Rheumatology Flashcards
Differentials for an acute monoarthritis
Gout, pseudogout, pyogenic septic arthritis, reactive arthritis, haemarthrosis, osteomyelitis, monoarticular rheumatoid arthritis, osteonecrosis
Define Adult-onset Still’s disease and give its features
An idiopathic autoinflammatory condition which commonly presents in young adulthood - characterised by a triad of arthritis (commonly knees, wrists and fingers), spiking fevers and a transient salmon-pink maculopapular rash
Adult-onset Still’s disease investigations
Hyperferritinaemia (1500 to >10,000), marked raised inflammatory markers, leucocytosis w/ neutrophilia
What are the Yamaguchi classification criteria?
Criteria used to diagnose Adult-onset Still’s disease - need to meet 5 criteria (including 2 major):
Major:
- Fever >39 for more than 7 days
- Arthralgias or arthritis for >2 wks
- Characteristic rash
- Leucocytosis w/ >80% neutrophils
Minor:
- Sore throat
- Lymphadenopathy
- Hepato/splenomegaly
- Raised aminotransferase
- -ve RF and ANA
Exclusion:
- Malignancy
- Infections
- Other CNT disorders
- Familial autoinflammatory conditions
- Drug reactions
Adult-onset Still’s disease management
NSAIDs and aspirin for mild disease, low-dose corticosteroids added when that isn’t enough
Severe disease w/ life-threatening organ manifestations require high-dose pulsed IV corticosteroids
Biological therapies = IL-1, TNF-alpha or IL-6 blockers
Ankylosing spondylitis features
- Inflammatory back pain - often early morning stiffness (gets better w/ activity), w/ tenderness of the sacroiliac joints and limited range of spinal motion on examination
- Enthesitis (inflammation of where ligaments attack to bone) - esp of Achilles tendon and plantar fascia
- Peripheral arthritis (1/3 or pts) - hips and shoulders
- Extra-articular involvement = anterior uveitis, aortitis and aortic regurgitation, upper lobe pulmonary fibrosis, IgA nephropathy
Ankylosing spondylitis physical examination findings
- Restricted movements in lumbar spine
- Dorsal kyphosis of the thoracic spine may be present as the disease progresses - can lead to reduced chest expansion
- C-spine movements can be globally reduced, with the neck forced into flexion by dorsal kyphosis
Ankylosing spondylitis imaging results
Pelvic X-ray:
- Sacroiliitis (better seen on MRI)
- Ankylosis or fusion of sacroiliac joint is seen in advanced disease
Lumbar X-ray:
- Squaring of vertebral bodies
- Syndesmophyte formation (bony bridges) between adjacent vertebrae + ossification of the spinal ligaments
- Complete fusion of the vertebral column (bamboo spine) in advanced disease
Ankylosing spondylitis management
Conservative: - critical to improve and maintain posture, flexibility and mobility
- Exercise
- Physio
Medical:
- NSAIDs + PPI 1st line
- DMARDs (sulfasalazine and methotrexate) for pts w/ peripheral joint disease - these don’t improve spinal inflammation
- Local steroid injection
- Biologics - anti-TNF 1st line, ant0IL17 2nd line
Antiphospholipid syndrome features
Mneumonic= CLOT
C = clots - usually venous thromboembolisms, but can be arterial
L = Livedo reticularis - a mottled, lace-like appearance on the skin of the lower limbs
O = obstetric loss
T = Thrombocytopenia
Antiphospholipid syndrome management
Prophylactic lose-dose aspirin
Control of thromboembolic RFs
In pts who have a venous thromboembolism = life long warfarin is used with a INR target of 2-3
How long should mechanical back pain take to clear up?
