RHEUMATOLOGY Flashcards
What is rheumatoid arthritis?
An autoimmune systemic inflammatory disorder affecting mainly the joints of the hand but also associated with extra-articular features. Symmetrical deforming polyarthritis
What are the HLA associations of Rh A?
HLA DR1
HLA DR4
What is the epidemiology of Rh A?
Females
30-50yo
What is the PC of RhA?
Gradual joint pain, swelling
Worst in the mornings
Impairs function and movement
+ systemic symptoms: weight loss, fever, fatigue.
Early signs on OE for RhA
SYMMETRICAL swelling of joints
Warm + Tender joints
MCP, PIP commonly affected
Decrease in range of movement
Late signs on OE for RhA
SYMMETRICAL deformed arhtopathy Ulnar deviation of finger Radial deviation of wrist "swan neck" "boutoinnere" "z" of thumb Rheumatoid nodules - hard, firm SC nodules found commonly on elbows and palms (associated with RhF)
What investigations do you carry out for RhA?
RhF + Anti-CCP + (500 SBA book says this is the most sensitive specific investigation for RhA) ANA + Bloods: low HB, high Plt High ESR + CRP
Joint aspiration to exclude septic arthritis.
What do you expect to see on Xray for RhA
“DONS”
Deformity
Osteopenia
Narrowing of joint space
Swelling of soft tissues
Which joints are usually spared on new onset RhA?
DIP
500 SBA
Define psoriasis
• A chronic inflammatory skin disease, which has characteristic lesions and may be complicated by arthritis
Recognise ALL the signs/symptoms of psoriasis
- Itching and occasionally tender skin
- Pinpoint bleeding with removing scales (Auspitz phenomenon)
- Skin lesions may develop at sites of trauma/scars (Koebner phenomenon)
- Discoid/Nummular psoriasis - symmetrical, well-demarcated erythematous plaques with silvery scales over extensor surfaces (knee, elbows, scalp, sacrum)
- Flexural psoriasis - less scaly plaques in axilla, groins, perianal and genital skin
- Guttate psoriasis - small drop-like lesions over trunk and limbs
- Palmoplantar psoriasis - erythematous plaques with pustules on palms and soles
- Generalised pustular psoriasis - pustules distributed over limbs and torso
Nail signs:
o Pitting
o Onycholysis
o Subungual hyperkeratosis
Signs of psoriatic arthritis
o Asymmetrical oligoarthritis o Symmetrical polyarthritis o Distal interphalangeal joint predominance o Arthritis mutilans o Psoriatic spondylitis
Mostly affects DIP and is assymetrical (5% of psoriasis pts will develop psoriatic arthritis) - from 500 SBA
Define osteoarthritis
• Age-related degenerative joint disease when cartilage destruction exceeds repair, causing pain and disability
Recognise the presenting symptoms of osteoarthritis
- Joint pain and discomfort - Use-related *
- Stiffness or gelling after inactivity
- Difficulty with certain movements *
- Feelings of instability
- Restriction walking, climbing stairs and doing manual tasks
• Systemic features are usually absent
Recognise the signs of osteoarthritis on physical examination
• Local joint tenderness
• Bony swellings along joint margins
o Heberden’s Nodes - DISTAL interphalangeal joint
o Bouchard’s Nodes - PROXIMAL interphalangeal joint
• Crepitus and pain during joint movement
• Joint effusion
• Restriction of range of joint movement
What are the classic OA signs on joint xray?
FOUR classic features: o Loss of joint space (narrowing) o Osteophytes o Subchondral cysts o Subchondral sclerosis
Explain the aetiology/risk factors of infective arthritis
- May be idiopathic
- In most cases there is RECENT systemic infection ** allowing for haematogenous spread
• Risk Factors o Recent orthopaedic procedures o Osteomyelitis o Diabetes o Immunosuppression o Alcoholism
Common causative organisms of septic arthritis
Neisseria gonorrhoeae
o Viruses • Rubella • Mumps • Hepatitis B • Parvovirus B19
o Fungi
• Candida
Recognise the presenting symptoms of infective arthritis
• Fever
• Excruciating joint pain
• Joint redness, swelling and loss of joint function
• Usually a monoarthropathy (usually affecting one large joint)
o NOTE: it may cause a polyarthropathy in the immunosuppressed
• Tuberculous arthritis develops more slowly and is more chronic
Recognise the signs of infective arthritis on physical examination
- Painful, hot, swollen
- Immobile joint
- Erythema
- Severe pain prevents passive movement
- Pyrexia
- Look for signs of aetiology
Identify appropriate investigations for septic arthritis
• Joint Aspiration (IMPORTANT)
o In infective arthritis, the aspirate will be grossly purulent **
o Send synovial fluid for MC&S
• Microscopy - rule out crystal arthritis
o PCR may be used if a viral cause is suspected
• Bloods o FBC - high WCC, high neutrophils o High CRP and ESR o Blood cultures M MC&S o Viral serology may be useful
• Plain Joint Radiographs
o Affected joint may look normal initially
o Can show signs of damage following the infection
• MRI Scan
o Useful for detecting osteomyelitis
Define reactive arthritis
• Characterised by a sterile arthritis occurring after an extra-articular infection (commonly GI or urogenital).
