Rheumatology Flashcards
1
Q
Describe the term Seropositive Inflammatory Arthritis.
A
Umbrella term for joint inflammation caused by autoantibodies e.g. RA (anti-CCP and RF), SLE (ANA, dsDNA, Anti-Sm, Anti-Ro, Anti-SRP), Scleroderma (anti-Scl70 + anti-centromere), Sjogren’s (Anti-La + Anti-Ro) and Dermatomyositis (anti-Jo)
2
Q
Positive Anti-CCP is seen in what condition?
A
RA
3
Q
Positive dsDNA is seen in what condition?
A
SLE
4
Q
Positive Anti-SM is seen in what condition?
A
SLE
5
Q
Positive anti-Ro is seen in what condition?
A
SLE; Sjogren’s
6
Q
Positive anti-La is seen in what condition?
A
Sjogrens
7
Q
Anti-centromere Abs being positive is suggestive of what condition?
A
Systemic sclerosis (limited)
8
Q
Anti-Scl70 Ab being positive is indicative of what condition?
A
Systemic sclerosis (diffuse)
9
Q
What does anti-Jo1 Ab suggest?
A
Myositis
10
Q
What does positive Anti-cardiolupin Ab suggest?
A
Anti-Phospholipid Syndrome
11
Q
What does positive Lupus anticoagulant suggest?
A
Anti-phospholipid syndrome
12
Q
What does positive Lupus anticoagulant suggest?
A
Anti-phospholipid syndrome
13
Q
What is the difference between cANCA and pANCA?
A
Two types of ANCA, each target a different protein within the cytoplasm of neutrophils and monocytes.
pANCA targets MPO
cANCA targets PR3
14
Q
What group of conditions are present if ANCA Abs are present?
A
Small vessel vasculitis (GPA; EGPA; MPA)
15
Q
State 3 RFs for SLE.
A
- Female sex
- Young onset: 15-45 years
- Drugs: Procainamide/Sulfasalazine/Isoniazid/ Phenytoin/ Carbamazepine
- Infection: EBV
16
Q
How may Lupus present?
A
- Fever
- Tiredness
- Weight loss
Skin • Butterfly rash: cheeks + bridge of nose • Vasculitic lesions • Urticaria • Photosensitivity • Alopecia
Nervous system • Epilepsy • Migraine • Cerebellar ataxia • CN lesions • Polyneuropathy
Lungs
• Pleurisy/ pleural effusion
CV • Pericarditis/ Pericardial effusion • Myocarditis • Thrombosis • Atherosclerosis
GI • Abdominal pain • Vomiting • Diarrhoea • Dysphagia • Mesenteric vasculitis
Kidneys
• Glomerulonephritis
17
Q
What skin lesion is typically present in Lupus?
A
Butterfly rash on cheeks and nasal bridge. Rash is photosensitive.
Urticaria
Vasculitis lesions
Alopecia
18
Q
What blood test(s) are clinically suggestive of SLE?
A
• Antibodies: Anti-dsDNA; ANA; Anti-Ro; Anti-La and Anti-RNP may be seen in SLE
19
Q
How do you treat SLE with joint symptoms and systemic disease?
A
• Anti-malarial: Hydroxychloroquine \+ • NSAIDs: Ibuprofen/Naproxen \+ • Corticosteroids: Prednisolone
± (Systemic disease)
• Immunosuppressants: Azathioprine/ Mycophenolate mofetil
20
Q
How would you treat SLE which causes severe organ disease?
A
Severe Organ Disease
• IV Steroids
+
• Cyclophosphamide
and seek help from HDU
21
Q
Describe Sjogrens syndrome.
A
Autoimmune disease featuring immunologically mediated destruction of epithelial exocrine glands via lymphocytic infiltration which also causes polyarthritis. Sjogrens syndrome may be primary or secondary to another autoimmune disorder – SLE, RA.
22
Q
How may Sjogrens syndrome present?
A
- Dryness of the mouth (xerostomia)
- Dry eyes (keratoconjunctivitis sicca)
- Parotid gland swelling (parotitis)
- Arthralgia and fatigue
- Raynaud’s Phenomenon
- Angular stomatitis may occur
23
Q
What are the diagnostic criteria for Sjogren’s Syndrome?
A
4 or more of:
• Ocular symptoms- dry eyes > 3 months (keratoconjunctivitis sicca), foreign body sensation, use of tear substitutes > 3 x daily
• Ocular signs- Schirmer’s test performed without anaesthesia (essentially see how wet some filter paper gets)
• Oral symptoms-dry mouth, recurrently swollen salivary glands, liquids aids used to swallow
• Oral signs- abnormal salivary scintigraphy
• Autoantibodies: Anti-Ro or Anti La antibodies
Mnemonic: Eyes, (Anti-)Ros and Mouth
24
Q
What are the diagnostic criteria for Sjogren’s Syndrome?
A
4 or more of:
• Ocular symptoms- dry eyes > 3 months (keratoconjunctivitis sicca), foreign body sensation, use of tear substitutes > 3 x daily
• Ocular signs- Schirmer’s test performed without anaesthesia (essentially see how wet some filter paper gets)
• Oral symptoms-dry mouth, recurrently swollen salivary glands, liquids aids used to swallow
• Oral signs- abnormal salivary scintigraphy
• Autoantibodies: Anti-Ro or Anti La antibodies
Mnemonic: Eyes, (Anti-)Ros and Mouth
25
State the name of the test for eye dryness in Sjogrens syndrome.
Schirmer test
26
What does a Schirmer test consist of?
Schirmer’s test performed without anaesthesia (essentially see how wet some filter paper gets)
Filter paper placed in lower conjunctival sac with positive being < 5mm
27
How do you manage Sjogrens syndrome?
* Artificial tears/lubricating eye drops
* Pilocarpine- can stimulate salivary gland production (however also produces flushing)
* Hydroxychloroquine can help with arthralgia and fatigue
28
What are the side effects of Pilocarpine?
