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immunopath > Random Immunopath > Flashcards

Random Immunopath Flashcards

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

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2
Q

Chondrocalcinosis

A

(Calcification of fibro and hyaline cartilage)

= Pseudogout

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3
Q

Atophy

A 
Type 1 IgE mediated allergy (most common allergy)
10% population. 
- Asthma
- Hay Fever
- Eczema
- Foods
- Urticaria and angioedema 
- Anaphylaxis
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4
Q

Attacks synovial joints

A

Rheumatoid Arthritis, often progresses to synovial joint destruction. Also targets tendon sheathes and bursae

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5
Q

Anti-bodies to double stranded DNA?

A

Highly Specific for SLE

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6
Q

Stiffness WORSE in the Morning

A

Rheumatoid Arthritis

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7
Q

Treatment or hyperacute rejection?

A

Organ Removal

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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)

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9
Q

Role of Activated Macrophages in transplant rejection (HVGD)?

A

Injure graft cells and vasculature.

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10
Q

25% of those with this go blind?

A

Giant Cell Arthritis

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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.

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12
Q

Erosions

A

Sign of Joint Destruction in Rheumatoid Arthritis

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13
Q

Steroid Dosing

A

High (20-40mg p/day)
Medium
Low (250mg given every 24-48 hrs

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14
Q

Classification of Transplant Rejection?

A

Hyperacute
(Accelerated Acute)
Acute
Chronic

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15
Q

Arthropathy in sacro-iliac and apophysial (facet joints)

A

Ankylosing spondylitis

Ddx
HLA-B27+ Enteropathic Arthritis

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16
Q

Boutoniere deformities (V at interphalageal joint)

A

Sign of Joint Destruction in Rheumatoid Arthritis

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17
Q

Scl-70

A

A poor prognostic indicator for Scleroderma.

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18
Q

Artritis associated with
Abdo pain and melaena
Renal Disease (renal artery affected)
Rash

A

Polyarteritis Nodosa

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19
Q

Obliterative intimal fibrosis
Glomeruli
Coronary arteries

A

Vascular damage associated with Chronic Rejection

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20
Q

Tx in Giant Cell Arthritis affecting eye

A

Corticosteroids can reduce initial inflammation.

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21
Q

Autoimmune reaction resulting in rejection of graft?

A 
Humeral Rejection
Type II (Autoantibody)  immune response.
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22
Q

Cystic Changes in Bone and Synovium

A

Osteoarthritis

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23
Q

Arthritis of elderly with heavy male predominance.

A

Gout

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24
Q

Antibody deficiencies

A 
XLA
CVID
Hyper-IgM
IgA
Specific antibody deficiency
Transient hypogammaglobulinaemia
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25
Q

Z-deformities (thumb)

A

Sign of Joint Destruction in Rheumatoid Arthritis

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26
Q

Inflammation & organ dysfunction due to Immune Complex deposition in the vasculature of affected organs

A

SLE

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27
Q

“Pencil in cup”

A

Erosive but central not peripheral

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28
Q

Arthritis found in 10-15% of IBD patients

A

Enteropathic Arthritis

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29
Q

X-linked (young men) Typically presents as a skin infection with +ve organisms (staph aureus, fungi, tc) with deep abscess formation

A

Chronic Granulomatous Disease

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30
Q

Type of rejection, common (10-30%) in Kidney transplants?

A

Acute Rejection

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31
Q

Fibrinoid necrosis of vessels and Cortical Infarction in transplant rejection?

A

Hyperacute Rejection

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32
Q

6wks affecting fewer than 4, mostly larger joints, ANA positive?

A

Oligoarthritis (Juvenile Idiopathic Arthritis Subtype)

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33
Q

Uurate overproduction due to:
Diet
Enzyme defects (known and unknown)
Decreased excretion

A

Primary Gout (90%)

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34
Q

Rheumatoid Nodules

A

Rheumatoid Arthritis

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35
Q

Polyconal Immunoglobulin

A

Treatment in humoral acute rejection.
On CD4 staining.
Also used = Polygonal Immunoglobulin and Anti-CD20

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36
Q

Non-complement fixing rejection

A

Accelerated Acute Rejection

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37
Q

Syndrome common in Turkey, Iran and Japan?

A

Behcet’s Syndrome

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38
Q

Subluxations

A

Sign of Joint Destruction in Rheumatoid Arthritis

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39
Q

Arthropathy associated with Haemachromatosis?

A

Pseudogout

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40
Q

Ability of a test to indicate a negative result in the absence
of disease.

A

Specificity

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41
Q

Joint Effusions

A

Sign of Joint Destruction in Rheumatoid Arthritis

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42
Q

Role of Cytotoxic T-cells in transplant rejection (HVGD)

A

Kill cells in the grafted tissue, causing parenchymal and, perhaps more importantly, endothelial cell death (resulting in thrombosis and graft ischemia).

