Rheumatology Flashcards
(198 cards)
1
Q
What are some risk factors for Osteoarthritis?
A
- Obesity
- Age
- Occupation
- Trauma
- Female
- Family history
2
Q
What is osteoporosis thought to be caused by?
A
Imbalance between the cartilage being worn down and the chondrocytes repairing it
3
Q
What are the 4 key Xray changes for osteoporosis?
A
LOSS
Loss of joint space
Osteophytes
Subarticular sclerosis
Subchondrial cysts
4
Q
How does OA present?
A
Joint pain and stiffness (worsened with activity in contrast to inflammatory arthritis)
Deformity
Instability
5
Q
What are some commonly affected joints for OA?
A
- Hips
- Knees
- Sacro-iliac joints
- DIPJ
- Wrist
- Cervical spine
6
Q
What are some signs in the hands for OA?
A
Herbeden’s nodes (DIP)
Bouchard’s nodes (PIP)
Squaring at base of thumb at carpo-metacarpal joint
Weak grip
Reduced range of motion
7
Q
What are the management options for OA?
A
Lifestyle: weight loss, physio to inprove strength. OT and orthotics
Analgesia: Paracetamol and topical NSAIDs (PPI for protection) or topical capsaicin (chilli pepper extract), THEN opiates e.g. codeine and morphine
Intra-articular steroid injections temporary reduction in inflammation
Joint replacement
8
Q
What is rheumatoid arthritis?
A
Autoimmune condition resulting in chronic inflamamtion of the synovial lining of joints, tenson sheaths and burse
It’s an inflammatory arthritis
9
Q
How is the inflammation in RA distributed?
A
Symmetrical distribution affecting muliple joints
10
Q
What is the genetic association of RA?
A
HLA DR4
11
Q
What is Rheumatoid Factor?
A
Autoantibody targetting the Fc portion of the IgG antibody.
The Fc portion is used to bind to cells of the immune system
12
Q
What is the result of RA?
A
Activation of the immune system against the patients own IgG causing systemic inflammation.
13
Q
What class of immunoglobulin is Rheumatoid Factor?
A
Usually IgM
14
Q
What antibodies are more sensitive and specific to RA than rheumatoid factor?
A
Anti-CCP antibodies
15
Q
How does RA present?
A
Symmetrical distal polyarthropathy: pain, stiffness, swelling
16
Q
What are the key symptoms of RA?
A
Pain
Swelling
Stiffness
17
Q
What are the associated systemis symptoms of RA?
A
Fatigue
Weight loss
Flu like illness
Muscle aches and weakness
18
Q
What is palindromic rheumatism?
A
Self limiting short episodes of inflammatory arthritis with joint pain, stiffness and swelling (only lasting 1-2 days then completely resolving)
Having RF and anti-CCP may indicate progression to full RA
19
Q
Which joints are commonlmy affected by RA?
A
PIP joints, MCP joints (DIPJ almost never affected)
Wrist and ankle
Metatarsophalangeal joints
Cervical spine
Large joints can be affected e.g. knee, hips and shoulders
20
Q
Are the distal IPJ ever affected by RA?
A
No - it will be OA
21
Q
What is atalantoaxial subluxation?
A
The axis and atlas shift caused by local synovitis
Sublaxation can cause spinal cord compression and is an emergency
22
Q
What are the hand symptoms of RA?
A
Z shaped thumb
Swan neck deformity
Boutonnieres deformity
Ulnar deviation of the fingers at knuckle
23
Q
How does Boutonnieres deformity occur?
A
Teat in the central slip of the extensor components of the fingers. This means that when the patient tries to straighten their finger, the lateral tendons that go around the PIP (called the flexor digitorum superficialis tendons) pull on the distal phalynx without any other supporting structure, causing the DIPs to extend and the PIP to flex
24
Q
What are some extra articular manifestations of RA?
A
- Pulmonary fibrosis
- Bronchiolitis obliterans (inflammation causing small airway destruction)
- Felty’s syndrome (RA, neutropenia, splenomegaly)
- Sjogrens syndrome AKA sicca syndrome
- Anaemia of chronic disease
- CVD
- Episcleritis and scleritis
- Rheumatoid nodules
- Amyloidosis
25
What are the investigations for RA?
- Check rheumatoid factor
- If RF negative, check anti-CCP antibodies
- Cehck inflammatory markers e.g. CRP and ESR
- X ray hands and feet
26
What imaging can be used for RA?
**Ultrasound** scan to confirm **synovitis** - useful when clinical examinations are unclear
## Footnote
**Xray**
27
What are the Xray changes in RA?
Joint destruction and deformity
Soft tissue swelling
Periarticular osteopenia
Boney erosions
28
When should a **referral** for **rheumatoid arthritis** be made?
Adult with **persistent synovitis** even if negative RF, anti-CCP antibodies and inflammatory markers
29
What is the DAS28 score?
**Disease activity score** (28 joints are assessed)
Points are given for **swollen joints, tender joints, ESR/CRP results**
Useful in monitoring disease acitivity and response to treatment
30
Which RA patients have a worse prognosis?
