14. Autoimmune MSK Flashcards

1
Q

What is sarcoidosis?

A

Chronic granulomatous disorder of unknown cause. Characterised by T lymphocytes and macrophages driving non-caseating granuloma formation (no necrosis in granulomas).

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2
Q

How does sarcoidosis present?

A

Fever
Erythema nodosum
Polyarthralgia
Bilateral hilar lymphadenopathy
Pulmonary - dry cough and progressive dypnoea
Extra pulm - organomegaly, uveitis, LUPUS PERNIO, subcut nodules, arrhythmia, symptoms of hypercalcaemia

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3
Q

Investigations for sarcoidsosis?

A

Increased ESR
Lymphopenia
Hypercalcaemia from high neutrophils
Raised ACE!! Very specific as lung damage

CXR -
Stage 1 - bihilar lymphadenopathy
2 - BHL and infiltrates
3. infiltrates alone
4. extensive fibrosis

Biopsy diagnostic

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4
Q

How to treat sarcoidosis?

A

Conservative

High dose corticosteroids

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5
Q

What is SLE?

A

Wrongful response to host that causes immune complex formation with poor clearance. Type III hypersensitivity reaction.
Related to HLA-DR3

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6
Q

How does SLE present?

A
Malar or discoid rash
Photosensitive rash
Oral ulcers
Arthritis
Serositis (pleuritis or pericarditis)
Renal disorders
Neuro disorder
Haem
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7
Q

SLE Investigations?

A

Anaemia, lymphopenia, thrombocytopenia
Raised ESR but normal CRP
ANA, anti-dsDNA and antihistone

C3 and C4 depletion

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8
Q

SLE Treatment?

A

Maintain with NSAIDS and hydroxychloroquine

Mild - low dose steroids
Moderate - DMARDS
Severe - high dose steroids

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9
Q

What is sjorgren’s syndrome?

A

Chronic inflammatory disorder characterised by lymphocytic infiltration and destruction of exocrine glands

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10
Q

How does sjorgren’s present?

A
Keratoconjunctivitis sicca - dry eye
Xerostomia - dry mouth
Parotid swelling
Polyarthritis
Raynaud's
Vasculitis
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11
Q

Investigations for Sjogren’s?

A

Schirmer’s test
Autoantibodies - anti-ro and anti-la but not specific
Salivary gland biopsy diagnostic

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12
Q

Treatment for Sjogren’s?

A

Dry eye - eye drops
Dry mouth - salivary substitutes
Severe disease - immunosuppressants e.g. steroids

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13
Q

What is systemic sclerosis?

A

Multisystemic autoimmune disease by abnormalities in non-inflammatory bloods vessels, fibrosis of skin and internal organs.

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14
Q

What are the types of systemic sclerosis?

A

Limited - hands, face and feet

Diffuse - wide spread organ involvement

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15
Q

How does systemic sclerosis present?

A

Puffy, taut skin with no wrinkles
Renal scleroderma
Pulmonary fibrosis
GI reflux, dysmotility

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16
Q

Investigations for systemic sclerosis

A

Anti-centromere in limited

Anti-scl-70 and anti-rna polymerase for diffuse

17
Q

How to manage systemic sclerosis?

A

Suppress immune system and treat symptoms

18
Q

What is poly/dermatomyositis?

A

Rare condition characterised by onset of progressive, symmetrical proximal muscle weakness. Often associated with paraneoplastic phenomena e.g. ovarian cancer

19
Q

Polymyositis pathology?

A

Endomysial inflammation with CD8+ T cells

20
Q

Dermatomyositis pathology?

A

Perimysial inflammation with CD4+ T cells

21
Q

How does polymyositis present?

A

Progressive symmetrical muscle weakness

22
Q

How does dermatomyositis present?

A

Gottron’s papules
Facial erythema
Shawl rash
Mechanics hands - dark, thick, irregular patches

23
Q

How do we investigate poly/dermatomyositis?

A

Muscle enzymes - CK and aldolase
EMG
Muscle biopsy - inflammation
Autoantibodies - anti-srp, anti-mi2 and anti-jo1

24
Q

What is giant cell arteritis/temporal arteritis?

A

Large vessel granulomatous vasculitis of external carotid artery branches
Usually comes hand in hand with PMR

25
Q

What is polymyalgia rheumatica?

A

Inflammatory condition manifesting as pain and morning stiffness of neck, shoulder and hip girdle

26
Q

How does GCA present?

A
Unilateral headache
Scalp tenderness
Tongue/jaw claudication
Amaurosis fugax
Unilateral blindness
27
Q

How does PMR present?

A

Subacute onset of bilateral aching, tenderness and morning stiffness
Fever
Weight loss
Anorexia

28
Q

How do we investigate GCA?

A

ESR, CRP
FBC - normocytic anaemia

Biopsy after managed to confirm

29
Q

How do we investigate PMR?

A

ESR

CRP (sensitive)

30
Q

How do we treat GCA?

A

High dose methylprednisolone if blindness starting

Normal pred if before blindness

31
Q

How do we treat PMR?

A

Rapid response to steroids within a week

Bone protection if steroids needed for 2+ years

32
Q

What is Behcet’s disease?

A

Systemic inflammatory vasculitis associated with HLA-B51

33
Q

How does behcet’s present?

A

Recurrent aphthous ulcers
Genital ulcerations
Erythema nodosum

34
Q

How do we investigate behcet’s disease?

A

Usually clinical diagnosis

Can do pathergy testing (needle in forearm, papule forms in 48 hours)

35
Q

How do we manage behcet’s?

A

Orogenital ulceration - topical steroids

Organ involvement - steroids plus immunosuppressant (azathioprine)

36
Q

What is polyarteritis nodosa?

A

Necrotising inflammation of medium sized arteries without vasculitis in arterioles, venules or capillaries.

37
Q

How does polyarteritis nodosa present?

A
Systemic features
Skin - rash, ulcers, nodules
Renal - stenosis, ischaemia, HTN
Cardio
GI - pain, melaena
Livedo reticularis
38
Q

How do we investigate polyarteritis nodosa?

A
FBC - elevated WCC
HBV - positive HbsAg potentially
ESR, CRP
MRA - string of pearls appearance
Renal biopsy
39
Q

How do we treat polyarteritis nodosa?

A

Prednisolone