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43
Q

Negatitive birefringment

A

Gout

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44
Q

Gouty Tophi

A

Chronic Arthritis Gout

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45
Q

Tests in Septic Arthritis?

A

Aspirate
Stain
Culture

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46
Q

Paraprotein in blood and/or urine

A

Multiple Myeloma

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47
Q

Rejection characterised by loss of function w/o fever?

A

Acute Rejection.

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48
Q

Lip Biopsy in Sjogrens

A

Periductal and perivascular CD4+ TH cells, B cells, plasma cells , fibrosis

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49
Q

Arthritis usually sparing hips and rest of spine?

A

Rheumatoid Arthritis

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50
Q

HLA and ABO compatibility min requirement?

A

Pancreas Transplant

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51
Q

Butterfly rash

A

Systemic Lupus Erythematous

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52
Q

Patient in upper limbs may have a weak or absent pulse

A

Takayasu Arteritis

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53
Q

Absence of T cells (

A

Severe Combined Immunodeficiency (SCID)

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54
Q

Rejection marked by necrotizing vasculitis with thrombosis (when less acute mimics arterioscleotic thickening)

A

Humeral Acute Rejection (Antibody medicated)

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55
Q

Tx for Polymyalgia rheumatica?

A

Responsive to steroids

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56
Q

CD11a, 11b, 11c and CD18

A

Adhesion molecules (Neutrophil Function Disorder)

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57
Q

Histological signs of Polyartritis Nodosa?

A

Fibrinoid Necrosis

Multiple Thrombosis

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58
Q

ABO Compatibility Essential

HLA as possible

A

Bone Marrow Transplant

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59
Q

Indications for Glucocorticoids

A

Significant Acute Inflammation

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60
Q

Types of Chronic GVHD?

A

Progressive (worst Px)
Interrupted
De novo (best Px)

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61
Q

Rejection Antigens?

A

MHC (Minor HC antigens account for GVHD in Human Leucocyte Antigen - identical Bone Marrow Transplant)
ABO
Others

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62
Q

Small Vesses Vasculitis?

A

Microscopic Polyangitis
Wegner’s Granulomatosis
Churg-Straus
Henoch-Schonlein Purpura

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63
Q

Investigation for Anaphylaxis?

A 
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

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64
Q

ELISA meaning?

A

Enzyme Linked ImmunoSorbant Assay

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65
Q

Chondrocyte Proliferation?

A

Osteoarthritis

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66
Q

Arthritis affecting articular cartilage, with a periarticular bone response

A

Osteoarthritis

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67
Q

HLAB4 is a haplotype of this arthritis.

A

Rheumatoid Arthritis

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68
Q 
Anti-nuclear antibody (ANA)
Anti-dsDNA antibody
ENA antibodies
Anti-RBC antibody
Anti-cardiolipin & lupus anticoagulant
A

All auto-antibodies of Systemic Lupus Erythematous

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69
Q

DR4 and DR1

A

Rheumatoid Arthritis

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70
Q

ANA patterns

A

Homogenous
Speckled
Centromere
Nucleolar

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71
Q

Autoimmune form of arthritis

A

Rheumatoid Arthritis

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72
Q

Ulnar deviation (fingers towards little finger)

A

Sign of Joint Destruction in Rheumatoid Arthritis

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73
Q

Detection of Anti-Nuclear Antibodies?

A

Indirect Immunofluoresence

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74
Q

Aldosterone

A

Main Natural Glucocorticoid

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75
Q

Recurrent infections – RTIs (lungs & ears chronic OM)

Meningitis and septic arthritis

A

Agammaglobinaemia.

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76
Q

T-Cell Mediated Rejection?

A

Cellular Rejection (as opposed to humeral)

Cytotoxic T-Cells
\+
Cytokine Sectreting CD4+ T-Cells
\+
Activated Microphages.
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77
Q

Vasculitis

A

Rheumatoid Arthritis

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78
Q

MPO-ANCA

A

Anti-myeloperoxidase ANCA

=Microscopic PolyAngiitis
&
=Churg-Strauss Δ
- necrotising vasculitis
- asthma
- allergic rhinitis
- pulmonary infiltrates
- increased circulating eosinophils
- necrotizing granulomas (extrvascular)
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79
Q

No HLA, but ABO compatibility required?

A

Cardiac and Liver transplant

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80
Q

Process which has reduced the incidence of hyperacute transplant rejection?

A

Cross Matching

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81
Q

Type 2 Allergy

A

Autoantibody

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82
Q

RNP

A

100% Mixed Connective Tissue Disease

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83
Q

Monitoring of query “Hyperacute rejection’?

A

U/S Doppler ofr BF or Duplex

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84
Q

Squamous cell carinoma a side effect of which type of drug?

A

Immunosuppressants

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85
Q

Type of arthritis that increase in prevalence with increase in latitute?

