Clinical Relevance: Connective tissue disorder

Question 1

Describe the clinical features of autoimmune connective tissue

Answer 1

• Arthralgia
• Sicca symptoms
• Raynaud’s
• Respiratory
• Cardiac
• Skin / mucous membrane
• Muscle
• Haematological abnormalities
Fatigue

Question 2

Describe the clinical features of
connective tissue disorders

Answer 2

• Fatigue – all! Especially Sjogren’s
• Arthralgia – all
• Sicca symptoms – Sjogren’s but remember secondary
• Raynaud’s – lupus, Sjogren’s, SS etc and remember primary
• Respiratory – lupus, SS
• Cardiac – lupus, SS,
• Skin – lupus, SS, dermatomyositis
• Muscle – poly/dermatomyositis, lupus
• Haematological abnormalities - lupus

Question 3

Compare polymyositis and PMR

Answer 3

Polymyositis
• Any age
• proximal muscle involvement ie shoulder and hip
• CPK elevated due to muscle damage
• Spectrum includes skin involvement
• Rare and needs referral

Polymyalgia rheumatica
• Over age of 50
• Shoudler and hip pain
• NO muscle involvement • Normal CPK
• EMS and high inflammatory markers
• Spectrum includes GCA (vasculitis)
• PMR is common and often managed by GPs

Question 4

Describe how tendons insert onto bone and briefly describe the presentation of the common clinical conditions which arise.

Answer 4

• Anatomy
◦ 4 transitional tissues
• Tendon, Fibrocartilage, Mineralised fibrocartilage (Sharpey’s fibres), Bone
• Effects of age
◦ Degeneration
◦ Trauma
◦ Vascular reaction

Biomechanics of tendon

Enthesitis
Natural history
Therapy

Question 5

Supraspinatus tendon

Answer 5

• Inserts onto greater tuberosity
• Allows abduction of the shoulder
• Often occurs in aged tendons

Clinical Presentation
• Weak shoulder abduction
• Unable to keep arm in space
• Drop arm sign

Question 6

Tennis Elbow

Answer 6

• Common extensor origin
• Pain on resisted extension

Question 7

Golfers Elbow

Answer 7

• Common flexor origin
• 10 x less common than tennis elbow
• Pain on resisted flexion

Question 8

Jumpers Knee

Answer 8

• Overusesyndrome
• Pain at inferior pole of patella
• Can also occur at insertion of quadraceps

Question 9

Achilles Tendonitis

Answer 9

• Pain over insertion of tendon onto Os-Calcis
• Achillestendon transmits forces up to 10 x body weight

Question 10

Connective tissue

Answer 10

• Highly Specialised
• Mechanical support, movement
• Contains blood vessels, nerves
• Arena for fighting infection
• Skin
• Tendon
• Cartilage
• Loose connective tissue
• Regulates cell behaviour

Question 11

What goes wrong with collagen….

Answer 11

Developmental
• –‘Chondrodystrophies’
• –‘Collagenopathies’

Structural
• –Osteogenesis Imperfecta
• –Ehlers Danlos Syndrome
• –Marfan’s syndrome

Maintenance & repair
• –Osteoporosis
Osteoarthritis
Tendinopathy

Question 12

Need to know… connective tissue disorder

Answer 12

HYPERMOBILITY
EDS
TYPE V COLLAGEN DEFECT

MARFAN SYNDROME
VARIABLE SYMPTOMS
SCOLIOSIS
CHEESY DEFORMITIES
ARACHNODACTYLY (long fingers)
Tall, THIN
JOINT HYPERMOBIKITY
CRANIOFACIAL ABNORMALITIES
CARDIOVASCULAR DEFECTS
OCULAR DEFECTS
AORTIC DEFECTS

Question 13

Tissue transfer: reconstructive ladder

Answer 13

• Free flap
• Pedicled flap
• Local random pattern flap
• Full Thickness Skin Graft
• Split Thickness Skin Graft
• Suture – healing by primary intention
• Dressings - healing by secondary intention

Question 14

Vasculitis

Answer 14

• Primary ◦ GCA
◦ GPA (Wegener’s) ANCA

• Secondary ◦ Infection
◦ Malignancy ◦ Drugs etc

Question 15

Antibodies

Answer 15

ANA
dsDNA
Jo1
Ro
La
Scl-70 Centromere