LAB 9 - Autoimmunity Flashcards
Compare and contrast hypersensitivity and autoimmunity.
Hypersensitivity: excessive response to a previous sensitization/exposure to antigen
Autoimmunity: not recognizing self antigens
Systemic sclerosis
Etiology: antigen activates CD4+ T cell -> cytokines released -> activating fibroblasts & collagen deposition
Dense Collagen deposits in skin
Thickening of skin Fibrosis - Pulmonary, esophageal, GI, endothelium Raynaud's Phenomenon Polyneuropathy Swollen Hands/forearms/face/trunk Ulcers
Female:Male = 3:1 50-60 y.o.
EARLY = distal extremity skin changes LATE = edema/fibrosis
Limited: only 1 organ/tissue system
Diffuse: starts diffues/widespread - everywhere; more than 1 organ system; rapid progression
CREST Syndrome:
C - Ca deposits
R - Raynaud’s
E - Esophageal dysmotility (collagen deposits in smooth muscle)
S - Sclerodactyly (tough fingers)
T - Telangestasia (dilated BV resulting from vascular injury)
Sjogren Disease
most often in Women (50-60 y.o)
Salivary & Lacrimal - other exocrine glands too
synovitis, diffuse pulmonary fibrosis, and peripheral neuropathy
Primary (alone) vs Secondary (with other Autoimmune disorders - RA & SLE)
- Eyes: cornea inflamed, eroded, and ulcerated
- Mouth: atrophy of mucosa with inflammatory fissuring and ulceration; dental caries.
- Nose: dryness and crusting leading to ulcerations and even perforation of the nasal septum.
- Parotid gland enlargement in 50% of patients (unilateral or bilateral).
Tests: rheumatoid factor (RF) + anti-Ro + anti-La + ESR=42 mm/hr Schirmer’s test + (lacrimal gland secretion flow rate)
keratoconjunctivitis
Inflammation of cornea and conjunctiva
Dry eyes
xerostomia
dry mouth
Mikulicz syndrome
abnormal enlargement of glands in head & neck
Lymphoma, Sjorgren’s, sarcoidosis, Goiter
Amyloidosis
Deposition of mis-folded proteins (in the cross-linked Beta pleated sheet form)
Primary - on its own => MI
Secondary - result of another disease - chronic infection/inflammation
Congo Red dye => Apple Green under polarized LM
Transthyretin-derived (senile, AS)
Amyloid Light-chain (AL)
Alzheimer’s Chronic inflammation, Cancer, Type I Diabetes
Dx with Rectal Biopsy
RF
Rheumatoid factor
Autoantibody related to Rheumatoid Arthritis (& other autoimmune diseases)
Antibody agains Fc portion of IgG
Anti-Ro
Anti-Ro - + in SLE & Sjorgrens
Anti-La
Anti-La - Sjorgren’s Syndrome
ESR
Erythrocyte Sedimentation Rate
the rate at which red blood cells sediment in a period of one hour
Men = 12-19 Women = 18-23
ANA
Anti-nucleate antibodies
Anti-Ro - + in SLE & Sjorgrens Anti-La - Sjorgren's Syndrome Anti-Sm - specific for SLE Anti-nRNP - SLE Anti-Scl-70 - scleroderma Anti-dsDNA - polymyositis & dermatomyositis Anti-histone - drug-induced Lupus Anti-centromere - CREST syndrome & cutaneous systemic sclerosis
Non-Hodgkin’s B Cell Lymphoma
types of cancer derived from blood cells (all except for Hodgkin’s Lymphoma)
Raynaud’s Phenomenon
excessively reduced blood flow in response to cold or emotional stress, causing discoloration of the fingers, toes, and occasionally other areas
stays white
i.e Christina Tsoriano
anti-topoisomerase antibody
autoantibodies directed against topoisomerase and found in several diseases, most importantly scleroderma
BUN
Blood urea nitrogen
Checks kidney function
Normal = 6-20 mg/dL
Urea nitrogen is what forms when protein breaks down
Creatinine
Serum = Blood creatinine levels Urine = Urine creatinine levels
Checks kidney function
Normal = 0.6-1.3 mg/dL (blood)
Total Protein
Total amount of protein in blood
Albumin & Globulins
Nutritional problems, kidney/liver disease
Normal = 6-8g/dL
Orthopnea
ortho = straight pnoia = breath
SOB when lying flat
Has to sleep propped up in bed or sitting in a chair
Nocturia
having to get up at night to urinate
Albumin
Albumin (from Liver) circulating in blood serum
Decreased = Kidney/Liver diseases
Increased = High protein diet, dehydration
Normal = 3.5-5.5g/dL
Waldenström’s macroglobulinemia
cancer affecting B cells that produce excessive IgM, thickening of the blood
- Unknown cause that causes adenopathy, hepatosplenomegaly, peripheral neuropathy and hyperviscocity (too much stuff in blood -> thickens)
Globulin
Liver function
Increased:
Acute infection
Multiple myeloma
Chronic inflammatory disease
(RA or SLE)
Waldenstrom macroglobulinemia
Polymyositis/Dermatomyositis
many muslces
muscle destruction b CD8+ T cell attack
Dermatomyositis = muscle & Skin
Perivascular muscular atrophy with mononuclear cell infiltrate
Immunifluorescence = patchy deposits of Ig
CPK
Creatine phosphokinase
Enzyme found in heart, brain, and skeletal muscle (non regenerative tissues)
Elevated levels indicate tissue damage to heart, brain or skeletal muscle
Normal = 10 - 120 mcg/L
Grotten’s lesions
Bilateral red papules on elbows, knuckles and knees
Characteristic antibody for dermatomyositis
Anti-Jo-1
Juvenile dermatomyositis (JDM)
Pediatric counterpart of dermatomyositis
S/s:
1) distinctive rash - Grotten’s lesions
2) vasculitis from muscle inflammation
