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Home , Arthritis mutilans, Palindromic rheumatism

Postgrad Med J: first published as 10.1136/pgmj.31.362.627 on 1 December 1955. Downloaded from 627

THE RARER By F. DUDLEY HART, M.D., F.R.C.P. Westminster Hospital, London

Pains referable to muscle, bone or joint may (i) Malignant or submalignant conditions; car- occur in almost all diseases in the medical diction- cinoma of bronchus, lymphadenoma. ary; were one to list the causes of very (j) Primary haematological conditions; leu- few conditions would be left out of the differential kaemia, haemophilia and other bleedingconditions, diagnosis. It is necessary, therefore, to confine thrombocytopenic purpura. ourselves to those conditions which show definite (k) Allergic conditions; anaphylactoid purpura swelling of joint structures accompanied by some (Osler-Sch6nlein), serum sickness. degree of pain or discomfort and' some disability. (1) Vitamin deficiency; scurvy, rickets. Even so the list is long and only those more likely (m) Endocrine disorders; acromegaly. to be encountered will be considered. The im- (n) Drug induced ; e.g. sulpha portance of recognizing these rare arthropathies drugs, penicillin. Protected by copyright. lies not only in the fact that they may need specific This list is not intended to be comprehensive but also that misdiagnosed as rheumatoid and does not include bony metastastic disease, or the true diagnosis may be delayed osteomyelitis, bursitis, Paget's disease, reflex several weeks or months or even occasionally years dystrophy, herpes zoster, the osteochondroses or a to the detriment of the patient. It is all too easy host of other disorders which may cause pain but in any speciality in to think only in terms rarely a true arthropathy. Only some of the of a handful of disease processes common in that conditions listed above will be considered. speciality and to forget that the whole field of medicine may contribute cases very similar in Palindromic external features but of quite different aetiology. This disease was described by Hench and A list of such arthropathies might include: Rosenberg as a specific entity, the essential feature (a) Those syndromes which are merely a variant of which was a recurrent swelling of the joints of rheumatoid and do not need to be unaccompanied by , the joint structures consi(ered as an no or

entity apart; Felty's syndrome, suffering degenerative inflammatory change http://pmj.bmj.com/ , Still's disease, Sj6gren's syn- with the passage of time. While Hench and drome. Rosenberg insisted that their cases were not a (b) Those syndromes which, though due to a variant of , the majority ofsuch variety of cifferent causes, are commonly mani- cases seen in this country are in the final analysis festations of rheumatoid arthritis; e.g. Palindromic and after the passage of time shown to be examples rheumatism, intermittent hydrarthrosis. of this disorder. Rheumatoid arthritis is a (c) Reiter's (Brodie's) syndrome. remitting-relapsing disease and this palindromic