Cholinergic parasympathomimetic agent thus SLUDGE
```
Sweating
Lacrimation
Urinary disorders
Defaecation
GI upset
Emesis
```
Flushing of skin
29
Describe Scleroderma.
Autoimmune disorder which affects multiple systems involving the activation of immune system featuring upregulation of adhesion molecules (VCAM, E-selectin, ICAM-1) which stimulates production of ECM via fibroblasts yielding abnormal connective tissue growth.
30
What type of molecules are upregulated in systemic sclerosis?
Upregulation of adhesion molecules - VCAM, E-selectin, ICAM-1 thus ECM production via fibroblasts
31
What are the two types of Scleroderma?
- Limited: Begins in hands, fingers, face and progresses to body (Anti-Centromere)
- Diffuse: Less common but more aggressive; earlier organ involvement with arthralgia and myalgia occurring as well as pulmonary disease and cardiac disease (Anti-Scl70)
32
What are the pentad of features in Limited Systemic Sclerosis?
CREST
Calcinosis cutis
Raynaud's
Oesophageal dysmotility
Sclerodactyly
Telangiectasia
33
What symptoms may be present in diffuse scleroderma (in addition to limited scleroderma)?
Diffuse Scleroderma
• Cough
• SOBE
• Rales at lung bases
* Dysphagia
* Heartburn/Reflux
* Bloating
* Malabsorption
* Bowel dysmotility
* Esophageal dysmotility
* Arthralgia
* Inflammatory arthritis
* Myositis
34
What investigation may confirm oesophageal dysmotility in Systemic Sclerosis?
• Barium swallow: Reduced peristalsis; Gastroparesis; Reduced sphincter tones; Strictures
35
What investigation may confirm thoracic involvement in Systemic Sclerosis?
• CXR: Interstitial infiltrates; Cardiomegaly
36
What autoantibodies will be positive in Systemic Sclerosis?
• Serum auto-antibodies: ANA; Anti-Scl70 (anti-topoisomerase I) positive = diffuse; Anticentromere Ab positive = limited
37
How would you manage Systemic Sclerosis?
Tx dependent on Sx and Organ involvement
Renal Crisis
• ACEi: Captopril/ Enalapril/ Lisinopril
+
• Antihypertensives: CCBs (Lercanidipine)
2nd
• RRT: Dialysis or Transplant
Raynaud’s phenomenon
• Supportive: Exercise/Warmth
+
• CCB: Nifedipine
± (Ulcers)
• PDE5: Sildenafil
GI
• PPI: Omeprazole/ Esomeprazole
± (Gastroparesis)
• Prokinetic agent: Domperidone/ Octreotide
Myopathy/ Synovitis/ Arthritis
• Oral corticosteroid: Prednisolone
```
Interstitial lung disease
• Immunomodulator: Cyclophosphamide/Mycophenolate mofetil/Azathioprine
+
• Oral corticosteroid: Prednisolone
+
• Oxygen
```
Cardiac arrhythmia
• Antiarrhythmics
38
How may you medically treat Raynaud's Phenomenon?
Nifedipine
Sildenafil
39
How may you manage ILD in Diffuse Systemic Sclerosis?
```
• Immunomodulator: Cyclophosphamide/Mycophenolate mofetil/Azathioprine
+
• Oral corticosteroid: Prednisolone
+
• Oxygen
```
40
Describe Antiphospholipid Syndrome.
Autoimmune disease caused by antibodies targeting the phospholipid cell membrane which causes arterial and venous thrombosis. This can be primary or secondary to other immune disorders.
41
How may Antiphospholipid Syndrome present?
* Stroke
* TIA
* MI
* Valvular heart disease
* DVT
* Budd-Chiari Syndrome (occlusion of hepatic veins ascites, abdo pain + hepatitis)
• Recurrent miscarriages = 3≤
42
What is the triad of clinical features observed in Budd-Chiari Syndrome?
Ascites
Abdominal Pain
Hepatomegaly
43
What would bloods show in a patient with Anti-Phospholipid Syndrome?
• APTT: Increased
• FBC: Thrombocytopenia
• Antibodies: Anti-cardiolipin; Lupus anticoagulant (LA)
-> LA is an autoimmune phospholipid antibody
44
Which clotting pathway is increased in Anti-phospholipid syndrome?
What blood test reflects this?
Intrinsic
APTT thus problem with clotting factors 8, 9, 11 and 12
45
How do you manage a patient with Antiphospholipid Syndrome?
Aspirin 75mg
Warfarin
46
How do you manage Anti-phospholipid syndrome in a pregnant woman?
LMWH 1mg/kg ('check BNF')
47
Describe Polymyositis and Dermatomyositis.
Autoimmune disorder featuring necrosis of skeletal muscle fibres (polymyositis) and/or skin (dermatomyositis) which is of unknown aetiology (idiopathic) characterised by proximal muscle weakness, myalgia, dysphagia, SOB and Shawl sign (V-shaped superior truncal rash), Heliotrope rash (reddish purple rash around eyelids), Gottron’s papules (violaceous bumps on outside of joints)
48
What classic dermatological clinical features of Dermatomyositis are there?
Gottron's papules
Shawl Sign
Heliotrope rash
49
What is the term for the rash around the eyes in Polydermatomyositis?
Heliotrope rash
50
What is the term for the V-shaped truncal rash in Polydermatomyositis?
Shawl sign
51
What is the term for violaceous bumps on the outside of the joints?
Gottron's papules
52
What investigations would you run in a suspected case of polymyositis/dermatomyositis?
