Random Immunopath Flashcards
(337 cards)
1
Q
Joint pain and inflammation Fever Rash (salmon-pink) Lymphadenopathy Organomegaly Serositis / Hepatitis
Patient 5yo and more likely female
A
Systemic Onset JIA or Stills Disease
2
Q
Chondrocalcinosis
A
(Calcification of fibro and hyaline cartilage)
= Pseudogout
3
Q
Atophy
A
Type 1 IgE mediated allergy (most common allergy) 10% population. - Asthma - Hay Fever - Eczema - Foods - Urticaria and angioedema - Anaphylaxis
4
Q
Attacks synovial joints
A
Rheumatoid Arthritis, often progresses to synovial joint destruction. Also targets tendon sheathes and bursae
5
Q
Anti-bodies to double stranded DNA?
A
Highly Specific for SLE
6
Q
Stiffness WORSE in the Morning
A
Rheumatoid Arthritis
7
Q
Treatment or hyperacute rejection?
A
Organ Removal
8
Q
Issues in Organ Transplant besides rejection
A
Technical
Cold ischaemia time
Reperfusion injury
Functional status and co-morbidity of recipient
Rejection/Immunosuppression (Infection, Post-transplant-lymphoproliferative disease PTLD (EBV- mediated), Neoplasia - HPV SCC, HHV8 -Kaposi, Lymphoma)
Recurrence (viral, primary FSGS, mesangiocapillary GN, diabetic and IgA nephropathy)
9
Q
Role of Activated Macrophages in transplant rejection (HVGD)?
A
Injure graft cells and vasculature.
10
Q
25% of those with this go blind?
A
Giant Cell Arthritis
11
Q
ABO
A
Most important blood type system (or blood group system) in human blood transfusion. The associated anti-A and anti-B antibodies are usually IgM antibodies, which are produced in the first years of life by sensitization to environmental substances, such as food, bacteria, and viruses.
12
Q
Erosions
A
Sign of Joint Destruction in Rheumatoid Arthritis
13
Q
Steroid Dosing
A
High (20-40mg p/day)
Medium
Low (250mg given every 24-48 hrs
14
Q
Classification of Transplant Rejection?
A
Hyperacute
(Accelerated Acute)
Acute
Chronic
15
Q
Arthropathy in sacro-iliac and apophysial (facet joints)
A
Ankylosing spondylitis
Ddx
HLA-B27+ Enteropathic Arthritis
16
Q
Boutoniere deformities (V at interphalageal joint)
A
Sign of Joint Destruction in Rheumatoid Arthritis
17
Q
Scl-70
A
A poor prognostic indicator for Scleroderma.
18
Q
Artritis associated with
Abdo pain and melaena
Renal Disease (renal artery affected)
Rash
A
Polyarteritis Nodosa
19
Q
Obliterative intimal fibrosis
Glomeruli
Coronary arteries
A
Vascular damage associated with Chronic Rejection
20
Q
Tx in Giant Cell Arthritis affecting eye
A
Corticosteroids can reduce initial inflammation.
21
Q
Autoimmune reaction resulting in rejection of graft?
A
Humeral Rejection Type II (Autoantibody) immune response.
22
Q
Cystic Changes in Bone and Synovium
A
Osteoarthritis
23
Q
Arthritis of elderly with heavy male predominance.
A
Gout
24
Q
Antibody deficiencies
A
XLA CVID Hyper-IgM IgA Specific antibody deficiency Transient hypogammaglobulinaemia
25
Z-deformities (thumb)
Sign of Joint Destruction in Rheumatoid Arthritis
26
Inflammation & organ dysfunction due to Immune Complex deposition in the vasculature of affected organs
SLE
27
“Pencil in cup”
Erosive but central not peripheral
28
Arthritis found in 10-15% of IBD patients
Enteropathic Arthritis
29
X-linked (young men) Typically presents as a skin infection with +ve organisms (staph aureus, fungi, tc) with deep abscess formation
Chronic Granulomatous Disease
30
Type of rejection, common (10-30%) in Kidney transplants?
Acute Rejection
31
Fibrinoid necrosis of vessels and Cortical Infarction in transplant rejection?
Hyperacute Rejection
32
6wks affecting fewer than 4, mostly larger joints, ANA positive?
Oligoarthritis (Juvenile Idiopathic Arthritis Subtype)
33
Uurate overproduction due to:
Diet
Enzyme defects (known and unknown)
Decreased excretion
Primary Gout (90%)
34
Rheumatoid Nodules
Rheumatoid Arthritis
35
Polyconal Immunoglobulin
Treatment in humoral acute rejection.
On CD4 staining.
Also used = Polygonal Immunoglobulin and Anti-CD20
36
Non-complement fixing rejection
Accelerated Acute Rejection
37
Syndrome common in Turkey, Iran and Japan?
Behcet's Syndrome
38
Subluxations
Sign of Joint Destruction in Rheumatoid Arthritis
39
Arthropathy associated with Haemachromatosis?