(d) Psoriatic arthropathy. syndrome when we have seen it has merely been on September 27, 2021 by guest. (e) The disseminated ' collagen' or ' para- rheumatoid arthritis turned, as it were, off and rheumatic' disorders; disseminated lupus ery- on, demonstrating the essential reversibility of this thematosus, polyarteritis nodosa, scleroderma, disease in its early stages. These patients have in dermatomyositis. their tissues the ' reversibility factor' operating (f) Manifestations of a generalized granulo- perhaps every few days; a patient with swollen matous disease; e.g. , syphilis, yaws, painful joints on Monday may be completely free leprosy, sarcoidosis. from signs and symptoms on Wednesday, only to (g) Manifestations of otherbacterial infective dis- be affected again the next day. In time the com- orders; undulant fever, enteric fever, meningo- plete restitution to normality fails to occur and coccal fever, gonorrhoea. rheumatoid arthritis of the established usual type (h) The neuropathies; Charcot's tabetic joint, slowly develops. We have seen cases where the syringomyelia. palindromic phase has lasted 30 years only to Postgrad Med J: first published as 10.1136/pgmj.31.362.627 on 1 December 1955. Downloaded from 628 POSTGRADUATE MEDICAL JOURNAL December 1955 merge gradually into the pattern of a classical Finnish Army some 13 days after the onset of rheumatoid arthritis after this time. While other flexner dysentery. Many cases but by no means all disorders, for example lymphadenoma and un- follow exposure to venereal infection, though the dulant fever, may give a similar picture and while gonococcus is not isolated; in some cases the dis- there are reported cases of an apparently specific order commences two to four weeks after successful primary palindromic syndrome those patients we penicillin therapy of a known gonococcal infection. have seen with this disorder have in almost all The L or pleuropneumonia-like organism has been cases in the course of time been revealed as considered by some workers as the possible causa- sufferers from rheumatoid arthritis. tive agent but the bulk of evidence is against it. Though the male sex is predominantly affected, Intermittent Hydrarthrosis female cases do occur. The skin condition, While many conditions may produce inter- keratoderma blenorrhagicum, commonly co-exists, mittent effusion into joints, as with palindromic as it does with gonorrhoeal infection. Indeed, in rheumatism, most cases in our experience eventu- these days of readily-available rapidly-effective ally demonstrate other signs of rheumatoid disease, antibiotic therapy one wonders if many of these either arthritis or , and become classical cases of Reiter's syndrome do not follow gonococcal examples of one or other of these disorders. The infection immediately treated. The latent interval knees are the most common joints affected, and the development after a variety of pelvic unilaterally or bilaterally. infections makes it appear to be a non-specific A typical history is that of a male patient I have arthropathy fired off by a variety of infections. seen who had suffered recurrent swelling of one or Nevertheless, on occasion its almost epidermic both knees for nine years. The swelling was not form suggests a more specific aetiological agent. several at a but in a number of cases of painful and lasted only days time, Ford, following up Protected by copyright. considerable inconvenience was experienced. Skin gonococcal and non-gonococcal urethritis, found tests for allergic reactions revealed a reaction to that four developed changes in the sacro-iliac certain dusts, long courses of which given by joints after earlier arthritis of the ankles and feet. injection failed to affect the subsequent course of Such cases he considers a variant of ankylosing events though every natural remission was hope- spondylitis, as did Buckley some years previously. fully attributed to the latest injection given. It is clear that much work remains to be done in Spinal stiffness and pain gradually increased and he this rather untidy corner in the field of was eventually correctly diagnosed as a case of . . In both this disorder and in rheumatoid arthritis such intermittent swelling Psoriatic Arthropathy may precede the classical features by several There is still a difference of opinion as to this months or years. Nevertheless, not all cases can condition, whether it is merely a variant of rheuma- be accounted for by these two disorders and a toid arthritis in association with the skin condition number of other underlying causes are occasionally or whether it is a separate disease entity. In many found, for instance-trauma, tuberculosis or cases it appears to be no different from any other http://pmj.bmj.com/ undulant fever. Some patients may suffer also case of rheumatoid arthritis, in others it presents a from an associated recurrent iridocyclitis; this more irregular asymmetrical form with a tendency again suggests a rheumatoid aetiology, ankylosing to involve the terminal interphalangeal joints and spondylitis being more common in this association thumbs. This involvement of all finger joints, than rheumatoid arthritis. metacarpo-phalangeal, proximal and distal inter- phalangeal, gives rise to the 'sausage' finger Reiter's described Dr. at a of the (Brodie's) Syndrome by Francon meeting on September 27, 2021 by guest. Benjamin Brodie described six patients suffering Heberden Society. Where involvement of the from , urethritis and conjunctivitis distal joint occurs there is frequently, but not early in the i9th century; Ioo years later, in I9I6, invariably, the tell-tale pitting or more diffuse Reiter described a single similar case which involvement of the nail in that digit. There is no followed bacillary dysentery. This syndrome has doubt that the association between psoriasis and a been exciting considerable interest lately as cases rheumatoid type of arthritis is more common than come to the fore in war time and the years could be accounted for by mere chance. Wassman immediately following; nevertheless, singularly (I949) found psoriasis in only 0.43 per cent. of little is generally agreed on in this disorder and the Io,ooo medical patients, but inI3.I per cent. of aetiology is still obscure. ,000o patients with rheumatoid arthritis; Cecil Certain features are apparent. The triad may (i949) found it in 5.6 per cent. of 930 rheumatoid follow infective disease of the lower colon and sufferers. Fawcett (I950) has described fhe rectum; Paronen described many cases in the radiological features. Postgrad Med J: first published as 10.1136/pgmj.31.362.627 on 1 December 1955. Downloaded from December I955 DUDLEY HART: The Rarer Arthropathtes 629

