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ARTHRITIS AND
ARTHRITIDES
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DEFINITIONS ARTHRITIS - greekArthro (joint)
itis (inflammation)
ARTHRITIDESPlural form
Group of disorders involving the joints
which may be either inflammatory or
non-inflammatory in nature
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TYPES OF ARTHRITIDES Osteoarthritis Rheumatoid Arthritis
Septic Arthritis
Gouty Arthritis Pseudogout
Other Crystal Arthropathies
Psoriatic Arthritis
Juvenile Idiopathic Arthritis
Adult Still Disease
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RHEUMATOID ARTHRITIS RA is the second most common form of
chronic arthritis
Affects approximately 1% of the adultpopulation worldwide
RA is an inflammatory disease of unknown
etiology Potentially crippling disease shortens
survival
Significantly compromises quality of life
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CLINICAL MANIFESTATIONS Gender: Female (3:1 ratio)
Age: Late childbearing years in women
(sixth to eighth decade in men)
Onset: Insidious (builds up over several
weeks to months)
Joint symptoms must persist for at least 6weeks
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CLINICAL MANIFESTATIONS The hands are a major site of involvement
in almost all patients with RA
Distribution: Symmetric small jointsMCP, PIP, and MTP (spares DIP) joints
Systemic: Fatigue, possible weight loss,
occasional low-grade fevers Symptoms: Joint stiffness (worse in
morning), pain, swelling
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HAND INVOLVEMENT Advanced rheumatoid
arthritis with severe
joint deformitiesincluding:
subluxation at the
metacarpophalangeal
joints
swan-neck deformities
(hyperextension at the
PIP joints).
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EXTRA-ARTICULAR
INVOLVEMENT Rheumatoid nodulesseen in approximately one-quarter of patients
with RAalmost exclusively in those patients who are
seropositive for rheumatoid factor
subcutaneous and typically occur on extensor
surfaces and other pressure points or over joints
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RHEUMATOID NODULE Rheumatoid nodules at
the elbow
Rheumatoid nodulesare firm and nontender
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EXTRA-ARTICULAR
INVOLVEMENT Digital infarcts and leukocytoclasticvasculitis - features of RA- associated
small vessel vasculitis CAD - high morbidity and mortality in
patients with RA
Reasons are not entirely clearsignificant risk factors include:
chronic inflammation with an elevated C-reactive
protein
medications used, and sedentary lifestyle
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EXTRA-ARTICULAR
INVOLVEMENTCARDIOVASCULAR Pericardial effusionpresent in 50% by
echocardiography; usually asymptomatic
Constrictive pericarditismay be seen in
long standing cardiac involvement
PULMONARY
Pleural effusion
Diffuse Interstitial Fibrosis
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EXTRA-ARTICULAR
INVOLVEMENT Keratoconjunctivitis sicca (dry eyes) fromsecondary Sjgren syndrome is the most
common ophthalmologic manifestation ofRA
Scleritis also occurs in RA and is usually
painfulSevere involvement can lead to thinning of the
sclera (bluish discoloration as the deep pigment
shows through)
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EXTRA-ARTICULAR
INVOLVEMENTNEUROLOGIC carpal tunnel syndrome
tarsal tunnel syndrome
Subluxations at C1C2 can produce
myelopathy
Rheumatoid nodules in the central nervous
system are rare and usually asymptomatic
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EXTRA-ARTICULAR
INVOLVEMENTFELTY SYNDROME triad of RA, splenomegaly, and neutropenia
seen in patients with severe seropositive
disease
may be accompanied by hepatomegaly,
thrombocytopenia, lymphadenopathy, andfevers
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LABORATORY FINDINGS
AND IMAGING Anemia of Chronic Disease Thrombocytosis
Acute Phase ReactantsElevated
ESR
C-Reactive protein
WBC elevated
(except in Felty syndrome)
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LABORATORY FINDINGS
AND IMAGING Rheumatoid Factorautoantibody directed against the constant (Fc)
region of IgGpositive in about 50% of cases at presentation
additional 2035% of cases become positive in
the first 6 months
not unique to RA and occurs in many other
diseases
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Differentials of Positive
Rheumatoid Factor Rheumatic diseases RA, Sjgren syndrome, SLE
Infections Viral: Hepatitis C, EBV, parvovirus, influenza, others
Bacterial: Endocarditis, osteomyelitis, others
Chronic inflammatory conditions
Liver disease, inflammatory bowel disease
Aging
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LABORATORY FINDINGS
AND IMAGING Anti-CCP - (anticyclic citrullinated peptideantibodies)
present in 6070% of patients with RA atdiagnosis
9098% specific for RA
present in the serum years before RA isdiagnosed
correlate strongly with erosive disease
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LABORATORY FINDINGS
AND IMAGING Typical findings occurlater in the disease course
and include :
bony erosions, cysts
osteopenia, joint space
swelling
calcifications
narrowed joint space
and deformities
separations, fractures.
