Part 12

The acute form of rheumatoid arthritis closely resembles the milder varieties of acute articular rheumatism or the best marked examples of the subacute form of that disease. But it presents the following particulars, by which it may generally perhaps, but not always, be distinguished: while the temperature, the thirst, the furring of the tongue, the frequency of the pulse, the articular pains and tenderness, etc., are less developed than in acute articular rheumatism, there is wanting the profuse and continued perspiration, the early involvement of the endo- or pericardium in the inflammation, and the prompt prostration of the strength so commonly witnessed in that disease. On the other hand, while the rheumatoid affection may involve the larger joints--knees, ankles, elbows, and wrists--it almost certainly implicates the smaller joints of the fingers, and often of the toes. There is apt to be greater effusion into the synovial capsules (McLeod's capsular rheumatism) and into the synovial sheaths and bursæ about the affected joints than in ordinary acute or subacute rheumatism; further, the inflammation does not migrate from joint to joint, but obstinately persists in several of them, and more especially in the wrist and in the metacarpo-phalangeal joints of the index and middle finger, perhaps also in the ankles and in the metatarso-phalangeal articulation of the great toe. Instead of disappearing in four to six weeks, the articular inflammation continues, although the pain may abate very much, and the capsules of the joints continue swollen and rather tense. The muscles of the extremities waste, and are the seat of painful reflex spasms which interfere with the movements of the joints; and although the patient is capable of moving about, and is free from all febrile disturbance, one or several of his joints remain permanently swollen, painful, and crippled. Perfect restoration of all the affected joints seldom if ever occurs. In common with other observers, I have met with this acute form most frequently in young women twenty to thirty years of age--several times in connection with recent delivery or rapid child-bearing, or lactation; once after what was regarded by the medical attendant as an attack of acute rheumatism occurring not long after labor. It has been observed in children, and is not uncommon after forty. These patients usually suffer in their general health--become weak, pale, depressed in spirits, and lose flesh. In several cases of this form marked intervals of improvement have occurred; the local disease has ceased to progress, and tolerable comfort has been experienced, perhaps, till pregnancy, delivery, or lactation again determined a fresh outbreak of the disease. Sometimes, however, this acute form steadily advances, and in a year or two establishes changes in the cartilaginous and osseous structure of the affected joints. Such a case I met in a lady of twenty-one who had had a good deal of anxiety as a mathematical teacher, and whose illness set in during vacation while at the seaside. It proved obstinately progressive for several years, until several of the larger joints, as well as the smaller, were badly crippled.

