Histopathology Flashcards
1
Q
Types of non-traumatic haemorrhage? (2)
A
- Intraparenchymal Haemorrhage - into the substance of the brain
- Subarachnoid Haemorrhage
2
Q
Intraparenchymal Haemorrhage
a) Causes (5)
b) most common site
c) Symptoms (4)
A
a) - HTN (50%)
- atherosclerosis
- arteriovenous malformations
- Amyloid angiopathy
- CADASIL
b) basal ganglia
c) Raised ICP - headache
- focal neurology
- vomiting
- reduced GCS
3
Q
What is CADASIL?
Associations? (4)
A
- Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy
Associations - progressive dementia
- mood disorders
- migraine
- recurrent subcortical cerebral infarctions
4
Q
Arteriovenous Malformation
a) where
b) who
c) presentation
d) high/low pressure?
e) treatment
A
a) anywhere in the CNS
b) 2nd - 5th decades
c) - haemorrhage
- seizures
- headache
- focal neurological deficits
d) high pressure - MASSIVE bleed
e) surgery - arteriovenous shunting
5
Q
Cavernous Angioma - WHAT?
a) where
b) who
c) presentation
d) high/low pressure?
e) treatment
A
“Well-defined malformative lesion composed of closely packed vessels with no parenchyma interposed between vascular spaces”
a) anywhere in the CNS
b) >50 years familial association
c) headache, seizures, focal deficits, haemorrhage
d) low pressure - recurrent bleeds
e) surgery
6
Q
Subarachnoid Haemorrhage
a) Cause
b) who - sex, age
c) most common site
d) Symptoms (3)
e) associations (4)
A
a) ruptured Berry aneurysm (85%)
b) F > M
c) internal carotid bifurcation
d) 1. thunderclap headache
2. vomiting
3. LOC
e) 1. PKD
2. Ehlers Danlos
3. Aortic coarctation
4. vascular malformations
7
Q
Types of traumatic brain haemorrhage? (2)
A
- extra dural
2. sub dural
8
Q
Extradural Haemorrhage
a) history
b) pathology
c) symptoms (2)
d) intervention
A
a) traumatic injury to side of head
b) skull fracture -> ruptured middle meningeal artery
c) 1. lucid interval
2. LOC
d) surgical evacuation
9
Q
Subdural Haemorrhage
a) history
b) pathology
c) associations (2)
d) symptoms
A
a) previous minor trauma
b) damaged bridging veins -> slow venous bleed -> brain atrophy
c) 1. elderly
2. alcoholism
d) fluctuating level of conciousness
10
Q
Concussion?
A
transient LOC and payalysis
recovery in hours or days
11
Q
Diffuse axonal injury
A
vegetative state
post-traumatic dementia
12
Q
Meningism (4)
A
- stiff neck - Brudzinski’s sign (flexion of knees when chin on chest)
- photophobia
- headache
- positive Kernig’s sign (inability to extend knee when hip is flexed)
13
Q
Myelitis
- what
- causative agent
A
- disturbance of the nerve transmission of the spinal cord
- Poliovirus
14
Q
Encephalitis
a) what
b) causative agents (3)
c) symptoms (5)
A
a) inflammation of the brain parenchyma
b) -HSV 1
- Rabies
- arboviruses
c) drowsiness
seizures
behavioural changes
headache
fever
15
Q
Meningitis
A
inflammatory process in the CSF and meninges
16
Q
Pathogenesis of neurological deficit in meningitis? (5)
A
- direct bacterial toxicity
- indirect inflammatory process, cytokine release, oedema
- shock
- seizures
- hypoperfusion
17
Q
Common causative agents of bacterial meningitis in NEONATES? (3)
A
- Group B Strep
- E Coli
- Listeria (gram +)
18
Q
Common causative agents of bacterial meningitis in 1 month - 6 years? (2)
A
- strep pneumoniae (gram +)
2. haemophilus influenza
19
Q
Common causative agents of bacterial meningitis in adolescents? (2)
A
- Neisseria meningitisis (gram -)
2. Strpe pneumonniae (gram +)
20
Q
Common causative agents of bacterial meningitis in ELDERLY? (2)
A
- Strep pneumoniae
2. E Coli
21
Q
Meningococcal septicemia?
