Rheumatology 2 Flashcards
(104 cards)
1
Q
What is ankylosing spondylitis?
A
Seronegative spondyloarthropathy
Chronic autoimmune inflammatory disease of the axial skeleton
2
Q
What is the typical onset for AS?
A
20s-30s
3
Q
Give four risk factors for AS?
A
HLA-B27 positivity
Male gender
FH of AS or psoriasis
Recent GU infection
4
Q
Give three aspects of the pathogenesis of AS.
A
Lymphocyte and plasma cell infiltration
Local bone erosion and enthesitis
Syndesmophytes and bamboo spine
5
Q
What is the typical presentation of AS?
A
Pain before the age of 35 (lower back, buttocks, anterior chest)Morning stiffness relieved by exercise
Can wake patient from night
Asymptomatic between episodes
6
Q
What is the characteristic of peripheral joint involvement in AS?
A
Asymmetrical
7
Q
Give three signs of AS.
A
Question mark posture - thoracic kyphosis with retention of lumbar lordosis
reduced chest expansion
Paraspinal muscle wasting
Schober’s test<5cm
8
Q
What are the 4As of other manifestations of AS?
A
Achilles tendonopathy Anterior uveitis Apical lung fibrosis Aortic regurgitation Atlanto-axial subluxation
9
Q
What is the initial investigation in AS and what is found?
A
X-Ray
Sacroilitis, sclerosis and fusion
10
Q
Why is MRI used in AS?
A
Shows sacroiliitis and enthesitis better
11
Q
What medications are used in AS?
A
Adalimumab SC
Certolizumab pegol SC
Etanercept SC
Infliximab IV
12
Q
What is the name of the heterogenous group of heritable connective tissue disorders?
A
Ehlers-Danlos Syndrome
13
Q
How many subtypes of EDS are there?
A
13
14
Q
What are the most important types of EDS?
A
- Classical
- Classical-like
- Cardiac-valvular
- Vascular
- Hypermobile (50%)
- Kyphoscoliotic
15
Q
What are the skin symptoms of EDS?
A
Increased skin elasticity and fragility
Easy bruising
Widening scars
Skin splitting on forehead, knees, and elbows
16
Q
What are the join symptoms of EDS?
A
Laxity and hypermobility
Spontaneous dislocations and subluxations - shoulder, knee, TMJ
Pes planus
17
Q
Give some other symptoms of the various types of EDS.
A
Cardio - dizziness, palpitations, dysautonomia
MSK - herniation, hypotonia, kyphoscoliosis
Other - uterocervical prolapse, fatiguability
18
Q
How is EDS diagnosed?
A
Hypermobile type - clinical
Other types - molecular genetic testing
19
Q
What is the management of EDS?
A
Physiotherapy
Pain management and CBT
Certain forms use celiprolol.
20
Q
What is celiprolol?
A
Beta-1 adrenoreceptor antagonist
Prevents arterial dissections and ruptures
21
Q
What is the mutation in Marfan’s syndrome?
A
Fibrillin 1 gene
Found on chromosome 15q21
2/3 familial transmission, 1/3 sporadic mutations associated with advanced paternal age
22
Q
What are signs of Marfan’s syndrome?
A
Dolichostenomelia (long arms and legs compared to trunk)
Tall and thin
Arachnodactyly
Thoracolumbar and sacral striae
23
Q
What are patients with Marfan’s at risk of?
A
Pleural rupture and pneumothorax
Cardio - AAA/dissection/aortic or mitral valve problems
Eyes - Lens dislocation, closed angle glaucoma
Hypermobility and arthralgia
24
Q
How are Marfan’s patients monitored?
A
Annual echo
Cardiac MRI/CT every 5 years
25
Apart from managing the complications, how are Marfan's patients treated?
Prophylactic beta-blockers
26
What is the name of the inflammatory condition characterized by severe bilateral pain and morning stiffness of the shoulder, neck, and pelvic girdle?