What is Reiter’s syndrome
Reiter’s Syndrome is defined as a TRIAD of:
o Reactive arthritis
o Urethritis
o Conjunctivitis
Explain the aetiology/risk factors for reactive arthritis
• Associated with infections: o GI • Salmonella • Shigella • Yersinia • Campylobacter
o Urogenital
• Chlamydia trachomatis (60%)*
• It is thought that initial activation of the immune system by a microbial antigen is followed by an autoimmune reaction that involves the skin, eyes and joints
Which HLA is associated with Reactive arthritis
• HLA-B27 allele is identified in 70-80% of patients
Recognise the presenting symptoms of reactive arthritis
- Symptoms can develop 3-30 days after infection
- Burning or stinging when passing urine (due to urethritis)
- Arthritis
- Low back pain (due to sacroiliitis)
- Painful heels (due to enthesitis and plantar fasciitis)
- Conjunctivitis
Recognise the signs of reactive arthritis on physical examination
• Signs of Arthritis
o Asymmetrical oligoarthritis
o Often affects the lower extremities
o SausageMshaped digits
• Signs of Conjunctivitis
o Anterior uveitis M painful red eye
• Oral Ulceration
• Circinate Balanitis
o Scaling red patches on the glans
• Keratoderma Blenorrhagica o BrownishMred macules o Vesicopustules o Yellowish-brown scales o Found on the SOLES and PALMS
• Others
o Nail dystrophy
o Hyperkeratosis
o Onycholysis
Identify appropriate investigations for reactive arthritis
• Bloods
o FBC
o High ESR and CRP
o HLA-B27 testing
• Stool or Urethral Swabs and Cultures
o May be negative by the time the arthritis develops (because the arthritis occurs post-infection)
• Urine
o Screen for Chlamydia trachomatis
• Plain X-Rays
o Useful in chronic cases
o Erosions seen at the entheses (insertion of tendons into bone)
• Joint Aspiration
o To exclude septic or crystal arthritis
Which crystals deposit in the joints in pseudogout?
Calcium pyrophosphate dihydrate
near the surface of chondrocytes
What diseases are RF for gout?
Haemochromatosis, Wilson’s, Hyperparathyroidism, Acromegaly, Hypophosphotemia, Hypomagnesemia
(you would expect to find pseudgout in a young patient if they have one of these diseases)
In which demographic group is pseudogout mostly found?
Elderly females (x2)
How does pseudogout present?
Acute painful swollen joint knee/wrist (ankle, shoulder)
What are the signs OE of pseudogout
Red, hot, swollen, tender, resticted range of movement
What investigations do you carry out for pseudogout?
Bloods: raised WCC, raised ESR, (Mg2+, PTH, Pho)
Blood and joint aspirate culture: to exclude septic arthritis
Joint aspirate -
Xray
What would the joint aspirate under polarised light
Positive birefringence - rhomboid/brick shaped crystals
What is a key feature on Xray for pseudogout?
CHONDROCALCINOSIS !!!
It will differentiate it from other things like: gout, OA, septic arhritis as there is some overlap in presentation and signs between them.
Define ankylosing spondylitis
• Seronegative inflammatory arthropathy affecting preferentially the axial skeletal and large proximal joints
Which joints are mainly affected in AS?
axial skeletal and large proximal joints
What is the HLA associated with AS?
HLA-B27
What’s the pathophysioogy o
o Inflammation starts at the ENTHESES* (where ligaments attach to vertebral bodies)
o Persistent inflammation leads to reactive new bone formation*
o Changes begin in the lumbar vertebrae and progress superiorly
o Vertebral bodies become more square
o Syndesmophytes* (vertical ossifications bridging the
margins between adjacent vertebrae)
o Fusion of syndesmophytes and facet joints
o Calcification of anterior and lateral spinal ligaments
What is the classic demographic of an AS pt?