a) causative agent?
b) signs (3)
A
a) N.meningitidis (gram -)
b) - non-blanching, petichial rash (80%)
- maculopapular rash (13%)
- no rash (7%)
22
Q
Bacterial meningitis LP
a) appearance
b) predominant cell
c) glucose
d) protein
A
a) turbid/cloudy
b) neutrophils
c) low
d) high
23
Q
TB meningitis LP
a) appearance
b) predominant cell
c) glucose
d) protein
A
a) fibrin web
b) lymphocytes
c) low
d) high
24
Q
Viral meningitis LP
a) appearance
b) predominant cell
c) glucose
d) protein
A
a) clear
b) lymphocytes
c) normal
d) normal
25
Tuberculosis meningitis
a) acute or chronic?
b) who?
c) diagnostic test
d) associations (3)
a) chronic
b) immunosuppressed patients
c) Ziehl-neelson stain LP
d) - granulomas
- abscesses
- cerebritis
26
Common causative agents of viral meningitis in NEONATES? (3)
1. Coxsackie's
2. Mumps
3. HIV
27
Common causative agents of viral meningitis in children? (4)
1. measles
2. mumps
3. varicella-zoster
4. EBV
28
Increased risk of spinal infections? (7)
1. increased age
2. IVDU
3. DM
4. long-use systemic steroids
5. organ transplantation
6. malnutrition
7. malignancy
29
Commonest form of adult brain tumours?
metastatic lesions
30
Common primaries with CNS metastases? (3)
1. Breast
2. Lung
3. Malignant melanoma
31
Commonest group of primary CNS tumours?
astrocytomas
32
Neurofibromatosis type II
a) tumour
b) associations (3)
a) meningioma
b) 1. cafe-au-lait macules
2. Lisch nodules (iris)
3. freckling
33
Ependymoma
a) what
b) causes
a) ventricular tumour
| b) hydrocephalus
34
Pilocytic astrocytoma
a) who
b) where
c) characteristic appearance
a) children
b) cerebellum
c) piloid "hairy" cell
35
Oligodendroma
a) who
b) appearance (3)
a) adults
| b) soft, gelatinous, calcified
36
Dementia
global impairment of cognitive function and personality without impairment of conciousness. This impairment goes beyond what might be expected for normal ageing. Includes memory impairment and at least one of the following cognitive disturbances - aphasia, agnosia, apraxia, or a disturbance in executive functioning.
37
Aphasia
language disorder - expressive or repetitive e.g. finding words
38
Agnosia
loss of ability to recognise an object/people
39
Apraxia
inability to carry out a learned task
40
Pathological misfolded proteins associated with AD (2)
1. neurofibrillary Tau tangles
| 2. B-amyloid
41
MRI changes in AD (4)
1. generalised cerebral atrophy
2. widened sulci
3. narrowed gyri
4. enlarged ventricles
42
Symptomatic medications used in AD (3)
1. anti-cholinesterases
2. nACR agonists
3. glutamate anatgonists
43
Symptoms associated with Lewy Body dementia (4)
1. day-to-day fluctuations
2. visual hallucinations
3. Parkinsonism
4. recurrent falls
44
Triad of PD
1. bradykinesia
2. rigidity
3. dyskinesia
45
Pathology of PD
loss of dopaminergic neurons of the substantia nigra
46
Protein associated with familial PD?
