Rheumatology Flashcards by Hari Babu

What are the typical ages of patients affected with giant cell arteritis?

Primarily affects people aged >50

Peaks at around age 80

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Which arteries are typically affected by GCA? Name 5 arteries/groups of arteries.

External carotid branches (e.g. temporal and occipital arteries)

Ophthalmic

Vertebral

Distal subclavian and axillary arteries

Thoracic aorta

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What is the pathogenesis of GCA?

Antigenic stimulation via danger pattern recognition on vessel-specific Toll-like receptors on vascular dendritic cells in artery adventitia

CD83+ activated vascular dendritic cells produce T cell-attracting chemokines (CCL19 and CCL21) drawing T cells into the media

Peripheral blood monocytes differentiate into macrophages in vessel wall and produce IL-6 and IL-1B, as well as IFy, CXCL9, 10 and 11, PDGF, FGF and VEGF

IL-6 induces T cells to differentiate into Th17 cells

Vasc smooth muscle cells express NOTCH ligands, which interact with NOTCH receptors on T cells and amplify inflammatory response, switching to synthetic myofibroblasts that proliferate in intima, narrowing vessel lumen

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What are the 4 different clinical patterns of GCA?

Isolated cranial GCA (headache, jaw claudication, scalp tenderness) - most cases

Symptomatic LVV (claudication, pulseless limb) with or without cranial signs

Isolated fever or inflammatory response

Isolated PMR with vasculitis on imaging

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Do GCA patients with LVV require higher or lower doses of steroids?

Higher

Typically tend to be younger, less likely to present with headaches, lower risk of visual loss

However, higher risk of relapse

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Which arteries are typically occluded in GCA that result in visual loss?

Posterior ciliary arteries, resulting in anterior ischaemic optic neuropathy

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What are the diagnostic criteria for GCA as per the American College of Rheumatology?

3 of 5 present:

Age at disease onset >50 years

New headache - localised

Temporal artery abnormality

Elevated ESR by >50

Abnormal artery biopsy

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What 3 acute phase reactants would you measure in GCA, and what are the estimated optimal cut-off levels?

ESR - 50

CRP - 20

Platelets - 300

CRP + platelets = good diagnostic utility for GCA

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What is the most significant long term risk of GCA?

Aortic aneurysm

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As well as steroids, what medications should be considered for treatment of GCA? Name 7.

Aspirin - positive role in cerebrovascular disease

Methotrexate

Cyclophosphamide

Azathioprine

Leflunomide

Tocilizumab (anti IL-6)

Ustekinumab

TNFa not recommended for GCA

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What are the mandatory (3) and additional criteria for diagnosing polymyalgia rheumatica (6)?

Mandatory

age > 50
aching in both shoulders
abnormal CRP/ESR, or both

Additional (> 4 points w/o USS, > 5 with USS)

morning stiffness lasting >45 mins (2 points)
hip pain or reduced range of motion (1 point)
negative RF/CCP (2 points)
absence of peripheral synovitis (1 point)
USS findings - one shoulder with subdeltoid bursitis, biceps tenosynovitis, or glenohumeral synovitis, or at least one hip with synovitis or trochanteric bursitis (1 point)
subdeltoid bursitis, biceps tenosynovitis, or glenohumeral synovitis in both shoulders (1 point)

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What are the cut-offs for PMR activity score (PMR-AS)?

PMR-AS < 7 is low disease activity

PMR-AS > 10 defines a flare and suggests adjustment in glucocorticoid dosage

PMR-AS > 17 is high disease activity

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How is PMR treated?

Prednisolone 15 mg/day for 4 weeks, then reduced by 2.5 mg every 2 weeks to 10 mg and subsequent reduction by 1 mg per month

Also can give methotrexate to use less GC, particularly to reduced osteoporotic fracture risk

Etanercept has a modest benefit in GC naive PMR patients

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Name 5 extra-articular features associated with axial sponyloarthritis.