6 weeks
Red flag symptoms for back pain
- New onset when aged <20 or >55
- Thoracic or cervical spine pain
- Pain is progressive and not relieved by rest - suggestive of infection or cancer
- Spinal (rather than paraspinal) tenderness
- Early morning stiffness >30 min = ankylosing spondylitis
- Abnormal LL neuro signs = cord compression
- Trauma or sudden onset pain in pts w/ RFs for osteoporosis = possible vertebral fracture
Define Behcet’s disease
A rare multiorgan disease caused by systemic vasculitis of arteries and veins of all sizes, hypercoagulability and neutrophil hyperfunction - has an unknown cause, but is more common in Turks, Mediterranean’s and the Japanese
Behcet’s disease features
- Recurrent oral/genital ulceration
- Anterior or posterior uveitis
- Erythema nodosum
- Vasculitis
- Acneiform lesions
- Superficial thrombophlebitis at venepuncture sites
- Pathergy = pustules at sites of puncture or minor trauma
- Arthralgias and arthritis
Uncommon:
- Neuro involvement = cerebral vasculitis, venous sinus thrombosis
- Reso involvement = pulmonary vasculitis, PE, pulmonary haemorrhage
- GI vasculitis = abdo pain, mesenteric angina, constipation
- Cardiovascular involvement = MI, deep vein thrombosis
Behcet’s disease investigations
No specific test - more of a diagnosis of exclusion/clinical signs
Pathergy test (skin prick using a sterile needle then reassessing 48hrs later to see if papule/pustule has formed) can be used but is unreliable
Behcet’s disease management
Depends on the pt - some need immunosupression, some only need cover during flare-ups
Medication used = steroids, colchicine, azathioprine, methotrexate, biologics
Gout crystals - appearance and make up
Negatively birefringent, needle-shaped crystals - made up of monosodium urate
Pseudogout crystals - appearance and make up
Positively birefringent, rhomboid shaped crystals - made up of calcium pyrophosphate dihydrate
What is the double-contour sign?
The most sensitive US finding for gout = a hyperechoic irregular band over the superficial margin of the articular cartilage
Define Enteric arthritis
An inflammatory arthritis associated w/ IBD - can occur before, during or after the diagnosis of IBD
Patterns of Enteric arthritis
Axial spondyloarthritis:
- Gradual onset lower back pain and stiffness, worse in the mornings and improves w/ exercise
- Sacroiliac tenderness reduced ROM and reduced chest expansion on examination
Acute peripheral arthritis of IBD:
- Asymmetric, oligoarticular arthritis predominantly of the LL
- Often transient and migratory join inflammation - episodes are usually self-limiting
- Strongly associated w. flares of IBD
Chronic peripheral arthritis of IBD:
- Chronic, polyarticular inflammation w/ disease independent of IBD activity
- Usually symmetrical and most commonly affects the metacarpophalangeal joints, knees and ankles
Enteric arthritis investigations
Bloods:
- Raised inflammatory markers
- RF and ANA -ve
- p-ANCA +ve seen in 55-70%
Radiology:
- Chronic peripheral arthritis = may show erosions of affected joints
- Axial disease = similar to ankylosing spondylitis
Enteric arthritis management
DMARDs for peripheral disease
TNF-alpha/IL12/IL23 inhibitors for axial disease
Define Eosinophilic granulomatosis w/ polyangiitis
A granulomatous small and medium-vessel vasculitis
Eosinophilic granulomatosis w/ polyangiitis features
Criteria:
- Adult onset asthma
- Eosinophilia >10% on peripheral bloods
- Paranasal sinusitis
- Pulmonary infiltrates
- Histological confirmation (small granulomas w/ necrotising vasculitis + eosinophilic infiltrates)
- Mononeuritis multiplex or polyneuropathy
Eosinophilic granulomatosis w/ polyangiitis management
Glucocorticoids
Familial Mediterranean fever features
- Recurrent fevers
- Abdo pain (peritonitis)
- Pleuritic pain (pleuritis)
- LL arthritis
- Orchitis
- Erysipeloid rash
Symptoms usually last 1-3 days and are slef-limiting
Familial Mediterranean fever investiagations
Rule out other causes + genetic testing for MEFV gene
Familial Mediterranean fever management
Symptomatic treatment during attacks
Long-term colchicine reduces risk of developing AA amyloidosis
Define Felty syndrome
A rare variant of RA, defined as the presence of 3 key features = highly active RA (often w/ extra-articular disease), splenomegaly and neutropenia
Define Fibromyalgia
A chronic, complex and widespread pain syndrome
Fibromyalgia features
- Cardinal symptom = chronic, widespread body pain (constant dull ache)
- Fatigue
- Cognitive disturbance (problems w/ focus and memory AKA fibro fog)
- Mood disorders
- Sleep disturbances (including insomnia)
What USS sign is characteristic of Giant cell arteritis?