- Younger onset
- Male
- More joints and organs affected
- Presence of RF and anti-CCP
- Erosions seen on X-ray
31
What is the 1st line treatment for RA flare ups?
Short course of steroids
NSAIDS/COX 2 inhibitors (with PPI)
32
What is the stepwise progressive use of DMARDs in RA?
**1st:** Methotrexate, leflunomide or sulfasalazine (hydroxychloroquine in mild disease)
**2nd**: Use 2 in combination
**3rd:** Methotrexate plus biologic therapy e.g. TNF alpha inhibitor
**4th:** methotrexate plus **rituximab**
33
What type of drugs are infliximab, adalimumab?
**Anti-TNF** (biologic therapies)
34
What are the biologics worth remembering?
**Adalimumab, infliximab and etanercept** (all TNF inhibitors)
35
Name some other classes of biologic therapies?
Anti-TNF
Anti-CD20
Anti-IL6
JAK inhibitors
36
What are the risks with biologics?
Serious infection
Reactivation of latent TB and hep B
37
How does **methotrexate work**?
**Interferes with metabolism of folate** also suppressing the immune system.
Taken once a week along with 5mg folic acid on diff day to methotrexate
38
What are some side effects of methotrexate?
- Mouth ulcers
- Liver toxicity
- Pulmonary fibrosis
- Bone marrow suppression and leukopenia
- Teratogenic (harmful to pregnancy)
39
How does leflunomide work?
**Immunosuppressant** - interfers with production of **pyrimidine** (component of RNA and DNA)
40
What are some side effects of leflunomide?
- Mouth ulcers and mucositis
- Increased blood pressure
- Rashes
- Peripheral neuropathy
- Teratogenic
- Liver toxicity
- Bone marrow suppression and leukopenia
41
What is sulfasalazine?
Immunosuppressant and anti-inflammatory
42
What are some **side effects of sulfasalazine**?
Temporary male infertility (reduced sperm count)
Bone marrow suppression
43
What is **hydroxychloriquine**?
Traditionally an anti-malarial but also an immunosuppressant **interferes with toll-like receptors** disrupting antigen presentation
Thought to be **safe in pregnancy**
44
What are some **side effects of hydroxychloroquine**?
- Nightmares
- Reduced visual acuity
- Liver toxicity
- **Skin pigmentation**
45
How do anti-TNF drugs work?
Block TNF - a cytokine involved in inflammation
46
What are some anti-TNF drugs?
- Adalimumab
- Infliximab (both monoclonal antibodies)
- Enteracept (protein that binds TNF to Fc portion of IgG)
47
What are some **side effects of anti-TNF drugs**?
- Severe infection and sepsis
- Reactivation of TB anf Hep B
48
What is Rituximab?
Monoclonal antibody that targets CD20 protein on surface of **B cells** (used for immunosuppression in auti-immune conditions and cancers related to B cells)
49
What are the unique side effects of:
**Methotrexate**
**Leflunomide**
**Sulfasalazine**
**Hydroxychloroquine**
**Anti-TNF medications**
Rituximab: Night sweats and thrombocytopenia
**Methotrexate:** pulmonary fibrosis
**Leflunomide:** Hypertension and peripheral neuropathy
**Sulfasalazine:** Male infertility (reduces sperm count)
**Hydroxychloroquine:** Nightmares and reduced visual acuity
**Anti-TNF medications:** Reactivation of TB or hepatitis B
Rituximab: Night sweats and thrombocytopenia
50
What hand joints are typically affected in psoriatic arthritis (inflammatory arthritis)?
**Wrists, DIPs** (MCP NOT AFFECTED UNLIKE RA)
51
What are some **recognised patterns** of **rheumatoid arthritis**?
**Symmetrical polyarthritis** = similarly to rheumatoid arthritis, more common in women. The **hands, wrists, ankles and DIP** joints are affected. The **MCP** joints are **less commonly** **affected (unlike rheumatoid)**.
**Asymmetrical pauciarthritis** affecting **mainly the digits** (**fingers and toes**) and feet. Pauciarthritis describes when the **arthritis only affects a few joints**.
**Spondylitic** pattern is more common in men. It presents with:
* Back stiffness
* Sacroiliitis
* Atlanto-axial joint involvement
**Other areas** can be affected:
* Spine
* Achilles tendon
Plantar fascia
52
How does psoriatic arthritis present?
**Plaques on the skin**
**Pitting** of the nails
**Onycholysis** (separation of nail from nail bed)
**Dactylitis** (inflammation of full finger)
**Enthesitis** (inflammation of the entheses, which are the points of insertion of tendons into bone)
53
What are some other associations of psoriatic arthritis?
Conjunctivitis / anteior uveitis
Aortitis
Amyloidosis
54
What are the **xray changes** for **psoriatic arthritis**?
**Periostitis =** inflammation of the periosteum causing a thickened and irregular outline of the bone
**Ankylosis** = bones joining together causing joint stiffening
**Osteolysis** = destruction of bone
**Dactylitis** = inflammation of the whole digit and appears on the xray as soft tissue swelling
**Pencil-in-cup appearance**
55
What is **arthritis mutilans**?