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1.2.51 30.4.52 23.9.53 14.4.54 Psoriatic arthropathy: progress of a typical lesion in the terminal lesion in the terminal interphalangeal joint of the index finger. Protected by copyright. Although there are certain points of difference polymorphonuclear but possibly also lymphocytic. in psoriatic and rheumatoid arthritis, features It is quite characteristic in appearance and when common to the two disorders predominate. Until once seen is readily recognized subsequently. more is known of the aetiology of this whole group It is important to re-emphasize that these cases of disorders, little is to be gained by dogmatic may be apparently classical cases of rheumatoid statements either way. arthritis, clinically, radiologically and histologically on biopsy. The importance of correct diagnosis Disseminated Lupus Erythematosus lies not only in the therapy instituted but in the This disorder is much more frequently diagnosed much more serious prognosis. today than it ever was previously. Although the disease may well be more prevalent than before its Caplan's Syndrome apparent increase is probably largely due to the fact In a study of the X-rays of the lungs of coal that the profession has been made more aware of miners in a Welsh community Caplan (i953) dis- http://pmj.bmj.com/ its existence in the last six years. The discovery of covered that there appeared to be an association the Hargraves or L.E. cell in 1948 helped greatly between a characteristic nodular type of pulmonary in correct diagnosis for disseminated lupus fibrosis and rheumatoid arthritis. Miall (I954, erythematosus may present in a variety of ways, 1955) found that the prevalence of rheumatoid one of which is classical rheumatoid arthritis. The arthritis did not differ significantly in miners or condition should always be suspected if an non-miners. As progressive massive fibrosis was apparent case of rheumatoid arthritis (a) is more very common among the miners, over 20 per cent. ill than arthritic-high fever, for instance, being in the older age group, it seemed unlikely that the on September 27, 2021 by guest. present with severe systemic upset and only fibrosis as such predisposed to rheumatoid relatively slight physical signs referable to the arthritis. Pulmonary tuberculosis appeared to be joints; (b) has the butterfly lilac-red rash over more common in the rheumatoid arthritis group of nose and cheeks; (c) has marked abnormality of patients and Miall suggests that the tissue re- the plasma protein ratio; (d) has symptoms and/or sistance of this group might be different and an signs of visceral involvement. inherited altered type of reaction might be Should any of the above make one suspect responsible for the pulmonary fibrosis in the disseminated lupus erythematosus a search should rheumatoid sufferer. Were this so one might be made for the L.E. cell. Th's cell is a poly- expect to see more examples in other communities, morphonuclear leucocyte with a large inclusion but lung lesions are, on the whole, rare in rheuma- body consisting of partially depolymerized desoxy- toid arthritis. Further statistical information may ribonucleic acid derived from other cells, usually prove or disprove the significance of the associa- Postgrad Med J: first published as 10.1136/pgmj.31.362.627 on 1 December 1955. Downloaded from 630 POSTGRADUATE MEDICAL JOURNAL December I955 tion of what are in any event two very common one or more joints as a result of haemorrhage into disorders. At present, though of extreme interest, the joint cavity or articular structures. Only slight further information is required before the causal trauma is required and the patient may be unaware association can be considered proven, but it is of of having injured the part. The commonest joints interest that Ball (I955) found the incidence of the to be affected are the knee, ankle and elbow, and differential agglutination test increased in pro- though no age group is immune in most cases such gressive pulmonary fibrosis. episodes diminish in frequency as time passes and the disease lessens in intensity. Haemorrhage into Undulant Fever a small joint, such as an interphalangeal articula- In brucellosis arthralgia is common, arthritis tion, is more productive of destructive change than rare, but suppurative or non-suppurative lesions when bleeding has occurred into a largerjoint, such may occur in bone, joint or periosteum. The as the knee, where repeated effusions can occur spine is relatively commonly involved. Joint without leaving any permanent disfiguration or symptoms are said to be more common in melitensis restriction in range of movement. In some cases, infections. In brucellosis the large joints are more however, synovial proliferation occurs with erosion commonly affected than the small ones, hip for of the articular cartilage, fibrosis of joint capsule example more than knee, knee more than ankle. and permanent contraction. A late chief of mine Sudden pain with effusion into the joint may used to teach that if a so-called haemophilic (over occur, the joint becoming tender, hot and swollen the age of 25) had full range of movement of all but without redness. Transient flitting pains may joints, particularly knees, the diagnosis was also occur, simulating rheumatic fever. As noted incorrect. Like most aphorisms, while often true, above true arthritis is rare as compared with it is not infrequently quite untrue for in the past arthralgia, true chronic arthritis rarer still. Indeed, I have seen two cases with full of