Halo sign on temporal artery USS
What is the definitive investigation for Giant cell arteritis?
Temporal artery biopsy - 3-5 cm to avoid missing skip lesions
Giant cell arteritis management
High dose prednisolone (40-60mg) ASAP to avoid blindness and stroke - followed by weaning program
Give bisphosphonates + PPIs due to prolonged steroid regime
Can give low-dose aspirin to decrease risk of stroke and blindness
Types of Gout
- Acute gout
- Tophaceous gout - usually on a background of longstanding recurrent gout, have tophi on the4 pinnae or on pressure points
- Nephrolithiasis = uric acid renal stones
- Uric acid nephropathy
Acute pseudo/gout management
- NSAIDs (500mg BD of Naproxen or 120mg BD of Etoricoxib)
- Colchicine 500 micrograms BD
- Intra-articular corticosteroids
- Oral corticosteroids (Prednisolone 30mg OD for 5-7 days)
Gout prophylaxis
- Lifestyle changes
- Avoiding triggers
- Review meds which may cause hyperuricaemia (Thiazide and loop diuretic, low-dose salicylates, chemo)
- If possible swap anti-HTN drugs to losartan or amlodipine which have urate-lowering effects
- Allopurinol (start 2 wks after an attack as can cause another attack is started before then) if fit criteria - aim for urate levels <300
Indications for prophylactic allopurinol in gout
- More than 2/3 attacks per year
- Chronic tophaceous gout
- X-ray changes show chronic destructive joint disease
- Urate nephrolithiasis
- Severe and disabling polyarticular attacks
- Inherited syndrome s(e.g. Lesch-Nyhan)
Define Granulomatosis w/ polyangiitis
A small and medium-vessel systemic vasculitis
Granulomatosis w/ polyangiitis features
URT involvement:
- Chronic sinusitis
- Epsitaxis/nasal discharge/mucosal ulcerations
- Saddle-nose deformity - secondary to perforation of the nasal septum and disruption of the supporting cartilage of the nose
LRT involvement:
- Cough
- Haemoptysis
- Pleuritis
p-ANCA/c-ANCA glomerulonephritis:
- Haematuria
- Proteinuria
Granulomatosis w/ polyangiitis investigations
Bloods:
- ANCA +ve
- Anaemia
- Leucocytosis
- Raised CRP/ESR
Urine:
- Haematuria
- Proteinuria
- Cellular casts
- CXR = Bilateral nodules, cavities and infiltrates
Renal biopsy = Pauci-immune, focal, segmental necrotising glomerulonephritis
Granulomatosis w/ polyangiitis management
- High-dose glucocorticoids (1st line)
- Cyclophosphamide
- Rituximab
- Plasma exchange
- Azathioprine/methotrexate/rituximab for maintenance
Define Henoch-Schonlein Purpura
Small-vessel vasculitis commonly occurring in children - often following a resp tract infection
Henoch-Schonlein Purpura features
- Arthralgia/arthritis
- Abdo pain
- Palpable purpura on buttocks and extensors
- Renal disease (microscopic haematuria or an isolated proteinuria)
Henoch-Schonlein Purpura investigations
Skin biopsy of lesions - will show presence of neutrophils and monocytes
Henoch-Schonlein Purpura management
- Analgesia
- Corticosteroids
- Refer to renal if renal involvement
Henoch-Schonlein Purpura complications
- Pulmonary or GI haemorrhage
- Renal impairment leading to ESRD
- Neuro complications e.g. headache and seizures
- Eye complications e.g. keratitis or uveitis
Define Hypermobility syndrome
Hypermobility = the ability to move joints beyond the normal range of movement - if this causes the pt to have symptoms secondary to the flexible joints, then it’s a syndrome
Hypermobility syndrome features
- Pain or stiffness in the joins or muscles
- Frequent sprains or dislocations
- Poor balance
- Thin, stretchy skin
- GI upset
Hypermobility syndrome investigtions
Use the Beighton score to assess joints for hypermobility
Hypermobility syndrome management
Strengthen muscles to protect the joints, physio involvement
Define IgG4-realted disease
