Chapter 14: The Arthropathies

Causes of Joint Pain

The Arthropathies
Lab Testing for the Arthropathies Articular Disorders
Affecting the Heel Synovial Fluid Analysis
Charcot Foot
THE ARTHROPATHIES
Causes of Joint Pain: A Summary
1. Joint disease: the arthropathies

2. Bone disease: fractures, primary or secondary tumors, osteochondritis,

osteomyelitis, etc.

3. Soft-tissue lesions: sprains and strains, tenosynovitis, overuse

syndrome, direct trauma, bursitis, "soft tissue rheumatism"

4. Arthralgia: defined as joint pain in the absence of objective joint disease,

seen with
a. The Arthropathies- either preceding the development of local signs or in
some conditions in which there may be no local signs. Important examples
are polymyalgia rheumatica and temporal arteritis, SLE, and polyarteritis
nodosa.
b. Infections- particularly viral and rickettsial
i. Viral: influenzia (25% cases), glandular fever, psittacosis, yellow fever,
sandfly fever
ii. Rickettsial: all types of typhus
iii. Bacterial: septicemia, subacute bacterial endocarditis, thyphoid
salmonella, gonorrhea
iv. Spirochaetal infections: secondary syphilis, leptospirosis, relapsing fever
v. Protozoan/metazoan: kala-azar and other tropical diseases
c. Drugs- immunizations, serum sickness
d. Protein abnormalities- e.g. mixed IgG IgM cryoglobulinemia

5. Referred pain: particularly common in the knee due to hip and prostate
disease and in the shoulder due to some abdominal conditions, esophageal
conditions, cardiac conditions, neurologic conditions neurological conditions
and pulmonary conditions

6. Psychogenic: Joint pain may be a manifestation of psychological

disturbance and "rheumatism" may become a source of complaint in the
anxious or neurotic patients

The Arthropathies
1. Anklosing Spondylitis (Marie Strumpell Disease): a chronic condition
of the spine (bamboo spine) and sacro-iliac joints in which early
inflammatory changes are followed by progressive restriction of spinal
movement, with radiological calcification of spinal ligaments (seronegative
disorder).
a. Signs: chest expansion reduced (< 5cm), limited spinal movements,
tender bony points (heel)
b. Symptoms: gradual onset of low backache and/or pain in buttocks
c. X-ray: shows bamboo spine, and "whiskering" of ischial tuberosities
d. Labs: ESR >, mild anemia, (-) latex fixation, synovial fluid shows
inflammatory/neutrophils
e. Tx: Exercises, NSAIDS

2. Arthrogryphosis Multiplex Congenita: a rare congenital disorder

characterized by stiff, deformed joints and muscle wasting.
a. Clinical features: painless deformities (equinovarus feet, flexion
deformities of knees), stiff joints
b. Tx: correction of deformities, surgical and nonsurgical

3. Avascular necrosis: a group of conditions in which bone infarction is not

associated with sepsis but is usually due to interference with blood supply,
either by abnormalities of the vessel wall such as arteritis, pressure on the
vessels from outside, trauma, thrombosis, or embolism. Clinical disease
results only when the surface of the joint is involved causing arthritis. The
condition may be:
a. Traumatic: following fractures
b. Secondary to existing arthropathies: RA, severe OA, psoriatic arthropathy,
neuropathic joint
c. Secondary to systemic conditions: sickle cell disease (particularly sicklecell
thalassemia and sickle-cell-hemoglobin C disease), high dose steroid
therapy, pregnancy, extensive burns endocarditis, Caisson disease, and
scleroderma
d. Idiopathic: avascular necrosis may occur in middle aged men and is the
cause of Perthes' disease in children
e. Types: Legg- Calve'- Perthe's disease (femoral capital epiphyses), Blount's
disease (proximal tibial epiphysis), Osgood Schlatter's disease (tibial
tuberosity), Sever's disease (calcaneal apophysis), Freiberg's disease (2nd
metatarsal head), Diaz/ Mauchet (talus), Islen's disease (base of 5th
metatarsal), Thieman's disease (phalanges), Kohler's disease (navicular and
patella), Treve's disease (medial sesamoid), Buchman's disease (medial
cuneiform)
f. X-rays: small areas of infarction which appear sclerotic or porotic, areas of
joint surface collapse into underlying infarction producing flattening, necrotic
osteochondral fragments may separate completely or partially
g. Tx: immobilation, physical therapy, surgery

