Rheumatology Flashcards
Vasculitides:
- 2 large vessel?
- 2 medium vessel?
- 3 ANCA small vessel?
- 2 immune complex mediated small vessel?
- 1 which only affects the smallest vessels (capillaries)?
Large: GCA, takayasu
Medium: polyarteritis nodosa, kawasaki
ANCA small: microscopic polyangitis, GPA, eGPA
Immune complex small: IgA (HSP), cryoglobulinaemic vasculitis
Capillaries: anti-GBM
Shared features in ANCA vasculitis?
Fever, weight loss Purpuric rash Arthralgia GN Mononeuritis multiplex Lung opacities (CXR)
Specific features of GPA?
(Wegener’s)
- cANCA (PR3)
- ENT - epistaxis, crusting, oral ulcers, sinusitis, saddle deformity
- LRT - dyspnoea, cough, haemoptysis
- Uveitis
- Necrotising GN
Specific features of eGPA?
(Churg-Strauss)
Similar to GPA + late-onset asthma + eosinophilia
Paranasal sinusitis
Mononeuritis multiplex/polyneuropathy
pANCA (anti-MPO)
Specific features of microscopic polyangitis?
Similar vasculitic picture
Necrotising GN v common
pANCA 60%
cANCA 40%
Rx ANCA vasculitides?
Cyclophosphamide + steroids (1st line)
Plasma exchange 2nd line
In GCA what is raised on blood test?
What is seen on CK, MR angio and PET CT?
ESR, CRP, PV raised
CK, MR angio and PET CT all normal
HSP where do they often get pain?
Rx?
Colicky abdo pain
(prupura on buttocks/legs)
Self-limiting
Kawasaki disease management? (3)
High dose aspirin
IVIG
Echocardiogram (coronary aneurysm)
Apart from vasculitis what else may pANCA be positive in? (anti-MPO)
UC (70%)
PSC (70%)
Anti-GBM (25%)
Crohn’s (20%)
General 1st line investigations for suspected vasculitis?
Urinalysis - blood and protein
Bloods:
- FBC - normochromic/cytic anaemia & raised PLT
- U&E - renal impairment
- CRP - raised
CXR - nodular, fibrotic or infiltrative lesions
Kidney/lung biopsies may be taken by specialists to aid diagnosis
Rare life-threatening complication of RA?
Extra-articular manifestations?
Atlanto-axial subluxation
lung fibrosis
rheumatoid nodules
CVD
ocular stuff
XR of RA?
What other imaging may be of use?
What may be seen in hands?
Early - normal
- Periarticular osteopenia/osteoporosis
- Periarticular erosions
- Subluxation
- May be loss of joint space
USS - synovitis
Hands - ulnar deviation, Z-shaped thumb, swan neck/boutonniere
Initial Ix for RA?
What else is RF positive in?
RF and anti-CCP (ACPA)
Have similar sensitivity (70%) but anti-CCP much more specific and can be detected 10 years before development of RA
RF also pos in:
- Felty’s syndrome (RA, splenomegaly, neutropaenia)
- Sjrogen’s
- infective endocarditis
- SLE
- systemic sclerosis
- general population (5%)
Azathioprine:
- What may be tested for in people before starting it?
- SE?
- Drug interaction?
- Safe in pregnancy?
Thiopurine methyltransferase (TPMT) deficiency - look for individuals prone to azathioprine toxicity
SE:
- myelosuppression
- nausea/vomiting
- pancreatitis
- BCC/SCC skin cancer
Allopurinol - use lower doses of azathioprine
Yes
Antibodies in SLE?
ANA - 99%, not specific but useful to rule out
anti-dsDNA (70% sensitive, 99% specific)
anti-Smith/Sm (30% sensitive, 99% specific)
RF (20%)
Also anti-U1 RNP, anti-Ro, anti-La
Monitoring of SLE?
Raised ESR
CRP usually normal in active disease - raised CRP may indicate underlying infection
C3 and C4 complement levels LOW during active disease (used up in immune complex formation)
anti-dsDNA titres (but only present in 70%)
Systemic sclerosis:
- what small bowel complication can occur?
- Usual causes of death?
- Monitoring
- Management of Raynaud’s, Renal, GI and ILD problems with SSc?
