Rheumatology 2 Flashcards
General groups of symptoms suffered by sle patients?
General - fatigue, malaise, fever
Skin - malar rash, photosensitivity, discoid rash
Vessels - raynauds, purpuria
Blood - haemeolytic anaemia
Joints - arthritis
Muscles - myalgia
Hair - alopecia
Lungs - pleurisy, pnemonitis, fibrosis
Heart - pericariditis, valve disease, ihd/cve, venous thrombosis
Kidney - nephrotic or nephritic syndrome, renal artery stenosis
Cns - depression, epilepsy, migraines, cn lesion
Eyes - sjogrens,
Gi - mouth ulcers, autoimmune hepititis
Markers of SLE?
ANA +ve -95%
DsDNA +ve - 60%
ENA +ve - 15-30%
Clues of sle on normal bloods?
Esr raised, crp normal Complement low (used up)
Why may ANA be raised?
SLE RA Sjogrens Scleroderma Normal varient
How is ana expressed? What does this mean?
As a titre - the number of times it can be diluted and still detectable thus 1:160 is more significant that 1:40
What may cause a raised RF?
Sjogrens RA SLE Infection Normal
What are ENA? Main types?
Anti-extractable nuclear antigen Anti-la Anti-ro Anti-sm Anti-jo
What conditions can caused raised ena anti-ro/la/sm?
Sjogrens, sle,
What conditions will cause a raised anti-jo?
Polymyositis, dermatomyositis
Causes of SLE?
Hereditary
Drug induced
Risk factors for SLE
Female Uv light EBV Afrocaribbean HLA +ve
Arthritis pattern in SLE?
RA like - small joints, symmetrical
Not erosive nor does it cause deformity
Treatments of SLE generally
NSAIDS
Topical corticosteroids
Oral corticosteroids for flares
Immunosupressants - hydroxychloroquinine, azothioprine, methotrexate, ciclosporin.
What would indicate the need for high dose corticosteroids in a lupus flare?
Renal problems
Cerebral problems
Haemolytic ananemia
In lupus where does the malar rash spare?
Nasolabial folds
What condition is frequently associated with SLE? What is it?
Antiphospholipid syndrome
Again, autoantibodies against apoptotic blebs
What does antiphospholipid syndrome cause?
Arterial and venous thrombosis
Recurrent miscarriage
What test is positive in antiphospholipid syndrome?
Antiphospholipid antibodies - uses dilute russel viper venom time drvvt
Treatment for antiphospholipid syndrome
Low dose aspirin
Warfarin if previous thrombosis
Sc heparin and aspirin when pregnant
What condition is frequently associated with RA? What is it?
Sjogrens syndrome
Autoimmune reaction against glands
What symptoms with sjogrens syndrome?
Dry eyes Dry mouth Dry skin Dry vagina Raynauds Fatigue
Positive tests in sjogrens?
Schirmers tear test
Ana, anti-ro and anti-la
Treatment of sjogrens
Artificial tears
Saliva replacement
Hydroxycholoroquinine for fatigue or arthralgia
What condition causes malaise weight loss and fever accompanied by symmetrical shoulder and pelvis muscle weakness and waisting?
Polymyositis
What condition accompanies polymyositis? What s+s does it have in addition?
Dermatomyositis
Purple helitrope rash on eyelids with oedema
Rough papules on fingers - gottrons
Nailfold erythema
Complications of poly and dermato myositis
Resp failure from resp muscle involvement
Dysphonia from laryngeal muscle involvement
Dysphagia from oesophageal muscle involvement
High risk of malignancy - either predating onset or following
Diagnosis of poly and dermatomyositis
- raised muscle enzymes - CK, lactate dehydroginase
- ana, rf
- emg
- mri
- muscle biopsy
Treatment of poly and dermatomyositis
High dose prednisolone
Taper and cover with steroid sparing agent
IV immunoglobulins
Bed rest
Pain distribution in fibromyalgia?
