autoimmune Flashcards
What does autoimmune refer to?
abnormal immune response directed toward a self-antigen
What is activated during an autoimmune response
B or T lymphs or both
T or F autoimmune diseases are idiopathic.
T
What are possible factors that may contribute?
- Genetic susceptibility to class 1 or 2 antibodies
- enviroment
- infections
- decreased cytokine production
- loss of regulatory T cells
What is ankylosing spondylitis?
rheumatic disorder producing inflammation of axial skeleton and large peripheral joints
ankylosing spondylitis affects whom?
- M:F ratio 3:1
20-40 y/o with a genetic link
ankylosing spondylitis
symptoms
back pain stiffness decreased chest expansion low grade fever, fatigue, anorexia weight loss/ anemia iritis
starts in thoraco-lumbar and expands sup. and inf.
ankylosing spondylitis has what lab test?
- HLA B27: not specific
ESR, CRP, IgG
ankylosing spondylitis shows what in diagnosis?
sclerosis in Si joints marginal sydesmophytes shiny corner sign bamboo spine trolly tracts dagger sign poker spine
all on xray`
autoimmune hemolytic anemia
what happens
autoantibodies attack RBC’s
autoimmune hemolytic anemia
who?
any age
more females
autoimmune hemolytic anemia
signs and symptoms
severe anemia
splenomegaly
increased unconjugated bilirubin
jaundice
autoimmune hemolytic anemia
tests
- Coomb’s
- Direct
- antiglobulin test
- CBC: increased MCHC
autoimmune hemolytic anemia
diagnosis
usually warm antibodies
celiac causes?
nontropical sprue/ celiac sprue
decreased gluten tolerance
celiac affects whom?
sensitivity to gluten w/ a genetic history
Celiac causes
signs
diarrhea
fatigue
weight loss
mouth ulcers
celiac
lab test
tTGA-IgA (antibody)
tissue transglutaminase
celiac
diagnosis
removal of gluten products
Crohn’s causes
regional enteritis
inflammation of distal ileum and colon, but may be entire GI tract
Crohn’s affects whom?
Female = Male
under 30 y/o
peak at 14-24 y/o
Crohn’s
signs and symptoms
right lower quadrant pain diarrhea anorexia weight loss malnutrition 1/3 fistulas and fissures
INFLAMMATION AND OBSTRUCTION
Crohn’s T or F
lab test is IgM antibody agglutination test
F
no test
Crohn’s
diagnosis
barium enema xray
string sign
apthous & linear ulcers in GI
skip and cobblestone lesions
What is DM1?
Juvinile Diabetes mellitus
panceas produces little or no insulin due to genetic or immune-related destruction of beta cells
DM1 affects whom?
people under 30
DM1
signs and symptoms
DKA (diabetic ketoacidosis)
polyuria and polydipsia
weight loss
DM1
tests
blood glucose
Hgb A1c
HLA-Dr3
HLA-DR4
DM1
diagnosis
labs
case history
clinical presentation
fibromyalgia produces what signs and symptoms?
- stiff, achy, tender pain in muscles tendons and adjacent soft tissuee
- poor sleep and fatigue
- anxiety
- IBS (irritable bowel syndrome)
fibromyalgia affects whom?
more females
young to middle aged
T or F
fibromyalgia has no known tests.
T
fibromyalgia
diagnosis
1. CH possible precipitating factors: 1. stress 2. trauma 3 virus 4 lyme 5. cold 6 damp must exclude other pathologies
T or F Goodpasture’s is a hypersensitivity disorder.
