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MDD > Multisystem connective tissue disease > Flashcards

Multisystem connective tissue disease Flashcards

1
Q

what is the pathology of SLE

A

Autoimmune condition, with autoantibodies directed at a range of tissues, however the aetiology is unknown

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2
Q

what is the only universal symptom of SLE

A

fatigue

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3
Q

what are some common features of SLE

A

Skin involvement - 75%
malar rash
photosensitivity
alopecia

Oral involvement - 20%
mucosal ulcers

CNS involvement - 60%
depression/anxiety
infarctions
grand-mal seizure

Renal involvement - 30%
glomerular disease
interstitial nephritis

Haematology
normocytic hypochromic anaemia
thrombocytopenia
leukopenia

MSK
arthritis (90%) - fingers wrists knees
myalgia
symmetrical polyarthritis

Lung - 50%
recurrent pleurisy /pleural effusions
pulmonary fibrosis

Heart involvement
myositis
pericarditis
cardiomyopathy

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4
Q

what investigations are done if lupus is suspected

A

FBC – normocytic hypochromic anaemia, thrombocytopenia, leukopenia

ESR/CRP - CRP normal, ESR may be raised
U+E – deranged if there is renal involvement
Serum ANA – almost always positive

Specific
Sm - indicates renal disease
Serum RF – positive in 25%
Anti-dsSNA – specific for SLE but only 50% sensitivity
RNA antibodies (anti ro, anti-la) often present
Serum complement levels – C3/C4 reduced In active disease
APL antibodies – many patients will have anti cardiolipin antibodies indicating a comorbid antiphospholipid antibodies

Urine Dip
Check for renal involvement

Histology

Characteristic histology/immunoflourescence scan in skin/kidney biopsies

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5
Q

whats the management of lupus

A

Avoid excessive sunlight and CV risk factors

Monitor for signs of infection and treat early

Mild disease
NSAIDS
Hydroxychloroquine - Monitor eyes as may cause retinopathy
In combination help skin disease, fatigue, and arthralgia

More severe disease  
Any cardiac/renal involvement  
Prednisolone  
DMARD  
e.g. azathioprine (child bearing age), methotrexate etc
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6
Q

what is antiphospholipid syndrome

A

Presence of autoantibodies with a specificity for phospholipids, predisposing to thrombosis

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7
Q

what are features of antiphospholipid syndrome

A

Venous thrombosis, most commonly in arms/legs predisposing to PE

Arterial thrombosis, much rarer than venous
20% of strokes in those <45 are due to APL syndrome

Pregnancy loss, particularly in 2nd or 3rd trimester

Livedo reticularis - ‘lace like’ rash in face, looks a bit like crazy paving

Thrombocytopenia

Migraines

Epilepsy

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8
Q

what investigations should be done if antiphospholipid syndrome considered

A

ESR – normal

ANA – negative

APTT – increased

Coombs test – positive

Anticardiolipin antibodies – diagnostic if +ve
2 high titres are required at least 12 weeks apart

Lupus anticoagulant antibodies (found in 20%)

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9
Q

how do you treat antiphospholipid syndrome in those with a history of thrombosis

A

Those with a history of severe thrombosis
Warfarin
Target INR 3-4

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10
Q

how do you treat antiphospholipid syndrome in those with no thrombotic history

A 
With no thrombotic history  
Low dose aspirin 
Lifestyle advice  
Avoid prolonged immobilisation 
Avoid oestrogen containing drugs
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11
Q

how do you treat antiphospholipid syndrome in pregnancy

A

In pregnancy

LMWH and aspirin given throughout

Early delivery with specialist care

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12
Q

what is the prognosis of antiphospholipid syndrome

A

Poor

1/3 having organ damage within 10 years of diagnosis

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13
Q

what is primary sjogrens

A

Dry eyes (keratoconjunctivitis sicca) in the absence of autoimmune disease

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14
Q

what is secondary sjogrens

A

Presence of dry eyes + autoimmune disease

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15
Q

what autoimmune diseases are most commonly associated with secondary sjogrens

A

RA

SLE

Scleroderma

Polymyositis

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16
Q

what are symptoms of sjogrens

A

Dry eyes and mouth

Salivary and parotic gland enlargement

Vaginal dryness

Associated systemic features

Arthralgia

Raynaud’s

Oesophageal motility issues

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17
Q

what investigations should be done for sjogrens

A

Schirmers smear test
Specialised filter paper placed inside lower eyelid <1cm in 5 minutes
Indicates defective tear production