**Severe form** of psoriatic arthrits in **phalanxes**
Osterlysis of the bone around the joint causing progressive shortening of digit = **telescopic finger**
56
What are the **medication options** for **psoriatic arthritis**?
NSAIDs for pain
DMARDs (methotrexate, leflunomide or sulfasalazine)
Anti-TNF medication (entanercept, infliximab or adalimumab)
**Ustekinumab** is last line (after anti-TNF medications) = monoclonal antibody targetting IL 12 and 23
57
Reactive arthritis causes what ?
What **gene** is it linked to?
Used to be known as **Reiter's syndrome** - synovitis occurs in joint
Acute **monoarthritis** (usually lower limb = leg)
No infection in the joint (unlike sepsis)
Linked to **HLA B27 gene** (part of **seronegative spongyloarthropathies**)
58
What are the most common causes of reactive arthritis?
**Gastroenteritis**
STIs
59
What are the symptoms of reactive arthritis?
Balanitis (dermatitis of head of penis)
Anterior uveititis
Bilateral conjunctivitis
60
What is the management for reactive arthritis?
Give **abx** until septic arthritis is ruled out
Aspirate joint and send for gram staining, cultures, sensitity and **crystal examination**
61
What is the management of reactive arthritis?
NSAIDs
Steroid injections
Systemic steroids may be required
**Recurrent cases** = **DMARDs** or **anti-TNF medications**
62
What are the seronegative spondyloarthropathies?
- Ankylosing spondylitis
- Psoriatic arthritis
- Reactive arthritis
All related to HLA B27
63
What joints are affected in Ankylosing Spondylitis?
**Sacroiliac joints**
**Vertebral column**
64
What is the classic finding on X-Ray for Ank Spondylitis?
Bamboo spine (due to fusion of spine)
65
What is the presenting feature of Ankylosing Spondylitis?
Lower back pain and stiffness
Sacroiliac pain
In 20s usually and affects men: women equally
66
When is the pain from Ankylosing spondylitis worse?
What is a **key complication** of **AS**?
Night and in morning (worse with rest)
## Footnote
**Vertebral fractures**
67
What are some associations of anylosing spondylitis?
**Systemic symptoms** = weight loss, fatigue
**Chest pain** = involvement of costovertebral and costosternal joints
**Enthesitis** (causing **plantar fasciitis** and **achilles tendonitis**)
**Dactylitis**
**Anterior Uveitis**
**Aoritits**
**Heart block** (fibrosis of heart's conductive system)
**Pulmonary fibrosis**
68
How is Schober's test performed?
What indicates a normal ROM?
Patient **stand straight** - find **L5 vertebrae** - mark **10cm** above and **5cm below** (15cm apart - pt bends forwards and assess distance)
**\>20cm** between points on back
69
What **investigations** are there for **Ank Spond**?
**Inflammatory markers (CRP and ESR)** raised
**HLA B27** genetic testing
**X ray** of the spine
**MRI** of spine can show **bone marrow oedema** early in disease before any X-ray changes
70
What are the **x-ray changes** for **Ank Spond**?
- **Bamboo spine**
- **Squaring** of the vertebral bodies
- **Subchondral sclerosis and erosions**
- **Syndesmophytes =** areas of bone growth where the ligaments insert into the bone
- **Ossification** of the ligaments, discs and joints. This is where these structures turn to bone.
- **Fusion** of the facet, sacroiliac and costovertebral joints
71
What is the management of Ank Spond?
**NSAIDs** to help with pain (if not adequately treated afrer 2-4 weeks of max dose then switch to another NSAID)
**Steroids** (oral / IM / joint injections)
**Anti-TNF** (e.g. etanercept or monoclonal antibody against TNF e.g. **infliximab**, **adalimumab** or **certolizumab pegol**)
**Secukinumab** is a **monoclonal antibody** against **IL-17** (recommended if response to NSAIDs and TNF inhibitors is inadequate)
72
What are some additional management steps for patients with ANK SPOND?
- Physio
- Exercise
- Avoid smoking
- Bisphosponates for osteoporosis
- Treat complications
73
What is **systemic lupus erythematosus**?
Inflammatory autoimmune **connective tissue** disorder
(erythematosus refers to typical red **malar rash** across face)
74
What is the disease course of SLE?
Relapsing-remitting
75
What are the leading causes of death in SLE?
Cardiovascular disease
Infection
(**chronic inflammation** cause shortened life expectancy)
76
What antibodies are present in SLE?
**Anti-nuclear antibodies** (antibodies to proteins within persons own cell nucleus)
77
How does SLE present?
- Fatigue
- Weight loss
- Arthralgia
- Myalgia
- Fever
- **Photosensitive malar rash**
- Lymphadenopathy
- SOB
- Pleuritic chest pain
- Mouth ulcers
- Hair loss
- Raynauds
78
What investigations are there for SLE?
**- Autoantibodies**
**- FBC** (anaemia of chronic disease)
**- C3 and C4 levels** (decreased in active disease)
**- CRP and ESR** (raised with active inflammation)
**- Immunoglobulins** (raised due to activation of B cells with inflammation)
**- Urine protein**: creatinine ratio for proteinuria
**- Renal biopsy** for lupus nephritis
79
What autoantibody is SLE associated with?