T
T or F
Goodpasture’s affects kidneys first then lungs
F
vice-versa
Goodpasture’ affects whom?
both sexes equally
18-30
50-65
Goodpasture’s has what signs and symptoms?
hemoptysis/ pulmonary hemorrhage
hematuria/ glomerulonephritis
iron deficiancy anemia
Goodpasture’ tests
- urinalysis
- blood: BUN/Creatinine
anti-basement
antibodies
HLA-DRw
Goodpasture’s
diagnosis
chest x-rays: bilateral patchy consolidations
What is the most common form of Graves’ disease?
thyrotoxicosis
Graves’ is caused by
hypermetabolism of thyroid and increased thyroid hormones
Graves’ affects whom?
more females than males
genetic history of thyroid or AI disease
Graves’ exhibit what signs and symptoms
exoptalmia
goiter
pretibial myxedema
skin: warm, moist, fine
increased sweating
frequent bowels
fatigue/weight loss/ insomnia/ nervous
Graves’ tests
TSH
free T4
Thyroid antibodies: LATS/ TSI/ TGI
Graves’ diagnosis
history
physical exam
lab tests
Plummer’s ds. (2nd most common cause of thyrotoxicosis
What is the most common aquired demylenating neuropathy?
Guillian Barre
Gullian Barre affects whom?
anyone
Gullian Barre AKA
Landry’s ascending paralysis
Gullian Barre signs and symptoms
symmetric weakness and paresthesia starting in legs and moving to arms
DTR’s lost
respiratory and autonomic dysfnx.
Can gullian barre be fatal?
yes
Gullian Barre has what tests?
increased CSF protein
evidence of recent infection: most common campylobacter jejuni
Gullian Barre diagnosis
nerve biopsy: shows de and remyelination
clinical symptoms.
Hashimoto’s akas
thyroiditis
myxedema
Hashimotos’s is
chronic thyroid gland inflammation with lymphocytic infiltration due to Ai
Hashimoto’s affects whom
females
Hashimoto’s signs ans symptoms
weight gain/ puffiness enopthalmia slurred speech/ thick toungue cold intolerance constipation
Hashimoto’s lab tests
- free T$/T3
- TSH
- TPO antibodies (antithyroid peroxidase)
- anti-microsomal antibodies
- anti-thyrogloulin antibodies
hashimoto’s diagnosis
history
physical exam
lab tests
WHAt does ITP mean?
Immue thrombocytopenic purpura
Antibodies attack platelets during
ITP
ITP affects whom?
children: acute
adults: chronic
ITP
signs and symptoms
sudden mucosal bleeding (epistaxis, gum, increased menstaration)
subcutaneous bleeding (petechia, eccymosis)
ITP
lab tests
NO GOLD STANDARD
autoantibodies:platelet ratio
decreased platelets
CBC:normal RBC and WC
ITP
diagnosis
bleeding symptoms
must rule out SLE
platelet antibody test is non specific
What is the most common demylenating disease of the CNS?
multiple sclerosis
What is MS possibly associated with?
temperate climate
MS affects whom?
F:M = 2:1
rare in children and adults over 50
MS
signs and symptoms
numbness, tingling, pain, burning, itching
cognitive problems
late: slurred speech and dementia
MS
lab test
CSF: oligoclonal bands
increased IgG
MS
diagnosis
MRI: high signal (white) plaque lesions at the brain and spinal cord
Lhermitte’s sign: pos
What causes decreased AcH receptors that lead to episodes of muscles weakness?
Myasthenia Gravis
Myasthenia Gravis affects whom?
more females than males
20-40
Myasthenia Gravis
signs and symptoms
Eyes; ptosis/ diplopia
muscle fatigue post exercise
dysartria, dysphagia, proximal limb weakness
later: quadriparesis bulbar symptoms (voice chocking nasal regurgitation
Myasthenia Gravis
lab test
AcH receptor antibody
Myasthenia Gravis
diagnosis
case history: signs and symptoms
edrophonium IV drug:mm function improves
electrophysiological testing
What is a skin disorder characterized by bullae and erosions of surface and mucosla areas?
Pemphigus
T or F
pemphigus can be fatal.