4. Drug Induced SLE (the Hydralazine Syndrome): a condition

resembling SLE but induced by procainamide, hydralazine, isoniazid, oral
contraceptives, penicillin, sulfonamides, tetracycline, griseofulvin,
phenylbutazone, reserpine and methyldopa. It is suggested that patients
with this condition have a heredity "lupus diathesis" which is made manifest
only when the drug is given. Arthritis occurs in 50% of the cases and can
occur synchronously or up to 4 weeks prior to the skin rash.
a. X-rays: normal
b. Labs: L.E. cells (90%), A.N.A. (100%)
c. Tx: stop the drug, steroids for symptoms prn

5. Erthema Nodosum: an acute self-limiting condition characterized by the

development of crops of tender nodules in the skin of the lower leg and
arthritis occurring in 50% of the cases occurring with the nodules or
preceding them by up to 4 weeks. The nodules are first bright red, later dark
red, and then fade like bruises. This condition occurs in sarcoidosis (common
in Great Britain), various infections (particularly strep, measles, etc.),
ulcerative colitis, malignant diseases, and drug sensitivities.
a. Joints affected: knees and ankles most commonly
b. Symptoms: sudden onset of pain without swelling
c. Signs: variable (none to red swollen joints), fever common
d. X-rays: normal
e. Labs: >ESR, LA Fix occasionally (+), must do complete work up to find
cause
f. Tx: rest, MAIDS

6. Gaucher's Disease: a rare condition transmitted as an autosomal

recessive trait characterized by the abnormal accumulation of
glucocerebrosides in reticulo-endothelial cells. The characteristic feature is
the Gaucher cell, a lipid filled macrophage that contributes to the arthritis
and eventually avascular necrosis. Severity is variable and can occur at any
age.
a. Joints affected: hips are most common, 70% unilateral
b. Symptoms: pain and stiffness
c. Signs: hip held in flexion and adduction with limb shortening
d. Course: untreated hip involvement will progress to complete destruction of
the joint with secondary OA
e. X-ray: areas of porosis/sclerosis, aseptic necrosis, Ehrlenmeyer flask
appearance of lower end of femur
f. Labs: anemia, sometimes leukopenia and thrombocytopenia, bone biopsy
shows Gaucher cells (lipid-filled "bubbly' macrophages)
g. Tx: rest, immobilization with no weight- bearing in early cases, surgery in
advanced cases

7. Gonococcal arthritis: arthritis due to gonococcal infection of the joints,

beginning 3-17 days after being infected.
a. Joints affected: knee is the most common area, ankles(40%), asymetrical,
polyarticular (75%), "Gonorrheal heel"
b. Symptoms: sudden onset of severe pain and swelling, fever, rigors
c. Signs: Warmth, erythema, tenderness, edema of joint and periarticular soft
tissues
d. Course: effective treatment produces complete cure in 1-4 weeks
e. Associations: skin lesions (50% cases) maculopapular, hemorrhagic
f. X-ray: normal in the acute stage
g. Labs: mild leukocytosis, >ESR, gonococcal antibodies detectable (90%),
organism may be recovered from the blood and genital tract
h. Tx: Penicillin (procaine penicillin 1-2 megaunits + benzylpenicillin 1
megaunit daily for 5 days) or tetracycline 250mg. q.d.s. for 7 days. VDRL
should be done before treatment, since antibiotics may mask early signs of
concurrent Syphilis.
8. Gout: a disorder of purine metabolism, characterized by hyperuricemia
and the deposition of urate crystals in joints, resulting in acute attacks of
arthritis. In the later stage there is deposition of urate in the soft tissues and
the kidney, with a chronic arthritis.
a. Joints affected: 1st m.p.joint (75%), monoarticular (90%)
b. Symptoms: prodromal irritability with sudden onset of pain often 3-6 am,
precipitated by surgery, trauma, starvation, alcoholic excess, and drugs
(ASA, thiazides), usually males>females
c. Signs: red/hot/swollen/tender joint, between attacks the joints are normal
until tophaceous stage, must differentiate from septic arthritis
d. Variants: Secondary gout (5%) cases due to myeloproliferative disorders
(polycythemia rubra/vera) and during the treatment of malignant disease.
Other variants are renal failure, Lesch-Nyhan syndrome and glycogen
storage disease (von Gierke's disease)
e. X-ray: normal in early stages, with the joint space preserved until late in
the disease, periarticular swelling and punched out/radiolucent areas in the
affected joints
f. Labs: synovial fluid and tophaceous deposits contain needle-shaped
crystals which are strongly negatively birefringent, uric acid greater than
6mg./100ml, >ESR (uric acid serum levels may not be elevated during acute
attack)
g. Tx: responds to indocin, phenylbutazone or colchicine (.6 mg./hr until relief
or NVD), if no response then ACTH. Later uricosurics or allopurinol (used in
tophaceous gout)

