Rheumatology Flashcards
RA definition
Associations
Clinical presentation
Signs
Extra-articular manifestations
Chronic multisystem inflammatory disorder of unknown cause with characteristic symmetrical, deforming peripheral polyarthritis, chiefly manifesting as synovitis
Associations
• Smoking
• Goes into remission during pregnancy
Clinical presentation
• Symmetrical joint pain, stiffness, swelling – these 3 indicate synovitis
o Stiffness worse in the morning (>1hr)
• Usually small joints of the hand (PIP’s, MCPs, Wrists) and feet (MTPs) followed by larger joints
• Systemic: Malaise, weight loss, low grade pyrexia
• Onset insidious over weeks/months (10-20% acute)
Signs
• Symmetric polyarthritis affecting small joints of hand +/- deformity
• Tenderness/swelling/warmth/redness of joints (synovitis or fluid)
o Synovitis – boggy joint swelling
• Rheumatoid nodules (hands, elbows, ears lungs)
• Later: Ulnar deviation of fingers, dorsal wrist subluxation, Boutonniere and Swan neck, Z thumb
Extra-articular manifestations
Respiratory Pulmonary fibrosis, pleural effusions, Pulmonary nodules
Felty’s syndrome Triad of RA, Splenomegaly + Leukopenia (SANTA)
Skin Nodules, Palmar Erythema, Vasculitis (rare but can have mesenteric, cerebral etc.)
Eyes Keratoconjunctivitis sicca, Episcleritis, Scleritis
Secondary Sjogren’s syndrome (+ dry mouth)
Cardiac Pericarditis/Pericardial effusion
Bone Osteoporosis
Renal Membranous nephropathy, Secondary amyloidosis
Haem Anaemia of chronic disease (normocytic, normochromic)
Felty’s syndrome: Triad of RA, Splenomegaly + Leukopenia
Neuro Carpal tunnel syndrome (entrapment due to synovitis), Peripheral neuropathy, Mononeuritis multiplex
Amyloidosis
Atlanto-axial joint subluxation – endangers spinal cord - must do AP and latera imaging pre-procedures
RA investigations
Diagnostic criteria
Monitoring of RA
• FBC, CRP + ESR (If acutely raised, can suggest a flare)
• RF: Autoantibody (IgM/IgA/IgG) against Fc portion of IgG. +ve in 75% of RA
• Anti CCP(cyclic citrullinated protein): More specific
o Seropositivity indicates a more progressive disease
• LFTs: Medications may have toxicity in liver
• Renal function: Extra articular manifestations, some medication excreted renally
X- Ray o Loss of joint space o Erosions o Soft tissue swelling o Soft bones (osteopenia) US /MRI allow much earlier radiological diagnosis
Diagnosis: ACR/EULAR Criteria - 6/10 (1) Joint distribution (0-5) 1 large joint 0 2-10 large joints 1 1-3 small joints (large joints not counted) 2 4-10 small joints (large joints not counted) 3 >10 joints (at least one small joint) 5 (2)Serology (0-3) -ve RF AND -ve ACPA 0 Low +ve RF OR low +ve ACPA 2 High +ve RF OR high +ve ACPA 3 (3) Symptom duration (0-1) <6 weeks 0 6 weeks 1 (4) Acute phase reactants (0-1) Normal CRP AND normal ESR 0 Abnormal CRP OR abnormal ESR 1
Monitoring: DAS28
RA Management
Initial management
MDT: PT/OT, Podiatry, Rheumatologists, CNS’s, GP, Neuro, Sugeons • Exercise + Physio + OT • Pharmacological o NSAIDS o DMARDs - within 3 months of symptoms o Biologics o Steroids • Surgical: May relieve pain, improve function and prevent deformity
General approach
Early, aggressive approach
Symptom relief: NSAIDs, PT
Acute flare: Corticosteroids
Initial therapy
DMARD monotherapy +/- a short-course of bridging prednisolone. In the past dual DMARD therapy was advocated as the initial step.