{81} The primary chronic form is much the more frequent, although between it and the acute variety there are many intermediate grades. For weeks or months the patient may experience numbness or formication and rheumatic pains in the limbs, perhaps with a sense of stiffness in the joints, especially felt after rest or the day after unusual fatigue. Then one or more joints--most frequently the metacarpo-phalangeal of the fingers--become painful, swollen, tender when touched, and inordinately hot; these symptoms may subside under rest or treatment, and after weeks or months recur, either without known cause or from exposure, fatigue, or some impairment of the health. Usually, the original joint is again affected, but frequently one or two more of the same on the other hand suffer likewise. More or less complete remissions of the pain and local inflammation now tend to take place from time to time and alternate with exacerbations or fresh attacks of the local disturbance, and the disease extends, as it were, centripetally and more or less symmetrically to the wrists, then to the elbows, and then to the shoulders, or from the toes to the ankles and thence to the knees--although there is no invariable sequence of this kind--and next to the hands; the knees are specially liable to invasion. Of Haygarth's 34 cases, in 2 the knees alone suffered, and "in all or nearly all the rest the hands, chiefly the fingers, were probably affected." In Charcot's 45 cases the début took place in the small joints of the hands and feet 29 times; in the hands, feet, and one large articulation, 7 times; in one large joint, and later in the fingers, 9 times. Even in this primary chronic form there is usually in the earlier stages some effusion into the joints; the soft parts of the articulation are thickened and swollen; obscure fluctuation in the smaller and very distinct fluctuation in the larger joints may be felt. The pain may be severe, especially at night, and during the exacerbations of the disease it varies greatly in its degree and persistency. The position and shape of the joints are altered, partly by spasmodic retraction of the muscles, and more or less by the effusion into the capsules and adjacent bursæ and sheaths, and the thickening of the soft parts covering the articulations. As the disease progresses further deformities ensue from the growth of new bone around the heads of the bones, the absorption of the articular cartilage, the development of masses of cartilage in the hypertrophied synovial processes and beneath the synovial membrane at the margin of the bones; the relaxation of the articular ligaments; and the displacements and subluxations of the unshapely bones composing the joint. The great wasting of the muscles of the member affected has some share in producing its unnatural appearance. In the advanced stage there is more or less abiding pain, soreness, and stiffness in the affected articulations, violent cramps are experienced in the course of the adjacent muscles, and pains either along the nerves or vaguely down the limbs. Crackings or creakings are to be heard, and grating is to be felt during the movements of the joints; these movements become more and more restricted, so that an immobility almost equal to that of true bony ankylosis is established, this result seldom occurring except amongst the carpal, tarsal, tibio-tarsal, and the vertebral articulations. Interlocking of the osteophites formed on and around the articular surfaces, and in other cases union of these surfaces by the interposition of newly-formed fibrous tissue, produce a spurious ankylosis {82} destructive of the articular functions. In the very advanced stages the feet, ankles, and legs are often considerably enlarged and the integument thickened by a chronic oedematous infiltration, or the bones and soft parts are atrophied and the integument is pale, smooth, and attenuated, resembling parchment or the condition seen in certain stages of scleroderma and tightly drawn over the wasted rigid fingers. This primary chronic form is especially apt to progress steadily for many years, the joints earliest affected becoming gradually more distorted and crippled, and fresh joints becoming invaded until there may hardly remain a single sound articulation in the limbs, or even in the body; and at length the patient may be unable to feed himself or masticate or raise his chin from his sternum or rotate his head or stand.

The deformities of the several joints, being largely the result of muscular contraction, observe certain general types, which, however, are not peculiar to the disease, but occur in various affections of the nerve-centres, involving paralysis or spasm or both. Charcot has carefully described those met with in the hands, and I must refer to his masterly article upon chronic articular rheumatism for his account of them.

(1) It must suffice to say here that the predominant features of the hand in chronic rheumatoid arthritis are the following: The first phalanx of the fingers is either flexed upon the metacarpus or extended, and the terminal phalanx in like manner is either markedly flexed or extended upon the second, or these two phalanges are maintained in a straight line, while the first phalanx is, as usual, decidedly flexed upon the metacarpus. In all these varieties the hand is pronated; there is a great tendency to deviation of the fingers toward the ulnar border of the hand, although sometimes the deformed fingers stand out, not unlike a bunch of parsnips. The thumb escapes longer than the other fingers, and its metacarpo-phalangeal joint is usually flexed, rarely extended.

(2) The great toe, enlarged at the metacarpo-phalangeal articulation, is usually drawn to the outer border of the foot, across and above, but rarely below, the other toes, and the foot is usually abducted and flattened, the prominent internal border resting on the ground. The wrist, elbow, and knee-joints are generally flexed; the distal ends of the ulna and radius, more or less enlarged, project backward; the semi-flexed tibia is drawn backward on the femur and rotated outward, thus rendering the internal condyle of the femur prominent and displacing the patella toward the external condyle, and foreign bodies may frequently be felt in the enlarged knee- and elbow-joints. Finally, the extremities of the affected bones will, as a rule, be found enlarged and misshapen, and nodosities, rims, tips, ridges, and stalactiform growths of new bone may be felt on them.

