MRCP Revision Rheumatology

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Your complete guide to passing the MRCP
Rheumatology
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MRCP Revision Rheumatology
Table Of Contents
Rheumatoid arthritis 2
Gout & Pseudogout 7
SLE - Systemic Lupus Erythematosus - Investigations and
Management 10
The Seronegative Spondyloarthropathies 13
Vasculitis 17
Large vessel vasculitis 17
Medium vessel vasculitis 19
Small vessel vasculitis 20
Systemic sclerosis 24
Raynaud's Syndrome 25
Bechet’s disease 25
Sjogren’s Syndrome 26
Dermatomyositis & Polymyositis 27
Antiphospholipid syndrome 28
Osteoarthritis and Septic arthritis 29
MRCP favourites – must know autoimmune conditions 31
MRCPREVISION.COM M RCP Revision Rheum at ol ogy

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Rheumatoid arthritis
Rheumatoid arthritis is a chronic and progressive in ammatory arthropathy of auto-
immune aetiology. It causes disabling pain and in ammation of the small joints of the
hands and feet most commonly, along with other multi-system complications. If left
untreated, RA can result in progressive joint destruction. Rheumatoid arthritis is
common, affecting an estimated 1% of the population in the UK.
Clinical features
Autoimmune symmetrical polyarthritis
Swollen, painful joints – particularly hands and feet
Morning stiffness
On examination:
Positive squeeze test
Boutonniere’s and swan neck deformity indicate advanced disease
Epidemiology
Prevalence: 1%
Age of onset: 30-50 years of age
Females are at highest risk (3F: 1M)
Genetics
Increased risk associated with HLA-DR4
Pathology
TNF-alpha, an in ammatory cytokine, plays a signi cant role in the
in ammatory response in RA. This has led to the use of TNF-inhibitors in the
management of RA
Investigations and diagnosis of RA
Clinical - in ammatory polyarthropathy with predilection for small joints
CRP/ESR elevated
XR - Juxta-articular osteoporosis
Antibodies – Rheumatoid factor and anti-CCP

Antibodies in Rheumatoid arthritis

Rheumatoid factor:
RF is an IgM antibody which reacts with the Fc portion of IgG
Tests for RhF: Rose-Waaler test (sheep red cell agglutination), latex
agglutination
RhF is positive in 80% of patients with RA
Rheumatoid factor is a prognostic marker – high levels of RhF suggest
progressive disease but RF does NOT re ect current disease activity
RhF is also positive in: Felty’s syndrome, Sjögren’s syndrome, SLE,
systemic sclerosis – it is non-speci c
Anti-cyclic citrullinated peptide antibody (Anti-CCP)
Can be present for up to 10 years before the diagnosis – this may have
clinical implications such as allowing early treatment with anti-TNF alpha
medications
NICE guidance: Check anti-CCP in patients in whom RA is suspected but
rheumatoid factor is negative
Prognosis
A poor prognosis is suggested by:
Antibodies: High titres of rheumatoid factor and anti-CCP positive
HLA-DR4 positive
Early XR changes
Insidious onset
Presence of extra-articular features
Management of rheumatoid arthritis – current guidelines
Flares: PO/IM steroids
Maintenance:
1st LINE: Commence DMARD monotherapy at diagnosis (co-prescribe a
bridging steroid when initiating DMARD)
Monitor disease response with:
CRP levels
DAS-28 score
2 line: Alternative DMARD
nd
3rd line: If there is failure of 2 or more DMARDs, consider TNF-inhibitors
or monoclonal antibody therapy

DMARDs and their side effects

Methotrexate
Myelosuppression
Liver cirrhosis
Methotrexate pneumonitis
Monitoring: FBC, LFTs
Sulfasalazine
Oligospermia
Interstitial lung disease
Heinz body anaemia
Le unomide
Hypertension
Liver impairment
Interstitial lung disease
Hydroxychloroquine
Retinopathy
Corneal deposits
TNF-inhibitors
TNF-inhibitors are indicated in the management of rheumatoid arthritis
following the failure of 2 or more DMARDs
Etanercept
Mechanism: acts as a decoy receptor for TNF-alpha
- Tip: EtaneRCEPT - decoy RCEPTor
Adverse effects: demyelinating disease, reactivation of latent TB
In iximab
Mechanism: Monoclonal antibody which binds to & neutralises TNF-alpha
Adverse effects: Reactivation of TB
Adalimumab
Rituximab
Mechanism of action: Rituximab is an anti-CD20 monoclonal antibody causing
B-cell depletion – reduces antibody mediated disease activity
Adverse effects: Infusion reactions