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DIAGNOSIS American College of Rheumatology
Criteria for Rheumatoid Arthritis
Morning stiffnessaArthritis of three joint areasa
Arthritis of the handsa
Symmetric arthritisa
Rheumatoid nodules
Serum rheumatoid factor
Radiographic changes
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ACR Diagnostic Criteria The first four criteria need to be present for
at least 6 weeks
This time requirement was imposed becausea number of conditions, most notably viral-
related syndromes, can cause self-limited
polyarthritis indistinguishable from RA These also manifest with the presence of RF
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DIFFERENTIAL DIAGNOSIS Viral syndromes,
especially hepatitis Band C, Epstein-Barr
virus, parvovirus,
rubella
Psoriatic arthritis,
reactive arthritis
Tophaceous gout
Systemic lupuserythematosus
Calcium
pyrophosphate disease
Polymyalgia
rheumatica Paraneoplastic
syndromes
Osteoarthritis,especially hereditary
osteoarthritis of the
hand
Sarcoidosis, Lymedisease, rheumatic
fever,
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COMPLICATIONS significant morbidity and premature
mortality
50% of RA patients have had to stopworking after 10 years
Patients who have anti-CCP antibodies,
who are rheumatoid factor positive, or whohave HLA-DR alleles expressing the shared
epitope have a worse prognosis with more
erosions and more extra-articular disease
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COMPLICATIONS Once deformities are found on examination
or erosions on radiography, the damage is
largely irreversible Erosions develop in the majority of patients
in the first 1 or 2 years of disease
the rate of radiographic damage can beaffected by early therapy
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KEYS TO OPTIMAL OUTCOME Early diagnosis
DMARD therapy as early as possible
Strive for remissions in all patients
Recognize and treat comorbid conditions
Cooperation and communication between
primary care physician and rheumatologist
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Juvenile Rheumatoid Arthritis one of the more common chronic illnesses
of childhood
major cause of functional disability and eyedisease leading to blindness
in contrast to adult rheumatoid arthritis
(RA), large joints such as the knees, wrists,and ankles are more prominently involved
than small joints
less frequent complaints of joint pain
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Juvenile Rheumatoid ArthritisThree major types of presentation:
Oligoarthritis (60 percent)
Polyarthritis (30 percent)Systemic onset disease (10 percent)
Subcutaneous nodules and rheumatoid
factor (RF) seropositivity are unusual antinuclear antibody (ANA) seropositivity
is a hallmark of the disease
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Juvenile Rheumatoid Arthritis Characteristic : Inflammation and ankylosis
of the posterior apophyseal joints of the
upper cervical spine Atlantoaxial subluxation
Rare neurologic sequelae
70 to 90 percent of children enter adulthoodwithout serious disability
small percentage of children will have a
recurrence of arthritis as adults
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Criteria for Chronic Arthritis
in Children (ACR and ILAR)ILAR (JIA)ACR (JRA)CHARACTERISTIC73ONSET TYPES1
9
COURSE SUBTYPES
< 16 years< 16 yearsAGE AT ONSET (Arthritis)> 6 weeks> 6 weeksDURATION (Arthritis)YesNoINCLUDES JASYesNoINCLUDES JPsAYesNoINCLUDES IBDNoNoINCLUDES Reactive ArthritisYesYesExclusion of other diseases
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Juvenile Rheumatoid Arthritis Age at onset is characteristically young (1
to 3 years
Girls account for the majority of patientsoverall at least twice as often as boys
Incidence of JRA is 10 to 20 cases per
100,000 children Etiology and pathogenesis of JRA are
unclear
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Juvenile Rheumatoid ArthritisLABORATORY FINDINGS:
Normocytic, hypochromic anemia during
periods of active disease Increased erythrocyte sedimentation rate
(ESR) and C-reactive protein (CRP) levels
Tests for ANAs are positive inapproximately 40 percent of children
Synovial fluid examination is usually
typical of inflammatory fluid
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Juvenile Rheumatoid ArthritisRADIOLOGIC EXAMINATION
Soft tissue swelling
Juxta-articular osteoporosis
Periosteal new-bone formation
Marked bony overgrowth due to
enlargement of the epiphyses occurs at the
interphalangeal joints
Widening of the phalanges from periosteal
new-bone apposition
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Juvenile Rheumatoid Arthritis Juvenile rheumatoid arthritis
in a 2.5year-old girl
Note the extensive periostealchanges particularly in the
fourth metacarpal and in all
the proximal phalanges.