9. Hemophilia: a group of disorders of blood coagulation mechanisms due

to deficiencies of various factors. The most common form is inherited by
males, the females being unaffected carriers. It is inherited as an X-linked
recessive trait. Attacks of arthritis are due to hemorrhage into joints and
recurrent hemoarthrosis leads to a degenerative arthropathy which is
characteristic of hemophilia.
a. Joints affected: knee (70%), ankle (20%), monoarticular, asymmetrical
b. Symptoms: sudden onset of very severe pain sometime after mild trauma
c. Signs: joint is red, warm, swollen and very tender, after repeated attacks
there is limited ROM and crepitus, eventually muscle wasting
d. Course: complete recovery but later deformities and degenerative
changes
e. X-ray: normal with early attacks, later small superficial erosions and cysts
appear with a loss of joint space and flattening of joint surfaces
f. Labs: prolonged PTT time, confirmed by Factor VIII assay, blood stained
synovial fluid
g. Tx: complete immobilization while in the acute stage, give missing factor
(cyroprecipitate or fresh plasma), analgesics, avoid aspiration, gentle
physiotherapy

10. Hypothyroidism: deficiency of thyroid hormone may cause a variety of

rheumatic manifestations: pain and stiffness in proximal muscles,
polyarthritis, monoarthritis associated with osteolytic lesions usually in
children, carpal tunnel syndrome, secondary gout, hypercholecterolemia.
a. Joints affected: knees, wrists, ankles, usually bilateral and symmetrical
b. Labs: confirm by > cholesterol, < thyroxin level, abnormal EKG
c. Tx: thyroxin

11. Neuropathic Joint (Charcot): disorganization of a joint and destruction

of joint surfaces associated with diminished pain sensation which is most
commonly due to: tabes dorsalis, diabetes mellitus, syringomyelia,
Charcot-Marie-Tooth disease, meningomyelocele, hemiplegia, and
leprosy.
a. Joints affected: monoarticular, depends on localization of pain loss
b. Symptoms: 50% have acute onset of pain and swelling, pain is absent in
the later stages
c. Signs: acute stage, red/warm/tender/swollen joint; chronic stage, bony
swelling and recurrent effusion/instability/crepitus/grotesque deformities
d. Course: acute inflammatory stage lasts for up to 6 months, with
subsequent slow progression for years until deformities occur
e. X-ray: sclerosis of the bone ends, loss of the joint space, loose bodies,
massive osteophytes, periarticular calcification, fractures
f. Labs: essentially normal chemistries. Do immunologic tests (antibodies to
listed bacterial infections)
g. Tx: rest in the acute stage, MAIDS, stabilization with braces and splints,
arthrodesis for instability

12. Conditions associated with neuropathic joints:

a. Tabes Dorsalis: knee is most common site (70%), with ankle and feet
(30%), Argyll Robinson pupil (80%), absent DTR's
b. Syringomyelia: 50% have cervical spondylosis on x-ray, loss of pain and
temperature in the upper extremities, equinus deformities c. Diabetes
Mellitus: 1 % of diabetics and 5% with diabetic neuropathy have a
neuropathic joint, foot is most common site (80%), absent ankle jerk,
sensory loss

13. Osteoarthritis: a common degenerative condition of a joint surface

associated with aging. OA may be primary or secondary to: obesity,
unrecognized congenital hip dislocation, any condition causing irregularity of
the joint surface including avascular necrosis, repeated trauma, septic or
other arthritis. There is a wide spectrum of clinical manifestations:

a. Secondary OA Primary OA Primary generalized OA

one joint one to six joints polyarticular
definite cause no definite cause Heberden's nodes
Heberden's nodes usually
(sometimes)
b. Joints involved: D.I.P. joints of the hands, lumbar spine, 1st M.P.J., P.I.P. or
M.C.P. of hands

NOTE* Heberden's nodes occur over the distal ipj's, and Bouchar’ s nodes
occur over the proximal ipj's (Bouchard's nodes are associated with gastric
dilatation)

c. Symptoms: pain with or after movement/relieved by rest, worse towards

evening, stiffness of affected joints after immobility, but not generalized
morning stiffness (post static dyskinesia)
d. Signs: bony swelling and tenderness, acute episodes may be accompanied
by warmth/erythema/effusion
e. Course: chronic with exacerbations, sometimes related to trauma
f. X-ray: degenerative changes with non-uniform loss of joint space/loss of
subchondral bone (subchondral sclerosis)/subchondral cysts (do not break
through the cortex)/osteophytes/later, irregularity of the joint surface/joint
mice
g. Labs: ESR normal, synovial fluid: see chart
h.Tx: NSAIDS, physiotherapy, surgery if symptoms severe and persistent