year rangeProtected by copyright. some workers (Spink, 1948) found no evidence of a movement of all joints after many episodes of specific chronic arthritis in his series of cases. A haemarthrosis. In one series of 98 cases of similar clinical picture may rarely be given by haemophilia (Thomas, 1936) arthritis developed in many other generalized stream infections, 79 per cent. and more than one-half of these cases such°as enteric fever, meningococcal fever, small- incurred permanent deformity. pox, pneumococcal or staphylococcal septicaemia. Acute haemarthrosis may occur in any other The field is well surveyed in the Tenth Rheumatism bleeding disease, e.g. thrombocytopenic purpura. Review (I953). The joint affection seen in children with leukaemia Neoplastic Arthropathy s now well recognized. Hypertrophic pulmonary osteopathy was observed by Hippocrates and was described by Acromegaly Bamberger in 1889 as an ossifying . The arthropathy associated with acromegaly has Occurring in a variety of conditions, for example been well described by Kellgren, Ball and Tutton carcinoma of heart (1952). These authors consider that this type of bronchus, congenital disease, http://pmj.bmj.com/ bronchiectasis or chronic lung abscess, it presents joint disease is a distinct entity. In a few patients no great diagnostic difficulties in most cases. It is they found that massive bony outgrowths inter- for a with a fered with joint movement, giving rise to a clinical not uncommon, however, patient lung of restriction in articular More or mediastinal lesion, usually a bronchial car- picture range. cinoma, to present not only with sub-periosteal commonly they found striking overgrowth of the proliferation of bone but with synovitis, peri- articular cartilage and soft tissues with remodelling articular of the bones which in some cases showed atrophic articular swelling and even erosion of of and other surfaces. Knees and ankles are more particularly changes. Softening ligaments on September 27, 2021 by guest. involved and the knees may be greatly swollen structures caused in some cases a curious gela- with effusion into the joints while arms and hands tinous or rubbery consistency, with the clinical usually show only coarse thickening of the entire picture of limb pains, instability of joints and hand and forearm. After removal of the primary recurrent synovial effusions. Histological studies lesion the arthropathy may subside rapidly. Such showed no inflammatory changes in the joints but patients with primary lung lesions are commonly rather a general overgrowth of all the limb sent up to rheumatism units because of the structures. The illustrations in this article show secondary arthritic features, the primary lesion well the excessive and abnormal mobility of the been overlooked. joints and some of the characteristic radiological having pictures seen in this condition. Haemophilic Arthritis The great majority of haemophilics suffer at Summary some stage of their disease an acute effusion into Only a very light and superficial survey of a few Postgrad Med J: first published as 10.1136/pgmj.31.362.627 on 1 December 1955. Downloaded from December I955 DUDLEY HART: The Rarer Arthropathies 631 of the rarer has been in CECIL, R. L. (I949), Chicago M. S. and C. Bull., 51, 747. arthropathies attempted FAWCETT, J. (I95o), Brit. J. Radiol., 23, 440. order to emphasize that joint structures may be- 'Rheumatism Review' (x953), Ann. int. Med., 39, 767. come affected in a very large number of disorders SHERMAN, M. S. (I952), J. Bone Jt. Surg., 34a, 831. in medicine and , and that in only relatively WASSMAN, K. (I949), Ann. Rheum. Dis., 8, 70. few of these is the disease of these Disseminated Lupus Erythematosus primarily joint HARGRAVES, M. M., RICHMOND, H., and MORTON, R. structures. The differential diagnosis of a painful (1948), Proc. Staff Meet. Mayo Clinic, 23, 25. swollen joint or joints takes in a large part of the Caplan's Syndrome whole field of unless one is alive to all BALL, J. (1955), Ann. Rheum. Dis., x4, I59. medicine; CAPLAN, A. (1953), Thorax, 8, 29. the possibilities many underlying primary con- MIALL, W. E. (1954), Quart. J. Med., 23, 465. ditions will be missed. MIALL, W. E. (I955), Ann. Rheum. Dis., 14, 150. Undulant Fever BIBLIOGRAPHY 'Arthritis Review' (I953), Ann. int. Med., 39, 514. Important references of general interest in each subject are given SPINK, W. W. (1948), Ibid., 29, 238. below: Neoplastic Arthropathy BAMBERGER, E. (1889), Wien. Klin. Wschr., 2, 226. HENCH, P. S., and ROSENBERG, E. F. (1944), Arch. int. Med., BERG, R. (I949), Dis. of Chest., 16, 483. 73, 293. JARVINEN, K. A., and KUMLIN, T. (1954), Ann. Rheum. Dis., ROPES, M. W., and BAUER, W. (I945), New Eng. J. Med., 233, 13, 52. 592, 6i8. THOMAS, C. PRICE (I955), Practitioner, 174, 301. Intermittent Hydrarthrosis Haemophilic Arthritis 'Rheumatism Review' (I953), Ann. int. Med., 39, 776. BOWIE, M. A. (I953), 'Comroe's Arthritis,' p. 876, 5th edition, Reiter's (Brodie's) Syndrome edited by J. L. Hollander. Henry Kimpton, London. BRODIE, B. C. (Ix88, i836), 'Pathological and Surgical observa- COHEN, H. (1954), 'Textbook of Rheumatic Diseases,' p. 281, tions on diseases of the joints,' ist edition, I818, p. 54, 4th 2nd edition, edited by W. S. C. Copeman. E. & S. Livingstone, edition, I836, Longman, London. London. BUCKLEY, C. W. (I943), Brit. med. j., ii, 4. THOMAS, H. B. (1936), J. Bone Jt. Surg., I8, 140. FORD, D. K. (I953), Ann. Rheum. Dis., 12, I77. Acromegaly PARONEN, I. (I948), Acta med. Scand. (Suppl.). ATKINSON, F. R. B. (1932), ' Acromegaly,' London. Protected by copyright. REITER, H. (1916), Deutsch. med. Wchnschr., 42, 1535. KELLGREN, J. H., BALL, J., and TUTTON, G. H. (1952), Psoriatic Arthropathy Quart. J. Med., 21, 405. BAUER, W., BENNETT, G. A., and ZELLER, S. W. (1941), WAINE, H., BENNETT, G. A., and BAUER, W. (I945), Amer Trans. Ass. Amer. Phys., 56, 349. J. med. Sci., 209, 67I.

