Fibromyalgia and Connective tissue disease Flashcards
Define Fibromyalgia
A chronic pain syndrome which commonly features widespread pain, increased pain sensitivity, and stiffness.
Disrupted and unrefreshing sleep, constant fatigue, and tender points.
Name 3 conditions commonly associated with fibromyalgia
Depression Anxiety RA, AS, and SLE (25%) Chronic fatigue syndrome Chronic headaches syndrome IBS
Outline the diagnostic criteria for fibromyalgia
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
Name 3 risk factors for fibromyalgia
Female (10:1)
Middle aged
Low socioeconomic group
Outline the management of fibromyalgia
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
Define Scleroderma
A multisystem autoimmune connective tissue disease featuring vascular and fibrotic changes.
Outline the vascular features seen in Scleroderma
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
Outline the fibrotic features seen in Scleroderma
Cytokine release activates fibroblasts
Increased collagen synthesis: fibrosis in lower dermis and internal organs
List the clinical features of Scleroderma
Raynaud’s phenomenon (100%)
Limited cutaneous scleroderma (70%)
Diffuse cutaneous scleroderma (30%)
Describe Limited cutaneous scleroderma
- 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%)
What is Raynaud’s phenomenon?
Vasospasm of digital arteries causes fingers/toes to turn white then blue, with numbness or pain.
Describe Diffuse cutaneous scleroderma
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
What antibodies are associated with Limited cutaneous scleroderma?
Anti-centromere antibodies (70-80%)
What antibodies are associated with Diffuse cutaneous scleroderma?
Anti-Scl-70 antibodies (40%) are highly specific, also known as anti-topoisomerase
Anti-RNA polymerase (20%)
Outline the management of Scleroderma
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
Define polymyositis and dermatomyositis
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
Describe the clinical features of adult polymyositis
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
Describe the clinical features of adult dermatomyositis
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%)
What is the association between polymyositis/ dermatomyositis and malignancy?
Relative risk of cancer
- 4 for males
- 4 for females
Consider in recurrent, refractory, or ANA-ve dermatomyositis
Name 3 investigations used in suspected polymyositis/ dermatomyositis
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
Outline the management of polymyositis and dermatomyositis
Prednisolone 0.5-1.0 mg/kg for at least 1 month after myositis becomes inactive
Steroid sparring agents e.g. MTX and azathioprine