• Muscle biopsy: T-cell infiltration and necrosis
• Serum muscle enzymes (CK; ALT; aldolase): Elevated
• Abs: Anti-JO positive; ANA positive; anti-SRP positive
-> Antisynthetase Ab = Anti-Jo (Ab in polymyositis and ILD); Anti-signal recognition particle = Anti-SRP (present in necrotising myopathy)
• ESR: Normal; Raised
• EMG: characteristic changes
• MRI: Muscle inflammation
53
How do you treat an acute bout of Polymyositis/Dermatomyositis?
Oral corticosteroids: Prednisolone 1/12
Immunosuppressants: Azathioprine/ Methotrexate/ Ciclosporin
54
Describe Undifferentiated Autoimmune Rheumatic Disease.
Seropositive inflammatory arthritis which combines features of more than one arthritic rheumatoid disease.
55
What clinical feature is almost always present in Undifferentiated Autoimmune Rheumatic Disease?
Raynaud's (90%)
56
Describe Rheumatoid Arthritis.
Chronic inflammatory condition affecting small joints of hands and feet which is characterised by joint pain, swelling and persistent morning swelling resulting in reduced mobility and reduced quality of life.
57
What finger swellings are more prevalent in RA? Which joint do these impact?
Bouchard's Nodes
PIP
58
Which deformities may be present in RA? Describe the joints and the deformity in each example.
* Swan neck deformity: DIP hyperflexion + PIP hyperextension
* Boutonniere’s deformity: DIP hyperextension + PIP hyperflexion
59
Outline the clinical features of Felty's Syndrome.
Splenomegaly
RA
Neutropenia
60
The presence of pneumoconiosis and RA is termed...
Caplan Syndrome
61
What investigations would you order in an individual with suspected RA?
* Rheumatoid factor (RF): Positive
* Anti-cyclic citrullinated peptide (anti-CCP) Ab: Positive
* XR/CT: Erosions
62
How may you manage RA?
• DMARDs: Methotrexate/ Sulfasalazine/ Leflunomide
±
• Corticosteroid: Prednisolone
+
• NSAID: Ibuprofen/Naproxen/ Diclofenac
Pregnancy
• Corticosteroid: Prednisolone
63
Describe what a Seronegative Arthritis is.
Umbrella term for conditions associated with predilection for axial (spinal and sacroiliac) inflammation with asymmetrical peripheral arthritis, in the absence of antibodies (seronegative) with enthesis inflammation.
64
Describe ankylosing spondylitis.
Type of seronegative Spondyloarthritis which is a chronic, progressive disease characterised by severe pain, spinal stiffness, fatigue, sleep disturbance, SI joint tenderness and kyphosis.
65
What is the term of the diagnostic criteria used in Ankylosing Spondylitis?
Calin Criteria
66
Outline the Calin Criteria.
* Age < 40 years
* Back pain > 3 months
* Insidious onset
* Improves with exercise
* Early morning stiffness
67
What gene mutation is strongly associated with ankylosing spondylitis?
HLA-B27
68
Outline the presentation of Ankylosing Spondylitis.
* Inflammatory back pain: Early morning back stiffness; improves with exercise; insidious onset; back pain > 3 months
* Fatigue
* SI joint tenderness
* Kyphosis:
* Enthesitis
* Iritis/Uveitis
69
What investigations would have positive findings in Ankylosing Spondylitis?
* CRP: Raised
* XR-Pelvis: Sacroiliitis
* XR-Cervical/Thoracic/Lumbar spine (lateral): erosions; sclerosis; syndesmophytes; bamboo spine (late disease)
70
How would you manage Ankylosing Spondylitis?
• Supportive: Stretches/ Group sessions/ Assess for CV risk
+
• NSAIDs: Naproxen/ Ibuprofen
± (Local IA inflammation/enthesitis)
• IA Corticosteroid injection: Hydrocortisone
71
How would you manage Ankylosing Spondylitis in the case of peripheral joint involvement?
DMARDs
Biologics
72
What are the guidelines for using Biologics in Ankylosing Spondylitis?
• Biologics: Adalimumab/ Infliximab/ Etanercept
73
Describe Psoriatic Arthritis.
Type of seronegative inflammatory arthritis which is associated with psoriasis characterised by joint pain, joint stiffness, history of psoriasis and family history of psoriasis.
74
What is Arthritis Mutilans?
Deformity of joints of hands and feet, usually in conjunction with Psoriatic Arthritis, resulting in shortening of digits due to deformity at MCP and IP joints resulting in retraction of skin transversely into the space left - known as 'opera glass' hands or feet.
75
How may Psoriatic Arthritis present?
* Joint pain: prolonged morning stiffness ≥ 30 minutes; improves with use; worsened with prolonged rest
* Joint stiffness
* Dactylitis (uniform swelling of entire digit)
* Entheseal inflammation (pain at site of tendon attachment)
* Spinal stiffness + reduced ROM: Schober test
76
What investigations may suggest Psoriatic Arthritis?
Seronegative, unremarkable joint fluid aspirate and radiological finds on XR
77
How would you manage Psoriatic Arthritis?
Supportive: Physiotherapy; Exercise
Medical: NSAIDs; DMARDs; Steroids IA; Biologics
Surgery: joint fusion; arthroplasty
78
Describe Reactive Arthritis?
Inflammatory, seronegative arthritis occurring after exposure to GI and GU infections characterised by joint pain, joint stiffness and possibly a classical post-infectious triad (Reiter’s Syndrome): arthritis, urethritis and conjunctivitis
79
Outline the 3 clinical features of Reiter's Syndrome.
Arthritis
Uveitis
Conjunctivitis
'Can't see, can't wee, can't climb a tree'
80
How may Reactive Arthritis present?
* Joint pain
* Joint stiffness
* Skin rash: Keratoderma blenorrhagicum (vesicular lesion becoming plaque/pustular on volar surfaces)
* Circinate balanitis (painless ulcers on glans of penis)
81
How may Reactive Arthritis be diagnosed?
Clinical diagnosis
Aided by exclusion of other differentials... seronegative; unremarkable arthrocentesis
82
How may you manage Reactive Arthritis?