Pain above and below the waist
Aching and constant
Tender trigger points
Associated symptoms in fibromyalgia
Fatigue Depression Sleep disturbance IBS, ME Headaches
Treatment of fibromyalgia?
Reassurance
Exercise
Depression treatment
Pain relief (gabapentin, amitryptyline, nsaids)
What is scleroderma? What are the subdivisions?
Systemic sclerosis.
Limited cutaneous scleroderma
Diffuse cutaneous scleroderma
Features of limited cutaneous scleroderma?
C - calcinosis
R - raynauds
E - esophogeal involvement
S - sclerodactyly (swollen tight digits)
T - telangiectasia (visible blood vessels in the cheeks)
Tight skin on hands, feet and face with microstomia and flexion deformities of fingers.
Can cause pulmonary hypertension
What is diffuse cutanious sclerosis?
Global tight thick skin
Organ fibrosis - lungs (fibrosis), heart, GI (atony), renal (htn),
What tests may be positive in scleroderma?
Anaemia
Rf
Ana
Anti centromere antibodies
Other than bloods what other tests should be done in suspected scleroderma?
Cxr and spirometry
Barium swallow
Management of scleroderma?
Symptomatically
Lubrication and stretches for skin
Steroids or immunosupression if lungs involved
PPI and prokinetic drugs for gi involvement
Supplementation for malabsorption
ACEi and steroids for kidneys
Vasodilators for pulmonary hypertension
Conditions associated with raynauds
Idiopathic RA Scleroderma SLE Sjogrens Poly and dermatomyositis Anorexia nevosa Athrosclerosis Beta blockers Exposure to vibration Trauma Malignancy
Treatment of raynauds
Hand warmers
Avoid rapid temp change
Calcium channel blockers
What is the pathology behind SLE?
Apoptotic bleb removal inefficient so taken up by lymphocytes
Results in autoantibody production by lymphocytes
Lack of recognition of self antibodies in thymus
Immune complexes form in circulation, complement activated, cytokines increase.
Generic characteristics of the seronegative arthropathies?
-RF Associated with HLA B27 Effect the spine and SI joint Asymmetrical arthritis Enthesis Dactylitis Iritis, aortic regurgitation, oral ulcers, IBD
What are the seronegative spondyloarthropathies?
AS
Reactive arthritis
Psoriatic arthritis
Pathology of ankalosing spondylitis?
Immune cell infiltration of bones and ligaments
Causes erosions and enthesitis
New bone forms over where ligaments were
Presentation of ankalosing spondylitis back pain?
Gradual onset lower back pains Worse overnight and in the mornings Relieved by exercise Radiates to hip and buttocks Loss of spinal movement in all directions
Tendons most commonly effected by enthesitis in AS?
Achilles
Plantar
Signs of ankalosing spondylitis
\+ve schobers Kyphosis Retains lumber lordosis when bending forwards Question mark posture Bamboo spine
What chest problems effect ankalosing spondylitis patients?
Costochondritis
Lack of expansion
Pulmonary fibrosis
Treatment of ankylosing spondylitis
Physiotherapy Nsaids Local steroids Methotrexate for peripheral arthritis Infliximab if persistent
What proportionof psoriasis patients develop psoriatic psoriasis?
5-8%
How does psoriatic arthritis present (arthritis only!)?
Asymetrical oligoarthritis
Effects the dipj
Effects the spine in 15%
Associated features of psoriatic arthritis?
Dactylitis
Psoriasis!
Radiological changes in psoriatic arthritis?
Central erosions forming pencil in cup
Treatment of psoriatic arthritis?
Nsaids
Intraarticular steroids
Dmards - methotrexate, sulphasalazine
Anti tnf
Issue with using nsaids in psoriatic arthritis?
Can worsten skin
What infections does reactive arthritis tend to follow?
Urethritis
STIs - gonorrhoea
Dysentry
How does reactive arthritis present?
Asymetrical lower limb arthritis
Days to weeks after infection
What is reactive arthritis associated with? Triad?
