ankylosing spondylitis and autoimmune CT diseases Flashcards
what is ankylosing spondylitis?
a chronic, progressive, inflammatory athropathy where patients present with severe pain and stiffness which may ultimately lead to spinal fusion
what is the aetiology of ankylosing spondylitis?
HLA B27
what are the S&S of AS?
typical patient = <40 year old man with gradual onset low back pain which is worse at night, with spinal morning stiffness that is relieved by exercise
pain radiates from sacro iliac joint to the hip/buttocks
progressive loss of spinal movement
some progress to kyphosis and neck hyperextension (question mark posture)
Enthesis
- inflammation at points where ligaments/tendons insert to bone
iritis/uveitis in 40-50%
tenderness at sacro-iliac joint
what InV are done in AS?
Pelvic and spine X-ray
- sacroilitis in early disease
- erosions
- scarring
- sclerosis
- bamboo spine
HLAB27 positive
FBC, ESR and CRP
- normocytic anaemia
- Increased ESR and CRP
MRI
what is the treatment of AS?
education, exercise, PT
NSAIDs
Analgesia (paracetomol and codiene)
Intraarticular corticosteroid injections
MTX or sulfasalazine
Biologics - rituximab or etanercept (anti-TNF)
what are the 11 diagnostic signs and symptoms of SLE?
you need 4 out of;
1 - malar rash
2 - discoid rash
3 - photosensitivity
4 - oral ulcers
5 - non-erosive arthritis
6 - serositis e.g pleuritis, pericarditis
7 - renal disorder - proteinuria or cellular casts
8 - CNS disorder - seizures, psychosis
9 - haematological disorder - haemolytic anaemia, leukopenia, lymphopenia, thrombocytopenia
10 - immunological disorder - Anti dsDNA, Anti-SM, antiphospholipid
11 - antinuclear antibody
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other features include
- lymphadenopathy
- weight loss, fatigue, malaise
- alopecia
what drugs can cause SLE?
isonazid hydralazine procainamide quinidine phenytoin
skin and lung signs normally
what InV are done in SLE?
FBC
- anaemia, leukopenia, thrombocytopenia
Antinuclear antibodies, Anti dsDNA, Anti-SM,
decreased C3 and C4
U&E’s and creatinine
- increased in renal impairment
Chest CT
- lung fibrosis or effusions
Urinalysis
- haematuria, casts or proteinuria
what are the treatments for SLE?
NSAIDs corticosteroids hydroxychloroquine MTX IVIG plasmapharesis
what is Sjogren’s syndrome and its S&S?
a systemic autoimmune disorder characterised by the presence of dry eyes and mouth as a consequence of lymphocyte infiltration into the lacriminal and salivary glands
additional S&S inc
- dry skin, nose, throat and vagina
- arthralgias and myalgias
- peripheral neuropathies
- pulmonary, thyroid and renal disorders
what InV are done in sjogren’s syndrome?
Schirmer’s test
- quantatively measures tears
- +ve if <5mm of paper wetted after 5 mins
Anti-60 KD (SS-A), Anti-RO, Anti-La (SS-B)
sialometry
- decreased salivation
what is polymyositis and dermatomyositis ?
idiopathic inflammatory myopathy characterised by insidious onset of progressive symmetrical proximal muscle weakness and autoimmune mediated striated muscle inflammation associated with myalgia and arthralgia
polymyositis = muscle weakness dermatomyositis = muscle weakness and skin rash
what are the S&S of polymyositis and dermatomyositis?
proximal and symmetrical muscle weakness
Gottron’s papules
- Violet/dusty red flat topped papules and plaques over the dorsal surfaces of knuckles, wrist, elbow, knees and malleoli. may be slightly scaling
Heliotrope rash with/without peri-orbital oedema
shawl sign - macular erythema in neck, posterior shoulders and upper back
Holster’s sign - lateral hip or thigh
Nailfold erythema
mechanics hands - hyperkeratosis, scaling, fissuring of hands and palmar aspect of fingers
photosensitivity
Pruritis
cutaneous calcinosis
what is a sign that distinguishes dermatomyositis and SLE?
pruritis
how is polymyositis and dermatomyositis InV?
muscle enzymes in plasma
- CK, aldolase, AST, ALT elevated
EMG
- characteristic fibrillation potentials
Muscle biopsy
- perivascular or interfasicular inflammation
- DIAGNOSTIC
myositis specific antibodies
- Anti-MI2
- Anti-JO1
