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Behcets disease
Behcets disease was not that important for someone that lives
in USA or Europe, but nowadays because of migration it is, but
for Middle East countries it is important, because all the time
we see patients with Behcets disease.
doesnt mean anything because this is not from the criteria for
the diagnosis, because as we said the diagnosis is CLINICALLY.
As an autoimmune disease, what happens is that there is
aberrant immune response triggered by exposure to an agent
possibly infectious, this will lead to increase immune complexes
and Cytokines & Increased CD8/CD4 ratio with decrease CD4
suppressor subpopulations.
In a way or another, Behcets disease is related to blood
vessels; we dont know the exact mechanism but they are prone
to have venous thrombosis more than arterial although they are
not in hypercoagulability state, but what causes the thrombosis
is that their endothelial system is abnormal, they have
inflammation that cause activation of the endothelial system
with low activated protein C levels & vascular endothelial
growth factor levels are high & then we have thrombosis, so it
is a form of vasculitis which is seen with lymphocytic
infiltration of mucocutaneous lesion and neutrophilic infiltrate in
Pathergy Test ** explained later
The clinical manifestations in general vary
between people, some have mild disease &
others have very severe disease (males have
very bad disease)
Most important one is Oral Ulcers which
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Genital ulcers
Pathergy test
One of the clues for Behcets disease is that when we put an
IV line for the patient he will develop a reaction around the
site of the puncture in the form of erythematous papule or
pustule of 2 mm or more, whereas normally we dont have
such reactions, it is seen in (50-75) % of eastern patients &
(10-20) % of north European patients.
Ocular lesions
Differential diagnosis:
Common Oral ulcers
Inflammatory Bowl Diseases(Crohns & UC)
Rheumatic diseases such as SLE( can cause oral &
genital ulcers BUT they are painless, but if they have
secondary infection they will be painful)
Vasculitis
Drug reactions such as Methotrexate
Pemphigus and pemphigoid
How to diagnose??
The major criterion is oral ulcers & the minors are genital
& Now
Gout
Gout in acute attacks cause severe pain (worse than the
infections pain), the cause is the tissue deposition of Mono
Sodium Urate (MSU) crystals due to super saturation of the
ECF with the MSU.
Hyperuricemia is a prerequisite for Gout meaning that you
cant get Gout without the presence of hyperuricemia
before, normally before puberty the uric acid level is very low
(2-3 mg) in both males & females, on puberty the level will
jump & in males will be more than females {in males >7mg &
in females >6mg}, the females level of uric acid will be less
than males till the menopause.
Only (15-20) % of patients who have hyperuricemia will
develop Gout.
Uric acid is an end product of purine metabolism, but human do
not have enzyme Uricase to convert it to allantoin (highly
soluble) to be secreted, the problem in hyperuricemia is either
overproduction(endogenous {in certain syndromes that are very
rare, they have positive family history & some mental problems
such as Nyhans syndrome} or exogenous) or undersecretion of
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uric acid (the problem is in the kidney & even if we have mild
renal impairment the uric acid excretion will be affected, renal
impairment counts for 90% of Gout patient, the idea here is
that the kidney has different threshold for uric acid excretion,
meaning that if we have 2 persons, the 1st excrete 900 mg of
uric acid in 24 hours when the serum uric acid level is 7 mg,
but the second one needs it to be 10 or 11 to excrete the
same amount, so hell have hyperuricemia because of
undersecretion) but we need hyperuricemia for prolonged time
to trigger Gout, for example patients with acute renal failure
dont develop Gout but they do in chronic renal failure.
The higher the level of uric acid & the longer the period
with hyperuricemia, the higher the risk to develop Gout.
The typical presentation for Gout is a male patient who is
30 or above years old, hypertensive, & on diuretics, he has
mild renal impairment, & now he is complaining of pain & he
gives you a history of prior similar pain (attacks of very
severe pain that can awakens him from sleep & he describes
it as someone had hit him with a hummer!! But indeed
there is no trauma).
Gout is a disease of adult men with peak in 5th decade, it is
very rare before puberty and in premenopausal women, &
family history may contribute in 20% of patients (such as in
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4. Thiazide
5. Lasix(furosemide)
6. Ethambutol
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Stages of Gout:
1) Prolonged asymptomatic undiagnosed Gout.
2) Intermittent Gout: Attacks of severe pain that are self
aborted within 7-10 days.
3) Chronic tophaceous Gout: they have deposits of uric acid
outside, common site is the ears.
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Podegra is the Gout of the big toe (1st MTP joint) mainly;
the big toe has a lot of diseases such as RA, osteoarthritis,
trauma, congenital anomalies, Gout, pseudo gout & infection.
It has several characteristics such as: Acute onset Severe
pain Erythema Very tender 5May be febrile 6Resolve in (310) days.
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Olecranon bursitis
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Differential diagnosis:
Pseudogout
Septic arthritis: the most dangerous one, it may lead
to death
Reactive arthritis
Other inflammatory arthritis
pseudogout & Gout arent very dangerous diseases, they
cause severe pain but they wont kill patients, in such patients
we do aspiration by needle, & the best diagnostic method is by
doing synovial fluid analysis.
