Rheumatology Flashcards
Using the mnemonic CHIMP, name the drugs that can cause Drug Induced Lupus
Carbamazepine/Chlorpromazine Hydralazine Isoniazid/Infliximab Minocycline Penicillamine/Procainamide/Pyrazinamide
Name the two ‘special tests’ for Carpal Tunnel Syndrome
Tinnel’s test
Phalen’s test
Give two rheumatological causes of Neutropenia
SLE DMARD Toxicity (neutropenic sepsis)
Name three inflammatory markers and what they indicate
ESR - Erythrocyte Sedimentation Rate (reflects presence of fibrinogen and immunoglobulins)
PV - Plasma Viscocity (same as above)
CRP - Short lived protein good for monitoring progression
Give 4 subtypes of ANA and what they’re raised in
Anti DsDNA - SLE
Anti RO - Sjogren’s
Anti Centromere - Systemic Sclerosis
Anti Jo-1 - Polymyositis
What is ANCA?
Anti Neutrophil Cytoplasmic Antibody
Against enzymes in neutrophils
What is HLA - B27?
Surface antigen associated with Ankylosing Spondylitis, Iritis and Juvenile Arthritis
What is Polymyositis?
Inflammation of striated muscle
How does Polymyositis present?
Insiduous Onset
(Painless) Proximal Muscle Weakness
Raynauds
Dysphagia/Dysphonia/Resp Weakness
How does Dermatomyositis present?
Muscle and Skin signs
Skin Signs being Helitrope Rash (lilac rash around eyes), Shawl Sign (macular rash), Nail Fold Infarcts
Describe the 5 Diagnostic Criteria of (Dermato)Myositis, and how many is required for each
Symmetrical Proximal Muscle Weakness
Raised serum muscle enzyme levels (CK,ALT,AST)
Electromyograph showing fibrillatory changes
Biopsy evidence
PM requires 3
DM requires 2 + Skin Changes
Name 4 investigations you could do for Polymyositis and what they would show
Bloods - Raised ALT (normal LFTs)
Bloods - Raised ANA - Anti Jo1
Electromyograph - Fibrillatory Changes
Muscle MRI - Muscle Oedema
How is Polymyositis managed?
Initial - High dose Prednisolone
Followed by DMARDs if resistant
How is Dermatomyositis managed long term?
Hydroxychloroquine and sun protection
What is Fibromyalgia?
Disorder of central pain processing associated with Allodynia (painful response to non painful stimulus), and Hyperaesthesia (exaggerated response to mildly painful stimulus)
Describe three different aetiological ideas for Fibromyalgia
Sleep Deprivation - Hyperactivation in response to painful stimuli
Patient CSF - Increased Substance P and decreased NA and Serotonin
PET Scan - Abnormal central dopamine response to pain
Give 5 presentations of Fibromyalgia
Widespread Pain Unrefreshed Sleep Migraines Depression Joint/Muscle Stiffness
Fibromyalgia is purely a clinical diagnosis, how could you manage it?
Physio
CBT
Low dose Amitryptyline/Pregabalin
What is Giant Cell Arteritis?
Chronic vasculitis in large and medium sized vessels occurring in over 50s
Why do you get Jaw Claudication in GCA?
Inflammation of arteries supplying the muscles of mastication
Give 5 presentations of GCA
Headache (unilateral over temple) Scalp Tenderness Jaw Claudication Amaurosis Fugax Malaise
Name three investigations you could do for GCA. What would they show?
Bloods - Raised ESR and CRP
Temporal Artery Biopsy - Necrotising arteritis (skip lesions)
Doppler - Decreased pulsation of temporal artery
Name three risk factors for GCA
Age (>60)
Female
White
How would you manage GCA?
1) Prednisolone 60-100mg for 2 weeks before tapering (if visual loss then use IV Methylprednisolone for 3 days)
2) Low Dose Asparin (for VTE risk)
What is Gout?
Accumulation of Monosodium Urate crystals in joints and soft tissues causes an inflammatory arthritis
Give two long term manifestations of Gout
Urate Nephropathy
Uric Acid Nephrolithiasis
Give 2 modifiable and 2 non modifiable risk factors of Gout
Non Mod - Age>40, Male
Mod - Increased Alcohol, Thiazide Diuretics
What would a synovial fluid sample show of Gout?
Yellow and Turbid
Polarised Light Microscopy - Negatively bifringent needles
What would an Xray of Gout show?
Early stage swelling
Late Erosions in juxta-articular bone
Preserved joint space
Give 3 pharmacological options to TREAT Gout
NSAIDs (Diclofenac, Naproxen)
Steroids
Colchicine (good when NSAIDs CI)
Give 2 pharmacological options to PREVENT Gout. What should you tell the patient?