a-synuclein
47
Multiple System Atrophy
a) what
b) presentation
c) areas affected (3)
a) degenerative neurological disorder
b) Parkinsonism with poor response to Levodopa
c) 1. Shy Drager - autonomic dysfunction
2. Striatonigral - difficulty with movement
3. olivopontocerebellar - difficulty with balance & coordination
48
Pick's Disease
a) what
b) inheritance pattern
c) protein involved
d) symptoms
a) frontotemporal atrophy
b) autosomal dominant
c) Tau
d) behavioral changes, inhibition, apathy
49
Multiple Sclerosis
a) what
b) who
c) presentation (2)
d) classification (2)
e) pathology
f) proteins (2)
a) autoimmune demyelinating disease
b) F>M, 20-40 years
c) focal neurology 1. optic neuritis
2. poor coordination
d) 1. relapsing & remitting
2. primary progressive
e) plaques with myelin loss
f) 1. Myelin Basic Protein
2. Proteo-lipid Protein
50
Fracture repair stages (4)
1. Pro-callus - organisation of haematoma
2. formation of fibrocartilaginous callus
3. mineralisation of fibrocartilaginous callus
4. remodelling of bone along weight-bearing lines
51
Factors affecting fracture healing (6)
1. fracture type
2. neoplasm
3. metabolic disorder
4. vitamin deficiency
5. drugs
6. infection
52
Common site of osteomyelitis in adults (3)
1. vertebrae
2. jaw (2o to dental abscess)
3. toe (2o to DM ulcer)
53
Common bacteria causing osteomyetlitis?
a) adults (3)
b) children (2)
c) rare (2)
a) 1. Staph aureus
2. Klebsiella
3. E.Coli
b) 1. H influenzae
2. GBS
c) 1. TB
2. syphilis
54
Normal calcium range
2.2-2.6mmol/L
55
DEXA T score in Osteopenia
1 - 2.5 SD below normal
56
DEXA T Score in Osteoporosis
> 2.5 SD below normal
57
Blood results in Osteoporosis
Norm Ca
Norm Phosphate
Norm ALP
58
Blood results in Osteomalacia
Low Ca
Low Phosphate
High ALP
59
Features of Osteomalacia in an ADULT (2)
1. Bony pain
| 2. Proximal muscle weakness
60
Features of Osteomalacia in a CHILD (6)
1. bony pain
2. bowing tibia
3. rachitic rosary
61
What is Paget's disease?
disorder of bone turnover
62
Features of Paget's disease (2)
1. lysis
| 2. sclerosis
63
Risk factors Paget's (3)
1. male
2. >50 years
3. caucasian
64
x-ray findings in osteomalacia?
Looser's zones
65
Blood results in Paget's disease?
Norm Ca
Norm Phosphate
HIGH ALP
66
X-ray features found in Paget's disease (6)
1. cotton wool skull
2. osteoporosis circumstripta
3, picture frame vertebra
4. ivory vertebra
5. lucency of pelvis
6. sclerosis of pelvis
67
Histological features of Paget's (2)
1. huge osteoclasts
| 2. mosaic pattern of lamellar bone
68
Blood results in hyperparathyroidism
HIGH Ca
LOW phosphate
Norm ALP
69
Causes of primary hyperparathyroidism (2)
1. parathyroid adenoma
| 2. chief cell hyperplasia
70
Causes of secondary hyperparathyroidism (3)
1. chronic renal failure
2. vit d deficiency
3. malabsorption
71
X-Ray features in hyperparathyroidism (3)
1. Brown's tumours
2. salt and pepper skull
3. subperiosteal resorption of phalanges
72
clinical features of hyperparathyroidism (6)
HYPERcalcemia
1. Stones - renal
2. Bones - pain
3. Groans - pancreatitis
4. Moans - depression, confusion
5. polyuria
6. polydipsia
73
histological feature of hyperparathyroidism
osteitis fibrosa cystica (OFC)
74
What is renal osteodystrophy?
ANY skeletal changes associated with CKD
75
Blood results in renal osteodystrophy?
LOW Ca
| HIGH Phosphate
76
Clinical features of renal osteodystrophy? (3)
1. metabolic acidosis
2. 2 hyperparathyroidism
3. alluminium deposition
77
Factors affecting fracture healing (6)
1. fracture type
2. neoplasm
3. metabolic disorder
4. drugs
5. vitamin deficiency
6. infection
78
What is pseuodarthritis?
non-healed fracture "false joint"
79
Symptoms of osteomyelitis? (6)
1. pain
2. oedema
3. malaise
4. fever
5. rigors
6. leukocytosis
80
X-ray changes in osteomyelitis
- when
- what
- 10 days post onset
| - lytic destruction of bone
81
Rare causes of osteomyeltitis (2)
1. TB
| 2. syphilis
82
Lyme's Disease
Inflammatory arthropathy as part of a multisystem illness resulting from a tickbite
83
What skin rash is associated with Lyme's disease?
erythema chronicum migrans
84
Where are Heberden's nodes?