**Anit-nuclear antibodies** ANA = 85% (also autoimmune hepatitits)
**anti-dsDNA** (very specific to SLE and vary with disease activity)
80
How is SLE diagnosed?
What can occur secondary to SLE
Confirm presence of **Antinuclear anitbodies** and clinical features suggestive of SLE
**Secondary** = **antiphospholipid antibodies** and **antiphospholipid syndrome**
81
What are some **complciations of SLE**?
- **CVD:** hypertension from inflammation of vessels
- **Infection**: secondary to immunosuppressants
- **Anaemia of chronic disease** (affecting bone barrow causing normocytic anaemia) - leukopenia, neutropenia, thrombocytopenia
- **Pericarditis**
- **Pleuritis**
**- Interstitial lung disease**
- **Lupus nephritis**
- **Neuropsychiatry SLE** (due to inflammation of CNS - presents with optic neuritis, transverse myelitis - inflammation of spinal cord or **psychosis**)
**Reccurent miscarriage** (and intrauterine growth restrictiom, pre-eclampsia and pre-term labour)
**VTE** associated with **antiphospholipid** **syndrome** secondary to SLE
82
What are the treatment options for SLE?
**Autoimmune condition** so anti-inflammatories and immunosuppression
- NSAIDs
- Steroids
- Hydroxychloroquine
- Suncream and sun avoidance for the rash
83
What are some other commonly used **immunosuppressants** in SLE?
- Methotrexate
- Mycophenolate mofetil
- Azathioprine
- Tacrolimus
- Leflunomide
- Ciclosporin
84
What **biologic therapy** can be used for SLE?
**Rituximab** - monoclonal antibody targetting **CD20 protein** on surface of **B cells**
**Belimumab** - monoclonal antibody targetting **B-cell activating factor**
85
What is **discoid lupus erythmatosus**?
Non-cancerous chronic skin condition - more common in women - increased risk of developing SLE
86
How does discoid lupus erythromatosus present?
Photosensitive lesions
Scarring alopecia
Lesions = inflammed, erythmatous, patchy, crusty
87
What is the management of discoid lupus erythromatosus?
- Sun protect
- Topical steroids
- Intralesional steroid injection
- Hydroxychloroquine
88
What is the difference between **systemic sclerosis** and **scleroderma**?
They can be used interchangably but there is a local version of **sclerodoma** which only **affect the skin**
89
What causes systemic sclerosis?
What type of disease is systemic sclerosis?
Unsure
## Footnote
**Autoimmune inflammatory and fibrotic connective tissue disorder**
90
What is the mneumonic for **limited cutaneous systemic sclerosis** (as opposed to diffuse cutanrous systemic sclerosis)?
**CREST**
**C**alcinosis
**R**aynaud's phenomenon
o**E**sophageal dysmotility
**S**clerodactyly
**T**elangiectasia
91
What does diffuse cutaneous systemic sclerosis affect?
All features of CREST including
**Cardiovascular problems** HTN and coronary artery disease
**Lung problems**: pulmonacy HTN, pulmonary fibrosis
**Kidney problems**: glomerulonephritis
92
What are the physical manifestations of systemic sclerosis?
**Scleroderma** (hardening of the skin)
**Scterodactyly** (hardening of the hands - tight skin restricts range of motion, can break and ulcerate)
**Telangiectasia** (dilated blood vessels of the skin)
**Calcinosis** (calcium deposits under the skin, most commonly on fingertips)
**Raynaud's** phenomenon
**Oesophageal dysmotility** (connective tissue dysfunction in oesophagus associated with swallowing difficulties)
**Systemic hypertension** (connective tissue dysfunction in systemic and pulmonary arterial systems)
**Pulmonary fibrosis** (presents as gradual dry cough and SoB)
93
What are the autoantibodies associated with systemic sclerosis?
**Antinuclear antibodies** (not specific to SS)
**Anti-centromere antibodies** (associated with limited cutaneous systemic sclerosis)
**Acti-Scl-70 antibodies** (associated with **diffuse cutaneous systemic sclerosis**)
94
How is a diagnosis of systemic sclerosis made?
**- Clinical features**
**- Antibodies**
**- Nailfold capillaroscopy** (avascular areas and micro-haemorrhages indicate SS)
95
What are some non-medical management options for systemic slerosis?
Avoid smoking
Skin stretching to maintain range of motion
Regular emollients
Avoid cold triggers for Raynaud's
Physio
OT for adaptations to daily living
96
What medication can be used for systemic sclerosis?
**Steriods and immunosuppressants** (no standardised and proven treatment)
Nifedipine (for Raynaud's)
Analgesia for joint pain
Pro-motility medications (e.g. metoclopramide) for GI symptoms
Antibiotics for skin infections
Antihypertensives for hypertension (ACEi)
Treat pulmonary artery hypertension
97
What is **polymyalgia rheumatica**?
Inflammatory condition causing pain and stiffness in the shoulders, pelvis and neck.
Strongly linked to **GCA**, both respond well to steroids
98
Who does polymyalgia rheumatic normally affect?
**\>50** y/o caucasian women
99
What are the core features of polymyalgia rheumatica?