OA Management
- MDT + holistic approach
• Weight loss, Exercise, PT
• Paracetamol, TOPICAL NSAIDs
• Podiatry: shoes with good support - Medications - Analgesia
• Oral NSAIDs, Opioids, Capsaican cream – WHO pain ladder
• Intraarticular injections (steroids or hyaluronic acid)
• Capsaicin cream or other topical analgesics - Criteria for referral for Joint Replacement Surgery (arthroplasty) = definitive treatment
1) Pain – limits walking, present at night, dependent on strong analgesia
2) X-rays - significant changes
3) Failed conservative management
What are seronegative spondyloarthropathies?
Name their features and the conditions
RF and ant CCP -ve. 3 key common features:
1) Axial skeleton inflammation e.g. sacrolillitis
2) Enthesitis – inflammation of tendon insertions
3) HLA-B27 association
Conditions
1) AS
2) Psoriatic arthritis
3) Reactive arthritis
4) Enteropathic arthritis
Ankylosing Spondylitis definition
Clinical presentation
Signs
Extra-articular manifestations
Chronic inflammatory disease of the spine, sacroiliac and axial joints
• Chronic Sacroiliitis
• Seronegative as RF -ve
Epidemiology • M:F = 2-3:1 • >95% AS patients are B27+ve • American Indians > Caucasians > Afro Caribbean/Oriental Presents at 20-30yo
Clinical presentation
• Insidious onset of Lower back pain + stiffness peristing >3 months
• Early morning stiffness, worse with rest + improves with exercise
• Reduced spinal movements – hyperextended neck, Loss of lumbar lordosis, Flexed hips and knees
• Peripheral arthritis (rarely at outset) but enthesitis common
o Plantar fasciitis, Achilles Tendonitis
• Fatigue
Signs
• Tender sacroiliac joints
• Restricted lumbar spinal movements (Schober’s test) - a line is drawn 10 cm above and 5 cm below the back dimples (dimples of Venus). The distance between the two lines should increase by more than 5 cm when the patient bends as far forward as possible
• Reduced lateral flexion
• Increased Occiput-Wall distance (>5cm)
• Reduced chest expansion
• Stooped Posture (loss of lumbar lordosis, thoracic kyphosis, cervical extension)
• Peripheral joint arthritis
• Enthesitis (Achilles tendon, plantar fascia)
Extra-articular manifestations • Atlantic axial instability • Anterior uveitis • Atypical lung fibrosis: Also caused by TB, Sarcoid • Aortic regurgitation • AV conduction defects • Amyloidosis - IgA nephropathy • Autoimmune bowel disease and UC?
AS
Investigations
Management
Chronic inflammatory disease of the spine, sacroiliac and axial joints
• Chronic Sacroiliitis
• Seronegative as RF -ve
Investigations • Bloods o Normocytic anaemia o HLA B27 +ve o ESR/CRP: can be normal • Imaging o MRI most useful: Irregularity (erosions), sclerosis of SI joints followed by fusion Squaring of vertebral bodies, Romanus lesion Syndesmophytes (ossification of the annulus fibrosis) Spinal fusion: Bamboo spine • Specialist tests o Slit lamp examination of eyes
Management
• 1st line: Simple analgesic, NSAIDs + PT (crucial), Support group
• Biologics (if BASDAI >4)
o anti-TNF works in most (after 2 NSAIDs tried 12 weeks apart)
o Secukinumab (anti-IL-17 mAb)
• DMARDs and steroids only work for those with peripheral joint disease – don’t work in spine!!
o Periph. Joint Disease Sulphasalazine
• Surgery has little role except occasionally in fusing painful or unstable bits of spine
Psoriatic arthritis definition
Clinical presentation + Signs
Patterns of joint disease
Investigations
Management
Inflammatory arthritis occurring in association with psoriasis
Clinical presentation
• Psoriasis precedes arthritis in 75%, synchronous in 15%, arthritis precedes in 10%
• Psoriasis (may be minor and not apparent)
• Nail changes: Pitting and onycholysis
• Arthritis – pain, stiffness in joints (Severity of arthritis reflects extent of skin disease in some patients)
arthritis mutilans (severe deformity fingers/hand, ‘telescoping fingers’)
Patterns of joint disease – 5 classic types
1) ASYMMETRIC OLIGOARTHRITIS = classic
2) Polyarthritis (mimics RA)
3) DIP joint arthritis (mimics OA)
4) Axial arthritis (mimics AS)
5) Arthritis mutilans – destructive bone eating form, causing ‘telescoping of the digits
Investigations
• Acute phase response – often normal
• RF -ve (usually)
• X-Rays (Depends on the disease pattern)
May be axial disease (similar to AS – sacroiliitis, syndesmophytes etc)
May be peripheral arthritis (similar to RA except DIP joint involvement)
Asymmetric involvement
o May be asymptomatic erosions in feet so x-ray both hands and feet
?Pencil in cup abnormality?
