Rheumatology Flashcards
Rheumatoid arthritis
symmetrical, painful swollen joints in the hands and feet (DIPJ not affected)
morning stiffness lasting >30 minutes
positive squeeze test
rheumatoid nodules - lungs, elbow and knuckles
anti-CCP
extra-articular manifestations of RA
ocular: keratoconjuctivitis, episcleritis, scleritis, corneal ulceration
respiratory: pulmonary fibrosis/nodules, pleural effusion, infection secondary to immunosuppression, methotrexate pneumonitis
CVS: pericarditis, vasculitis, peripheral neuropathy
systemic: fatigue, fever, lymphadenopathy, weight loss, PO, depression
X-ray changes in RA
early: loss of joint space, juxta-articular OP, soft tissue swelling
late: periarticular erosions and subluxations
poor prognostic features in RA
positive RF, poor functional status at presentation, anti-CCP, HLA DR4, X-ray showing early erosions (<2years), insidious onset, rheumatoid nodules, being female
management of RA
2 DMARDs (one of which is methotrexate) and a short-term glucocorticoid e.g. methotrexate + sulfasalazine + prednisolone
TBF-inhibitors e.g. infliximab used if that inefficient
Reactive arthritis
aka Reiter’s syndrome
HLA-B27 associated seronegative spondyloarthropathy
typically develops <4 weeks post (sexually transmitted) infection
no organism can be retrieved from joint aspiration
classical triad: urethritis, conjunctivitis and arthritis
Rx: analgesia, NSAIDs, intra-articular steroids
sulfasalazine and methotrexate if persistent disease (symptoms rarely last more than 12 months)
Gout
caused by chronic hyperuricaemia
risk factors include CKD, DM, obesity, thiazide diuretics, furosemide, alcohol and Lesch-Nylan syndrome
affects small joints
causes needle-like uric acid crystals that are negatively birefringent
Rx: NSAIDs, colchicine (causes diarrhoea) and allopurinol prophylaxis
Pseudo-gout
risk factors include hyperparathyroidism, hypothyroidism, haemochromatosis, acromegaly, Wilson’s Disease
affects larger joints
causes rhomboid calcium phosphate crystals that are weakly positively birefringent, chondrocalcinosis on X-ray
Rx: aspirate to exclude septic arthritis, NSAIDs and steroids
use of allopurinol as gout prophylaxis
inhibits xanthine oxidase
started 2 weeks after an acute attack has settled (if already taking, continue throughout attack). initial dose of 100mg OD is then titrated every few weeks to aim for a serum uric acid <300micromol/L
INTERACTS WITH CYCLOPHOSPHAMIDE
Systemic Lupus Erythematous features
“A CHRONIC RASH”
ANA positive in 99%
CVS - pericarditis, myocarditis, HTN
Respiratory - pleurisy, fibrosing alveolitis
Others - fever, fatigue, lymohadenopathy, oral ulcers, weight loss
Nuropsychiatric - anxiety, depression, psychosis and seizures
Immunological - anti-dsDNA, antiphospholipid syndrome, anti-Smith
Compliment levels (C3 and C4) decrease during active disease
Renal - glomerulonephritis, proteinurea, lupus nephritis (a severe complication)
Arthritis - 2 or more joints (non-erosive) and arthralgia
Skin - malar (butterfly) rash, photosensitivity, discoid rash and livedo reticularis (lace like rash)
Haematology - neutropenia, thrombocytopenia, lymphopenia, increased ESR but CRP is normal
SLE presentation
glove and sweater
gloves: raynauds, joint pain and swelling, hand rash
sweater: proximal muscle weakness, hair loss, mouth ulcers, pleuritic/ pericardial pain, truncal rash, uveitis
SLE management
high factor sunblock hydroxychloroquine (decreases disease activity and increases survival) NSAIDs (unless has renal disease) azathioprine/methotrexate/mycophenolate belimimab (monoclonal antibody)
what is discoid lupus?
lupus that only affects the skin
drug induced lupus
renal and NS involvement is rare
usually resolves when drug is stopped
features: arthralgia, myalgia, malar rash, pleurisy, ANA positive, ds-DNA negative, upto 90% have anti-histone antibodies
offending drugs –> procainamide, hydralazine, isoniazid and phenytoin
antiphospholipid syndrome
features: "CLOTS" coagulation defect livedo reticularis obstetric (recurrent miscarriages) thrombocytopenia persistent aniphospholipid antibodies (anti-cl and LA antibodies) Rx: anticoagulation
Sjogren’s syndrome
autoimmune disorder affecting exocrine grands (gives dry mucosal surfaces) RF positive (100%), ANA positive (70%), anti-RO and anti-LA (most classical)
Rx: artificial tears and saliva, lubricant, hydroxychloroquine to reduce arthralgia and skin symptoms
fibromyalgia
widespread pain and fatigue (tender in >11/18 locations)
Rx: education, CBT, pregabalin, duloxetine and amitriptyline
ankylosing spondylitis
HLA-B27 associated spondyloarthropathy
commonly in males 20-30y/o
lower back pain and stiffness (worse in morning and improves with exercise) of insidious onset
other features (the A’s): apical fibrosis, anterior uveitis, aortic regurgitation, achilles tendonitis, AV node block, amyloidosis
x-ray: sacroiliitis, squaring of lumbar vertebrae, “bamboo spine” in late stages
Rx: exercise and NSAIDS +/- bisphosphonates
then tnf alpha blockers e.g. etanercept
Psoriatic arthritis
features: “ROADS”
