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Forbidden clone
Altered antigen
Sequestered Antigen
Immunologic deficiency theory
Genetic influence
Forbidden clone
Hematologic abnormalities.
Immunologic Findings
Lupus Erythematosus (LE) cell, neutrophil
which has engulfed the antibody-coated
nucleus of another cell.
First classic test to aid in diagnosis.
Not utilized anymore, may still see in older
references.
Over activity of B cells main immunologic
characteristic.
Antinuclear antibodies produced.
More than 28 antibodies associated with LE
have been identified.
Level of antibody production correlates with
severity of symptoms.
Estrogen enhance B cell activation.
Immunologic Findings
Decrease in absolute number of T
cells
Accumulation of immune complexes
with activation of complement lead
to kidneydamage.
Drug induced lupus may occur,
discontinue drug, symptoms usually
disappear.
Laboratory Diagnosis
Screening test for anti-nuclear
antibodies (ANA) first test done.
Antibodies directed against nuclear
material of cells.
Flourescent anti-nuclear antibody (FANA)
most widely used, extremely sensitive, low
diagnostic specificity.
Animal or human cells fixed to slide.
Add patient serum and incubate.
Wash to remove unreacted antibody.
Add anti-human globulin labeled with
fluorescent tag or enzyme.
Antinuclear Antibody Test
Antinuclear antibodies
(ANA) are
autoantibodies against
various cell nucleus
antigens and are
found in patients with
autoimmune diseases
such as SLE.
Some of ANA are
considered to be
useful for diagnosis of
autoimmune diseases.
Antiphospholipid Antibodies
Antiphospholipid antibodies may be
present and are of two types.
Anticardiolipin.
Lupus anticoagulant, if present, may
cause spontaneous abortion and
increase
Risk of clotting, platelet function may
be affected.
Treatment
Characterized by HYPERTHYROIDISM.
Nervousness, insomnia, depression,
weight loss, heat intolerance,
breathlessness, fatigue, cardiac
dysrhythmias, and restlessness.
Women more susceptible, occurs most
frequently between 30 and 40 years of
age.
Genetic link suspected.
Graves Disease
Diagnosis may be straightforward, since
the "classic face" with its triad of
hyperthyroidism,, goiter
hyperthyroidism goiter,, and
exophthalmos is easily recognized.
Goiter is usually symmetric, smooth, and
nontender
The hyperthyroid state, which is by far the
most common component of Graves'
disease, can cause a wide variety of
multisystem derangements that often
result in diagnostic confusion.
Exophthalmos
Exophthalmos, also called proptosis
Exophthalmos, proptosis,, is a
characteristic finding in thyroid eye
disease,, and has been reported to occur
disease
in 34% to 93% of patients
Signs Symptoms
Nervousness and increased activity,
Grave's disease patients may suffer a
fast heartbeat, fatigue, moist skin,
increased sensitivity to heat,
shakiness, anxiety, increased appetite,
weight loss, and sleep difficulties.
They also have at least one of the
following: an enlargement of the
thyroid gland (goiter), bulging eyes, or
raised areas of skin over the shins.
Laboratory Testing
Presence of thyroid-stimulating
hormone receptor antibody, causes
release of thyroid hormones.
Key findings are elevated total and
free T3 (triiodothyronine) and T4
(thyroxine), the thyroid hormones.
Thyroid stimulating hormone (TSH)
is reduced due to antibody
stimulation of the thyroid.
Treatment
Medication.
Radioiodine therapy to destroy the
thyroid.
Surgical removal of thyroid
Insulin Dependent Diabetes
Mellitus
Autoimmune process causes
destruction of cells in the pancreas
resulting in insufficient insulin
production.
Occurs before age 20, peak onset
between 10 and 14 years.
Inherited susceptibility.
Environmental influences include
possibility of viral infections.
Complications
It is a chronic autoimmune
neuromuscular disease characterized
by varying degrees of weakness of
the skeletal (voluntary) muscles of
the body.
It is the most common primary
disorder of neuromuscular
transmission
Symptoms
Facial weakness,
Difficulty chewing and swallowing,
Inability to maintain support of
trunk, neck or head.
Myasthenia Gravis
Antibody mediated damage to acetylcholine
receptors in skeletal muscles leading
toprogressive muscle weakness.
Acetylcholine released from nerve endings to
generate muscle contraction.
Antibody combines with receptor site, blocking
acetylcholine binding.
Receptors destroyed by action of antibody and
complement.
Myasthenia Gravis
Laboratory Testing
Autoantibodies to the Acetylcholine receptor
(AChRAb) can be detected in 80-80-90% of
patients with myasthenia gravis.
The assay measures antibodies that precipitate
solublized muscle AChR that has been
complexed with radiolabeled alpha-
alpha-
bungarotoxin (BTX). Antibodies that bind to
the receptor regions that are not sterically
blocked by the BTX are detected.
Goodpastures Syndrome
CREST syndrome
Calcinosis
Raynauds
Esophageal dysmotility
Sclerodactyly
Telangiectases
Calcinosis
The buildup of calcium deposits in the tissues.
It may occur under the skin of the fingers, arms,
feet, and knees, causing pain and infection if the
calcium deposits pierce the surface of the skin.
Raynauds Phenomena
is a problem of poor blood flow to fingers and
toes.
Blood flow decreases because blood vessels in
these areas become narrow for a short time, in
response to cold or to emotional stress.
Results in: finger sensitivity, toe
oe sensitivity cold
sensitivity, changes in skin color, finger pain, toe
pain, fingertip ulcers, toe ulcers
Esophageal Dysmotility