Management
• Peripheral joint disease – mild: PT,OT, Podiatery NSAIDs, consider steroid injections
• Progressive peripheral joint disease: DMARDs, NSAIDs, PT, Steroid injections
o DMARDs:
Methotrexate or Leflunomide (both help skin)
Sulfasalazine
o 2nd line: Biologics - anti-TNF (Adalimumab), Ustekinumab (Anti Il-12 + 23 monoclonal Ab), Secukinumab (Anti Il-17 monoclonal antibody)
o “Targeted synthetics” – Apremilast (PDE4 inhibitor)
Reactive arthritis definition
Clinical presentation + Signs
Patterns of joint disease
Investigations
Management
Sterile joint inflammation initiated by infection in which the CAUSATIVE ORGANISM CANNOT BE ISOLATED FROM JOINT.
Triad: reactive arthritis, urethritis, conjunctivitis.
Clinical presentation + Signs
• Arthritis developing 3-30 days post GI/GU infection
• Usually self-limiting but recurrent episodes may occur – in most resolution within 2-6 months
• Chronic symptoms in up to 20%
• Associated features
o Conjunctivitis
o Urethritis - Burning or stinging passing water,
o Lower back pain (sacroiliitis)
o Painful heels (enthesitis, plantar fasciitis)
o Oral ulcers
o Circinate balanitis
o Keratoderma blennorrhagicum
Investigations
• ESR/CRP may be raised
• RF -ve
• HLA-B27 may or may not be positive
Management
• Simple analgesics, NSAIDs, Opiods
• Steroids – short term use (if relapse on steroid reduction DMARDs may be required e.g. sulfasalazine 1st line)
• Biologics – anti TNF-alpha effective in some patients
Enteropathic arthritis definition
Clinical presentation + Signs
Patterns of joint disease
Investigations
Management
Arthropathies associated with pathology in the large and small bowel
• IBD mainly (Chron’s, UC)
• Infectious enteritis (Reactive Arthritis)
• Intestinal bypass surgery
• Whipple’s disease (Caused by Tropheryma whippelii)
• Coeliac disease
Clinical presentation
• Arthralgia but usually much less arthritis
• Asymmetrical pauci-articular arthritis
• Knee/ankle>Upper limb
• Spondylitis may occur (similar to AS)
• Associated features
o Enthesitis
o Anterior Uveitis
o Skin lesions (erythema nodosum, pyoderma gangrenosum)
o Clubbing and other manifestations of IBD
Investigations
• FBC: May have Fe deficiency or anaemia of chronic disease
• LFTs: May be abnormal in association with iBD
• CRP/ESR: Raised
• RF: -ve usually
• Haematinics (low iron, low ferritin), low vit D (poor absorpition)
• X-Rays: non erosive osteopenia
• Endoscopy
Management
(1) Treat IBD
(2) Medications
Simple analgesics, NSAIDs (may exacerbate IBD), opiods
DMARDs
• Sulfasalazine (mesalazine) useful in IBD because the ‘sulfa’ treats the join and the ‘salazine’ treats the bowels!