Bloods  
RF – usually raised  
ANA – raised in 70% 
Anti-ro – positive in 70% 
Anti-la – positive In 30%
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18
Q

what management is there for sjogrens

A

Artificial tears + saliva replacement solutions

identify and treat underlying disorder if secondary

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19
Q

what is the pathophysiology of scleroderma/systemic sclerosis

A

Perivascular fibrosis leads to ischaemic damage in a range of tissues

the skin is most commonly affected (dermal thickening leads to hardened skins)

Major organs (kidneys/heart/oesophagus/kidneys damaged)

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20
Q

what are the 2 main subtypes of scleroderma + their % split

A

limited cutaneous scleroderma - 70%

diffuse cutaneous scleroderma - 30%

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21
Q

what are some features of limited cutaneous scleroderma including early and late symptoms

A

Long history of raynauds with skin tightening at the extremities

Face may be involved causing microstomia (smaller oral cavity due to tightening)

Early symptoms
Fatigue
GORD
Ulcers on digital tips

Later symptoms
Oesophageal strictures
Small bowel malabsorption
Pulmonary fibrosis/hypertension

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22
Q

what was scleroderma previously known as, and what does it stand for

A

CREST syndrome

C - calcinosis 
R - reynauds
E - oesophageal dysmotility 
S - sclerodactlyl
T - telangectasia
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23
Q

what is the main life threatening feature of scleroderma

A

pulmonary hypertension

24
Q

whats the management for limited cutaneous scleroderma

A

Digital sympathectomy /vasodilators for the Raynauds

Procedure where the nerves to the fingers are cut

Removal of calcinoses

Treatment of oesophageal problems

25
Q

what are features of diffuse sytemic sclerosis

A

Short history of Raynauds

Rapidly progressive skin sclerosis – peaking at about 2 years

Systemic symptoms greater  
Lethargy  
Weight loss 
Anorexia 
Complications will occur within the first 3 years  
Myocardiac fibrosis 
Pulmonary fibrosis  
Renal fibrosis
26
Q

whats the management for diffuse systemic sclerosis

A

Immunosuppression

Aiming to prevent complications so can be gradually withdrawn after a year if the disease subsides

Sympathectomy/vasodilators helpful for symptoms

27
Q

what investigations tend to be diagnostic in scleroderma

A

Limited cutaneous scleroderma
Anti-centromere antibodiy positive in 60%

Diffuse cutaneous scleroderma
Anti-SCL-70 antibodies In 40%
Anti-RNA polymerase antibodies
These can be present in limited cutaneous scleroderma however less commonly

28
Q

whats the prognosis of scleroderma

A

Highest mortality of any of the autoimmune rheumatic diseases

Limited cutaneous scleroderma has the better survival rate (70% 10 year) due to its limited organ involvement compared to diffuse scleroderma

29
Q

what are examples of inflammatory myopathies

A

polymyositis

dermatomyositis

30
Q

what gene phenotype is associated with inflammatory myopathies

A

HLA-B8/DR3

31
Q

what type of muscle is affected in polymyositis

A

striated muscle

32
Q

what are features of polymyositis

A

Inflammation of striated muscle, causing proximal muscle weakness

Generally causes weakness in absence of any pain

There may be associated muscle wasting

Onset can be insideous or acute

Associated with malaise, fever and weight loss

Patient will have difficulty
Squatting
Climbing stairs

if left untreated respiratory muscle involvement will lead to respiratory failure

33
Q

what is the presentation of dermatomyositis

A

polymyositis + associated skin involvement

Classic heliotropic rash (purple colouration of eyelids)

Associated periorbital oedema

Vasculitis patches over the knuckles (Gottron’s papules)

34
Q

what conditions are inflammatory myopathies associated with

A

RA

SLE

systemic sclerosis

various malignancies (most commonly breast, lung and colorectal)

