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Musculoskeletal > Rheumatology: Connective Tissue Disease > Flashcards

Rheumatology: Connective Tissue Disease Flashcards

1
Q

What is a connective tissue disease?

A

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

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2
Q

How does SLE arise?

A

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

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3
Q

Who does SLE most commonly affect?

A

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

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4
Q

What are the 4 factors which contribute to SLE?

A

Genetic, Environmental, Immunological and Hormonal

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5
Q

What are the constitutional features of SLE?

A

Fatigue, fever, malaise, weight loss and poor appetite

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6
Q

What constitutional feature is always present in SLE?

A

Fatigue

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7
Q

What are some Mucocutaneous features of SLE?

A 
Malar rash (made worse by IV exposure)
Butterfly rash 
Ulcers on hard palate, lips and tongue
Alopecia
Reynaud's
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8
Q

What are some Musculoskeletal features of SLE?

A

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

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9
Q

What are some Pulmonary features of SLE?

A

Pleuritic pain, Pleural effusions, pulmonary infarcts and hypertension

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10
Q

What are some Cardiac features of SLE?

A

Pericarditis, Libman-Sachs, pulmonary hypertension, cardiomyopathy

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11
Q

What is Libman-Sachs?

A

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

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12
Q

What are some Glomerulonephritis features of SLE?

A

No symptoms until late stage renal failure

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13
Q

What are some Neurological features of SLE?

A

Depression is common, headaches, stroke

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14
Q

What are some Haemaotogical features of SLE?

A

Lymphadenopathy is common but variable

low lymphocyte count in majority

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15
Q

What are the antibody tests in SLE?

A

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

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16
Q

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

A

1:160

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17
Q

What is the link between anti-ENA and pregnancy?

A

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

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18
Q

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

A

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

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19
Q

What other anti-ENA antibodies are there?

A

Anti-Sm, Anti-Ro and Anti-RNP

20
Q

What is double stranded DNA antibody specific for?

A

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

21
Q

What screening tests should be conducted in SLE?

A

ANA antibodies, FBC, urinalysis

22
Q

How should SLE be monitored?

A

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

23
Q

What is the general management of patients with SLE?

A

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

24
Q

What is the drug choice in mild SLE?

A

topical steriods and NSAIDs

25
Q

What is the drug choice in moderate SLE?

A

oral steriods, aziothioprine, methotrexate

26
Q

What is the drug choice in severe SLE?

A

IV steriods, cyclophophamide, Rituximab

27
Q

What is Sjogren’s syndrome?

A

Lymphocytic infiltrate in exocrine organs

28
Q

What are the typical features of Sjogren’s syndrome?

A

dry eyes, mouth and joint pain

29
Q

Name some of the other manifestations of Sjogren’s syndrome

A

Fatigue, arthralgia, Raynaulds

30
Q

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

A

Schimer test, positive anti-ro, anti-LA

31
Q

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

A

Sjogrens but it is mostly undiagnosed

32
Q

Are serious complications common in Sjogren’s?

A

No, rare

33
Q

What treatment should be given in Sjogren’s?

A

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

34
Q

What are the main features of Systemic sclerosis?

A

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

35
Q

How can systemic sclerosis be categorised?

A

Into localised, limited and diffuse

36
Q

What can be seen in limited Systemic sclerosis?

A

face, forearms, hands and feet affected

  • Pinching of the nose ‘breaking’
  • Tightening of the skin around the mouth
  • Telangiectasia
37
Q

What can be seen in diffuse Systemic sclerosis?

A

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

38
Q

What is Anti-scl-70 antibodies related to?

A

diffuse systemic sclerosis

39
Q

What are anti-centromere antibodies associated with?

A

limited systemic sclerosis

40
Q

What are the treatments for systemic sclerosis?

A

Vasodilators for Reynaulds

ACEi for renal involvement

41
Q

What happens to the other organ manifestations in systemic sclerosis?

A

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

42
Q

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

A

Recurrent venous/arterial thrombosis and recurrent foetal loss

43
Q

What is Catastrophic Anti-phospholipid syndrome?

A

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

44
Q

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

A

Livedo Reticularis

45
Q

What is the management for

a) Thrombosis
b) Pregnancy loss

A

a) life long anticoagulation with warfarin

b) aspirin and heparin during pregnancy

Musculoskeletal (13 decks)

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