Immunopathology Awkwards Flashcards
HLAB4 is a haplotype of this arthritis.
Rheumatoid Arthritis
HLA-DRB1
Rheumatoid Arthritis.
TH17
Neutrophil recruitment – Rheumatoid Arthritis
TH1
Interferon Gamma (y) – Rheumatoid Arthritis
RANKL
Rheumatoid Arthritis
TNF
Rheumatoid Arthritis
Anti-Citrullinated Peptide Antibodies (ACPA)
Rheumatoid Arthritis (Specific)
COL2A1 gene
Familial Osteoarthritis
WNT signalling, Prostaglandin pathways
Osteoarthritis
Caplans Syndrome
Rheumatoid arthritis (RA) and pneumoconiosis combination.
Manifests as intrapulmonary nodules, which appear homogenous and well-defined on chest X-Ray
Felty’s Syndrome
Combination of heumatoid arthritis, splenomegaly and neutropenia.
ANA
Oligoarthritis (Juvenile Idiopathic Arthritis Subtype)
RF+
Polyarthritis (Juvenile Idiopathic Arthritis Subtype)
Ddx
10% of Psoriatic Arthritis
Sjogrens (75%)
Stills Disease
Systemic Onset JIA
HLA-B27
95% w/Ankylosing spondylitis are HLA-B27+
8% of Caucasian population = HLA-B27+
80% w/Reactive Arth/Reiters are HLA-B27+
Enteropathic Arthritis w/sacroiliitis or spondylitis
Psoriatic Arthritis
HLA-CW6
Psoriatic Arthritis
HLA-B8
Sjogrens
HLA-DR3
Sjogrens
Anti-Ro
Sjogrens (90%) also RF (75%)
Lip Biopsy in Sjogrens
Periductal and perivascular CD4+ TH cells, B cells, plasma cells , fibrosis
Anti-La
Sjogrens (60-90%)
SLE (10-15%)
MPO-ANCA
Anti-myeloperoxidase ANCA
=Microscopic PolyAngiitis & =Churg-Strauss Δ - necrotising vasculitis - asthma - allergic rhinitis - pulmonary infiltrates - increased circulating eosinophils - necrotizing granulomas (extrvascular)
PR3-ANCA
Anti-proteinase-3 ANCA = Wegener’s Granulomatosis - Upper airways (necrotising granulomas) - Necrotising granulomatous vasculitis (small-medium vessels) - Necrotising glomerulonephritis
FcR-bearing cells (T-Cells – primarily Cytotoxic)
Accelerated Acute Rejection
Anti-CD20
Treatment used in humoral acute rejection.
On CD4 staining.
Also seen = Polyconal Immunoglobulin, Plasmapharesis
Minor histocompatibility antigens
Non-MHC encoded polymorphisms
Cytokine polymorphisms
Mechanism of GVHD which avoid “Matching”
Occasionally seen in autologous transplants die t re-emergence of autoreactive t-cells
HLA-DRB1*04
Polymyalgia Rheumatica
HLA-DRB1*01
Polymyalgia Rheumatica
P-ANCA, purpura, lungs, kidneys, cvs, git, necrotizing glomerular nephritis, exogenous antigens from drugs , blood and infections.
Microscopic Polyangitis
Triad:
Renal Disease
Chronic Sinusitis
Pneumonitis
ANCA+
Wegner’s Granulomatosis (Tx = Rapid Immunosup)
Typically affects vessels of respiratory tract, strongly associated with asthma, eosinophilia and pulmonary infiltrates. May also have GI Sx (e.g. colitis, bleeding) +/- cardiac +/- peripheral neuropathy Sx’s.
P-ANCA+ and IgE
Churg-Straus Syndrome (Tx = Glucocortiocids +Support)
Anti-nuclear antibody (ANA) Anti-dsDNA antibody ENA antibodies Anti-RBC antibody Anti-cardiolipin & lupus anticoagulant
All auto-antibodies of Systemic Lupus Erythematous
Anti-Sm (30%)
Anti-Ro (30%)
Anti-La
30% specific (except La) each for SLE
All are anti-ENA (extractable nuclear antigen).
Jo-1
30% of Polymyosistitis
RNP
100% Mixed Connective Tissue Disease
Scl-70
A poor prognostic indicator for Scleroderma.
IgA immune complexes deposited in mesangium.
IgA nephropathy.
Anti-GBM antibody
Goodpastures
Anti-CCP
Rheumatoid Arthritis
DR4 and DR1
Rheumatoid Arthritis
Principle Antibodies in Rheumatoid Arthritis
Anti CCP (Specific 97%, sensitive 88%) RF (Specific 65%, Sensitive 50-90%)
Anti-PR3+
Granulomatosis with Polyangitis (GPA)
Anti-ACHr (receptor)
Myasthenia Gravis
Anti-MUSK (muscle specific tyrosine kinase)
Found in some anti-AchR negative MG
IgA EMA
Coeliac (90-100% sensitive and specific) Dermatitis herpetiformis (50-70% sensitive, 90-100 specific)
False negative – gluten free diet
Dx of Multiple Myeloma
Monoclonal expansion of paraprotein secreting plasma cells in bone marrow
IgG 60% of cases
IgA 20% of cases
Light chains alone 15-20% of cases
NADPH oxidase (Phox)
Chronic Granulomatous Disease
CD11a, 11b, 11c and CD18
Adhesion molecules (Neutrophil Function Disorder)
Atophy
Type 1 IgE mediated allergy (most common allergy) 10% population. - Asthma - Hay Fever - Eczema - Foods - Urticaria and angioedema - Anaphylaxis
Type 2 Allergy
Autoantibody
Type 4
Patch Test (Angry Back Syndrome)
Graft Rejection Pathophysiology
APC-TCR = CD4 proliferation, release of IL2 and recruitment of Cytotoxic CD8+Tcells