Bilateral shoulder pain, **radiating to elbow**
Pelvic girdle pain
Interferes with sleep
Worse with movement
At least 45 min morning stiffness
100
What are some **other features** of **polymyalgia rheumatica**?
Systemic symptoms = weight loss, fatigue, low grade fever and low mood
Upper arm tenderness
Carpel tunnel syndrome
Pitting oedema
101
What are some differentials for shoulder, neck and oelvis stiffness and inflammation?
- OA
- RA
- SLE
- Myositis (from conditions like polymyositis or medications like statins)
- Cervical spondylosis
- Adhesive capsulitis
- Hyper / hypothyroidism
- Osteomalacia
- Fibromyalgia
102
How is polymyalgia rheumatica typically diagnosed?
Clinical presentation and response to steroids (ESR, plasma viscosity and CRP)
103
What **additional investigations** for **polymyalgia rheumatica** before starting steroids to rule out other conditions?
**FBC**
**U&Es**
**LFTs**
**Calcium** raised in hyperparathyroidims / cancer and low in osteomalacia
**Serum protein electrophoresis** for myeloma and other protein disorders
**TSH**
**CK** for myositis
**Rheumatoid factor** for RA
Urine dipstick
**ANA** for SLE
**anti-CCP** for RA
**Urine Bence Jones protein** for myeloma
**CXR** for lung and mediastinal abnormalities
104
What is the **treatment for PMR**?
**Steroids** (expecting an improvement in symptoms)
**Assess 1 week** = if poor response then probably not PMR and stop, look for other diagnosis
**Assess 3-4 weeks** = 70% improvement expected, inflammatory markers normal = PMR (if no improvement then start reducing regime of steroids)
105
What is the osteoporosis protection / gastric protection when on steroids?
Bisphosphonates
Calcium
Vitamin D
PPI
106
What is **GCA**?
**Systemic vasculitis** of the **medium** and **large** arteries (usually affects temporal arteries)
107
What are the symptoms of GCA?
- Severe unilateral headache
- Scalp tenderness
- Jaw claudication
- Blurred vision
- Irreversible painless complete sight loss
108
What are the associated symtoms of GCA?
Fever
Muscle aches
Fatigue
Weight loss
Loss of appetite
Peripheral oedema
109
How is a definitive diagnosis of GCA made?
- Clinical presentation
- Raised ESR
- Temporal artery biopsy findings
110
What is found on the biopsy for GCA?
**Multinucleated giant cells**
111
What do the blood tests show for GCA?
What imaging for GCA?
- **Normocytic anaemia** and thrombocytosis
- **LFTs** raised ALP
- **C reactive protein** is usually raised
**Duplex ultrasound** of temporal artery shows **hypoechoic halo** sign
112
What is the initial management for GCA?
**Steroids** - reduce risk of permanent sight loss (and PPI) - review after 48 hrs
## Footnote
**Aspirin**
113
Who to refer a patient with GCA to?
**Vascular surgeons** (temporal artery biopsy)
**Rheumatology** for diagnosis
**Opthamology** if visual symotoms
114
What are the sick day rules and treatment card for patients on steroids?
Increase dose when sick and take at treatment card with you
115
What are some complications of GCA?
- Vision loss
- Stroke
- Relapse
- Steroid side effects
- **Aortitis** leading to **aortic aneurysm** and **aortic dissection**
116
What are **polymyositis** and **dermatomyositis**?
**Autoimmune disorders** where there is inflammation of the muscles (**myositis**) and or skin (in dermatomyositis)
117
What is the **key investigation** for polymyositis and dermatomyositis?
**Creakine Kinase \> 1000** (caused by myositis)
118
What are some other causes of a raised CK?
Rhabdomyolysis
AKI
MI
Statins
Strenuous exercise
119
What can polymyositis / dermatomyositis be caused by?
Paraneoplastic syndrome from malignancy (lung, breast, ovarian, gastric)
120
What is the presentation of polymyositis?
- **Muscle pain**, **fatigue** and **weakness**
- **Proximal muscles** normally affects
- Shoulder and pelvic girdle
- Develops over **weeks**
**Polymyositis** occurs **without any skin features** whereas **dermatomyositis** is associated with **involvement of skin**
121
What are the **skin features** of **dermatomyositis**?
- **Gotton lesions** (scaly erythomatous patches) on the knuckles, elbows and knees
- **Photosensitive erythmatous rash** on back, shoulders and neck
- Purple rash on face and eyelids
- Periorbital oedema (swelling around eyes)
- Subcutaneous calcinosis (calcium deposits in subcut tissue)
122
What antibodies are seen in polymyositis and dermatomyositis respectively?
**Polymyositis** = anti Jo-1 antibodies
**Dermatomyositis** = anti-Mi-2 antibodies and anti-nuclear antibodies
123
What is the diagnosis of polymyositis basedon ?
- Clinical presentation
- Elevated CK
- Autoantibodies
- Electromyography (EMG)
- **Muscle biopsy**
124
What is the first line medication for polymyositis/dermatomyositis?
**Corticosteroids**
- Immunosuppressants (e.g. azathioprine)
- IV immunoglobulins
- Biological therapy (e.g. infliximab or etanercept)
125
What is antiphospholipid syndrome?