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Juvenile Rheumatoid ArthritisTREATMENT:
Conservative management to control the
clinical manifestations of the disease and toprevent deformity
family-centered, community-based, and
coordinated Treatment in most children is prolonged
over many years
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Juvenile Rheumatoid ArthritisMEDICAL MANAGEMENT:
NSAIDS Intravenous IgG
Methotrexate Sulfasalazine
Glucocorticoids
Etanercept
Hydroxychloroquine
Gold compounds
D-Penicillamine
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PSORIATIC ARTHRITIS chronic inflammatory arthritis that is
commonly associated with psoriasis
At least 5% of patients with psoriasisdevelop psoriatic arthritis
etiology of psoriatic arthritis is unknown
As in the pathogenesis of many otherautoimmune disorders, an infectious trigger
has been suspected
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CLINICAL MANIFESTATIONS Majority of patients with psoriatic arthritis
present with an oligo- or monarthritis
Often the DIP joints become stiff, swollen,and tender in an asymmetric fashion
When present, involvement of the DIPs
helps to distinguish psoriatic arthritis fromrheumatoid arthritis.
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PSORIATIC ARTHRITIS
DIP involvement
Nearly always associated
with nail psoriasis
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CLINICAL MANIFESTATIONS Other joints that are affected by psoriatic
arthritis include the knees, hips, andsternoclavicular joints
Most patients will progress to additional
joint involvement in the absence ofeffective treatment
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CLINICAL MANIFESTATIONS Dactylitis, or "sausage digit"
complete swelling of a single digit in the hand
or footcommon in psoriatic arthritis, occurring in one-
third to one-half of patients at some point in
their disease
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Dactylitis diffuse swelling of the
entire digit likely due
to a combination of
both arthritis and
tenosynovitis.
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CLINICAL MANIFESTATIONS Enthesitis
an inflammatory process at the site of the
insertion of tendons into boneoccurs in up to 40% of psoriatic arthritis
patients
soft tissue swelling usually accompanied by
tenderness to palpation and sometimes by
overlying erythema and warmth
common site for enthesitis is the insertion of the
Achilles tendon into the calcaneus
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CLINICAL MANIFESTATIONS Skin and nail changes
All forms of psoriasis are associated with
arthritisclassic psoriasis vulgaris is seen most
frequently
erythematous plaques that produce scaling with
scratching
nail involvement is common in psoriatic
arthritis and includes: ridging, pitting,
onycholysis, and hyperkeratosis
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CLINICAL MANIFESTATIONS
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CLINICAL MANIFESTATIONSSpondyloarthropathy
involvement of the sacroiliac joints and
axial skeleton is less common thanperipheral arthritis
Inflammation of the sacroiliac joints
(sacroiliitis) in psoriatic arthritis is usuallyunilateral
common site of skeletal involvement in
psoriatic arthritis is the cervical spine
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LABORATORY FINDINGS There are no laboratory tests diagnostic for
psoriatic arthritis
20% of patients have hyperuricemia acute phase reactants such as the C-reactive
protein and the erythrocyte sedimentation
rate may be elevated Synovial fluid analysis reveals
inflammatory fluid
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LABORATORY FINDINGSRheumatoid Factor
Patients with psoriatic arthritis usually do
not have rheumatoid factor But up to 10% of patients with psoriatic
arthritis will test positive
Antibodies to cyclic citrullinated peptideshave only rarely been reported in patients
with psoriatic arthritis
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IMAGING STUDIES The most common radiographic findings in
psoriatic arthritis are:
joint space narrowing and erosions involvingthe distal interphalangeal and proximal
interphalangeal joints