The general condition in this chronic form varies in different individuals, and there is no characteristic disturbance of the functions, such as obtains in chronic gout. There is no elevation of temperature, unless to a slight degree during an active crisis of the disease; the tongue may be clean, the pulse tranquil, the appetite and digestion satisfactory, and {83} the urine normal or perhaps pale and of low density. Fuller, however, says that "more generally the complexion is sallow and the skin sluggish, and evidence of mischief is furnished by yellowishness of the conjunctivæ, constipation of the bowels, a pale and unhealthy character of the dejections, excessive flatulence after meals, turbidity of the urine, and fulness of the pulse." My own experience hardly harmonizes with this, and I have seen many persons suffering for years from the general and partial form in the enjoyment of excellent general health. Should, however, the disease develop in a person the subject of menorrhagia or other uterine disorder, or of repeated child-bearing, or after prolonged mental anxiety, some disturbance of the general health fairly referable to such disturbing conditions may be certainly looked for. In the advanced stages the prolonged suffering and confinement often induce anæmia, dyspepsia, and failing health.

More numerous and exhaustive analyses of the perspiration, urine, and blood in the disease are needed. There is no uniform condition of the skin; general perspirations, chiefly at night, often obtain, but I know of no authoritative report as to the chemical reaction of the sweat in this disease; Garrod and Charcot vouch for an absence of uric acid in the blood, while Marrot found both this acid and the urea below the normal quantity in the urine, although the acid increased notably under baths of high temperature.

Certain affections other than the articular have been occasionally observed in persons suffering from rheumatoid arthritis, but many even of those authors who regard the disease as a form of rheumatism speak of these affections as coincidences, and not as essential manifestations of the disease. Charcot and Besnier, however, maintain the latter to be their true relation to the articular affection which they regard as chronic rheumatism. The two authors just named allege that all the visceral localizations that occur in acute articular rheumatism may obtain in the nodular form, but that such localizations are infinitely less frequent and serious than in the acute, subacute, or simple chronic forms of articular rheumatism--that endo- and pericarditis undoubtedly do occur in nodular rheumatism, and appear especially where there is an exacerbation of the disease and where there is some approach to the acute state. As Charcot has adduced these cardiac affections in proof of the rheumatic nature of rheumatoid arthritis, it is deserving of mention that he had personally met with but two instances of endocarditis and five of pericarditis, four of the latter having been discovered not during life, but in nine autopsies, and that he cites only eight other cases of endo- or pericarditis which had been either published or reported to him. He admits too that there had generally been in these cases, at some former period, an attack of acute rheumatism. Besnier, Homolle, Malherbe, Vidal, and Colombel, in their articles upon the disease under consideration, do not cite a single case in which they have seen cardiac disease in rheumatoid arthritis. On the other hand, McLeod, Garrod, Fuller, Flint, Senator, and Pye-Smith either deny or ignore the occurrence of cardiac disease as a manifestation or complication of this disease. My personal {84} experience coincides with that of those authorities last cited, except in one instance, and that is open to the objection that the patient's father had had acute articular rheumatism, the mother was the subject of chronic deforming arthritis, and the patient had experienced during many winters an affection which began in the smaller joints and permanently damaged them; when first seen by me he had chronic disease of the aortic valves. He may have had true articular rheumatism as well as rheumatoid arthritis. His father had experienced the one, his mother the other. If those instances be excluded in which a former attack of acute rheumatism might be adduced in explanation of the supervention of cardiac disease, but few cases will remain to suggest that rheumatoid arthritis may develop endo- or pericarditis; and when it is borne in mind that in several ways the cardiac affections may have arisen as mere coincidences of the rheumatoid affection, it is well to wait for further evidence before accepting as proved the occurrence of cardiac affections as local manifestations of rheumatoid arthritis. Garrod's observation is still pertinent: "The form of the disease in which acute cardiac inflammation has occurred may be rather that of true articular rheumatism of a very subacute character."

Nor is the evidence at all satisfactory in favor of any special tendency to the following affections, much less of their being local manifestations of rheumatoid arthritis: viz. pleuritis (McLeod, Fuller), asthma (Charcot), chronic laryngitis (Garrod), grave cerebral or spinal disturbances (McLeod, Fuller, Vidal), paralysis agitans, locomotor ataxia, sciatica, trifacial neuralgia, and albuminous nephritis.