rheumatoid like pattern (does affect DIPJ), (asymmetrical) oligoarthritis, arthritis mutilans, DIPJ involvement, spondylitis or sacroiliitis
RF negative, increase IgA
x-ray: pencil cup deformity of distal phalanx
Rx: topical treatment for rash, oral prednisolone or local injection of methylprednisolone during flare
+/- NSAIDs and COX2 inhibitors
tnf-alpha inhibitors (infliximab) for active enthesis and dactylitis
scleroderma
aka systemic sclerosis
ANA positive in 90%
- limited cutaneous sclerosis (~70%) - face and distal limbs, associated with anti-centromere antibodies. crest is a subtype: calcinosis, Raynaud’s phenomenon, oesophageal dysmotility, sclerodactyly, telangiectasia
- diffuse cutaneous systemic sclerosis (~30%) - trunk and proximal limbs, associated with scl-70/anti-RNA polymerase-3 antibodies, HTN, lung fibrosis and renal involvement - poor prognosis
—> pulmonary fibrosis, malabsorption, reduced SB motility leading to infections, intestinal obstruction, cardiac tamponade, renal crisis due to accelerated HTN
Rx: immunosuppression
- prednisolone/methotrexate/ciclosporin/azathioprine
- cyclophosphamide/mycophenolate for pulmonary involvement
- cyclical ABx for gut bacterial overgrowth
- PPIs
- ACEi to control BP
- pt education and PT
polymyalgia rheumatica
frequently occurs with giant cell arthritis
typically >60y/o, rapid onset, aching morning stiffness and proximal limb muscles, polyarthralgia, lethargy, depression, low-grade fever, anorexia and night sweats
ESR is used to monitor response to treatment (along with symptoms)
Rx: prednisolone (gives a dramatic response, if doesn’t work consider a new diagnosis)
calcium and vitamin d supplements and bisphosphonates
vasculitis types
large vessel (giant cell, takaydasu's arteritis) Rx: steroids medium vessel (polyarteritis nodosa, Kawasaki disease) Rx: immunosuppression small vessel (wegner's - cANCA, churg-strauss - pANCA, microscopic polyangiitis, Goodpasture's, IgA vasculitis) Rx: immunosuppression variable (behcet's and corgan's syndrome)
smoking cessation helps all
Giant cell arteritis
headache, temporal artery and scalp tenderness (when
brushing hair), jaw claudication, amaurosis fugax
Rx: prednisolone STAT or IV methylprednisolone if visual symptom history
typically lasts two years and then has complete remission
Behcet’s syndrome
oral ulcers, genital ulcers and anterior uveitis
HLA B51 and MICA6 allele associated
Rx: azathioprine (if eye/vasculitis)
TNF-alpha inhibitors (if CNS involvement)
topical corticosteroids (for ulcerations)
NSAIDs (for joint involvement and muscle pain)
surgery - aneurysm repair, repair of GI perforations glaucoma, cataracts or retinal detachment
Takyasu’s arteritis
affects aorta and its branches
upper limb claudication with diminished or absent pulses
raised ESR in acute phase
Buergers disease
thrombotic occlusions of small and medium vessels in the lower limb, common in young male smokers
proximal pulses are present but pedal pulses are lost, tortuous corkscrew shaped collateral vessels are seen on angiography
Polyarteritis nodosa
systemic necrotising vasculitis affecting small and medium muscular arteries
common in populations with a high Hep B prevalence
renal disease in 70%
angiography shows saccular/fusiform aneurysms
Wegners granulomatosis
c-ANCA
ulceration, nodules and purpura may be seen
methotrexate
DMARD
dihydrofolate reductase inhibitor (prevents DNA synthesis by preventing folate conversion to purines and thymidylate)
ADR:
-myelosuppression (FBC monitoring)
-liver cirrhosis (LFT monitoring)
-pneumonitis
-teratogenic (not taken in pregnancy, must be stopped 3 months before conception in females AND males)
TAKEN ONCE A WEEK
NEVER WITH TRIMETHOPRIM
sulfasalazine
DMARD 5-aminosalicylate, possibly inhibits T cell proliferation, IL-2 production, neutrophil, chemotaxis and degranulation ADR: -rashes -oligospermia -Heinz body anaemia -interstitial lung disease - if allergic to aspirin then may be allergic -safe in pregnancy
leflunomide
DMARD ADR: -liver impairment -ILD -HTN -not safe in pregnancy
hydroxychloroquine
DMARD reduces activation of dendritic cells ADR: -retinopathy (needs a baseline ophthalmic examination) -corneal deposits -safe in pregnancy
prednisolone
glucocorticoid ADR: -cushingoid features -OP -impaired glucose tolerance -HTN -catarcts
etanercept
TNF-alpha inhibitor (recombinant human protein)
ADR:
-demyelination
-reactivation of TB
given SC
infliximab
TNF-alpha inhibitor (monoclonal antibody - reduces cytokine cascade, reduces inflammation, reduces angiogenesis and reduces joint destruction)
ADR: reactivation of TN
given IV
adalimumab
TNF-alpha inhibitor (monoclonal antibody - reduces cytokine cascade, reduces inflammation, reduces angiogenesis and reduces joint destruction)
ADR: reactivation of TB
given SC
rituximab
anti-CD20 monoclonal antibody, causing B-cell depletion
ADR: infusion reactions are common
NSAIDs
nonselective COX inhibitors
can be taken up to 32 weeks in pregnancy
ADR: bronchospasm in asthmatics, dyspepsia/peptic ulceration (co-prescribe a PPI)
Allopurinol
Xanthine Oxidase inhibitor (prevents purine metabolism)
ADR: can cause steven-johnson syndrome