Young male
Recognise the presenting symptoms of ankylosing spondylitis
• Lower back and sacroiliac pain **
• Pain pattern
o Worse in the morning **
o Better with activity **
o Worse when resting **
- Disturbed sleep
- Progressive loss of spinal movement
- Symptoms of asymmetrical peripheral arthritis
- Pleuritic chest pain (due to costovertebral joint involvement)
- Heel pain (due to plantar fasciitis)
- NonMspecific symptoms (e.g. malaise, fatigue)
Recognise the signs of ankylosing spondylitis on physical examination
- Reduced range of spinal movement (particularly hip rotation)
- Reduced lateral spinal flexion
- Schober’s Test
- Tenderness over the sacroiliac joints
What is Schober’s test
o Two fingers are placed on the patients back about 10 cm apart
o The patient is asked to bend over
o The distance between the two fingers should increase by > 5 cm on forward
flexion
o Reduced movement would suggest ankylosing spondylitis - AS is a clinical diagnosis !!!!
What are the signs OE for later stages of AS?
• LATER STAGES:
o Thoracic kyphosis
o Spinal fusion
o Question mark posture
What are the signs of exrta-articular disease of AS?
• Signs of Extra-Articular Disease: 5 As o Anterior uveitis o Apical lung fibrosis o Achilles tendinitis o Amyloidosis o Aortic regurgitation
Identify appropriate investigations for ankylosing spondylitis
• Bloods
o FBC - anaemia of chronic disease
o Rheumatoid factor - negative *
o ESR/CRP - high *
• Radiographs
o Anteroposterior and lateral radiographs of the spine
• May show Bamboo spine*
o Anteroposterior radiograph of sacroiliac joints
• Shows symmetrical blurring of joint margins
o LATER STAGES on XRAY:
• Erosions
• Sclerosis
• Sacroiliac joint fusion
o CXR - check for apical lung fibrosis
• Lung Function Tests
o Assess mechanical ventilatory impairment due to kyphosis
Generate a management plan for giant cell arteritis
- High dose oral prednisolone IMMEDIATELY to prevent visual loss
- Reduce the dose of prednisolone gradually
- Many patients will need to be kept on a maintenance dose of prednisolone for 1-2 yrs
- Low dose aspirin (with PPIs and gastroprotection) - reduces risk of visual loss, TIAs and stroke
- Annual CXR for up to 10 yrs to look for thoracic aortic aneurysms
What is polymyositis ?
Connective tissue disease characterised by inflammation of muscles
What causes polymyositis?
Autoimmune
Possible viral triggerQ
What is the epidemiology of polymyositis?
30-60yo Female
How does one present with polymyositis
Progressive muscle weaknes gradually getting worst over the past few months
(NO RASH)
How does a dermatomyositis rash look like?
• Blue-purple discolouration of the upper eyelids with periorbital oedema
• Flat red rash involving the face and upper trunk
• Raised purple-red scaly patches over the extensor surfaces of joints and fingers
o Rash may affect knees, shoulders, back and upper arms
o Rash may be exacerbated by sunlight
o Proximal myopathy
o Muscle pain and tenderness in early disease
What are the IX for polymyositis
CK is raise (50% more)
EMG
Musclebx - defnitive diagnostic test
Which auto-ab are related to polymyositis
Anti-Jo1
Myosite specific Ab
Which auto-ab are related to dermatomyositis
Anti-Mi2
ANA
Define SLE
• Multi-system inflammatory autoimmune disorder
What are the American College of Rheumatology criteria
• 4/11 of the diagnostic criteria of the American College of Rheumatology provides high sensitivity and specificity for the diagnosis of SLE:
SOAP BRAIN MD
o Serositis
o Oral ulcers
o Arthritis (non-erosive)
o Photosensitivity
o Bloods (haemolytic anaemia/ leukopaenia/ thrombocytopaenia)
o Renal disease (urine casts/proteinuria)
o ANA
o Immunological disorder (anti-dsDNA /anti-Sm /anti-phospholipid)
o Neurological disease (psychosis/seizures)
o Malar rash
o Discoid rash
Recognise the presenting symptoms and signs of SLE
• General Symptoms o Fever o Fatigue o Weight loss o Lymphadenopathy o Splenomegaly
- Raynaud’s phenomenon
- Oral ulcers
• Skin Rash
o Malar rash
o Discoid lupus (red scaly patches)
o Atypical rashes (e.g. photosensitivity, vasculitis, urticaria, purpura)
• Systemic Involvement
o Musculoskeletal - arthritis, tendonitis, myopathy
o Heart - pericarditis, myocarditis, arrhythmias, Libman-Sacks endocarditis
o Lung - pleurisy, pleural effusion, basal atelectasis, restrictive lung defects
o Neurological - headache, stroke, cranial nerve palsies, confusion, chorea
o Psychiatric - depression, psychosis
o Renal - glomerulonephritis
Identify appropriate investigations for SLE
• Bloods o FBC o U&E o LFT o Raised ESR * o Raised/Normal CRP * o Clotting o Complement
- Urine M haematuria, proteinuria, red cell casts
- Joints M plain radiographs
- Heart and Lungs M CXR, ECG, echocardiogram, CT
- Kidneys M renal biopsy (if glomerulonephritis suspected) • CNS M MRI scan, lumbar puncture
State the autoantibodies associated with SLE
• Autoantibodies o Anti-dsDNA (60%) o Rheumatoid factor (30M50%) o Anti-ENA o Anti-RNP o Anti-SM o Anti-Ro o Anti-La o Anti-histone o Anti-cardiolipin
a 55yo female presetns with dry cough and SOB which have been present and have progressed the past few months.