NSAIDs
83
How may enterohepatic arthritis present?
* Joint pain: Mono (one joint) or Oligoarthritis
* Joint stiffness
• Known diagnosis of UC or CD
84
What investigations may aid your diagnosis of Enterohepatic arthritis?
* Arthrocentesis: Negative; Sterile
* Rheumatoid factor: Negative
* CRP: Elevated
* XR: Erosions
85
How would you manage enterohepatic arthritis?
```
• DMARD: Methotrexate/ Sulfasalazine/ Leflunomide
±
• Corticosteroids: Prednisolone
±
• Analgesia: Ibuprofen/ Naproxen
```
86
UVB converts 7-dehydrocholesterol into?
Cholecalciferol
87
Where is cholecalciferol stored?
WAT
88
Where is Cholecalciferol activated?
Liver, by 25-alpha hydroxylase
89
What converts cholecalciferol to 25-hydroxycholecalciferol?
25-alpha hydroxylase
90
What is the location for the formation of calcitriol?
Kidneys
91
What is the reactant used to convert 25-hydroxycholcalciferol into calcitriol?
1 alpha hydroxylase
92
Describe Osteoporosis.
Bone disease caused by reduction in bone mass and micro-architectural deterioration of bone tissue leading to fragility and increased fracture risk
93
Outline the difference between osteopenia and osteoporosis.
Osteopenia is a reduced bone mass between -1 to -2.5 SDs whilst Osteoporosis is a reduced bone mass greater than -2.5 SDs
94
State 5 RFs for Osteoporosis.
- Female sex
- Caucasian
- Increasing age
- Hypogonadism
- FHx Hip Fx
- Disease: DM; Multiple myeloma; Cushing’s syndrome; Hyperthyroidism; Hyperparathyroidism
- Immobilisation
- Low BMI
- Smoking
- Alcohol abuse
- Vitamin D intake
- Drugs: Heparin/Ciclosporin/ Anticonvulsants
95
How may Osteoporosis present?
* Fractures: Vertebral/ Proximal femur/ Distal radius (Colles’ Fx)
* Kyphosis
96
What is the gold standard investigation for Osteoporosis?
* DEXA: T-score < 2.5 SDs
| * XR: Osteopenia; Fx
97
What tool is used to assess Osteoporotic fracture risk?
FRAX Score
98
How may you manage a patient with Osteoporosis?
Bisphosphonates are first line with Vitamin D and Calcium as an Adjunct.
Second line is Denosumab, a RANKLi
If there is a high risk fracture and vertebral fractures, PTH-R antagonist Teriparatide could be used
In a postmenopausal woman, Oestrogen may be added
99
When might you use Teriparatide in an Osteoporotic patient?
High risk fractures with T-Score <3 and vertebral fractures
100
Describe Osteopetrosis.
Condition caused by reduced bone resorption by osteoclasts leading to craniofacial abnormalities, chest wall deformities and skeletal enlargement
101
List some clinical features of Osteopetrosis.
* Facial nerve paralysis
* Macrognathia
* Teeth infections
* Vision loss
* Deafness
* Cranial nerve palsies
102
What is the gold standard investigation for Osteopetrosis?
DEXA
XR
103
Outline the management for a patient with osteoporosis.
```
• Analgesia: Ibuprofen
+
• Corticosteroids: Prednisolone
+
• Vitamin D: Ergocalciferol
±
• Surgery
```
104
In adults, what is hypomineralisation of the bone secondary to vitamin D deficiency called?
Osteomalacia
105
In children, hypomineralisation of the bone secondary to Vitamin D deficiency is termed?
Rickets Disease
106
How may Osteomalacia/Rickets present?
* Proximal muscle weakness
| * Bone deformities: bowing + genu valgum
107
What investigations would you order in a patient with suspected Osteomalacia?
* Vitamin levels: OHD low
* Serum biochemistry: ALP high
* XR: Defective mineralization; Looser’s pseudofractures (low-density bands perpendicular to cortex) seen in pelvis and femur
108
What is the term for low-density bands seen perpendicular to the cortex as a clinical feature of Osteomalacia?
Looser's Pseudofractures
109
How would you manage Osteomalacia?
• Vitamin replacement: 25-hydroxyvitamin D at 50 000U for 8/52
110
What is the second treatment of choice for Osteomalacia?
Why may someone use this?
Ergocalciferol (Vitamin D2(
Cultural, dietary or religious - does not contain gelatine
111
State some side effects of Vitamin D.
Abdominal pain
Headache
Nausea
Skin reactions
112
What disease features aberrant remodelling due to osteoclast resorption and osteoblast deposition causing weaker bone with sclerotic and lytic areas observed on XR.
Paget's disease of the bone
113
Outline the clinical features of Paget's disease.
* Bone pain
* Deformities: skull enlargement; tibial bowing
* Nerve compression: Deafness; paraparesis
* Fractures
* Osteogenic sarcoma
* High-output cardiac failure
114
Outline the process by which high-output cardiac failure may occur in Paget's disease.
Hyperactive osteoblasts deposit new bone matrix which is hyperperfused thus increased blood flow there, reducing blood in systemic circulation hence reduced EDV and F-S Principle compromised
115
How do you manage Paget's Disease?
• Bisphosphonates: Zoledronate
116
What investigations would positively identify Paget's disease?
* Bone markers: ALP raised; Normal Calcium; Normal Phosphate
* XR: Bone enlargement; distortion; sclerotic changes; osteolytic areas
* Radionuclide bone scan: Increased uptake of bone-seeking radionuclides
117
Describe osteonecrosis.
Bone disease causing death of bone and marrow cells due to hypoperfusion
118
Outline 3 causes of osteonecrosis.
* Drugs: Glucocorticoids/ Bisphosphonates
* Sickle cell disease
* Trauma
* Radiation
* HIV infection
119
Describe Osteochondritis dissecans.