DIPJ
85
Where are Bouchard's nodes?
PIPJ
86
Most common sites of OA?
1. Vertebrae
2. hips
3. knees
87
What is RA?
autoimmune, severe, chronic, relapsing synovitis
88
Gene complexes associated with RA?
HLA DR1 & DR4
89
Blood results common with RA? (3)
1. anaemia of chronic disease
2. raised ESR
3. 80% RF +ve
90
Characteristic deformities associated with RA (6)
1. radial deviation of wrist
2. ulnar deviation of fingers
3. Boutonniere deformity
4. Swan neck deformity
5. Z thumbs
6. Rheumatoid nodules
91
Histological findings in RA (4)
1. Thickening of synovial membranes ( villous)
2. Hyperplasia of surface synoviocytes
3. Intense inflammatory cell infiltrate
4. Fibrin deposition and necrosis
92
X-ray findings in RA (4)
1. soft tissue swelling
2. juxta-articular osteopenia
3. decreased joint space
4. bony erosions
93
Pathognonomic of RA?
morning stiffness
94
Crystals found in gout?
needle shaped, negatively birefringent, urate crystals
95
Common joint affected in gout?
great toe "podagra"
96
Gout
a) Short term treatment
b) Long term treatment
c) Conservative
a) Colchicine
b) Allopurinol
c) - lower alocohol intake
- low purine intake
97
Common joints affected in pseudogout? (2)
1. shoulder
| 2. knee
98
Crystals found in pseudogout?
rhomboid shaped, positively birefringent, calcium pyrophosphate crystals
99
Osteosarcoma
1. who
2. where
3. x-ray findings (2)
4. benign/malignant
1. adolescents
2. knee (60%)
3. -Codman's triangle
- sunburst appearance
4. malignant
100
Chondrosarcoma
1. who
2. where
3. x-ray findings (2)
4. benign/malignant
1. >40yrs
2. pelvis
3. - lytic lesions
- fluffy calcification
4. malignant
101
Tumour marker for Ewing's sarcoma?
CD99
| MIC2
102
Translocation in Ewing's sarcoma?
EWS/FLI1 fusion 11/22 translocation
103
Ewing's tumour
- who
- where
- x-ray appearance
- benign/malignant
1. teenagers
2. long bones, pelvis
3. onion skinning
4. malignant
104
Giant cell tumour
- who
- where
- type
- F>M 20-40years
- knee
- osteoclast-type
105
Enchondroma
benign tumour of cartilage
106
Common site of enchondroma? (2)
1. hands
| 2. feet
107
Ollier's syndrome
mutliple enchondromas
108
Marffuci's syndrome (2)
multiple enchondromas + haemangiomas
109
X-ray features of enchondroma's (3)
1. lytic lesion
2. popcorn calcification
3. O ring sign
110
Common site of osteochondroma
Knee (50%)
111
Exostosis
benign outgrowth of cartilaginous tissue on bone
112
Diaphyseal aclasis- (3) features
Hereditary multiple exostoses
1. multiple exostoses
2. short stature
3. bone deformities
113
Fibrous dysplasia
- what
- who
- age
- site
- bone replaced by fibrous tissue
| - female
114
X-ray findings in fibrous dysplasia (2)
1. Soap bubble osteolysis
| 2. Shepherd's crook deformity
115
Albright Syndrome (3)
1. polyostotic dysplasia
2. cafe au lait
3. precocious puberty
116
Osteiod osteoma
- who
- site
- malignant or benign
- adolescence
- tibia/femur
- benign
117
Osteoma
- who
- site
- feature
- middle aged
- head/neck
- bony outgrowths attached to normal bone
118
Gardner's syndrome (3)
1. GI polyps
2. multiple osteomas
3. epidermoid cysts
119
X-ray feature in osteoblastoma
speckled mineralisation
120
Common sites of simple bone cyst (2)
1. humerus
| 2. femur
121
4 layers of epidermis
1. stratum corneum
2. stratum granulosum
3. stratum spinosum
4. stratum basale
122
Hyperkeratosis
increase in keratin in stratum corneum
123
Parakeratosis
nuclei present in stratum corneum
124
Acanthosis
increase in stratum spinosum
125
Acantholysis
decrease in cohesions between keratinocytes
126
Spongiosis
intercellular oedema
127
Lentiginous
linear pattern of melanocyte proliferation within epidermal basal cell layer - reactive or neoplastic
128
Pemphigus Vulgaris
- who
- where (2)
- appearance
- involvement of mucous membranes?