asymmetric, paralleling the pattern of the
clinical arthritis
metacarpophalangeal joints and wrists are
usually spared, in contrast to rheumatoid
arthritis
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IMAGING STUDIES Arthritis mutilans
describes the end stage
of the destructive
process
loss of bony
architecture allows
complete subluxationand telescoping of the
involved digit
Occurs in about 5 %
of cases
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TREATMENT NSAIDS
Disease modifying anti-rheumatic drugs
methotrexate, sulfasalazine, azathioprine,antimalarials ( hydroxychloroquine), and
cyclosporine
Biological TherapiesTumor necrosis factor- (TNF-)
Alefacept
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Juvenile Idiopathic Arthritis (JIA) Refers to the group of disorders that cause
chronic arthritis in children
Chronic synovitis is present in any one jointfor more than 6 weeks in a child less than
16 years of age
Chronicity of the joint involvement in JIAdistinguishes this group of disorders from
the many short-term causes of joint pain and
swelling that can occur in childhood.
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Juvenile Idiopathic Arthritis (JIA) Incidence of 122 children per 100,000
Prevalence of anywhere from 8150 per
100,000 Most common chronic rheumatic disease of
childhood
Significant cause of both short-term andlong-term disability
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Types of JIA Systemic onset JIA (SOJIA)
Oligoarticular (previously known as
pauciarticular) JIA Polyarticular rheumatoid factor positive JIA
Polyarticular rheumatoid factor negative JIA
Juvenile psoriatic arthritis
Enthesitis-related arthritis
"other."
Oli ti l JIA
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Oligoarticular JIA
Occurs in very young children Asymmetric pattern of joint involvement is
common
Fewer than five joints total can be affected Morning stiffness is a prominent finding
Affected joint is often swollen and quite
large, the child frequently has less pain thanone would anticipate
Knees are the most commonly involved
joints
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Polyarticular JIA Affects children whose arthritis begins in
five or more joints at onset
Rheumatoid factor test is a usefulprognosticator
Rheumatoid factor positive patients are
usually teenage girls with symmetric small-joint arthritis involving hands and feet
These girls may have rheumatoid nodules,
with aggressive erosive joint disease
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Polyarticular JIA Polyarticular rheumatoid factor negative
JIA affects younger children
More girls are affected than boys
Arthritis may or may not be symmetric Usually affects large joints predominantly
no associated extra-articular features and
uveitis is rare
Affected patients have active arthritis for
many years without erosive change on x-
ray.
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Systemic onset JIA (SOJIA) High daily spiking fever
Evanescent rash present during febrile
episodes that disappears when thetemperature is back to normal
Severe myalgias
Polyserositis Affects children and adults at any age,
without preference for girls or boys.
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Systemic onset JIA (SOJIA) Hallmarks of this disorder are the daily
spiking fever in a "rabbit ears" pattern in
association with an evanescent, salmon-colored, macular rash on the trunk and
extremities
Marked leukocytosis, very highsedimentation rates, and elevated ferritin
levels are the rule in this disease
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Juvenile Psoriatic Arthritis
Presents with any of the following signs and
symptoms:
sacroiliitisdistal interphalangeal joint synovitis
dactylitis, "sausage" digit
nail pittingpsoriatic rash
positive family history of psoriasis
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Enthesitis-related JIA
Patients with juvenile onset spondylitis,
reactive arthritis, and the arthritis associated
with inflammatory bowel disease Boys from 8 years of age onward are most
often affected
Pain, stiffness, and loss of flexibility in thespine, in addition to synovitis of peripheral
joints, predominantly in lower extremities.
