autoimmune Flashcards
examples of autoimmune disease
-brain- multiple sclerosis, guillaun-barre syndrome, autism
-thyroid- thyroiditis, hashimoto’s disease, graves disease
-bones- rheumatoid arthritis, ankylosing spondylitis, polymyalgia rheumatica
-muscles- muscular dystrophy, fibromyalgia
-skin- psoriasis, vitiligo, eczema, scleroderma
-nerves- peripheral neuropathy, diabetic neuropathy
-GI tract- celiacs, crohns, ulceratic colitis, diabetes type 1
-blood- leukemia, lupus erythematosus, hemolytic dysglycemia
autoimmune
-Receptors on T & B cells of the immune system are antigen specific with diversity derived from gene recombination
-Cells have memory and on a subsequent engagement with an antigen the cells exhibit more rapid and robust responses
-Immune response to self-antigens as in autoimmune & failure to recognize pathogens and eliminate
-Autoimmune Diseases: immune response to self antigens occur in the context of a genetic predisposition resulting in disease expression. Can be organ or system.
Immunodeficiency: primary vs secondary
Primary are a direct consequence of structural or functional derangement in the immune network.
Secondary are a consequence of an alteration in the immune status resulting in manifestation of infectious disease.
systemic autoimmune diseases: connective tissue: systemic Lupus Erythematosus
-Multisystem autoimmune disease, associated with the production of antibodies to a variety of nuclear and cytoplasmic antigens
-Antibodies to ds DNA, causes inflammation to skin, joints, kidney, cardiovascular system, ervous system,lung and hemopoietic cells.
-Diagnosis with criteria of signs (rash, arthritis, oral ulcers, etc) proteinuria, cytopenia
-+ anti-ds DNA
-+ antiphospholipid antibody
-+ anti-Sm antibody
-false positive syphilis (boards q)
-abnormal (speckled) ANA -> usually first test
-butterfly rash
-arthritis, raynauds, lupus nephritis, heart problems, pleural effusions
systemic autoimmune diseases: connective tissue: sjogren syndrome
-Systemic connective tissue disorder
-MC in women than is men
-an autoimmune exocrinopathy involving the lacrimal GLANDS, salivary glands and less often the pancreas
-Inflammation contributes to sicca syndrome, dry eyes and mouth.
-Positive ANA, positive SS-A, positive SS-B, positive rheumatoid factor
-Can be primary or associated with another autoimmune disorder.
-Test for sicca
-Dry eye Schirmer test- tissue paper put up to eye to measure how far down the wet travels
-Biopsy of salivary gland
-2.5% develop non-Hodgkin Lymphoma
-joint pain, abnormal liver, digestive problems, muscle pain, vaginal dryness, neurological problems, recurrent bronchitis
-fertility problems due to lack of fluid
systemic autoimmune diseases: connective tissue: scleroderma
-Excessive and widespread deposition of COLLAGEN in many organ systems of the body
skin, GI tract, kidney(renal ischemia), lung and muscles:
- tissue fibrosis,
- proliferative and occlusive vasculopathy of the small blood vessels
- specific autoimmune response associated with distinctive autoantibody profile
-Subtypes including CREST (Calcinosis, Raynaud, Esophageal dysmotility, Sclerodactyly and Telangiectasia)
-pulmonary HTN -> CHF, lung problems -> can die
-Positive ANA, antibody Scl-70
-Calcinosis- calcium deposits in skin
-Raynauds phenomenon
-Esophageal dysfunction- acid reflex and decrease motility
-Sclerodactyly- thickening and tightening of skin on fingers and hands
-Telangiectasis- dilation of capillaries causing red marks on surface of skin
systemic autoimmune diseases: connective tissue: inflammatory muscle diseases
-3 types:
-1. dermatomyositis. Heliotrope rash, shawl sign, Gottron papules. Complement vasculopathy.
-2. Polymyositis. T-cell medicated muscle injury
-3. Inclusion body myositis. Older
-Pulmonary interstitial fibrosis in 10%,
-3/5 criteria present -> proximal muscle weakness, muscle pain and tenderness on palpation, EMG abnormal, elevated serum/plasma concentrations of muscle enzymes, muscle bx with cellular inflammation
-Positive ANA, anti-Jo
-calcification in skin, joint pain, arthritis, arrhythmias, myocarditis, pericarditis, gastroesophageal reflex disease, dysphagia, aspiration pneumonia
-progressive muscle wasting
-fever, fatigue, weight loss
systemic autoimmune diseases: connective tissue: mixed connective tissue disease
Combined features of SLE, sclerosis, and polymyositis
- combo of scleroderma, rheumatoid arthritis, lupus, sjogrens
Sx:
-Arthralgias, myalgias, fatigue & Raynaud phenomenon.
-Pulmonary (interstitial pneumonitis, Pulmonary HTN, Intersitital Fibrosis, diaphragm & esophagus disfucntion)
Dx
- by clinical features: must have synovitis or myositis
- positive ANA, anti-U1 RNP
systemic autoimmune diseases: connective tissues: rheumatoid arthritis
-primarily affects the synovial joints often starting as a synovitis.
-Young women.
-3/7 criteria with morning stiffness.
-Increased serum Rheumatoid Factor, anti- CCP
wasnt on slide:
→ 3/7 criteria w/ morning stiffness 6+ weeks
● Arthritis in 3+ small joints
● Morning stiffness >30 mins
● Arthritis of small joints in hands
● Rheumatoid nodules
● Symmetrical arthritis, often w/ synovitis
● Positive test for RF
● Radiographic changes affected joints
systemic autoimmune diseases: connective tissue: amylodosis
-Extracellular deposition of low-molecular-weight fibrils from a soluble circulating precursor giving a waxy appearance to the infiltrated organs.
-Congo red stain on microscopy, abdominal fat pad,
-Multiorgan involvement Factor X coagulopathy
-Male 2:1, onset 40’s
-fatigue
-SOB
-protein in urine
-BP changes
-dizzy
-pain
-kidney issues
-stiff heart
-diarrhea/constipation
-weight loss
systemic autoimmune diseases: connective tissue: cryoglobulinemia
-Immunoglobulins in the serum that precipitate at a temp below 37C, resolves on warming.
Sx:
-Immune complex vascultitis, arthritis, neuropathy, renal involvement
-red blotchy rash**
-occurs at cold temp and resolves with warm
Dx:
-Wintrobe tube collects cyroglubulin reported as a cryocrit.
positive ANA flow chart
-order the whole panel at once
-anti-jo differentiation is based on symptoms
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diseases of immune system: X-linked agammaglobulinemia
-Absence of b lymphocytes in males
-Recurrent infections
disease of immune system: common variable immunodeficiency
-Male & females in adult
-B cells are dysfunctional
-Recurrent infections, immune hemolytic anemia, neutropenia, pernicious anemia, B cell Lymphoma
-chronic cough
-swollen lymph nodes
-breathing trouble
-pain in ear
-nausea, vomiting, and diarrhea, weight loss
disease of immune system: hyper IgM syndrome
-low IgG and IgA with normal IgM OR high IgM, normal B cells
inability to switch antibody production from IgM to other types of antibodies (IgG, IgA, and IgE). This leads to an excess of IgM antibodies and a deficiency of other types