A chronic multisystem fibroinflammatory disease characterised by a unifying histological features = tumefacient lesions w/ dense lymphocytic infiltrates and storiform fibrosis - thought to be due to the production of IgG4
IgG4-realted disease features
Pts typically present w/ subacute swelling of the glandular tissue and allergic features:
- Salivary gland swelling
- AI Pancreatitis
- Lymphadenopathy
- Aortitis
- Retroperitoneal fibrosis (lower back pain, LL oedema, urethritic obstructions)
IgG4-realted disease management
Steroids
Define Marfan syndrome
An A. dominant CNT disorder caused by a missense mutation of the fibrillin 1 gene
Marfan syndrome features
Physical appearance:
- Tall and thin, with unusually long limbs
- High-arched palate
- Arachnodactyly (tested w/ thumb or wrist sign)
Cardiovascular:
- Thoracic/abdo aortic aneurysms/dissections
- Aortic regurgitation
- Aortic root dilatation
- Mitral valve prolapse/regurgitation
- Spontaneous pneumothoraxes
Ophthalmic:
- Lens dislocations
- Closed-angle glaucoma
MSK:
- Hypermobility
- Contractures
- Pectus deformities
- Kyphoscoliosis
- Protusio acetabuli
Neuro:
-Dural ectasia hernias
-Spontaneous CSF leaks
Marfan syndrome diagnostic criteria
Use 2010 Ghent criteria
Marfan syndrome managemnt
Conservative:
- Genetic testing and counselling
- Education around avoiding risky activities
- Surveillance echo’s
- Annual ophthalmological assessment
Medical:
- Strict BP control
- Avoid giving fluoroquinolones due to risk of aortic aneurysms or dissections
Surgical = treat issues that occur
Define Microscopic polyangiitis
One of the more common forms of arteritis affecting smaller arteries - due to p-ANCA directed against myeloperoxidase
Microscopic polyangiitis features
- Constitutional symptoms = WL, fevers, malaise, arthralgia, myalgia
- Necrotising glomerulonephritis
- Skin lesions =palpable purpura of the LL
- Mononeuritis multiplex
- Lung involvement = pulmonary capillaritis leading to alveolar haemorrhage
Microscopic polyangiitis management
High dose glucocorticoids + cyclophosphamide or rituximab
Define Polyarteritis nodosa
A segmental transmural necrotising vasculitis of medium-sized muscular arteries - associated w/ Hep B
Polyarteritis nodosa features
- Mononeuritis multiplex
- Renal infarcts and failure
- Skin lesions = livedo reticularis, arthritis, myalgias
- MSK = arthralgias, arthritis, myalgias
- GI = abdo pain, abnormal LFTs
- MI/HF
- Stroke
- Seizures
Pulmonary arteries are NOT affected
Polyarteritis nodosa management
Oral corticosteroids for moderate, IV pulsed high-dose corticosteroids for severe, immunotherapy (azathioprine, methotrexate, cyclophosphamide, rituximab)
Polyarteritis nodosa complications
Can destroy the arterial media and internal elastic lamina - leads to aneurysm formation
Polymyalgia Rheumatica features
- Shoulder/hip girdle stiff (in the mornings) for > 1 hr, w/ associated inflammatory pain
- Low-grade fever
- Anorexia
- WL
- Malaise
- Associated w/ GCA
- No muscle weakness - if there is weakness then more likely to be dermato/myositis
Polymyalgia Rheumatica management
Low-dose corticosteroids
Subtypes of inflammatory myopathies
- Polymyositis
- Dermatomyositis
- Amyotrophic dermatomyositis
- Antisynethetase syndrome (polymyositis w/ antisynthetase abs)
- Inclusion-body myositis
Polymyositis features
- Main feature = bilateral, proximal muscle (hip and shoulder) weakness developing over weeks to months
- Myalgia and tenderness in 1/3 of pts
- Muscle bulk reflexes are preserved until very late
- As disease progresses pt get: pharyngeal or oesophageal involvement, resp muscle involvement
Dermatomyositis features
Symptoms of polymyositis + skin rashes:
