Rheumatoid Arthritis

Rheumatoid Arthritis
• This is perhaps the most common form of
inflammatory arthropathy seen in India. Among
adult population below the age of 50 years this is
the most common form of arthritis.
• The exact cause and triggering factor are not
fully known.

• Several evidences point to autoimmune mecha-

nisms as the basic pathological processes and
viral infections as triggering factors.
• There is increased frequency of HLA DRW4, DR4 and
DR1 in these subjects. HLA DR4 is also correlated
with the severity of the disease.

• Genetically predisposed individuals produce IgM or

IgG antibodies on microbial or other forms of
challenge. These antiglobulin antibodies which are
known as rheumatoid factor (RF).
Pathology
• Articular lesions: Most marked changes are seen in the joints.
The earliest change :
• the synovial membrane which shows congestion,
infiltration by mainly CD4+ lymphocytes, plasma cells and
macrophages. Synovial effusion occurs.
• Muscles around the joint also show mononuclear cell
infiltration.
• Synovium shows fibrinoid degeneration surrounded by
infiltration with fibroblasts and mononuclear cells.
• The synovial membrane is thickened, hyperemic, and
edematous. It proliferates to form villi which fill the joint.
• Later, vascular proliferation invades the
articular cartilage to form a pannus.
• Irreversible destruction of cartilage occurs in the
later stages of the disease.

• The proliferated synovial membrane which

invades cartilage, ligaments and subchondral
bone centripetally liberates proteolytic enzymes
which aggravate the destructive process.
•Extra-articular lesions: Other tissues are
affected to varying degrees. Basic pathology
is the same as in the synovium. Lesions are
seen in the skin, lungs, heart, liver, nervous
system, and eyes.
 Clinical Features
• Females are affected more than males in a ratio of
4:1.

• The disease is more common in the fourth and

fifth decades.
• The disease passes through different stages :

• 1. Onset to 6 months -early disease

• 2. 6 months to 3 years -established disease
• 3. Above 3 years -advanced disease.
•Early symptoms are non-specific and they
include undue fatigability, weight loss, poor
appetite, transient myalgias, and paresthesia.
 Articular involvement:
• The onset is generally insidious and the disease presents as
a chronic symmetrical poly- arthritis.
• include: (1) acute polyarthritis,
• (2) oligoarthritis,
• (3) acute monoarticular arthritis,
• (4) chronic monoarticular arthritis, and
• (5) systemic disease with fever, sweating, leukocytosis and
pleural effusion in addition to arthritis.
 Swan neck deformity
• hyperextension of the
proximal
interphalangeal joints
and flexion of the
distal interphalangeal
joints.
boutonnière or button hole
• flexion of the proximal
interphalangeal joint
and hyperextension of
the distal
interphalangeal joint
deformity.
 dropped fingers
• The extensor tendons
may undergo
attrition, these result
in loss of extension of
the fingers
Hitch-hiker’s thumb

•The thumb
may show a
‘Z’-shaped
deformity
 Baker’s cysts

• Baker’s cysts are tense cysts developing in the

popliteal fossae as a result of collection of
synovial fluid. These may occasionally rupture
giving rise to a painful and tender swelling on
the calf. Lateral subluxation of the knee joint is
also very common.
 Hammer toe
• flexion at the proximal
interphalangeal joint and
hyperextension at the
metatarsophalangeal
joint.
Hallux valgus

• lateral deviation of
the big toe
atlantoaxial subluxation.
• the atlantoaxial joint the transverse ligament of the
atlas may be weakened leading to atlantoaxial
subluxation.
• This leads to pain in the neck and pain referred to
the temporal and retro-orbital regions. There may
also be a “clunking sound” in the neck on flexion. It
is dangerous to manipulate the neck to elicit this
sign.
Temporomandibular arthritis

•pain on mastication.
 Extra-articular Manifestations
• Skin: Painless and nontender subcutaneous nodules
ranging in size from a few millimeters to a few
centimeters develop around the extensor aspects of
the elbow and other subcutaneous bony surfaces
and over tendons in 20- 30 percent of cases. They
are almost always associated with a positive
serology.
Eyes

•Scleritis is common
 Respiratory system

•Caplan’s syndrome is a special phenomenon

occurring in subjects with rheumatoid
disease who develop pneumoconiosis. Large
coalescing nodules develop in the lungs.
These are demonstrable by X-rays
 Cardiovascular system

•Lesions include pericarditis, aortic

regurgitation, and conduction
defects.
Nervous system
• carpal tunnelsyndrome and tarsal tunnel
syndrome
• Mononeuritis multiplex
• foot drop and wrist drop may develop.
Renal Lesions

• Type 1 renal tubular acidosis

• Secondary amyloidosis is a late complication
 Laboratory Investigations

• ESR may exceed 100/mm hours.