Anterior uveitis - common

IBD

Psoriasis

Apical fibrosis

Aortic regurgitation

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What are the typical XR changes you can expect to see in the spine with AS? Name 3.

Squaring

Syndesmophytes (will progress to bridging syndesmophytes)

Romanus lesion (shiny corners)

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When can patients with axial spondyloarthritis commence biologics?

After failing 12 weeks of NSAID and exercise therapy with on-going active disease

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Name 6 biologics that can be used in the treatment of axial spondyloarthritis.

Adalimumab

Certolizuma pegol

Etanercept

Golimumab (funded by PBS)

Infliximab

Secukinumab is effective for AS (IL-17A inhibitor)

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What are the PBS funding criteria for golimumab on non-radiographic axial spondyloarthropathy?

nr-axSpA as defined by ASAS criteria

Elevated inflammatory markers

Sacroiliitis with inflammation or oedema on non-contrast MRI

Bone marrow oedema on STIR and T1 images (non-contrast)

Failed NSAIDs and exercise for 3 months

BASDAI 4 or more

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What are the classification criteria for axial spondyloarthritis?

Patients must have at least 3 months of back pain with age of onset < 45

Sacroiliitis on imaging plus at least 1 SpA feature OR HLA-B27 positive plus at least 2 other SpA features:

inflammatory back pain
arthritis
enthesitis
uveitis
dactylitis
psoriasis
Crohn’s/colitis
good response to NSAIDs
family history for SpA
HLA-B27
elevated CRP

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What are the classification criteria for peripheral spondyloarthritis?

Arthritis, enthesitis or dactylitis PLUS

1 or more SpA features

uveitis
psoriasis
Crohn’s/colitis
preceding infection
HLA-B27
sacroiliitis on imaging

OR at least 2 other SpA features

arthritis
enthesitis
dactylitis
inflammatory back pain ever
family history for SpA

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What is the definition of a positive MRI sacroiliac joint?

Subchondral bone marrow oedema

Acute (bilateral) sacroiliitis

STIR sequencing

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How is psoriatic arthritis diagnosed?

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Presence of established inflammatory articular disease (peripheral arthritis, enthesitis or axial) plus three points from:

present skin psoriasis (2 points)
past of family Hx of psoriasis (1 point)
dactylitis (1 point)
nail changes - pitting, onycholysis (1 point)
RF negative (1 point)
juxta-articular new bone formation on XR (1 point)

What correlates better to psoriatic arthritis, skin or nail disease?

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Nail disease

Name 2 co-morbidities associated with psoriatic arthritis.

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Cardiovascular disease

Metabolic syndrome

Obesity additionally is associated with worse outcomes

What treatments can be given for psoriatic arthritis? 6 categories

NSAIDs - symptom relief CsDMARDs - methotrexate, sulfasalazine, leflunomide (but little data for these) Anti-TNF - adalimumab, certolizumab pegol, etanercept, golimumab, infliximab Anti-IL17 - secukinumab, ixekizumab Anti-p40 subunit IL12/23 - ustekinumab Anti-p19 subunit IL23 - guselkumab Also apremilast, a phosphodiesterase 4 inhibitor, but this is not PBS listed

Name 5 organisms that can cause reactive arthritis.

Chlamydia Shigella Salmonella Campylobacter Yersinia

Name 8 extra-articular manifestations of reactive arthritis.

Keratoderma blennorrhagica Circinate balanitis Mouth ulcers Conjunctivitis Uveitis Aseptic urethritis Cervicitis Prostatitis

What are the treatment options for reactive arthritis? 5 options.

If chlamydia - treat, contact tracing NSAIDs Intra-articular glucocorticoids (systemic if unwell) DMARDs - sulfasalazine for chronic disease, or methotrexate

Name 3 features of IBD that might predispose patients towards having spondyloarthritis.

Having other extra-intestinal features Complications of bowel disease Large bowel involvement (for Crohn’s)

What is the most likely cause of death in systemic sclerosis?

