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
What is polymyalgia rheumatica?
Inflammatory condition manifesting as pain and morning stiffness of neck, shoulder and hip girdle
26
How does GCA present?
``` Unilateral headache Scalp tenderness Tongue/jaw claudication Amaurosis fugax Unilateral blindness ```
27
How does PMR present?
Subacute onset of bilateral aching, tenderness and morning stiffness Fever Weight loss Anorexia
28
How do we investigate GCA?
ESR, CRP FBC - normocytic anaemia Biopsy after managed to confirm
29
How do we investigate PMR?
ESR | CRP (sensitive)
30
How do we treat GCA?
High dose methylprednisolone if blindness starting | Normal pred if before blindness
31
How do we treat PMR?
Rapid response to steroids within a week | Bone protection if steroids needed for 2+ years
32
What is Behcet's disease?
Systemic inflammatory vasculitis associated with HLA-B51
33
How does behcet's present?
Recurrent aphthous ulcers Genital ulcerations Erythema nodosum
34
How do we investigate behcet's disease?
Usually clinical diagnosis | Can do pathergy testing (needle in forearm, papule forms in 48 hours)
35
How do we manage behcet's?
Orogenital ulceration - topical steroids | Organ involvement - steroids plus immunosuppressant (azathioprine)
36
What is polyarteritis nodosa?
Necrotising inflammation of medium sized arteries without vasculitis in arterioles, venules or capillaries.
37
How does polyarteritis nodosa present?
``` Systemic features Skin - rash, ulcers, nodules Renal - stenosis, ischaemia, HTN Cardio GI - pain, melaena Livedo reticularis ```
38
How do we investigate polyarteritis nodosa?
``` FBC - elevated WCC HBV - positive HbsAg potentially ESR, CRP MRA - string of pearls appearance Renal biopsy ```
39
How do we treat polyarteritis nodosa?
Prednisolone