Pseudogout
40
Ability of a test to indicate a negative result in the absence
of disease.
Specificity
41
Joint Effusions
Sign of Joint Destruction in Rheumatoid Arthritis
42
Role of Cytotoxic T-cells in transplant rejection (HVGD)
Kill cells in the grafted tissue, causing parenchymal and, perhaps more importantly, endothelial cell death (resulting in thrombosis and graft ischemia).
43
Negatitive birefringment
Gout
44
Gouty Tophi
Chronic Arthritis Gout
45
Tests in Septic Arthritis?
Aspirate
Stain
Culture
46
Paraprotein in blood and/or urine
Multiple Myeloma
47
Rejection characterised by loss of function w/o fever?
Acute Rejection.
48
Lip Biopsy in Sjogrens
Periductal and perivascular CD4+ TH cells, B cells, plasma cells , fibrosis
49
Arthritis usually sparing hips and rest of spine?
Rheumatoid Arthritis
50
HLA and ABO compatibility min requirement?
Pancreas Transplant
51
Butterfly rash
Systemic Lupus Erythematous
52
Patient in upper limbs may have a weak or absent pulse
Takayasu Arteritis
53
Absence of T cells (
Severe Combined Immunodeficiency (SCID)
54
Rejection marked by necrotizing vasculitis with thrombosis (when less acute mimics arterioscleotic thickening)
Humeral Acute Rejection (Antibody medicated)
55
Tx for Polymyalgia rheumatica?
Responsive to steroids
56
CD11a, 11b, 11c and CD18
Adhesion molecules (Neutrophil Function Disorder)
57
Histological signs of Polyartritis Nodosa?
Fibrinoid Necrosis
| Multiple Thrombosis
58
ABO Compatibility Essential
| HLA as possible
Bone Marrow Transplant
59
Indications for Glucocorticoids
Significant Acute Inflammation
60
Types of Chronic GVHD?
Progressive (worst Px)
Interrupted
De novo (best Px)
61
Rejection Antigens?
MHC (Minor HC antigens account for GVHD in Human Leucocyte Antigen - identical Bone Marrow Transplant)
ABO
Others
62
Small Vesses Vasculitis?
Microscopic Polyangitis
Wegner’s Granulomatosis
Churg-Straus
Henoch-Schonlein Purpura
63
Investigation for Anaphylaxis?
```
Mast cell tryptase
Demonstration of mast cell degranulation
Elevated levels seen from 1-12 hrs after rxn
90% specific
Sensitivity less good
```
Usually IgE-mediated
Food allergy
Other stimuli not excluded
64
ELISA meaning?
Enzyme Linked ImmunoSorbant Assay
65
Chondrocyte Proliferation?
Osteoarthritis
66
Arthritis affecting articular cartilage, with a periarticular bone response
Osteoarthritis
67
HLAB4 is a haplotype of this arthritis.
Rheumatoid Arthritis
68
```
Anti-nuclear antibody (ANA)
Anti-dsDNA antibody
ENA antibodies
Anti-RBC antibody
Anti-cardiolipin & lupus anticoagulant
```
All auto-antibodies of Systemic Lupus Erythematous
69
DR4 and DR1
Rheumatoid Arthritis
70
ANA patterns
Homogenous
Speckled
Centromere
Nucleolar
71
Autoimmune form of arthritis
Rheumatoid Arthritis
72
Ulnar deviation (fingers towards little finger)
Sign of Joint Destruction in Rheumatoid Arthritis
73
Detection of Anti-Nuclear Antibodies?
Indirect Immunofluoresence
74
Aldosterone
Main Natural Glucocorticoid
75
Recurrent infections – RTIs (lungs & ears chronic OM)
| Meningitis and septic arthritis
Agammaglobinaemia.
76
T-Cell Mediated Rejection?
Cellular Rejection (as opposed to humeral)
```
Cytotoxic T-Cells
+
Cytokine Sectreting CD4+ T-Cells
+
Activated Microphages.
```
77
Vasculitis
Rheumatoid Arthritis
78
MPO-ANCA
Anti-myeloperoxidase ANCA
```
=Microscopic PolyAngiitis
&
=Churg-Strauss Δ
- necrotising vasculitis
- asthma
- allergic rhinitis
- pulmonary infiltrates
- increased circulating eosinophils
- necrotizing granulomas (extrvascular)
```
79
No HLA, but ABO compatibility required?
Cardiac and Liver transplant
80
Process which has reduced the incidence of hyperacute transplant rejection?
Cross Matching
81
Type 2 Allergy
Autoantibody
82
RNP
100% Mixed Connective Tissue Disease
83
Monitoring of query “Hyperacute rejection'?
U/S Doppler ofr BF or Duplex
84
Squamous cell carinoma a side effect of which type of drug?
Immunosuppressants
85
Type of arthritis that increase in prevalence with increase in latitute?