T
Pemphigus affects whom?
middle aged and elderly
high incidence in brazil
Pemphigus
signs and symtoms
Bullae and extensive skin erosions
starts in mouth and goes all over any stratified epithelium
painful ulcers
Pemphigus
lab tests
Tzanck test
immunoflorescent IgG on lesions
Pemphigus
diagnosis
CH of mucosal ulcers
Nikolsky sign: epidermis detaches from underlying skin
What develops in 50% of patients with Giant cell arteritis (GCA)?
Polymalgia rheumatica
Polymalgia rheumatica causes
pain and stiffnes in proximal muscles without atrophy and weakness
Polymalgia rheumatica affects whom
people over 50
Polymalgia rheumatica
signs and symptoms
pain and stiffness in muscles of: back, neck, hip, shoulders, thighs
no weakness or atrophy
malaise, fever, weight loss
stiffness after inactivity (gelling phen.)
Polymalgia rheumatica
lab test
increased ESR and CRP
normal:CK-MM, AST
CBC:normocytic normochromic anemia
Polymalgia rheumatica
diagnosis
differential: RA and MM
normal: EMG, mm. biopsy
What is characterized by inflammation and degenerative changes in muscle (poly) and skin (derm) that leads to symetrical weakness & atrophy of limb girdles?
polymyositis & dermatomyositis
polymyositis & dermatomyositis affects whom?
F:M= 2:1
40-60
5-15
polymyositis & dermatomyositis signs and symptoms
polymyositis: proximal muscle weakness and atrophy
fever/fatigue/weight loss
dermatomyositis: rash
periorbital edema w/ heliotrope hue (purple)= pathognomonic
lesions: dusky and red
skin may split
polymyositis & dermatomyositis
lab test
All mm. enzymes
increased CK, AST, LDH
thymic antibodies: Jo-1, PM-1
polymyositis & dermatomyositis
diagnosis
CH proximal mm weakness skin rash increased mm enzymes muscle biopsy: definitive EMG changes
Chronic recurrent skin pathology with dry, well-marginated scaly, silvery plaques and patches describes what?
Psoriasis
Psoriasis affects whom?
10-40 y/o
but really any age
Psoriasis signs and symptoms
characteristic lesions: scalp, extensor surfaces buttoks, sacral area, penis
Psoriasis lab tests include skin antibodies IgM and IgG. T or F
F no tests
Psoriasis
diagnosis
insection of lesions
case history
What is the difference between Raynaud’s disease and Raynaud’s phenomenon?
disease is bilateral w/ no underlying cause
phenomenon has an recognizable underlying cause
Raynaud’s is characterized by
spasm of arterioles with intermitten pallor and cyanosis
Raynaud’s affects whom?
more females
20-40
Raynaud’s signs and symptoms
arteriole spasm (acral: nose, fingers, tongue)
intermitten pallor or cyanosis
What tests for Raynaud’s?
no tests
Raynaud’s diagnosis
presentation: blanching or cyanosis of digits precipitated by cold or emotions
can occur with scleraderma bone resorption to the distal tufts of the digit
Rheumatoid Arthritis aka
felty syndrome
Felty’s=
splenomegaly & neutropenia
Still’s disease=
Juvenile RA
What is characterized by symmetric inflammation of peripheral joints advancing to destruction of joints & periarticular areas?
RA
RA affects whom?
females more than males
30-50
genetic link
RA
signs and symptoms
slow, progressive joint stiffness
fatigue, weakness, anorexia
“peripheral joint involvement advancing to tissues and organs”
RA
lab tests
IgM RF: not specific for RA
increased ESR, CRP
joint fluid: increased PMN’s HLA’s
DRbeta1
DR4
anemia: N, N (normocytic normochromic)
RA diagnosis
Based on 4 of 7 criteria that must be present for 6 weeks: 1. stiffness greater than one hour 2. arthritis in 3 or more joints 3. arthritis in hand joints 4. systemic arthritis 5 rheumatoid nodules 6. Serum RF antibody 7. x-ray changes rat bite reosions, deviations, ligamentous laxity
What is characterized by dry mouth, eyes, mucous membranes and often accompanies rheumatoid pathologies?
