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Stage 4 CSIM > Multi-System Disease > Flashcards

Multi-System Disease Flashcards

1
Q

Describe the aetiology of SLE

A

Not fully understood
Loss of tolerance to self
production of immune complexes - autoantibodies and antigens
These are the deposited in affected organs

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2
Q

How might SLE present?

A

CONSTITUTIONAL SYMTPOMS
fever, fatigue, weight loss
MUCOUS MEMBRANES
ulceration of hard palate
SKIN
acute lupus malar rash with sparing of nasolabial folds
ALOPECIA
REYANUDS PHENOMENON
VASCULITIS
RENAL
~50% of patients, lupus nephritis
LUNG
pleuritis, pneumonitis, ILD, pulmonary HTN, alveolar haemorrhage
CARDIAC
pericarditis, non-infective endocarditis (Libman Sacks Syndrome), myocarditis, CAD
NEURO
cognitive dysfunction, delirium, psychosis, seizures, headache, peripheral neuropathies
OPTHALMOLOGIC
keratoconjunctivitis sicca (secondary sjogrens)
HAEMATOLOGIC
anaemia, leukopenia, thrombocytopenia, lymphadenopathy, splenomegaly
GI TRACT
oesophagitis, lupus hepatitis, acute pancreatitis

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3
Q

Describe Jaccoud’s Arthropathy

A

Seen in 90-95% of patients with Lupus at some point in the disease
Deforming NON-EROSIVE Arthropathy
Characterised by ulnar deviation of the 2nd - 5th fingers with MCP subluxation
Thought to be caused by ligamentous laxity
when hand relaxed - looks normal
when patient tries to move hands and use ligaments - hand looks deformed

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4
Q

What are the 6 screening questions you should ask in a patient who you suspect CTD?

A 
Hair Loss
Dry Eyes
Rashes (esp photosensitive)
Mouth Ulcers
Raynauds
Joint Pains
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5
Q

What could you argue is one of the most important tests in a patient with SLE to look at disease activity

A

Urine Dipstick
= MUST TEST - FIRST SIGN OF RENAL INVOLVEMENT
look for proteinuria and haematuria for nephritis

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6
Q

What specific blood test results would you expect to see in a patient with SLE?

A

Anti-nuclear antibody ANAs (increased - useful in distinguishing SLE from other CTDs)
Anti ds DNA antibody (increased in active disease)
Complement levels (decrease in active disease)
Lymphocytes (classically reduced in active lupus)

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7
Q

How do you diagnose anti-phospholipid syndrome?

A

AT LEAST ON CLINICAL FEATURE
Vascular event e.g. DVT, VTE, stroke, TIA
Pregnancy morbidity including fetal death after 10 weeks gestation, pre-eclampsia or placental insufficiency, multiple (3 or more) embryonic losses (

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8
Q

List the aetiology of antiphosphlipid syndrome

A

Can be PRIMARY
Or SECONDARY - most commonly to SLE
should always ask SLE patients if they have a history of VTE or pregnancy morbidity

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9
Q

To diagnose SLE you need 4 out of 11 of the diagnostic criteria (at least one clinical and one immunologic), what are the 11 diagnostic criteria?

A

1- Malar Rash
2 - Discoid Rash
3- Photosensitivity
4- Oral Ulcers
5 - Non-erosive arthritis
6 - Serositis e.g. pleuritis, pericarditis
7 - Renal Disorder - persistent proteinuria or cellular clasts
8 - CNS Disorder - seizures or psychosis
9 - Haematological Disorder - haemolytic anaemia, lymphopenia, thrombocytopenia
10 - Immunological Disorder anti dsDNA antibody
11 - ANA positive

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10
Q

If the APTT is prolonged in a patient with ?antiphopholipid antibody syndrome, what further test should be done?

A

A lupus anticoagulant effect that effects APTT will not correct in a mixing study with normal plasma, then do dRRVT (will also be prolonged)

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11
Q

What patient population does SLE usually affect?