A

Rheumatoid Arthritis

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86
Q

SLE affects who?

A

Females x9 more
20-30’s
60% Concordance in Twins.

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87
Q

Low Risk CMV group in BMT

A

CMV- Recipient, CMV- Donor

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88
Q

Sequence for BMT Transplantation

A
  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-)
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89
Q

Difference between direct and indirect cellular rejection?

A

Own (indirect) versus donor (direct) antigen presenting cells (APC’s) which are presenting the MHC (always from donor).

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90
Q

Caplans Syndrome

A

Rheumatoid arthritis (RA) and pneumoconiosis combination.

Manifests as intrapulmonary nodules, which appear homogenous and well-defined on chest X-Ray

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91
Q

ALG (Anti-lymphocyte globulin)

A

ALG is used as a treatment for vascular acute transplant rejection. Along with ATG

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92
Q
  • Upper airways (necrotising granulomas)
  • Necrotising granulomatous vasculitis (small-medium vessels)
  • Necrotising glomerulonephritis
A

Wegners Granulomatosis

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93
Q

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

A

Behcet’s Syndrome

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94
Q

Chrystal Arthritides

A

Pseudogout

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95
Q

ATG (Anti-Thymocyte Globulin)

A

Anti-thymocyte globulin is used in the treatment of vascular acute rejection. Along with ALG.

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96
Q

Podgara

A

Acute Arthritis Gout

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97
Q

Test for paraproteins?

A

Electrophoresis

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98
Q

Risk lymphoma 1.16 (MALTOMA)

A

Sjogrens

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99
Q

Asymmetrical arthritis of lower limb

A

Enteropathic Arthritis

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100
Q

Xenograft

A

From animal e.g. Pig heart valves

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101
Q

Pain on movement of joint

A

Rheumatoid Arthritis

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102
Q

HLA-B27

A

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

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103
Q

The Secondary Osteoarthritis.

A

Metabolic – Gout &
Pseudogout (Chondrocalcinosis)
Infectious / Septic Arthrits

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104
Q

Rejection in

A

Acute GVHD

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105
Q

HLA-B8

A

Sjogrens

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106
Q

Treatment for chronic granulomatous disease?

A

Prophylaxis (co-trimoxasole, itraconasole)

BMT

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107
Q

Transplant rejection over 1-5 days

A

Accelerated Acute Rejection

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108
Q

Tightening of skin around the mouth
Portal system of the liver
White out lung disease (acute vascular information)
GI fibrosis and dysmotilty

A

Chronic GVHD

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109
Q

Anti lymphocyte Globulins

A

Immunosuppressant

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110
Q

Arthritis within weeks after shigella, salmonella, yersinia, campylobacter or chlamydia.

A

Reactive Arthritis/Reiter Syndrome

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111
Q

Common causative organisms in Septic Arthritis?

A

Haemophilus influenzaepredominates 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 withSalmonellaat any age.

Gram negative bacilli (elderly),

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112
Q

Kaposi sarcoma a s/e of what drugs? Where else seen?

A

Immunosuppressants/HIV progression to AID’s

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113
Q

Degenerative arthritis

A

Osteoarthritis

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114
Q

Mortality in BMT patients?

A

20-40%

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115
Q

Umbilical Cord Blood

A

Cord blood is collected because it contains stem cells, which can be used to treat hematopoietic and genetic disorders.

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116
Q

Dx of Multiple Myeloma

A

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

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117
Q

> 20% Abnormal looking BM plasma cells

A

Multiple Myeloma

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118
Q

COL2A1 gene

A

Familial Osteoarthritis

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119
Q 
Local factors affecting loading and wear (point loading)
“Secondary Osteoarthritis”
	Injury
	Obesity
	Instability
	Hypermobility
	Joint dysplasia
	Neuropathy
A

Secondary Osteoarthritis

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120
Q

Large cause of mortality in gout?

A

Gouty nephropathy (20% of gout mortality)

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121
Q

IgA immune complexes deposited in mesangium.

A

IgA nephropathy.

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122
Q

Pathogenesis of Gout

A

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.
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123
Q

“telescopic fingers” “whittling”

A

Arthritis mutilans due to osteolysis seen in 5% of Psoriatic Arthritis cases

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124
Q

FcR-bearing cells (T-Cells – primarily Cytotoxic)

A

Accelerated Acute Rejection

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125
Q

BMT graft sources

A

Allogenic (Match sibling or unrelated doner)

Autologous

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126
Q

PR3-ANCA

A 
Anti-proteinase-3 ANCA
= Wegener’s Granulomatosis
- Upper airways (necrotising granulomas)
- Necrotising granulomatous vasculitis (small-medium vessels)
- Necrotising glomerulonephritis
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127
Q

Seronegative destruction of articular cartilage and bony ankylosis

A

Ankylosing spondylitis.