- bacterial overgrowth (Rx Rifampicin or Co-amox)
- Lung or renal involvement (renal crisis from accelerated HTN)
- Renal monitoring, pulmonary function tests, echocardiogram (fibrosis of heart muscle)
Raynaud’s: CCB or bosentan
Renal: ACEI (regardless of age)
GI: PPI (reflux from oesophageal dysmotility)
ILD: Cyclophosphamide
5 drugs which can cause drug-induced lupus?
Ig?
Most common: hydralazine, procainimide
Less common: Isoniazid, minocycline, phenytoin
ANA and anti-histone +ve
Anti-dsDNA -ve
4 XR findings in ank spond?
- subchondral erosions
- subchondral sclerosis
- squaring of lumbar vertebrae
- syndesmophytes (ossification of outer annulus fibrosus fibres)
Systemic sclerosis:
- difference in symptoms between limited and diffuse?
- antibodies?
- what is scleroderma?
Limited: CREST
Scleroderma affects distal limbs, tends not to affect above elbows
Diffuse:
Scleroderma affecting proximal limbs and trunk
Pulmonary fibrosis, pulmonary hypertension, kidney failure
Limited: anti-centromere
Diffuse: anti-Scl-70
Scleroderma - cutaneous involvement only, no internal organ involvement. Plaques are called morphoea
Sulfasalazine:
- people with allergies to what drugs may also have allergy to it?
- SE? (5)
- is it safe in pregnancy/breastfeeding?
Aspirin
Oligospermia SJS pneumonitis/lung fibrosis heinz body anaemia Myelosuppression
Yes
Behcet’s classic triad?
Other symptoms?
Tests?
Oral ulcers, genital ulcers, anterior uveitis
Also: erythema nodosum, thrombophlebitis, colitis, arthritis, aseptic meningitis, acne-like lesions
No diagnostic tests
Pinpricks with needles may lead to pustule formation at puncture site
SE hydroxychloroquine?
Is it safe in pregnancy?
bulls eye retinopathy - baseline ophthalmological exam necessary and annual screening
Yes
Heart defect with ehlers danlos syndrome?
Aortic Regurg
SE methotrexate? Pregnancy? Monitoring? What should be co-prescribed? What CANNOT be given with it?
Pneumonitis/pul fibrosis
Myelosuppression
Mucositis
Liver fibrosis
Both women and men must stop methotrexate for 6 months before trying to conceive
Weekly FBC, LFT and U&E until dose stabilised, then every 2-3 months (drug given weekly)
Folic acid 5mg
Trimethoprim - risk of bone marrow aplasia and fatal pancytopaenia
Caution with high dose aspirin as well - can increase effects of methotrexate by reducing excretion
When can anti-TNF drugs be considered in ank spond?
When they have failed on 2 different NSAIDs and meets the criteria for active disease on 2 occasions at least 12 weeks apart
What screening tests must be done for Rituximab?
Cardiac echo - can cause cardiac complications - esp of LVD
How many DMARDs must be tried before TNF inhibitors?
Name 3 TNFI?
What DAS28 values mean remission and high activity?
2, including MTX
Etanercept, infliximab, adalimumab
<2.6 = remission >5.1 = high activity
OA XR signs?
LOSS
Loss of joint space
Osteophytes
Subchondral sclerosis
Subchondral cysts
1st - paracetamol + topical NSAID (if hand/knee)
2nd - NSAIDs/COX-2 inhibitors, opioids, capsaicin cream, intra-articular steroid injections
(PPI with NSIAD/COX-2, don’t give these drugs if on cardioprotective aspirin)
Non-pharm - support braces, TENS, in-soles, walking aids
If conservative fails then joint replacement
Schober’s test?
Put dots in midline 10cm above PSIS and 5cm below - bend forward to touch toes, distance between dots should increase by 5+cm if normal
Hand features of OA?
Painless Heberden’s (distal) and Bouchard’s (proximal) nodes - bony swellings (osteophytes)
Squaring of thumb base - deformity of CMC joint resulting in fixed adduction
CMC and PIP joints most commonly affected
Stiffness <30 mins. Intermittent ache provoked by use. Usually no functional impairment
Is obesity a CI for joint replacement in OA?
Weight bearing after op?
Thromboprophylaxis?
4 standard pieces of advice post-op for hip replacement?