Abatacept
Mechanism of action: Reduced T cell proliferation and cytokine production
- Tip: Aba T a C ept = reduced T cells and Cytokines
Extra-articular manifestations of rheumatoid arthritis
Respiratory manifestations
Pulmonary brosis
Pulmonary nodules
Pleural effusions (exudative)
Caplan syndrome: Massive brotic nodules occur following occupational
exposure to coal dust
Bronchiolitis obliterans
Ocular manifestations
Keratoconjunctivitis sicca – Dry, red irritated eyes, blurred vision and
visual fatigue
- The MOST COMMON ocular complication
Scleritis – red + painful
Episcleritis – red
Corneal ulcers, keratitis
Others:
Accelerated cardiovascular disease – high risk of ischaemic heart disease
in RA
Osteoporosis
Depression
Felty’s syndrome
Amyloidosis AA

Rheumatoid arthritis in Pregnancy

Advice: patients should be advised, where possible, to defer pregnancy until
symptoms are well controlled
Disease course: Typically symptoms will improve during pregnancy, but often
are post-partum
SAFE DMARDs & management during pregnancy:
Sulfasalazine
Hydroxychloroquine
Low dose steroids
Methotrexate, le unomide should be avoided
NSAIDs should not be used after 32 weeks – closure of ductus arteriosus
Risk: Atlanto-axial subluxation – involve obstetric anaesthetist
Rheumatoid Arthritis (Credit: James Heilman, MD, CC BY-SA 3.0 via Wikimedia Commons)

Gout & Pseudogout

Gout and pseudogout are the most common crystal-induced in ammatory
arthropathies. Crystals of urate (gout) and calcium pyrophosphate (pseudogout) are
deposited within joints resulting in an immune response. They present in a similar
manner with acute onset, red, hot and swollen joints which can be exquisitely
painful.
Gout
Pathology:
In ammatory arthritis – microcrystal synovitis
Cause: Deposition of MONOSODIUM URATE MONOHYDRATE within the
synovium (as a result of hyperuricaemia)
Most common joint affected: 1st METATARSOPHALANGEAL JOINT (70%)
Risk factors include factors which cause increased urate synthesis (often related to
high cell turnover), or impaired urate excretion.
Drugs: Aspirin, thiazides, furosemide, cytotoxics, ciclosporin
Lead: reduces excretion of urate
Co-morbidities: CKD, myeloproliferative disorders, psoriasis
Lesh-Nyhan syndrome
Pathology: De ciency in hypoxanthine-guanine
phosphoribosyltransferase (HGPTase)
Inheritance: X-linked recessive
Features: Self mutilation (head banging/biting), renal failure, orange/sandy
urine, renal failure, learning disability
Investigations:
X-ray
Punched out lesions
Juxta-articular sclerosis
Joint effusions
Microscopy: Needle shaped, negatively birefringent crystals
Other clinical factors: soft tissue tophi

Acute management:
1st LINE: NSAID (naproxen) or COLCHICINE (esp. if renal impairment)
Colchicine – common side effects = diarrhoea
NSAID – should be prescribed with PPI cover
2 Line: PO Prednisolone – 15mg OD
nd
Allopurinol should be continued during the acute attack

Urate lowering therapy: should be commenced after the FIRST attack of gout
1st LINE: Allopurinol
Mechanism of action: XANTHINE OXIDASE INHIBITOR
Commence 2 weeks following the rst attack, and co-prescribe
colchicine cover whilst initiating therapy
Starting dose approx. 100mg OD
This should be titrated until uric acid level < 300 umol/L
2 line: Febuxostat - Mechanism of action: XANTHINE OXIDASE
nd
INHIBITOR
Refractory gout treatment options:
Uricase
Pegloticase infusion
Other management:
Losartan helps to reduce uric acid levels, so is a useful options in
patient with both gout and hypertension
High vitamin C diet should be recommended
Gout 1st MTP (credit: Gonzosft, CC BY 3.0 DE via Wikimedia Commons)