Usually affect sacro-iliac and apophysial joints

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128
Q

Anti-GBM antibody

A

Goodpastures

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129
Q

Suspected mechanism behind chronic rejection?

A

Recipient cells present MHC from the recipient organs

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130
Q

Lenght of post BMT neutropenia?

A

Should lift be 21 days

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131
Q

Should be excluded first in Dx of pseudogout?

A

Septic Arthritis

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132
Q

Iritis and conjunctivitis

A

Psoriatic Arthritis

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133
Q

RF+

A

Polyarthritis (Juvenile Idiopathic Arthritis Subtype)
Ddx
10% of Psoriatic Arthritis

Sjogrens (75%)

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134
Q

HLA-DR3

A

Sjogrens

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135
Q

Arthritis that peaks age 40, but can become symp in 40’s

A

Rheumatoid Arthritis

136
Q

Time to B-Cells post BMT?

A

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
Q

Treatment for Rheumatoid arthritis?

A

Total Joint Replacement

Joint Fusion.

138
Q

Time to T-Cells post BMT?

A

Depends on T-Cell
1-24 for most.
Some never return.
Depends too on immunosuppression

139
Q

Investigations in Sjogrens?

A

Lip biopsy

140
Q

Intraarticular sodium urate chrystals

A

Gout (w/hyperuricaemia)

141
Q

Anti-CCP

A

Rheumatoid Arthritis

142
Q

Bone destruction with osteoporosis or lytic lesions

A

Often seen in Multiple Myeloma

143
Q

Anti-PR3+

A

Granulomatosis with Polyangitis (GPA)

144
Q

Cortisol

A

Main Natural Glucocorticoid

145
Q

Anti-ACHr (receptor)

A

Myasthenia Gravis

146
Q

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

A

Hereditary Angioedema.

147
Q

Routes to septic arthritis?

A

Direct inoculation through injury
Direct spread from adjacent infection
Haematogenous spread

148
Q

Treatment for Acute GVHD?

A

Aggressive and early

Steroids + Anti-T-Cell agents + Anti-TNFalpha

149
Q

Ability of a test to indicate a positive result in the presence
of disease.

A

Sensitivity

150
Q

Anti-CD20

A

Treatment used in humoral acute rejection.
On CD4 staining.
Also seen = Polyconal Immunoglobulin, Plasmapharesis

151
Q

Most common glomerular disease?

A

IgA nephropathy.

152
Q

Erythema of hands and fee (cutaneous) and of eyes and mouth (muco) t, e in children?

A

Query Kawasaki disease.

153
Q

HLA-B51

A

Behcet’s Syndrome

154
Q

Graft manipulation for BMT

A
  1. T-Cell Depletion (less GVHD, Less successful engraftment, Less graft v tumour)
  2. Stem Cell (CD34+) Enrichment
155
Q

Artritis affecting mostly small and medium arteries?

A

Polyarteritis Nodosa

156
Q

Important test pre-intubation

A

Sign of RA on the Neck = X-Ray, Lateral and an AP. Shows narrowing, bony erosions etc.

157
Q

Question mark spine

A

Ankylosing spondylitis

158
Q

Granulomatous thickening of the aortic arch, which primarily affects the brachial artery yeilding a pulsless upper limb

A

Takayasu Arteritis

159
Q

Sex affected more by kawasaki?

A

Male

160
Q

Histological Pathogenesis of Rheumatoid Arthritis.

A
  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
Q

Tx for Aplasia/neoplasias/Herediatary immunodeficineies or metablic abnormalities?

A

Bone Marrow Transplantation

162
Q

IgA EMA

A 
Coeliac (90-100% sensitive and specific)
Dermatitis herpetiformis (50-70% sensitive, 90-100 specific)

False negative – gluten free diet

163
Q

NADPH oxidase (Phox)

A

Chronic Granulomatous Disease

164
Q

Secondary Amyloidosis

A

Protein Misfolding. Seen in…

Rheumatoid Arthritis.

165
Q

Type 4

A

Patch Test (Angry Back Syndrome)

166
Q
  • necrotising vasculitis
  • asthma
  • allergic rhinitis
  • pulmonary infiltrates
  • increased circulating eosinophils
  • necrotizing granulomas (extrvascular)
A

Churg Strauss Syndrome

167
Q

Mass of boggy inflammed synovium that can erode cartilage, known as what? Seen in What?

A

PANNUS

Rheumatoid Arthritis

168
Q

Treatment for acute transplant rejection?

A 
Cellular
	Steroids
Vascular
	ATG, ALG
Humoral
	C4 staining 
	Polyconal immunoglobulin, plasmapharesis, antiCD20
169
Q

Deep, achy pain
Worsens with use
Short-lived morning stiffness

A

Osteoarthritis

170
Q

Anti-La

A

Sjogrens (60-90%)

SLE (10-15%)

171
Q

Schirmer Tear Test

A

Sjogrens

172
Q

Rejection that often relapses?

