Rheum 2 Flashcards
What is the number 1 characterization of Seronegative Spondyloarthropathies
sacroiliitis (SI) joint stiffness - axial arthritis
Enthesitis
- what is it?
- what rheum disorder is it seen in?
inflammation of the ligamentous-, tendinous-, fibrous-osseous junctions (where they insert into bone)
- seen in Seronegative Spondyloarthropathies
genetic association of Seronegative Spondyloarthropathies?
- RF?
- ANA?
HLA-B27
neg RF and ANA
Synovium of Seronegative Spondyloarthropathies
increased expression of TNFα
For Seronegative Spondyloarthropathies (ankylosing spondylitis AS)
Chances of developing AS is…
_____ % if HLA-B27 positive
_____% if HLA-B27 positive with a first-degree relative with AS
2%
20%
Reactive arthritis, presentation?
Asymmetric, oligoarticular, lower ext arthritis!!
Dactylitis
Reiter’s syndrome associated with what disorder?
Reactive arthritis
Dactylitis, what is it? what is it associated with?
Sausage digits - reactive arthritis
HLA-B27 Unfolded Protein Hypothesis Endoplasmic Reticulum Stress Response
is associated with what disorder?
Reactive arthritis
Fundamental defect of SLE?
misdirected recognition of self as foreign, resulting in an autoimmune process; T cell and B cell process
Which lymphocytes are involved in the autoimmune process of SLE?
T and B cell
In SLE, Antibody responses toward autoantigens are _____-driven and require ______
antigen
CD4+ T cells
In SLE, Loss of T cell tolerance permits what?
B cell stimulation: peripheral abnormality in self-reactive lymphocyte deletion or anergy (state of unresponsiveness)
greatest risk factor of SLE?
what other genetic factor is associated with SLE?
C4A null allele
HLA-DR3, +ANA
What are ANAs?
Hallmark of abnormal antibody production in SLE (not specific), Antibodies are directed to multiple nuclear antigens
- dont mix it up, C4A null allele still the most imp risk factor of SLE
What hypersensitivity is SLE?
II and III (lumpy bumpy)
Anti-phospholipid antibodies in SLE are associated with what?
increased clotting
Types of cells in Vasculitis?
infiltrating lymphs, monocytes, histiocytes, eosinophils, and PMNs
Pathophysiology of vasculitis
Immune complexes: inflamm→ PAFs→ ↑vascular permeability→ IC deposition; palpable purpura
- antigen driven inflammation
ANCA Vasculitis:
c-ANCA
- what are located in primary granules of PMNs, and what disorder is it associated with?
Cytoplasmic ANCA (c-ANCA):
Proteinase-3 (PR3) in primary granules of PMNs
- Associated with generalized GPA (Wegener’s)
ANCA Vasculitis:
p-ANCA
- what are located in primary granules of PMNs, and what disorder is it associated with?
Perinuclear ANCA (p-ANCA):
Myeloperoxidase (MPO) in primary granules of PMNs
Associated with microscopic polyangiitis (MPA)
Antineutrophil Cytoplasmic Antibodies (ANCAs): play a role in what?
Likely play a role in amplifying the inflammatory vascular response
Polymyositis / Dermatomyositis (PM/DM)
Inflammatory myopathies characterized by: Muscle weakness (proximal) and low endurance
DM presents with typical skin rashes:
Gottron’s papules Heliotrope rash V-sign and shawl-sign Mechanic’s hands Periungual changes/erythema
Anti-synthetase Syndrome
PM or DM presenting with:
Interstitial lung disease (ILD): 60%
Anti-synthetase antibodies:
- list the enzyme class, then the most common one
Anti-aminoaCYl-tRNA synthetases (CYtoplasm)
Most common: Anti-Jo-1 = anti-histadyl-tRNA synthetase
Are the antibodies in Anti-synthetase Syndrome pathologic?
Not pathologic or myotoxic antibodies
*recall, RF-IgG immune complexes ARE pathogenic
Polymyositis
- what type of distribution?
- Distribution in which T cells?
Endomysial distribution of inflammatory cells (CD8+ T cells) surrounding and invading muscle fibers
Dermatomyositis
- what type of distribution?
- Distribution in which T cells?
Perivascular (CD4+ T cells) and perifascicular inflammatory infiltrate
What rheum disorder is associated with a viral etiology?
Has live virus been cultured?
PM/DM
Viral particles by EM and viral RNA detected in muscle from PM/DM patients. NO live virus has been cultured from muscle