Pseudogout
Pathology:
In ammatory arthritis – microcrystal synovitis
Cause: Deposition of CALCIUM PYROPHOSPHATE DIHYDRATE crystals
Most commonly affects larger joints: Knees, ankles, elbows, wrists
Risk factors:
Increasing age
HAEMOCHROMATOSIS
Investigations:
Microscopy: Rhomboid shape, positively birefringent crystals
X-ray: CHONDROCALCINOSIS / calci cation of cartilage/meniscus
Management:
Rule out septic arthritis
Manage with NSAIDs or steroids (PO/IA/IM)

SLE - Systemic Lupus Erythematosus -

Investigations and Management
Systemic lupus erythematosus is a chronic multisystem disorder of autoimmune
aetiology which can present with a wide range of clinical features. SLE affects
approximately 1 in 1000 people in the UK.
Pathophysiology:
Lupus is an autoimmune mediated condition
Type 3 hypersensitivity condition
Antigen: antibody complexes form (immune complexes) & are subsequently
deposited in tissues around the body. As such it is a multi-system disorder.
Genetics: HLA-DR3, DR2 and B8 associated with increased risk of SLE
Epidemiology:
Females are at increased risk (F9:1M)
Afro-Caribbean patients affected most commonly
Peak age of onset: 20-40 years
Clinical features of lupus:
General: Fever, fatigue, lymphadenopathy, ulcers
Dermatological:
Photosensitive rash
Butter y/malar rash (sparing the nasolabial folds)
Non-scarring alopecia
Livedo reticularis
Arthralgia
Cardiovascular: Pericarditis (more commonly than myocarditis)
Respiratory: Fibrosing alveolitis
Renal:
DIFFUSE PROLIFERATIVE GOLMERULONEPHRITIS
Wire-loop appearance: endothelial/mesangial proliferation
Subendothelial immune complex deposits
Granular appearance on immuno uorescence

Investigating SLE:
ANA – 99% sensitivity
Anti-dsDNA – 99% speci c
Anti-smith – 99% speci c
Other: Anti-RO (SS-A), anti-La (SS-B), anti-U1RNP
Monitoring disease activity:
ESR and anti-dsDNA levels can be used for monitoring disease activity in
SLE
In active disease CRP can be normal (so if it is high this might suggest
concurrent infection).
Low C3 and C4 levels re ect active disease due to consumption
Pregnancy in SLE:
Maternal antibodies can cross the placenta
As a result, the foetus is at risk of developing NEONATAL LUPUS
ERYTHEMATOSUS
- This can lead to CONGENITAL HEART BLOCK
ANTI-RO +ve patients are at the highest risk of NLE.
SLE photosensitive malar rash (credit: Doktorinternet, CC BY-SA 4.0via Wikimedia

Commons)

Drug Induced Lupus

Certain medications can result in a lupus like disease, referred to as drug
induced lupus
Clinical features:
MALAR RASH
PLEURISY
Arthralgia, myalgia
Renal and neurological involvement is very rare in DIL
Investigations:
100% of patients are ANA +ve
dsDNA negative - differentiates DIL from SLE
90% of patients are ANTI-HISTONE +VE
Management:
The mainstay of management of drug induced lupus is stopping the
causative medication. The medications associated with the highest risk of
DIL include:
PROCAINMIDE
HYDRALAZINE
ISONIAZID
MINOCYCLINE
PHENYTOIN
Discoid lupus erythematosus
Discoid lupus erythematosus is an autoimmune condition which results in the
presence of scaly, discoid lesions which primarily affects the scalp, face and
ears. The rash can be photosensitive.
DLE results in SCARRING ALOPECIA
DLE is characterised by the presence of FOLLICULAR KERATIN PLUGS
5% of patients with discoid lupus will go on to develop SLE
Management:
1st LINE: TOPICAL STEROIDS
2nd LINE: HYDROXYCHLOROQUINE
Avoid sun exposure