A

Accelerated Acute Rejection

173
Q

Autologous

A

Self Organ Donation (graft etc)

174
Q

Anti-MUSK (muscle specific tyrosine kinase)

A

Found in some anti-AchR negative MG

175
Q

Anti-endothelial and smooth muscle antibodies

A

Kawakaski

176
Q

DTH

A

Trigger DTH (delayed type hypersensitivity), with increased vascular permeability and local accumulation of mononuclear cells (lymphocytes and macrophages).

177
Q

Necrotising Vasculitis?

A

Polyartrirs Nodosa

178
Q

RANKL

A

Rheumatoid Arthritis

179
Q

Joint Space Narrowing

A

Sign of Joint Destruction in Rheumatoid Arthritis

180
Q

P-ANCA, purpura, lungs, kidneys, cvs, git, necrotizing glomerular nephritis, exogenous antigens from drugs , blood and infections.

A

Microscopic Polyangitis

181
Q

Arthritis, more common in females than males

A

Rheumatoid Arthritis

182
Q

Immune Complexes

A

Rheumatoid Arthritis

183
Q

Young men

A

Buerger’s Disease(Thromboangitis Obliteran)

184
Q

Triad:
Renal Disease
Chronic Sinusitis
Pneumonitis

ANCA+

A

Wegner’s Granulomatosis (Tx = Rapid Immunosup)

185
Q

WNT signalling, Prostaglandin pathways

A

Osteoarthritis

186
Q

Rejection between 6-90 days?

A

Acute Rejection

187
Q

Immunocompetent donor T-cells recognize a mismatch with recipient HLA and initiate an immune response against an immunologically vulnerable host

A

Graft Versus Host Disease (GVHD)

188
Q

Rate of GVHD in BMT?

A

30-50%

189
Q

Rejection over months/years

A

Chronic Rejection

190
Q

Seronegative (in 90%) arthritis associated with psoriasis 20-40yo?

A

Psoriatic Arthritis (10% RF+)

191
Q

Definition of transplant rejection?

A

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
Q

Rheumatoid Factor

A

Seen in 80% of Rheumatoid Arthritis
non-specific
Antibodies (usually IgM) against IgG Fc portion
Marker for activity

193
Q

Small vessel IgA & immune complex mediated vasculits often following upper-RTI in young children

Giveaway is lesions on the buttocks and legs

A

Henoch-Schonlein Purpura

194
Q

Association between GCA and polymyalgia rheumatica?

A

50% of patients with Giant Cell Arteritis have symptoms of PMR and up to 20% of PMR patients have biopsy findings of GCA

195
Q 
Bilateral neck, shoulder and low back
pain and stiffness. 
Worse in the morning
Malaise, tiredness, weight loss, depression and fever. 
Elevated ESR and CRP.
A

Polymyalgia Rheumatica

196
Q

TH1

A

Interferon Gamma (y) – Rheumatoid Arthritis

197
Q

TNF

A

Rheumatoid Arthritis

198
Q

Short Activng Glucocorticoid?

A

Prednisolone
Hydrocortisone
Methylprednisolone
Tramcinolone.

199
Q

Low Magnesium

A

Pseudogout

200
Q

Calcium pyrophosphate dihydrate deposition disease

A

Pseudogout

201
Q

Rejection involving complement fixing

A

Hyperacute Rejection

202
Q

Granulomatous arteritis of the aorta and larger vessels with a predilection for the extracranial branches of the carotids

A

Giant Cell Arthritis

203
Q

Long Acting Glucocorticoid?

A

Dexmethasone

204
Q

MEDICATION TO AVOID IN STEROIDS?

A

NSAIDS

205
Q

Aortitis

A

Ankylosing spondylitis

206
Q

Cyclosporine (FK506)

A

Immunosuppressant

207
Q

Complications and Tx of X-linked Agammaglobinaemia?

A

IVIg and antibiotics

Complications…
Bronchiectasis
Chronic Sinus Damage

208
Q

Treatment of SCID?

A

Isolation in +pressure
Antibiotics
Bone Marrow Transplant, possibly gene therapy.

209
Q

Minor histocompatibility antigens
Non-MHC encoded polymorphisms
Cytokine polymorphisms

A

Mechanism of GVHD which avoid “Matching”

Occasionally seen in autologous transplants die t re-emergence of autoreactive t-cells

210
Q

Salivary gland enlargement F>M

A

Sjogrens

211
Q

Serology of Rheumatoid Arthritis

A
  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
Q

TH17

A

Neutrophil recruitment – Rheumatoid Arthritis

213
Q

HLA-DRB1*04

A

Polymyalgia Rheumatica

214
Q

Rate of 5-10y graft survival?

