents amore irregular asymmetrical form with a tendencyto involve the terminal interphalangeal joints andthumbs. This involvement of all finger joints,metacarpo-phalangeal, proximal and distal inter-phalangeal, gives rise to the 'sausage' fingerdescribed by Dr. Francon at a meeting of theHeberden Society. Where involvement of thedistal joint occurs there is frequently, but notinvariably, the tell-tale pitting or more diffuseinvolvement of the nail in that digit. There is nodoubt that the association between psoriasis and arheumatoid type of arthritis is more common thancould be accounted for by mere chance. Wassman(I949) found psoriasis in only 0.43 per cent. ofIo,ooo medical patients, but inI3.I per cent. of,000o patients with rheumatoid arthritis; Cecil

(i949) found it in 5.6 per cent. of 930 rheumatoidsufferers. Fawcett (I950) has described fheradiological features.

December 1955628

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December I955 DUDLEY HART: The Rarer Arthropathtes 629

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1.2.51 30.4.52 23.9.53 14.4.54Psoriatic arthropathy: progress of a typical lesion in the terminal lesion in the terminal interphalangeal

joint of the index finger.

Although there are certain points of differencein psoriatic and rheumatoid arthritis, featurescommon to the two disorders predominate. Untilmore is known of the aetiology of this whole groupof disorders, little is to be gained by dogmaticstatements either way.Disseminated Lupus Erythematosus

This disorder is much more frequently diagnosedtoday than it ever was previously. Although thedisease may well be more prevalent than before itsapparent increase is probably largely due to the factthat the profession has been made more aware ofits existence in the last six years. The discovery ofthe Hargraves or L.E. cell in 1948 helped greatlyin correct diagnosis for disseminated lupuserythematosus may present in a variety of ways,one of which is classical rheumatoid arthritis. Thecondition should always be suspected if anapparent case of rheumatoid arthritis (a) is moreill than arthritic-high fever, for instance, beingpresent with severe systemic upset and onlyrelatively slight physical signs referable to thejoints; (b) has the butterfly lilac-red rash overnose and cheeks; (c) has marked abnormality ofthe plasma protein ratio; (d) has symptoms and/orsigns of visceral involvement.

Should any of the above make one suspectdisseminated lupus erythematosus a search shouldbe made for the L.E. cell. Th's cell is a poly-morphonuclear leucocyte with a large inclusionbody consisting of partially depolymerized desoxy-ribonucleic acid derived from other cells, usually

polymorphonuclear but possibly also lymphocytic.It is quite characteristic in appearance and whenonce seen is readily recognized subsequently.

It is important to re-emphasize that these casesmay be apparently classical cases of rheumatoidarthritis, clinically, radiologically and histologicallyon biopsy. The importance of correct diagnosislies not only in the therapy instituted but in themuch more serious prognosis.Caplan's Syndrome

In a study of the X-rays of the lungs of coalminers in a Welsh community Caplan (i953) dis-covered that there appeared to be an associationbetween a characteristic nodular type of pulmonaryfibrosis and rheumatoid arthritis. Miall (I954,1955) found that the prevalence of rheumatoidarthritis did not differ significantly in miners ornon-miners. As progressive massive fibrosis wasvery common among the miners, over 20 per cent.in the older age group, it seemed unlikely that thefibrosis as such predisposed to rheumatoidarthritis. Pulmonary tuberculosis appeared to bemore common in the rheumatoid arthritis group ofpatients and Miall suggests that the tissue re-sistance of this group might be different and aninherited altered type of reaction might beresponsible for the pulmonary fibrosis in therheumatoid sufferer. Were this so one mightexpect to see more examples in other communities,but lung lesions are, on the whole, rare in rheuma-toid arthritis. Further statistical information mayprove or disprove the significance of the associa-

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tion of what are in any event two very commondisorders. At present, though of extreme interest,further information is required before the causalassociation can be considered proven, but it is ofinterest that Ball (I955) found the incidence of thedifferential agglutination test increased in pro-gressive pulmonary fibrosis.Undulant Fever

