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[221] and Charcot[222] vouch for an absence of uric
acid in the blood, while Marrot[223] found both this acid and the urea
below the normal quantity in the urine, although the acid increased
notably under baths of high temperature.
[Footnote 221: Reynolds's _Syst. Med._, i. 918.]
[Footnote 222: _Loc. cit._, p. 190.]
[Footnote 223: _Contribution à l'Étude des Rheum. Artic., Examen de
l'Urine et du Sang_, Paris, 1879, p. 42.]
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.[224] 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."
[Footnote 224: _Loc. cit._, 172-175; Besnier, _loc. cit._, 699.]
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.[225]
[Footnote 225: To mention only some of the many sources of cardiac
disease other than rheumatism may be adduced scarlet and other fevers,
extension of inflammation from the pleura or lung and other sources of
local irritation, powerful or oft-repeated muscular efforts, Bright's
disease, senile degeneration, etc.]
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,[226]
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.
[Footnote 226: See Ord's case, II., _Brit. Med. Journal_, i., 1880,
158.]
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,[227] 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."
[Footnote 227: _Loc. cit._, 198.]
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.[228] Forster's[229] and Ziegler's[230] 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.
[Footnote 228: _Illustrations of the Effects of Rheumatic Gout_,
London, 1857.]
[Footnote 229: Forster, _Handbuch der Path. Anat._, p. 1000.]
[Footnote 230: _Virchow's Archiv_, 1877.]
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.[231] 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.[232] Moncorvo[233]
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.
[Footnote 231: _Brit. Med. Jour._, 1880, 156.]
[Footnote 232: _The Med. Record_, London, 1877, 797.]
[Footnote 233: _Du Rheumatisme Chronique Noueux des Enfans_, Paris,
1880.]
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.[234]
[Footnote 234: _Guy's Hospital Reports_, 3d Series, xix. 348.]
The evidence in favor of the doctrine that true articular rheumatism
transmits an hereditary tendency to rheumatoid arthritis does not
appear to be conclusive, although it is highly thought of by those who
regard the latter disease as a variety of rheumatism. Some
considerations of an opposing character deserve mention. Acute
articular rheumatism has very rarely passed continuously into
rheumatoid arthritis, and very rarely has been followed at short
interval by that disease; and in such exceptional cases the antecedent
affection may have been really the acute form of rheumatoid arthritis,
which closely resembles acute articular rheumatism. Trastour,[235]
Vidal,[236] Charcot,[237] and others admit that acute rheumatism can
hardly be placed amongst the antecedents of the rheumatoid affection.
Garrod[238] with some others states that now and then acute rheumatism
acts as an exciting cause of it, which appears to have been Fuller's
view;[239] he had repeatedly known it to commence apparently as a
sequel of acute rheumatism. However, Ord met with a case in which the
lesions of rheumatoid arthritis were present in a typical form in a
patient who had mitral disease as a result of acute rheumatism, the
arthritis having begun as a continuation of the acute attack.[240]
[Footnote 235: _Thèse de Paris_, 1853, p. 41.]
[Footnote 236: _Ibid._, 1855, p. 9.]
[Footnote 237: _Leçons Cliniques_, p. 214.]
[Footnote 238: Reynolds's _Syst. Med._, 1870, i. 920.]
[Footnote 239: _Lib. cit._, 333.]
[Footnote 240: _Brit. Med. Jour._, 1880, i., 158.]
That so common an affection as articular rheumatism should occur in the
family or personal history of a patient the subject of the rheumatoid
arthritis is not improbable; nasal catarrh and many other very common
diseases must be frequent antecedents of the rheumatoid affection, yet
are not causes of it. Much the same remarks apply to the view that gout
in the parents may transmit a tendency to rheumatoid arthritis in the
offspring. The experience of English physicians in this matter is
hardly reliable, owing to the great prevalence of gout in England. In
Canada and many parts of the United States, however, while gout is a
rare disease, rheumatoid arthritis is a common one, and the writer has
not found an intimate relationship to obtain between the two
affections. It is not intended to deny that when the children of
rheumatic or gouty parents fail in health owing to their inherited
constitutional disease, they become liable to rheumatoid arthritis, for
feeble health predisposes to that affection.