14. Osteochondritis Dissecans: a condition characterized by the

separation of avascular osteochondral fragments from the surface of the
joints. If separation is complete, loose bodies are found within the joint.
a. Joints affected: knee is most common (85%), monoarticular (75%), ankle
frequently
b. Symptoms: pain (mild) worse after exercise
c. Signs: often none, effusion occasionally
d. Course: acute a isode recovers after a few months with OA following after
a few years (50%)
e. X-ray: separation of a fragment or fragments of bone from the joint
surface, sclerosis of the fragment and the crater from where it comes from,
osteoarthritic changes later
f. Labs: uneventful
g. Tx: immobilization in non-weight bearing cast/ spontaneous healing in
younger cases, with surgery for fixation of a fragment or removal of loose
bodies

15. Paget's Disease of Bone (osteitis deformans): a disorder of

unknown etiology affecting one or more bones particularly the skull, femur,
and tibia. The condition is rare before the age of thirty. Approx. 80% of
patients with radiological changes also have symptoms, usually bone pain
and deformities.Areas of increased density and of decreased density.
a. Symptoms: pain indistinguishable from OA, limp
b. Course: chronic and slowly progressive leading to OA
c. X-ray: 1st change is localized porosis (in skull called osteoporosis
circumscripta), bone often expanded and thickened, with fractures of the
long bones. Distinct border between normal and abnormal bone
d. Labs: >Alk Phos, Ca normal (ALK PHOS reaches extremely high serum
levels)
e. Tx: analgesics, disodium etidronate (inhibits bone resorption and
mineralization)
16. Palindromic Rheumatism: an uncommon condition, probably a variant
of RA, characterized by recurrent/acute/self-limiting attacks of arthritis.
a. Joints affected: hands, wrists, knees and feet, with each patient having 1-3
prominent sites
b. Symptoms: sudden onset of severe pain and stiffness
c. Signs: swollen/red/tender joint
d. Course: attacks occur at irregular intervals, averaging 20 per year. There
may be years of freedom between attacks or hundreds of attacks in one
year. Attacks last a few hours/days, seldom more than 1 week, with
complete recovery follows the attack
e. X-rays: normal
f. Labs: >ESR, latex fix (+) 50% cases
g. Tx: indomethacin, phenylbutazone or ASA for acute attacks, gold therapy
may produce remission

17. Psoriatic Arthritis: a common skin disease (psoriasis) with associated

arthritis in 10% of cases, with the skin manifestation preceeding the arthritis
by many years. A seronegative disease. Sometimes joint symptoms precede
skin lesions
a. Joints affected: polyarticular, with small joints of the hands predominating
(70%). There are three patterns (distal type- d.i.p.j.'s and p.i.p.j.'s,
seronegative indistinguishable type- joint involvement indistinguishable from
RA, deforming type- causes arthritis mutilans)
b. Symptoms: pain is seldom prominant except in the deforming type
c. Signs: acute stage- red/hot/swollen joint (sausage toe), chronic stage-
swollen joints
d. X-rays: erosions within the joint, sclerosis of the joint margins, proliferation
of bone, cysts, ankylosis, destruction of bone ends in the deforming type,
mushrooming of the joint surfaces
e. Labs: ESR normal except in the acute stage, (+) HLA-B27 (in many cases),
latex fix (-)
f. Associations: pitting of the nails (90%), hyperuricemia (30%)
g.Tx: analgesic and NSAIDS, avoid systemic steroids, avoid antimalarials
cause exfoliative dermatitis)

18. Pyrophosphate Arthropathy: a condition caused by the deposition of

crystals of calcium pyrophosphate dihydrate (CPPD) into joints. This condition
does not resemble gout and should not be called pseudogout. The term
chondrocalcinosis articularis should be reserved for the radiological
appearance of cartilage calcification. There are three clinical patterns of the
disease: recurrent acute arthritis, chronic degenerative arthritis
(resembles RA), and chronic arthritis with acute episodes. Typical onset is
age 60.
a. Joints affected: knee is most common site, can affect ankles/toes/fingers,
usually monoarticular and symmetrical b. Symptoms: pain and stiffness,
sudden onset
c. Signs: acute red/ hot/ swollen joint
d. Course: attacks last weeks/months
e. Associations: diabetes mellitus present in up to 50% cases, hypercalcemia,
hemochomatosis
f. X-ray: affected joints eventually show calcification of cartilage and
degenerative changes
Labs: synovial fluid changes (see synovial fluid chart), >ESR, leukocytosis,
crystals seen microscopically in fluid and biopsy-crystals polarize
g. Tx: injection of joints with hydrocortisone, indomethacin

