Rheumatology Flashcards
What antibodies is SLE characterised by?
Anti-nuclear antibodies
(alsoAnti-double stranded DNA - specific to SLE)
What are the symptoms of SLE:
Hair loss
Photosensitive malar rash
SOB
Splenomegaly
Joint pain
Lymphadenopathy
Myalgia
Fever
Weight loss
Fatigue
What is the leading cause of death in SLE?
Cardiovascular disease (chronic inflammation in the blood vessels leads to hypertension)
What are the first line treatments for SLE?
NSAIDS
Steroids
Hydroxychloroquine
What is discoid lupus erythematosus?
Non cancerous chronic skin condition, associated with an increased risk of developing systemic lupus erythematosus
DLE risk factors
Female
Young
Dark skin
Smoker
What is the treatment for DLE?
Topical steroids
Intralesional steroid injections
Hydroxychloroquine
Suncream
What is systemic sclerosis
autoimmune inflammatory and fibrotic connective tissue disease
What are the features of limited cutaneous systemic sclerosis (i.e. CREST)?
C - calcinosis
R - Raynaud’s
E - oesophageal dysmotility
S - sclerodactyly
T- telagiectasia
What is the difference between limited and diffuse cutaneous systemic sclerosis?
Diffuse also affects the CVS system, lungs and kidneys
What antibodies are most associated with systemic sclerosis?
ANA
Anti-centromere antibodies - limited cutaneous
Anti-Scl-70 antibodies - diffuse cutaneous
What is nail fold capillaroscopy
Looks at the health of the peripheral capillaries - used to exclude systemic sclerosis in patients with Raynaud’s
What is poly myalgia rheumatica strongly associated with?
Giant cell arteritis
What are the main features in PMR?
Shoulder girdle pain and stiffness
Neck Pain
Pelvic girdle pain and stiffness
What is the key investigation for diagnosing polymyositis?
Creatine Kinase, should be under 300 but in polymyositis it is often in the 1000s
What are the causes of a raised creatine kinase?
Rhabdomyolysis
AKI
MI
Statins
Strenuous exercise
What malignancy can cause polymyositis?
Lung, breast, ovarian, gastric
What are the symptoms of polymyositis?
Muscle pain
Fatigue
Weakness
Bilateral, proximal muscles (pelvic and shoulder girdle)
Develops over weeks
What are the features of dermatomyositis?
Gottron lesions (scaly erythematous patches) on the knuckles, elbows and knees
Photosensitive erythematous rash on the back, shoulders and neck
Purple rash on the face and eyelids
Periorbital oedema
Subcutaneous calcinosis
What antibodies are associated with poly and dermatomyositis?
Anti-Jo-1
Anti-Mi-2
Anti-nuclear
How do you diagnose polymyositis?
Creatine kinase, autoantibodies, clinical presentation, EMG
Definitive: Muscle biopsy
What is the complication of anti-phospholipid syndrome?
Hyper-coagulable state -> thrombosis
Recurrent miscarriage
What antibodies are associated with APS?
Lupus anticoagulant
Anticardiolipid antibodies
Anti-beta-2 glycoprotein I antibodies
What is Lived reticular?
Purple lace like rash in APS
What is Libmann-Sacks endocarditis?
Non-bacterial endocarditis
Associated with SLE and APS
What is the management for APS?
Long term warfarin, INR range of 2-3
Pregnant women: LMWH plus aspirin (NOT WARFARIN)
What is Behçet’s disease?
Recurrent oral and genital ulcers
Complex inflammatory condition: can affect lots of areas
What gene is Behçet’s linked to?
HLA B51
How can Behçets affect the eyes?
Anterior or posterior uveitis
Retinal vasculitis
Retinal haemorrhage
How can Behçet’s affect the MSK system?
Morning stiffness
Arthralgia
Oligoarthritis (swelling without joint destruction)
How can Behçet’s affect the CNS?
Memory impairment
Headaches and migraines
Aseptic meningitis
Meningoencephalitis
What is the key investigation in Behçet’s?
Pathergy test
Sterile needle creates a subcutaenous abrasion on the forearm
Review in 24-48 hours - any weal greater than 5mm?