Allopurinol and Febuxostat (XANTHINE OXIDASE INHIBITORS)
Do not start the drugs in an acute attack
Cover with NSAIDs once started as they may precipitate an attack
Give 4 contraindications to the use of NSAIDs
BARS B- Bleeding A - Asthma R - Renal Dysfunction S - Stomach (Peptic Ulcer or Gastritis)
What is the pathophysiology of Pseudogout?
Calcium Pyrophosphate crystals
Positive bifringent rods
What is Hypermobility Spectrum Disorder?
Pain syndrome in people with joints that move beyond normal limits
Give 5 presentations of Hypermobility Spectrum Disorder
Pain around joints (worse after activity) Hernias Drooping Eyelids Recurrent dislocations Marfans/EDS
What gene is defective in Marfans?
Fibrillin
Describe 3 aetiologies of OA
- Failure of normal cartilage due to prolonged abnormal or excessive loading
- Damaged cartilage failing under normal conditions
- Cartilage breaks up due to defective bone putting on more weight
Describe the cartilage of OA on a microscopic level
Decreased elasticity
Decreased cellularity
Decreased tensile strength
The pain of OA cannot originate from the cartilage itself as it is avascular and aneural, therefore where does it originate from?
Microfractures of bone
Low grade synovitis
Capsular distension
Muscle spasm
Give 4 presentations of OA
Pain and Crepitus on movement
Joint Gelling
Background Ache at rest
Joint Swelling
Describe the XRay findings of OA
Loss of joint space
Osteophytes
Subchondral Sclerosis
Subchondral Cysts
Give 2 conservative and 2 pharmacological managements of OA
Conservative - Movement and Strengthening exercises, Walking stick
Management - Paracetamol, Topical NSAIDs and Capsaicin
What is Osteoporosis?
Low bone mass, deterioration of bone tissue and disruption of bone architecture leading to reduces bone strength and increased fracture risk
What are the two types of Osteoporosis?
1 - Post menopausal women, oestrogen withdrawal increases osteoclast activity
2 - Increased age reduces osteoblast function
Using the mnemonic SHATTERED, what are the risk factors for Osteoporosis?
Steroids Hyperthyroidism Alcohol and Tobacco Thin Testosterone Early Menopause Renal Failure Erosive Bone Disease (Myeloma) Dietary Malabsorption
Suspected Osteoporosis patients are given a DEXA scan, to produce a T and Z score, what are they?
T score - Number of standard deviations from the mean bone density of a healthy person. above -1 SD is normal
between -1 and -2.5 SD is defined as mildly reduced bone mineral density (BMD) compared with peak bone mass (PBM)
at or below -2.5 SD is defined as osteoporosis
Z Score - comparing your DEXA with someone of the same age, less than -2 SD indicates Osteoporosis
Give 3 conservative managements of Osteoporosis
Quit smoking
Increase weight bearing exercises
Calcium and Vit D rich diet
What is the first pharmacological choice for managing Osteoporosis? What should you advise the patient with this medication?
Oral Bisphosphonates such as Alendronic Acid
Stay upright and wait 30 mins before food after taking
Possible SE include Jaw Osteonecrosis and GI Upset
Other than Bisphosphonates, describe two other pharmacological options for Osteoporosis
Teriparatide - Recombinant PTH
Denosumab - MAB decreasing bone reabsorption given subcut biannually
What is Polymyalgia Rheumatica?
Idiopathic inflammatory condition characterised by severe bilateral pain and morning stiffness of shoulder/neck/pelvic girdle
What other rhumatological condition does PMR commonly co-exist with?
50% association with GCA
Give 3 symptoms of PMR
Subacute (<2wks) proximal limb pain and stiffness
Difficulty rising from chair/combing hair
Night time pain
What is the difference in presentation between PMR and Polymyositis?
PMR - No weakness, just pain
Polymyositis - Generally no pain, just weakness
What blood parameters would be raised in PMR?
Raised CRP, ESR and PV
How would you manage PMR?
15mg Prednisolone daily before tapering, adjusting to symptoms (dramatic response within 5d)
What is Raynaud’s Phenomenon?
Painful vasospasm of digits resulting in colour changes
What is the difference between Raynaud’s Syndrome and Raynaud’s Phenomenon/Disease?
Syndrome - Idiopathic, Symmetrical and Bilateral
Disease - Asymmetrical and lasts longer
What are the three colour changes in Raynauds?
Pale - Ischaemia
Blue - Deoxygenation
Red - Hyperaemia
Describe Primary and Secondary Raynauds. How can you differentiate between?
