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
Presentation of Behcets?
Cant see, cant pee, cant eat spicy Anterior Uveitis, genital ulcers and oral ulcers Thrombophlebitis, + DVT, arthritis, erytherma nodosum
26
Epidemiology of SLE?
20-40YO, Female | Afro-carribean
27
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
28
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
29
triad for Anti-phospholipid syndrome?
Thomboembolism, thrombocytopaenia, recurrent miscarriage
30
Where are other autoabs present?
``` Anti-CCP = RA Anti-Jo-1 = myositis Anti-centromere = CREST Syndrome ASMA = AI hepatitis ```
31
What is systemic sclerosis?
A rare connective tissue disroder characterised by widespread small blood vessel damage + fibrosis in skin and internal organs
32
Features of pre-scleroderma?
Raynauds | ANA abs
33
Features of scleroderma sine scleroderma?
Internal organs fibrosis without skin
34
Features of limited cutaneous systeic sclerosis (CREST)
``` Calcinosis Raynauds Eosophageal dysmotility Scerlodactyly Telangiectasia ``` Anti-centromere ABs
35
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)
36
Ix for systemic sclerosis?
Check organ function to see involvement ``` Lungs = CXR, CT GI = endoscopy Kidneys = U&Es X ray for joints skin = biopsy ```
37
What is Sjogrens?
Autoimmune destruction of the exocrine glands Females, associated with other AI conditions
38
Aetiology of Sjogrens?
Primary sjogren syndrome (PSS) Secondary to other AI e.g. RA, SLE, systemic slerosis ANA +VE
39
Px for Sjogrens?
DRY EYES DRY mouth ``` Tiredness and muscle aches Parotitis Renal tubular acidosis Vaginal dryness - dyspareunia FLAWS ```
40
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
41
What is polymyositis?
Gradual onset, diffuse proxmal msucle weakness, resp muscles and dysphagia, dysphonia and X rash
42
WHat is deratomyositis?
Gradual onset, Proximarl msucle weakness Rash = macular mrash, gottrons papules and heliotrope rash
43
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 ```
44
Epidemiology of sarcoidosis?
Afro-carribean and scandinavians | >50 YO
45
presentation of sarcoidosis?
``` Uveitis Pupus pernio SOB + Dry cough erytherma nodosum Arthralgia Arrhythmias ``` FLAWS
46
Ix for sarcoidosis?
Bloods = high calcium, high ACE and high ESR CXR = bilateral lymphadenopathy Pulmonary fibrosis Biopsy = non-caseating granuloma