In brucellosis arthralgia is common, arthritisrare, but suppurative or non-suppurative lesionsmay occur in bone, joint or periosteum. Thespine is relatively commonly involved. Jointsymptoms are said to be more common in melitensisinfections. In brucellosis the large joints are morecommonly affected than the small ones, hip forexample more than knee, knee more than ankle.Sudden pain with effusion into the joint mayoccur, the joint becoming tender, hot and swollenbut without redness. Transient flitting pains mayalso occur, simulating rheumatic fever. As notedabove true arthritis is rare as compared witharthralgia, true chronic arthritis rarer still. Indeed,some workers (Spink, 1948) found no evidence of aspecific chronic arthritis in his series of cases. Asimilar clinical picture may rarely be given bymany other generalized blood stream infections,such°as enteric fever, meningococcal fever, small-pox, pneumococcal or staphylococcal septicaemia.The field is well surveyed in the Tenth RheumatismReview (I953).Neoplastic Arthropathy

Hypertrophic pulmonary osteopathy wasobserved by Hippocrates and was described byBamberger in 1889 as an ossifying periostitis.Occurring in a variety of conditions, for examplecarcinoma of bronchus, congenital heart disease,bronchiectasis or chronic lung abscess, it presentsno great diagnostic difficulties in most cases. It isnot uncommon, however, for a patient with a lungor mediastinal lesion, usually a bronchial car-cinoma, to present not only with sub-periostealproliferation of bone but with synovitis, peri-articular swelling and even erosion of articularsurfaces. Knees and ankles are more particularlyinvolved and the knees may be greatly swollenwith effusion into the joints while arms and handsusually show only coarse thickening of the entirehand and forearm. After removal of the primarylesion the arthropathy may subside rapidly. Suchpatients with primary lung lesions are commonlysent up to rheumatism units because of thesecondary arthritic features, the primary lesionhaving been overlooked.

Haemophilic ArthritisThe great majority of haemophilics suffer at

some stage of their disease an acute effusion into

one or more joints as a result of haemorrhage intothe joint cavity or articular structures. Only slighttrauma is required and the patient may be unawareof having injured the part. The commonest jointsto be affected are the knee, ankle and elbow, andthough no age group is immune in most cases suchepisodes diminish in frequency as time passes andthe disease lessens in intensity. Haemorrhage intoa small joint, such as an interphalangeal articula-tion, is more productive of destructive change thanwhen bleeding has occurred into a larger joint, suchas the knee, where repeated effusions can occurwithout leaving any permanent disfiguration orrestriction in range of movement. In some cases,however, synovial proliferation occurs with erosionof the articular cartilage, fibrosis of joint capsuleand permanent contraction. A late chief of mineused to teach that if a so-called haemophilic (overthe age of 25) had full range of movement of alljoints, particularly knees, the diagnosis wasincorrect. Like most aphorisms, while often true,it is not infrequently quite untrue for in the pastyear I have seen two cases with full range ofmovement of all joints after many episodes ofhaemarthrosis. In one series of 98 cases ofhaemophilia (Thomas, 1936) arthritis developed in79 per cent. and more than one-half of these casesincurred permanent deformity.

Acute haemarthrosis may occur in any otherbleeding disease, e.g. thrombocytopenic purpura.The joint affection seen in children with leukaemias now well recognized.AcromegalyThe arthropathy associated with acromegaly has

been well described by Kellgren, Ball and Tutton(1952). These authors consider that this type ofjoint disease is a distinct entity. In a few patientsthey found that massive bony outgrowths inter-fered with joint movement, giving rise to a clinicalpicture of restriction in articular range. Morecommonly they found striking overgrowth of thearticular cartilage and soft tissues with remodellingof the bones which in some cases showed atrophicchanges. Softening of ligaments and otherstructures caused in some cases a curious gela-tinous or rubbery consistency, with the clinicalpicture of limb pains, instability of joints andrecurrent synovial effusions. Histological studiesshowed no inflammatory changes in the joints butrather a general overgrowth of all the limbstructures. The illustrations in this article showwell the excessive and abnormal mobility of thejoints and some of the characteristic radiologicalpictures seen in this condition.

SummaryOnly a very light and superficial survey of a few

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December I955 DUDLEY HART: The Rarer Arthropathies 631of the rarer arthropathies has been attempted inorder to emphasize that joint structures may be-come affected in a very large number of disordersin medicine and surgery, and that in only relativelyfew of these is the disease primarily of these jointstructures. The differential diagnosis of a painfulswollen joint or joints takes in a large part of thewhole field of medicine; unless one is alive to allthe possibilities many underlying primary con-ditions will be missed.