19. Reiter's Disease: a triad of arthritis/conjunctivitis/urethritis which

usually follows non-specific (non-gonococcal) urethritis, occurs in males (20-
1). A dermatitis may also be present. A seronegative disease.
a. Joints affected: knee (90%) and ankle (75%) most common, feet (40%),
polyarticular/asymetrical
b. Symptoms: acute onset of joint pain and swelling, dysuria, and penile
discharge
c. Signs: red/hot/swollen joints in the acute stage
d. Course: 1st attack resolves within 6 months, rarely becomes chronic, 50%
relapse, 20% have continued relapsing/chronic arthritis
e. Associations: urethritis, conjunctivitis, circinate balanitis, keratoderma
blenorrhagica, tenosynovitis (usually achilles), plantar fasciits, heel pain,
back pain
f. X-ray: periarticular osteoporosis with erosions, fluffy periosteal new bone
formation (calcaneal spur)
g. Labs: >ESR, (-) latex fixation, (+) HLA-B27 (in many cases), joint fluid is
inflammatory, gonococcus found in GU tract in some cases. Other bacterial
infections may be present. Reiters disease is also seen in AIDS
h. Tx: rest, analgesics, MAIDS, antibiotics (if indicated)

20. Rheumatoid Arthritis: a chronic and widespread chronic deforming

polyarthritis characterized by bilateral/symmetrical joint involvement,
erosions on x-ray, positive latex fixation tests, and pathologically a chronic
proliferative synovitis with villous hypertrophy, infiltration of lymphocytes
and plasma cells, and lymphoid nodules. Occurs in 6% females and 2%
males world-wide. An autoimmune disease a. Diagnostic criteria:
• morning stiffness
• pain on motion or tenderness in 1 joint
• swelling in 1 joint, swelling in another joint
• symmetrical joint swelling
• subcutaneous nodules
• x-ray changes typical of RA
• poor mucin ppt. from synovial fluid
• characteristic histologic changes in the synovial membrane
• positive Rheumatoid factor
• characteristic histologic changes showing palisading granulomas

Definite rheumatoid arthritis= 5 of the above

Probable rheumatoid arthritis= 3 of the above
b. Joints affected: small joints of the hands and feet (75%) especially the
m.p.j.'s, and the p.i.p.j's, usually bilateral and symmetrical. Also calcaneus
affecting the posterior-superior surface near the achilles insertion (forms a
posterior calcaneal recess) with non-well demarcated heel spur. Larger,
proximal joints are frequently involved.
c. Symptoms: insidious onset of joint pains and stiffness, generalized
morning stiffness, accompanied by general ill health, fatigue and weight loss
which can precede joint symptoms by a few months.
d. Signs: swollen/tender joints with limited ROM due to effusion or synovial
thickening, muscle wasting around the affected joints
e. Special features: ulnar deviation hands, boutonniere deformity of p.i.p.j.,
baker's cyst of knees, atlanto-axial subluxation of cervical spine, crico-
arytenoid joint involvement causeshoarseness/dyspnea/dysphasia/bronchitis
f. Course: either episodic or persistent, episodic has average patient having 3
attacks of arthritis once every 2 years each lasting 6 months; persistent has
chronic arthritis with partial remissions and exacerbations, joints tend to
become burnt out
g. Non-articular manifestations:
i. Periarticular soft tissues- nodules (Haygarth's nodes at affected joints),
tenosynovitis, bursitis, synovial cysts, muscle wasting, ligamentous laxity
ii. Skin- tight like scleroderma, leg ulcers
iii. Eyes- Sjogren's syndrome
iv. Heart- pericarditis, granulomatous lesions in myocardium
v. Nerve- carpal tunnel syndrome/tarsal tunnel syndrome
vi. Blood- anemia, Felty's syndrome (splenomegaly/leukopenia)
h. X-ray: early changes include periarticular osteoporosis, joint space
widening; later changes include uniform joint space narrowing thinning of the
cortices synovial erosions, pseudocysts that erode through the cortex (OA
cysts have a sclerotic boarder), subluxations and deviations joint fusions and
widespread osteoporosis
i. Labs: anemia, >ESR, latex fixation (+) 80%, ANA (+), RA (+), synovial fluid
changes (see chart), blood protein (plasma fibrinogen and globin increased;
decreased total protein)
j. Tx: NSAIDS, rest modalities, intraarticular steroid injections, synovectomy,
gold injections (Myocrisin) @ 10, 20, 30, 40 mg I.M. at weekly intervals then
50 mg. weekly up to 1 g., then a maintenance dose of 50 mg. monthly if
results good (must check WBC's)

