Connective tissue diseases

Question 1

SLE - features (8)

Answer 1

4/11 ACR criteria - SOAP BRAIN MD

• Serositis - pleruritic chest pain (plerual inflammation)
• oral ulcers
• arthritis
• photosensitivity
• Blood disorders - pancytopenia
• renal involvement
• ANA +ve
• Immune path - anti DsDNA, anti-smith, anti-histones
• Neuro symptoms
• Malar - butterfly rash
• Discoid rash

Question 2

ANA ab in SLE - specificly what can the abs be? (3)

Answer 2

1. Anti Ds DNA
2. Anti-smith - most specific but only 30% pts have
3. Anti-histone - drug related e.g. hydralazine

Question 3

What happens to glomerulus in SLE?

Answer 3

Immune complex deposition results in thickened GBM - membranous GNpathy

Question 4

What is this?

Answer 4

Libman Sacks endocarditis - strands of fibrin, neutrophils,lymphocytes (but no bacteria)

Question 5

SLE: gender, ethnicity, HLA assc.

Answer 5

Autoimmune multisystem disorder, high incidence in those with complement def, can be drug induced. F> M, afroC.

HLA DR2/3 assc.

Question 6

Scleroderma ‘hard skin’.

Features (5)

Answer 6

Scleroderma also autoimmune disorder, get tight skin due to excess collagen deposition & fibrosis

F -

• Calcinosis - painful lesions on tips of fingers
• Raynauds - triphasic colour change
• Esophageal dysmotility - also due to collagen deposition
• Sclerodactyl - tightening of skin of fingers (cant pull it)
• Telangectasia

Question 7

Scleroderma: can be diffuse or limited - what are the differences (abs & distribution)

What is the pattern of immunofluorescence

Answer 7

• Diffuse - involves trunk, anti DNA topoisomerase (Scl70)
• limited form - anticentromere ab

nucleolar pattern of IF

Question 8

What will histology of Scleroderma show? (2)

Answer 8

• Collagen excess deposition everywhere
• onion skin thickening of arterioles - intimal thickening, present with renal crises - BP>200

Question 9

Mixed connective tissue disorder has features of 4 disorders, what are they?

Answer 9

• SLE
• Scleroderma
• Dermatomyositis
• Polymyositis

(myositis presents with prox muscle weakness & tenderness > raised CK)

Question 10

Features of dermatomyositis (2)

Answer 10

• Heliotrope rash
• Gottron papules - erythematous rash over knuckles

Mixed connective tissue disorders have speckled ANA pattern

AntiJo-1 ab