Among the more frequent complications may be mentioned migraine, certain cutaneous affections, more especially psoriasis, prurigo, lichen, and some diseases of the eye, chiefly iritis, which is apt to be relapsing, and sometimes episcleritis. It is remarkable that iritis very seldom occurs as a complication of acute articular rheumatism.

The so-called rheumatic nodules occur also in chronic rheumatoid arthritis. It is not yet established that they are peculiar to rheumatism and to rheumatoid arthritis. Dr. Stephen Mackenzie has seen them in one instance in tertiary syphilis, the patient not having had arthritis, rheumatism, or chorea.

2d. The partial or oligo-articular form of rheumatoid arthritis, like the general or polyarticular variety, is usually a primarily chronic affection, insidious in its invasion and slow in its progress. It is chiefly observed in old persons, especially men (senile arthritis), affects frequently a single joint, and chiefly the hip, but occasionally the knee, shoulder, or spinal column, either as a consequence of special injury or of the wear and tear of life, or exposure to cold and wet, or even of what seemed to be simple acute or subacute articular rheumatism or gonorrhoeal rheumatism. When not the result of injury, two or three joints may suffer, both hips or knees, or hip and some of the vertebræ, hip, knee, and ankle of the same limb, and so on. Even in those cases in which the disease for a long time is confined to a single joint and may have been caused by an injury, other joints, finally, are apt to become {85} affected, often in a symmetrical order. So that it may seem almost general, or at least polyarticular, just as the converse sometimes happens in the general rheumatoid arthritis of long standing, where the disease becomes greatly aggravated in one articulation and produces great deformity and destruction of it, the others remaining as they were.

The symptoms of this partial chronic form are very much those of the general form already described, but there is usually in the early stages less heat, tenderness, and swelling of the affected joint; the pain is less acute, but more abiding, and, with the exception of more or less stiffness or impeded movement in the joint, it may be the only sign of disease present, so that at this stage of the affection it may be taken for simple chronic articular rheumatism. But the disease persists; the voluntary movements become more painful and difficult; slight exercise of the joint is followed promptly by fatigue and aggravation of the pain, and yet the articular surfaces may be pressed together, and flexion and extension be practised, without causing much suffering. Slowly and continuously alterations take place in the affected articulation; with but little heat or redness it enlarges steadily, the soft parts becoming infiltrated and thickened, or effusion taking place into the capsule; the articular surfaces become irregularly depressed by the growth of osteo-cartilaginous rings, osseous nodosities, and stalactiform processes upon them, and these irregularities, together with one or several loose bodies, may be felt in the joint. The enlargement of the articulation becomes more apparent, owing to the wasting of the muscles of the limb; its movements become more and more restricted and difficult, although perhaps not more painful, and are attended with creakings and gratings perceptible to the ear and hand; and at last nearly all movement of the joint may be prevented by the alterations in the shape of the epiphyses, or by the interlocking of the osseous outgrowths, or in rare cases by actual union of the bones. This form constitutes, par excellence, arthritis deformans. In many instances there is little effusion throughout the process, notwithstanding the grave deformity in progress; hence the term dry arthritis. Even the partial form is sometimes more active in its invasion, as when it very rarely succeeds acute or subacute articular or gonorrhoeal rheumatism, or, more frequently, follows an injury.

The duration of the partial form is usually very protracted; it may be ten or twenty years. Exacerbations of the disease occur from time to time, in the intervals of which the patient may be free from pain, although the affected joints are seriously crippled.

The affection is not in itself fatal; the patient may attain an advanced age and die of some intercurrent disease, such as dysentery, pneumonia, cerebral hemorrhage, or other affection incident to old age.

A description of the features presented by partial rheumatoid arthritis affecting the hip (morbus coxa senilis), the shoulder, and other joints rather appertains to works on surgery, and only a glance at the evidences of the disease in the vertebral column (spondylitis deformans) will here be given. When the cervical vertebræ are implicated the power of rotating the head from side to side is usually preserved and is attended with a crackling noise, while the rest of the cervical region is stiff and the head cannot be bent forward; when the dorsal or lumbar vertebræ suffer the back becomes bent, the patient stoops greatly and cannot stand {86} erect, and his body is shortened and more or less twisted. A careful examination will discover not only the great rigidity of the spine, and as it were fusion en masse of its joints, but in these persons the bony outgrowths may be felt. Occasionally the alteration in the vertebræ by compressing the cord or its membranes, or the spinal nerves and ganglia, may produce neuralgic pains in the cervical, dorsal, lumbar, or sciatic nerves, wasting of the muscles, more or less paralysis, and even vasomotor disturbances.