PERIPHERAL VASCULAR DISORDERS (Postgraduate Medical Journal)

Price: 3s. 8d., post free http://pmj.bmj.com/ THE INVESTIGATION OF PERIPHERAL RAYNAUD PHENOMENON VASCULAR DISORDERS R. P. Jepson, F.R.C.S. Peter Martin, V.R.D., M.Chir., F.R.C.S.(Ed.) COLLATERAL CIRCULATION IN THE PERIPHERAL ARTERIOGRAPHY AND LIMB AORTOGRAPHY C. J. Longland, M.V.O., M.S., F.R.C.S.

Robert E. Steiner, M.B., Ch.B., D.M.R., on September 27, 2021 by guest. and David F.R.C.S. SYMPATHECTOMY AND PALLIATIVE F.F.R., Messent, M.B., OPERATIONS FOR OCCLUSIVE ARTERIAL DISEASE THE MEDICAL MANAGEMENT OF A. J. Slessor, M.V.O., F.R.C.S. OBLITERATIVE ARTERIAL DISEASE OF THE LEGS BLOOD VESSEL GRAFTING Robert Semple, M.D., M.R.C.P. C. G. Rob, M.C., M.Chir., F.R.C.S. Published by THE FELLOWSHIP OF POSTGRADUATE MEDICINE 60, Portland Place, London, W.1.