Connective Tissue Diseases Flashcards
what are autoantibodies
antibodies generated by the immune system against the bodys own proteins
what is another name for conditions with auto-antibody production
seropositive conditions
condition associated with anti-CCP antibody
rheumatoid arthritis
conditions associated with anti-nuclear antibody (5)
SLE
sjogrens
systemic sclerosis
MCTD
autoimmune liver disease
condition associated with anti-double stranded DNA antibody
SLE
condition associated with anti-Sm antibody
SLE
conditions associated with anti-Ro antibody
SLE, sjogrens
condition associated with anti-La antibody
sjogrens
condition associated with anti-centromere antibody
systemic sclerosis (limited)
condition associated with anti-Scl-70 antibody
systemic sclerosis (diffuse)
condition associated with anti-neutrophil cytoplasmic antibody
small vessel vasculitis
who is SLE more common in
Women
Asian, African, Caribbean and Hispanic ethnicity
Young to middle-aged adults
pathophysiology of SLE
anti-nuclear antibodies generate a chronic inflammatory response
name some non-specific symptoms of SLE
malar rash - worsened by sunlight
joint pain
muscle pain
non-scarring alopecia
raynauds
jaccouds arthropathy
describe the arthritis seen in SLE
synovitis or tenderness of at least 2 joints with >30 mins of early morning stiffness
what is jaccouds arthropathy
non-erosive reversible joint disoder
name a renal complication of SLE
nephritis
what is the first line inevstigation for SLE
antinuclear antibody titre
name some antibodies that may be tested for if ANA positive in suspected SLE
anti-dsDNA
anti-Ro
anti-smith
blood results in a patient with SLE
leukopenia, thrombocytopenia, haemolytic anaemia
low complement (C3+4)
proteinuria in suspected SLE
indicated lupus nephritis
investigation for lupus nephritis following positive urine dipstick
biopsy
general management of SLE
sun protection
minimise steroid use
monitoring !!!
management of mild-moderate SLE
hydroxychloroquine
management of acute flairups of SLE
immunosuppressants, short stints of oral steroids
management of severe organ damage associated with SLE
IV steroids and cyclophosphamide
how do we monitor SLE
anti-dsDNA and complement levels, urinalysis for blood or protein
complication of SLE
avascular necrosis, usually of the femoral head
what is sjogrens syndrome
autoimmune condition that affects the exocrine glands
who is more likely to present with sjogrens syndrome
middle aged women
symptoms of sjogrens syndrome
dry eyes, mouth, throat
vaginal dryness in pre-menopausal women
joint pain
fatigue
name 2 clinical signs of sjogrens syndrome
bilateral parotid gland enlargement
unexplained increase in dental carries
investigations for sjogrens syndrome
bloods: anti-ro and anti-la antibodies
management of sjogrens syndrome
symptom control: tear and salivary replacement, analgesia and exercise for pain and fatigue
complications of sjogrens
increased risk of lymphoma
peripheral neuropathy
ILD
what are the 2 main types of systemic sclerosis
limited and diffuse
who usually presents with systemic sclerosis
women aged 30-50
skin involvement in limited systemic sclerosis
face, hands, forearms and feet
raynauds preceeding systemic sclerosis
limited
clinical presentation of systemic sclerosis
CREST
calcinosis, Raynaud’s, oesophageal dysfunction, sclerodactyly, telangiectasia
what is sclerodactyly
thickening of the skin of the fingers and hands which can cause loss of dexterity of hands
name some complications of systemic sclerosis
pulmonary arterial hypertension
pulmonary fibrosis
non-medical management of systemic sclerosis
avoid smoking
skin stretching, regular emollients
physiotherapy for joints
what is used to treat symptoms of raynauds
nifedipine
clinical presentation of mixed connective tissue disease (6)
- Raynaud’s
- Arthralgia/arthritis
- Myositis
- Sclerodactyly
- Pulmonary hypertension
- ILD
3 main investigations for mixed connective tissue disease
bloods, antibodies, imaging
bloods in mixed connective tissue disease
FBC: anaemia, leukopenia
hypergammaglobulinemia
raised inflammatory markers
who usually presents with anti-phospholipid syndrome
women
bloods in anti-phospholipid syndrome
thrombocytopenia
prolonged aPTT