Rheumatology

Rheumatoid arthritis - antibodies? When would you do XRs? XR findings?

Antibodies – RF 70-80%, Anti-CCP antibody (test for this if suspecting and RF negative)

XR – hands and feet of all pts with suspected RA

-          Periarticular osteopenia

-          Bony erosions

-          Soft tissue swelling

-          Joint destruction, loss of joint space

RA - disease activity score? Management - initial, assessing response, flare, 2nd line treatment?

DAS28

Management:

Initially DMARD monotherapy (methotrexate, sulfasalazine) +/- short course prednisolone

CRP and DAS28 to assess response

Flare – oral/IM corticosteroids

TNF-inhibitor (infliximab) if inadequate response to at least 2 DMARDs including MTX

Next line - Rituximab

What is Felty’s syndrome?

Triad of RA, splenomegaly, neutropenia

Methotrexate - adverse affects? Co-prescribed with? Monitoring? What meds would you avoid?

        ·        Adverse effects – myelosuppression, pneumonitis, liver fibrosis

        ·        Taken weekly – co-prescribe with folic acid – not taken on same day

        ·        FBC, U&E, LFT monitoring before starting, weekly until stabilised and then every 2-3 months

        ·        Avoid with trimethoprim, co-trimoxazole – risk of marrow aplasia

Osteoarthritis - XR changes? Management?

        ·        XR – loss of joint space, subchondral sclerosis, subchondral cysts, osteophytes forming at joint margins

        ·        Management – topical NSAIDs → oral NSAIDs

Ankylosing spondylitis - gene? Feature? XR changes? Management?

  HLA-B27

Reduced lateral flexion of lumbar spine

XR – sacroiliitis, squaring of lumbar vertebrae, bamboo spine (late, uncommon), syndesmophytes, CXR – apical fibrosis

NSAIDs are 1^st line management. DMARDs only useful if peripheral joint involvement

SLE - type of hypersensitivity reaction? ESR and CRP? Associated antibodies and complements? Management?

        ·        Type 3 hypersensitivity reaction

        ·        High ESR, normal CRP

        ·        ANA, anti-dsDNA, anti-Ro, anti-Sm, anti-a

        ·        Low C3, low C4

        ·        Hydroxychloroquine – retinopathy risk – baseline ophtho exam and annual screening

        ·        Corticosteroids, immunosuppressants if severe

Psoriatic arthritis - which joints? XR changes?

        ·        DIPS (spared in RA)

        ·        XR – periostitis, ankylosis, osteolysis, dactylitis, pencil-in-cup deformity

Reactive arthritis - triad? Causative organisms? When do sx present and how long do they last?

        ·        Arthritis, urethritis, conjunctivitis

        ·        Post-STI (chlamydia), post-dysenteric (shigella, salmonella)

        ·        Sx within 4 weeks of initial infection, last 4-6 months

Anti-phospholipid syndrome - features? Associated antibodies? APTT?

        ·        Venous and arterial thromboses, recurrent fetal loss, thrombocytopenia

        ·        Antibodies – lupus anticoagulant, anticardiolipin, anti-beta-2 glycoprotein I – at least 1 on 2 occasions 12 weeks apart

        ·        Paradoxical rise in APTT – prolonged

Antiphospholipid syndrome - primary thromboprophylaxis? Secondary thromboprophylaxis? Pregnancy/planning pregnancy management?

        ·        Primary thromboprophylaxis – low-dose aspirin, LMWH in high-risk scenarios

        ·        Secondary thromboprophylaxis – initial VTE = lifelong warfarin INR target 2-3. Recurrent VTE while on warfarin = lifelong warfarin, target INR 3-4, consider adding aspirin

        ·        Arterial thrombosis = lifelong warfarin INR target 2-3

        ·        Pregnancy/planning = low dose aspirin plus LMWH

Sjogren’s - associated antibodies? Investigations? Management?

        ·        Anti-Ro, anti-La

        ·        RF 50%, ANA 70%

        ·        Schirmer’s test, histology – focal lymphocytic infiltration

        ·        Artificial saliva and tears, pilocarpine can stimulate saliva production

Systemic sclerosis - 3 types? What is affected in each? Associated antibodies with each?

Limited cutaneous

-          Raynaud’s, scleroderma affects face and distal limbs mostly

-          Subtype is CREST – Calcinosis, Raynaud’s, Esophageal dysmotility, Sclerodactyly, Telangiectasia

-          ANA, anti-centromere

Diffuse cutaneous

-          Scleroderma affects trunk and proximal limbs mostly

-          ANA, anti-scl-70

-          Common cause of death resp involvement, renal disease (captopril usually used – rapid onset and short half-life)

Scleroderma (without internal organ involvement)

-          Tightening and fibrosis of skin

Polymyalgia rheumatica - age? Onset? Symptoms? Management?

>60, onset <1 month

Morning stiffness proximal limb muscles

15mg prednisolone daily 1-2 years – usually respond dramatically

F/u after 1 week

Giant cell arteritis - features? Fundoscopy findings? Biopsy findings? Management?

