Flashcards in Connective Tissue Disease Deck (86):
What are connective tissue disease characterized by?
the presence of spontaneous over-activity of thewh immune system
what type of hypersensitivity is SLE?
who is affected more by lupus- males or females?
what are the 3 main environmental factors which could contribute to the development of SLE?
-virus (eg eptein-barr virus)
-silica dust (found in cigarette smoke)
why might defective apoptosis lead to the production of auto-antibodies?
cell death happens less efficiently so remnants are not cleared away quickly. This allows extended exposure to nuclear and intracellular antigens which might trigger the production of auto-antibodies.
where do the immune complexes get deposited in SLE?
in the wall of blood vessels
what is serositis?
inflammation of a lining ie pericarditis, peritonitis, pleuritis
what are the 5 main constitutional symptoms of SLE? (vague/non specific- most common symptoms)
what are the 7 main mucocutaneous features of SLE?
-discoid lupus erythematosus
-subacute cutaneous lupus
what is typically spared in an SLE malar rash?
what is different about discoid lupus?
can be solely cutaneous symptoms
what are the 4 main musculoskeletal features of SLE?
-deforming arthropathy (Jaccoud's arthritis)
how is non-deforming polyarthritis of SLE differentiated from rheumatoid arthritis even though they have the same distribution?
SLE polyarthritis has no sign of erosion on radiological investigation
What is Jaccoud's arthritis?
a reversibly deforming arthropathy (not erosive)
what are the 5 main pulmonary features of SLE?
-pleurisy (causing pleural effusion)
-diffuse lung infiltrate and fibrosis
-pulmonary infarct (causing haemoptysis)
is a patient with SLE more likely to get a transudate or exudate pleural effusion?
exudate (due to inflammation, transudate more due to leakage)
what are the 4 main cardiac features of SLE?
what is Libman-Sachs endocarditis?
sterile (nonbacterial) endocarditis, valve vegetation but no microbes on lood cultuees
How do you detect glomerulonephritis in a patient with SLE?
what are the 5 main neurological features of SLE?
-cerebral ischamie (TIA or CVA)
-cranial or peripheral neuropathy
what are the 4 main haematological features?
why do patients with SLE have a susceptibility to infection?
-intrinsic factors (impaired immunity)
-extrinsic factors (ie immunosuppressive treatments)
what are the main immune tests to do in suspected SLE?
-anti-double stranded DNA antibody
-anti-extractable nuclear antigens (eg anti-Ro, anti-La, anti-Sm and anti-RNP)
which is more specific for lupus: anti-ds DNA or ANA
(rarely get false positives)
which is antibody is present in more SLE patients- anti-ds DNA or ANA?
what antibody is important in pregnant patients with SLE because of pathogenic effects on foetus?
what can anti-Ro cause in a neonate?
congenital heart block (in utero)
what happens to anti-ds DNA titre and complement titre when SLE starts to become active again?
anti-ds DNA titre increase
complement titre decreases
why do complement levels decrease in the body during active SLE?
complement is being used up
what is the drug treatment of SLE?
-NSAIDs and simple analgesia
when are steroids used in SLE?
for acute flare up, never used long term
what is the function of rituximab (a biologic)?
B cell depletion therapy
what autoantibodies can be present in anti-phospholipid syndrome?
how do you diagnose anti-phospholipid syndrome?
1 laboratory sign AND 1 clinical sign
[on 2 occasions, 12 weeks apart]
-arterial or venous thrombosis
-pregnancy loss with no other explanation from 10 weeks +
-3 pregnancy losses with no other explanation below 10 weeks
-pregnancy loss due to eclampsia, severe pre-eclampsia or placental insufficiency
why do patients with anti-phospholipid syndrome get blood clots when many have lupus anti-coagulant factor?
lupus anti-coagulant factor actually is a pro-coagulation factor in vivo
it only inhibits coagulation in vitro
who tends to get anti-phospholipid syndrome?
what type of skin rash can be a feature of anti-phospholipid syndrome?
(a vascularitic skin rash with a marbling appearance)
what is the the platelet count like in patients with anti-phospholipid syndrome usually?
