Autoimmune disease

Question 1

What are the features of systemic sclerosis?

Answer 1

Scleroderma
Internal organ fibrosis
Microvascular abnormalities

Question 2

What are the two main patterns of systemic sclerosis and which autoantibodies are they associated with?

Answer 2

90% of both types are ANA positive

• Limited cutaneous systemic sclerosis - associated with anti centromere antibody - sclerodema tends to involve face, hands and feet
• Diffuse cutaneous systemic sclerosis - associated with anti-topoisomerase antibodies (SCL 70) - scleroderma affects the trunk and proximal limbs predominently, Hypertension, lung fibrosis and renal involvement

Question 3

What is the management for systemic sclerosis?

Answer 3

No cure
For organ involvement use IV cyclophosphamide
Monitor BP and renal function
Regular ACE-i or ARBs reduce the risk of renal crisis
IV iloprost or lantoprost causes dilation for raynaud’s

Question 4

What antibodies are sensitive and specific for rheumatoid arthritis?

Answer 4

Rheumatoid factor is sensitive

Anticyclic citrullinated peptide is 96% specific

Question 5

What is the autoantibody associated with SLE?

Answer 5

There is anti nuclear antibody in >95% of cases

Anti-double stranded DNA is not as senstitive but is highly specific

Question 6

What autoantibodies are present in antiphospholipid syndrome?

Answer 6

Anticardiolipin antibody

Question 7

What autoantibodies are present in Sjogren’s syndrome?

Answer 7

There is ANA in 70%

Anti Ra and Anti La are often present

Question 8

What autoantibodies are present in primary biliary cholangitis?

Answer 8

These are antimitochondrial antibodies