Primary - young female and generally no underlying disease
Secondary - more severe pain, older, co-morbidities
Differentiated by raised ESR in secondary only
Give 5 triggers of Raynauds
Exposure to cold Medications (Beta Blockers) Vasculitis Heavy Vibrating Tools Diseases (SLE, Scleroderma, Sjogren's)
Give two conservative and two pharmacological treatments for Raynauds
Conservative - Stop smoking, avoid the cold
Pharmacological - Topical GTN, CCBS (Nifedipine)
Give two complications of Raynauds
Digital Ulcers
Digital Ischaemia
In four steps, describe the pathophysiology of RA
1) Self antigens become citrullinated and recognised by B and T cells which subsequently release RF and Anti CCP
2) Macrophages and Fibroblasts become activated to release TNFalpha
3) Infammatory cascade activated
4) Synoviocytes proliferate, growing over articular cartilage, restricting nutrients and damagin cartilage
Give 3 common presentations of RA
Pain worsened by movement
Morning stiffness>30 mins
Fatigue
Give 5 late signs of RA
Ulnar Deviation Z Thumb Rheumatoid Nodules (on elbows) Carpal Tunnel Swan Neck (DIPJ) and Boutonnieres (PIPJ)
Using the mnemonic CAPS, describe some extra-articular manifestations of Rheumatoid Arthritis
C- Carpal Tunnel, CVD
A - Anaemia, Amyloidosis, Arteritis
P- Pericarditis, Pleural Disease
S - Sjogren, Scleritis, Splenomegaly (Feltys)
XRays can be used in later stages of RA, what would you see?
L - Loss of Joint Space
E - Erosions
S - Swelling
S - Sublaxation
Name a scoring system used for RA
ABCD
Joint Involvement, Serology, Acute Phase Reactants, Duration of Symptoms
When would you give Biologics in RA? Give 3 examples and their targets
When you have trialled atleast 2 DMARDs
Infliximab (TNFalpha)
Rituximab (CD20)
Tocilizumab (IL6)
What is Sjogren’s Syndrome?
Autoimmune inflammatory disorder characterised by diminished lacrimal and salivary gland secretion (lymphocytic infiltration and fibrosis of exocrine glands)
What cancer does Sjogren’s Syndrome increase your risk of?
Non Hodgekin’s Lymphoma
Using the mnemonic MADFRED, describe the signs and symptoms of Sjogrens
Myalgia Arthralgia Dry Mouth Fatigue Raynauds Enlarged Parotids Dry Mouth
Name three investigations for Sjogren’s, and what a positive result would show.
Blood tests - Anti RO antibodies
Schirmer’s Test - Reduced tear volume
Salivary gland biopsy - Lymphocytic infiltration
Management of Sjogren’s is normally conservative, give 3 examples
Avoid dry/smoky atmospheres Artificial tears (hypromellose) Artificial saliva
What is the risk with Anti RO antibodies in pregnancy?
Can cross placenta and cause heart block/foetal loss/neonatal lupus
What are Spondyloarthropathies?
Group of conditions affecting spine and peripheral joints associated with HLA - B27
Includes: Ankylosing Spondylitis, Psoriatic Arthritis, Reactive Arthritis, Enteric Arthritis
What is Ankylosing Spondylitis?
Chronic inflammation of spine and sacroiliac joint
What is the ‘typical’ clinical picture of Ankylosing Spondylitis?
Man less than 30y/o
Progressive back pain
Worse at night with morning stiffness, improving throughout the day
Reduced spinal movement and chest expansion
What happens to the curves of the back with Ankylosing Spondylitis?
Loss of lumbar lordosis
Increasing thoracic kyphosis
Extreme cases - Q mark
Using the 5A’s mnemonic, name the extra articular features of Ankylosing Spondylitis
Anterior Uveitis Aortic Incompetence AV Block Apical Lung Fibrosis Amyloidosis
Name two clinical tests and two radiological investigations for Ankylosing Spondylitis
Schober’s Test - mark 10cm above and 5cm below the PSIS and ask the patient to bend forward. In a normal patient this distance should increase to atleast 20cm
Forced external hip rotation - pain
MRI - Bone marrow oedema
XRay - Bamboo Spine
What is the mainstay of treatment for Ankylosing Spondylitis?
NSAIDs and Physio
What are the conditions for starting biologic therapy in Ankylosing Spondylitis?
Had to have tried 2 NSAIDs, for 6 weeks each
VAS>4 (Visual Analogue Scale)
BASDAI>4 (Bath Ankylosing Spondylitis Disease Activity Index)
Describe the presentation of Psoriatic Arthritis
Oligoarthritis with Dactylitis (Sausage FIngers)
Can be symmetrical
What would imaging of Psoriatic Arthritis show in it’s early and late stages?
