Fibromyalgia and Connective tissue disease

Question 1

Define Fibromyalgia

Answer 1

A chronic pain syndrome which commonly features widespread pain, increased pain sensitivity, and stiffness.

Disrupted and unrefreshing sleep, constant fatigue, and tender points.

Question 2

Name 3 conditions commonly associated with fibromyalgia

Answer 2

Depression
Anxiety
RA, AS, and SLE (25%)
Chronic fatigue syndrome
Chronic headaches syndrome
IBS

Question 3

Outline the diagnostic criteria for fibromyalgia

Answer 3

Severe pain in 3-6 different areas, or milder pain in 7+ areas
Constant symptoms for at least 3 months
No other causes for symptoms

Question 4

Name 3 risk factors for fibromyalgia

Answer 4

Female (10:1)
Middle aged
Low socioeconomic group

Question 5

Outline the management of fibromyalgia

Answer 5

Clear explanation of diagnosis
Reassurance it is not arthritis

Centrally acting analgesia: Amitriptyline, pregabalin, gabapentin, paracetamol, tramadol

Reversal of sleep disturbance
Physically orientated rehabilitation programme
Pain management programmes
Psycho-physiotherapy: improve QoL and function

Question 6

Define Scleroderma

Answer 6

A multisystem autoimmune connective tissue disease featuring vascular and fibrotic changes.

Question 7

Outline the vascular features seen in Scleroderma

Answer 7

Early widespread vascular damage
Cytokine release causes vasoconstriction, increased vascular permeability, and migration of cells to extracellular matrix.

Small blood vessel damage:
Widespread obliterative arterial lesions
Chronic ischaemia

Question 8

Outline the fibrotic features seen in Scleroderma

Answer 8

Cytokine release activates fibroblasts

Increased collagen synthesis: fibrosis in lower dermis and internal organs

Question 9

List the clinical features of Scleroderma

Answer 9

Raynaud’s phenomenon (100%)
Limited cutaneous scleroderma (70%)
Diffuse cutaneous scleroderma (30%)

Question 10

Describe Limited cutaneous scleroderma

Answer 10

• Calcinosis: calcium deposits in skin
• Raynaud’s
• Esophageal and gut dysmotility
• Sclerodactyly: thickened tight skin of fingers/toes
• Telangiectasia

Skin involvement limited to face (beak-like nose and small mouth), forearms, hands, and feet.
Pulmonary HTN (21%)

Question 11

What is Raynaud’s phenomenon?

Answer 11

Vasospasm of digital arteries causes fingers/toes to turn white then blue, with numbness or pain.

Question 12

Describe Diffuse cutaneous scleroderma

Answer 12

Diffuse skin involvement
Raynaud's -> Skin sclerosis -> Atrophy
Early organ fibrosis:
-Lung fibrosis (41%) and pulmonary HTN (17%)
-Myocardial fibrosis
-Oesophageal
-Renal crisis: AKI and malignant HTN

Malabsorption, anal incontinence, and pseudo-obstruction can occur

Question 13

What antibodies are associated with Limited cutaneous scleroderma?

Answer 13

Anti-centromere antibodies (70-80%)

Question 14

What antibodies are associated with Diffuse cutaneous scleroderma?

Answer 14

Anti-Scl-70 antibodies (40%) are highly specific, also known as anti-topoisomerase
Anti-RNA polymerase (20%)

Question 15

Outline the management of Scleroderma

Answer 15

Organ-based:
Raynaud’s: hand warms and nifedipine
Oesophagus: PPIs
Symptomatic malabsorption: Nutritional supplements
Renal: ACEi
Pulmonary HTN: Sildenafil + oxygen and warfarin
Pulmonary fibrosis: Azathioprine + prednisolone

Question 16

Define polymyositis and dermatomyositis

Answer 16

Polymyositis is a rare disorder of unknown cause, featuring inflammation of striated muscle -> proximal myopathy.

Dermatomyositis is polymyositis with additional skin involvement.

Both associated with HLA-B8/DR3

Question 17

Describe the clinical features of adult polymyositis

Answer 17

Commoner in women (3:1)

Acute phase: Proximal muscle weakness*, malaise, weight loss, and fever
Late phase: Dysphonia, dysphagia, respiratory failure
-pharyngeal, laryngeal, and respiratory muscles

Wasting of shoulder and pelvic girdle muscles
Often sparring of face and distal limb muscles

Question 18

Describe the clinical features of adult dermatomyositis

Answer 18

Commoner in women

Characteristic rashes
Eyelids: Heliotrope (purple) discoloration with periorbital oedema
Fingers: Purple-red raised vasculitic patches (Gottron’s papules)
Nail fold erythema
Macular rash/Shawl sign

Common: Myositis, myalgia, polyarthritis, Raynaud’s
Ulcerative vasculitis and calcinosis of subcutaenous tissues (25%)

Question 19

What is the association between polymyositis/ dermatomyositis and malignancy?

Answer 19

Relative risk of cancer

2. 4 for males
3. 4 for females

Consider in recurrent, refractory, or ANA-ve dermatomyositis

Question 20

Name 3 investigations used in suspected polymyositis/ dermatomyositis

Answer 20

Needle muscle biopsy: Fibre necrosis and regeneration

Serum creatine kinase: usually raised
ESR and CRP may be raised
ANA usually positive in dermatomyositis
Myositis-specific antibodies
Anti-synthetase antibodies

EMG: spontaneous fibrillation at rest, polyphasic potentials on voluntary contraction, salvos of repetitive potentials on mechanical stimulation of nerve

PET scan for malignancy

Question 21

Outline the management of polymyositis and dermatomyositis

Answer 21

Prednisolone 0.5-1.0 mg/kg for at least 1 month after myositis becomes inactive
Steroid sparring agents e.g. MTX and azathioprine