The Seronegative Spondyloarthropathies

The seronegative spondyloarthropathies are a collection of joint disorders in which
patients are RHEUMATOID FACTOR NEGATIVE. This includes reactive arthritis
(previously known as Reiter’s), psoriatic arthritis, enteropathic arthritis and
ankylosing spondylitis. They present with symptoms typical of in ammatory
arthropathies affecting the back and/or peripheral joints including morning stiffness
and pain which improves with exercise.
Genetics: Seronegative spondyloarthropathies are signi cantly associated with the
presence of HLA-B27
Features:
Asymmetrical peripheral arthritis
Enthesopathy
Sacroiliitis
Extra-articular manifestations
Reactive arthritis (Reiter’s syndrome)

Features of reactive arthritis:
Reactive arthritis / Reiter’s syndrome is classically characterised by the
presence of a TRIAD of:
CONJUNCTIVITIS
URETHRITIS
ASYMMETRICAL OLIGOARTHRITIS
Tip: Reactive arthritis - can’t see, can’t pee, can’t climb a tree...
Symptoms usually last for less than 12 months before resolution
Causes:
Reactive arthritis most commonly follows an infection of the gastrointestinal or
genitourinary tracts
Post-STI (especially men) – CHLAMYDIA TRACHOMATIS - This is
referred to as SARA – sexually acquired reactive arthritis
Post-dysentery (M=F) – CAMPYLOBACTER, SHIGELLA, SALMONELLA,
YERSINIA

Management of Reiter’s syndrome:

1st LINE: NSAIDs (+/- paracetamol or intra-articular steroids)
2nd line: Sulfasalazine, methotrexate rarely used
Psoriatic arthropathy
10-20% of patients with cutaneous psoriasis will develop psoriatic arthritis, though
often patients with psoriatic arthropathy will have no skin lesions
Psoriatic arthropathy can be classi ed into 3 main types including:
Rheumatoid-like – accounts for 40% of cases – MOST COMMON
Asymmetrical oligoarthritis – accounts for 30% of cases
DIP joint disease – accounts for 10% of cases
Investigations:
X-ray:
DIPs are most affected > MCPs/PIPs – this differentiates it from
rheumatoid arthritis!
JUXTA-ARTICULAR PERIOSTITIS
- PENCIL IN A CUP APPEARANCE
Management:
The management of psoriatic arthritis is essentially as per the management of
rheumatoid arthritis (i.e. DMARDs are the mainstay), though the prognosis is better.
Enteropathic arthritis
Enteropathic arthritis occurs in patients with IBD (Crohn’s or ulcerative colitis), and so
questions may describe a history of joint pain + recent weight loss, diarrhoea etc.
Clinical features:
Crohn’s/UC – i.e. history of diarrhoea, weight loss, abdominal pain, PR bleeding
etc.
Sacroiliitis
Enthesopathy – Achilles tendonitis, plantar fasciitis
Asymmetrical arthritis
Extra-articular manifestations

Ankylosing Spondylitis
Genetics: As above, signi cant association with HLA-B27 (positive in 90% of
patients – but also positive in 10% or ‘normal’ patients, so not much value
diagnostically).
Clinical features of ankylosing spondylitis:
Typically a young male patient age 20-35
Lower back pain
Morning stiffness, improves with exercise
Night time back pain may be present
Examination ndings:
Reduced lateral exion
Reduced chest expansion
Reduced forward exion
Schober’s test – failure to increase by > 5cm
Extra-articular features of Ankylosing spondylitis
Tip: Extra-articular features can be remembered as “The A’s of Ankylosing
spondylitis”
Apical brosis
Anterior uveitis
AVN block on ECG
Aortic regurgitation
Amyloidosis A
And cAuda equina syndrome – a rare but serious complication of
ankylosing spondylitis
Ankylosing spondylitis - Bamboo

spine resulting from vertebral body
fusion (credit: Stevenfruitsmaak, CC
BY-SA 3.0 via Wikimedia Commons)

Investigations:
As above, HLA-B27 is NOT used in the diagnostic work up
1st LINE: PLAIN XR OF SACROILIAC JOINT
- Findings include sacroiliitis, squaring of the vertebra, syndesmophytes &
bamboo spine
2nd line: If XR is negative but clinical suspicion remains, perform an MRI L/S
spine (also shows bone marrow oedema in the SI)
Other investigations: Spirometry – restrictive pattern
Management:
Exercise is extremely important
Pharmacological
1st LINE: NSAIDs
DMARDs (inc. TNF-inhibitors) are only effective for the management of
associated peripheral joint arthritis and will not improve back
pain/stiffness.