Problem with antiphospholipid antibodies where the blood becomes **prone to clotting**
126
When does antiphospholipid syndrome commonly occur?
**Secondary to SLE**
127
What are the main associations of **antiphospholipid** syndrome?
**Thromobosis**
Pregnancy complications e.g. **recurrent miscarriage**
128
Which **antibodies** as associated with **antiphospholipid syndrome**?
Lupus anticoagulant
Anticardiolipin antibodies
Anti-beta-2 glycoprotein I antibodies
129
What are some associations of antiphospholipid syndrome?
DVT
PE
Stroke
MI
Renal thrombosis
Recurrent miscarriage, stillbirth, preeclampsia
Livedo reticularis (purple rash)
**Libmann-Sacks endocarditis** (type of non-bacterial endocarditis causing **vegetation** on the valves of the heart - **mitral** is commonly affected)
**Thrombocytopenia** (low platelets)
130
How is the **diagnosis** of **antiphospholipid syndrome** made?
**Lupus anticoagulant**
**Anticardiolipin antibodies**
**Anti-beta-2 glycoprotein I antibodies**
131
What is the management of antiphospholipid syndrome?
- Long term **warfarin** aiming for 2-3 (LMWH if pregnant e.g. enoxaparin plus aspirin)
132
What is **Sjogren's syndrome**?
**Autoimmune** condition affecting the **exocrine glands** leading to **dry mucous membranes** e.g. dry mouth, eyes, vagina
133
What is primary sjogren's?
Disease occurs in isolation
134
What is secondary Sjogren's ?
Occurs related to SLE or RA
135
What antibodies is Sjogren's associated with?
Anti-Ro
Anti-La
136
What is the **Schimer's test** for Sjogren's?
Filter paper under eyelid (but in eye), leave for 5 mins and see how far tear travels - should be **15mm** in healthy young adult (less than 10mm is significant)
137
What is the management of Sjogren's ?
Artificial tears
Artifical saliva
Vaginal lubricants
Hydroxychloroquine to halt disease progression
138
What are some complications of Sjogren's?
**Eye infections** = conjunctivits / corneal ulcers
**Oral problems** = dental cavities and candida infections
**Vaginal problems** = candidiasis and sexual dysfunction
Along with:
* Pneumonia and bronciectasis
* NHL
* Peripheral neuropathy
* Vasculitis
* Renal impairment
139
What vasculitis affects the small vessels?
**Henoch-Schonlein purpura**
Eosinophilic Granulomatosis with Polyangiitis (**Churg-Strauss syndrome**)
**Microscopic polyangiitis**
Granulomatosis with polyangiitis (**Wegener’s granulomatosis**)
140
What vasculitis affect the medium sized vessels?
**Polyarteritis nodosa**
**Eosinophilic Granulomatosis with Polyangiitis** (**Churg-Strauss syndrome**)
**Kawasaki disease**
141
Which **vasculitis** affect the **large vessels**?
**Giant cell arteritis**
**Takayasu’s arteritis**
142
What **symptoms** are shared amongst most types of **vasculitis**?
**Purpura.** These are purple-coloured non-blanching spots caused by blood leaking from the vessels under the skin.
**Joint and muscle pain**
**Peripheral neuropathy**
**Gastrointestinal disturbance** (diarrhoea, abdominal pain and bleeding)
**Renal impairment**
**Anterior uveitis and scleritis**
**Hypertension**
143
What are some **systemic manifestations** of **vasculitis**?
**Fatigue**
**Fever**
**Weight loss**
**Anorexia** (loss of appetite)
**Anaemia**
144
What are the tests for Vasculitis?
**Inflammatory markers**: CRP and ESR
**Anti-neutrophil cytoplasmic antibodies** _(ANCA)_
145
What is **p-ANCA** (anti-MPO) associated with?
**Microscopic polyangiitis** and **Churg-Strauss syndrome**
146
What is **c-ANCA** associated with?
**Wegener’s** granulomatosis
147
What is the treatment of **vasculitis**?
**Steroids** (oral e.g. prednisolone, hydrocortisone, nasal spray, inhaled for lung e.g. Churg-Strauss)
**Immunosuppressants:** cyclophosphamide, methotrexate, azathioprine, rituximab
148
What is **Henoch-Schonlein purpura**?
Why does the rash occur?
What is it triggered by?
What are the **classic 4 features**?
What is the **management**?
**IgA vasculitis** presenting with purpuric rash on lower limbs
**Rash** due to **IgA deposits** in blood vessels of affected organs e.g. skin, kidbeys, and GI tract
**Triggered** by **upper airway infection** (e.g. tonsilitis)/ **GI**
Most common in children under 10 y/o
**Features** = purpura, joint pain, abdo pain, renal involvement
**Management** = simple analgesia, rest and proper hydration (benefits of steroids = unclear)
149
How does Eosinophilic Granulomatosis with Polyangiitis present (**aka Churg-Strauss syndrome**)?
How does it present?
What is a characteristic finding?
**Small and medium sized vasculitis** affecting **lungs** and **skin**
Presents with **s****evere asthma** in late teenage ears
Characteristic finding = **elevate eosinophil levels**
150
How does **microscopic polyangiitis** present?