Polymyalgia rheumatica
27
What is the main risk factor for PMR?
Giant cell arteritis
28
What is the prodrome of PMR?
Flu-like
| Sudden, over 1-2 weeks
29
What is the diagnostic criteria for PMR?
1) Age over 50
2) Symptoms > 2 weeks
3) Bilateral shoulder and/or pelvic girdle aching WITHOUT WEAKNESS
4) Morning stiffness > 45m
5) Raised ESR/CRP
30
What bloods are required in diagnosis of PMR and exclusion of other conditions?
```
ESR, CRP, viscosity
FBC, UEs, LFTs, TFTs, Bone profile
Protein electrophoresis
Creatinine kinase
RF
ANA and anti-CCP
```
31
What other investigations are required in PMR?
Urinalysis and Bence Jones
CXR
USS shoulders/hips
32
What is the treatment of PMR?
PMR is extremely sensitive to steroids.
| Steroid regimen - PO prednisolone gradually decreasing over 1-2 years
33
What should be done if a PMR patient is high risk for fragility fractures?
Perform DEXA
| Give bisphosphonates with calcium and Vit D supplements
34
What is myositis?
CTD involving inflammation of striatal muscle
35
What are the types of polymyositis?
Dermatomyositis
Polymyositis
Necrotizing autoimmune myopathy
Sporadic inclusion body myositis
36
What are the main symptoms of myositis?
Diffuse proximal weakness
Fatigue and myalgia
Distal muscles spared until late in disease
37
How does diffuse proximal weakness present?
Difficulty rising from chair
Climbing steps
Lifting objects
Combing hair
38
What can myositis progress to?
Pharyngeal weakness - dysphagia
Laryngeal weakness - dysphonia
Respiratory failure and lung fibrosis
39
What are the signs of dermatomyositis, excluding the muscle symptoms?
Heliotrope discolouration of eyelids and periorbital oedema
Gottron's papules
Ulcerative vasculitis
Photosensitive macular rash in shawl pattern
Scales and shininess
40
What do lab tests show in myositis?
Elevated CK, aldolase, LDH
PM: anti-Jo1
DM: ANA, anti-Mi2
41
How is myositis diagnosed?
EMG - spontaneous fibrillation potentials at rest
Muscle biopsy - fibre necrosis and inflammatory infiltrate
MRI - inflammation
42
What should be excluded in elderly patients with dermatomyositis?
Malignancy
43
What is the treatment of myositis or dermatomyositis?
High dose systemic steroids
Immune suppression - azathioprine, cyclophosphamide, mycophenolate mofetil
IVIG
44
What is pseudogout?
Joint inflammation caused by deposition of calcium pyrophosphate dihydrate crystals
45
Attacks of pseudogout can be precipitated by....
Dehydration
Intercurrent illness
Dysregulation of calcium-phosphate homeostasis
Surgery or trauma
46
How does pseudogout present?
Acute monoarticular/oligoarticular arthritis
Typically knees or wrists
Similar but milder than gout
There may be fever
47
How does Chronic CPP arthritis present?
Destructive changes more severe than OA
| Neuropathic joint
48
How is pseudogout present?
X-Ray - linear opacification of articular cartilage
| Joint aspiration - purulent, neutrophils, crystals.
49
What are the characteristics of joint crystals in pseudogout?
Intracellular and extracellular weakly positive birefringent rhomboid crystals.
50
How is pseudogout managed?
Symptomatic - ice packs, rest, therapeutic aspiration, NSAIDs, colchicine
51
What percentage of psoriasis patients develop a seronegative spondyloarthropathy?
5-25%
52
Apart from an inflammatory arthritis, how does psoriatic arthritis present?
```
Dactylitis
Enthesitis
Nail changes
Anterior uveitis
Sacroilitis
Aortitis
```
53
What are the different types of psoriatic arthritis?