Rheumatoid Arthritis
86
SLE affects who?
Females x9 more
20-30's
60% Concordance in Twins.
87
Low Risk CMV group in BMT
CMV- Recipient, CMV- Donor
88
Sequence for BMT Transplantation
1. Matchining (fully matched sibling can be used whole)
2. Viral Status (esp. CMV)
3. Conditioning of Recipient (eliminate residual immune system. Note: not required in Sever Combined Immune Deficiency (SCID).
4. Immunosuppression
5. Growth factor support.
6. Supportive blood products (CMV-)
89
Difference between direct and indirect cellular rejection?
Own (indirect) versus donor (direct) antigen presenting cells (APC’s) which are presenting the MHC (always from donor).
90
Caplans Syndrome
Rheumatoid arthritis (RA) and pneumoconiosis combination.
Manifests as intrapulmonary nodules, which appear homogenous and well-defined on chest X-Ray
91
ALG (Anti-lymphocyte globulin)
ALG is used as a treatment for vascular acute transplant rejection. Along with ATG
92
- Upper airways (necrotising granulomas)
- Necrotising granulomatous vasculitis (small-medium vessels)
- Necrotising glomerulonephritis
Wegners Granulomatosis
93
Genital ulcers
Eye: Uveitis (anterior or posterior) Retinal vasculitis
Skin: Erythema nodosum, Pseudofolliculitis, Papulopustular lesions
Pathergy
Minor skin injury precipitates pustules in a couple of days
±Constitutional symptoms
Behcet's Syndrome
94
Chrystal Arthritides
Pseudogout
95
ATG (Anti-Thymocyte Globulin)
Anti-thymocyte globulin is used in the treatment of vascular acute rejection. Along with ALG.
96
Podgara
Acute Arthritis Gout
97
Test for paraproteins?
Electrophoresis
98
Risk lymphoma 1.16 (MALTOMA)
Sjogrens
99
Asymmetrical arthritis of lower limb
Enteropathic Arthritis
100
Xenograft
From animal e.g. Pig heart valves
101
Pain on movement of joint
Rheumatoid Arthritis
102
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
103
The Secondary Osteoarthritis.
Metabolic – Gout &
Pseudogout (Chondrocalcinosis)
Infectious / Septic Arthrits
104
Rejection in
Acute GVHD
105
HLA-B8
Sjogrens
106
Treatment for chronic granulomatous disease?
Prophylaxis (co-trimoxasole, itraconasole)
| BMT
107
Transplant rejection over 1-5 days
Accelerated Acute Rejection
108
Tightening of skin around the mouth
Portal system of the liver
White out lung disease (acute vascular information)
GI fibrosis and dysmotilty
Chronic GVHD
109
Anti lymphocyte Globulins
Immunosuppressant
110
Arthritis within weeks after shigella, salmonella, yersinia, campylobacter or chlamydia.
Reactive Arthritis/Reiter Syndrome
111
Common causative organisms in Septic Arthritis?
Haemophilus influenzae predominates in children under age 2 years,
Staph. Aureus: older children and adults,
Gonococcus: young adulthood. mainly in sexually active women
Individuals with sickle cell disease are prone to infection with Salmonella at any age.
Gram negative bacilli (elderly),
112
Kaposi sarcoma a s/e of what drugs? Where else seen?
Immunosuppressants/HIV progression to AID's
113
Degenerative arthritis
Osteoarthritis
114
Mortality in BMT patients?
20-40%
115
Umbilical Cord Blood
Cord blood is collected because it contains stem cells, which can be used to treat hematopoietic and genetic disorders.
116
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
117
>20% Abnormal looking BM plasma cells
Multiple Myeloma
118
COL2A1 gene
Familial Osteoarthritis
119
```
Local factors affecting loading and wear (point loading)
“Secondary Osteoarthritis”
Injury
Obesity
Instability
Hypermobility
Joint dysplasia
Neuropathy
```
Secondary Osteoarthritis
120
Large cause of mortality in gout?
Gouty nephropathy (20% of gout mortality)
121
IgA immune complexes deposited in mesangium.
IgA nephropathy.
122
Pathogenesis of Gout
Hyperuricemia
Urate precipitates in joints forming crystals
1. Activation of complement and kinen system
Neutrophil chemotaxis
Release LTB4, prostaglandins and free radicals causes tissue inflamm.
Neutrophils phagocytose crystals, causing lysis of neutrophils .
Release of lysosomal enzymes leads to tissue inflaCammation.
2. Phagocytosis by macrophages
Leads to release of IL-1Beta and other cytokines in joint (resulting in further neutrophil chemotaxis)
Release of proteases leads to tissue inflammation.