Vasculitis
Classi cation
Large vessel vasculitis: Temporal arteritis, Takayasu’s arteritis
Medium vessel vasculitis: PAN, Kawasaki, Beurger's
Small vessel vasculitis: ANCA associated vasculitis, immune complex vasculitis
Large vessel vasculitis

Temporal Arteritis
Pathology: Vasculitis of the super cial temporal artery (branch of the external
carotid)
Presentation:
50% of patients have a history of polymyalgia rheumatica (suggested by pain
and stiffness in the proximal muscles esp. in the mornings)
Headache, claudication (chewing)
Super cial tenderness (e.g. while showering/combing hair)
Visual disturbance - as a result of anterior ischaemic optic neuropathy
Bloods: ESR + CRP raised
Temporal artery biopsy - diagnostic investigation of choice
Disadvantages of TA biopsy include the possibility of SKIP lesions
Histology: Granulomatous arteritis with mononuclear cell in ltrates and
GIANT CELL formation
Contraindications: Steroid use for > 30 days (reduces yield)
Complications: CN7 palsy, auriculotemporal nerve injury.
Management:
High dose steroids - prednisolone
Urgent ophthalmology review - same day review if any visual symptoms

Polymyalgia Rheumatica
Clinical features:
Age > 60yrs
Acute (< 1/12) history of morning proximal STIFFNESS and aching (not so
much weakness)
Low mood
Night sweats
Polyarthralgia
Investigations: ESR > 40 (CK, EMG normal)
Management: Prednisolone 15mg OD
Takayasu's Arteritis
Presentation:
Young, Asian female
Systemic upset
INTERMITTENT CLAUDICATION
Examination ndings:
UNEQUAL BPs in upper limbs
Early diastolic murmur - aortic regurgitation
Carotid bruit
Associated conditions: renal artery stenosis
Management: Steroids

Medium vessel vasculitis

Polyarteritis Nodosa (PAN)
Pathology
Necrotising in ammation of the 'medium' sized blood vessels
Results in the formation of aneurysms
Presentation
Classically a middle aged man with a history of HEPATITIS B
MONONEURITIS MULTIPLEX - painful, peripheral neuropathy affecting 2 or
more peripheral nerves
Hypertension
Livedo reticularis
Haematuria
Acute renal failure
Investigations:
pANCA (20% positive), HEPATITIS B SEROLOGY (30% positive)
Angiogram: Beading + microaneurysms
Kawasaki's
Presentation: Children presenting with fever and most of the following 5 features:
Conjunctival injection
Red/cracked lips
Strawberry tongue
Cervical lymphadenopathy
Redness and desquamation of the skin of palms and soles
Complications: CORONARY ARTERY ANEURYSM
Investigations: Echocardiogram is the investigation of choice for CA aneurysm
Management:
High dose aspirin (risk of Reye's syndrome)
IV immunoglobulins

Beurger's (thromboangiitis obliterans)

Risk factors: SMOKING
Presentation:
Classically, a smoker develops symptoms of limb ischaemia (ulcers,
claudication)
Raynaud's
Thrombophlebitis
Small vessel vasculitis

Small vessel vasculitis can be further divided into ANCA associated vasculitis (GPA,
EGP, MP) and immune complex vasculitis (HSP, Goodpastures, cryoglobulinaemia).
Granulomatosis with Polyangiitis (Wegener's)

Pathology: Autoimmune disease process resulting in necrotising , granulomatous
in ammation of the vessels of the upper respiratory tract, lower respiratory tract
and kidneys.
Features:
URT - SINUSITIS, epistaxis, nasal crusting, SADDLE NOSE
LRT: HAEMOPTYSIS
Renal: RAPIDLY PROGRESSIVE (CRESCENTIC) GLOMERULONEPHRITIS -
acute renal failure
Vasculitis rash, proptosis, cranial nerve lesions
Investigations: cANCA (90%), pANCA (25%)
CXR: cavitations
Renal biopsy: Epithelial crescents affecting Bowman's capsule
Management:
Steroids
Cyclophosphamide
Plasma exchange
Survival 8-10 yrs