21. Septic Arthritis: Infection of a joint with pyogenic bacteria. Most

common organism is Staph aureus (50%). Can occur in any age group but
more frequent in kids and geriatrics (should be considered when examining
any monarticular erythematous, hot, swollen joint)

NOTE* The bacteriology in septic arthritis is most frequently as follows:

Neonates: Streptococcus and gram negative organisms a.
Children (6months to 5 yrs): H. influenza common
Teenagers: Neisseria gonorrhea have higher incidence
Adults: P. aeruginosa common after puncture wound
Adults with sickle-cell: Salmonella common
Compromised patients (burn victims, drug users): Serratia marcescens
pyarthrosis
Joints affected: knee most common, 90% monarticular
b. Symptoms: rapid. onset of pain and swelling, can have elevated
temperature, malaise, tachycardia, and confusion
c. Signs: swelling with effusion/tenderness/warmth and painful limitation of
motion, febrile
d. Course: untreated, leads to joint destruction (OM/ankylosis)
e. X-rays: normal initially, osteoporosis after 2 weeks
f. Labs: >WBC's, synovial fluid (see chart), blood culture and synovial fluid
culture (+)

NOTE* The joint aspirate should undergo the following studies:

C&S, gram stain, examination for crystals, WBC count and differential

NOTE* The WBC's in joint aspirate in a septic joint is usually higher then
100,000, however, in gonococcal arthritis the WBC's is usually below
50,000
g. Tx: antibiotics, rest, aspirations (?? damage to the joint), open
debridement (permits lysing of adhesions and debridement of necrotic bone
or soft tissue present, arthrotomy performed in patients with advancing
osteomyelitis

22. Sickle-Cell Disease: a condition inherited as an autosomal

intermediate, in which hemoglobin A is replaced by hemoglobin S.
Heterozygotes are not anemic and do not have arthritis. Homozygotes are
severely anemic and have thrombotic crises which commonly cause arthritis
as a result of local bone infarcts. Usually seen in blacks with the onset in the
first 10 years of life.
a. Joints affected: polyarticular, hands and feet common sites, often
migratory
b. Symptoms: sudden onset of severe joint pains or backache
c. Signs: often none, occasionally affected joints are swollen/red/tender,
fever common in a crises, painful ankle ulcers
d. Course: joint pain subsides spontaneously within days, life span greatly
reduced
e. X-rays: areas of osteoporosis or sclerosis, periosteal proliferation affecting
the shafts of the metatarsals
f. Labs: in vitro sickling tests and hemoglobin electrophoresis confirm the
diagnosis
g. Tx: analgesics, IV bicarbonate may help, urea compounds,
pentoxophylline, avoid hypoxia/hypotension/tourniquets

23. Still's Disease (Juvenile Rheumatoid Arthritis): a chronic polyarthritis

resembling R.A. clinically and histologically, but beginning before the age of
16 (peak ages, 1-3 & 10-15).
a. Joints affected: as with the adult + involvement of the cervical
spine/TMJ's/d.i.p.s. (more)
b. commonly affected, less symmetry than RA
c. Symptoms: pain often mild
d. Signs: joints warm/tender/red/swollen with effusion and synovial
thickening, fever (50%)
e. Course: better prognosis than in adults especially if earlier onset
f. X-ray: erosions less common, periosteal reaction may occur in phalanges
and mets
g. Labs: >ESR, latex fixation (+) in 20% cases
h. Tx: ASA, rest, maintain full ROM, gold or immunosuppressive drugs only in
severe progressive cases, short course A.C.T.H. useful for severe relapse

24. Systemic Lupus Erythematosis (S.L.E.): an autoimmune condition

characterized by the presence of antinuclear factor and other auto-
antibodies. The diagnosis is often made on the basis of multi-system
involvement.
a. Joint pain occurs in 90% cases, and arthritis usually occurs at onset but
may precede other systemic features by up to 20 years.
b. Joints affected: Commonly seen in the p.i.p.j.'s, also in the wrists, knees,
ankles and elbows, is polyarticular with a distribution resembling RA
c. Symptoms: sudden onset of pain and stiffness which may be precipitated
by exposure to sunlight or stress, morning stiffness (50%)
d. Signs: 50% have none, but the characteristic finding is slight soft tissue
swelling, fever common
e. Course: 70% survive 5 years and 50% 10 years, prognosis is much worse
with renal involvement
f. Associations: rash of face (butterfly), renal disease, pericarditis, Raynaud's
phenomenon, and liver disease
g. X-ray: usually normal
h. Labs: >ESR, ANA (+) 100%, LE cells present (80%), latex fixation (+) 30%
i. Tx: prednisone 30-60 mg/day initially reducing slowly to a maintenance
dose of about 15 mg.day