123
“telescopic fingers” “whittling”
Arthritis mutilans due to osteolysis seen in 5% of Psoriatic Arthritis cases
124
FcR-bearing cells (T-Cells – primarily Cytotoxic)
Accelerated Acute Rejection
125
BMT graft sources
Allogenic (Match sibling or unrelated doner)
| Autologous
126
PR3-ANCA
```
Anti-proteinase-3 ANCA
= Wegener’s Granulomatosis
- Upper airways (necrotising granulomas)
- Necrotising granulomatous vasculitis (small-medium vessels)
- Necrotising glomerulonephritis
```
127
Seronegative destruction of articular cartilage and bony ankylosis
Ankylosing spondylitis.
| Usually affect sacro-iliac and apophysial joints
128
Anti-GBM antibody
Goodpastures
129
Suspected mechanism behind chronic rejection?
Recipient cells present MHC from the recipient organs
130
Lenght of post BMT neutropenia?
Should lift be 21 days
131
Should be excluded first in Dx of pseudogout?
Septic Arthritis
132
Iritis and conjunctivitis
Psoriatic Arthritis
133
RF+
Polyarthritis (Juvenile Idiopathic Arthritis Subtype)
Ddx
10% of Psoriatic Arthritis
Sjogrens (75%)
134
HLA-DR3
Sjogrens
135
Arthritis that peaks age 40, but can become symp in 40's
Rheumatoid Arthritis
136
Time to B-Cells post BMT?
Slow production for 1st 3months
If rapid in 1st 3mts then suspect autologous reconstitution or lymphoproliferative disease.
Plateaus out at between 6-9 months
137
Treatment for Rheumatoid arthritis?
Total Joint Replacement
| Joint Fusion.
138
Time to T-Cells post BMT?
Depends on T-Cell
1-24 for most.
Some never return.
Depends too on immunosuppression
139
Investigations in Sjogrens?
Lip biopsy
140
Intraarticular sodium urate chrystals
Gout (w/hyperuricaemia)
141
Anti-CCP
Rheumatoid Arthritis
142
Bone destruction with osteoporosis or lytic lesions
Often seen in Multiple Myeloma
143
Anti-PR3+
Granulomatosis with Polyangitis (GPA)
144
Cortisol
Main Natural Glucocorticoid
145
Anti-ACHr (receptor)
Myasthenia Gravis
146
Failure in inactivation of complement and kinin systems
Angioedema (deep tissue swelling)
Any part of body
Airway & gut (recurrent abdominal pain)
No urticaria or itch (tingling described)
Late childhood or teenage years
Hereditary Angioedema.
147
Routes to septic arthritis?
Direct inoculation through injury
Direct spread from adjacent infection
Haematogenous spread
148
Treatment for Acute GVHD?
Aggressive and early
| Steroids + Anti-T-Cell agents + Anti-TNFalpha
149
Ability of a test to indicate a positive result in the presence
of disease.
Sensitivity
150
Anti-CD20
Treatment used in humoral acute rejection.
On CD4 staining.
Also seen = Polyconal Immunoglobulin, Plasmapharesis
151
Most common glomerular disease?
IgA nephropathy.
152
Erythema of hands and fee (cutaneous) and of eyes and mouth (muco) t, e in children?
Query Kawasaki disease.
153
HLA-B51
Behcet's Syndrome
154
Graft manipulation for BMT
1. T-Cell Depletion (less GVHD, Less successful engraftment, Less graft v tumour)
2. Stem Cell (CD34+) Enrichment
155
Artritis affecting mostly small and medium arteries?
Polyarteritis Nodosa
156
Important test pre-intubation
Sign of RA on the Neck = X-Ray, Lateral and an AP. Shows narrowing, bony erosions etc.
157
Question mark spine
Ankylosing spondylitis
158
Granulomatous thickening of the aortic arch, which primarily affects the brachial artery yeilding a pulsless upper limb
Takayasu Arteritis
159
Sex affected more by kawasaki?
Male
160
Histological Pathogenesis of Rheumatoid Arthritis.
1. Synovial edema and sparse mononuclear infiltrate.
2. Synovial aggregates of mononuclear cells, CD4+ THELPER Cells, Plasma cells, Dendritic cells, Macrophages
3. Synovial hyperplasia with villous change
4. Increased synovial vascularity
5. Organising fibrin on synovial surface and neurtophils on synovial surface and in joint space.
6. Osteoclast activation = osteoporosis, junta-articular erosions, sub-chondral cysts.
7. Mass of boggy inflammed synovium PANNUS, can erode cartilage, bone and ligament.
8. Joint destroyed. Loss of articular cartilage. Fibrous ankylosis or Unstable
161
Tx for Aplasia/neoplasias/Herediatary immunodeficineies or metablic abnormalities?
Bone Marrow Transplantation
162
IgA EMA
```
Coeliac (90-100% sensitive and specific)
Dermatitis herpetiformis (50-70% sensitive, 90-100 specific)
```
False negative – gluten free diet
163
NADPH oxidase (Phox)
Chronic Granulomatous Disease
164
Secondary Amyloidosis
Protein Misfolding. Seen in...
| Rheumatoid Arthritis.