PMH: rheumatoid arthritis
OE: Bilateral fine inspiratory crackles
What is this
Pulmonary fibrosis
Is an extra-articular manifestation of RhA
Is also a side effect of methotrexate (a disease-modifying anti rheumatic drug) a drug used in RhA
Define anti-phospholipid syndrome
• Characterised by the presence of antiphospholipid antibodies (APL) in the plasma, venous and arterial thrombosis, recurrent foetal loss and thrombocytopaenia
Explain the aetiology/risk factors of anti-phospholipid syndrome
- Antiphospholipid antibodies (APL) are directed against plasma proteins bound to phospholipids
- APL may develop in susceptible individuals following exposure to infectious agents
- Once APL are present, a second event is needed for the syndrome to develop
- APL has effects on a number of coagulation factors (e.g. protein C, annexin V, platelets, fibrinolysis)
- Complement activation by APL is critical for the complications
Summarise the epidemiology of antiJphospholipid syndrome
- More common in YOUNG WOMEN
- Accounts for 20% of strokes in < 45 yrs
- Accounts for 27% of women with > 2 miscarriages
Recognise the presenting symptoms of antiJphospholipid syndrome
• RECURRENT MISCARRIAGES * • History of: o Arterial thromboses (stroke) o Venous thromboses (DVT, PE) * • Headaches (migraine) • Chorea • Epilepsy
(at a young age)
Recognise the signs of antiJphospholipid syndrome on physical examination
• Livedo reticularis = A skin finding consisting of a mottled reticulated vascular pattern that appears as a lace-like purplish discolouration of the skin
- Signs of SLE (e.g. malar rash, discoid lesions)
- Signs of valvular heart disease
Identify appropriate investigations for antiJphospholipid syndrome
• FBC - low platelets
• ESR - usually normal
• U&Es - can get APL nephropathy
• Clotting screen - high APTT
• Presence of antiphospholipid antibodies may be demonstrated by:
o ELISA testing for anticardiolipin antibodies
o Lupus anticoagulant assays
Which auto-ab are associated with Sjrogens
• Autoantibodies
o Rheumatoid factor
o ANA
o AntiMENA (extractable nuclear antigens)
What is Sjorgens
Characterised by inflammation and destruction of exocrine glands (usually salivary and
lacrimal glands).
o NOTE: when associated with other autoimmune diseases, Sjogren’s syndrome is
termed secondary
How does sjogrens present
- Dry eyes (keratoconjunctivitis sicca) - they will be gritty and sore
- Dry mouth - leads to secondary dysphagia
- Dry upper airways M leads to a dry cough and recurrent sinusitis
- Fatigue
- Fever
- Weight loss
- Depression
- Dry skin or hair
- Dry vagina - may cause dyspareunia
- Reduced GI mucus secretions leads to reflux oesophagitis, gastritis and constipation
What is Schirmer’s Test
o A strip of filter paper is placed under the eyelid
o Positive for Sjogren’s syndrome if < 10 mm of the strip is wet after 5 mins
What is Henoch-Schonlein purpura
A small vessel vasculitis which usually occurs in children after an upper respiratory tract infection.
purpura, arthritis, gut symptoms, glomerulonephritis, IgA deposition