A

(80%)

215
Q

Artritis affecting large, small and medium arteries in children?

A

Kawakaski

216
Q

Syngeneic

A

Monozygotic (identical) siblings

217
Q

Presensitisation?

A

Now Countered by cross-matching
Irradiated to leucocyte deplete

Questionable increase in tolerance

218
Q

Acute pain and swelling in knee, wrist or shoulder

A

Pseudogout

219
Q

Lymphomas as s/e?

A

Immunosuppressants

220
Q

Vaccination post BMT?

A

Follow guidelines

No BMT.

221
Q

HLA-DRB1*01

A

Polymyalgia Rheumatica

222
Q

Cyanotic, mottled and flacid graft on removal of vascular clamps in Operating Room?

A

Hyperacute Rejection

223
Q

Anti-Citrullinated Peptide Antibodies (ACPA) (AKA ACCP)

A

Rheumatoid Arthritis (Specific)

224
Q

Sirolimus

A

TOR (Target of Rapamycin) inhibition.

Thereby blocking IL2

225
Q

Methods for improving graft survival?

A
  1. Better Matching

2. Immunosuppression

226
Q

Tx for Allergens

A

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
Q

Maculopapular rash +

Hepatosplenomegaly

A

Acute GVHD

228
Q

Arthritis affecting young adults?

A

Polyarteritis Nodosa

229
Q

Crepitus

A

Osteoarthritis

230
Q

Age of onset in Multiple Myeloma?

A

After age 50 yrs

231
Q

Subchondral Sclerosis

A

Osteoarthritis

232
Q 
Extensively matched 
ABO (ABO mismatched are possible)
\+
HLA (mostly DR)
\+
X-matched for preformed antibodies
A

Kidney Transplant

233
Q

Indirect cellular rejection

A

Usually chronic rejection

234
Q

Prednisone and Cortisone

A

Biologically inactive synthetic
11 keto group requires activation to the 11-hydroxy group before having effect on cytosolic receptor.

Activated forms = Prednislone, Cortisol.

235
Q

Pneumonitis

A

Rheumatoid Arthritis

236
Q

Treatment for Pseudogout

A

Supportive

237
Q

Arthritis of mostly men 20-40

A

Reactive Arthritis/Reiter Syndrome
Or
Ankylosing Spondylitis

Ddx
Possible Rheumatoid (early)
238
Q

Felty’s Syndrome

A

Combination of heumatoid arthritis, splenomegaly and neutropenia.

239
Q

Lymph and Cardiac Signs of Kawasaki Disease?

A

Lymph = Cervical Lymphadenopathy

Coronary Arteries = Aneurysm and MI

240
Q

Arthritis in PIP’s, 1sr MCP and 1st MTP joints

A

Osteoarthritis

241
Q

Similar in appearance to systemic scleosis and Sjogrens?

A

Chronic GVHD

242
Q

Loss of cartilage with subchondral bony change

A

Osteoarthritis

243
Q

Monoclonal Antibodies (e.g. Monoclonal Anti-CD3)

A

Immunosuppressant

244
Q

Agents used in Transplant Immunosuppression

A

Glucocorticoids
Calcineurin Inhibitors (Cyclosporine, Tacrolimus)
Antiproliferative/antimetabolic agents
Monclonal Antibody Therapies (cell specific)

245
Q

Seronegative arthritis more common in males, average more common in mid-20’s

A

Ankylosing spondylitis

246
Q

Small non-weight bearing joints (Hands and Feet, C-Spine)

A

Rheumatoid Arthritis

247
Q

btk

A

Brutons Tyrosine Kinase
Vital in maturation of Pro-BCell to Pre-Bcell
Defect = agammaglobulinaemia = IgA, IgG and IgM def

248
Q

ACR/EULAR 2010 Criteria

A 
Rheumatoid Arthritis
Points for:
	Joint Involvement
	Serology
	Acute phase reactants
	Duration of symptoms
249
Q

Pathogenesis an Tx targets of Acute Rejection?

A

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
Q

Increase PTH

A

Pseudogout

251
Q

ANA reactivity clarification?

A

ELISA, RIA and Immunoblotting

252
Q

ESR adjusted for age and CRP if this condition is suspected.

A

Giant Cell Arthritis

253
Q

Hyperuricaemia

A

Gout (w/intraarticular sodium urate chrytals)

10% have hyperuricaemia, only 0.5% have gout

254
Q

Gold Standard in Allergen Dx

A

Allergen Challange

255
Q

Arthritis with temporal relationship to outbreaks of…

Parainfluenza
Parvovirus B19
Mycoplasma
Chlamydia

A

Polymyalgia Rheumatica

256
Q

Overproduction of Urate

  • Increased cell turnover
  • Inborn errors of metabolism

Reduced excretion
-Chronic renal failure

A

Secondary Gout (10%)

257
Q

ANA

A

Oligoarthritis (Juvenile Idiopathic Arthritis Subtype)

258
Q

Cartilage Matric Metalloproteases

A

Rheumatoid Arthritis

259
Q

Joint disorder common in 6wks

A

Juvenile Idiopathic Arthritis

260
Q

Cell Cycle Inhibitors

A

Mycophenolate Mofetil
Inhibits Inosine, needed for nucleotide synthesis
Used in SLE

Azathioprine
Metabolised to 6MP in the liver.

