Name 3 signs of joint damage in rheumatoid arthritis
Ulnar deviation of fingers Dorsal subluxation at the wrist Boutonnieres deformity Swan neck deformity of fingers Z deformity of thumb Rupture of hand or feet extensor tendons
What immunological investigations should be done in suspected rheumatoid arthritis?
Rheumatoid factor
ANA
Anti-cyclic citrullinated peptide antibodies
What are the early radiological signs of rheumatoid arthritis?
Soft tissue swelling
Peri-articular osteopenia
Name three extra-articular complications of rheumatoid arthritis
Vasculitis Pleurisy Pericarditis Dry eye syndrome Felty's syndrome Anaemia Cervical myelopathy Infections
What are the principals of managing rheumatoid arthritis?
Refer to rheumatology early
Analgesia with NSAIDs + PPI
Don’t delay referral
Measure disease activity and screen for comorbidities
Give DMARDs early (methotraxate and another and short term steroids at first)
Biological therapies if 2 DMARDs tried for 6 months and failed
Encourage exercise
COnsider surgery if on maximum therapy but still uncontrolled pain, or significantly restricted function
What is the diagnostic criteria for lupus?
4 out of: Arthritis of at least 2 joints (non-erosive) Renal disorder ANA Serositis Haematological disorder Photosensitivity Oral and nasopharyngeal ulcers Immune disorder with positive ds-DNA and positive anti-phospholipid antbody Neurological eg seizures or psychosis Malar rash Discoid rash
Briefly describe the pathophysiology of SLE?
Inate susceptibility based on immune regulatory genes, complement levels, HLA type and hormone levels combine with environmental triggers such as drugs, UV exposure and microbial response (Eg to EBV)
This triggers autoimmune proliferation with hyperactive T and B cell activation, increased CD4:CD8, defective clearance of immune complexes and impiared tolerance
THis leads to production of autoantibodies with apoptosis, self-exposure and self-recognition
How should lupus activity be monitored?
Anti-dsDNA antibody (high) Complement levels (C3 low) ESR (high, CRP low) BP Urine for protein and casts FBC U&E LFTs
What are the features of the discoid rash of lupus?
Erthematous papules or pustules on head and neck. Leads to hyperpigmented keratotic papules and then atrophic, depressed scarring and depigmentation
What are the features of the malar rash of lupus?
Fixed erythematous flat or raised rash over malar eminences, sparing the nasolabial folds
What immune markers are positive in lupus?
ANA
Anti ds-DNA
ENAs inc Ro and La
Anti-phospholipid antibody
What are the features of antiphospholipid syndrome?
Seen in SLE. Antibodies cause coagulation defect, livedo reticularis, recurrent miscarrdiages and thrombocytopenia
What is the significance of a woman with SLE being Ro positive?
Ro can cross the placenta and cause congential heart block
What are the principles of managing SLE?
Hydroxyquinalone for joints and skin involvement.
DMARD such as methotrexate, azathioprine or ciclosporin, covered by prednisolone
Avoid sunlight and UV exposure
Avoid NSAIDs or use sparingly
Monitor bloods for cytopenias and toxicity of drugs
Monitor for opportunistic infections
Aggressive goals for BP and lipid levels to try and minimise cardiovascular risk
What are some causes of raised ANA?
Normal variant rheumatological disease Malignancy Hepatic disease Haematological disorder eg ITP Drugs eg isoniazid Pulmonary disease Chronic infection Other eg graves disease and T1DM
What is the triad of granulomatosis with polyangitis?
Renal disease
Lung disease
Upper airway involvement
What are some upper airway features of granulomatosis with polyangitis?
Tracheal obstruction (med emergency) Epistaxis Sinusitis Saddle nose from septal destruction Nasal obstruction
What are some renal features of granulomatosis with polyangitis?
Rapid glomerulonephritis with proteinuria and haematuria
What are some pulmonary features of granulomatosis with polyangitis?
Cough
Hameoptysis that may be severe in pulmonary haemorrhage
Other than the main triad, what are some other features of granulomatosis with polyangitis?
Arthritis
Peripheral neuropathy
Scleritis
Purpura
What investigations should be done in suspected granulomatosis with polyangitis and what will there abnormalities be?
FBC and plasma viscosity (PV raised) U&Es, LFTs and CRP (CRP raised ANCA (positive) Urinalysis - proteinuria and haematuria CXR - may be signs of pulmonary haemorrhage Lung function tests CT of sinuses and orbit COnsider renal biopsy - granulomata, glomerular necrosis, vasculitis
How should granulomatosis with polyangitis be managed?
