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Autoimmunity
conditions in which damage to organs/tissue results from the presence of autoantibody/autoreactive cells
SLE manifestations
arthritis
erythematous skin rash
nephritis
neuropsychiatric
hematologic abnormalities
cardiac abnormalities
fatigue, weight loss, malaise, fever, anorexia
SLE lab features
presence of ANAs, circulating immune complexes, decreased complement (C3 & C4) levels, cyoglobulins, circulating anticoag & nonspecific elevation of IgG levels
Fluorescent ANA (Indirect immunoflrescence)
- most widely used and accepted because of high sensitive
- screening is done: 1:40 or 1:80 dilution of px serum
- titer of > or equal 160 is generally considered to be clinically significant
Homogenous/diffuse
uniform staining of the entire nucleus
Peripheral/Rim
greater staining intensity surrounding the nucleus
Speckled
discrete, fluorescent specks throughout the nuclei
Nucleolar
prominent staining of the nucleoli
Centromere
numerous discrete speckles (46 speckles) are seen in the nuclei
Anti-dsDNA
Staining pattern: homogenous/diffuse, peripheral/rim
Significance: active stage of SLE
Gold standard: IFA staining of Crithidia luciliae
Anti-DNP (deoxynucleoprotein)
Staining pattern: homogenous/diffuse, peripheral/rim
Significance: appearance of LE cells, found in 90% untreated SLE
Anti-Smith/Anti-Sm
Staining pattern: coarsely speckled
Significance: diagnostic of SLE
Anti-RNP
Staining pattern: coarsely speckled
Significance: SLE, RA, Sjorgen's syndrome, mixed connective tissue disease
Anti-histones (nuclear proteins)
Staining pattern: homogenous/diffuse
Significance: drug-induced lupus
Anti-Ro (SS-A)
Staining pattern: finely speckled
Significance: cutaneous/neonatal SLE
Anti-La (SS-B)
Staining pattern: finely speckled
Significance: SLE and Sjogren's syndrome
Anti-nucleolar RNA
Staining pattern: nucleolar
Significance: SLE, Sjogren's syndrome, Systemic sclerosis
Anti-centromere
Staining pattern: discrete, speckled
Significance: SLE, mixed connective tissue disease, CREST syndrome
Rheumatoid factor (IgM), Anti-CCP (cyclic citrullinated peptide)
Rheumatoid Athritis
Anti-thyroglobulin; anti-microsomal
Hashimoto's thyroiditis (HYPOTHYROIDISM)
Anti-TSHr; anti-microsomal
Grave's disease
Anti-islet cells
Type I DM (insulin-dependent/juvenile)
Anti-MBP (myelin basic protein)
Multiple Sclerosis
Anti-acetylcholine receptor
Myasthenia gravis (HL-B8) - muscle weakness
Anti-glomerular basement membrane
Goodpasture's syndrome - glomerulonephritis
Anti-mitochondrial
Primary Biliary Cirrhosis - obstruction of bile duct
Anti-smooth muscle
Chronic active hepatitis - autoimmune hepatitis
Anti-parietal cells
Pernicious anemia (Vit. B12 deficiency)
Anti-salivary/lacrimal glands
Sjorgen's syndrome
Anti-centromere
Scleroderma/CREST - thickening of skin/tissue
Primary Immunodeficiencies (PID)
inherited dysfunctions of immune system
(PID) Category 1: (SCID) Severe Combined Immunodeficiency
- most serious of the congenital immunodeficiencies
- present in early infancy; life span: 2 YEARS MAX
(PID) Category 1: Purine-Nucleosidde Phosphorylase Deficiency
- decreased number of T cells due to accumulation of deoxyguanosine triphosphate, a toxic purine metabolite
- moderate to severe CMI
- normal to mildly impaired HI
(PID) Category 2: Wiskott-Aldrich Syndrome (WAS)
- smallest platelets
- triad: immunodeficiency, eczema, thrombocytopenia
- lethal in childhood bc of infx, hemmorhage, or malignancy
(PID) Category 2: DiGeorge anomaly/Congenital Thymic Aplasia
- zinc deficiency
- thymus development abnormality
- quantitative defect in T cells
(PID) Category 2: Ataxia-Telangiectasia (AT)
- cerebral ataxia (involuntary muscle movements)
- telangiectasias (capillary swelling)
- abnormal GENES produce defect of both humoral & cellular immunity
(PID) Category 3: Transient hypogammaglobulinemia of Infancy
- low lvls of immunoglobulins (5-6 months of age)
- IgG most affected
(PID) Category 3: Selective IgA Deficiency
- most common CONGENITAL IMMUNODEFICIENCY
- susceptible to respiratory of GI infections
(PID) Category 3: Bruton's tyrosine kinase (BTK) deficiency
- X-linked inheritance (males mostly)
- lack circulating mature CD19+ B cells
- lack immunoglobulins of all classes
(PID) Category 3: Common Variable Immunodeficiency
- IgA & IgG deficiency
- spuelike syndrome & malabsorption
(PID) Category 3: Isolated IgG subclass deficiency
- most common: IgG4
- least: IgG1
(PID) Category 5: Chronic Granulomatous Disease (CGD)
- defective bacterial digestion (catalase + bacteria)
- X-linked or autosomal recessive
- inability to produce superoxide
- defective NADPH oxidase system
Neutrophil Oxidative Burst Assay
measures oxidation to rhodamine by the respiratory burst of the leukocyte
Nitroblue tetrazolium test (NBT)
spx: buffy coat
rgt: Bacterial suspension + NBT dye
(+) result: BLUE FORMZAN ppt
normal: 80-100% blue ppt
CGD: <50% blue ppt
(PID) Category 5: Leukocyte Adhesion Deficiency
- deficiency of CD18
- abnormal motility/chemotaxis, adhesion, and endocytosis
Plasma Cell Dyscrasia
- overproduction of a single immunoglobulin component (Myeloma protein - M)
Multiple Myeloma
- most serious & common of the plasma cell dyscrasia
- malignant plasma cells (CD38, CD56, CD138)
- presence of Bence-Jones protein in urine ( neg on urine strip, positive on SSA ppt)
- hypercalcemia, renal insufficiency and anemia
- 10% of bone marrow cells
Multiple Myeloma turbidity
ppt @ 40-60C = turbid
redissolves @ 100C = no turbidity
Waldenstrom macroglobulinemia
- malignant proliferation of IgM producing plasma cells
- HYPERVISCOSITY due to obstruction of blood vessels
- elevated serum monoclonal protein (macroprotein or IgM paraprotein) that migrates in the gamma region during SPE
Cryoglobulins
precipitate at cold temperatures and can occlude small vessels in the extremities in cold weather