Eosinophilic Granulomatosis with Polyangiitis (Churg-

Strauss)
Features:
SINUSITIS
Asthma like symptoms, wheeze
Mononeuritis multiplex
Investigations:
Bloods - eosinophilia
Antibodies: pANCA (60%)
Prescribing precautions: Avoid leukotriene receptor antagonists (can precipitate
disease)
Tip: Questions may describe a patient with a presumed diagnosis of asthma, who's
condition deteriorates after GP introduces montelukast.
Microscopic Polyangiitis
Features:
Fever
Systemic upset
Mononeuritis multiplex
Renal impairment - raised creatinine, haematuria
Rash - palpable, purpuric rash
Labs: pANCA (70%), cANCA (40%)
HSP
Pathology: IgA mediated small vessel vasculitis
Clinical features: Often a child, post infection
PALPABLE PURPURIC RASH (with oedema), affecting the EXTENSORS AND
BUTTOCKS
Abdominal pain
Polyarthritis

Features of IgA nephropathy - AKI, haematuria

Prognosis: Self-limiting. 1/3rd of patients will have relapse.
Management: Supportive, analgesia
Goodpasture’s Syndrome
Pathophysiology:
IgG ANTI-GBM ANTIBODIES VS TYPE 4 COLLAGEN
Genetics: HLA-DR2 association
Features:
Renal - rapidly progressive (crescentic) glomerulonephritis - AKI, haematuria,
proteinuria
Pulmonary haemorrhage - haemoptysis, elevated TRANSFER FACTOR
Renal biopsy: Linear deposits of IgG along the basement membrane
Management:
Plasma exchange
Steroids
Cyclophosphamide
Cryoglobulinaemia
Pathology: Reversible precipitation of immunoglobulins occurring at 4 degrees C (&
dissolve at 37). Cryoglobulinaemia can be classi ed as type 1, type 2 or type 3.
Type 1 cryoglobulinaemia:
Monoclonal antibodies (IgG or IgM)
Causes: myeloma, Waldenstrom's
Type 2 cryoglobulinaemia:
Mixed mono- + polyclonal antibodies
Rheumatoid factor positive
Causes include HEPATITIS C
Type 3 cryoglobulinaemia
Rheumatoid factor positive
Causes include rheumatoid arthritis, Sjögren’s

Clinical features:
Raynaud's
Purpuric rash
Arthralgia
Renal failure: diffuse glomerulonephritis
Bloods:
Low complement (C4)
Raised ESR
Treatment: Immunosuppression, plasmapheresis
Hypocomplementemic Urticarial Vasculitis
Anti-C1q vasculitis
Features: Low complement levels, urticaria

Systemic sclerosis
Systemic sclerosis is an autoimmune condition characterised by sclerosis and
hardening of the skin and other connective tissue. There are 3 distinct patterns of
systemic sclerosis, including limited cutaneous SS, diffuse cutaneous SS,
scleroderma.
90% of patients with any form are ANA positive.
Limited cutaneous systemic sclerosis

Features:
Sclerosis affects the FACE + DISTAL LIMBS
Raynaud’s is a common & early feature
Subtypes include CREST
Antibody: ANTI-CENTROMERE antibodies
Diffuse cutaneous systemic sclerosis

Sclerosis affects the TRUNK + PROXIMAL LIMBS
Complications:
INTERSTITIAL LUNG DISEASE (affects up to80% pts)
Pulmonary artery hypertension
Hypertension, CKD
Antibody: SCL-70 antibodies
Scleroderma
Features:
Tightening of the skin, gives 'shiny' appearance
Can be linear or morphea (plaques/ patches of brosis or localised scleroderma
- from increased collagen deposition)
No organ involvement

