MSK Vasculitides Flashcards

1
Q

What is GCA/temporal arteritis and Epidemiology?

A

Granulomatous inflammation of the large erteries affecting the external carotid artery, most commonly temporal

> 50YO, women
Associated with polymyalgia rheumatica

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2
Q

Presenation of GCA?

A

Unilateral headache, scalp tenderness, visual distrurbances e.g. loss of vision
Jaw claudication

Symptoms of PMR
FLAWS

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3
Q

Ix for GCA?

A
Increased ESR (before steroids)
Teemporal artery biopsy (skip lesions)
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4
Q

Diagnostic for GCA?

A

> 3 of age >50, new headache, ESR>50mm/hr, temporal artery abnormaility (tender, nonpulsatile), positive artwey biopsy (Multinucleated cells)

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5
Q

Mx for GCA?

A

High dose oral prednisolone even without definitive diagnosis

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6
Q

What is PMR and epidemiology?

A

inflammatory rheumatological condition with unknown cause with blatreal hip and shoulder girth pain and morning stiffness (NO WEAKNESS)

> 50YO, women
With GCA

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7
Q

Presenation of PMR?

A

Bilateral hip/shoulder girdle pain, morning stiffness
Subacute onset >2weeks
FLAWS

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8
Q

Ix and management for PMR?

A

Increased ESR/CRP, normal CK

Corticosteroids + bone protection

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9
Q

What is Takayasus arteritis?

A

large vessel vasculitis causing occlusion of the aorta and its main branches.

<40YO, asian, females

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10
Q

Presentation of Takayasus?

A

Limb claudication, Signs = absent pulses, bruits, unequal BP
Headache, TIA, Stroke
FLAWS

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11
Q

Ix for Takayasus?

A

Increased ESR and CRP

Angiography = CTA or MRA

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12
Q

What is polyarteritis nodosa and aetiology?

A

vasculitis affecting the medium sized vessels e.g. arterioles, capillaries and venules

Idiopathic or HEP B INFECTION

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13
Q

Presentation of polyarteritis nodosa?

A
Renal = HTN, renal failure, haematuria
Abdo = abdo pain, PR bleeding
Skin = livedo reticularis
Nerves = peripheral neuropathy
Athralgia
Systemic FLAWS
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14
Q

Ix for polyarteritis nodosa?

A

CTA/MRA see rosary sign

Bloods for Hep B, WCC and anaemia

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15
Q

How to split up small vessel vasculitis?

A

ANCA +ve = Churg strauss, wegeners, MPA

ANCa -VE = hpa, goodpastures

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16
Q

What is Wegeners granulomatosis?

A

Granulomatosis with polyangitis

Autoimmune necrotising granulomatous vasculitis characterised by triad organ involvement

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17
Q

Wegeners organ involvement?

A
URT = rhitis, epistaxis
LRT = haemoptysis, SOB
Kidneys = haematuria, dysuria
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18
Q

Ix for Wegeners?

A

cANCA
RBCs casts in urinalysis
CXR = cavitating lesions

SADDLE NOSE Deformity on examination

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19
Q

What is Chrug strauss syndrome?

A

Eosinophilic granulomatosis with polyangiitis.

Autoimmune vasculitis characterised by asthma and eosinophilia

20
Q

Presentation of Churg-strass?

A

Allergic (asthma/rhinitis) -> eosinophilic (tissue damage in lungs/GIT) -> Vasculitis (widespread organ damage)

21
Q

Ix for Chrug-strauss?

A

Increased pANCA

Very high eosinophils

22
Q

Presentation of microscopic polyangitis?

A

Small vessel ANCA associated vasculitis

Similar to Wegeners but no URT

Haemoptis and SOB, Haematuria and proteinuria, FLAW and mononeuritis multiplex

23
Q

Ix for microscopic polyangitis?