A

Women of child bearing age

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12
Q

Describe Reynauds Phenomenon

A

Episodic vasospasm of extremities triggered by cold and emotional stress, fingers go white (decreased circulation due to vasospasm), then blue (as recirculated blood is deoxygenised very quickly to hypoxic tissue) and then painful and red (as tissues go back to normal)
Primary Reynauds is common 6-10% of general population
Secondary Reynauds has many causes

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13
Q

List some of the causes of Secondary Reynauds and when are you more likely to think of a secondary cause?

A

CHEMICAL OR DRUG EXPOSURE
e.g. beta blockers, COCP, chemotherapy
OCCLUSIVE ARTERIAL DISORDERS
e.g. external compression, carpal tunnel syndrome, buergers disease, thrombosis and embolization
OCCUPATION
e.g. vibrating machinery, cold
INTRAVASCULAR
e.g. ploycythemia vera, leukaemia, thrombocytosis,
MISCELLANEOUS
e.g. malignancy, anorexia nervosa
INCREASING AGE OF ONSET - more likely to be pathological process than primary.

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14
Q

Describe the spectrum of scleroderma

A

Localized Scleroderma -Thickening of skin and dermis
Systemic Sclerosis
Limited Systemic Sclerosis (CREST)
Diffuse Systemic Sclerosis

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15
Q

Describe the clinical features of LIMITED SYSTEMIC SCLEROSIS

A

Formerly known as CREST syndrome - anti centromere
Calcinosis (deposits of calcium phosphate in fingertips)
Raynauds
E(oesophageal dysmotility)
Sclerodactyly (distal) + scleroderma face and neck
Telangectasia
(now can also get pulmonary hypertension, fibrotic lung disease and mid gut disease)

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16
Q

Describe the clinical features of DIFFUSE SYSTEMIC SCLEROSIS

A 
Raynauds
Digital Ulceration
Scleroderma proximal to elbows
Interstitial Lung Disease
GI
Renal Cardiac 
Scl-70 and RNA polymerase III antibodies
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17
Q

If you had a patient with Reynauds, what test could you do to look for progression to systemic sclerosis?

A

Nail Fold Capillaroscopy - changes very strongly associated with progression to systemic sclerosis.

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18
Q

Why do you have to be careful when prescribing steroids in a patient with systemic sclerosis?

A

Can precipitate a RENAL CRISIS
Hypertension, rapidly deteriorating renal function, flash pulmonary oedema, seizures
Can protect patients from this by giving them ACE inhibitors

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19
Q

Describe what is meant by Sicca Symptoms

A

Lymphocytic infiltration of exocrine glands

Mucosal Dryness - eyes, mouth, larynx, pharynx and vagina

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20
Q

What are the causes of Sicca Symptoms?

A

Primary Sjogrens Syndrome
most strongly associated - systemic autoimmune
Secondary Sjogrens Syndrome
e.g. to RA, SLE, systemic sclerosis, MCTD

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21
Q

What disease are people with Primary Sjogrens Syndrome at a 20% increased risk from?

A

Lymphoma (often MALT - mucosal associated lymphoma)

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22
Q

What symptoms do you get in Primary Sjogrens Syndrome?

A 
Fatigue
Dry Eyes 
Dry Mouth
Vaginal Dryness
Parotid and Submandibular Gland Swelling
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23
Q

What is the typical autoantibody profile of Primary Sjogrens Syndrome?

A 
RF+
ANA+
anti Ro +
anti La+
IgG++++
ESR ++++ (>100)
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24
Q

What investigations can be done to investigate Primary Sjogrens Syndrome?

A 
Autoantibodies
Schirmer Test
Parotid and submandicular gland USS
Minor labial gland biopsy
Salivary Flow
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25
Q

Myalgia is commonly associated with many autoimmune diseases, what symptoms would make you more concerned and make you think of an inflammatory myopathy?

A 
Muscle Weakness (usually proximal and symmetrical)
Raised Muscle Enzymes
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26
Q

Describe the salient points of polymyositis

A 
Inflammation of striated muscle
Proximal Muscle Weakness
Distal muscles spared until late disease
Occular muscles usually spared
NO RASH
Creatine Kinase raised, also AST and ALT (produced by muscle)
20% have Jo-1 mutation - should be investigated for interstitial lung disease
Definitive Test = muscle biopsy
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27
Q

Describe the salient points of dermatomyositis.