Pulmonary arterial hypertension

What pattern of interstitial lung disease is most likely to be seen in systemic sclerosis?

Non-specific interstitial pneumonitis

Name 2 high risk phenotypes in systemic sclerosis for the development of ILD.

Early dc-SSc and anti-Scl70 Early dc-SSc and elevated CRP

How is SSc-ILD graded via HRCT?

<20% = limited disease >20% = extensive disease Indeterminate percentage + FVC 70% or above = limited disease Indeterminate percentage + FVC <70% = extensive disease

How often should patients with early diffuse SSc with ILD be monitored?

Use spirometry and DLCO Every 3-4 months for 3-5 years after disease onset, then yearly No advantage in serial HRCT scans if PFTs are stable

What are the options for treatment of SSc-ILD?

Mycophenolate 3g/day or if intolerant, mycophenolic acid Oral or IV cyclophosphamide for 6-12 months followed by MMF or azathioprine for severe or progressive disease unresponsive to MMF Consider nintedanib

Which antibodies would you expect to see in scleroderma renal crisis?

Anti-RNA polymerase III

Which drug can precipitate scleroderma renal crisis?

Cyclosporin A

Name 4 classes of drugs that can be used in SSc-PAH for treatment.

PDE-5 inhibitors (sildenafil, tadalafil) Endothelin receptor antagonists (ambrisentan, bosentan, macitentan) Prostacyclin analogues (iloprost, treprostinil, IV epoprostenol for class III or IV disease) Riociguat No role for CCB monotherapy

What is the treatment of scleroderma renal crises?

Mainstay of treatment is ACEi Benefit of combining this with bosentan or plasma exchange If CNS involvement (encephalopathy) - add nitroprusside

Name 6 non-pharmacological therapies for treating Raynaud’s phenomenon.

Treat underlying cause e.g. cryoglobulinaemia Keep hands warm/hand warmers

Name pharmacological therapies for Raynaud’s phenomenon (3 stages).

First line -

Which SSc patients benefit from autologous HSCT?

Non-smokers non-responsive to standard treatment along with either: - dcSSc within the first 4-5 years with mild-to-moderate organ involvement - lcSSc with progressive visceral involvement

Name 2 complications from SSc affecting the small bowel

Pseudo-obstruction Bacterial overgrowth

Name 1 complication from SSc affecting the liver.

Primary biliary cirrhosis

Name 2 complications from SSc affecting the large bowel.

Wide-mouth diverticulae Faecal incontinence

Name 3 diseases that can result following TREX1 gene mutations.

SLE Aicardi-Goutiere’s syndrome - early onset encephalopathy RVCL - retinal vasculopathy with cerebral leukodystrophy

Name 4 complement deficiencies that can predispose patients to SLE.

C1q C2 C4A C4B 90% risk of SLE in homozygotes for C1q deficiency

Name 3 drugs that can cause drug-induced lupus where you are more likely to see anti-histone antibodies.

Procainamide Hydralazine Isoniazid

Name 3 features of Jaccoud’s arthropathy.

Ulnar deviation Swan-neck deformity Tenosynovitis

What is the most common manifestation of cardiac disease in SLE?

Pericarditis

What are the two most specific auto-antibodies seen in SLE, and what manifestations are they associated with?

Anti-Smith - renal and CNS disease Anti-dsDNA - disease activity, nephritis, TNFi induced disease

Name 4 triggers for SLE.

UV light/phototherapy Sulphonamides Stress Oestrogen-containing preparations

What is belimumab, and what is it used in?

Used in SLE mAb against BAFF/BLyS (B-cell activating factor/B lymphocyte stimulator)

Name 7 contra-indications to pregnancy in SLE.

Severe restrictive lung disease (FVC > 1L) Severe pulmonary hypertension (sPAP >50) Advanced renal insufficiency (>2.8 Cr mg/L) Advanced heart failure Previous severe pre-eclampsia or HELLP Stroke within last 6 months Severe disease flare within 6 months

Rheumatology Flashcards

(54 cards)