A

Bloods = pANCA, U&Es, increased Cr
urinalysis for haematuria and proteinurua
CXR for cavitating lesions
Renal biopsy = glomerulonephritis

24
Q

What is Behcets syndrome?

A

Multisystem disroer causing vasculitis of small, medium and large vessels

Eastern mediterranean
Men 20-40

BLA B51 Strong genetics

25
Q

Presentation of Behcets?

A

Cant see, cant pee, cant eat spicy

Anterior Uveitis, genital ulcers and oral ulcers

Thrombophlebitis, + DVT, arthritis, erytherma nodosum

26
Q

Epidemiology of SLE?

A

20-40YO, Female

Afro-carribean

27
Q

Presetation of SLE?

A
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

28
Q

Ix for SLE?

A

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

29
Q

triad for Anti-phospholipid syndrome?

A

Thomboembolism, thrombocytopaenia, recurrent miscarriage

30
Q

Where are other autoabs present?

A
Anti-CCP = RA
Anti-Jo-1 = myositis
Anti-centromere = CREST Syndrome
ASMA = AI hepatitis
31
Q

What is systemic sclerosis?

A

A rare connective tissue disroder characterised by widespread small blood vessel damage + fibrosis in skin and internal organs

32
Q

Features of pre-scleroderma?

A

Raynauds

ANA abs

33
Q

Features of scleroderma sine scleroderma?

A

Internal organs fibrosis without skin

34
Q

Features of limited cutaneous systeic sclerosis (CREST)

A
Calcinosis
Raynauds
Eosophageal dysmotility
Scerlodactyly
Telangiectasia

Anti-centromere ABs

35
Q

Features of diffuse cutaneous systemic sclerosis?

A

Skin changes involving trunk
Raynauds
Tendon friction
Lung, heart, GI and renal disease

Ab = anti-topoisomerase 2 (Anti-scle-70)

36
Q

Ix for systemic sclerosis?

A

Check organ function to see involvement

Lungs = CXR, CT
GI = endoscopy
Kidneys = U&Es
X ray for joints
skin = biopsy
37
Q

What is Sjogrens?

A

Autoimmune destruction of the exocrine glands

Females, associated with other AI conditions

38
Q

Aetiology of Sjogrens?

A

Primary sjogren syndrome (PSS)
Secondary to other AI e.g. RA, SLE, systemic slerosis
ANA +VE

39
Q

Px for Sjogrens?

A

DRY EYES
DRY mouth

Tiredness and muscle aches 
Parotitis
Renal tubular acidosis
Vaginal dryness - dyspareunia
FLAWS
40
Q

Ix for Sjogrens?

A

SCHRIMERS TEST
Abs = ANA in 70%, Rf in 50%, SSA in 70% PSS, SSB in 30% PSS

Biopsy + histology for focal lymphocytic infiltration

41
Q

What is polymyositis?

A

Gradual onset, diffuse proxmal msucle weakness, resp muscles and dysphagia, dysphonia and X rash

42
Q

WHat is deratomyositis?

A

Gradual onset,
Proximarl msucle weakness

Rash = macular mrash, gottrons papules and heliotrope rash

43
Q

Ix and organs in poly/dermatomyositis?

A

Organs = GI, Lungs and cardiac disease

Ix = CK
MUSCLE BIOPSY = GOld
EMG
autoantibodies
Poly = Anti-Jo
Dermato = ANA, ANti-MI-2, Anti-JO
44
Q

Epidemiology of sarcoidosis?

A

Afro-carribean and scandinavians

>50 YO

45
Q

presentation of sarcoidosis?

A
Uveitis
Pupus pernio
SOB + Dry cough
erytherma nodosum
Arthralgia
Arrhythmias

FLAWS

46
Q

Ix for sarcoidosis?

A

Bloods = high calcium, high ACE and high ESR

CXR = bilateral lymphadenopathy
Pulmonary fibrosis

Biopsy = non-caseating granuloma