It may be here remarked that Landré-Beauvais and Haygarth described more particularly that form of the disease which, beginning in the small joints of the extremities, tends to extend to the larger joints in a centripetal way, and to involve many of them—peculiarities which have given rise to the epithets progressive polyarticular chronic rheumatism, peripheral arthritis deformans, and which is the form of the disease usually described by physicians as rheumatic gout, rheumatoid arthritis, nodular rheumatism, and by the other names just mentioned. On the other hand, Key, Colles, Adams in his earlier paper, and R. W. Smith described the disease as it affects the larger joints, hip, shoulder, or knee, to one or two only of which it may be confined; and as this variety is frequently observed in elderly persons, and in them often involves the hip, it is often spoken of as senile arthritis, malum senile articulorum, morbus coxe senilis, mono-articular arthritis deformans, partial chronic rheumatism, and has been described by surgeons rather than by physicians. However, even when beginning in the hip or shoulder, the disease is apt to involve several of the intervertebral articulations, and not unfrequently to extend to other joints than the one first affected, and even to the peripheral joints. Its progressive and general nature is thus evidenced, whether it invade from the beginning a single large joint or several symmetrical small articulations. Finally, on this topic Charcot has insisted that Heberden's nodi digitorum contributes a special form of the disease under consideration, and proposes to call it Heberden's rheumatism or nodosities.218
218 Lectures on Senile Diseases, Syd. ed., 1881, p. 137.
Rheumatoid arthritis presents the clinical varieties or groupings of phenomena just mentioned, at times quite distinctly appreciable from one another, but sometimes more or less blended, yet even then manifesting in their periods of invasion and early stages an adhesion to all of these typical groupings. Charcot has especially dwelt upon these: 1st, the general or polyarticular and progressive form; 2d, the partial or oligo- or mono-articular form; 3d, Heberden's nodosities.
1st. The symptoms and clinical history of general or polyarticular and progressive rheumatoid arthritis. This is the most common form of so-called chronic rheumatic arthritis, the classical rheumatic gout, or rheumatisme noueux, and it may declare itself, as Garrod and Fuller pointed out, very rarely in an active or acute form, or, as it usually does, in a chronic and insidious form.
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.
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 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.219 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.
219 Lectures on Senile Diseases, Syd. ed., trans. 1881. Figs. 1 and 2, Pl. II., on the hand, give good illustrations of these deformities.
(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.220