**Small vessel vasculitis** causing **renal failure** also affecting **lungs** causing SoB and haemoptysis
151
How does **granulomatosis with polyangiitis** (**Wegener’s granulomatosis**) present?
**Small vessel vasculits** affecting **resp tract** and **kidneys**
Epistaxis, hearing loss, saddle shaped nose (due to perforated nasal septum),
Cough, wheeze, haemoptysis
**Glomerulonephritis**
152
How does Polyarteritis Nodosa present?
What is it associated with?
**Medium sized vasculitis** associated with **hep B/C** and **HIV**
Affects skin, GI, kidneys and heart causing **renal impairment, strokes** and **MI**
Causes **livedo reticularis** (mottled, purple, lace rash)
153
Where does **Kawasaki** disease affect?
What are the clinical **features**?
What is a **key complication**?
A **medium vessel vasculitis** affecting young children \< 5 years old
**Features** = persistent high fever \> 5 days, erythematous rash, bilateral conjunctivitis, erythema and desquamation (skin peeling) of palms and soles
**Strawberry tongue** (red tongue with prominent papillae)
**Complication** = **coronary artery aneurysms**
**Treatment** = aspirin and IV immunoglobulins
154
How does **Takayasu’s arteritis** present?
How is it **diagnosed**?
**Large vessel vasculitis** mainly **affecting the aorta** and pulmonary arteries (also called **pulseless disease**)
Presents before 40 years with non-specific symptoms e.g. fever, malaise and muscle aches
**Diagnosis** = CT or MRI angiography
**Doppler ultrasound** of carotids can detect carotid disease
155
How does **Behçet’s disease** present?
**Oral and genital ulcers** (also cayses inflammation in skin, GI tract, lungs, blood vessels, MSK, CNS)
156
What gene is linked to Behçet’s disease?
**HLA B51**
157
What are the differentials to mouth ulcers?
**Simple aphthous ulcers**
**Inflammatory bowel disease**
**Coeliac disease**
**Vitamin deficiency** (B12, folate, or iron)
**Herpes simplex ulcers**
**Hands, foot and mouth** disease (coxsackie A virus)
**Squmaous cell carcinoma**
158
What are the **features of Behçet’s disease**?
**Mouth ulcers**
**Genital ulcers** (kissing ulcers on two opposing surfaces)
**Skin inflammation** - erythema nodosum, papules and pustules, vasculitic type rashes
**Eyes** - uveitis, retinal vasculitis, retinal haemorrhages
**MSK system** - morning stiffness, arthralgia, oligoarthritis
**GI system** - inflammation and ulceration affecting ileum, caecum, ascending colon
**Central nervous system** - memory impairment, headaches and migraines, aseptic meningitis, meningoencephalitis
**Veins** - inflammation and vein thrombosis (e.g. Budd Chiari syndrome, DVT, thrombus in pulmonary veins, cerebral venous sinus thrombosis
**Lungs** - pulmonary artery aneurysms can rupture (and be fatal)
159
How is **Behçet’s disease diagnosed**?
**Clinical diagnosis**
**_Pathergy test_** - subcut abrasion is made on arm and examined after for reaction (weal for 5mm or more - testing for **non-specific hypersensitivity** in skin, positive in **Behcet's disease**, **Sweet's syndrome** and **pyoderma gangrenosum**)
160
What is the **management of Behçet’s disease**?
What is the prognosis?
**Topical steroids** for **mouth ulcers** (e.g. **soluble betamethasone**)
Systemic steroids (i.e. oral **prednisolone**)
**Colchicine** usually effective as anti-inflammatory to treat symptoms
Topical anaesthetics for genital ulcers (e.g. **lidocaine** ointment)
Immunosuppressants - **azathioprine**
Biologics e.g. **infliximab**
**Prognosis** = relapsing-remitting with normal life expectancy
161
What are the most commonly affected joints in the hand by gout?
What causes it?
What are **gouty tophi**?
How does gout present?
DIP joints
Chronically **high uric acid** (urate crystals are deposited in the joint)
**Gouty tophi** are subcut deposits of uric acid
**Presents** = single acute, hot, swollen and painful joint (differential = septic arthritis)
162
What are some risk factors for gout?
- Male
- Obesity
- High purine diet (e.g. meat and seafood)
- Alcohol
- Diuretics
- FH
163
Which joints are affected by gout?
1st metatarsalphalangeal joint (base of big toe)
Wrists
1st carpometacarpal joint (base of thumb)
Can affect large joints
164
How is gout diagnosed?
Aspiration of fluid from joint
165
What will aspirated fluid from gout show?
- No bacterial growth
- **Needle shaped** crystals
- **Negative birefringent** of polarised light
- **Monosodium urate crystals**
166
What does a joint Xray show for gout?
**Maintained** joint space
**Lytic lesions** in the bone
**Punched out erosions** with sclerotic borders
167
What is used to treat gout?
When is colchicine used? Whats a side effect of this?
Acute = NSAIDs, Colchicine, steroids
Used when **NSAIDs not appropriate** e.g. renal impairment / significant heart disease
Side effect = **diarrhoea**
168
What is used as prophylaxis against gout?