A

Proximal muscle weakness with distal sparing until late disease and occular sparing
Usually systemic upset - fever, arthralgia, weight loss
RASH - blue purple discolouration of upper eyelids, periorbital oedema, flat red rash on face and upper trunk, raised purple/red scaly patches on extensor surfaces (Gottrons Papules)
Investigations - again raised CK, AST and ALT, muscle biopsy is definitive test, also thorough malignancy screen - STRONG ASSOCIATION WITH MALIGNANCY.

28
Q

What antibody is associated with mixed connective tissue disease?

A

RNP

29
Q

What clinical features have a higher incidence in MCTD?

A

Erosive Arthritis

Pulmonary Artery Hypertension

30
Q

In one sentence describe Sarcoidosis

A

A multisystem disorder with unknown aetiology, characterised by non-caeseating granulomas in involved organs

31
Q

What is the organ most commonly affected in Sarcoidosis?

A

Lung

32
Q

Describe the staging of pulmonary involvement in sarcoidosis

A 
STAGE 0
Normal CXR
STAGE 1
Bilateral Hilar Lymphadenopathy (BHL)
STAGE 2
BHL + Infiltrates
STAGE 3
Dense pulmonary infiltrates with functional limitation +/- cor pulmonale + no BHL
STAGE 4 
Fibrosis
33
Q

How does acute sarcoidosis usually present?

A

With erythema nodosum and polyarthralgia

34
Q

If you see BHL on CXR what next investigation should be ordered?

A

HRCT

35
Q

What are the 4 differential diagnoses when you see BHL on a CXR

A

Sarcoidosis
Lymphoma
Pulmonary TB
Malignant Infiltration of Mediastinal LN e.g. from carcinoma of bronchus

36
Q

Describe the investigations you would do in Sarcoidosis.

A

BLOODS
Raised ESR, serum ACE, LFTs, lymphopenia, hypercalcaemia, and raised immunoglobulin’s
CXR + HRCT
ECG and lung function tests
Tissue Biopsy - will show non-caeseating granulomas

37
Q

Describe the management of sarcoid

A

BHL only
No Tx - most recover spontaneously
Acute Sarcoidosis
Bed rest and NSAIDs
Indications for corticosteroids include
parenchymal lung disease, uveitis, hypercalcaemia, neurological or cardiac involvement. High Dose 4-6 weeks and then taper down based on clinical picture
In refractory disease - immunosuppressant’s, or lung transplantation.

38
Q

What are the names of the large vessel vasculitises and Ix to diagnose them?

A

Temporal Arteritis - ESR, biopsy

Takayasu’s Arteritis - ESR, MRI

39
Q

What are the names of the medium vessel vasculitises and Ix to diagnose them?

A

Polyarteritis Nodosa - ESR, renal angiography

Kawasaki’s Disease -

40
Q

What are the names of the small vessel vasculitises?

A

ANCA positive
Wegeners -renal biopsy, red cell clasts, c-ANCA
Microscopic Polyangitis - renal biopsy, red cell clasts, p-ANCA
Churg Strauss Syndrome
ANCA negative
Henonch-Schonlein Purpura

41
Q

Describe the salient points of Henoch-Schonlein Purpura

A

IgA mediated autoimmune hypersensitivity vasculitis if childhood (peak 4-6 years)
Often preceding URTI
Main Sx - purpura, arthritis, adbo pain and Gi bleed, orchitis and nephritis

42
Q

What are the red flag symptoms that make you think of vasculitis?

A

Rash - palpable purpura (commonest)
PUO and other constitutional Sx
Inflammatory arthritis/general arthralgia
Myositis/Myalgia
Neuropathy - mononeuritis, polyneuritis
Glomerulonephritis
End organ ischamiea - abdo pain, acute visual loss
Anaemia, raised inflammatory markers with no ?cause
Multi-system disease

43
Q

Describe the pathophysiology of vasculitis?

A

Inflammatory condition of blood vessel walls

Leads to either destruction (aneurysm or rupture) or stenosis - which leads to end organ damage in various organs.

44
Q

What are the cutaneous manifestations associated with cushings syndrome?