(still risk of thrombosis)
what is the pharmacological treatment of anti-phospholipid syndrome?
for patients with prev episode of thrombosis- lifelong anti-coagulation (with heparin)
for patients with recurrent pregnancy loss- aspirin + heparin during future pregnancy
What is Sjogren's syndrome?
an autoimmune condition which causes a lymphocytic infiltration of exocrine glands leading to xerostomia and keratoconjuctivitis sicca
(+ potentially other areas of dryness)
what are the 2 main autoantibodies associated with Sjogren's syndrome?
what test is useful for testing xerostomia in suspected sjogren's sundrome?
why can anti-Ro cause teratogenic effects?
anti-Ro can cross the placenta
who tends to get primary sjogren's syndrome?
what is the pharmacologicaly treatment of Sjogren's syndrome?
-punctal plugs + eye drops
-steroids + immunosuppression (only for severe case)
what is the function of pilocarpine?
what type of patients is pilocarpine contra-indicated in?
What is scleroderma/systemic sclerosis?
an autoimmune condition which causes fibrosis of the skin and internal organs
why does fibrosis of the skin and internal organs occur in scleroderma/systemic sclerosis?
due to excess deposition of colllagen
what vasculopathy of the hands occurs in almost every patient with scleroderma?
What are the 2 classifications of scleroderma/systemic sclerosis?
1. localised scleroderma
2. systemic scleroderma
what is the difference between localised and systemic scleroderma?
in localised scleroderma it is isolated to the skin, systemic scleroderma has systemic involvement
what are the 2 types of localised scleroderma?
what are the 3 types of systemic scleroderma?
limited systemic sclerosis
diffuse systemic sclerosis
systemic sclerosis sine scleroderma
What are the features of limited systemic sclerosis?
E- esophageal dysmotility
+ pulmonary hypertension
what is calcinosis?
depositions of calcium nodules in the sin
what is raynauds?
an exaggerated vasoconstrictive process in the hands in the cold
what is sclerodactyly?
skin thickening on the fingers
what is telangiectasia?
dilated capillaries on the hands, face and mucous membranes
what antibodies are associated with limited systemic sclerosis?
What is diffuse systemic sclerosis?
systemic sclerosis with early significant organ involvement and skin changes
what antibodies are associated with diffuse systemic sclerosis?
what is systemic sclerosis sine scleroderma?
systemic sclerosis with organ involvement but no skin changes
who is more likely to get scleroderma- M or F?
what are the treatments for scleroderma?
pulmonary hypertension: calcium channel blockers, bosentan, sildenafil, iloprost
interstitial lung disease: immunosuppression
reflux disease: PPI
what is the main con of using steroids in the treatment of scleroderma?
may provoke a hypertensive crisis
What is the autoantibody associated with mixed connective tissue disease?
what is the autoantibody associated with polymyositis?
for all connective tissue disease, what does the treatment hugely depend on? (what is the one exception to this)
depends on patients manifestations (which are very variable)
hydroxychloroquine in SLE is the one exception- ALWAYS used if possible
in connective tissue disease, what are the main risk factors to try and reduce?
cardiovascular risk factors
what connective tissue disease is associated with increased lymphoma risk?
what are the 3 phases of cutaneous involvement of scleroderma and what does this lead to?
skin becomes thickened and tight
compare limited and diffuse systemic sclerosis in terms of location of skin changes?
limited- skin involvement confined to face, hands, forearms and feet
diffuse- can involve trunk (devlops more rapidly)
compare limited and diffuse systemic sclerosis in terms of development of organ involvement?
limited- organ involvement occurs late
diffuse- early organ involvement
compare limited and diffuse systemic sclerosis in terms of antibody association?
limited- anti-centromere antibody
diffuse- anti-Scl-70 antibody
what is the treatment for raynauds/digital ulcers in scleroderma?
calcium channel blockers
what is the treatment for renal involvement in scleroderma?
what is the treatment for acid reflux in scleroderma?
proton pump inhibitos
what is the treatment for interstitial lung disease in scleroderma?
(usually with cyclophosphamide)
What is catastrophic anti-phospholipid syndrome?
a rare, serious and often fatal manifestation of APS characterised by multiorgan infarcations over a period of days-weeks
how can you distinguish between a malar rash and acne rosacea?
malar rash- sparing of the nasolabial folds
(malar rash forms where sun has fallen on the face- photosensitivity)
what treatments are used for mild SLE disease?
what treatments are used for moderate SLE disease?
what treatments are used for severe SLE disease?
what are the 2 reasons a patient with diffuse scleroderma might have shortness of breath?