- cause
- middle aged
- flexures, head
- flaccid, painful blisters
- yes
- IgG mediate autoantibodies against antigens in the epidermis (Superficial)
129
Dermatitis herpetiformis
- who
- where (3)
- appearance
- involvement of mucous membranes?
- associated conditions (2)
- young adults
- elbows, knees, buttocks
- vesicles, papules, excoriations
- rare
- gluten-sensitivity, lymphoma
130
Discoid eczema
- who
- where
- what
- M>F
- limbs
- pruitic coin-shaped lesions
131
Atopic dermatitis/eczema
- infants - where
- adults - where
- chronic
- histology
- face, scalp
- flexural areas
- strong atopic FH, lichenification
- spongiosis
132
```
Contact dermatitis
- type of hypersensitivity reaction?
appearance (3)
- where
- common allergens (3)
- histology
```
- type IV
- 1. pruritis
2. erythema
3. oedema
- ear lobes, wrists, neck
- 1. nickel
2. rubber
3. PPD
- spongiosis
133
Yeast involved in seborrhoeic dermatitis?
Malassezia furfur
134
Seborrhoeic dermatitis
- where a) infants
b) adults
- histology (2)
a) cradle cap - yellow scales on scalp
b) eyebrow/eyelid/face/anterior chest/external ear
- 1) acanthosis
2) dilated dermal capillaries
135
Psoriasis
- what
- where (3)
- histology (3)
- associations (2)
- chronic, inflammatory dermatosis with well-demarcated red scaly plaques with silver-scale
- extensor aspect of knees, elbows, scalp
- 1) parakeratosis
2) clubbing of rete's ridges "test tubes in a rack"
3) Munro's microabscesses
- 1) nail changes
2) arthritis
136
Auspitz's sign
rubbing psoriatic plaques causes pin-point bleeding
137
Koebner phenomenon
psoriatic lesions form at sites of mild trauma
138
Flexural psoriasis (4)
groin, axillae, sub-mammary folds, natal cleft
139
Guttate psoriasis
- what
- where
- who
- association
- "rain-drop" plaque distribution
- widespread
- children
- post- Strep throat
140
Erythrodermic psoriasis
- widespread, life-threatening
| - systemis symptoms
141
Pustular psoriais
- who
- where
- sterile deep-seated lesions
- smokers
- palms & soles
142
Nails changes associated with psoriasis (3)
1. pitting
2. onkyiolysis
3. subungual hyperkeratosis
POSH
143
Bullous Pemphigoid
- who
- where (2)
- appearance
- immune mediated
- involvement of mucous membranes?
- cause
- elderly
- trunk, limbs
- tense, fluid-filled blisters
- IgG
- no
- autoantibodies against antigens between dermis and epidermis (Deep)
144
Lichen Planus
- appearance (5 P's)
- where (4)
- histology
```
- Pruritic
Purple
Polygonal
Papules
Plaques
- forearms, legs, palmar wrists, oral mucosa
- saw-toothing of Rete ridges
```
145
Wickam's striae
fine white network of streaks seen on oral mucosa in lichen planus
146
PemphiguS
bullae are Superficial
147
PemphigoiD
bullae are Deep
148
Erythema multiforme
- what
- appearance (2)
- where (2)
- causes (2)
- self-limiting inflammatory condition
- annular target lesions (most common)
pleomorphic: macules, papules, weals, vesicles, bullae, petechiae
- hands, feet
- 1) Infection - Herpes simplex, mycoplasma
2) Drugs - Penicillin, anti-malarials
149
Dermatitis herpetiformis
- association
- appearance
- distribution (3)
- Coeliac disease
- pruritic vesicles
- extensor surfaces of elbows, knees, buttocks
150
Pathophysiology of dermatitis herpetiforms
IgA mediated, bind to BM and cause subepidermal bullae
151
Seborrhoeic keratosis
- benign or malignant?