If positive and skin is hypersensitive: it is positive in Behçet’s, Sweet’s syndrome and pyoderma gangrenosum
What is the management for Behçet’s?
Steroids (topical or systemic)
Colchicine
Topical anaesthetics
Immunosuppressants such as azathioprine
Biologic therapy such as infliximab
What crystals form in pseudo gout?
Calcium pyrophosphate crystals
What is seen in aspirated fluid in pseudo gout?
Calcium pyrophosphate crystals
Rhomboid shaped crystals
Positive birefringent of polarised light
What are the joint X-ray changes in pseudo gout? (LOSS)
Loss of joint space
Osteophytes
Subarticular sclerosis
Subchondral cysts
What is Paget’s disease of the bone?
Excessive bone turnover due to excessive activity of both osteoclasts and osteoblasts
This is uncoordinated, so: patchy areas of high density (sclerosis) and low density (lysis)
Enlarged and misshapen bones with structural problems
What bones does Paget’s particularly affect?
Axial skeleton
How does Paget’s disease of the bone present?
Bone pain
Bone deformity
Fractures
Hearing loss can occur if it affects the bones of the ear
What is osteoporosis circumscripta?
Well defined osteolytic lesions that appear less dense compared with normal bone
What does cotton wool appearance of the skull imply?
Paget’s disease of the bone: poorly defined patchy areas of sclerosis and lysis
What are V-shaped defects in the long bones suggestive of?
Paget’s disease of the bone: V-shaped osteolytic bone lesions within healthy bone
What do you see on blood tests in Paget’s disease of the bone?
Raised alkaline phosphatase
Normal calcium
Normal phosphate
How do you manage Paget’s disease of the bone?
Bisphosphonates
What are the two main complications of Paget’s disease of the bone?
Osteogenic sarcoma
Spinal stenosis and spinal cord compression
What are the four key x-ray changes in osteoarthritis?
LOSS mnemonic:
Loss of joint space
Osteophytes
Subarticular sclerosis
Subchondral cysts
What are the main signs of osteoarthritis?
Heberden’s nodes
Bouchard’s nodes
Squaring at CMC joint
Weak grip
Reduced range of motion
What type of joint is the carpometacarpal joint?
saddle joint
What are the symptoms of osteoarthritis?
Pain and stiffness in classic joints, worse on activity and no morning stiffness
What is the common gene associated with rheumatoid arthritis?
HLA DR4
What are the antibodies in RA and which is more sensitive?
Rheumatoid factor and Anti-cyclic citrullinated peptide ( anti-CCP)
What hand signs can you see in patients with advanced rheumatoid arthritis?
Z-shaped deformity to the thumb
Swan next deformity (hyperextended PIP and flexed DIP)
Boutonniere deformity (hyperextended DIP and flexed PIP)
Ulnar deviation of the fingers at the MCP joints
What is Felty’s syndrome?
Triad of rheumatoid arthritis, neutropenia, splenomegaly
What x-ray changes can you see in RA?
Periarticular osteopenia
Bony erosions
Soft tissue swelling
Joint destruction and deformity (in more advanced disease)
What do DMARDs stand for?
Disease-modifying anti-rheumatic drugs
What is the treatment algorithm for RA?
- Monotherapy (e.g. methotrexate., leflunomide or sulfasalazine)
- Combination treatment with multiple cDMARDs
- Biologic therapies (usually alongside methotrexate)
What are the safest DMARDs in pregnancy?
Hydroxychloroquine (v mild) and sulfasalazine, but they should take extra folic acid.
What is the target of infliximab, etanercept, golimumab, certolizumab, and adalimumab?
TNFi: Tumour necrosis factor inhibitors
What is the target of rituximab?
Anti-CD20 on B cells
What is tumour necrosis factor?
A cytokine involved instimulating inflammation
What are some of the side effects of methotrexate and leflunomide?
Mouth ulcers
liver toxicity
Bone marrow suppression
teratogenic
What are some of the side effects of sulfasalazine?
Orange urine
Reversible male infertility
Bone marrow suppression
What are some of the side effects of hydroxychloroquine?
Retinal toxicity
Blue-grey skin pigmentation
Hair lightening
How does hydroxychloroquine work?
Interferes with Toll-like receptors, disrupting antigen presentation, increasing pH in the lysosomes of immune cells
What is a side effect of anti-TNF medications?