165
Type 4
Patch Test (Angry Back Syndrome)
166
- necrotising vasculitis
- asthma
- allergic rhinitis
- pulmonary infiltrates
- increased circulating eosinophils
- necrotizing granulomas (extrvascular)
Churg Strauss Syndrome
167
Mass of boggy inflammed synovium that can erode cartilage, known as what? Seen in What?
PANNUS
| Rheumatoid Arthritis
168
Treatment for acute transplant rejection?
```
Cellular
Steroids
Vascular
ATG, ALG
Humoral
C4 staining
Polyconal immunoglobulin, plasmapharesis, antiCD20
```
169
Deep, achy pain
Worsens with use
Short-lived morning stiffness
Osteoarthritis
170
Anti-La
Sjogrens (60-90%)
| SLE (10-15%)
171
Schirmer Tear Test
Sjogrens
172
Rejection that often relapses?
Accelerated Acute Rejection
173
Autologous
Self Organ Donation (graft etc)
174
Anti-MUSK (muscle specific tyrosine kinase)
Found in some anti-AchR negative MG
175
Anti-endothelial and smooth muscle antibodies
Kawakaski
176
DTH
Trigger DTH (delayed type hypersensitivity), with increased vascular permeability and local accumulation of mononuclear cells (lymphocytes and macrophages).
177
Necrotising Vasculitis?
Polyartrirs Nodosa
178
RANKL
Rheumatoid Arthritis
179
Joint Space Narrowing
Sign of Joint Destruction in Rheumatoid Arthritis
180
P-ANCA, purpura, lungs, kidneys, cvs, git, necrotizing glomerular nephritis, exogenous antigens from drugs , blood and infections.
Microscopic Polyangitis
181
Arthritis, more common in females than males
Rheumatoid Arthritis
182
Immune Complexes
Rheumatoid Arthritis
183
Young men
Buerger’s Disease(Thromboangitis Obliteran)
184
Triad:
Renal Disease
Chronic Sinusitis
Pneumonitis
ANCA+
Wegner’s Granulomatosis (Tx = Rapid Immunosup)
185
WNT signalling, Prostaglandin pathways
Osteoarthritis
186
Rejection between 6-90 days?
Acute Rejection
187
Immunocompetent donor T-cells recognize a mismatch with recipient HLA and initiate an immune response against an immunologically vulnerable host
Graft Versus Host Disease (GVHD)
188
Rate of GVHD in BMT?
30-50%
189
Rejection over months/years
Chronic Rejection
190
Seronegative (in 90%) arthritis associated with psoriasis 20-40yo?
Psoriatic Arthritis (10% RF+)
191
Definition of transplant rejection?
Aka HOST versus graft
“A process whereby the transplant recipient’s immune system recognizes the graft as foreign and attacks it with potential adverse effects on its viability and function”
192
Rheumatoid Factor
Seen in 80% of Rheumatoid Arthritis
non-specific
Antibodies (usually IgM) against IgG Fc portion
Marker for activity
193
Small vessel IgA & immune complex mediated vasculits often following upper-RTI in young children
Giveaway is lesions on the buttocks and legs
Henoch-Schonlein Purpura
194
Association between GCA and polymyalgia rheumatica?
50% of patients with Giant Cell Arteritis have symptoms of PMR and up to 20% of PMR patients have biopsy findings of GCA
195
```
Bilateral neck, shoulder and low back
pain and stiffness.
Worse in the morning
Malaise, tiredness, weight loss, depression and fever.
Elevated ESR and CRP.
```
Polymyalgia Rheumatica
196
TH1
Interferon Gamma (y) – Rheumatoid Arthritis
197
TNF
Rheumatoid Arthritis
198
Short Activng Glucocorticoid?
Prednisolone
Hydrocortisone
Methylprednisolone
Tramcinolone.
199
Low Magnesium
Pseudogout
200
Calcium pyrophosphate dihydrate deposition disease
Pseudogout
201
Rejection involving complement fixing
Hyperacute Rejection
202
Granulomatous arteritis of the aorta and larger vessels with a predilection for the extracranial branches of the carotids
Giant Cell Arthritis
203
Long Acting Glucocorticoid?
Dexmethasone
204
MEDICATION TO AVOID IN STEROIDS?
NSAIDS
205
Aortitis
Ankylosing spondylitis
206
Cyclosporine (FK506)
Immunosuppressant
207
Complications and Tx of X-linked Agammaglobinaemia?
IVIg and antibiotics
Complications...
Bronchiectasis
Chronic Sinus Damage
208
Treatment of SCID?
Isolation in +pressure
Antibiotics
Bone Marrow Transplant, possibly gene therapy.
209
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
210
Salivary gland enlargement F>M
Sjogrens
211
Serology of Rheumatoid Arthritis
1. Rheumatoid factor (80%)
non-specific
Antibodies (usually IgM) against IgG Fc portion
Marker for activity
2. APCA (anti-citrullinated peptide antibodies)
Specific for RA
212
TH17
Neutrophil recruitment – Rheumatoid Arthritis
213
HLA-DRB1*04
Polymyalgia Rheumatica
214
Rate of 5-10y graft survival?
