conditions
characterized by the presence of crystals in
and
around the joints, bursae and tendons.
Three clinical disorders in particular are
associated with
this phenomenon:
Gout: urate crystal deposition disorder.
Pseudogout: calcium pyrophosphate
dihydrate
(CPPD) deposition disease.
Gout
This is a disorder of purine metabolism characterized
by hyperuricaemia, deposition of monosodium urate
monohydrate crystals in joints and periarticular tissues
and recurrent attacks of acute synovitis.
Hyperuricemia caused by
Overproduction
Underexcretion
No Gout w/o crystal deposition
Pathology
The term hyperuricaemia is therefore
generally reserved for individuals with
a serum urate concentration which is
significantly higher than that of the
population to which they belong (more
than two standard deviations above
the mean); this is about 0.42 mmol/L
for men and 0.35 mmol/L for women
in Western Caucasian peoples.
Clinical gout
Urate crystals are deposited in minute clumps in
connective tissue, including articular cartilage; the
commonest sites are the small joints of the hands
and feet.
asymptomatic
acute inflammatory reaction.
common sites are around the
1.metatarsophalangeal joints of the big toes
2.the Achilles tendons
3.the olecranon bursae
4.the pinnae of the ears.
Classification
Primary gout (95% of cases) occurs in the
absence of any obvious cause and may be due
to constitutional under-excretion (the vast
majority)
or over-production of urate.
Secondary gout (5%) results from prolonged
hyperuricaemia due to acquired disorders such
as myeloproliferative diseases, administration of
diuretics or renal failure.
Clinical features
diagonsis
Chronic gout
Recurrent acute attacks may eventually
merge into polyarticular gout.
Tophi may appear around joints, over the
olecranon and in the pinna of the ear.
A large tophus can ulcerate and discharge
its chalky material.
Joint erosion causes chronic pain, stiffness
and deformity.
Renal lesions include calculi and
parenchymal disease.
Treatment
Interval therapy
simple measures such as losing weight, cutting out alcohol
and eliminating diuretics.
Urate-lowering drugs are indicated if acute attacks recur at
frequent intervals, if there are tophi or if renal function is
impaired. They should also be considered for
asymptomatic hyperuricaemia if the plasma urate
concentration is persistently above 6 mg/dL (0.36 mmol/L).
. Uricosuric drugs (probenecid or sulfinpyrazone) can be
used if renal function is normal.
However, allopurinol, a xanthine oxidase inhibitor, is
usually preferred, and for patients with renal complications
or chronic tophaceous gout allopurinol is definitely the
drug of choice.
Surgery
With prolonged urate-lowering therapy, adjusted
to maintain a normal serum uric acid level (less
than 0.36 mmol/L), tophi may gradually dissolve.
However, ulcerating tophi that fail to heal with
conservative treatment can be evacuated by
curettage; the wound is left open and dressings
are
applied until it heals.
Calcium pyrophosphate
dihydrate deposition
CPPD deposition encompasses three
overlapping conditions:
(1) chondrocalcinosis the appearance
of calcific material in articular cartilage
and menisci;
(2) pseudogout a crystal-induced
synovitis; and
(3) chronic pyrophosphate arthropathy
a type of degenerative joint disease.
Asymptomatic chondrocalcinosis
Calcification of the menisci is common in elderly
people and is usually asymptomatic.
When it is seen in association with osteoarthritis
this does not necessarily imply cause and effect;
both are common in elderly people and they are
bound to be seen together in some patients.
X-rays may reveal chondrocalcinosis in other
asymptomatic joints.
Chondrocalcinosis in patients under 50 years of
age should suggest the possibility of an underlying
metabolic disease or a familial disorder
Diagnosis
Pseudogout must be distinguished from other acute inflammatory
disorders such as gout and infection. Diagnosis rests on identifying the
characteristic crystals in synovial fluid
Chronic pyrophosphate arthropathy can resemble other types of
polyarticular arthritis and will come into the differential diagnosis of
rheumatoid arthritis and polyarticular osteoarthritis
Metabolic disorders such as hyperparathyroidism, haemochromatosis and
alkaptonuria
may be associated with calcification of articular cartilage and
fibrocartilage as well as joint symptoms.
It is important to exclude such
generalized disorders before labelling a patient as just another case of
chondrocalcinosis.
Gout
Pseudogout
Smaller joints
Large joints
Pain intense
Pain moderate
Joint inflamed
Joint swollen
Hyperuricaemia
Chondrocalcinosis
Calcium
pyrophosphate
crystals
pyrophosphate crystals
(b) are rhomboidshaped, slightly
smaller than urate
crystals and showing
weak positive
birefringence.
Pathology
Minute BCP crystals are deposited in articular cartilage and in
damaged tendons and ligaments
most notably around the shoulder and knee.
The deposits grow by crystal accretion and may be detectable by xray.
In long-standing cases the calcific deposit has a chalky consistency.
The mini-tophus may be completely inert, but in symptomatic cases
it is
surrounded by an acute vascular reaction and inflammation.
Crystal shedding into joints may give rise to synovitis.
More rarely this is complicated by the development of a rapidly
destructive, erosive arthritis; bits of articular cartilage and bone
or fragments of a meniscus may be found in the
synovial cavity.
X-rays
With periarthritis, calcification may be seen in tendons or ligaments
close to the joint, most commonly in the rotator cuff around the
shoulder.
Articular cartilage and fibrocartilaginous menisci and discs never
show the type of calcification seen in CPPD deposition disease, but
loose bodies may be seen in synovial joints.
Erosive arthritis causes loss of the articular space, with little or no
sclerosis
or osteophyte formation.
The typical picture of rapidly destructive arthritis is one of severe
erosion and destruction of subchondral bone.
In advanced cases the joint may become unstable and, eventually,
dislocated.
Treatment
Acute periarthritis should be treated by rest and NSAIDs.
Resistant cases may respond to local injection of
corticosteroids; this treatment should be used only to
weather the acute storm repeated injections for lesser pain
may dampen the repair process in damaged tendons.
Persistent pain and tenderness may call for operative
removal of the calcific deposit or decompression of the
affected tendon or ligament.
Erosive arthritis is treated like osteoarthritis.
However, rapidly progressive bone destruction calls for early
operation: in the case of the shoulder, synovectomy and softtissue repair; for the hip, usually total joint replacement.