• Methotrexate or Azathioprine
‘Biologics’
• Infliximab / adalimumab (etanercept doesn’t work on the bowel)
Steroids only for acute flares
Surgery - colectomy for refractory UC, avoided wherever possible in Crohn’s disease
What is amyloidosis
Types
- Involvement
- Presentation
- Management
Diagnosis
Group of disorders characterised by extracellular deposits of a protein in abnormal fibrillar form, resistant to degradation
Types
(1) AL amyloid (primary amyloidosis)
- kidneys (proteinuria and nephrotic syndrome), heart (restrictive cardiomyopathy, arrhythmia, angina), nerves (peripheral and autonomic neuropathy), cut (macroglossia, malabsorption/weight loss, perforation, haemorrhage, obstruction and hepatomegaly)nerves (peripheral and autonomic neuropathy), GI (macroglossia, malabsorption/weight loss, perforation, haemorrhage, obstruction and hepatomegaly), vascular (Purpura, especially peri-orbital)
- associated with myeloma
- Rx: optimise nutrition; oral melphalan + prednisolone extends median survival from 13 months to 17
(2) AA amyloid (secondary amyloidoses)
Amyloid derived from amyloid A, an acute-phase protein, reflecting chronic inflammation in RA, UC/Chron’s, familial Mediterranean fever and chronic infections - TB, bronchiectasis, osteomyelitisTB, bronchiectasis, osteomyelitis
- kidney, liver, spleen affected
- may present with proteinuria, nephrotic syndrome or hepato-splenomegaly
- Macroglossia not seen and cardiac involvement rare
- Manage the underlying condition optimally
Diagnosis: biopsy of affected tissue and positive conger red stain with a red green birefringence under polarised light microscopy
- isotope scan will also show areas of deposition
What is Behcet’s disease
Features
Diagnosis
Management
Systemic inflammatory disorder of unknown calls, associated with HLA-B5/B51
Features
Cardinal feature equals oral ulceration. Diagnosis requires oral ulceration and any two of:
- Genital ulcers
- Defined eye lesions (including anterior/posterior uveitis or retinal vasculature)
- Defined skin lesions (erythema nodosum, popular pet pustular lesions)
- Positive skin pathergy test
- Oral ulcers – Aphthous or herpetiform
Other manifestations: mono/poly arthritis affecting knees, ankle, wrists and elbows; Gastro symptoms: diarrhoea, Abdo pain and anorexia; pulmonary and renal lesions, thrombophlebitis, vasculitis and a brainstem syndrome
Diagnosis
Mainly clinical
Pathogen test: needle prick leads to papule formation within 48 hours (highly specific)
Bloods: High ESR + CRP but not antibodies
Management
Colchicine for oral/genital ulcers
Azathioprine or Cyclophosphamide for systemic disease
Gout
Definition
Presentation
Causes
Associations
Investigations
Management
Disorder of curing metabolism characterised by hyperuricaemia. Deposition of monosodium urate crystals in joints – acute arthritis
Presentation
Typically acute mono arthropathy with severe joint inflammation
– MTP joint of the big toe (pad agra)
– other joints affected: ankle, foot, small joints of hand, wrist, elbow or knee
- Can be precipitated by trauma, surgery, starvation, infection or diuretics.
Long-term (chronic tophaceous gout). Urate deposits = top e.g. in pinna, tendons and joints + renal disease (stones, interstitial nephritis may occur)
Causes
DART: Diuretics, Acohol, Renal disease, Trauma
Hereditary, high dietary purines, alcohol execess, diuretics, salicyclates, cytotoxic’s
Associations
CVD, HTN, DM, chronic renal failure
Investigations
Light microscopy of synovial fluid: negatively birefringent Urate crystals
Serum urate: May be raised
High WCC + ESR/CRP in acute attack
Radiographs: only soft tissue swelling in early stages. Later, well-defined punched out emotions are seen in juxta articular bone. No sclerosis and joint spaces are preserved until lateonly soft tissue swelling in early stages. Later, well-defined punched out erosions are seen in juxta articular bone. No sclerosis and joint spaces are preserved until late
Gout management
Acute
– NSAIDs e.g. diclofenac - continue until pain and inflammation subsides
– colchicine (if NSAIDs CI) 500 µg 2 to 3 times per day
– corticosteroids if they both don’t work
Chronic
– Allopurinol: xanthene oxidase inhibitor
- Febuxostats - 2nd line xanthene oxidase inhibitor
Pseudogout definition
Presentation
Risk factors
Investigation findings
Management
Features
- typically of larger joints in elderly patients
– usually spontaneous and self-limiting but can be provoked by illness, surgery or trauma
Polarised light microscopy of synovial fluid shows weekly positively birefringent crystals.