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Enthesitis-related JIA
Heel pain, rash including pyoderma
gangrenosum or ulcers, and acute, painful
uveitis can occur HLA-B27 gene can be found in many
patients in this category, but is not
diagnostic.
Adult onset Still disease (AOSD)
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Adult-onset Still disease (AOSD) Multisystem inflammatory disease thattypically begins with a sore throat
Fever that spikes in "rabbit ears" pattern
with daily return to normal.
Salmon-colored macular rash only
occurring with fever.
Arthritis, splenomegaly, pleuritis,pericarditis, and marked leukocytosis
common.
Pharyngitis often the initial symptom
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Adult-onset Still disease (AOSD) Other constitutional symptoms soon follow,
including profound fatigue, weight loss, and
anorexia Malignancy and infectious causes of these
symptoms must be excluded
cause of AOSD has yet to be identified
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Adult-onset Still disease (AOSD)Laboratory Work-up:
No definitive lab test for AOSD
Marked leukocytosis (>15,000/L) with apredominance of neutrophils (>80%)
Markedly elevated erythrocyte
sedimentation rate (>90) Marked elevation of the serum ferritin
(above 3000 mg/mL) is seen in over 70% of
AOSD patients
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Arthritis Associated with
Systemic Disease
Arthropathy of Acromegaly
Excessive production of growth hormone by
an adenoma in the anterior pituitary glandProliferation of cartilage, periarticular
connective tissue, and bone
Results in several musculoskeletalabnormalities, including osteoarthritis, back
pain, muscle weakness, and carpal tunnel
syndrome.
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Arthropathy of Acromegaly
Arthropathy resembling osteoarthritis is a
common feature
Affects most often the knees, shoulders,hips, and hands
Single or multiple joints may be affected
Carpal tunnel syndrome occurs in about halfof patients
median nerve is compressed by the excessive
growth of connective tissue in the carpal tunnel
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Arthropathy of Hemochromatosis Disorder of iron storage
Excessive amounts of iron are absorbed
from the intestine Leads to iron deposition in parenchymal
cells, which results in tissue damage and
impairment of organ function
Arthropathy of
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Arthropathy of
Hemochromatosis Arthropathy is an osteoarthritis-like
disorder
Affects the small joints of the hands,
followed later by larger joints such asknees, ankles, shoulders, and hips
The second and third metacarpophalangeal
joints of both hands are often the first jointsaffected
provide an important clue to the possibility of
hemochromatosis.
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Arthropathy of
Hemochromatosis
Treatment of hemochromatosis is repeated
phlebotomy
Little effect on the arthritis Administration of acetaminophen and
nonsteroidal anti-inflammatory drugs
(NSAIDs)
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Hemophilic Arthropathy
Sex-linked recessive genetic disorder
characterized by
the absence or deficiency of factor VIII(hemophilia A, or classic hemophilia)
or the absence of factor IX (hemophilia B, or
Christmas disease)
Hemophilia A is by far the more common
type, constituting 85% of cases
Hemophilic Arthropathy
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Hemophilic Arthropathy Spontaneous hemarthrosis is a commonproblem with both types of hemophilia and
can lead to a chronic deforming arthritis
Hemarthrosis is not common in otherinherited disorders of coagulation, such as
von Willebrand disease or factor V
deficiency
The frequency and severity of hemarthrosis
are related to the degree of clotting factor
deficiency
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Hemophilic Arthropathy Hemarthrosis appears after 1 year of age,
when the child begins to walk and run
Joints most commonly affected are theknees, ankles, elbows, shoulders, and hips
Small joints of the hands and feet are
occasionally involved Characterized by warm, tensely swollen,
and painful joint
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Hemophilic Arthropathy The patient holds the affected joint in
flexion and guards against any movement
Blood in the joint remains liquid because ofthe absence of intrinsic clotting factors
Resorbed over a period of a week or longer
Joint function usually returns to normal orbaseline in about 2 weeks
Recurrent hemarthrosis leads to the
development of a chronic arthritis
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Hemophilic ArthropathyTREATMENT:
Immediate infusion of factor VIII or IX at
the first sign of joint or muscle hemorrhage patient is placed at bed rest
Analgesic doses of an NSAID and local
icing may help with the pain
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