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Rhematoid Disease |
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Rheumatoid disease is a multisystem connective
tissue disease of which the dominant effects
are on the joints, giving rheumatoid arthritis; The disease is more common in
women
than in men, and there is a genetic predisposition, although the cause or causes
of this remain unknown.
Rheumatoid disease also affects the skin, lungs, blood vessels, eyes, and the
haemopoietic and
lymphoreticular systems .
The treatment for rheumatoid disease may itself cause
pathology,
e.g. steroid-induced osteoporosis, analgesic- related ulceration of stomach, and
drug-induced renal disease.
Rheumatoid disease may cause primary disease, or damage may be caused by drug
treatment.
Rheumatoid arthritis is a symmetrical polyarthritis,characterized by the
destruction of articular cartilage
and its replacement by chronic inflammatory pannus.
In most cases, the disease first attacks the finger joints, followed,
in order of frequency, by the metatarsophalangeal joints and the joints of the
ankles, wrists, knees,
shoulders, elbows and hips. However, the disease may also affect joints such as
the
temporomandibular joint, the synovial joints of the spine(particularly the upper
cervical spine),
and occasionally the joints of the larynx.
The early pathological change in rheumatoid arthritis is rheumatoid synovitis.
The synovium is swollen and
shows a villous pattern. There is a great increase in chronic inflammatory cells
(mainly lymphocytes and plasma cells) in the synovial stroma, often with an
exudate comprising fluid, which
produces an effusion in the joint space, and fibrin which is deposited on the
synovial surface. Soft tissue
swelling from synovial inflammation can be marked.
With time, there is articular cartilage destruction; vascular granulation tissue
grows across the surface
of the cartilage (pannus) from the edges of the joint, and the articular surface
shows loss of
cartilage beneath the extending pannus, most marked at the joint margins.
The inflammatory pannus causes focal destruction of bone. At the edges of the
joint there is osteolytic
destruction of bone, responsible for 'erosions' seen on radiographs. This phase
is associated with joint deformity.
The characteristic deformity and soft tissue swelling associated with
long-standing rheumatoid disease of the hands.
Skin is involved in rheumatoid disease by the formation of rheumatoid nodules or
the presence of vasculitis.
The most frequent skin lesion in rheumatoid disease is the rheumatoid nodule, a
subcutaneous firm
nodule located usually over the extensor aspect of the forearm but also found
occasionally in the skin
overlying other bony prominences. The rheumatoid nodules are composed of
extensive areas of degenerate
collagen that are surrounded by a giant cell granulomatous reaction.
These nodules are seen in approximately 25% of cases of rheumatoid arthritis,
particularly in
patients with severe arthritic disease and other visceral involvement such as
pulmonary fibrosis.
Occasionally, the nodules can antedate the development of arthritis.
The other important skin manifestation is the development of vasculitis. This is
either
an acute neutrophilic vasculitis presenting with purpura and occasional foci of
ulceration,
or is a lymphocytic vasculitis producing a more low-key erythematous patchy
rash. The cutaneous
vasculitis may be part of a more generalized vasculitis affecting other organs,
particularly
lymphocytic vasculitis.
Rheumatoid disease can cause dry-eye syndrome and degeneration of the sclera
In rheumatoid disease, both the lacrimal and mucous glands may be affected by
lymphocytic
inflammation, leading to the destruction of the glands and to dry eyes (keratoconjunctivitis
sicca).
The lack of tears leads to secondary inflammation of the cornea.
The degeneration of collagenous tissue in the eye, analagous to that which is
present in rheumatoid
nodules, may occur, causing scleritis.
In severe cases, this progresses to cause perforation of the globe, termed
scleromalacia perforans.
Pulmonary involvement in rheumatoid disease causes interstitial fibrosis.
The most common form of pulmonary involvement in rheumatoid disease is
interstitial pneumonitis
and fibrosing alveolitis, which leads, eventually, to a pattern of interstitial
pulmonary fibrosis
that is very similar to that seen in progressive systemic sclerosis, 'honeycomb
lung'.
In addition, patients may develop lesions that are very similar to the
subcutaneous
rheumatoid nodule, both within the lung and on the pleural surfaces.
Intrapulmonary rheumatoid granulomas are particularly common in patients who
already have
industrial lung disease due to the inhalation of various types of silica; the
association of
coalminers' lung with rheumatoid granulomas in sero-positive miners is called
Caplan's syndrome.
Anaemia of chronic disorders is common in rheumatoid disease, and a minority of
patients
develop hypersplenism or lymphadenopathy Anaemia is very common in rheumatoid
disease,
usually being of the normocytic hypochromic type that is characteristic of
chronic disease.
Despite the hypochromia there is no regular iron deficiency, but there
is probably a defect of iron incorporation and utilization. The bone marrow may
show
follicular lymphocytic aggregates.
Lymph nodes may be enlarged in rheumatoid disease as a result of follicular
hyperplasia.
Enlargement of the spleen is usual, but it is rarely great enough to be palpable
except in
cases of Felty's syndrome, in which splenomegaly, lymphadenopathy, anaemia and
leukopenia are present
in a patient with seropositive rheumatoid arthritis; there is an increased
susceptibility to infection,
and sepsis is an important and common cause of death in these patients. |
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