NOTE* Some medications precipitate SLE (see earlier chapter)

25. Ulcerative Colitis: a condition in which severe ulcerative inflammation

of the colon causes fever, anemia, and the passage of blood/mucus from the
bowel. Arthritis is of 4 types, colic arthritis, ankylosing spondylitis, arthritis
associated with erythema nodosum, and pseudohypertrophic osteo-
arthropathy. In ulcerative colitis 10% develop arthritis, in Crohn's disease 4%
develop arthritis
a. Joints affected: knees and ankles, usually 1-3 joints in each attack, usually
asymetrical
b. Symptoms: sudden onset of pain/swelling, often associated with relapse of
colitis
c. Signs: effusion, painful limitation of motion
d. Associations: skin lesions (erythema nodosum/pyoderma gangrenosum)
e. X-ray: joints normal
f. Labs: >ESR, Synovial fluid shows WBC's up to 40,000
g. Tx: NSAIDS and analgesics, appropriate tx for colitis
h. Younger patients may go on to develop GI adenocarcinomas after 10 or
more years of disease.

Laboratory Testing for the Arthropathies

1. Synovial Fluid analysis: should be approached with caution so as to
prevent hemoarthrosis or septic joint. When the joint is punctured an
attempt should be made to extract all the fluid present. The analysis of the
joint fluid is of some importance in the diagnosis of certain diseases. (See
chart)

2. Serologic tests:
a. Erythrocyte Sedimentation Rate: nonspecific, reflects the presence of
tissue injury and inflammation, as a screening test may be overrated
b. C-reactive protein (CRP): nonspecific test for inflammation, a most
sensitive indicator of rheumatic fever
c. Rheumatoid factor: are a heterogenous group of antiglobulin antibodies
primarily found in patients with rheumatoid arthritis. The antibodies react to
specific antigenic determinants on the crystallizable fragment of human IgG.
RF has been found among several classes of immunoglobulins, including IgM,
IgA, IgG, and IgE
d. Latex fixation: are also used to detect RF, with IgG used as the antigen
e. Antinuclear antibodies (ANA): are a heterogenous group of antibodies
detecting many nuclear constituents; including deoxyribonucleic acid (DNA),
deoxynucleoprotein (DNP), histone, and ribonucleicacid (RNA). The ANA
antibodies were 1st noted in the sera of patients with SLE. Only a test
reactive for double- stranded DNA is diagnostic for SLE
f. Histocompatibility antigens (HLA-B27): is associated with the
seronegative diseases
g. VDRL: screening test for syphilis (but not specific for it)
h. ASO titer: done with CRP where Rheumatic fever is suspected
i. LE prep: rarely done today because of the accuracy of the ANA

3. CBC and differential

4. Uric Acid: May be normal during acute gouty attacks 5. Test for
Lyme disease (Lyme titer)

6. Test for AIDS: ELISA (and If reactive then do a Western Blot)

Articular Disorders Affecting the Heel

Systemic inflammatory disorders such as RA, anklosing spondylitis, psoriatic
arthritis, Reiter's syndrome, gout, and Behcet's syndrome have all been
reported to cause heel pain. Signs and symptoms of these disorders include
local soft tissue swelling and occasional erythema associated with diffuse
aching type pain. Various symptom complexes highlight certain arthritides
(like Reiter's). Radiographic erosive and proliferative bone changes in these
patients include posterior and inferior calcaneal spurring, retrocaicaneal
swelling, Achilles tendon thickening, and posterior and inferior calcaneal
erosions. These changes occur in various combinations and each disease
entity has its own usual but not exclusive calcaneal target site.
 Calcaneal target sites
A: Rheumatoid Arthritis
B: Ankylosing spondylosis and psoriasis
C: Reiter's syndrome
McGlamry ED: Comprehensive Textbook of Foot Surgery, Williams & Wilkins, Baltimore, 1987, with permission
 SYNOVIAL FLUID
After aspiration note volume, clarity, turbidity, blood-staining, and viscosity. Divide as
follows:

2 ml. in a tube containing At least 1 ml. in a sterile Remainder: examine a wet

EDTA for cell count and container for microscopy and film for crystals under
differential culture. Ask for urgent Gram polarized light. Note
stain or special culture media presence or absence of clot
if indicated formation after standing

RESULTS: The characteristics of synovial fluid depend upon the presence or absence of
inflammation of synovium. Non-inflammatory fluid is clear, viscous, fails to clot on standing,
and contains less than 1000 cells per c.mm., predominantly mononuclears. Inflammatory
fluid is non-viscous, may clot, and contains an increased number of white blood-cells. These
changes may be slight or gross, depending on the severity of the inflammatory process.
Fluid with a high whitecell count is turbid and this does not necessarily mean that it is septic.
The characteristics of inflammatory and non-inflammatory fluid are summarized in the
following table. Synovial fluid characteristics in individual arthropathies are shown with other
laboratory features of the conditions.

