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Generalities
Presence of one or more auto-antibodies
Directed against: membrane, cytoplasm, nucleus
ANA: hallmark of SRD
Many of the diseases have distinctive profiles of
auto-antibodies with diagnostic specificities
Inflammatory process: non-infectious in nature
NB: (+) LE cell prep & (+) ANA test : SLE is strongly
suspected
Anti-dsDNA (native)
Confirmatory test for SLE (about 2/3 of patients)
Also: to monitor SLE patients in response to therapy
Reference level: <25 IU/mL (considered negative for
SLE)
NB: Increased also in: chronic hepatitis, infectious
mononucleosis, biliary cirrhosis
Auto-antibodies in SLE
ANA
Anti-ds-DNA
Anti-Sm
Anti-leukocyte antibody
Anti-phospholipid antibody (lupus anticoagulant)
Believed to affect the coagulation cascade
o
by interfering with anti-coagulation factors,
as a result, the patient has a tendency to
form clots
ANA Fluorescent pattern
Homogenous - antideoxynucleoprotein
Peripheral - anti-ds-DNA
Nuclear membrane - DNA
Speckled - anti-ss-DNA
Pseudo ACA - anti-histone
Anticentromere - anti-Smith
Nucleolar - anti-RNP
Cytoplasmic - anti-nucleolar
Anti-nuclear lamins - anti-Scl-70
Anti-ribosomal - anti-proliferating nuclear Ag
Anti-centriole/centrosome/midbody - anti-mitotic
spindle
Mitotic spindle - anti-SS-A, SS-B, SS-C
Prognosis
-
Polymyositis
Primarily affects females (young to late middle age)
Clinically: muscle weakness with pain & tenderness
(shoulders, hips, neck)
Characterized by: inflammatory infiltrates in skeletal
muscle with necrosis & degeneration
NB: Dermatomyositis: when accompanied by skin changes
(maculo-papular or eczema-like skin lesion)
Serologically - presence of a number of auto-antibodies
directed against different tRNA synthetases
E.g., Antibodies to:
o
Jo-1 (histidyl tRNA synthetase) most
frequently detected
o
Threonyl & alanyl tRNA synthetase
Anti-PM-Scl (specific) also known as PM-1
o
Laboratory Findings
Increase in ESR (correlates with disease activity)
Increased in CPK (MM & MB) & aldolase (reflect
muscle injury & disease activity)
LDH & AST usually elevated
Increase LDH2 to LDH5 (esp LDH5): (indicates active
muscle necrosis)
Muscle biopsy: inflammation & muscle degeneration
& regeneration
Scleroderma
Antigen is not known
Female: male 3:1 (20-60y/o)
Most common symptom: Raynauds phenomenon*
(90% of patients)
CREST syndrome: milder form of scleroderma
Primarily: skin, but can lead to multi-organ
involvement (GIT, heart, lung, etc.)
NB: Scl-70: an ANA, 15-20%
-
Anti-Centromere Antibody
Very high percentage in patients with CREST
syndrome, (a variant, and milder form of
scleroderma)
Present only in a small minority of patients with
Scleroderma
Anti-Scleroderma Ab (Scl-70)
Diagnostic for scleroderma, present in 20-30% of
patients
Occasionally seen in: SLE, MCTD, SS, RA, Polymyositis
Increased levels in: INH, methyldopa, penicillin,
streptomycin, tetracycline, aspirin & propylthiouracil
usage
NB: Positive also for CREST syndrome
Sjogrens Syndrome
Most often occurs secondary to RA, SLE, scleroderma,
polymyositis
Partial or complete destruction of the salivary
&lacrimal glands by lymphocytes & plasma cells
In its primary form: manifested as
Keratoconjunctivitis sicca
o
o
Xerostomia
NB: Presence of RF & ANA , indicative of a systemic
disease
Antigenic alteration of salivary gland tissue sensitization,
lymphocytic infiltration, production of auto-antibodies against
the salivary & lacrimal glands damage
-
Anti-Nuclear RNP
One of the 4 anti-ENA (SS-A, SS-B and Smith)
Increased in: MCTD, SLE, discoid lupus
Antigens consist of RNA & protein
Rheumatoid Arthritis
Chronic, symmetric, erosive arthritis of the peripheral
joints
Female to male ratio 3:1 (30-50 years of age)
All patients: morning stiffness & joint pains that
improves through the day
Excessive production of Type 1 cytokines
RF: IgM (or IgG/IgA) to the Fc portion of IgG (these
are not specific but may enhance immune complexes)