Sjorgen's & Scleroderma

Question 1

List the autoimmune connective tissue diseases?

Answer 1

SLE
Sjorgen's
Systemic sclerosis
Myositis
Bechet's disease

Question 2

What is systemic sclerosis?

Pathophysiology?

Answer 2

An autoimmune multisystem disease

Cell-cell matrix reactions stimulate production of cytokines and growth factors
Which mediate proliferation and activation of vascular and connective tissue cells (fibroblasts)

Uncontrolled and irreversible proliferation of connective tissue

Resulting in thickening of vascular walls and narrowing of lumen

Question 3

What are the two types of systemic sclerosis?

Answer 3

Limited cutaneous SS (CREST syndrome)

Diffuse cutaneous SS

Question 4

Describe Limited cutaneous SS?

Which antibodies?

What condition to look out for?

Answer 4

Formerly known as CREST syndrome

Calcinosis
Raynaud's
Esophageal and gut dysmotility
Sclerodactyly
Telangiectasia

Skin involvement limited to hands, feet and face

Anti-centromere antibodies

Pulmonary hypertension

Question 5

Describe Diffuse cutaneous SS?

Which antibodies?

Answer 5

Diffuse skin involvement (can be whole body)
Raynaud’s

Organ fibrosis: lung, cardiac, GI, renal

Anti-topoisomerase-1
Anti-RNA

Poor prognosis

Question 6

Management of systemic sclerosis?

Answer 6

No cure

IV cyclophosphamide for organ involvement or progressive skin disease

Monitor BP and renal function

Question 7

What is Sjorgen’s syndrome?

Answer 7

Autoimmune condition in which there is lymphocytic infiltration of exocrine glands

Resulting in

• xeropthalmia
• xerostomia
• enlarged parotid glands

Question 8

Causes of Sjorgen’s syndrome?

Answer 8

Primary: develops in isolation

Secondary: to RA, SLE, scleroderma

Its believed to be a combo of immunological genetic, hormonal, inflammatory conditions

Question 9

Presentation of Sjorgen’s?

Answer 9

Dry eyes: gritty, sticky in morning

Dry mouth: difficulty eating dry food, altered taste, oral candidiasis

Parotitis and enlarged glands

Dry cough

Vaginal dryness

Systemic involvement:

• polyarthritis
• Raynaud’s
• lymphadenopathy
• vasculitis
• pancreatitis

Question 10

Investigations of Sjorgen’s?

Answer 10

FBC: anaemia of chronic disease

ANA +ve
Anti-phospholipid

Anti-Ro
Anti-La

Schirmer’s test: filter paper in lower conjunctiva, should be wet to 15mm after 5 mins in normal pt

Biopsy of salivary gland

Question 11

Management of Sjorgen’s?

Answer 11

Treat sicca symptoms

• artifical tears (hypromellose)
• drink lots
• artifical saliva
• good dental hygiene

NSAIDs
Hydroxychloroquine
For arthralgia

Anti-TNF

Question 12

What does sicca mean?

Answer 12

Dryness

Question 13

What is Raynaud’s phenomenon?

Answer 13

Peripheral digital ischaemia

Due to paroxsymal vasospasm

Precipitated by cold or emotion

Question 14

Causes of Raynaud’s?

Answer 14

Connective tissue disorders

• Systemic sclerosis
• SLE
• RA
• Dermatomyositis, polymyositis

Vibrating tools

Atheroma

Blood: thrombocytosis, polycythaemia vera

B blockers

Hypothyroid

Question 15

Presentation of Raynaud’s?

Answer 15

Fingers or toes

Aching
Turn pale, blue, then red

Question 16

Management of Raynaud’s?

Answer 16

Keep hands warm
Stop smoking

Nifedipine
Sildenafil
Prostacyclin

Chemical or surgical sympathectomy

Treat the cause