• Normochromic normocytic anemia, iron

deficiency anemia
• rarely hemolytic anemia may be present in
the acute phase.
Serology

• Rhumatoid factor: Antibodies in rheumatoid

disease, especially the IgM
• Rheumatoid factor is positive in 80% of
adults with rheumatoid arthritis.
• In 20% of cases antinuclear antibodies may
be demonstrable.
 ACCP

• Anti-citrullinated protein antibodies are

autoantibodies that are directed against peptides
and proteins that are citrullinated. They are
present in the majority of patients with
rheumatoid arthritis.
 Synovial fluid aspiration

•This reveals a turbid fluid with low viscosity

and poor mucin clot.
•The white cell count in the fluid exceeds
1500/cmm. The cells are mostly neutrophils
even though the synovial membrane is not
infiltrated by them.
Radiology
• Grade I:Soft tissue swelling which indicates synovitis with or
without juxta-articular osteoporosis which indicates active
inflammation.
• Grade II: Narrowing of joint space due to cartilage destruction.
• Grade III:Erosions which may be of 2 types:
i. Surface erosion at joint margins,
ii. Cystic erosion of the bone shaft.
• Grade IV: Marked irregularity of articular surfaces with
subluxation and secondary degenerative changes. Bony ankylosis
occurs only very rarely
Diagnosis :The diagnosis is to be made on
clinical examination and laboratory criteria.
 Differential diagnosis:
• rheumatic arthritis
• psoriatic arthritis
• Gout
• Osteoarthritis
• Allergic arthritis
• syphilitic arthritis,
• gonococcal arthritis,
• Reiter’s syndrome and
• ankylosing spondylitis.
PROGNOSIS

•In general the disease tends to become

recurrent and chronic and in many
cases it leads to considerable disability
and deformity. Severe rheumatoid
arthritis shortens life.
 Management
• Bed rest is essential during the acute phase of the disease.
• Local rest is ensured by splints which reduce muscle
spasm and deformities.
• Physiotherapy to relieve muscle spasm and maintain joint
mobility is an essential component of management during
all stages of the disease.
• The dose of fish oils required for the beneficial effects
varies from 10-20 g daily.
 Variants of Rheumatoid Arthritis
• Several arthritic syndromes occur which show some
resemblance to rheumatoid arthritis.

These include:
1. Juvenile idiopathic arthritis (JIA)
2. Felty’s syndrome.
3. Sjögren’s syndrome, and
4. Palindromic rheumatism.
Juvenile Idiopathic Arthritis (JIA)
• It is the most common cause of chronic
arthritis in childhood. This term encompasses
all idiopathic arthritis affecting children
below 16 years of age and lasting for more
than 6 weeks.
Types

•Oligoarticular
•Polyarticular
• In the oligoarticular types lower limb joints-knees,
hips, ankles and tarsal joints are affected
asymmetrically.

• In the polyarticular type both small and large

joints are affected, usually in a symmetrical
manner.
clinical features
• The clinical features may be confined to the skeletal
system, but in 10% the onset and course are characterized
by systemic manifestations such as high fever (>38.5°C)
transient erythematous rash, generalized
lymphadenopathy, hepatosplenomegaly, anemia and
weight loss.

• Fever is intermittent with periods of normal temperature in

between, during which the child appear to be normal.
• The serious complications include macrophage
activation syndrome characterized by onset of
thrombocytopenia, anemia, liver dysfunction
and rapid downhill course which may end
fatally.
 Felty’s Syndrome
• This is seen in older age groups. In addition to the classic
features of seropositive rheumatoid arthritis, splenomegaly
and neutropenia also occur.

• Splenomegaly may lead to hypersplenism. Splenectomy

may have to be considered to correct hypersplenism, in
addition to treatment for the rheumatoid state.
 SJÖGREN’S SYNDROME (SS)
• This is a chronic inflammatory autoimmune disease
characterized by mixed cellular infiltration of the
exocrine glands, particularly the lachrymal and salivary
glands- a form of autoimmune exocrinopathy
(autoimmune epithelitis).
• This results in dryness of the eyes (xerophthalmia),
dryness of the mouth (xerostomia) and frequently,
dryness of the nose, throat and vagina. This condition
predisposes to increased risk of mucosa- associated
lymphoid tissue lymphoma (MALT lymphoma).
• Viral infections may trigger off the
condition.

• ANAs or rheumatoid factor autoantibodies

will be present in the serum.
 Clinical features
1. Ocular dryness and consequent symptoms
2. Oral dryness with consequent symptoms
3. Arthralgias in 75%, arthritis in 10%
4. Skin manifestations- annular erythema on the face andtrunk
5. Vascular involvement- leading to Raynaud’sphenomenon in 35-
50%
6. Gastrointestinal symptoms -dysphagia, atrophicgastritis7. Renal
tubular acidosis
8. Hypothyroidism in 10-15%
Diagnosis
• Schirmer’s test is positive.
This test is based on the wetting of a piece of
filter paper by tears when applied to the
palpebral conjunctiva. It is positive if the length
of the wetting is < 5 mm in 5 minutes.
 Palindromic Rheumatism
• In this condition repeated attacks of joint pains,
redness and swelling occur. The attacks occur
suddenly within hours and may affect one joint
usually. The affected joint shows signs of
inflammation. These last for a few days and subside
without any residual lesions.
• The ESR is raised during the attacks and remains
high even during the intervals.

• After varying periods of time, typical rheumatoid

arthritis supervenes in many cases, whereas
spontaneous remission occurs in some.