- Radiographs: soft tissue calcium deposit (chondrocalcinosis)
– high white cell count and ESR/CRP in acute attack
Risk factors
- Hyperparathyroidism
- Haemochromatosis
- Acromegaly
- Wilson’s disease
Management
- Acute: NSAIDs, PO/Intrarticular steroids, (colchicine doesn’t work as well)
Chronic: As for RA
Vasculitis
Definition
Classification
• Idiopathic Autoimmune-driven inflammation, caused by inflammatory cell infiltration of the blood vessel wall, resulting in fibrinoid necrosis. Often granuloma formation. Can have severe consequences
o Vessel stenosis –> occlusion and distal infarction
o Aneurysm formation –> rupture of vessels and haemorrhage
• ANCA (antineutrophil cytoplasmic antibodies) are specific for vasculitis and help classification. – They bind to enzymes in the cytoplasm of neutrophils. 2 associated antigen/antibody subtypes:
(1) Anti-P3 antibodies (a-ANCA)
(2) Anti-MPO (p-ANCA
Classifications Large: GCA/PMR, Takayasu's arteritis Medium: PAN, Kawasaki Small (ANCA+): MPA, GPA, EGPA Small (ANCA-): HSP, Essential cryoglobulinaemia
GCA
Definition
Epidemiology
Clinical features
Investigation
Management
Definition
Vasculitis of aorta and major branches with a predilection for the branches of the external carotid (especially temporal artery) and internal carotid (especially ophthalmic artery)
Epidemiology
• Occurs in the elderly (>50 but generally >60yo)
• 2x as common in women
• Often co-exists with PMR
Clinical features
• Systemic upset
• Headache (usually unilateral, temporal) + scalp tenderness
• Jaw claudication (pain on chewing food)
• Visual disturbance (blurring/loss of vision)
• Polymyalgia symptoms.
• Temporal artery/scalp tenderness, thickening and/or loss of pulsation
Investigation
The diagnosis is clinical
• Raised CRP: ESR not reliable without CRP here
• Temporal artery biopsy (ideally within 7-14d of starting steroids): Inflammatory cell infiltrates, giant cells and granulomas
o Skip lesions may occur so don’t be put off if -ve (10%)
• Anaemia: Common feature
Management
• Prednisolone
o 60mg/day for GCA
o reduce dose by weekly decrements of 5mg. Once 10mg reached, reduce by 1mg every 2-4 weeks.
• Give prophylaxis against steroid induced osteoporosis: Bisphosphonates, Calcium and Vit D + GI protection
• Monitor bloods and clinical symptoms
• Consider steroid sparing agent (eg methotrexate or azathioprine) if significant dose of prednisolone required to control disease activity
PMR
Definition
Epidemiology
Clinical features
Investigation
Management
Clinical syndrome of proximal limb girdle pain and stiffness in middle-aged/elderly patients associated with an acute phase response and a rapid response to steroids
Epidemiology
• Mean age 70
• Female: male of 2:1.
Clinical features
• Onset usually sudden but sometimes insidious
• Systemic - fever, fatigue, anorexia, weight loss
• Bilateral proximal muscle pains (shoulder girdle and pelvis) and early morning stiffness
• Synovitis sometimes can occur.
• Muscle strength normal but difficult to assess because of pain
Investigation
• No specific test – combination of clinical findings, raised inflammatory markers
• Raised CRP, (ESR), ALP, normochromic normocytic anaemia etc
Management
• Prednisolone 15mg daily - rapid improvement (24-48 hours) with subsequent normalisation of abnormal blood tests.
• If poor response, alternative diagnosis should be considered.
• Gradually reduce steroids, titrating dose with symptoms and CRP
• Be aware of steroid toxicity - bone and GI protection, diabetes, hypertension
Takayasu’s arteritis
Definition
Epidemiology
Clinical features
Investigation
Management
A granulomatous arteritis of the aorta and great vessels causing stenosis, occlusion or aneurysm of the artery.
–> HTN, Absent peripheral pulses, strokes and HF
LARGE VESSEL VASCULITIS
Epidemiology
• Most common in Asia, particularly Japan.