- appearance
- who
- Benign
- well defined "stuck on" plaques
- middle age/elderly
152
Solar lentigo/Actinic Keratosis
- who (2)
- where (4)
- histology (5) SPAIN
- middle aged, high sun exposure
- face, distal limbs, bald scalp, dorsal hands
S - solar elastosis
P - parakeratosis
A - atypis
I - inflammation
N - not full thickness
153
Keratocanthoma
- appearance (3)
- presentation (2)
- histology
- crusty nodule with necrotic centre
- rapidly growing over 2-3 weeks, clear spontaneously
- similar to SCC
154
Bowen's Disease/SCC in situ
- appearance (3)
- risk factors (2)
- histology (2)
- red, flat, scaly patches
- 1. sun exposure
2. HPV 16
- 1. no dermal invasion
2. BM intact
155
SCC
- appearance (4)
- histology
1. nodular
2. hyperkeratotic
3. rapidly growing
4. may ulcerate
- dermal invasion through BM
156
BCC "Rodent ulcer"
- what
- appearance (3)
- risk factors (5)
- histology
- most common cancer in humans
- 1. rolled edge
2. telangectasia
3. pearly surface
- 1. age
2. fair skin
3. high UV exposure
4. radiation
5. immunosuppression
- palisading = nuclei align at outermost layer
157
Risk factors associated with malignant melanoma (5)
1. caucasian
2. female
3. age >30yrs
4. sun exposure in childhood
5. xeroderma pigmentosa
158
Histology of Malignant Melanoma (2)
1. atypical melanocytes
| 2. "buckshot appearance" of vertical pagetoid cells
159
Subtypes of Malignant Melanoma (4) SLAN
1. Superficial spreading melanoma - irregular border & variation in colour
2. Lentigo melanoma - more common in elderly
3. Acral melanoma - darker skins, occurs on palms/soles/subungual
4. Nodular malignant melanoma - 50-60yrs
160
Hutchinson's sign
pigmentation of the nail in acral melanoma
161
Breslow's thickness
- prognostic factor in melanoma
| 3. 5mm 50% 5 year survival
162
Pityriasis Rosea
- appearance (2)
- cause
- salmon pink herald patch, followed by oval macules afterwards in a Christmas tree distribution
- post-viral illness
163
Pityriasis vesicolor
- appearance (2)
- where (3)
- cause
- well defined macular lesions variable in colour
- chest, upper arms, back
- M.furur fungal infection
164
SJS
- what
- causes
- % body surface
- mucocutaneous necrosis at at least 2 mucosal sites
- drugs - abx, anticonvulsants
-
165
TEN
- what
- causes
- % body surface
- histology (2)
Toxic Epidermal Necrosis
drug-induced
>30%
full thickness epidermal necrosis with subepidermal detachment
166
What is SLE?
Systemic Lupus erythematosus
multisystemic autoimmune disorder where autoantibodies are made against a variety of autoantigens causing tissue damage due to immune complex formation & deposition and complement formation
167
Which type of hypersensitivity reaction is SLE?
Type III
168
Laboratory results in SLE? (2)
1. HIGH ESR
| 2. Norm CRP
169
HLA association in SLE?
HLA DR2/DR3
170
Autoantibodies involved in SLE? (3)
1. ANA
2. Anti-dsDNA
3. Anti-Sm
171
Histological features (5)
1. LE bodies
2. kidney - immune complex deposition
3. CNS - small vessel angiopathy
4. spleen - onion skin lesions
5. heart - Libman-Sack endocarditis
172
Signs & symptoms in SLE (11)
SOAP BRAIN MD
S - serositis
O - oral ulcers
A - arthritis
P - photosensitivity
```
B - blood disorders (AIHA, ITP, leucopenia)
R - renal involvement
A - ANA +
I - immune phenomena
N - neuro symptoms
```
M - malar rash
D - discoid rash
173
What condition in pregnancy is associated with SLE?