3d. Heberden's nodosities are certainly sometimes the effect of rheumatoid arthritis, implicating chiefly, often solely, the distal joints of the fingers, where it slowly forms two little hard nodules about the size of dried peas upon the side of the articulations. These are notably enlarged and their movements impaired, but pain is seldom experienced, and were it not for deviation of the end of the finger to one side or the knob-like excrescences upon the joints--appearances which much disfigure the hand--patients would not speak of the affection. In many cases these alterations likewise involve, but in a minimum degree, the first phalangeal articulations, and less frequently the metacarpo-phalangeal, and even some of the larger joints--the wrist, knee, or hip, etc. Like the other varieties of rheumatoid arthritis, this form occasionally has a more active invasion than is above mentioned, and may be attended by local pain, heat, and redness, or such symptoms may occur as exacerbations of the chronic disease.

Gout may precede these nodosities, or, as in the case of Charcot's, the latter may precede the former by several years. Finally, Charcot remarks that Heberden's nodosities are "often accompanied by asthma, migraine, neuralgia, especially of the sciatic nerve, and muscular rheumatism, and that these manifestations may alternate with the exacerbations of the disease."

MORBID ANATOMY.--Every component tissue of the articulations exhibits signs of a chronic inflammatory process. In the chronic form affecting the larger joints the synovial membrane is found more or less congested, opaque, and thickened; at the point of its reflection upon the bones its fringes are thickened and injected and their villosities greatly increased in number, length, and thickness, and in extreme instances have been aptly compared to the wool on a sheep's back. The cartilage-cells normally existing in the synovial fringes likewise proliferate and develop into cartilaginous growths, many of which become infiltrated with lime salts, or even ossified, and in this way originate some of the foreign bodies, pedunculated or sessile, which are found in the joints. These may be attached to the synovial fringes, or imbedded in the membrane itself, or set free by rupture of their pedicles. In some examples these neoplasms resemble in size small melon-seeds; in others they form irregular masses, many of which are as large as hazel-nuts.

At the outset there is frequently an increase of synovial fluid, richer in mucine than natural, which lessens considerably in the later stages and becomes a turbid, viscid fluid of a dirty white or reddish-yellow color, containing no pus, but degenerating epithelium and fragments of villosities and cartilage. In many cases, more especially of the partial {87} form, very little effusion into the articulations takes place (arthrite sèche).

The inflammatory irritation excites proliferating and degenerating processes in the cells and basis-substance of the cartilage covering the bones, and the changes described in connection with acute rheumatic arthritis ensue. Those parts of the cartilage covering the bones which suffer pressure in locomotion fibrillate on their surface, and either undergo mucous degeneration, resulting in ulceration and complete absorption, or are thinned and worn away by attrition. In either way the ends of the bones become laid bare. Those portions of the cartilage at the periphery of the joints which escape compression in the erect posture likewise proliferate, but, according to Cornil and Ranvier, in consequence of being covered by the synovial membrane the proliferating elements are retained in situ, instead of escaping into the articular cavity, and develop into actual cartilage, and may ultimately ossify. In this way irregular masses of cartilage (enchondromata) and bone (osteophytes) form around the heads of the bones, enlarging them considerably, altering their shape, encroaching upon the articular cavity as well as extending up the shafts of the bones, and displacing the capsules of the articulations. Similar productions of cartilage sometimes form in the thickened capsules and ligaments, especially in very protracted cases, or these parts become infiltrated with lime salts.

