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Multisystem connective tissue disease Flashcards

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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

Study These Flashcards
A

pulmonary hypertension

24
Q

whats the management for limited cutaneous scleroderma

Study These Flashcards
A

Digital sympathectomy /vasodilators for the Raynauds

Procedure where the nerves to the fingers are cut

Removal of calcinoses

Treatment of oesophageal problems

25
what are features of diffuse sytemic sclerosis
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
whats the management for diffuse systemic sclerosis
Immunosuppression Aiming to prevent complications so can be gradually withdrawn after a year if the disease subsides Sympathectomy/vasodilators helpful for symptoms
27
what investigations tend to be diagnostic in scleroderma
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
whats the prognosis of scleroderma
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
what are examples of inflammatory myopathies
polymyositis dermatomyositis
30
what gene phenotype is associated with inflammatory myopathies
HLA-B8/DR3
31
what type of muscle is affected in polymyositis
striated muscle
32
what are features of polymyositis
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
what is the presentation of dermatomyositis
polymyositis + associated skin involvement Classic heliotropic rash (purple colouration of eyelids) Associated periorbital oedema Vasculitis patches over the knuckles (Gottron's papules)
34
what conditions are inflammatory myopathies associated with
RA SLE systemic sclerosis various malignancies (most commonly breast, lung and colorectal)
35
what investigations should be done in inflammatory myopathies
``` 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
how do you manage inflammatory myopathies
Prednisolone/DMARDS until myositis is clinically inactive If not working IVIG is sometimes used
37
what is inclusion body myositis
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
what are features of systemic vasculitis
``` 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
what are some examples of large vessel vasculitides
Giant cell arteritis/temporal arteritis Takayasu's arteritis
40
what are features of takayasu's arteritis
Young adults Presents with upper limb claudication and stroke
41
what are some examples of medium vessel vasculidities
classic polyarteritis nodosa kawasakis disease
42
what are some features of polyarteritis nodosa
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
what are some features of kawasakis disease
Fever Rash Lymphadenopathy Palmar erythema strawberry tongue May cause coronary artery aneurysm
44
what are examples of medium/small vessel vasculidities
wegeners vasculitis/Granulomatosis with polyangiitis Churg-struass syndrome microscopic polyangitis henoch-schlonein purpura cryoglobinaemia
45
what are some featues of wegeners vasculitis
Lung, kidney, ENT involvement with granuloma formation seen on biopsies
46
what are some features of churg-strauss syndrome
Late-onset asthma Atopy Cardio-pulmonary involvement
47
what are some features of microscopic polyangitis
Commonly causes pulmonary renal syndrome Haemoptysis and haematuria
48
what are some features of cryoglobinaemia
Associated with hep C Rash Arthralgia Neuropathy
49
what are some features of henoch-schlonein purpura
purpuric rash on back/buttocks/back legs commonly in kids some associated kidney/joint/bowel disease
50
what are some investigations done for a suspected vasculitis
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
what specific test is associated with churg-strauss syndrome
P-ANCA
52
what specific test is associated with wegeners syndrome
C-ANCA
53
how is vasculitis typically treated
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
what is done for life threatening vasculitis episodes
plasma exchange
55
whats an important side effect of cyclophosphamide
infertility
56
what treatment is done in kawasakis disease
IVIG

MDD (22 decks)

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