BIBLIOGRAPHYImportant references of general interest in each subject are given

below:Palindromic RheumatismHENCH, P. S., and ROSENBERG, E. F. (1944), Arch. int. Med.,

73, 293.ROPES, M. W., and BAUER, W. (I945), New Eng. J. Med., 233,

592, 6i8.Intermittent Hydrarthrosis'Rheumatism Review' (I953), Ann. int. Med., 39, 776.Reiter's (Brodie's) SyndromeBRODIE, B. C. (Ix88, i836), 'Pathological and Surgical observa-

tions on diseases of the joints,' ist edition, I818, p. 54, 4thedition, I836, Longman, London.

BUCKLEY, C. W. (I943), Brit. med. j., ii, 4.FORD, D. K. (I953), Ann. Rheum. Dis., 12, I77.PARONEN, I. (I948), Acta med. Scand. (Suppl.).REITER, H. (1916), Deutsch. med. Wchnschr., 42, 1535.Psoriatic ArthropathyBAUER, W., BENNETT, G. A., and ZELLER, S. W. (1941),Trans. Ass. Amer. Phys., 56, 349.

CECIL, R. L. (I949), Chicago M. S. and C. Bull., 51, 747.FAWCETT, J. (I95o), Brit. J. Radiol., 23, 440.'Rheumatism Review' (x953), Ann. int. Med., 39, 767.SHERMAN, M. S. (I952), J. Bone Jt. Surg., 34a, 831.WASSMAN, K. (I949), Ann. Rheum. Dis., 8, 70.Disseminated Lupus ErythematosusHARGRAVES, M. M., RICHMOND, H., and MORTON, R.

(1948), Proc. Staff Meet. Mayo Clinic, 23, 25.Caplan's SyndromeBALL, J. (1955), Ann. Rheum. Dis., x4, I59.CAPLAN, A. (1953), Thorax, 8, 29.MIALL, W. E. (1954), Quart. J. Med., 23, 465.MIALL, W. E. (I955), Ann. Rheum. Dis., 14, 150.Undulant Fever'Arthritis Review' (I953), Ann. int. Med., 39, 514.SPINK, W. W. (1948), Ibid., 29, 238.Neoplastic ArthropathyBAMBERGER, E. (1889), Wien. Klin. Wschr., 2, 226.BERG, R. (I949), Dis. of Chest., 16, 483.JARVINEN, K. A., and KUMLIN, T. (1954), Ann. Rheum. Dis.,

13, 52.THOMAS, C. PRICE (I955), Practitioner, 174, 301.Haemophilic ArthritisBOWIE, M. A. (I953), 'Comroe's Arthritis,' p. 876, 5th edition,

edited by J. L. Hollander. Henry Kimpton, London.COHEN, H. (1954), 'Textbook of Rheumatic Diseases,' p. 281,

2nd edition, edited by W. S. C. Copeman. E. & S. Livingstone,London.

THOMAS, H. B. (1936), J. Bone Jt. Surg., I8, 140.AcromegalyATKINSON, F. R. B. (1932), ' Acromegaly,' London.KELLGREN, J. H., BALL, J., and TUTTON, G. H. (1952),

Quart. J. Med., 21, 405.WAINE, H., BENNETT, G. A., and BAUER, W. (I945), Amer

J. med. Sci., 209, 67I.

PERIPHERAL VASCULAR DISORDERS(Postgraduate Medical Journal)

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THE INVESTIGATION OF PERIPHERAL RAYNAUD PHENOMENONVASCULAR DISORDERS R. P. Jepson, F.R.C.S.Peter Martin, V.R.D., M.Chir., F.R.C.S.(Ed.)

COLLATERAL CIRCULATION IN THEPERIPHERAL ARTERIOGRAPHY AND LIMBAORTOGRAPHY C. J. Longland, M.V.O., M.S., F.R.C.S.Robert E. Steiner, M.B., Ch.B., D.M.R.,

F.F.R., and David Messent, M.B., F.R.C.S. SYMPATHECTOMY AND PALLIATIVEOPERATIONS FOR OCCLUSIVEARTERIAL DISEASE

THE MEDICAL MANAGEMENT OF A. J. Slessor, M.V.O., F.R.C.S.OBLITERATIVE ARTERIAL DISEASE OFTHE LEGS BLOOD VESSEL GRAFTINGRobert Semple, M.D., M.R.C.P. C. G. Rob, M.C., M.Chir., F.R.C.S.

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