While these processes are going on at the periphery and the centre of the cartilages, in its deeper layers the proliferating cells are undergoing ossification and rendering the ends of the bones very dense and compact, so that under the attrition to which they are exposed by the articular movements they acquire the smoothness, polish, and white aspect of ivory (eburnated). It is probable that the articular ends of the bones participate in this proliferation and development of bone, which increases their compactness and is followed by eburnation. That the bone itself does sometimes play a part in the hyperostosis which is in progress is shown by an increase of an inch in the length of the right ramus of the maxilla over that of the left in Adams's first plate. Forster's and Ziegler's later investigations confirm this view. Nor is the periosteum exempted from the proliferating process which may have long existed in the several articular tissues, as is shown by the considerable enlargement of the diameter of the shaft of the long bones and by the osteophytes which form on the exterior of the vertebræ and often unite several of them together by a series of osseous splints, interfering with the mobility of the spine. Notwithstanding this development of cartilage and bone upon the exterior of the articular extremities, the interior, especially in old people or in very chronic examples of the general form of the disease, or rarely in the partial form, undergoes degeneration and atrophy. The spongy substance becomes rarefied, thinned, and friable (osteoporosis), so that it has been easily cut or crushed, and it is frequently loaded with fat. True ankylosis of the diseased joints is rare, except in the very small articulations when kept at rest; even under this condition fibrous ankylosis is not of frequent occurrence.

Finally, the interarticular fibro-cartilages and ligaments and the long {88} tendon of the biceps degenerate and are absorbed. The muscles in protracted cases suffer simple atrophy, but are sometimes the seat of an interstitial accumulation of fat. Thus far, no lesions of the nerves supplying the diseased joints nor of the spinal cord have been discovered.

ETIOLOGY.--The causation of rheumatoid arthritis is involved in much obscurity--in part, because sufficient attention has not been paid to its clinical varieties. We will examine first the general progressive form which is the more common.

In women it prevails during the child-bearing period. It is probably oftenest developed between twenty and thirty, and continues to occur frequently up to the period of the menopause, fifty, after which it develops comparatively seldom. Of Ord's 33 cases, 10 were between twenty and thirty years; 11 between thirty and forty; 9 between forty and fifty; and 3 between fifty and sixty. Children are not exempt. E. C. Seguin saw three children of the same family suffering from the disease at ages from two and a half to four years. Moncorvo met with an example at two years and a half, Laborde at four, and Charcot at ten. It occasionally begins in both sexes after sixty.

It is pre-eminently a disease of females up at least to fifty; after that it is not infrequent in men, and is then often only partial, at least at first. The most frequent progressive form, however, does often occur even in boys.

It is probably more frequently observed in cold and damp climates than in those of opposite qualities, for cold is regarded as its most common cause. However, it is met with in India and other hot climates. Besnier asserts it is almost unknown in the tropics, but new investigations are needed on this point.

Direct hereditary predisposition exercises but little influence, according to Garrod, and we certainly often see the disease confined to a single member of a large family, although Seguin saw three young children of one family affected with it, their parents being free from any disease. Trastour three times saw the children of women who were afflicted with nodular rheumatism already suffering from articular rheumatism; and Charcot once saw the grandmother, the mother, and the granddaughter successively attacked. At present I have a patient whose mother at fifty-five and maternal grandmother at sixty became subjects of a crippling polyarticular affection; another of my patients informed me that his mother and a young sister were like himself victims of the disease. This direct transmission appears to be rare, judging from my own experience and from the few instances of it mentioned by writers. But very many authorities maintain that simple acute and chronic rheumatism and gout in the parents predispose to rheumatoid arthritis in the offspring (Charcot, Trastour, Besnier). Now, the facts given in support of this opinion are not numerous. Trastour found that out of 45 cases of nodular rheumatism the father or mother were rheumatic in 10 instances, but the form of the rheumatic affection is not stated. Charcot, Besnier, and Homolle, although believers in the doctrine, do not cite an example in proof. However, in Pye-Smith's 27 cases of osteo-arthritis, five stated that rheumatism had occurred in their families. Thus, two fathers {89} had had rheumatic fever, and one was rheumatic, and two sisters of different families had had rheumatic fever. Besides, the father of a sixth and the grandmother of a seventh had had gout.