Introduction
▪ Forbidden clone
▪ Altered antigen
▪ Sequestered Antigen
▪ Immunologic deficiency theory
▪ Genetic influence
Forbidden clone
▪ Some antigens in the body are hidden from cells of the immune
system.
▪ If there is damage to these organs causing exposure of these
sequestered antigens an immune reaction to these antigens may
occur.
Immunologic Deficiency Theory
▪ Patterns of reactivity:
▪ Homogenous-entire nucleus stained
▪ Peripheral-rim of nucleus stained
▪ Speckled-spots of stain throughout nucleus
▪ Nucleolar-nucleolus only stained
▪ False positives and negatives occur.
▪ If positive, perform profile testing.
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.
Homogeneous Pattern
▪ Smooth, even staining of the nucleus with or without
apparent masking of the nucleoli
Nucleolar
▪ 23 or 46 (or some multiple of 46) bright speckles or ovoid granules
spread over the nucleus of interphase cells
Peripheral
▪ Fluorescence is most intense at the periphery of the nucleus with a large
ring starting from the internal nuclear membrane and the rest of the
nucleus showing weaker yet smooth staining.
Speckled
▪ Large speckles covering the whole nucleoplasm, interconnected by a
fine fluorescent network.
Anti-nuclear antibodies detected by FANA
▪ Double-stranded DNA (ds-DNA) antibodies are most specific for SLE, correlate well
with disease activity.
▪ Antihistone antibody second major antibody found in SLE.
▪ Deoxyribonucleoprotein (DNP) antibody, responsible for LE cell phenomena and
available as a latex agglutination test.
▪ Anti-Sm antibody, specific for LE.
▪ SS-A/Ro and SS-B/La antibodies, most common in patients with cutaneous
manifestations.
▪ Anti-nRNP detected in patients with SLE as well as mixed connective tissue
disease.
▪ Presence of antibodies not diagnostic, may be present due to other diseases.
Anti-nuclear Antibodies by Immunodiffusion.
▪ Laboratory tests involve testing patients serum with red blood cells or latex
particles coated with IgG, agglutination is a positive result.
▪ Nephelometry and ELISA techniques are available to quantitate the RF.
▪ Erythrocyte Sedimentation Rate (ESR) used to monitor inflammation.
▪ C-Reactive protein (CRP) is utilized to monitor inflammation
Hashimoto's Thyroiditis
▪ 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
▪ Facial weakness,
▪ Difficulty chewing and swallowing,
▪ Inability to maintain support of trunk, neck or head.
Myasthenia Gravis
▪ Symptoms include:
▪ foamy,
▪ bloody, or dark colored urine,
▪ decreased urine output,
▪ cough with bloody sputum,
▪ difficulty breathing after exertion,
▪ weakness,
▪ fatigue,
▪ nausea or vomiting,
▪ weight loss,
▪ nonspecific chest pain
▪ and/or pale skin
Diagnosis
▪ Complete blood count (CBC)
▪ Blood urea nitrogen (BUN) and creatinine levels
▪ Urinalysis will be done to check for damage to the kidneys.
▪ Sputum test to look for specific antibodies.
▪ Chest x ray to assess the amount of fluid in the lung tissues.
▪ Lung needle biopsy and a kidney biopsy will show immune system deposits.
▪ Kidney biopsy can also show the presence of the harmful antibodies that attack the
lungs and kidneys
▪ Antiglomerular basement membrane (anti-GBM) antibody Enzyme immunoassay
(EIA)
▪ Antibodies to Neutrophil Cytoplasmic Antigens (ANCA) identified by
immunofluorescence
Sjogren's Syndrome
▪ CREST syndrome
▪ Calcinosis
▪ Raynaud’s
▪ Esophageal dysmotility
▪ Sclerodactyly
▪ Telangiectases
Calcinosis
▪ Clinical symptoms:
▪ Anemia
▪ Bleeding
▪ Hyperviscosity