Reactivation of TB
What is a side effect of rituximab?
Night sweats and thrombocytopaenia
What are some of the hand signs in psoriatic arthritis?
Nail pitting
Onycholysis
Dactylitis
What xray changes are seen in psoriatic arthritis?
periostitis
ankylosis
Osteolysis
Dactylitis
What gene is reactive arthritis linked to?
HLA B27
What is the rhyme which is helpful to remember the symptoms of reactive arthritis?
“Can’t see, pee or climb a tree” (conjunctivitis, urethritis, arthritis)
What are the 5 As associated conditions in ankylosing spondylitis?
Anterior uveitis
Aortic regurgitation
Atrioventricular block
Apical lung fibrosis
Anaemia of chronic disease
What is Schober’s test?
Test for spinal mobility (like in osce)
Standing straight, a point is marked 10cm above and 5cm below L5 vertebra. When bending over if this 15cm distance doesn’t increase to 20cm, this supports diagnosis of ankylosing spondylitis
What is the typical x-ray finding in ankylosing spondylitis?
Bamboo spine
Also:
squaring of the vertebral bodies
subchondral sclerosis and erosions
syndesmophtes
ossification fo the ligaments
fusion of the facet, sacroiliac and costovertebral joints
What is the treatment algorithm for ankylosing spondylitis?
- NSAIDS
- Anti-TNF
What is scleroderma?
Hardening of the skin
What are the main two patterns of disease in systemic sclerosis?
Limited cutaneous systemic sclerosis
Diffuse cutaneous systemic sclerosis
What are the features of limited cutaneous systemic sclerosis?
CREST syndrome:
Calcinosis
Raynoaud’s
oEsophageal dysmotility
Sclerodactyly
Telangietasia
What is the difference between limited and diffuse cutaneous systemic sclerosis?
Diffuse also affects CVS, lung, kidneys
What is the technique to decide whether Raynaud’s phenomenon is fine or caused by systemic sclerosis?
Nailfold capillaroscopy: magnify and examine the peripheral capillaries. Abnormal capillaries, avascular areas and micro-haemorrhages suggest systemic sclerosis
What medication can be used for Raynauds?
Calcium channel blockers, particularly nifedipine
What medications can worsen the symptoms of Raynauds?
Beta-blockers
What antibodies are positive in most patients with systemic sclerosis?
ANA (anti-nuclear antibodies)
What antibodes are most associated with limited cutaneous systemic sclerosis?
Anti-centromere antibodies
What antibodes are most associated with diffuse cutaneous systemic sclerosis?
Anti-Scl-70 antibodies
What are the characteristic features of polymyalgia rheumatica?
-Pain and stiffness:
worse in the morning
worse after rest
interfere with sleep
45 mins to ease in morning
Also: Systemic symptoms (e.g., weight loss, fatigue and low-grade fever)
Muscle tenderness
Carpel tunnel syndrome
Peripheral oedema
What is the treatment for polymyalgia rheumatica?
Low dose steroids for 1-2 years: 15mg prednisolone
What is the Don’t STOP mnemonic?
Don’t- don’t stop after 3 weeks of treatment: risks adrenal crisis
S- Sick day rules (increase dose)
T - treatment card to alert
O- osteoporosis prevention
P - PPI
What is the treatment for GCA?
40-60 mg prednisolone OD if no visual symptoms or jaw claudication
500mg-1000mg methylprednisolone daily if any visual symptoms of jaw claudication
Once controlled, steroid dose slowly weaned over 1-2 years
What are the characteristic skin changes in dermatomyositis?
Gottron papules on back of hands
Heliotrope rash affecting eyelids
What is the critical test for myositis?
Creatine kinase (in the 1000s for myositis)
What is the common antibody associated with polymyositis?
anti-Jo-1 antibodies
What are the specific antiphospholipid antibodies?
Lupus anticoagulant
Anticardiolipin antibodies
Anti-beta-2 glycoprotein I antibodies
What are the key complications of antiphospholipid syndrome?
VTE
Arterial thrombosis (stroke, MI, renal thrombosis)
Pregnancy-related complications (e.g. recurrent miscarriage, stillbirth and pre-eclampsia)
What is used to treat antiphospholipid syndrome?