35
Q

what investigations should be done in inflammatory myopathies

A 
Bloods 
Serum CK – raised  
ESR – rarely raised (5%) 
ANA – most are positive 
RF – positive in 50%  
Myositis specific antibodies  
Electromyography  
EMG shows characteristic pattern  
Fibrillations 
Myotonic discharges 
Positive sharp waves

MRI
Detects area of abnormal muscle
Needle muscle biopsy
Fibre necrosis with inflammatory infiltrate

Further investigations for malignancy screening
Full body CT

36
Q

how do you manage inflammatory myopathies

A

Prednisolone/DMARDS until myositis is clinically inactive

If not working IVIG is sometimes used

37
Q

what is inclusion body myositis

A

Affects white males over 50 , with the insideous onset of proximal and distal muscle wasting, which may be asymmetrical

ANA frequently less positive

Myositis specific antigens will not be positive

Muscle biopsy shows inflammatory infiltrate and vacuoles containing beta-amyloid (inclusion bodies)

Progressive and rarely responds to prednisolone/DMARD combos

38
Q

what are features of systemic vasculitis

A 
General 
malaise  
Fever 
Weight loss 
Myalgia 
Arthralgia

Skin
Palpable purpura
Ulceration

GI
Ulcers
Abdominal pain
Diarrhoea

Respiratory
Haemoptysis
Dyspnoea

ENT
Epistaxis
Crusting

Cardiac
Chest pain

Neuropathies

39
Q

what are some examples of large vessel vasculitides

A

Giant cell arteritis/temporal arteritis

Takayasu’s arteritis

40
Q

what are features of takayasu’s arteritis

A

Young adults

Presents with upper limb claudication and stroke

41
Q

what are some examples of medium vessel vasculidities

A

classic polyarteritis nodosa

kawasakis disease

42
Q

what are some features of polyarteritis nodosa

A

Multiple systemic symptoms

Multiple microaneurysms on angiography

Systemic vasculitic symptoms in the presence of hepatitis B signs and in the absence of pulmonary symptoms/signs suggests a diagnosis polyarteritis nodosa

43
Q

what are some features of kawasakis disease

A

Fever

Rash

Lymphadenopathy

Palmar erythema

strawberry tongue

May cause coronary artery aneurysm

44
Q

what are examples of medium/small vessel vasculidities

A

wegeners vasculitis/Granulomatosis with polyangiitis

Churg-struass syndrome

microscopic polyangitis

henoch-schlonein purpura

cryoglobinaemia

45
Q

what are some featues of wegeners vasculitis

A

Lung, kidney, ENT involvement with granuloma formation seen on biopsies

46
Q

what are some features of churg-strauss syndrome

A

Late-onset asthma

Atopy

Cardio-pulmonary involvement

47
Q

what are some features of microscopic polyangitis

A

Commonly causes pulmonary renal syndrome

Haemoptysis and haematuria

48
Q

what are some features of cryoglobinaemia

A

Associated with hep C

Rash

Arthralgia

Neuropathy

49
Q

what are some features of henoch-schlonein purpura

A

purpuric rash on back/buttocks/back legs

commonly in kids

some associated kidney/joint/bowel disease

50
Q

what are some investigations done for a suspected vasculitis

A

BP + urine dip - for prognosis

bloods
FBC: leukocytosis in primary disease/infection, leukopenia in CTDs
LFTs: hepatitis is often a secondary marker of vasculitis
Inflammatory markers
Immunology
C-ANCA associated with wegeners
P-ANCA associated with Churg-Strauss syndrome

51
Q

what specific test is associated with churg-strauss syndrome

A

P-ANCA

52
Q

what specific test is associated with wegeners syndrome

A

C-ANCA

53
Q

how is vasculitis typically treated

A

Depends on the size of the vessel involved

Corticosteroids are mainstay of treatment – prolonged dose reduction over 12 hours

Cyclophosphamide (low dose or pulse) is often used in ANCA positive disease to induce remission

IVIG is used in kawasakis

Plasma exchange may be done In life threatening conditions

54
Q

what is done for life threatening vasculitis episodes

A

plasma exchange

55
Q

whats an important side effect of cyclophosphamide

A

infertility

56
Q

what treatment is done in kawasakis disease

A

IVIG

MDD (22 decks)

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