- Symmetrical polyarthritis rheumatoid pattern (DIPs>MCPs)
- Oligoarthritis
- Lone DIP disease
- Arthritis mutilans
- Spondylitic pattern and sacroilitis
54
How is psoriatic arthritis diagnosed?
Increased ESR/CRP
HLA-B27 and IgA
Joint aspiration shows neutrophils
X-Ray for erosions
55
How is psoriatic arthritis managed?
Methotrexate address both skin and joint disease
| 2nd line: sulfasalazine, leflunomide
56
Why should hydroxychloroquine be avoided in psoriatic arthritis?
May cause an exfoliative dermatitis
57
What biologics should be considered in psoriatic arthritis?
TNF inhibitors
| After inadequate response to one DMARD, or patients with axial disease
58
What are the subgroups of reactive arthritis?
Post-enteric - campylobacter, salmonella, shigella, yersinia
| Post-venereal - HIV or chlamydia
59
How long after infection does reactive arthritis develop?
Minimum of 1 and maximum of 6 weeks
60
What is the typical arthritis in reactive arthritis?
Asymmetrical, predominantly lower extremity oligoarthritis
61
What are the other symptoms of reactive arthritis?
```
Malaise and fever
Reiter's syndrome
Nail changes
Mouth ulcers
Erythema nodosum/circinate balanitis
```
62
What is seen on bloods in reactive arthritis?
High CRP and ESR
HLA-B27 pos, RF and ANA neg
Normochromic normocytic anaemia, leucocytosis and thrombocytosis
63
What does joint aspiration show in reactive arthritis?
Polymorphonuclear leukocytes
64
What are important investigations in reactive arthritis?
Culture of stools, throat, and urogenital tract samples
65
What is the management of reactive arthritis?
Corticosteroids (IA or PO)
Abx
Limited use for sulfasalazine
66
What is the pathophysiology of systemic sclerosis?
Increased fibroblast activity resulting in the abnormal growth of connective tissue and collagen, causing vascular damage and fibrosis
67
What are the types of scleroderma?
Limited cutaneous systemic sclerosis
Diffuse cutaneous systemic sclerosis
Overlap systemic sclerosis
Systemic sclerosis sine scleroderma
68
What is the difference between limited and diffuse cutaneous systemic sclerosis?
Limited - skin affected only on face, forearms, and lower leg. Milder disease and slow progression.
Diffuse - skin also involves upper arms, thighs, or trunk. Rapid progression and high mortality
Both have symptoms included in CREST acronym
69
What does CREST stand for?
```
Calcinosis
Raynaud's
Esophageal dysmotility
Sclerodactyly
Telangiectasia
```
70
What are five signs of scleroderma?
```
Sclerodactyly
Fingertip pitting and ulceration
Calcinosis
Microstomia
Telangiectasia
Joint pain
```
71
What are the pulmonary features of scleroderma?
80% pulmonary fibrosis
| Pulmonary arterial hypertension is the leading cause of death
72
What are the GI features of scleroderma?
GORD
Watermelon stomach and GI bleed from gastric antral vascular ectasia
Reduced small bowel motility
73
What autoantibodies are associated with scleroderma?
Anti-topoisomerase 1 - diffuse
Anti-centromere - limited
Anti-RNA polymerase
ANA
74
What is the main management of scleroderma?
Immunotherapy - MTX, MMF, CYC
75
How is the following managed in scleroderma?
1) Raynaud's
2) Pulmonary fibrosis
3) PAH
4) Renal crisis
5) Contractures/calcinosis
1) Nifedipine
2) CYC or MMF infusion
3) Bosentan, ambrisentan, sildenafil
4) ACEIs plus dialysis
5) Surgical procedures, aluminium hydroxide
76
What is scleroderma associated with?
Breast, lung, and haematological malignancies
| Sjogrens syndrome
77
What are five risk factors for septic arthritis?