- Heliotrophic rash = lilac discolouration of the eyelids w/ periorbital oedema
- Gottron’s papules = scaly, erythematous papules over knuckles and extensors
- Macular erythematous rash on anterior chest and neck (V-sign) or upper arms and shoulders (shawl sign)
- Nailfold abnormalities
- Cutaneos vasculitis
- Calcinosis
Amyotrophic dermatomyositis features
Characteristic cutaneous symptoms of dermatomyositis (e.g. Heliotrophic rash, Gottron’s papule) w/out any muscle involvement
Antisynthetase syndrome features
- Cracking and fissuring of the skin on the finger pads
- Interstitial lung disease
- Raynaud’s
- Arthralgia/arthritis
- Systemic features
- Dermatomyositis-like rash
- Polymyositis
Inclusion-body myositis features
Bilateral, asymmetrical painless weakness of the distal muscles +/- dysphagia
Slowly progressive and poorly responsive to immunosuppression
Dermato/myositis investigations
- Raised creatine kinase + other enzymes
- Muscle biopsy is definitive test - polymyositis shows endomysial inflammatory infiltrates, dermatomyositis shows perivascular, perimysial inflammatory infiltrates
Elevated enzymes can be remebered with:
‘Dermato/mysotis turn your muscles into CLAAA’
- Creatine kinase
- Lactate dehydrogenase
- Aldolase
- ALT
- AST
Dermato/myositis management
Corticosteroids, immunosuppression (methotrexate, mycophenolate), hydroxychloroquine can help w/ skin disease, rehabilitation
Psoriatic arthritis management
Conventional synthetic DMARDs (csDMARDs):
- Methotrexate
- Leflunomide
Biological DMARDs (bDMARDs):
- If pt fails to respond to csDMARDs
- Anti-TNF agents
- Anti-IL12/23
- Anti-IL17
- CTLA4 inhibitors
Tofacitinib (Janus kinase inhibitor) if those don’t work
Causes of Raynaud’s phenomenon
Primary = idiopathic
Secondary:
-SLE
- Scleroderma
- Buergers disease
- Sjogrens syndrome
- RA
- Oclusive vascular disease e.g. atheroscerlosis
- Polymyositis
- Coagulopathies
- Thyroid disorders
- Pulmonary HTN
Raynaud’s phenomenon management
Conservative:
- Smoking cessation
- Warm gloves
- Avoiding cold exposure
Medical:
- Dihydropyridine CCB (e.g. nifidepine) = 1st line
- Phsophodiesterase inhibitors and IV prostacyclins = 2nd line
Surgical (in severe cases):
- Nerve blocks
- Digitil amputation
Define Reactive arthritis
A sterile inflammatory arthritis occuring w/in 4 weeks of an infection - most commonly chlamydia, shigella, yersinia or salmonella
Reactive arthritis features
- Asymmetrical oligoarthritis most commonly affecting he large joints of the lower limbs
- Constiutional symptoms
- Keratoderma blenorrhagica = dark maculopapular rash on palmes and soles
- Circinate balantitis = erosion of the glands penis
- Conjuctivitis/anterior uevitis
- Dactylitis
- Urethritis and sterile dysuria
Cant see, cant pee, cant climb tree
Reactive arthritis management
NSAIDs, DMARDs
Rheumatoid arthritis wider systemic features
- Haem = anaemia of chronic disease, AA amyloidosis
- Derm = rheumatoid nodules (firm, dark nodules around inflammation), small vessel vasculitis, pyoderma gangrenosum
- Ophthalmic = keratoconjunctivitis, Epi/scleritis
- Resp = pleural disease, rheumatoid nodules, ILD, methotrexate induced pneumonitis
- Cardiac = pericarditis, pericardial effusion, CVD
- Neuro = peripheral neuropathy (secondary to small-vessel vasculitis), mononeuritis multiplex (secondary to medium-vessel vasculitis)
Antibodies in Rheumatoid arthritis
Anti-CCP, RF
Rheumatoid arthritis management
Conservative:
- Physio
- Occupational therapy
Medical:
- Simple analgesia
- Oral or intra-articular corticosteroids
- DMARDs (1st line) = methotrexate, hydroxychloroquine, leflunomide, sulfasalazine
- Biologics (in addition if DMARD monotherapy doesn’t work) = Anti-TNF (infliximab), CTLA4 inhibitors, andi-CD20 (rituximab), anti-Il6 (tocilizumab)