Induce remission with high dose steroids and either ciclophosphamide or methotrexate
Maintain remission with low dose steroids and either methotrexate or azathioprine
What are the main features of polymyalgia rheumatica?
Pain, aching, tenderness and stiffness of pelvic and shoulder girdle, proximal muscle and neck (symmetrical and bilateral)
Fatigue, anorexia, malaise
Depression
Rapid response to steroids
How should polymyalgia rheumatica be managed?
15mg oral prednisolone slowly weaned over two years (+bisphosphonates, vit D, calcium and PPI)
What are the main features of giant cell arteritis?
New onset headache that's usually temporal Temporal artery and scalp tenderness Jaw claudication Amaurosis fugax \+/- signs of polymyalgia rheumatica
How should giant cell arteritis be managed?
40-60mg prednisolone asap, weaned for around 2 years. SHould be response within a week
How is suspected giant cell arteritis investigated?
ESR (Raised)
Temporal artery biopsy - giant cell granulomas
What is the pattern of colour change found in Raynaud’s phenomenon?
White from ischaemia
Blue from stasis of blood
Red from reactive hyperaemia
Name 3 causes of secondary Raynaud’s
SLE Systemic sclerosis Rheumatoid arthritis Polymyositis Vibration injury Atheroma obstruction THrombocytosis Polycythaemia vera Beta blockers Hypothyroidism
How is Raynaud’s managed?
Stop smoking
Hand warmers and warm socks
Vasodilators eg nifedipine
Surgical sympathectomy if severe
What is the pathology behind sjogren’s syndrome?
Autoimmune lymphocytic infiltration of exocrine glands, especially salivary and lacrimal. Polyclonal B lymphocytes are hyperreactive causing immune complexes and autoantibody formation
What antibodies are associated with sjogren’s syndrome?
Ro and La (predict systemic involvement
Rheumatoid factor
What is schirmer’s test?
USed to investigate possible sjogrens and assesses conjunctival dryness. Piece of filter paper placed under the lower lid and the distance that tears travel is measured. If less than 5mm in 5 minutes, test is positive
What is the typical feature of polymyositis?
Insidious, progressive, symmetrical and proximal muscle weakness
Apart from muscle weakness, name 3 features of polymyositis
Dysphonia
Dysphagia
Shortness of breath
What autoantibodies are involved in polymyositis?
ANA positive
Anti jo 1
How should polymyositis be managed?
Screen for malignancy COrticosteroids Bisphosphonates Immunosuppression eg with methotrexate Hydroxychloroquine for rash
Describe the pathophysiology of systemic sclerosis
Endothelial cell injury and vascular dysfunction of small and medium vessels
Immunologic derangement with perivascular infiltration of T cells
FIbroblast activation with excess production and deposition of collagen causing progressive tissue fibrosis and occlusion of microvasculature
What are the features of limited systemic sclerosis?
Calcinosis Raynauds Oesophageal and gut dysmotility Sclerodactyly Telangiectasia Microstomia Abnormal nail fold capillaries Pulmonary hypertension
What antibodies are associated with systemic sclerosis?
ANA
Anti-centromere antibodies
Anti-topoisomerase in diffuse
What is the pathology of gout?
Occurs due to a disorder in the metabolism of purines (into urate) either by reduced renal excretion or increased production
Increased levels of urate in the blood
Urate crystals are deposited at joints, connective tissue and urinary tract
Name 3 causes of gout
High dietary purine content Hereditary Diuretics Leukaemia Excess alcohol intake Cytotoxic agents and tumour lysis Renal impairment
How is gout diagnosed on microscopy?
Polarised light microscopy shows urate cystals (needle shaped) that are negatively birefringent
How is gout differentiated from psuedogout on microscopy?
Pseudogout involves rhomboid shaped crystals that are positively birefringent
What are the features of gout on x ray?
Soft tissue swelling initially
Chondrocalcinosis
Punched out erosions of peri-articular bone
How should an acute attack of gout be managed?
Rule out septic arthritis Self care if mild Strong NSAID eg diclofenac +PPI. Or Colchicine. Steroids if Renal impairment Paracetamol +/- codeine Measure urate after 4-6 weeks
How can recurrences of gout be prevented?