Relative - no extra adverse long-term outcomes in terms of joint survival
Weight bearing as tolerated ASAP
LMWH for 4 weeks post-op
- Avoid hip flexion >90 degrees
- avoid low chairs
- do not cross legs
- sleep on back for first 6 weeks
‘A’ features of ank spond? (7)
Anterior uveitis Apical fibrosis Aortic regurg AV node block Amyloidisos Achilles tendonitis Arthritis (peripheral)
Ix for ank spond?
Rx?
HLA-B27 (90% +ve)
Raised ESR CRP
1st - spinal XR
- sacroiliitis - subchondral sclerosis/erosions
- squaring of lumbar vertebrae
- syndesmophytes - ossification of annulus fibrosus
- bamboo spine (late and uncommon)
If XR normal but high clinical suspicion - MRI
- marrow oedema
CXR - apical fibrosis
RX: NSAID’s
Must try at least 2 before TNF inhibitors
DMARD’s only useful if peripheral arthritis
Regular exercise
Monitor spirometry for restrictive defect
What are the 4 seronegative arthropathies?
Ank spond
Psoriatic
Enteropathic
Reactive
All assoc w HLA-B27
Psoriatic arthritis
- pattern?
- other inflammations?
- XR?
- End stage?
- Rx?
Asymmetric oligoarthritis, usually affective small joints first - can affect DIP
Look for nail/skin changes
Dactylitis, enthesitis and tenosynovitis
- Erosions
- Tuft resorption
- Eventually pencil-in-cuo
Arthritis mutilans - telescoping
Same as RA - DMARDs, try MTX first
Joint replacement for large joints
Enteropathic arthritis?
Usually large joint, assoc w UC/Crohn’s
Rx involves controlling bowel symptoms
Reactive arthritis?
AKA Reiter’s Syndrome (nazi)
Large joint arthritis
Urethritis/recent diarrhoea
Uveitis
Also yellow/brown papules on palms/soles called keratoderma blenorrhagica, and circinate balanitis
Rx: NSAID’s, intra-articular steroids
Sulfasalazine if persistent
Rarely lasts past 12 months
Gout acute Rx?
After this?
NSAIDs or Colchicine 1st line
(Colchicine main Rx diarrhoea - and has slower onset)
Oral steroids 2nd line
Urate lowering therapy after 1st attack:
- allopurinol 1st line (lower dose if reduced GFR) - wait 2 weeks after acute to start
- febuxostat 2nd line
Antibodies in dermatomyositis?
Cutaneous features?
Other features?
Anti-Jo-1
Anti-Mi-2
Anti-SRP
Heliotrope rash (eyes) Shawl sign (over shoulders) Gottron's papules (dorsal MCP and along extensor tendons)
Proximal muscle weakness, ILD, dysphagoa, raynaud’s
Prednisolone +/- MTX/azathioprine
Sjogren’s syndrome:
- features?
- Ix?
- Rx?
- increased risk of?
Exocrine dysfunction - may be primary or secondary to RA.
- dry mouth/eyes/vagina
- Arthralgia/myalgia
- Raynaud’s
- Parotid swelling (recurrent)
- Sensory polyneuropathy
Ix: - RF, ANA, anti-Ro, anti-La (Ro and ANA most sensitive - 70%) - Schirmer's test - <5mm in 5 mins - histology - focal lymphocytic infiltration
- artificial tears
- pilocarpine (muscarinic agonist) may be used to stimulate saliva
60-fold increased risk lymphoid malignancy
General management of SLE?
Of acute flares?
Skin symptoms?
Nephritis?
Hydroxychloroquine
Acute - haemolytic anaemia, nephritis, pericarditis, CNS disease
- cyclophosphamide + pred
Skin - topical steroids
Nephritis - Cyclophosphamide or MMF
Anti-phospholipid syndrome:
- What is it?
- features?
- antibodies? (3)
- Rx?
Predisposition to both arterial and venous thrombosis with recurrent foetal loss and thrombocytopenia. Can be primary or secondary to others, usually SLE
Arterial/venous thrombosis
Recurrent foetal loss
Livedo reticularis
Thrombocytopaenia
Pulmonary hypertension (due to multiple/recurrent emboli)
Prolonged aPTT
- due to reaction of lupus anticoagulant with phospholipids involved in coag cascade
Anti-cardiolipin
Lupus anticoagulant
anti-beta 2 glycoprotein
Primary prophylaxis: low-dose aspirin
Secondary: Lifelong warfarin
Target INR 2-3 if initial
Recurrent - 3-4