Causative agents of Septic arthritis
- Staph aureus = 50% of cases
- Streptococci = 2nd most common (haematogenous spread)
- N. gonorrhoeae
- Pseudomonas = healthcare associated or IVDU
- Salmonella = if SCA
- Gram-ve’s
Special cases = TB, Lyme disease, fungi
Septic arthritis investigations
- Synovial fluid aspiration = gram stain, culture and microscopy
- 2x blood cultures
All done before giving Abx
Septic arthritis management
Empirical abx and then change based on culture
Pt should be treated for 2 wks w/ IV abx, and then orally for 4 more wks
If the joint is prosthetic = aggressive debridement and prolonged abx
Side effects of corticosteroids
C = Cushing’s syndrome
O = osteoporosis
R = retardation of growth
T = thin skin, easy bruising
I = immunosupression
C = cataracts and glaucoma
O = oedema
S = suppression of HPA axis
T = teratogenic
E = emotional disturbance (including psychosis)
R = rise in BP
O = obesity (truncal)
I = increased hair growth
D = DM
S = striae
Side effects of methotrexate
- GI disturbance
- Folate deficient anaemia
- Immunosuppression
- Pulmonary fibrosis
- Liver toxicity
- Interstitial pneumonitis
- Rash
- Teratogenic
Side effects of sulfasalazine
- Myelosuppression
- Nausea
- Rash
- Oral ulcers
- Decreased sperm count
Side effects of hydroxychloroquine
- Retinopathy
- Rash
Sjogren’s syndrome features
Ocular:
- Keratoconjunctivitis sicca = reduced tear secretion
- Causes dry, gritty eyes
Oral:
- Dry mouth
- Intermittent parotid gland swelling
Other exocrine glands:
- Vaginal dryness
- Reduces GI secretions = dysphagia, oesophagitis, gastritis
Extra glandular features:
- Systemic symptoms
- Arthritis = episodic, non-erosive
- Raynaud’s
- Cutaneous vasculitis
Causes of secondary Sjogren’s syndrome
- SLE
- RA
- Systemic sclerosis
- Poly/dermatomyositis
- AI thyroiditis
- PBC
- AI hepatitis
Sjogren’s syndrome investigations
Bloods:
- raised ESR/CRP
- RF +ve in 90%
- Anti-Ro and anti-La in 40-90%
Special tests:
- Schirmer’s test = filter paper on lower eyelid, with wetting of <5mm being +ve
- Rose Bengal staining of the cornea = demonstrates keratitis when using a split lamp
- Salivary flow rate monitoring w/ radiolabelled dye
- Salivary gland biopsy to confirm diagnosis
Sjogren’s syndrome management
Symptomatic control
No effective disease-modify therapies
SLE subtypes
SLE = multiple organ systems - usually +ve for ANA, anti-dsDNA
Subacute cutaneous lupus = skin only 0 ANA +ve or -ve, anti-SS-A and anti-SS-B +ve
Drug-induced lupus = systemic, usually sparing renal and neuro systems - anti-histones
Discoid lupus = skin only - usually ANA -ve
Dermatological manifestations of SLE
- Photosensitivity - usually a malar rash
- Discoid rash
- Recurrent mouth ulcers
- Raynaud’s phenomenon
- Non-scaring alopecia
- Cutaneous vasculitis - manifesting as splinter haemorrhages/purpura
MSK manifestations of SLE
- Non-reosive arthritis
- Flitting arthralgia w/ early morning stiffness
- Jaccoud’s arthropathy = tendon involvement leading to an RA-like deformity
Renal manifestations of SLE
Lupus nephritis (most common cause of SLE-related mortality) - starts as asymptomatic before presenting w/ either nephrotic or nephritic syndrome
Classification of Lupus nephritis
Minimal mesangial (class I) = mesangial immune deposits - no clinical manifestations
Mesangial proliferative (class II) = mesangial hypercellularity or matrix expansion, few subepithelial immune complexes - microscopic haematuria +/- proteinuria
Focal (class III) = <50% of glomeruli affected w/ hypercellularity and subendothelial/epithelial immune complex deposition - haematuria, proteinuria, reduced eGFR, HTN and nephrotic syndrome