Idiopathic subchondral bone lesion causing sequestration of bone and instability – generally in young, athletic patients.
120
What are the clinical features of Osteochondritis dissecans.
* Pain: on activity; ankle/ knee/ heel
* Effusion
* Crepitus
* ROM
* Young patient
121
How may you manage Osteochondritis Dissecans?
Stable
• Conservative: Ibuprofen ± Paracetamol; physiotherapy; technique correction
Unstable
• Surgery: Arthroscopy
122
What is Hyperparathyroidism?
Endocrine cause of bone disease featuring excessive secretion of parathyroid hormone (PTH) from the parathyroid gland which can be categorized into primary, secondary or tertiary dependent on biochemical abnormalities and aetiology.
123
Describe Hyperparathyroidism.
Endocrine cause of bone disease featuring excessive secretion of parathyroid hormone (PTH) from the parathyroid gland which can be categorized into primary, secondary or tertiary dependent on biochemical abnormalities and aetiology.
124
How do the types of Hyperparathyroidism differ from eachother?
- 1º: Hyperactive PT gland = RANKL secreted to RANK on Oc = activation thus osteoclast resorption yielding elevated Ca2+, elevated PTH, low Pi
- 2º: Low calcium feedback to PT gland thus PTH elevated, Ca2+ low, Pi normal and ALP may be elevated
- 3º: Untreated secondary hyperparathyroidism causes chronic elevated PTH, elevated Ca2+ and elevated Pi and ALP
- Malignant HyperPT: Squamous cell carcinoma of lung or renal cell carcinoma produces PTH-p to elevated Ca2+
125
A solitary adenoma secreting excess parathyroid hormone would be what type of Hyperparathyroidism?
Primary
126
Chronically untreated parathyroid cancer would be classified as what type of Hyperparathyroidism?
Tertiary
127
Dietary deficiency detected by low Ca2+ and elevated PTH would be what type of hyperparathyroidism?
Secondary
128
Give an example of a cancer which may cause malignant hyperparathyroidism.
Breast
Lung - Squamous Cell Carcinoma
Renal
Multiple Myeloma
129
What are the clinical features of hyperparathyroidism.
* Fatigue
* Malaise
* Depressed
* Thirsty
* Dehydrated
* Renal stones
* Abdominal pain
* Pancreatitis
* Ulcers (duodenal > gastric)
130
What electrolyte and hormone patterns are seen in primary hyperparathyroidism regarding?
Elevated PTH
Elevated Calcium
ALP high
Pi decreased (normal in CKD)
131
What electrolyte and hormone patterns are seen in secondary hyperparathyroidism regarding?
PTH: Elevated
Ca: Low
ALP: N
Pi: N
132
What electrolyte and hormone patterns are seen in tertiary hyperparathyroidism regarding?
PTH: High
Ca: Elevated
ALP: Elevated
Pi: Elevated
133
How would you treat primary hyperparathyroidism?
• Conservative: Fluid intake (reduce calculi)
+
• Cinacalet
+
Tx cause
134
How would you manage secondary hyperparathyroidism?
```
• Tx underlying condition
+
• Hyperphosphatemia: Binders/ Diet (Calcium acetate; Ferric citrate)
+
• Vitamin D intake
```
135
How would you treat tertiary hyperparathyroidism?
```
• Surgery
+
• Hyperphosphatemia: Binders/ Diet
+
• Vitamin D intake
```
136
Describe gout.
Syndrome caused by hyperuricemia and deposition of urate crystals causing attacks of inflammatory arthritis
137
State 3 RFs for Gout.
* Older age
* Male
* Consumption of meat, seafood and alcohol
* Use of diuretics
* Drugs: Cyclosporine (urate reabsorption)/ Pyrazinamide (urate reabsorption)/ NSAIDs (reduce GFR)
138
How does cyclosporine increase your risk of gout?
Increased urate reabsorption/reduced renal excretion
139
How does pyrazinamide increase the risk of gout?
Reduced urate reabsorption
140
How do NSAIDs increase the risk of gout?
Reduced eGFR
141
Outline the pathophysiology of Gout.
• Uric acid super-saturation = crystal formation -> DAMPs bind to macrophages and PRRs -> inflammatory response featuring TNF-a; IL-1ß; IL-8 and neutrophil influx
142
Outline the clinical features of gout.
* Severe pain: Rapid onset;
* Joint stiffness: Distributed in the feet (1st MCP/TMT and ankle); Monoarticular or oligoarticular (< 4 joints)
* Joint swelling
* Joint effusion
* Tenderness
* Tophi: Present on extensor surface joints – e.g. hands, elbows, knees, Achilles tendons
143
What investigation would you order for suspected cases of gout?
• Arthrocentesis: Leukocytosis; Needle-shaped crystals
144
How do you manage an acute episode of gout?
• NSAIDs: Naproxen/Ibuprofen/Diclofenac/ Celecoxib
145
How do you treat recurrent gout?
• Xanthine oxidase inhibitors: Allopurinol
+
• Uric Acid Transporter Inhibitor: Lesinurad
+
• NSAIDs: Naproxen/Ibuprofen/Diclofenac/ Celecoxib
146
Describe Pseudogout.
Type of seronegative, inflammatory arthritis which is a crystal arthritis caused by deposition of calcium pyrophosphate (CPP) crystals
147
State 3 RFs of Pseudogout
* Advanced age
* FHx of CPPD
* Hypomagnesemia
* Hypophosphatemia
* Hyperparathyroidism
* Wilson’s Disease
148
Outline the clinical presentation of pseudogout.
* Joint pain
* OA-like involvement: wrists/shoulders/knee
* Exacerbation of OA
* Joint effusion
* Erythema
* Swollen joint
149
What would the investigations and results would you anticipate in a case of Pseudogout?
