8/8 - Diseases of Immunity Flashcards
what disease has swan-neck and boutonniere deformities
rheumatoid arthritis
what is a chronic autoimmune disorder that principally attacks the joints, producing nonsuppurative prolierative and inflammatory synovitis
rhematoid arthritis
RA often progresses to ___ and in some cases ___
articular cartilage destruction; joint fusion (ankylosis)
extraarticulator lesions of ___ may involve skin, BV, lungs and heart leading to clinical manifestations that overlap w/ other autoimmune disorders including SLE and scleroderma
rhematoid artheritis
prevalence of RA in US
1% in US population
age of people affected by RA
2nd to 4th decades
what sex predominantly affects RA
women
what disease principally attacks joints; extraarticular lesions may involve skin, heart, BV and lungs
RA
what contributes to development, progression, and persistence of disease of RA
- genetic predisposition
- environmental factors
autoimmune response of RA is initiated by what
CD4+ helper T cells
pathologic changes of RA are mediated by what
antibodies against self antibodies against self antigens and inflammation induces cytokines, predoimnantly induced CD4+ t cells
activated ___ release inflammatory mediators that stimulate inflammatory cells leading to tissue injury in RA
CD4+ T cells
___ and ___ from ___ stimulate resident synovial cells to secrete proteases and destroy hyaline cartilage
TNF, IL-1, macrophages
RA begins with what symptoms
malaise, fatigue, and generalized musculoskeletal pain
joint involvement of RA begins when
after weeks to months - general symmetrical and effects small joints before larger ones
described joints involved in RA
swollen, warm, and painful
B)
which arthritis is better with movement? which arthritis doesn’t get better with movement?
osteoarthritis = movement is better
rheumatoid arthritis = movement doesn’t get better
what produces radial deviation of the wrist, ulnar deviation of fingers and flexion-hyperextension of the fingers
RA
in RA, what becomes edematous, thickened, and hyperplastic transforming its smooth contour to one covered by delicate and bulbous villi
synovium
characteristic features of RA tissue injury
- synovial cell: hyperplasia and proliferation
- dense inflammatory infiltrate
- increased vascularity
- fibrinopurulent exudate
- osteoclastic activity in subchondral bone
buzz word PANNUS = ___
RA
how does pannus cause RA
grows over synovium an causes erosion of articular cartilage. pannus then bridges opposing bones to form fibrous ankylosis which ossifies, resulting in bone fusion or bony ankylosis)
80% of patients have which antibodies in RA? what are these autoantibodies called?
IgM or IgA reacting against self-IgG = called rheumatoid factor (RF)
! what are diagnostic markers that can be detected in serum of up to 70% of RA patients
anti-citrullinated peptide antibodies (ACPAs)
RA tx
aimed at relieving pain and inflammation and slowing and arresting joint destruction
RA therapies
- corticosteroids
- immunosuppressants (methotrexate, TNF antagnoists)
what disease:
- dry eyes damage to eye surface
- dry mouth causes increased tooth decay
sjogren syndrome
what type is sjogren syndrome
type II
what is a secondary form of sjogren syndrome
dry eyes and mouth PLUS another autoimmune disease
the isolated disorder/primary form of Sjogren Syndrome is also called what
sicca syndrome
what diseases is sjogren syndrome most commonly associated with? what do other patients have?
most common: rheumatoid arthritis
other: SLE, scleroderma, vasculitis, and thyroiditis
characteristic decrease in tears and saliva in sjogren syndrome results in what
lymphocytic infiltration and fibrosis of lacrimal and salivary gladns
infiltrate in sjogren syndrome contains what
activated CD4+ helper T cells , some B cells including plasma cells
what is the initiating trigger of sjogrens syndrome
viral infection of salivary glands, which cause local cell death and release of tissue self antigens
___% of patients with sjogrens have rhematoid factor whether coexisting RA is present or not
75%
pathogenesis of sjogrens
- decreased saliva and tear from inflammatory destruction of exocrine glands
- lymphocytic infiltration and fibrosis of lacrimal and salivary glands
who does sjogrens most commonly occur in
women between 50 and 60, and rarely in chilcdren
what produces blurring of vision, burning and itching, and thick scretions
keratoconjuncitivitis associated w sjogrens
what results in difficulty in swallowing solid food, a decrease in in ability to taste, cracks and fissures in mouth, and dryness of oral mucosa
xerostomia associated w sjogrens
parotid gland enlargement is seen in what percent of sjogrens patients
50%
how does saliva appear in sjogrens
frothy, with lack of usual pooling saliva in floor of mouth
how does tongue appear in sjogren
fissured and exhibits atrophy of papillae
how does oral mucosa appear in sjogrens
red and tender and usually results in secondary candidiasis
buzz word “fruit-laden, branchless tree” = ___
sjogrens syndrome
what disease:
sialo graphic examination often reveals punctate sialectasis and lack of arborization of ductal system
sjogrens
presence of ___ can be helpful to clue in diagnosis
autoantibodies
positive rheumatoid factor found in ___ of sjogrens patients, regardless whether the patient has rheumatoid arthritis
60%
antinuclear antibodies (ANA) are present in ___% of sjogrens patients
75-80%
antibody SS-A (anti-Ro) and anti SS-B (anti-La) may be found in what patients
sjogrens syndrome
sjogren syndrome tx
most supportive:
- artifical tears
- artificial saliva
B)
about 5% of pt with sjogren syndrome develop ___ and incident that is 40-fold greater than normal
lymphoma
what type is systemic sclerosis/scleroderma
type II
systemic sclerosis is also called what
scleroderma
systemic sclerosis is characterized by what
- chronic inflammation
- widespread damage to small BV
- progressive interstitial and perivascular fibrosis in skin and multiple organs
what is most affected in systemic sclerosis? what else can be affected?
most affected: skin
other: GI tract, kidneys, heart, muscle, lungs