Lifestyle advice
Allopurinol if recurrent attacks or tophi/renal impiarment/renal stones
NSAID for at least 1 month to cover period of increased risk of attack
What are the 4 seronegative spondyloarthropathies?
Psoriatic arthritis
Ankylosing spondylitis
Enteropathic spondylitis
Reiter’s syndrome
Give 5 shared features of the seronegative spondyloarthropathies
Axial arthritis Rheumatoid factor negative Asymmetrical large joint oligo- or monoarthritis Enthesitis Dactylitis Extra-articular features
Which joints are predominantly affected in psoriatic arthritis?
Distal interphalangeal joints
What is arthritis mutilans?
Resoprtion of the distal phalynx
What changes are found on x ray in psoriatic arthritis?
Erosive changes
Osteolyis
Periosteal reaction
How is psoriatic arthritis managed?
NSAIDs
Corticosteroid injections
DMARDs, especially sulfasalazine and methotrexate
What is reiter’s syndrome?
A triad of conjunctivitis, arthropathy and urethritis
Name two organisms that commonly cause reactive arthritis?
Chlamydia trachomatis
Shigella
Campylobacter
Salmonella
How is reactive arthritis managed?
NSAIDs
Antibiotics if organism can be isolated or steroid injections
DMARD if no response in 6 months
What are the main features of the pain of ankylosing spondyloarthritis?
Lower back, radiating to the hips and buttocks Worse at night and on waking Improved by activity Gradual onset May wake them up at night
What features are found on x ray in ankylosing spondylitis?
Sacroiliitis - sclerosis and erosions
Syndesmophytes of the vertebra
Ligamentous calcification of the spine
What is the pharmacological management of ankylosing spondylitis?
NSAID
anti-TNF
?steroid injection
?DMARD
Give three features that should raise your suspicion of ankylosing spondylitis in a person with chronic lower back pain
Under 45 Present for >3 months Family history of spondyloarthropathy Inflammatory stiffness Buttock pain Costochondritis Enthesitis Asymmetric lower limb arthritis Anterior uveitis Symptoms wake them at night Rapid response to NSAIDs
Give three risk factors for the development of fibromyalgia?
Female
Divorced
Low household income
Low educational status
What is the diagnostic criteria for fibromyalgia?
Chronic (>3 months), widespread pain associated with unrefreshing sleep and fatigue in the absence of inflammation and with at least 11 tender points on palpation
What are the x ray features of osteoarthritis?
Loss of joint space
Osteophyte formation
Subchondral sclerosis
Subchondral cyst formation
What are the x ray features of rheumatoid arthritis?
Loss of joint space
Periarticular erosions
Subluxation
Juxta-articular osteoporosis
What are the x ray features of gout?
Soft tissue tophi
Punched out juxta-articular erosions with sclerotic edges
Eccentric erosions
Joint effusion
What are the x ray features of psoriatic arthritis?
Pencil in cup deformity Juxtaarticular periostitis Subluxation Enthesitis Marginal bone erosions Bone proliferation
What is the joint distribution of osteoarthritis of the hands?
Carpometacarpal
PIP
DIP
What is the joint distribution of rheumatoid arthritis of the hands?
MCP
PIP
What is the joint distribution of psoriatic arthritis of the hands?
DIP
Give 3 risk factors for the development of pseudogout
Increasing age Hypothyroidism Hyperparathyroidism Diabetes mellitus Haemochromatosis Wilson's disease Acromegaly Low magnesium Low phosphate
How is pseudogout differentiated from gout?
Pseudogout usually occurs in large joints, whereas gout is usually in smaller joints
On microscopy, gout shows negatively birefringent needle shaped urate crystals and pseudo-gout shows positively birefringent rhomboid CPPD crystals
What x ray changes are seen in pseudogout?
Chondrocalcinosis
How is psuedogout managed?
Similarly to gout
NSAIDs and analgesia
Steroids if necessary
What monitoring is required for methorexate?
Baseline and regular fbc, U&Es and LFTs
How should methotrexate be prescribed?
Weekly doses titrated to effect with folic acid co-prescribed
What monitoring is required for sulfasalazine?
Baseline FBC and LFTs and regularly monitor for the first 6 months of treatment
What is the mechanism of action of azathioprine?
COnverted to 6-mercaptopurine analogue which inhibits the synthesis of DNA and RNA
What monitoring should be done for azathioprine?
Baseline TPMT status, LFTs, FBC and U&Es
Regular monitoring of FBC and LFTs
What is the most important side effect of hydroxychloroquine use?
Retinopathy