Headache, jaw claudication

RAPD, can get amaurosis fugax

50% have PMR features

Fundoscopy – swollen pale disc and blurred margins

Temporal artery biopsy – granulomatous inflammation with multinucleated giant cells. If negative biopsy, cannot be fully ruled out due to skip lesions.

Start steroids before biopsy, same day ophtho review

40-60mg prednisolone (high dose steroids) daily if no visual/jaw symptoms

500mg-1000mg methylprednisolone daily otherwise

Treat for 1-2 years

Polymyositis/dermatomyositis - features? What is raised? Antibodies? Management?

Dermatomyositis - proximal muscle weakness (PMR doesn’t cause weakness), gottron’s papules on back of hands, heliotrope rash affecting eyelids

Poly is same but without skin involvement

CK raised

ANA positive

Anti-Jo-1 antibodies, anti-Mi-2 antibodies – myositis specific antibodies

Manage with high-dose steroids

Behcet’s disease - what is it? Triad? Gene variant? Investigations?

Complex multisystem disorder, presumed autoimmune-mediated inflammation of arteries and veins

Triad – oral ulcers, genital ulcers, anterior uveitis

Thrombophlebitis, DVT, arthritis, GI stuff 

Relapsing-remitting

HLA-B51

Oral and genital ulcers – red halo

Pathergy test suggestive of diagnosis, no definite test

Gout - drug causes? Diagnosing with uric acid? XR changes? Management of flare and long-term?

Drug causes – diuretics, ciclosporin, alcohol, pyrazinamide, aspirin

Uric acid >360 = diagnostic. <360 – if strongly suspected repeat 2 weeks after flare settled

XR – joint effusion, well-defined ‘punched-out’ erosions with sclerotic margins, preservation of joint space until late disease, no periarticular osteopenia (vs RA)

Flare – NSAID, colchicine

Allopurinol, febuxostat

Pseudogout - risk factors? XR changes?

Risk factors – dehydration, hyperparathyroidism, surgery, trauma, low magnesium

XR – chondrocalcinosis

Osteoporosis - if someone has had a fragility fracture when would you start bisphosphonates without DEXA?

>75

Paget’s disease - what is it? Blood results? XR findings? Complications? Management?

        ·        Excessive bone turnover

        ·        High ALP, normal calcium and phosphate

        ·        XR – osteolysis in early disease, mixed lytic/sclerotic lesions later

        ·        Skull XR – thickened vault, osteoporosis circumscripta

        ·        Can cause hearing loss, osteosarcoma, heart failure, spinal stenosis

        ·        Bisphosphonates

Osteomalacia - what is it? Blood results? XR findings? Management?

Softening of bones due to low vit D which causes decreased bone mineral content. (rickets in kids)

Low vit D, low phosphate, low calcium

High PTH, high ALP

XR – translucent bands (Looser’s zones or pseudofractures)

Osteogenesis imperfecta - inheritance pattern? Features? Blood results?

Autosomal dominant

Childhood onset, fractures following minor trauma

Blue sclera, deafness.

Normal calcium, phosphate, PTH, ALP

Calcium, phosphate, ALP, PTH in 1) Osteoporosis? 2) Osteomalacia? 3) Primary hyperparathyroidism? 4) CKD? 5) Paget’s disease?

Ehlers-Danlos syndrome - inheritance pattern? What does it affect? Features? Scoring system? Ix? Management?

        ·        Autosomal dominant – mostly affects type III collagen

        ·        Hypermobility and POTS, fragile skin, easy bruising, AR, mitral valve prolapse

        ·        Beighton score

        ·        ECHO recommended, can do genetic testing

        ·        Management – supportive, CVS monitoring

Marfan’s syndrome - inheritance pattern - features?

Autosomal dominant

Tall, high arched palate, arachnodactyly, pectus excavates, scoliosis, heart disease – dilatation of aortic sinuses, mitral valve prolapse, lungs – pneumothoraces, eye issues

Benign bone tumours? (3)

Osteoma – overgrowth of bone, typically on skull

Osteochondroma – bony projection on external surface of bone

Giant cell tumour – epiphyses of long bones, XR shows double bubble appearance

Malignant bone tumours? (3)

Osteosarcoma metaphyseal region of long bones prior to epiphyseal closure, XR shows Codman triangle and sunburst pattern

Ewing’s sarcoma – pelvis and long bones, XR shows ‘onion skin’ appearance

Chondrosarcoma – axial skeleton

Vasculitis - Small? Medium? Large?

Small

-          HSP – no thrombocytopenia!!

-          Microscopic polyangiitis – P-ANCA

-          Granulomatosis with polyangiitis – C-ANCA

-          Eosinophilic granulomatosis with polyangiitis – P-ANCA, high ESR, late-onset asthma

Medium

-          Polyarteritis nodosa – renal impairment, HTN, CVD, tender skin nodules

-          Kawasaki disease

Large

-          Giant cell arteritis – high ESR

-          Takayasu’s arteritis – aortic arch affected, pulseless disease

Before starting biologics what test would you do?

IGRA as can reactivate TB

What test would you do before starting azathioprine?

TPMT test to look for individuals prone to toxicity (TPMT metabolises it)

CFS - duration of sx for a diagnosis?

3 months

Raynaud’s management?

CCB 1^st line e.g. nifedipine