Sjogren’s syndrome
Sjogren’s syndrome affects whom?
more females
late 40s
Sjogren’s syndrome
signs and symptoms
occular symptoms (dryness) for more than 3 months
sicca=dryness
xerostomia= dry mouth
Sjogren’s syndrome
lab tests
schirmer test
rose bengal test
increased ESR, CRP
anemia develops
SS-B antibodies, but non-specific
Sjogren’s syndrome
diagnosis
CH
labs: non-specific
salivary gland test and biopsy
increased immune antibodies generally
What is a connetctive tissue disorder affecting joints, kidneys, heart, vessel walls and serous areas?
Systemisc Lupas Erythematosis
SLE affects whom?
more females
15-50
SLE
signs and symptoms
acute or chronic onset
constitutional symptoms (febrile rash/ alopecia “butterfly rash on face”
arthralgias
lung/kidney/heart (Libman-Sacks)
SLE
lab tests
ANA/FANA
Ds-DNA antibody
smith antibody
DNA and Smith Ab are specific but not sensitive
SLE
diagnosis
symptoms/ rash/ arthalgias
Lab Tests
can mimic RA of the hands due to ligamentous laxity which would include ulnar deviation, swan and boutonnieres deformity but will lack rat bit erosions
What is CREST involved with?
Scleroderma
What characterizes scleroderma?
diffuse fibrosis and degeneration of skin vascular supply, articulations and internal organs (GI, lung, heart, kidneys)
Scleroderma affects whom?
F:M = 4:1
30-50
Scleroderma signs and symptoms
thickening of the skin (esp. acral parts)
raynaud’s phenomenon
multi-organ: renal and lung
Scleroderma
lab tests
nonspecific: anti-Sci-70/ autoantibodies
eosinophilia
Scleroderma
diagnosis
clinical presentation: CREST
Calcinosis Raynauds Esophageal dysfunction Sclerodactyly Telangiectasia
x-ray: bone resorption to the distal tufts of the digits
Temporal arteritis aka
giant cell
What is characterized by cranial artery involvement (large blood vessels: carotid system) vessels with elastic tissue
Temporal arthritis
Temporal arthritis affects whom?
slightly more females
over 50
Temporal arthritis
signs and symptoms
bitemporal headache
visual disturbances
jaw claudication
polymyalgia rheumatica (40%)
Temporal arthritis
lab test
Big increase in ESR
anemia: N, N (normocytic, normochromic)
maybe increase in ALK. Phos
Temporal arthritis
diagnosis
biopsy of arteries
radiological exam of the tempotral artery with ultrasound yields a halo sign or a MRI with gadolinium based contrast
What is characterized by granulomatous arteritis of aorta and its branches?
Takyasu’s
Takyasu’s affects whom?
F:M = 8:1
15-30
Takyasu’s
signs and symptoms
non-descriptive constitutional (fatigue, malaise, night sweats, weight loss, arthralgias)
progression: vascular changes: syncope, TIA’s, HTN, decreased pulses
Takyasu’s
lab tests
NA non specific
increased ESR and CRP
increased IgM globulins
Takyasu’s
diagnosis
arteriograms showing occlusion or narrowing of aa
What is characterized by granulomatous inflammation of respiratory tract then progressins to vasculitis and glomerulonephritis?
Wegener’s granulomatosis
Wegener’s granulomatosis affects whom?
more males
any age
possible hypersensitivity history
Wegener’s granulomatosis
signs and symptoms
upper respiratory tract
nasal mucosa: red, raised, friable, bleeds
fever/fatigue/weight loss/ anorexia
Wegener’s granulomatosis
lab tests
ANCA (antineutrophilic cytoplasmic antibodies)
increased ESR and CRP
anemia
leukocytosis
renal function: increased creatinine/ BUN
Wegener’s granulomatosis
diagnosis
pulmonary & renal biopsies
lab tests