Warfarin (or LMWH in pregnancy)
What are the main antibodies in Sjögren’s?
Anti-SS-A (also called anti-Ro)
Anti-SS-B (also called anti-La)
What is the test for Sjogren’s?
The Schirmer test involves inserting folded filter paper under the lower eyelid with the end hanging out. Moisture from the eye will travel by diffusion along the filter paper. After 5 minutes, the distance that the moisture travels along the filter paper is measured. In a healthy young adult, 15mm is expected. Less than 10mm is significant.
What medication can be used to stimulate tear and saliva production in Sjogren’s?
Pilocarpine - stimulates muscarinic receptors, stimulating the parasympathetic nerves
What antibodies are linked to microscopic polyangiitis?
p-ANCA
What antibodies are linked to granulomatosis with polyangiitis?
c-ANCA
Which organs can polyangiitis affect?
Lungs and kidneys
What are the four classic features in HSP?
Pupura
Joint pain
Abdominal pain
Renal involvement (IgA nephritis)
Inflammation occurs due to IgA deposits in the blood vessels
What symptoms in the upper respiratory tract can granulomatosis with polyangiitis cause?
Nose: epistaxis
Ears: hearing loss
sinuses: sinusitis
What is eosinophilic granulomatosis with polyangiitis?
Primarily affects lungs and skin but also kidneys
Presents with severe asthma in middle age.
Raised eosinophils
What is Kawasaki disease?
medium vessel vasculitis
Symptoms:
Persistent high fever for more than 5 days
KWidespread erythematous maculopapular rash
Desqamation of palms and soles
Bilateral conjunctivitis
Strawberry tongue
What is the key complication of kawasaki disease?
Coronary artery aneurysms
What is the treatment for kawasaki disease?
Aspirin
IV immunoglobulins
What does aspirated joint fluid show in gout?
Monosodium urate crystals which are needle-shaped and negatively birefringent of polarised light
What does aspirated joint fluid show in pseudogout?
Calcium pyrophosphate crystals which are rhomboid shaped and positively birefringent
What are the x-ray changes in gout?
Joint space maintained
lytic lesions
punched out erosions
sclerotic borders with overhanging edges
When is prophylaxis started in gout?
Several weeks after the acute attack has resolved. However, once initiated, it is continued through any other acute attacks
What type of drug is allopurinol?
Xanthine oxidase inhibitors
What are the risk factors for gout?
Male
FHx
Obesity
High purine diet
Alcohol
Diuretics
CVS disease
Kidney disease
What are the joint x-ray changes in pseudogout?
Chondrocalcinosis (calcium deposits in the joint cartilage)
also LOSS mnemonic
What is osteomalacia?
Defective bone mineralisation - soft bones
(adult rickets)
How is Vitamin D made?
Created from cholesterol in the skin in response to UV radiation, also in food.
Kidneys convert it into its active form.
What are Looser zones in osteomalacia?
Fragility fractures that go partially through the bone
What is the treatment for osteomalacia?
Colecalciferol:
either:
50,000 IU once weekly for 6 weeks
or
4000 IU daily for 10 weeks
Thgen maintenance dose of 800-2000 IU daily
What is Paget’s disease of bone?
Excessive bone turnover, which is uncoordinated, leading to patchy areas of sclerosis and lysis
This results in enlarged and misshapen bones, structural problems and increased risk of pathological fractures.
What bones does Paget’s disease of the bone particularly affect?
Axial skeleton
What can patients present with in Paget’s disease of the bone?
Bone pain
Bone deformity
Fractures
Hearing loss
What x-ray findings are seen in Paget’s disease of the bone?
Bone enlargement and deformity
Osteoporosis circumscripta (well defined osteolytic lesions that appear less dense compared with normal bone)
Cotton wool appearance of the skull
V-shaped osteolytic defects in the long bone
What is raised on blood tests in Paget’s disease of the bone?
Raised alkaline phophatase
What is the main treatment of Paget’s disease of the bone?
Bisphosphonates
What are the key complications of Paget’s disease of the bone?
Hearing loss (if it affects the bones in the ear)
Heart failure (due to hypervascularity of abnormal bone)
Osteosarcoma
spinal stenosis and spinal cord compression