(80%)
215
Artritis affecting large, small and medium arteries in children?
Kawakaski
216
Syngeneic
Monozygotic (identical) siblings
217
Presensitisation?
Now Countered by cross-matching
Irradiated to leucocyte deplete
Questionable increase in tolerance
218
Acute pain and swelling in knee, wrist or shoulder
Pseudogout
219
Lymphomas as s/e?
Immunosuppressants
220
Vaccination post BMT?
Follow guidelines
| No BMT.
221
HLA-DRB1*01
Polymyalgia Rheumatica
222
Cyanotic, mottled and flacid graft on removal of vascular clamps in Operating Room?
Hyperacute Rejection
223
Anti-Citrullinated Peptide Antibodies (ACPA) (AKA ACCP)
Rheumatoid Arthritis (Specific)
224
Sirolimus
TOR (Target of Rapamycin) inhibition.
| Thereby blocking IL2
225
Methods for improving graft survival?
1. Better Matching
| 2. Immunosuppression
226
Tx for Allergens
Avoid allergen – if possible
```
Anti-histamines
Self injectable adrenaline
Steroids – onset of action 6hrs
Individualised plan
life threatening reaction?
Chance of re-exposure?
```
Desensitisation – venoms, pollens
227
Maculopapular rash +
| Hepatosplenomegaly
Acute GVHD
228
Arthritis affecting young adults?
Polyarteritis Nodosa
229
Crepitus
Osteoarthritis
230
Age of onset in Multiple Myeloma?
After age 50 yrs
231
Subchondral Sclerosis
Osteoarthritis
232
```
Extensively matched
ABO (ABO mismatched are possible)
+
HLA (mostly DR)
+
X-matched for preformed antibodies
```
Kidney Transplant
233
Indirect cellular rejection
Usually chronic rejection
234
Prednisone and Cortisone
Biologically inactive synthetic
11 keto group requires activation to the 11-hydroxy group before having effect on cytosolic receptor.
Activated forms = Prednislone, Cortisol.
235
Pneumonitis
Rheumatoid Arthritis
236
Treatment for Pseudogout
Supportive
237
Arthritis of mostly men 20-40
Reactive Arthritis/Reiter Syndrome
Or
Ankylosing Spondylitis
```
Ddx
Possible Rheumatoid (early)
```
238
Felty's Syndrome
Combination of heumatoid arthritis, splenomegaly and neutropenia.
239
Lymph and Cardiac Signs of Kawasaki Disease?
Lymph = Cervical Lymphadenopathy
| Coronary Arteries = Aneurysm and MI
240
Arthritis in PIP's, 1sr MCP and 1st MTP joints
Osteoarthritis
241
Similar in appearance to systemic scleosis and Sjogrens?
Chronic GVHD
242
Loss of cartilage with subchondral bony change
Osteoarthritis
243
Monoclonal Antibodies (e.g. Monoclonal Anti-CD3)
Immunosuppressant
244
Agents used in Transplant Immunosuppression
Glucocorticoids
Calcineurin Inhibitors (Cyclosporine, Tacrolimus)
Antiproliferative/antimetabolic agents
Monclonal Antibody Therapies (cell specific)
245
Seronegative arthritis more common in males, average more common in mid-20's
Ankylosing spondylitis
246
Small non-weight bearing joints (Hands and Feet, C-Spine)
Rheumatoid Arthritis
247
btk
Brutons Tyrosine Kinase
Vital in maturation of Pro-BCell to Pre-Bcell
Defect = agammaglobulinaemia = IgA, IgG and IgM def
248
ACR/EULAR 2010 Criteria
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Rheumatoid Arthritis
Points for:
Joint Involvement
Serology
Acute phase reactants
Duration of symptoms
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249
Pathogenesis an Tx targets of Acute Rejection?
Endothelium is main target of pathology
CD4+ and CD8+ infiltrate (glomerular and tubular damage and endothelitis)
Necrosis
Heart: Lymphocyte infiltrate and myocyte damage
Vascular response may be subacute proliferation and fibrosis
Responds to aggressive immunosuppression (particularly anti-T cell strategies)
Progression to chronic rejection common
250
Increase PTH
Pseudogout
251
ANA reactivity clarification?
ELISA, RIA and Immunoblotting
252
ESR adjusted for age and CRP if this condition is suspected.
Giant Cell Arthritis
253
Hyperuricaemia
Gout (w/intraarticular sodium urate chrytals)
10% have hyperuricaemia, only 0.5% have gout
254
Gold Standard in Allergen Dx
Allergen Challange
255
Arthritis with temporal relationship to outbreaks of...