```
Increasing age
Diabetes mellitus
Prior joint damage e.g. RA
Joint surgery
Immunodeficiency
```
78
What are the features of gonococcal septic arthritis?
Fever
Arthralgia
Multiple skin lesions
Tenosynovitis
79
What are the symptoms of septic arthritis?
Single swollen, red, hot joint
Severe pain on active or passive movement
Fever and rigors
80
How is septic arthritis diagnosed?
Blood cultures
Increased CRP and WCC
Synovial fluid: leukocytes, gram staining and culture, polarising microscopy
-X-ray: osteomyelitis, fat pad displacement, swelling of capsule and soft tissue, joint space widening due to effusion
81
What is the treatment of septic arthritis?
Surgical drainage
| High dose IV abx that cover s.aureus and strep A
82
What is Sjogren's syndrome?
Chronic lymphocytic infiltration of exocrine glands
83
What are two risk factors for Sjogren's syndrome?
HLA B8 or DR3
| Vitamin D deficiency
84
How does Sjogren's syndrome present?
```
Xerophthalmia
Xerostemia
Recurrent bilateral parotitis
Dry cough and dysphagia
Fatigue
```
85
How is Sjogren's syndrome diagnosed?
RF and ANA, anti-Ro and anti-La
| Schirmer test <5mm (normal - 15mm)
86
How is Sjogren's syndrome managed?
Artificial tears and saliva replacement
Avoid anti-cholinergics
Pilocarpine tablets
87
What are the two main aspects of pathophysiology of vasculitis?
Vessel wall destruction leading to aneurysm or rupture
| Stenosis leading to tissue ischaemia and necrosis
88
Name the large and medium vessel vasculitides?
Large: GCA, Takayasu's
Medium: PAN, Kawasaki
89
Name the ANCA-associated vasculitides.
Granulomatosis with polyangiitis
Eosinophilic granulomatosis with polyangiitis
Microscopic polyangiitis
90
Apart from those associated with ANCA, name three other small vessel vasculitides.
Cryoglobulinaemic vasculitis
Henoch-Schonlein Purpura (IgA vasculitis)
Hypocomplementaemic urticarial vasculitis
91
What are the two forms of ANCA and which conditions are they found in?
Cytoplasmic (c-ANCA) - GPA
| Perinuclear (p-ANCA) - MPA and EGPA
92
Name five presentations of a large vessel vasculitis.
```
Arm claudication
Diminished pulses
Jaw claudication
Aortic arch aneurysm
Visual loss
```
93
Which arteries does Takayasu arteritis affect?
Aorta and great vessels
94
How can Takayasu arteritis present?
Systemic upset including arthralgia and myalgia
Arm claudication and loss of pulses
Hypertension from renal artery stenosis
Stroke and visual change
95
How is Takayasu arteritis diagnosed?
F-FDG PET-CT scan
96
How is Takayasu arteritis and PAN managed?
PO prednisolone
| MTX/AZA/MMF if steroid resistant
97
What is polyarteritis nodosa?
Necrotizing vasculitis of medium sized arteries
98
What is a risk factor for PAN?
Hepatitis B
99
How does PAN present?
```
Systemic upset
Vasculitis rash and livedo reticularis, bullous and vesicular lesions
GI haemorrhage
Mononeuritis multiplex
Kidney and pancreas
```
100
What is GPA?
Necrotizing granulomatous inflammation usually involving the upper and lower respiratory tract.
101
How does GPA present?
```
Sinusitis, epistaxis, crusts, septal perforation, saddle nose, gingivitis
Haemoptysis
Glomerulonephritis and haematuria
Vasculitic skin change
MNM
```
102
How is GPA diagnosed?
Lung/nose biopsy
c-ANCA, CRP, ESR
CXR and high resolution CT
103
What is the treatment of GPA?
CYC
AZA maintenance
RTX relapse
104
What is the triad of features of Still's disease?
Persistent high spiking fevers
Joint pain
Salmon coloured bumpy rash