* Arthrocentesis: Rhomboid-shaped crystals
* XR: Linear deposits in fibro-cartilage or hyaline articular cartilage (chondrocalcinosis); rapid joint degeneration
* Serum Calcium: Normal; Elevated
* Serum PTH: Normal; Elevated
* Serum Mg: Normal; Elevated
150
How do you manage Pseudogout?
Acute
• NSAIDs: Naproxen/ Diclofenac
±
• Analgesia: Paracetamol
± (Monoarticular disease)
• IA Corticosteroids: Dexamethasone
```
Chronic
• Surgery: Joint replacement
±
• NSAIDs: Naproxen/ Diclofenac
±
• Analgesia: Paracetamol
```
151
How may Vasculitides be classified?
- Vessel size
- ANCA
- IgE
152
Define Takayasu's Arteritis.
Chronic progressive, inflammatory disease of the aorta causing occlusion. Takayasu’s arteritis commonly affects young females (15-35).
153
Outline the clinical features of Takayasu's Arteritis.
* Systemic Sx: Fever, malaise, fatigue
* Claudication pain
* Tenderness on palpation of artery
* Bruit
* Loss of pulses
154
How do you manage Takayasu's arteritis?
• Corticosteroids: Prednisolone 40-60mg
± (Stenosis)
• Surgery: Angioplasty
155
Define Polymyalgia Rheumatica.
Chronic inflammatory condition of the large vessels of unknown cause characterised by bilateral proximal muscle stiffness (neck and shoulders) and ache affecting > 50 age group
156
Outline the clinical features of Polymyalgia Rheumatica.
* Morning stiffness >1 hour; improve in day
* Myalgia: hip and shoulder girdle
* Systemic Sx: Fever, malaise, weight loss
* Often associated with GCA
157
What investigation and result is suggestive of Polymyalgia Rheumatica in clinical context.
* ESR: Elevated
| * CRP: Elevated
158
How do you manage Polymyalgia Rheumatica.
* Corticosteroids: Prednisolone 15mg
| * Bone protection: Vitamin D; Alendronic acid
159
1. Condition involving transmural inflammation of all 3 arterial layers with lymphocytes, macrophages and multinucleate giant cells.
2. Clinical features (3)
3. Gold standard investigation (1)
4. Investigation to confirm (2)
5. Treatment
1. GCA
2. Headache; Scalp tenderness; Visual changes; Jaw claudication
3. ESR
4. Biopsy of temporal artery
5. Corticosteroids
160
Cough, haemoptysis, malaise and oral ulceration featuring a blood film of schistocytes, haematuria, cANCA and Granulomas in the kidneys.
Diagnosis?
Clinical Features?
Ix to order?
Tx?
Granulomatosis with Polyangiitis (Wegener's Syndrome)
* Cough
* Chest pain: Pleurisy
* SOB
* Haemoptysis
* Malaise
* Arthralgia
* Haematuria
* Purpura
* Oral ulceration
* Pleural effusion
* Conjunctivitis
Urinalysis; Bloods; Abs; Autoantibodies; CXR; Kidney biopsy
Immunosuppressants - cyclophosphamide and prednisolone
161
Patient presenting with a cough, SOBE, haemoptysis and purpura on the leg. IgE is raised on bloods and ANCA is positive.
Diagnosis?
Triad of features?
Management?
Churg-Strauss Syndrome
Asthma + Eosinophilia + Vasculitis
Prednisolone
162
Young adult presenting with purpuric rash on buttocks, abdominal pain and vomiting. Usually well, last had a cold 2 weeks ago. IgA is positive and urinalysis shows blood and protein.
Diagnosis?
Treatment?
IgA Nephropathy/ HSP
Supportive - NSAIDs/ Anti-emetics/ Fluids
163
Patient presents with distal fingers being blue, dry eyes and dry mouth, a rash along the legs. Bloods show proteins which precipitate in the cold. Urinalysis shows nephritic syndrome.
Diagnosis?
Name of proteins?
Name of group of symptoms featuring dryness.
Treatment?
Cryoglubulinaemic vasculitis
Cryoglobulins
Sicca symptoms - keratoconjunctiva sicca; xerostomia; hypohydrosis
• Antivirals: Grazapravir + Elbasavir
+
• Corticosteroids: Prednisolone
164
Patient of Turkish descent presents with ulcers
165
35 y/o F with asymmetrical pain and stiffness in the fingers and feet with plaques on the extensor surface of elbow and some nail changes.
What is your diagnosis?
What are the nail changes observed?
What us the term for inflammation of an entire digit?
What may the most severe form with osteolysis and telescoping of the digits cause?
Psoriatic arthritis
Nail pitting; Onycholysis
Dactylitis
Arthritis mutilans
166
What are the causes of trochanteric bursitis?
Trauma
Inflammatory
Infection
Friction
167
What special test is used to assess hip weakness?
Trendelenberg Test
168
What movements elicit pain in trochanteric bursitis?
Abduction
Internal rotation
External rotation
169
What are the key radiographic features for OA?
Mnemonic: LOSS
Loss of joint space
Osteophytes
Subchondral sclerosis
Subchondral cysts
170
In Arthritis, swelling of the joints furthest along the fingers are known as?
Heberden's Nodes
171
In Arthritis, swelling of the joints nearest along the fingers are known as?
Bouchards's nodes
172
How is OA managed?
Supportive: weight loss; patient education; physiotherapy; orthotics
+
Medical: Oral analgesia; Topical analgesia; IA injections
±
Surgery: TKR; HA; HTO
173
Which is the most common gene association with RA?
HLA DR4
174
In which proportion of patients is RF positive?
70%
175
A 25 year old female presents with joint pain in her knuckles. The pain is present in the morning and improves as the day goes on. The pain lasts 1-2 days then resolves. These episodes have been happening for several months.
She is positive for anti-CCP and RF.
What is your diagnosis?