Raynaud's Syndrome
Pathophysiology:
Cold or stress results in excess vasoconstriction of the digital arteries
Primary = Raynaud’s disease
Secondary = Raynaud’s phenomenon (e.g. sharps syndrome)
Causes:
Connective tissue disorders: rheumatoid arthritis/ SLE/ SS
Leukaemia
Type 1 cryoglobulinaemia
Management:
1st LINE: CALCIUM CHANNEL BLOCKERS (NIFEDIPINE)
2nd line: IV Prostacyclin (EPOPROSTENOL infusions)
Raynaud's phenomenon in SLE (credit: Tcal at English Wikipedia, CC BY-SA 3.0 via Wikimedia Commons
Bechet’s disease
Pathology: autoimmune mediated in ammation of the arteries and veins
Presentation: Most commonly affects patients from middle-east (Turkey esp.) with a
positive family history, classic triad.
1. Oral ulcers
2. Genital ulcers
3. anterior uveitis
Other features include thrombophlebitis, deep vein thrombosis
Genetics: Association with HLA-B51
Diagnosis: Positive pathergy test suggests Bechet's but diagnosis is clinical

Sjogren’s Syndrome
Pathology: Autoimmune mediated destruction of EXOCRINE glands
Classi cation:
Primary Sjogren’s
Secondary Sjogren’s - rheumatoid arthritis, connective tissue disease
Presentation:
Dry eyes (Keratoconjunctivitis Sicca)
Dry mouth - xerostomia
Parotitis
Vaginal dryness
Arthralgia
Sensory polyneuropathy
Complications: LYMPHOID MALIGNANCY ( x 50 risk)
Antibodies:
Rheumatoid factor - 50% positive
ANA - 70% positive
ANTI-RO (SSA) - 70% Primary SS
Anti-La (SS) - 30%
Labs: Raised immunoglobulins, reduced C4
Diagnosis: SCHIRMERS TEST
Histology: focal lymphocytic in ltration
Management: pilocarpine, arti cial tears
Sjogrens - focal lymphoplasmacytic in ltration of the salivary

glands (credit: KGH assumed (based on copyright claims).,
CC BY-SA 3.0 via Wikimedia Commons)

Dermatomyositis & Polymyositis

Dermatomyositis
Dermatomyositis is an in ammatory condition resulting in symmetrical PROXIMAL
MUSCLE weakness and SKIN LESIONS.
Derm features - 'Dermato...'
HELIOTROPE periorbital rash
Macular rash affecting back and shoulders
Mechanics hands – Gottron’s red papules on extensor surfaces
Rashes are photosensitive
MSK features - '...myositis'
Symmetrical, proximal weakness +/- tenderness
Other features: ILD, Raynaud’s
Causes:
Idiopathic
MALIGNANCY - Ovarian, breast, lung (in 25% pts)
Investigations:
Raised CK
ANA positive (80%)
Antibodies vs AMINOACYL-tRNA SYNTHETASE including
Anti-MI-2 Antibodies
Anti-SRP antibodies
Anti-Jo1 antibodies (vs histidine tRNA ligase) (Note - anti-Jo-1 is more
associated with POLYMYOSITIS)
PATIENTS SHOULD BE SCREENED FOR MALIGNANCY
Management: Prednisolone
Polymyositis
A variant of dermatomyositis without skin manifestations
Antibodies: ANTI-JO1 (vs histidine tRNA ligase)

Antiphospholipid syndrome
APS can be primary or occur secondary to other diseases (notably SLE)
Clinical features:
ARTERIAL + VENOUS THROMBOSIS (MI/stroke, DVT/PE)
THROMBOCYTOPENIA
Recurrent MISSCARRIAGE / FETAL LOSS
Other features: Livedo reticularis
Investigations:
Antibodies:
LUPUS ANTICOAGULANT antibodies
Anti-cardiolipin antibodies
Anti-apolipoprotein antibodies
Paradoxically increased APTT (reaction of lupus anticoagulant with
phospholipids in the clotting cascade)
Management: THROMBOPROPHYLAXIS
Primary thromboprophylaxis - Aspirin 75mg OD
Secondary thromboprophylaxis
Initial VTE or arterial thrombus - LIFELONG warfarin (INR 2-3)
VTE whilst on warfarin - Lifelong warfarin (INR 3-4) AND aspirin
APS & Pregnancy
Complications: Recurrent miscarriage, foetal loss, IUGR, pre-eclampsia, placental
abruption, prematurity
Management: Proper thromboprophylaxis is essential – it increases rate of live birth
by 7x
LOW DOSE ASPIRIN - as soon as pregnancy con rmed
LMWH - as soon as FETAL HEART PRESENT ON US until 34 weeks