• 9 x more common in female
• Seen in young adults up to the age of 40.
Clinical features
• Systemic: fever, weight loss, arthralgia
• Occlusive symptoms due to ischaemia
• Vascular: Claudication of affected limb; HTN
• Neuro: Dizziness, headaches, visual disturbance, TIA/Stroke
• Cardiac: Angina, dyspnoea (HF)
• Other: Peripheral pulses may not be palpable, arterial bruits over any large artery
Investigation
KEY = BP DIFFERENCE >10mmHg between arms
• Raised inflammatory markers
• MR-angiography
• PET scan – used FDG as a marker for tissue with high glucose uptake
• Biopsy (temporal artery may be involved sub clinically)
Management
• Prednisolone, usually at a starting dose 1 mg/kg/day – gradually tapered
• Steroid sparing agents eg methotrexate, azathioprine
• Tocilizumab – refractory disease
• Surgical intervention of vascular stenoses: should not be attempted until immunosuppression given and inflammation controlled.
POLYARTERITIS NODOSA
Definition
Epidemiology
Clinical features
Investigation
Management
Necrotising arteritis of medium sized vessels (main visceral arteries) – associated with microaneurysm formation, thrombosis and infarction
Epidemiology
• Predominantly middle aged men.
• Association with Hepatitis B (or less commonly C) infection
Clinical features
• Constitutional: Fever, malaise
• CNS: CVA, mononeuritis multiplex
• Cardiac: MI
• GI: GI perforation, haemorrhage
• Renal: Haematuria, malignant hypertension
• Skin: Infarcts, nodules
• Testes: Pain
• Perinuclear-antineutrophil cytoplasmic antibodies (ANCA) are found in around 20% of patients with ‘classic’ PAN
• Hepatitis B serology positive in 30% of patients
Systemic vasculitic symptoms in the presence of hepatitis B signs and in the absence of pulmonary symptoms/signs suggests a diagnosis polyarteritis nodosa
Investigation
• Angiography: Microaneurysms in hepatic, intestinal or renal vessels
• Biopsy: Of affected organ, often kidney
• Raised inflammatory markers
• ANCA -ve
Management
• Control BP and refer
• High dose steroids
• Cyclophosphamide (severe life threatening disease)
• DMARDs (azathioprine, MTX, leflunomide)
• Hep B related PAN – plasma exchange + lamivudine
Granulomatosis with Polyangitis
Definition
Clinical features
Investigation
Management
Necrotising granulomatous vasculitis affecting small vessels usually with involvement of the upper and lower respiratory tract.
Previously called Wegener’s granulomatosis
Clinical features
• ENT:
o Nasal discharge, sinusitis, nasal mucosal ulceration (saddle nose)
o Otitis media, deafness
o Hoarseness, stridor
• Lungs:Cough, haemoptysis, pleuritic pain
• Renal: Glomerulonephritis
Investigation
• cANCA +ve ; Anti-PR3 (proteinase 3) antibodies +ve
• CXR (infiltrates, nodules, cavitation)
• Biopsy of kidney/lung/nasal tissue shows necrotising granulomas
Management
• Systemic immunosuppression and support of affected organs such as the kidneys.
• Induction treatment: steroids, cyclophosphamide/rituximab (organ threatening disease), plasma exchange (life threatening disease)
• Maintenance treatment: azathioprine / mycophenolate mofetil / methotrexate
Microscopic polyangitis
Definition
Epidemiology
Clinical features
Investigation
Management
Necrotising vasculitis of small vessels. No granulomas.
Glomerulonephritis and pulmonary capillaritis common.
Clinical features • Constitutional Fever, malaise • Lung: Pulmonary infiltrates, alveolar haemorrhage • Renal: Glomerulonephritis • Nerves: Mononeuritis multiplex
Investigation
• pANCA +ve; Anti-MPO (myeloperoxidase) antibodies +ve
• Biopsy of affected organ
Management
Systemic immunosuppression and support of affected organs such as the kidneys.
• Induction treatment: steroids, cyclophosphamide/rituximab (organ threatening disease), plasma exchange (life threatening disease)
• Maintenance treatment: azathioprine / mycophenolate mofetil / methotrexate