Anti-phospholipid syndrome
autoimmune hypercoagulable state promoting blood clots, increases risk of miscarriage and still birth
174
Scleroderma
"tight-skin"
multi-systemic autoimmune condition involving Th17 and Th2 cells promoting widespread inflammation which recruits fibroblasts and results in fibrosis
175
Limited scleroderma
- what
- signs and symptoms (5)
- fatal complication
```
- limited to skin changes in face and distal limbs to elbow & knees
C - calcinosis
R - Rayanud's phenomenon
E - oesophagealdismotility
S - sclerodactyly
T - telangiectasia
- pulmonary hypertension
```
176
Which autoantibody is involved in limited scleroderma?
anti-centromere
177
HLA association with scleroderma?
HLA DR6 & HLA DRw8
178
Which autoantibodies are involved in diffuse scleroderma? (3)
1. anti Scl-70
2. Fibrillarin
3. RNA polymerase
179
What difference is there in diffuse scleroderma?
skin changes can occur throughout the entire body with widespread organ involvement
180
Organ involvement associated with diffuse scleroderma? (3)
1. lungs - pulmonary fibrosis
2. renal
3. GI
181
Histological finding in limited scleroderma? (2)
1. increased collagen in skin and organs
| 2. onion skin thickening of arterioles
182
Type of hypersensitivity reaction involved in Dermatomyositis?
Type III
183
Type of hypersensitivity reaction involved in Polymyolitis?
Type IV
184
Autoantibody associated with dermatomyositis?
Anti-Jo 1 - tRNA synthetase
185
Autoantibody associated with polymyositis?
anti-signal recogition peptide antibody
186
Signs and symptoms associated with dermatomyositis? (4)
1. proximal muscle weakness
2. Rash a) heliotrope rash
b) Gottron's papules
3. malaise
4. pulmonary fibrosis
187
Investigations in dermatomyositis? (4)
1. skin biopsy
2. MRI
3. EMG - abnormal
4. CK - HIGH
188
Difference between dermatomyositis and polymyolitis?
DM - immune mediate vasculitis
| PM - immune complexes outside of capillaries cause damage to myofibres
189
Sarcoidosis
multisystem disease of unknown cause, characterised by non-caseating granulomas
190
Prevelance of sarcoidosis? (3)
F>M
afro-Caribbean
young adults
191
Histological findings in Sarcoidosis? (3)
1. non-caseating granulomas
2. Schaumann bodies
3. Asteroid bodies
192
Effects on lungs in sarcoidosis? (3)
1. bilateral hilar lymphadenopathy
2. fibrosis
3. lymphocytosis
193
Skin conditions in sarcoidosis? (3)
1. erythema nodosum
2. lupus pernio
3. skin nodules
194
Eyes conditions in sarcoidosis? (3)
1. anterior/posterior uveitis
2. uveoparotid fever
3. keratoconjuncitivitis sicca
195
Uveoparotid fever?
bilateral uveitis + parotid gland enlargement + facial nerve palsy
196
Effects on liver in sarcoidosis? (4)
1. hepatitis
2. hepatomegaly
3. cholestasis
4. cirrhosis
197
Effects on heart in sarcoidosis? (3)
1. dysrhythmias
2. cardiomyopathy
3. conduction defects
198
Laboratory results in Sarcoidosis? (3)
1. hypercalcemia
2. HIGH ESR
3. HIGH ACE
199
Why hypercalcemia in sarcoidosis?
Vit D hydroxylation is activated by the macrophages
200
Effects on the kidneys in sarcoidosis? (2)
due to hypercalcemia...