Osteoarthritis and Septic arthritis

Osteoarthritis
Pathophysiology
Degradation of articular cartilage and remodelling of bone
Carpometacarpal and DIPJs affected more than the PIPJs (in contrast to RA
where DIPJs are spared), bilateral.
Examination ndings
Heberden’s nodes
Bouchard’s nodes
Investigations: XR shows LOSS
L – loss of joint space
O – osteophytes
S – subchondral sclerosis
S - subchondral cysts
Management
Conservative strategies – weight loss and exercise
1st LINE: Paracetamol (AND topical NSAIDs if knee/hand affected)
2nd Line: Oral NSAID (co-prescribed with PPI), opioids, capsaicin cream, intra-
articular steroid injections
Other management strategies: Joint replacement, glucosamine (not
recommended by NICE)
Septic arthritis
Microbiology
Staph Aureus is the most common causative organism overall
Neisseria Gonorrhoea is the most common organism in young, sexually active
patients
Pathophysiology
Haematogenous spread of microorganism to joint space is more common than
direct inoculation
The most commonly affected joint is the knee

Investigations
Joint aspiration for MCS
Blood cultures
Management
1st Line: Flucloxacillin – total course 6-12 weeks
Penicillin allergy: Clindamycin
Plus – needle decompression +/- lavage

MRCP favourites – must know autoimmune

conditions
Mixed Connective Tissue Disease / Sharp's Syndrome
Pathology: A multisystem autoimmune disease
Features of SLE, systemic sclerosis and myositis
Antibodies: ANTI-U1 RIBONUCLEOPROTEIN antibodies
Presentation:
Raynaud's
Polyarthralgia
Myalgia
Dactylitis "sausage ngers"
Rash
Oesophageal dysfunction / swallowing dif culties
Management: DMARDs
Relapsing Polychondritis
Pathology: Episodic in ammation of cartilage, particularly of the EARS, nose, joints
Features:
Ears - Auricular chondritis, hearing loss, vertigo
Nose - nasal chondritis - saddle nose
Respiratory tract - hoarse voice, aphonia, wheeze
Joints - arthralgia
Management:
Steroids can be used to induce remission
Maintenance - DMARDs

Familial Mediterranean Fever

Pathology: Recurrent polyserositis
Inheritance: autosomal RECESSIVE
RFs: Turkish, Armenian, Arabic descent
Clinical features:
Patients have 'attacks' lasting 1-3 days with symptoms including...
Fever
Pleurisy
Abdominal pain, peritonitis
Pericarditis
Arthritis
Erysipeloid rash on the lower limbs
Management: COLCHICINE is 1st line
Pseudoxanthoma Elasticum
Inheritance: autosomal RECESSIVE
Features:
Retinal angioid streaks
'Plucked chicken skin appearance'
Cardiovascular complications: MV prolapse, ischaemic heart disease
Gastrointestinal bleeding

Still’s Disease
Pathology: A systemic autoimmune disease
Clinical features – a triad of:
Fever
Joint pain
Maculopapular salmon rash
Investigations:
FERRITIN elevated
Autoantibodies: Rheumatoid factor and ANA are negative
Diagnosis: Yamaguchi criteria
Management:
1st Line: NSAIDs for 1 week
2nd line: Add steroids to NSAIDs
3rd line: Methotrexate/anti-TNF
Palindromic Rheumatism
Relapsing/remitting monoarthritis of large joint

MRCP Revision Rheumatology

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MRCP Revision Rheumatology

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MRCP Revision

Your complete guide to passing the MRCP

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Antibodies in Rheumatoid arthritis

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Allopurinol should be continued during the acute attack

Gout 1st MTP (credit: Gonzosft, CC BY 3.0 DE via Wikimedia Commons)

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SLE photosensitive malar rash (credit: Doktorinternet, CC BY-SA 4.0via Wikimedia

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Osteoarthritis and Septic arthritis

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Familial Mediterranean Fever

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