A

Purple Abdominal Straie
Facial Acne
Plethora

45
Q

What are the cutaneous manifestations associated with Addisons Disease?

A

Hyperpigementation - Buccal Muscoa, Palmar Creases,

46
Q

What are the cutaneous manifestations associated with Hypothryoidism?

A

Peaches and Cream Complexion
Loss of Lateral 1/3 eyebrow
Dry Skin
Periorbital oedema

47
Q

What are the cutaneous manifestations associated with Hyperthryoidism?

A

Pretibial Myxoedema (mucin deposits in skin)
Acropachy (mucin deposits in nail bed)
Eye signs - exopathlamos - Graves

48
Q

What are the cutaneous manifestations associated with Porphyria?

A

Sun exposed- Blistering, Scarring, Fragility

Facial hirsutism

49
Q

What are the cutaneous manifestations associated with Hyperlipidaemia?

A

Xanthalasma
Tendon Xanthomas (familial hypercholesterolaemia)
Eruptive Xanthomas (hypertriglyceridaemia)
Corneal Arcus

50
Q

What are the cutaneous manifestations associated with Diabetes?

A

Acanthosis nigricans - Also occurs in obesity
Necrobiosis lipoidica - thin skin, full of granulomas
Infections e.g. candida

51
Q

What are the cutaneous manifestations associated with Gout?

A

Tophi

and periarticular urate

52
Q

Describe what can cause neutrophilic dermatoses (have a bluey edge to lesion)

A

Pyoderma Gangrenosum
e.g. IBD, haematological malignancy, RA
Sweet’s syndrome = acute febrile neutrophilic dermatosis
e.g. Myeloma, Leukaemia, Other malignancies

53
Q

What are the 2 stages of cutaneous T cell Lymphoma (mycosis fungoides)

A

Patch and plaque stage - 5-30 years, PUVA phototherapy, looks like psoriatic plaques that are refractory to treatment.
Tumour stage - get abnormal T cells stuck in the skin

54
Q

Describe the cutaneous manifestations of the three stages of syphilis

A 
SPIROCHETES
Primary
Painless chancre
Secondary
rash - classically affects palms and soles, or can be systemic, can also see snail track ulcers
Tertiary
Gumma - form of granuloma, growth
55
Q

What is the name of the rash seen in Lyme Disease

A

Erythema Migrans

56
Q

Describe the triad of Reiters Syndrome and the classic cutaneous manifestations

A

Urethritis, arthritis, uveitis
Psoriasiform rashes
e.g. Circinate balanitis, Keratoderma blenorrhagica

57
Q

What are the cutaneous manifestations seen in IBD?

A

erythema nodosum

pyoderma ganrenosum

58
Q

Describe the salient points of Bechets Disease?

A

Orogenital ulcers, uveitis (triad)
Pyoderma Gangrenosum
Pathergy (skin condition in which a minor trauma such as a bump or bruise leads to the development of skin lesions or ulcers that may be resistant to healing)

59
Q

What are the cutaneous manifestations of sarcoid?

A

Erythema Nodosum
Lupus Pernio
Subcutaneous Nodules

60
Q

Describe the cutaneous manifestation associated with CTD

A

Photosensitivity Rashes
e.g. acute malar rash in lupus
Nailfold changes
Livedo

61
Q

What are the cutaneous manifestations of scurvy?

A

Corkscrew hairs
Perifollicular haemorrhages
Bleeding gums

62
Q

Describe the cutaneous manifestations of neurofibromatosis Type 1

A

Multiple café au lait spots
Axillary freckling
Lisch nodules (iris)

63
Q

What is the commonest cause of erythema nodosum?

A

Strep Throat

64
Q

What are the 5 types of acanthosis nigracans based on cause

A 
1 - Familial
2 - Endocrine 
3 - Obesity
4- Drug related
5 - Malignancy
65
Q

What malignancies are commonly implicated in dermatomyositis?

A

Breast, Lung, Ovarian, GI

66
Q

What is Lofgrens Syndrome?

A

BHL + polyarthralgia + erythema nodosum = acute presentation of sarcoid

Stage 4 CSIM (9 decks)

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