261
Q

Pulseless Syndrome

A

Takayasu Arteritis

262
Q

Joint pain in 5yo

A 
Systemic Onset JIA. Also...
Fever
Rash (salmon-pink)
Lymphadenopathy
Organomegaly
Serositis / Hepatitis
263
Q

Better HLA matching improves Graft survival?

A

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
Q

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.

A

Psoriatic Arthritis

265
Q

Arthropathy associated with Alkaptonuria disease?

A

Pseudogout

266
Q

Common site to observe synovial joint destruction

A

Metacarpal/interphalangeal joints

267
Q

High Risk CMV group in BMT

A

CMV+ Recipient

w/wo CMV+ Donor

268
Q

Transplants associated with GVHD?

A

BMT (30-50%)
Blood Transfusion
Solid Organs, commonly LIVER (immune organ CD4+8 cells, transplanted a lot) (others =occasionally)

269
Q

Sjograns w/o RA?

A

Primary Sjogrens

270
Q

Treatment for Accelerated Acute Rejection?

A

Immosuppression, but only partially effective.

Transplant removal often required.

271
Q

Calcification of fibro and hyaline cartilage

A

Pseudogout

272
Q

Sicca’s (Sjogrens) Syndrome

A

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
Q

Infections soon after birth

  • Recurrent bacterial
  • Persistent Viral
  • Opportunistic
  • Persistent Thrush
A

Severe Combined Immunodeficiency (SCID)

274
Q

Swan neck deformities

A

Sign of Joint Destruction in Rheumatoid Arthritis

275
Q

Dx for SLE?

A

Clinical Hx & examination
Urinalysis & urine microscopy
Assess function of potentially involved organs.

Autoantibodies (Indirect immunofluoresence)

DIFF= Skin /Renal

276
Q

Haematological features of SLE?

A

Immune thrombocytopaenia
Haemolytic anaemia
Autoimmune neutropaenia

277
Q

Stages of osteoarthritis?

A
  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
Q

Rejection involving preformed antibodies? Name the antibody types?

A

MHC 1 or ABO, mothers, previous transfusions and transplants)

279
Q

Stills Disease

A

Systemic Onset JIA

280
Q

Important consideration in IgE allergies

A

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
Q

HLA-DRB1

A

Rheumatoid Arthritis.

282
Q

Volar subluxation (enlargement at knuckles caused by tendon slip)

A

Sign of Joint Destruction in Rheumatoid Arthritis

283
Q

Citrulline

A

Rheumatoid Arthritis

284
Q

Arthralgia and arthritis with skin and haematological abnormalities?

A

Systemic Lupus Erythematous

285
Q

Symmetrical arthritis

A

Rheumatoid Arthritis

286
Q

Less severe symptoms that reiter but nonetheless following infection?

A

Enteropathic Arthritis

287
Q

Relatively refractory with progressive loss of graft function

A

Chronic Rejection

288
Q

Subchondral Cysts

A

Osteoarthritis

289
Q

Juxta-articular osteopaenia

A

Sign of Joint Destruction in Rheumatoid Arthritis

290
Q

Jo-1

A

30% of Polymyosistitis

291
Q

Time to NK cells post BMT?

A

1 month

292
Q

Treatment for Granulomatosis with Polyangitis?

A

Cyclophosphamide

293
Q

X-Chromosome (males) defect in B-Cell Maturation. Presents in early childhood.

A

XLA/Brutons Tyrosine Kinase Defect/X-linked agammaglobulinaemia

294
Q

Anti-Sm (30%)
Anti-Ro (30%)
Anti-La

A

30% specific (except La) each for SLE

All are anti-ENA (extractable nuclear antigen).

295
Q

Commonest primary systemic vasculitis

A

Giant Cell Arthritis

296
Q

MHC

A

Major Histocompatability Complex
Mediated Graft Versus Host disease in HLV identical BMT

Seen in Hyperacute rejection (MHC1)

297
Q

Treatment for hereditary angioedema

A

Treatment
C1-Inh replacement (acute attacks)
Modified androgens (danazol or stanazol)
Bradykinin receptor anatagonists

298
Q

Decreased range of movement

A

Osteoarthritis

299
Q

HLA-CW6

A

Psoriatic Arthritis

300
Q

Why are chronic rejections the most common?

A

Immunosuppresents so good it drags rejection out

301
Q

Virus associated with Polyartritis Nodosa?

