MSK Vasculitides Flashcards
What is GCA/temporal arteritis and Epidemiology?
Granulomatous inflammation of the large erteries affecting the external carotid artery, most commonly temporal
> 50YO, women
Associated with polymyalgia rheumatica
Presenation of GCA?
Unilateral headache, scalp tenderness, visual distrurbances e.g. loss of vision
Jaw claudication
Symptoms of PMR
FLAWS
Ix for GCA?
Increased ESR (before steroids) Teemporal artery biopsy (skip lesions)
Diagnostic for GCA?
> 3 of age >50, new headache, ESR>50mm/hr, temporal artery abnormaility (tender, nonpulsatile), positive artwey biopsy (Multinucleated cells)
Mx for GCA?
High dose oral prednisolone even without definitive diagnosis
What is PMR and epidemiology?
inflammatory rheumatological condition with unknown cause with blatreal hip and shoulder girth pain and morning stiffness (NO WEAKNESS)
> 50YO, women
With GCA
Presenation of PMR?
Bilateral hip/shoulder girdle pain, morning stiffness
Subacute onset >2weeks
FLAWS
Ix and management for PMR?
Increased ESR/CRP, normal CK
Corticosteroids + bone protection
What is Takayasus arteritis?
large vessel vasculitis causing occlusion of the aorta and its main branches.
<40YO, asian, females
Presentation of Takayasus?
Limb claudication, Signs = absent pulses, bruits, unequal BP
Headache, TIA, Stroke
FLAWS
Ix for Takayasus?
Increased ESR and CRP
Angiography = CTA or MRA
What is polyarteritis nodosa and aetiology?
vasculitis affecting the medium sized vessels e.g. arterioles, capillaries and venules
Idiopathic or HEP B INFECTION
Presentation of polyarteritis nodosa?
Renal = HTN, renal failure, haematuria Abdo = abdo pain, PR bleeding Skin = livedo reticularis Nerves = peripheral neuropathy Athralgia Systemic FLAWS
Ix for polyarteritis nodosa?
CTA/MRA see rosary sign
Bloods for Hep B, WCC and anaemia
How to split up small vessel vasculitis?
ANCA +ve = Churg strauss, wegeners, MPA
ANCa -VE = hpa, goodpastures
What is Wegeners granulomatosis?
Granulomatosis with polyangitis
Autoimmune necrotising granulomatous vasculitis characterised by triad organ involvement
Wegeners organ involvement?
URT = rhitis, epistaxis LRT = haemoptysis, SOB Kidneys = haematuria, dysuria
Ix for Wegeners?
cANCA
RBCs casts in urinalysis
CXR = cavitating lesions
SADDLE NOSE Deformity on examination
What is Chrug strauss syndrome?
Eosinophilic granulomatosis with polyangiitis.
Autoimmune vasculitis characterised by asthma and eosinophilia
Presentation of Churg-strass?
Allergic (asthma/rhinitis) -> eosinophilic (tissue damage in lungs/GIT) -> Vasculitis (widespread organ damage)
Ix for Chrug-strauss?
Increased pANCA
Very high eosinophils
Presentation of microscopic polyangitis?
Small vessel ANCA associated vasculitis
Similar to Wegeners but no URT
Haemoptis and SOB, Haematuria and proteinuria, FLAW and mononeuritis multiplex
Ix for microscopic polyangitis?
Bloods = pANCA, U&Es, increased Cr
urinalysis for haematuria and proteinurua
CXR for cavitating lesions
Renal biopsy = glomerulonephritis
What is Behcets syndrome?
Multisystem disroer causing vasculitis of small, medium and large vessels
Eastern mediterranean
Men 20-40
BLA B51 Strong genetics
Presentation of Behcets?
Cant see, cant pee, cant eat spicy
Anterior Uveitis, genital ulcers and oral ulcers
Thrombophlebitis, + DVT, arthritis, erytherma nodosum
Epidemiology of SLE?
20-40YO, Female
Afro-carribean
Presetation of SLE?
SOAP BRAIN MD Serositis (pericarditis/pleuritis) Oral ulcers Arthritis Photosensitivity Bloods (down) Renal (Proteinuria/casts/GN) ANA Immunological (Anti-dsDNA) Neuro (psychosis/seizures)
Malar rash
Discoid rash
Also FLAWS, lymphadenopathy, livedo reticularis, alopecia, raynauds
Ix for SLE?
Bloods = High ESR, U&Es, LFTs, FBC
Autoabs = ANA
Anti-dsDNA more specific
Anti-cardiolipin (-> anti-phospholipid syndrome)
Urinalysis = casts, proteinurea, haematuria
Imaging = CXR, joint XRAY
triad for Anti-phospholipid syndrome?
Thomboembolism, thrombocytopaenia, recurrent miscarriage
Where are other autoabs present?
Anti-CCP = RA Anti-Jo-1 = myositis Anti-centromere = CREST Syndrome ASMA = AI hepatitis
What is systemic sclerosis?
A rare connective tissue disroder characterised by widespread small blood vessel damage + fibrosis in skin and internal organs
Features of pre-scleroderma?
Raynauds
ANA abs
Features of scleroderma sine scleroderma?
Internal organs fibrosis without skin
Features of limited cutaneous systeic sclerosis (CREST)
Calcinosis Raynauds Eosophageal dysmotility Scerlodactyly Telangiectasia
Anti-centromere ABs
Features of diffuse cutaneous systemic sclerosis?
Skin changes involving trunk
Raynauds
Tendon friction
Lung, heart, GI and renal disease
Ab = anti-topoisomerase 2 (Anti-scle-70)
Ix for systemic sclerosis?
Check organ function to see involvement
Lungs = CXR, CT GI = endoscopy Kidneys = U&Es X ray for joints skin = biopsy
What is Sjogrens?
Autoimmune destruction of the exocrine glands
Females, associated with other AI conditions
Aetiology of Sjogrens?
Primary sjogren syndrome (PSS)
Secondary to other AI e.g. RA, SLE, systemic slerosis
ANA +VE
Px for Sjogrens?
DRY EYES
DRY mouth
Tiredness and muscle aches Parotitis Renal tubular acidosis Vaginal dryness - dyspareunia FLAWS
Ix for Sjogrens?
SCHRIMERS TEST
Abs = ANA in 70%, Rf in 50%, SSA in 70% PSS, SSB in 30% PSS
Biopsy + histology for focal lymphocytic infiltration
What is polymyositis?
Gradual onset, diffuse proxmal msucle weakness, resp muscles and dysphagia, dysphonia and X rash
WHat is deratomyositis?
Gradual onset,
Proximarl msucle weakness
Rash = macular mrash, gottrons papules and heliotrope rash
Ix and organs in poly/dermatomyositis?
Organs = GI, Lungs and cardiac disease
Ix = CK MUSCLE BIOPSY = GOld EMG autoantibodies Poly = Anti-Jo Dermato = ANA, ANti-MI-2, Anti-JO
Epidemiology of sarcoidosis?
Afro-carribean and scandinavians
>50 YO
presentation of sarcoidosis?
Uveitis Pupus pernio SOB + Dry cough erytherma nodosum Arthralgia Arrhythmias
FLAWS
Ix for sarcoidosis?
Bloods = high calcium, high ACE and high ESR
CXR = bilateral lymphadenopathy
Pulmonary fibrosis
Biopsy = non-caseating granuloma