Cant see (conjunctivitis), cant pee (urethritis), cant bend the knee (arthritis) Enthesitis Sacroillitis Spondylitis Skin lesions
What skin lesions appear in reactive arthritis?
Balanitis
Psoriasis like lesions
Nail dystrophy
Treatment of reactive arthritis?
Nsaids and intra articular steroids
Splint
Stool culture and sti screen
If persistant dmards and anti tnf
How can small vessel vasculitis be subdivided? What conditions fall into each?
ANCA -ve - henkch schonlein purpura
ANCA +ve - granulomatosis with polyangiitis, churg strauss
What is henoch schonlein purpura? How does it present? Treatment? Prognosis?
IgA deposition in small vessels following respiratory infection
Presents with - lower limb purpura, transient polyarthritis, abdominal pain, glomerulonephritis
Supportive treatment
1% develop chronic renal damage
What condition is on the spectrum of henoch schonlein purpura?
IgA nephropathy
Presentation of granulomatosis with polyangiitis
General malaise
Upper resp - rhinorrhoea, nasal ulceration
Lungs - cough, haemoptysis, pleuritic pain
Kidneys - haematauria, proteinurea
CXR findings of granulomatosis with polyangiitis
Cxr - nodular masses and cavitation that migrate!
Blood tests results on granulomatosis with polyangiitis
cANCA +++
pANCA +
Management of granulomatosis with polyangiitis
Steroids
Methotrexate
Azathioprine
Rituximab
Presentation of churg strauss
General malaise and fever Rhinitis Asthma Eosinophilia Vasculitis - purpura, digital ischemia Subcutanious nodules
Blood tests in churg strauss
pANCA +++
cANCA +
Treatment of churg strauss
Corticosteroids
Name two medium vessel vasculitis?
Polyarthritis nodosa
Kawasakis disease
How does kawasakis present?
Fever >5 days Bilateral conjunctiva reddening Dry red lips and oral cavity Cervical lymphadenopathy Redness and oedema to palms and soles
Treatment of kawasakis?
Complication of disease?
Complication of treatment?
Iv immunoglobulins and high dose aspirin
Coronary artery disease
Reyes syndrome
At risk groups for kawasakis?
Under 5
Japanese
Post infection
Two large vessel vasculitis disease
Polymyalgia rheumitica
Giant cell arteritis
Presentation of polymyalgia rheumitica
Sudden onset symmetrical proximal limb pain (shoulders, hips)
Normal strength
Worse in morning, eases over day
Systemic weight loss, fatigue, fever
Investigation findings in polymyalgia rheumitica
Raised crp and esr
Anaemia
Raised alp and ggt
Treatment of polymyalgia rheumitica
Corticosteroids
Prognosis of polymyalgia rheumitica?
Rapid (days) reduction in symptoms
May need long term steroids or steroid sparing agents to slowly reduce dose
Presentation of giant cell arthritis?
Severe headache Worse on chewing Tender scalp eg on brushing hair Jaw claudication Painless sudden loss of vision in one eye
Examination findings in temporal arteritis?
Tenderness and swelling to temporal artery
Loss of pulsatile temporal artery
Bloods in giant cell arteritis?
Crp ++++
High plasma viscosity
Problem with temporal artery biopsy for diagnosing temporal arteritis?
Lesions are patchy so can be missed
Treatment for giant cell arteritis
High dose prednisolone with bone protection
Steroid sparing agents long term
Who is at risk of giant cell arteritis
People with previous polymyalgia rheumitica
What is Bechet’s? How does it present?
Inflammation of blood vessels
Genital and mouth ulcers, red painful eyes, spots, headaches, arthralgia
Who is most effected by Behcets?
Mediteranian and middle eastern
What is Bechet’s? How does it present?
Inflammation of blood vessels
Genital and mouth ulcers, red painful eyes, spots, headaches, arthralgia
Who is most effected by Behcets?
Mediteranian and middle eastern