1) renal calculi
2) nephrocalcinosis
201
Takayasu's arteritis
- what kind of vasculitis
- who
- what
- symptoms (3)
- Large vessel
- Japanese women
- "pulseless" disease due to fibrosis of the aorta and its main branches, absent pulses in the upper extremities
- 1) absent pulse
2) bruits
3) claudication
202
Temporal Arteritis "Giant Cell Arteritis"
- what kind of vasculitis
- who
- what
- symptoms (4)
- association
- histology (3)
- laboratory findings (1)
- large vessel
- elderly
- chronic lymphocytic inflammation
- 1) scalp tenderness
2) temporal headache
3) blurred vision
4) jaw claudication
- polymyalgia rheumatica
- 1) granulomatous transmural inflammation
2) giant cells
3) skip lesions
- HIGH ESR
203
Polyartheritis Nodosa (PAN)
- what kind of vasculitis
- what
- type of hypersensitivty reaction
- pathology
- common feature
- associations
- organ spared
- histology (3)
- medium vessel
- necrotising arteritis causing fibrosis
- type III
- microanneurysms form everywhere
- renal involvement
- underlying Hep B
- lungs
- 1)polymorphs
2) lymphocytes
3) eosinophils
204
Kawasaki's Disease
- what kind of vasculitis
- who
- symptoms (5)
- severe complication
- medium vessel
- children 5 days
2) erythematous palms and soles with desquamation
3) strawberry tongue
4) cervical lymphadenopathy
5) oedema of lips, mouth, tongue
- coronary artery involvement causing aneurysm
205
Buerger's Disease
- what kind of vasculitis
- who (3)
- what
- symptoms (3)
- pathology
- medium vessels
- 1) men
2) heavy smokers
3)
206
```
Wegener's Granolomatosis
"Granulomatosis with polyangitis"
- type of vasculitis
- type of hypersensitivity reaction
- triad (ELK)
- autoantibody specific
- key features (3)
```
- small vessel
- type II
- 1) ENT - upper resp tract - sinusitis, epstaxis
2) Lung - lower resp tract - cavitation
3) Kidney
- cANCA
- 1) saddle nose
2) pulmonary haemorrhage
3) crescenteric glomerulonephritis
207
Churg Strauss
- type of vasculitis
- type of hypersensitivity reaction
- features (AEV)
- specifc autoantibody
- small vessel
- type II
Asthmas, Allergic rhinitis
Eosinophilia
Vasculitis
- pANCA (anti MPO)
208
Microscopic polyangitis
- type of vasculitis
- type of hypersensitivity reaction
- features (2)
- specific autoantibody
- small vessel
- type II
1) pulmonary haemorrhage
2) glomerulonephritis
- pANCA (anti MPO)
209
Henoch Schonlein Purpura HSP
- type of vasculitis
- Immune mediated
- who (2)
- preceding illness
- presentation (5)
- small vessel
- IgA
- children girls
- URTI
1) palpable pupuric rash over lower limb extensor surface and buttocks
2) collicky abdo pain
3) haematuria (gloemrulonephritis)
4) orchitis
5) arthritis
210
What is amyloidosis?
multisystem disorder caused by abnormal folding of proteins that are deposited as amyloid fibrils in tissues, disrupting their normal function
211
Primary amyloidosis
- type of amyloid
- presentation (4)
- light-chain amyloid
1) monoclonal Ig
2) free light chains in serum and urine
3) Bence jones proteins in urine
4) increased bone marrow plasma cells
212
Secondary amyloidosis
- type of amyloid
Causes (3)
- Amyloid A
1) autoimmune - RA, AS, IBD
2) Chronic infection - TB, osteomyelitis, IVDU
3) Non-immune - renal carcinoma, Hodgkin's
213
Haemodialysis associated amyloidosis is associated with deposition of which protein?
B-2-microglobulin
214
```
Familial Amyloidosis
- association
- inflammatory factor
serosal surfaces affected (3)
- type of amyloid
- predominant feature
```
- Familial Mediterranean Fever
- IL-1
1) pleura
2) peritoneum
3) synovium
- amyloid A
- renal deposition
215
Clinical features of Amyloidosis (5)
```
Renal - nephrotic syndrome
Cardio - conduction defects, heart failure
Liver/spleen - organomegaly
Tongue - macroglossia
Neuropathies - carpal tunnel
```
216
Histology associated with Amyloidosis?
Apple green birefringence with Congo Red stain under polarised light (due to misfolded beta pleat)
