arthritis and arthritides

7/28/2019 Arthritis and Arthritides

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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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