Rheumatology: Connective Tissue Disease

Question 1

What is a connective tissue disease?

Answer 1

The immune system goes into overdrive, producing antibodies. Symptoms vary and appear slowly

Question 2

How does SLE arise?

Answer 2

Increased and defective apoptosis. Formation of antibody antigen complexes and are deposited in the skin and kidneys, leading to inflammation and an immune response

Question 3

Who does SLE most commonly affect?

Answer 3

Can affect anyone but most commonly in Asian, Chinese and Afro-Carribean, Afro-Americans, unlikely in black Africans

Question 4

What are the 4 factors which contribute to SLE?

Answer 4

Genetic, Environmental, Immunological and Hormonal

Question 5

What are the constitutional features of SLE?

Answer 5

Fatigue, fever, malaise, weight loss and poor appetite

Question 6

What constitutional feature is always present in SLE?

Answer 6

Fatigue

Question 7

What are some Mucocutaneous features of SLE?

Answer 7

Malar rash (made worse by IV exposure)
Butterfly rash 
Ulcers on hard palate, lips and tongue
Alopecia
Reynaud's

Question 8

What are some Musculoskeletal features of SLE?

Answer 8

Non deforming polyarthritis/polyarthralgia (joint pain with no inflammation)
Jaccoud’s (reversible swan necking)

Question 9

What are some Pulmonary features of SLE?

Answer 9

Pleuritic pain, Pleural effusions, pulmonary infarcts and hypertension

Question 10

What are some Cardiac features of SLE?

Answer 10

Pericarditis, Libman-Sachs, pulmonary hypertension, cardiomyopathy

Question 11

What is Libman-Sachs?

Answer 11

On an echo, vegetations are seen on the heart valve but the blood cultures are negative as it is sterile

Question 12

What are some Glomerulonephritis features of SLE?

Answer 12

No symptoms until late stage renal failure

Question 13

What are some Neurological features of SLE?

Answer 13

Depression is common, headaches, stroke

Question 14

What are some Haemaotogical features of SLE?

Answer 14

Lymphadenopathy is common but variable

low lymphocyte count in majority

Question 15

What are the antibody tests in SLE?

Answer 15

Anti-ds DNA, Anti-Sm, Anti-Ro, Anti-RNP

Question 16

What is the maximum titre of ANA which is almost all patients with SLE?

Answer 16

1:160

Question 17

What is the link between anti-ENA and pregnancy?

Answer 17

If the mother is positive for this antibody then this can cause neonatal lupus (born with rash and heart block)

Question 18

What changes to antibodies are seen in flare ups of SLE?

Answer 18

Anti-ds DNA level increases in flare ups with decrease in complement C3/C4

Question 19

What other anti-ENA antibodies are there?

Answer 19

Anti-Sm, Anti-Ro and Anti-RNP

Question 20

What is double stranded DNA antibody specific for?

Answer 20

highly specific for lupus, occurs in 60% of patients and varies with disease activity

Question 21

What screening tests should be conducted in SLE?

Answer 21

ANA antibodies, FBC, urinalysis

Question 22

How should SLE be monitored?

Answer 22

need to assess whether it fluctuates or not, urine examination, blood biochemistry and FBC§

Question 23

What is the general management of patients with SLE?

Answer 23

counselling for both patients and family, regular monitoring, avoid sun exposure, pregnancy issues (SLE needs to be under control for at least a year)

Question 24

What is the drug choice in mild SLE?

Answer 24

topical steriods and NSAIDs

Question 25

What is the drug choice in moderate SLE?

Answer 25

oral steriods, aziothioprine, methotrexate

Question 26

What is the drug choice in severe SLE?

Answer 26

IV steriods, cyclophophamide, Rituximab

Question 27

What is Sjogren's syndrome?

Answer 27

Lymphocytic infiltrate in exocrine organs

Question 28

What are the typical features of Sjogren's syndrome?

Answer 28

dry eyes, mouth and joint pain

Question 29

Name some of the other manifestations of Sjogren's syndrome

Answer 29

Fatigue, arthralgia, Raynaulds

Question 30

What tests can be performed to confirm the diagnosis of Sjogren's

Answer 30

Schimer test, positive anti-ro, anti-LA

Question 31

What is more common, Sjogren's or Rheumatoid Arthritis?

Answer 31

Sjogrens but it is mostly undiagnosed

Question 32

Are serious complications common in Sjogren's?

Answer 32

No, rare

Question 33

What treatment should be given in Sjogren's?

Answer 33

Tailored to symptoms of the patient Hydrocholoquine for joint pains and fatigue Saliva replacement, eye drops, punctal plugs

Question 34

What are the main features of Systemic sclerosis?

Answer 34

Vasomotor disturbances: Raynauld's, fibrosis and subsequent atrophy of the skin and subcutaneous tissue

Question 35

How can systemic sclerosis be categorised?

Answer 35

Into localised, limited and diffuse

Question 36

What can be seen in limited Systemic sclerosis?

Answer 36

face, forearms, hands and feet affected - Pinching of the nose 'breaking' - Tightening of the skin around the mouth - Telangiectasia

Question 37

What can be seen in diffuse Systemic sclerosis?

Answer 37

Skin changes within one year of Raynaulds Truncal and aural skin involvement Early significant organ involvement Anti-Scl-70 antibodies

Question 38

What is Anti-scl-70 antibodies related to?

Answer 38

diffuse systemic sclerosis

Question 39

What are anti-centromere antibodies associated with?

Answer 39

limited systemic sclerosis

Question 40

What are the treatments for systemic sclerosis?

Answer 40

Vasodilators for Reynaulds | ACEi for renal involvement

Question 41

What happens to the other organ manifestations in systemic sclerosis?

Answer 41

sclerosis and fibrosis throughout, leading to strictures in GI tract and the fibrotic end of respiratory

Question 42

What are the main clinical manifestation of Anti-phospholipid syndrome?

Answer 42

Recurrent venous/arterial thrombosis and recurrent foetal loss

Question 43

What is Catastrophic Anti-phospholipid syndrome?

Answer 43

Rare, serious and fatal manifestation, characterised by a series of multi-organ infarctions over a period of weeks to days

Question 44

What is a common cutaneous finding in Anti-phospholipid syndrome?

Answer 44

Livedo Reticularis

Question 45

What is the management for a) Thrombosis b) Pregnancy loss

Answer 45

a) life long anticoagulation with warfarin | b) aspirin and heparin during pregnancy