SLE

Question 1

what happens to cause lupus?

Answer 1

Lupus is an auto-immune condition where the body creates auto-antibodies against a variety of auto-antigens which results in immune complexes. Inadequate clearance of these immune complexes results in immune response causing tissue inflammation and damage

Question 2

who does SLE more commonly occur in

Answer 2

Women

Those of Afro-caribbean, south asian and mexican descent

Question 3

when is SLE commonly diagnosed

Answer 3

during women’s reproductive years. between 40-50, often with a delay to diagnosis.

Question 4

what is the diagnostic criteria used for diagnosing SLE?

Which criteria must you have?

Answer 4

SLICC.
Need to have at least 1 clinical and 1 serological criteria and a total of 4, to get a diagnosis but if you have biopsy proven lupus nephritis and positive immunology this is also diagnostic.

Question 5

what are the common features of SLE?

Answer 5

constitutional symptoms (fatigue being a major complaint), cutaneous manifestations, arthralgia and arthritis

Question 6

what type of arthritis is seen in SLE

Answer 6

non-erosive.
May see Jaccoud’s artropathy sometimes: chronic non-erosive reversible joint disorder that may occur after repeated bouts of arthritis. inflammation of the joint capsule and subsequent fibrotic retraction, causing ulnar deviation of the fingers

Question 7

name some respiratory features of SLE

Answer 7

o	Pleurisy
o	Pleural effusions
o	Acute pneumonitis
o	Diffuse alveolar haemorrhage
o	Pulmonary hypertension
o	Shrinking lung syndrome

Question 8

name some CVS features of SLE

Answer 8

o	Pericarditis +/- effusion
o	Myocarditis
o	Valvular abnormalities 
o	Coronary heart disease – high risk of morbidity and mortality long-term
o	Risk of MI 50x

Question 9

name some neuropsychiatric manifestations of SLE

Answer 9

headache (unremitting), anxiety and mood disorder, seizure, demyelination, GBS, mononeuritis

Question 10

name some haematological consequences of SLE

Answer 10

o Anaemia of chronic disease
o Autoimmune haemolytic anaemia
o Thrombotic thrombocytopenic purpura (TTP) – MAHA, low platelets, fever, neuro/renal involvement - check aPLS antibodies
o Leukopenia
o Can have associated lymphadenopathy and splenomegaly
o Thrombocytopenia
o Mild or ITP

Question 11

name some ENA you might find in SLE

Answer 11

Ro/La, Ds-DNA, Dm

Question 12

what will you find with regard to complement in a patient with active SLE?

Answer 12

complement consumption (will have low levels). C3 is more specific than C4.

Question 13

what would be standard care for non-acute SLE

Answer 13

high-factor sunblock and hydroxychloroquine. If they had a skin flare you would try topical steroid first
NSAIDs (unless they have renal disease)

Question 14

what drugs would you use in moderate SLE

Answer 14

mild disease drugs - sunblock, hydroxychloroquine, NSAIDs.

Oral prednisolone or steroid-sparing agents such as methotrexate, azathioprine etc.

Question 15

If someone had severe SLE how would you treat them

Answer 15

mild disease drugs - sunblock, hydroxychloroquine, NSAIDs.
Oral prednisolone or steroid-sparing agents such as methotrexate, azathioprine etc
Add in high dose steroid, biologics, DMARDS, B cell therapy, cyclophosphamide etc.

Question 16

what would a bone marrow aspirate in someone with SLE show?

Answer 16

reactive bone marrow

Question 17

what pattern of ESR and CRP is commonly seen in SLE

Answer 17

ESR disproportionately elevated compared to CRP

Question 18

which investigations would you order 1st line in a suspected case of SLE

Answer 18

• FBC and differential
• Activated PTT
• Urea and electrolytes
• Erythrocyte sedimentation rate (ESR) and CRP
• Antinuclear antibodies, double-stranded (ds) DNA, Smith antigen (anti-Sm)
• Urinalysis
• Chest x-ray
• ECG
• Would consider complement levels and anti-phospholipid antibodies but not essential as 1st line

Question 19

what is Sjogren’s syndrome

Answer 19

Chronic auto-immune disorder where there is lymphocytic infiltration and fibrosis of exocrine glands, especially lacrimal and salivary glands.
Can be primary (females, 40-50) or secondary to connective tissue disease e.g. SLE

Question 20

list some features of Sjogren’s syndrome

Answer 20

decreased tear and saliva production. Parotid swelling, vagianl dryness leading to dyspareunia, dry cough

Question 21

list some systemic signs of Sjogren’s syndrome

Answer 21

polyarthritis/arthralgia, Raynaud’s syndrome, lyphadenopathy, vasculitis, peripheral neuropathy, fatigue.

Question 22

how do you test for Sjogren’s syndrome

Answer 22

Schirmer’s test - measures conjunctival dryness.
ANA usually +.
Anti-RO and Anti-LA antibodies may be present.

Question 23

how do you treat Sjogren’s syndrome

Answer 23

artificial tears, NSAIDs and hydroxychloroquine for arthralgia

Question 24

what is systemic sclerosis

what are the two types

Answer 24

auto-immune disease of the connective tissue characterised by scleroderma due to accumulation of collagen and by injuries to small arteries.
Localised and diffuse.
Localised - face, hands and feet

Question 25

how do you manage systemic sclerosis

Answer 25

no cure. Immunosuppression is used for organ involvement and any progressive skin disease.
Need to monitor BP due to risk of pulmonary hypertension as well as renal function.