Parainfluenza
Parvovirus B19
Mycoplasma
Chlamydia
Polymyalgia Rheumatica
256
Overproduction of Urate
- Increased cell turnover
- Inborn errors of metabolism
Reduced excretion
-Chronic renal failure
Secondary Gout (10%)
257
ANA
Oligoarthritis (Juvenile Idiopathic Arthritis Subtype)
258
Cartilage Matric Metalloproteases
Rheumatoid Arthritis
259
Joint disorder common in 6wks
Juvenile Idiopathic Arthritis
260
Cell Cycle Inhibitors
Mycophenolate Mofetil
Inhibits Inosine, needed for nucleotide synthesis
Used in SLE
Azathioprine
Metabolised to 6MP in the liver.
261
Pulseless Syndrome
Takayasu Arteritis
262
Joint pain in 5yo
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Systemic Onset JIA. Also...
Fever
Rash (salmon-pink)
Lymphadenopathy
Organomegaly
Serositis / Hepatitis
```
263
Better HLA matching improves Graft survival?
Yes but....
As drugs for immunosuppression have improved, HLA matching is not even attempted in some situations, such as heart, lung, and liver transplantation; in these cases, the recipient often needs a transplant urgently.
264
Arthritis usually DIP, dactylitis, sausage finger/TOE 33%.
Also sometimes ankles, knees, hips, SI and facet joints
Asymetric, Histo resembling RA but not as destr.
Psoriatic Arthritis
265
Arthropathy associated with Alkaptonuria disease?
Pseudogout
266
Common site to observe synovial joint destruction
Metacarpal/interphalangeal joints
267
High Risk CMV group in BMT
CMV+ Recipient
| w/wo CMV+ Donor
268
Transplants associated with GVHD?
BMT (30-50%)
Blood Transfusion
Solid Organs, commonly LIVER (immune organ CD4+8 cells, transplanted a lot) (others =occasionally)
269
Sjograns w/o RA?
Primary Sjogrens
270
Treatment for Accelerated Acute Rejection?
Immosuppression, but only partially effective.
| Transplant removal often required.
271
Calcification of fibro and hyaline cartilage
Pseudogout
272
Sicca's (Sjogrens) Syndrome
Chronic autoimmune disease in which the body's white blood cells destroy the exocrine glands, specifically the salivary and lacrimal glands, that produce saliva and tears, respectively.[2] The immune-mediated attack on the salivary and lacrimal glands leads to the development of xerostomia (dry mouth) and keratoconjunctivitis sicca (dry eyes), which takes place in association with lymphocytic infiltration of the glands.
273
Infections soon after birth
- Recurrent bacterial
- Persistent Viral
- Opportunistic
- Persistent Thrush
Severe Combined Immunodeficiency (SCID)
274
Swan neck deformities
Sign of Joint Destruction in Rheumatoid Arthritis
275
Dx for SLE?
Clinical Hx & examination
Urinalysis & urine microscopy
Assess function of potentially involved organs.
Autoantibodies (Indirect immunofluoresence)
DIFF= Skin /Renal
276
Haematological features of SLE?
Immune thrombocytopaenia
Haemolytic anaemia
Autoimmune neutropaenia
277
Stages of osteoarthritis?
1. Chondrocyte Proliferation
2. Mediator Release
3. Cartilage Remodelling
4. Secondary Changes in Bone and Synovium
(Cystic Changes)
5. Loss of cartilage with subchondral bony change
278
Rejection involving preformed antibodies? Name the antibody types?
MHC 1 or ABO, mothers, previous transfusions and transplants)
279
Stills Disease
Systemic Onset JIA
280
Important consideration in IgE allergies
Sensitisation does not mean necessarily mean clinical allergy!
Negative result does not exclude allergy
Patient may need a challenge test
Negative SPT does not exclude allergy
281
HLA-DRB1
Rheumatoid Arthritis.
282
Volar subluxation (enlargement at knuckles caused by tendon slip)
Sign of Joint Destruction in Rheumatoid Arthritis
283
Citrulline
Rheumatoid Arthritis
284
Arthralgia and arthritis with skin and haematological abnormalities?
Systemic Lupus Erythematous
285
Symmetrical arthritis
Rheumatoid Arthritis
286
Less severe symptoms that reiter but nonetheless following infection?
Enteropathic Arthritis
287
Relatively refractory with progressive loss of graft function
Chronic Rejection
288
Subchondral Cysts
Osteoarthritis
289
Juxta-articular osteopaenia
Sign of Joint Destruction in Rheumatoid Arthritis
290
Jo-1
30% of Polymyosistitis
291
Time to NK cells post BMT?
1 month
292
Treatment for Granulomatosis with Polyangitis?
Cyclophosphamide
293
X-Chromosome (males) defect in B-Cell Maturation. Presents in early childhood.
XLA/Brutons Tyrosine Kinase Defect/X-linked agammaglobulinaemia
294
Anti-Sm (30%)
Anti-Ro (30%)
Anti-La
30% specific (except La) each for SLE
| All are anti-ENA (extractable nuclear antigen).