Early - joint erosions (USS or MRI)
Late - Pencil in a cup (XRay)
What is Reactive Arthritis?
Arthritis occurs as a result of infection elsewhere in the body (eg post dysentry or post chlamydia)
Describe the clinical presentation of Reactive Arthritis
Occurs 2d-2w post infection
Involves conjunctivitis and urethritis (cant see cant pee cant bend at the knee)
How would you manage Reactive Arthritis?
Treat underlying infection
NSAIDs and joint infections
If not resolved in 2 years then consider DMARDs
Describe the two types of Enteropathic Arthritis
1 - Oligoarticular, Asymmetric, Responds to flares
2 - Polyarticular, Symmetric, Doesn’t respond to flares
How would you manage Enteropathic Arthritis?
Avoid NSAIDs (as they may precipitate an IBD flare) Use Infliximab (as it can treat both the IBD and the Arthritis)
Using the mnemonic IPAIN, describe the 5 classical features of inflammatory back pain
Insiduous Onset Pain at night Age of onset<40 Improved by exercise No improvement with rest
Describe the pathophysiology of SLE
Inadequate supression of T cells, and excessive B cell activity (along with production of antibodies against certain nuclear components)
Using the mnemonic SOAP BRAIN describe the signs and symptoms of SLE
Serositis, Oral Ulcers, Arthritis, Photosensitivity Blood disorders (lymphopenia), Renal involvement (glomerulonephritis), Autoantibodies, Immunulogic tests (low C3 and C4), Neurologic Disorders (seizures, psychosis)
What 3 investigations would you do with a blood sample for SLE?
Anti DsDNA titres (1:1600 - have to dilute 1600 times before undectable)
Low C3 and C4
Raised ESR and PV
Why is sun protection especially important in SLE?
UV alters the DNA making it more immunogenic
What medication would be used for the rash and arthralgia in SLE?
Hydroxychloroquine
Why is SLE more common in Women?
Oestrogen promotes autoimmunity by prolonging life of autoreactive B and T cells
Describe the pathophysiology of Systemic Sclerosis (AKA Scleroderma)
Increased fibroblast activity resulting in abnormal CT growth, fibrosis and vascular damage
90% ANA positive
Describe the presentation of LIMITED Scleroderma
CREST Calcinosis Cutis Raynauds Phenomenon Esophageal Dysmotility Sclerodactyly Telangiectasia
Pulmonary HTN
Describe the presentation of DIFFUSE Scleroderma
Sudden onset skin involvement proximal to elbows and knees
What would you expect to find when testing the serum of a Systemic Sclerosis patient?
Normal inflammatory markers
+ve Anti Centromere antibodies in Limited
How would you manage Systemic Sclerosis?
No cure - Psychological support
Nifedipine - Raynauds
Skin thickening - Methotrexate and Mycophenolate
What is a Morphea?
Localised Scleroderma
State the four subtypes of small vessel vasculitis and their respective markers
Granulomatosis with Polyangitis (c-ANCA) Microscopic Polyangitis (p-ANCA) Eosinophilic Granulomatosis (p-ANCA) IgA Vasculitis (IgA)
State two subtypes of medium vessel vasculitis
Polyarteritis Nodosa
Kawasaki
Describe two subtypes of large vessel vasculitis
GCA
Takayasu Arteritis
Describe four skin changes you might see in Vasculitis
Palpable Purpura
Digital Ulcers
Gangrene
Nail Bed Capillary Changes
Some ocular changes you might see in Vascultiis include: Scleritis, Uveitis and Episcleritis. How would these present?
Scleritis - Pain and photophobia
Uveitis - Photophobia
Episcleritis - Red and watery
What might be mistaken for vasculitis?
Atrial Myxoma (benign tumour in atria)
Describe the first and second line treatment of Vasculitis
1) Corticosteroids
2) DMARDs
Name 4 Seronegative Spondyloarthropathies, using the mnemonic PEAR
Psoriatic Arthritis
Enteropathic Arthritis
Ankylosing Spondylitis
Reactive Arthritis
What is Polyarteritis Nodosa?
Necrotising vascularise affecting small and medium size vessels
Not associated with ANCA
What is Polyarteritis Nodosa associated with?
Hep B
How would Polyarteritis Nodosa present?
Mononeuritis Multiplex
Purpura/Subcut nodules/ Ulcers
Post Prandial Abdo Pain (MI)
How is Polyarteritis Nodosa treated?
Steroids +/- Azathioprine or Cyclophosphamide