Connective Tissue Disease Flashcards
What structures does SLE mainly affect?
skin, joints, kidneys, blood cells and nervous system
How many points of criteria does a person need to be diagnosed with SLE?
at least four with one being clinical and one being laboratory
What is another way of diagnosing SLE other than the SILCC classification?
biopsy-proven lupus nephritis with positive ANA or anti-DNA
What are the six things that make up the immunologic criteria for SLE?
ANA, anti-DNA, anti-Sm, antiphospholipid Ab, low complement, direct Coombs test
What populations is SLE more present in?
Black people in the UK and the USA
Is SLE more common in men or women?
women (like 90%)
What is the 10 year survival rate in patients with SLE?
90%
What are the constitutional symptoms of SLE?
fever, fatigue and weight loss
What are the MSK features of SLE?
arthralgia, myalgia and inflammatory arthritis
what are the Muco-cutaneous features of SLE?
malar rash, butterfly rash, alopecia, photosensitivity, discoid lupus, subacute cutaneous lupus, oral/nasal ulceration and Raynaud’s
What are the renal symptoms of SLE?
lupus nephritis
What are the respiratory symptoms of SLE?
pleurisy, pleural effusion, pneumonitis, PE, pulmonary hypertension, interstitial lung disease
What are the haematological symptoms of SLE?
leukopenia, lymphopenia, anaemia and thrombocytopenia
What are the cardiac symptoms of SLE?
pericarditis, pericardial effusion, pulmonary hypertension, sterile endocarditis and IHD
What are the neuropsychiatric symptoms of SLE?
seizures, psychosis, headache, aseptic meningitis
What are the GI symptoms of SLE?
autoimmune hepatitis, pancreatitis and mesenteric vasculitis
What investigations would you do in SLE?
FBC, ANA, Anti-dsDNA, anti-Sm, anti Ro/LA/RNP, C3/4 levels, urinalysis and imaging
How do you manage skin disease and arthralgia hydroxychloroquine caused by SLE?
topical steroids and NSAIDs
How do you treat moderate SLE disease?
azathioprine, mycophenolate mofetil and corticosteroids
How do you treat severe organ disease in SLE?
IV steroids and cyclophosphamide
how do you treat patients with SLE where they have been unresponsive to previous treatment?
IV immunoglobulin and rituximab
What is Sjogren’s syndrome
an autoimmune condition characterised by lymphatic infiltrates in exocrine organs
What are the key symptoms in Sjogren’s syndrome?
dryness of mouth and eyes, arthralgia, fatigue, vaginal dryness and parotid gland swelling
what can Sjogren’s syndrome be secondary to?
RA and SLE
What conditions are you more at risk of if you have Sjogren’s?
Peripheral neuropathy, interstitial lung disease and lymphoma
How do you diagnose Sjogren’s?
Schirmer’s test and +ve anti-Ro and anti La antibodies and a +ve lip gland biopsy
How many of the American-European consensus group classification do you need to diagnose Sjogren’s?
four
Is treatment for Sjogren’s curative or managing symptoms?
managing symptoms
When would immunosuppression be involved in Sjogren’s?
when there is organ involvement like interstitial lung disease
what symptoms present in Systemic sclerosis?
vasomotor disturbances, fibrosis and atrophy of the skin and subcutaneous tissue
What causes systemic sclerosis?
excessive collagen deposition
What is a finding in most patients with systemic sclerosis?
Raynaud’s
What are the 3 phases of cutaneous involvement of systemic sclerosis?
1) oedematous, 2) indurative and 3) atrophic
What are the major cutaneous features of systemic sclerosis?
central sclerosis that affects the arms, face and/or neck
What are the minor cutaneous features of systemic sclerosis?
sclerodactyly, atrophy of the fingertips and bilateral lung fibrosis
How do you diagnose, systemic sclerosis?
1 major and 2 minor features
How do you classify systemic sclerosis?
limited or diffuse
How do you investigate systemic sclerosis?
test for autoantibodies including anti-centromere and anti-Scl-70
organ screening
How do you manage systemic sclerosis?
treat symptoms
What symptoms are featured in Mixed connective tissue disease that are seen in other conditions?
Raynaud’s, arthralgia/ arthritis, myositis, sclerodactyly, pulmonary hypertension and interstitial lung disease
What antibodies are associated with mixed connective tissue disease?
anti-RNP
How do you manage mixed connective tissue disease?
maybe Ca2+ blockers, or immunosuppression
What is anti-phospholipid syndrome?
recurrent venous or arterial thrombosis
How do patients with anti-phospholipid syndrome present?
multiple conditions associated with clots, catastrophic APS, late spontaneous foetal loss, migraine, livedo reticularis
How do you investigate anti-phospholipid syndrome?
thrombocytopenia and prolongation of APTT, lupus anticoag, anti-cardiolipin antibodies and anti-beta 2 glycoprotein
How do you treat those with anti-phospholipid syndrome?
anti-coagulates, heparin if preggo