NON-INFLAMMATORY INFLAMMATORY

E.g., Osteo-arthrosis Gout or

Rheumatoid Septic
or Traumatic Pyrophosphate
Arthritis, Arthritis
-Arthritis Arthropathy
Appearance Clear Often turbid Turbid Clear with Bakes
of fibrin

Colour Yellow Yellow/green Brown/green Yellow

Viscosity High Low Low Low

Clots ? No Yes Yes Yes

Approx. W.B.C. 1,000 30,000 100,000 10,000

(per mm.3)

Predominant cell Mononuclears Neutrophils Neutrophils Neutrophils

Crystals No No No Yes

Culture Sterile Sterile Positive Sterile

IDENTIFICATION OF CRYSTALS. Uric acid crystals are needle-shaped and strongly negatively
birefringent (blue across the plane of the first-order red compensator); pyrophosphate
crystals have square ends and are weakly positively birefringent (blue along the plane of the
compensator).

SPECIAL TESTS
1. Complement levels are low in rheumatoid arthritis and high in Reiter's disease.
2. Latex tests parallel serum titres in patients with rheumatoid arthritis; there is a high
incidence of false positives in other conditions and the test has little diagnostic value.
Charcot Foot
1. Disorders producing a Charcot joint: Diabetes mellitus (most common
cause), tabes dorsalis, leprosy, syringomyelia, spina bifida,
meningomyelcoele, congenital insensitivity to pain, chronic alcoholism,
spinal cord injury and compression (all have decrease in pain sensation in
the presence of uninterrupted physical activity in common)

2. Mechanism of destruction: Precipitated by a single injury or by

repetitive moderate stress applied to bones and joints. The results are
fractures, effusions, and ligamentous laxity followed by erosion of articular
cartilage, fragmentation, luxation, distintegration, and finally collapse of the
foot. The consequence of trauma is a hyperemic response, which promotes
additional resorption of bone and increases the susceptibility to further injury
and progressive deterioration

NOTE* Eichenholtz divided the disease process into 3 radiographically

distinct stages:
a. Development: The acute destructive period, which is distinguished by joint
effusions, soft tissue edema, subluxation, formation of bone and cartilage
debris (detritus), intra-articular fractures, and fragmentation of bone
b. Coalescence: Noted by a lessening of edema, absorption of fine debris,
and healing of fractures (reparative phase of healing has begun)
c. Reconstruction: Further repair and remodeling of bone take place in an
attempt to restore stability and homeostasis

Neuropathic osteoarthropathy can be arrested during the Development

stage if diagnosed before the disease has a chance to mature

3. Pathogenesis:
a. Sensory-motor neuropathy: Loss of protective sensation, absent DTR's,
diminished vibratory sense, muscle weakness, ankle equinus b. Autonomic
neuropathy: Sympathetic denervation loss of vasomotor control, increased
peripheral blood flow, increased artiovenous shunting, increased bone blood
flow, hyperemia
c. Minor trauma: Repetitive moderate stress, repetitive impulse loading,
trabecular microfractures, inadequately protected fractures and sprains,
surgery
d. Other factors: Metabolic abnormalities which weaken bone, renal
transplantation, immunosuppressive treatment, steroid-induced
osteoporosis, decreased cartilage growth activity, glycosylation of collagen

4. Diagnosis:
a. Medical history, clinical manifestation, and radiologic findings
b. Technium and Gallium scans, MRI (low signal Intensity in T, and T2-
weighted images within the bone marrow space adjacent to the
involved joint), and bone biopsy and cultures in equivocal cases
5. Treatment: Based on the acuteness of symptoms, the anatomic pattern
of bone and joint destruction, the degree of involvement (deformity,
fractures instability, etc.) and the presence of infection a. Immobilization: At
least 3 months of non-weight-bearing cast immobilization prior to resumption
of partial weight-bearing (always check the asymptmatic limb during this
time)
b. Orthoses and shoes: A patella tendonbearing brace with molded shoes to
decrease the load on the foot
c. Surgery: Ostectomy, arthrodesis
i. Criteria for surgery: Instability, deformity, recurrent ulceration, refractory
to conservative treatment, must be quiescent, circulation intact, no active
infection, medically stable