Diffuse (class IV) = >50% of glomeruli affected, same histo as above - haematuria, proteinuria, urine cellular casts, HTN. reduced eGFR, reduced C£/r, increased anti-dsDNA
Membranous (class V) = Global or segmental subepithelial immune deposits - nephrotic syndrome, expansive proteinuria w. minimal haematuria
Advanced sclerosing (class VI) = >90% glomerulosclerosis - CKD
Cardiovascular manifestations of SLE
- Pericarditis (most common)
- Myocarditis (rare)
- Increased CV risk factor
- Libman-Sacks (non-infective) endocarditis (very rare)
Respiratory manifestations of SLE
- Pleurisy (most common)
- Pleural effusions
- Pulmonary HTN (rare)
Neurological manifestations of SLE
- Cognitive impairment
- Seizures
- Migraines
- Peripheral neuropathy
- Cranial neuropathy
- Psychiatric symptoms
- Movement disorders
- Headaches
Haematological manifestations of SLE
- Anaemia of chronic disease (common)
- Lymphopenia (common)
- AI haemolytic anaemia
- Thrombocytopenia
- Leukopenia
- Reactive lymphadenopathy and splenomegaly (esp in active disease)
- Antiphospholipid syndrome
GI manifestations of SLE
- Aseptic peritonitis
- Hepatosplenomegaly
What ANA titre is considered significant for SLE?
1:160
SLE management
- 1st line = NSAIDs and hydroxychloroquine
- Add long-term corticosteroids + DMARD if not working
- Belimumab (inhibits B cell stimulation ) = biologic
DMARDs used = hydroxychloroquine, methotrexate, leflunomide, azathioprine, mycophenolate
Which drugs can induce Lupus?
- Sulfadiazine
- Hydralazine
- Procainamide
- Isoniazid
- Methyldopa
- Quinidine
- Minocycline
- Chlorpromazine
Limited cutaneous systemic sclerosis features
CREST syndrome
- Calcinosis
- Raynaud’s (typically occurs for years before onset of symptoms)
- Oesophageal dysmotility (dysphagia, GORD)
- Sclerodactyly
- Telangiectasia
Skin fibrosis is limited to the hands and forearms, feet and legs, and head a neck
Diffuse cutaneous systemic sclerosis features
- CREST features not limited across whole body
- Abnormalities w/ blood vessels
- Fibrosis of internal organs (pulmonary fibrosis, renal crisis, myocardial disease, bowel hypomotility, pericarditis w/ effusion)
- Digital ulcers
- Tendon friction rubs and contractures
Pts may present with acute onset hands/feet swelling + Raynaud’s
Cutaneous systemic sclerosis (both types) management
Symptomatic management - DMARDs (mycophenolate, methotrexate, cyclophosphamide) can be used to treat skin thickening, but with limited evidence
Define Takayasu’s arteritis
A chronic granulomatous vasculitis affecting large arteries, particularly the aorta and its main branches, this can cause stenosis, occlusions and aneurysms - most common in Asian women aged 10-40
Takayasu’s arteritis features
Depends on the vessel involved:
- Subclavian artery = upper limb claudication’s
- Carotid and vertebral artery = headaches and presyncope
- Pulmonary artery = pulmonary HTN
- Cardiac = angina, HF and valvular pathologies
Systemic symptoms
Vascular exam shows:
- Abnormalities in peripheral pulses
- Bruits over the central pulses
- Asymmetric BP
- Aortic regurgitation (due to root dilatation)
Takayasu’s arteritis investigations
Imaging is requred for diagnosis:
- Angiography
MR/CT angiography and FDG-PET are the modalities of choice
Takayasu’s arteritis management
High dose corticosteroids + bone/GI protection
Define Trochanteric bursitis
Inflammation of the bursa situated over the greater trochanter
Trochanteric bursitis features
- Lateral hip pain, aggravated by physical activity and more severe at night
- Swelling + tenderness in affected area
- +ve Trendelenburg test
Trochanteric bursitis management
- Physio
- Analgesia
- Corticosteroid injections
- Bursectomy in severe or refractory cases