Allopurinol (xanthine oxidase inhibitor)
169
What lifestyle changes are there to reduce risk of gout?
**Losing weight, staying hydrated, less alcohol**
**Less purine based foods** (meat and seafoods)
170
What joint is commonly affected in pseudogout?
What is it?
Knee
**Crystal arthropathy** caused by **calcium pyrophosphate crystals** aka **chondrocalcinosis**
171
What will aspirated fliud show from pseudogout?
- No bacterial
- **Calcium pyrophosphate** crystals
- **Rhomboid shaped**
- **Postivie birefringent** of polarised light
172
What is the **classic xray change in pseudogout**?
**Chondrocalcinosis** - thin white line in middle of joint space (**pathognomonic**)
173
What is the treatment of pseudogout?
NSAIDs, colchicine, joint aspiration, steroid injections, joint washout (if severe)
174
What are some risk factors for osteoporosis?
- Older age
- Female
- Reduced mobility and activity
- Low BMI
- Rheumatoid arthritis
- Alcohol and smoking
- **Long term corticosteroids**
175
Why does osteoporosis occur in post menopausal women?
**Oestrogen** is **protective** against osteoporosis
176
What tool gives the risk of a fragility fracture over the next 10 years in osteoporosis?
FRAX tool (using age, BMI, smoking, alcohol, FH)
177
What is bone mineral density measured using?
What score indicates bone density?
**DEXA scan (xrays)**
**T score at hip** is the most important (density compared to a healthy young adult)
**Z score** (compare to mean for age) or **T score** (compared to mean for healthy young adult - this is **most clinically important outcome)**
178
What are the lifestyle changes that a patient with osteoporosis can do?
Activity and exercise
Maintain a health weight
Adequate calcium intake
Adequate vitamin D
Avoiding falls
Stop smoking
Reduce alcohol consumption
179
What are some **medical options** for **osteoporosis**?
**Calcium and vitamin D** (colecalciferol)
**Bisphosphonates** (reduce activity of osteoclasts)
180
What are the key side effects to bisphosphonates?
**Reflux and oesophagel erosions** (take on empty stomach, sitting up 30 mins before any food)
Osteonecrosis of the jaw
Osteonecrosis of the external auditory canal
181
What are some example of some bisphosphonates?
Alendronate
Risedronate
Zoledronic acid
182
What are some **other medical options** for **osteoporosis**?
**Denosumab** = monoclonal antibody that blocks osteoclasts
**Strontium ranelate** = similar element to calcium that stimulates osteoblasts and blocks osteoclasts but increases risk of DVT, PE and MI
**Raloxifene**
**HRT** if menopause early
183
What is osteomalacia? Why does it happen?
What does it cause? What is it called in children?
Soft bone due to **defective bone mineralisation** as a result of low Vit D
Weak bone, bone pain, muscle weakness and fractures
Causes **rickets**
184
What is **vitamin D**?
A **hormone** created from cholesterol by the skin in response to UV radiation
185
Which patients are more likely to have vitamin D deficiency?
Patients with **malabsorption conditions** IBD
Kidneys are needed to make active form therefore vit D deficinency is common in **CKD**
186
What is vitamin D needed for?
Calcium and phosphate absorption from the intestines and kidneys, also for **regulating bone turnover**
187
How does parathyroid hormone interfere with problems due to low vitamin D?
Low calcium causes more PTH released which stimulates more resporption from the bones causing further mineralisation problems
188
What is the typical presentation of a patient with osteomalacia?
- Fatigue
- Bone pain
- Muscle weakness
- Muscle aches
- Pathological fractures
189
What is the **investigation** for **osteomalaci****a**?
**Serum-25-hydroxyvitamin D**
190
What are the other findings for osteomalacia?
Low serum calcium
Low serum phosphate
High ALP
High PTH
Xray may show **osteopenia** (more **radiolucent bones**)
**DEXA** show low bone mineral density
191
What is the treatment of osteomalacia?
**Colecalciferol**
192
What is Paget's disease?
**Disorder of bone turnover** - excessive - due to **overactive** osteoblasts and osteoclasts
Causing **enlarged** and **misshapen bones** with increased risk of **pathological fractures**
193
How does **Paget's disease present**?
Bone pain
Bone deformity
Fractures
Hearing loss if affecting bones of ears
194
What are the **xray findings for Paget's disease**?
Bone enlargement and deformity
**Osteoporosis circumscripta =** well defined osteolytic lesions that appear less dense with normal bone
**Cotton wool appearance** of the skull describes patchy areas of **increased density and decreased density**
**V-shaped defects** in **long bones**
195
What is the only lab abnormality in Pagets?
Raised ALP (normal calcium and phosphate)
196
What is the management for Paget's disease?
**Bisphosphonates**
NSAIDs for bone pain
(**Calcium and Vit D supplementation** particularily for bisphosphonates)
**Monitor serum ALP**
197
What are two complications of Paget's disease?
**Osteosarcoma** - bone cancer - presents with focal bones pain and swelling
**Spinal stenosis** - deformity causes spinal canal narrowing - diagnosed with MRI treated with bisphosphonates
198