295
Commonest primary systemic vasculitis
Giant Cell Arthritis
296
MHC
Major Histocompatability Complex
Mediated Graft Versus Host disease in HLV identical BMT
Seen in Hyperacute rejection (MHC1)
297
Treatment for hereditary angioedema
Treatment
C1-Inh replacement (acute attacks)
Modified androgens (danazol or stanazol)
Bradykinin receptor anatagonists
298
Decreased range of movement
Osteoarthritis
299
HLA-CW6
Psoriatic Arthritis
300
Why are chronic rejections the most common?
Immunosuppresents so good it drags rejection out
301
Virus associated with Polyartritis Nodosa?
Hepatitis B
302
Arthritis in Lumbar and Cervical Spine
Osteoarthritis
303
Arthritis associated with old age?
Osteoarthritis
304
Immune complex mediated disease affecting any organ with diverse manifestations and variable prognosis?
Systemic Lupus Erythematous
305
Plamaphaesis
Treatment in humoral acute rejection. On CD4 staining. Also used = Plasmapharesis and Anti-CD20
306
The Seronegative immune mediated joint disorders
Ankylosing spondylitis
Reactive arthritis/Reiter syndrome
Enteropathic arthritis
Psoriatic arthritis
307
Serosistitis
Inflammation of the serosa. Seen in...
| Rheumatoid Arthritis
308
Graft Rejection Pathophysiology
APC-TCR = CD4 proliferation, release of IL2 and recruitment of Cytotoxic CD8+Tcells
309
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Arthritis (sausage finger, bony spurs)
+
Nongonococcal urethritis/cervicitis (+/- circinate balanitis)
+
Conjunctivitis
```
Reactive Arthritis/Reiter Syndrome
Also,
+/- oral ulceration, heart block, aortic regurgition
50% is chronic and resembles Ankylosing Spondylitis.
310
Varients of SLE limited only to the skin?
Discoid lupus erythematosus
| Subacute cutaneous lupus
311
Principle Antibodies in Rheumatoid Arthritis
```
Anti CCP (Specific 97%, sensitive 88%)
RF (Specific 65%, Sensitive 50-90%)
```
312
Anti-IL2-Receptor Antibodies.
Daclizumab and Basiliximab
| Prophylaxis against organ rejection.
313
Signs and symptoms of PANNUS/Rheumatoid Arth?
(4 basic signs of inflammation)
1. Calor
2. Rubor (
3. Tumour (swelling)
4. Dolar (pain)
314
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)
315
Commonest malignant disorder of plasma cells
Multiple Myeloma
316
Calcineurin Inhibitors
Cyclosporine, Tacrolimus (100x more potent).
Intracell signalling pathways to block IL2.
Mainstay of organ transplant.
S/E's Nephrotoxicity, Neurotixcity.
317
Weakly positively birefringent rhomboid crystals on polarisation.
May be cloudy and associated with fever and ↑ peripheral WCC
Result from joint aspirate
| =Pseudogout
318
BMT graft types
Bone Marrow
Peripheral Stem Cells (mat. T-cells)
Cord Stem Cells
319
Skip Lesions
| Often associated with polymyalgia rheumatica
Giant Cell Arthritis
320
6wks affecting more than 4, mostly smaller joints, RF+ positive? With poorer prognosis
Polyarthritis (Juvenile Idiopathic Arthritis Subtype)
321
Allogeneic
Living or Cadaveric (Another genetically different person donor)
322
Arthropathy associated with Wilsons disease?
Pseudogout
323
Rate of 10-20y graft survival?
(50%)
324
Rejection marked by an interstitial mononuclear cell infiltrate with associated edema and parenchymal injury,
Cellular Acute Rejection
325
Graft rejection in less
Hyperacute Rejection
326
IgM, IgG, IgA normalisation post BMT?
6-24 months
327
Hot, Red, one joint?
Septic Arthritis
328
Mechanical degradation of joints
Osteoarthritis
329
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Interstitial fibrosis
Tubular atrophy
Vanishing bile duct syndrome
Bronchiolitis obliterans
Gross scarring and shrinkage
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Chronic Rejection
330
Anti-Ro
Sjogrens (90%) also RF (75%)
331
Arteritis far more common in the elderly (>50) and affect F>M, 2:1?
Giant Cell Arthritis
332
Low Phosphate
Pseudogout
333
Schober's test (limited flexion (
Ankylosing spondylitis
334
Older person
| Unilateral Headache, facial pain and scalp tenderness.
Giant Cell Arthritis
335
Uveitis
Ankylosing spondylitis
336
Rejection, can be humeral or cell mediated, but is more commonly cell mediated?
Accelerated Acute Rejection
337
Role of Cytokine Secreting CD4+ T-Cells in transplant rejection (HVGD)?
Trigger DTH (delayed type hypersensitivity), with increased vascular permeability and local accumulation of mononuclear cells (lymphocytes and macrophages).