Palindromic Rheumatism - likely to develop full rheumatoid arthritis
176
Outline the process by which Boutonnieres deformity occurs.
Tear in the central slip of extensor components of fingers.
When patients straighten fingers, lateral tendon around the PIP pull on the distal phalynx thus DIPs extend and and PIP flex
177
What is the referral criteria for Rheumatoid Arthritis?
Persistent synovitis > 3 months = MSK referral
178
How is rheumatoid arthritis managed?
Supportive: Referral; Review; Exercise; Physiotherapy
+
Medical: Methotrexate
2nd line = ± Hydroxychloroquine/Sulfasalazine
3rd line
Methotrexate + Adalimumab
4th line
Methotrexate + Ritixumab
179
Which type of biologic is Adalimumab?
Anti-TNFa
180
Which type of biologic is Infliximab?
Anti-TNFa
181
Which type of biologic is Rituximab?
Anti-CD20
182
Which type of biologic is Sarilumab?
Anti-IL6
183
Which type of biologic is Tocilizumab?
Anti-IL6
184
Which type of biologic is Tofacitinib?
JAKi
185
Which drug is taken as an adjunct when on Methotrexate?
Folic acid 5mg - take on a different day to the methotrexate
186
List 5 common side effects of Methotrexate.
```
Anaemia
Anorexia
Diarrhoea/Vomiting
Nausea
Headache
GI discomfort
Leucopaenia - infections
Pulmonary fibrosis
Teratogenesis
Hepatotoxicity
```
187
What are the side effects of Leflunomide?
```
Mouth ulcers
Rashes
Peripheral neuropathy
Liver toxicity
Bone marrow suppression and leukopenia
Teratogenic
```
188
What effect can Salfasalazine have on male sexual health?
Reduced sperm count
189
What are the side effects of hydroxychloroquine?
Nightmares
Reduced visual acuity (macular toxicity)
Liver toxicity
Skin pigmentation
190
What are the side effects of Rituximab?
Rituximab is a monoclonal antibody against CD20 protein on B cells
```
Vulnerability to severe infections
Night sweats
Thrombocytopaenia
Liver and lung toxicity
Peripheral neuropathy
```
191
Destruction of a whole joint due to severe psoriatic arthritis is known as?
Arthritis Mutilans
192
What are the different patterns in Psoriatic Arthritis?
Symmetrical - similar to RA, hands, wrist, ankles and DIP joints affected; mainly F
Asymmetrical - affects few joints (Pauciarthritis)
Spondylitic pattern: AA joint; back stiffness; sacroillitis; mainly M
193
What tool may be used to assess risk fo Psoriatic Arthritis in a patient with Psoriasis?
Psoriasis Epidemiological Screening Tool (PEST)
194
What are the clinical features of Reiter Syndrome?
Urethritis
Conjunctivitis
Arthritis
195
Which gene is associated with reactive arthritis?
HLA B27 gene
196
What is the management for reactive arthritis?
Tx cause
+
NSAIDs ± IA injection
197
Which gene mutation is associated with Ankylosing Spondylitis?
HLA B27
198
Advanced changes from ankylosing spondylitis may result in which radiographic feature being observed?
Bamboo spine due to fusion of the VBs
199
What proportion of patients positive for HLAB27 gene will get ankylosing spondylitis?
2%
200
What proportion of patients with ankylosing spondylitis have HLAB27 gene?
90%
201
What are the referral criteria for back pain?
Age <20 or >50
Thoracic pain
Weight loss; Night sweats; Itch
Widespread neurology
Structural deformity/Trauma
CES: Saddle anaesthesia; Urinary retention; Faecal incontinence; Bilateral radiculopathy
202
What clinical test is used to assess the spine in suspected Ankylosing Spondylitis?
Schober's Test
L5 vertebrae to 10cm above
>20cm = normal
203
What skin changes are noted in SLE?
Photosensitive malar rash
Mouth ulcers
Raynaud's Phenomenon
204
Which autoantibodies are specific to SLE?
Anti-dsDNA
205
Which criteria can help you make a diagnosis of SLE?
ACR
Positive ANA + clinical features of SLE
206
What are some of the complications presenting with SLE?
Anaemia
Neuropsychiatric SLE - optic neuritis, transverse myelitis, psychosis
CVD
Pericarditis
VTE
Interstitial lung disease
Pleuritis
Lupus nephritis
Anaemia of chronic disease - leucopenia, neutropenia, thrombocytopenia
Recurrent miscarriages
207
What is the first line treatment for mild SLE?
Hydroxychloroquine
208
What is the risk of developing SLE in a patient with DLE?
<5%
209
A 30 year old female patient presenting with lesions on the face, ears and scalp that are circular shaped and sensitive to light. There is some scarring alopecia and the areas can become hypo and hyperpigmented.
They are positive for dsDNA.
What is your most likely Ddx?
Discoid lupus erythematosus
210
How do you manage DLE?
```
Supportive: avoid sun; sunscreen
+
Topical steroid
+
Intralesional steroid (hyperkeratotic)
```
211
What are the two main types of Systemic Sclerosis?
Limited (anti-centromere) and Diffuse (anti-Scl70)
212
What are the features of limited cutaneous systemic sclerosis?
Mnemonic: CREST
```
Calcinosis cutis
Raynaud's phenomenon
Esophageal dysmotility
Sclerodactyly
Telangiectasia
```
213
A patient is positive for anti-Scl70 Abs, what is your ddx?
Diffuse systemic sclerosis
214
How can you examine the nail bed in suspected systemic sclerosis?
Nailbed microscopy
215
Which investigations are key to making a diagnosis of Polymyalgia Rheumatica?
Clinical features
AND
Inflammatory markers - ESR, CRP, plasma viscosity
216
How do you manage a patient with suspected polymyalgia rheumatica?