Management of acute Gout:
NSAIDs: such as ibuprofen (800mg), diclofenac,
COX2 inhibitors, indomethacin (most effective one),
& naproxen (500mg) at full dose, we use all NSAIDs
except paracetamol because it may kill the pain but
it wont affect the inflammation.
You should know the NSAIDs toxicity &
contraindications.
Steroids: are used for acute management if we want
to have fast results & if NSAIDs & colchicine use are
not warranted, they are given orally(such as
prednisolone 20-40 mg daily for 5-7 days), intra
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Tophaceous Gout
Chronic gout with joint damage and erosions
Hyperuricemia uric acid > 12mg/dl
24 hour urine excretion of >1100 mg uric acid
We use Probencid (Uricose uric acid), that leads to the
excretion of the uric acid in the urine we give it in certain
patients who are:
1-age <60
2-Creatinine clearance >50ml/min
3-24 hour urine of uric acid < 700mg(under excretion)
4-No history of renal stone
Also we have new drugs that are similar in their mechanism to
the Uricase enzyme that converts the uric acid to allantoin
that is excreted in the urine & it is found normally in animals
not in humans, of course it is very expensive drug & not
available in Jordan.
Allopurinol (xanthene oxidase inhibitor):
*We use it in hyperuricemia with the following conditions:
Urinary uric acid >1000mg
Uric acid nephropathy
Nephrolithiasis
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Before chemotherapy
Renal insufficiency GFR<50
Allergy to Uricosuric agents
**Average dose is 300mg.
*** In renal impairment you should use lower dose.
****It may precipitate acute gout when first used
*****Side effects can be very serious range from dyspepsia,
headache, diarrhea & rash to more severe including fever,
eosinophilia, interstitial nephritis, hepatitis, vasculitis, acute
renal failure, toxic epidermal necrolysis, & hypersensitivity
syndrome.
*Pseudogout:
Deposition of calcium pyrophosphate dehydrate crystals, it is
very difficult to differentiate between gout &pseudogout
clinically but you may differentiate by looking at the crystals
under the microscope, & usually gout is associated with
pseudogout, & those who have gout are at risk to develop
pseudogout.
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Gout
Neuropathic joints
picture of pseudogout
Aging
Amyloidosis
Trauma
Again
Spondylarthropathies
Common characteristics between the diseases in this group
are:
Inflammatory axial spine involvement
Asymmetrical peripheral arthritis (in RA we have
symmetrical peripheral arthritis)
Enthesopathy: means affecting the site of the insertion
of the tendon, in RA it affects the joint itself.
Inflammatory eye disease
Mucocutaneous features
Rheumatoid factor negative(in RA the rheumatoid
factor positive)
High frequency of HLA B27 AG
Familial aggregation: as other rheumatic diseases.
Examples:
Ankylosing Spondylitis
Psoriatic Arthritis: mimic RA as well as ankylosing
spondylitis.
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Reactive Arthritis
Reiters syndrome
syndrome
Usually the patients are young (<40), & the pain duration is
longer than 3 months.
This is the criteria for diagnosis, it may be changed soon:
one piece.
The stages of the disease in the last one he seems better because he had hip replacement.
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Inflammation of the
sacroiliac joint in its early
stage on the right, then it
will be completely fused as
in the left figure
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spondylitis patients
Reiters syndrome:
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Keratoderma Blenorrhagica
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Balanitis
Palate erosions
Tongue lesions
Asymmetrical Sacroiliitis
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Conjunctivitis
Plantar periostitis
Psoriatic arthritis:
It is a syndrome that mimics
ankylosing spondylitis without
peripheral arthritis, it may
presents with just sacroiliitis
without anything else, or it may
present as asymmetrical arthritis
especially with large joints like
spondylarthropathy or it may be
presented with sausage digit, or nail changes.
Affecting DIPs,PIPs,
sausage digit & NO rash
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Nail pitting
Swelling, joint
Dactylitis
destruction, Erosions in
DIPs, PIPs, MCPs
Treatment
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Scleroderma
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Raynauds phenomenon:
Reversible skin color changes from white to blue to red, 1st we
have severe vasospasm so no blood flow so white color, then
ischemia so blue color, then flushing so red color,, it is caused
by coldness & emotional stress, & it isnt necessary that
Raynaud will affect all the fingers, it may affect just 2 or 3
fingers with the least affection to the thumb, the major
problem in Raynaud that it may end up with necrosis.
Other causes of Raynauds: vasculitis, hyperviscosity,
polycythemia, & if we have compression on blood vessels or on
sympathetic & parasympathetic systems.
Complications: hypertension, high renin, microangiopathic
hemolytic anemia, & thrombocytopenia.
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CREST:
+ ANA (+ve) +
anticentromere
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& Finally
The End
Done by:
Kawther A. Al-alem
Group B8
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