A

Hepatitis B

302
Q

Arthritis in Lumbar and Cervical Spine

A

Osteoarthritis

303
Q

Arthritis associated with old age?

A

Osteoarthritis

304
Q

Immune complex mediated disease affecting any organ with diverse manifestations and variable prognosis?

A

Systemic Lupus Erythematous

305
Q

Plamaphaesis

A

Treatment in humoral acute rejection. On CD4 staining. Also used = Plasmapharesis and Anti-CD20

306
Q

The Seronegative immune mediated joint disorders

A

Ankylosing spondylitis
Reactive arthritis/Reiter syndrome
Enteropathic arthritis
Psoriatic arthritis

307
Q

Serosistitis

A

Inflammation of the serosa. Seen in…

Rheumatoid Arthritis

308
Q

Graft Rejection Pathophysiology

A

APC-TCR = CD4 proliferation, release of IL2 and recruitment of Cytotoxic CD8+Tcells

309
Q 
Arthritis (sausage finger, bony spurs)
\+
Nongonococcal urethritis/cervicitis (+/- circinate balanitis)
\+
Conjunctivitis
A

Reactive Arthritis/Reiter Syndrome
Also,
+/- oral ulceration, heart block, aortic regurgition

50% is chronic and resembles Ankylosing Spondylitis.

310
Q

Varients of SLE limited only to the skin?

A

Discoid lupus erythematosus

Subacute cutaneous lupus

311
Q

Principle Antibodies in Rheumatoid Arthritis

A 
Anti CCP (Specific 97%, sensitive 88%)
RF (Specific 65%, Sensitive 50-90%)
312
Q

Anti-IL2-Receptor Antibodies.

A

Daclizumab and Basiliximab

Prophylaxis against organ rejection.

313
Q

Signs and symptoms of PANNUS/Rheumatoid Arth?

A

(4 basic signs of inflammation)

  1. Calor
  2. Rubor (
  3. Tumour (swelling)
  4. Dolar (pain)
314
Q

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

A

Churg-Straus Syndrome (Tx = Glucocortiocids +Support)

315
Q

Commonest malignant disorder of plasma cells

A

Multiple Myeloma

316
Q

Calcineurin Inhibitors

A

Cyclosporine, Tacrolimus (100x more potent).
Intracell signalling pathways to block IL2.
Mainstay of organ transplant.

S/E’s Nephrotoxicity, Neurotixcity.

317
Q

Weakly positively birefringent rhomboid crystals on polarisation.
May be cloudy and associated with fever and ↑ peripheral WCC

A

Result from joint aspirate

=Pseudogout

318
Q

BMT graft types

A

Bone Marrow
Peripheral Stem Cells (mat. T-cells)
Cord Stem Cells

319
Q

Skip Lesions

Often associated with polymyalgia rheumatica

A

Giant Cell Arthritis

320
Q

6wks affecting more than 4, mostly smaller joints, RF+ positive? With poorer prognosis

A

Polyarthritis (Juvenile Idiopathic Arthritis Subtype)

321
Q

Allogeneic

A

Living or Cadaveric (Another genetically different person donor)

322
Q

Arthropathy associated with Wilsons disease?

A

Pseudogout

323
Q

Rate of 10-20y graft survival?

A

(50%)

324
Q

Rejection marked by an interstitial mononuclear cell infiltrate with associated edema and parenchymal injury,

A

Cellular Acute Rejection

325
Q

Graft rejection in less

A

Hyperacute Rejection

326
Q

IgM, IgG, IgA normalisation post BMT?

A

6-24 months

327
Q

Hot, Red, one joint?

A

Septic Arthritis

328
Q

Mechanical degradation of joints

A

Osteoarthritis

329
Q 
Interstitial fibrosis
Tubular atrophy
Vanishing bile duct syndrome
Bronchiolitis obliterans
Gross scarring and shrinkage
A

Chronic Rejection

330
Q

Anti-Ro

A

Sjogrens (90%) also RF (75%)

331
Q

Arteritis far more common in the elderly (>50) and affect F>M, 2:1?

A

Giant Cell Arthritis

332
Q

Low Phosphate

A

Pseudogout

333
Q

Schober’s test (limited flexion (

A

Ankylosing spondylitis

334
Q

Older person

Unilateral Headache, facial pain and scalp tenderness.

A

Giant Cell Arthritis

335
Q

Uveitis

A

Ankylosing spondylitis

336
Q

Rejection, can be humeral or cell mediated, but is more commonly cell mediated?

A

Accelerated Acute Rejection

337
Q

Role of Cytokine Secreting CD4+ T-Cells in transplant rejection (HVGD)?

A

Trigger DTH (delayed type hypersensitivity), with increased vascular permeability and local accumulation of mononuclear cells (lymphocytes and macrophages).

immunopath (3 decks)

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