Outline the process
Steroids - do a steroid taper
15mg 1 week, see if reduces
When symptoms controlled fully, begin taper
12.5mg for 3-4 weeks
10mg for 4-6 weeks
Reduce by 1mg every 4-8 weeks
If relapse at an interval, escalate or stay.
217
What aspects of oral prednisolone management must you consider?
Mnemonic: DONT STOP
Dont suddenly stop - Steroid dependent after 3 weeks thus risk of adrenal crisis.
Sick day rules
Treatment card
OP - bone protection
PPIs - GI protection
218
Which artery is affected in Giant Cell Arteritis?
Temporal artery
219
What gives a diagnosis of GCA?
Clinical diagnosis
Raised ESR >50mm/hour
Temporal biopsy: multinucleate giant cells
220
What is the management of Giant Cell Arteritis?
40-60mg Prednisolone started immediately
Review in 48 hours
221
Which antibodies are present in Dermatomyositits/Polymyositis?
Anti-Jo; ANA
222
what is the target INR for a patient with Antiphospholipid Syndrome?
2-3
223
What is Livedo Reticularis? Which condition is it associated with?
purple lace like rash that gives a mottled appearance to the skin
Antiphospholipid Syndrome
224
How is gout diagnosed?
Clinically
Joint aspiration - monosodium urate crystals, needle-shaped crystals, negatively birefringent of polarised light
225
What is the first line management of an acute gout flare up?
NSAIDs > Colchicine > Steroids
Secondary prevention: Lifestyle + Allopurinol; Febuxostat
226
How do you reach a definitive diagnosis of pseudogout?
Joint aspirate which shows no bacterial growth, positive birefringent of polarised light with calcium pyrophosphate crystals which are rhomboid shaped
227
What XR changes may be seen in pseudogout?
Chondrocalcinosis - thin whit line in middle of joint space due to calcium deposition
228
How should you take Oral Bisphosphonates?
Oral bisphosphonates are taken on an empty stomach sitting upright for 30 minutes before moving or eating to prevent this.
Prevents reflux/oesophageal erosions
229
What are the side effects of Bisphosphonates?
Reflux/GI upset
ONJ
ON of external auditory canal
Atypical fractures
230
Should bisphosphonates be continued continuously in a patient?
No, every 3-5 years a treatment holiday is required.
Check to see if BMD has improved and XR to check if fragility fractures.
Break for 18 months then re-assess.
231
What radiographic finds may be present in Osteomalacia?
Looser zones (fragility fractures going partially through the bone)
Bowing of the long bones
232
What are the thresholds for 25-hydroxyvitamin D?
>75 nmol/L = optimal
25-50nmol/L = vitamin D insufficiency
<25nmol/L = vitamin D deficiency
233
What is the treatment for Osteomalacia?
50 000IU once weekly for 6 weeks
234
What is the cause of Paget's Disease of the Bone?
Unknown
235
What are the phases involved in Paget's disease?
3 phases:
1) Osteoclast activity - excessive bone resorption
2) Mixed phase of osteoclastic and osteoblastic activity - increased levels of bone turnover
3) Final chronic sclerotic phase with bone formation > resorption
236
What radiographic finds would you see in Paget's disease?
Combinations of lytic bone regions and sclerosis
Osteoporosis circumscripta - well defined osteolytic lesions appearing less dense cf normal bone
Cotton wool appearance (lysis vs sclerosis)
V-shaped defects in long bones
237
Which biochemical marker will be markedly raised in Paget's disease of the bone?
ALP
238
What are the potential complications of Paget's disease of the bone?
Osteosarcoma
Spinal stenosis
Spinal cord compression
239
How may Vasculitis be classified?
Classified based on vessel affected: Large, Medium and Small Vessel
ANCA associated or non-ANCA associated
240
Which ANCA-associated vasculitis is detected by the presence of cANCA?
Mnemonic: Wegener was a classicist
cANCA
241
pANCA is directed against which cellular component?
MPO
242
cANCA is directed against?
PR3
243
What are the clinical features of Henoch-Schonlein Purpura?
```
Purpura
Joint pain
Abdominal pain
Renal involvement (nephritic syndrome)
```
244
What proportion of patients with Henoch-Schonlein Purpura will go on to develop end-stage renal failure within 6 months?
1%
245
What blood test finding may be suggestive of Churg-Strauss syndrome?
Raised mast cells
Raised eosinophils
IgE level elevated
246
What are the clinical features of Wegener's Granulomatosis?
Sinusitis
Recurrent epistaxis
Saddle shaped nose (from perforated nasal septum)
Cough, wheeze and haemoptysis
Glomerulonephritis
247
Which condition is polyarteritis nodosa most associated with?
HBV
248
Which skin rash is most associated with polyarteritis nodosa?
Livedo reticularis
249
What are the clinical features of Kawasaki disease?
```
Persistent high fever >5 days
Strawberry tongue
Erythematous rash
Bilateral conjunctivitis
Erythema and desquamation of skin at solar surfaces
```
250
What is the most concerning complication risk in Kawasaki disease?
Coronary Artery Aneurysms
251
How is Kawasaki's disease managed?
Aspirin
+
IVIG
252
What gene is associated with Behcet's Disease?
HLA B51 gene
253
What are the clinical features of Behcet's disease?
Ulcers: mouth; genital
Skin: Erythema nodosum; Papules and pustules; Vasculitic type rash
Eyes: Anterior/posterior uveitis; Retinal vasculitis; Retinal haemorrhage
MSK: arthralgia; oligoarthritis
GI system: ileum; caecum; ascending colon
CNS: memory impairment; headaches; migraines; aseptic meningitis; meningoencephalitis;
Veins: Budd-Chiari syndrome; DVT; CVS
Lungs: Pulmonary artery aneurysms
254
Which investigation may be used in Behcet's Disease?
Skin pathergy test - sterile needle to create a SC abrasion; review after 24-48 hours to look for a weal 5mm
