Connective Tissue Disease Flashcards
(56 cards)
How does antiphospholipid syndrome manifest clinically?
As arterial or venous thrombosis (common cause of these in people under 50)
Pregnancy loss with no other explanation or pre-term birth because of eclampsia, severe pre-eclampsia or with signs of placental insufficiency.l
Pregnancy loss is commonly late, in the second or third trimester but can be at any time
What do we need to diagnose antiphospholipid syndrome?
One positive lab test and one clinical feature present in the right patient context.
What lab tests can be used as part of the diagnosis for antiphospholipid syndrome?
Positive anti-cardiolipin (anti-phospholipid) antibodies.
And/or lupus anticoagulant activity
And/or anti-beta2-glycoprotein
These must be present on 2 occasions at least 12 weeks apart.
Is antiphospholipid syndrome more common in males or females?
Females X 3.5
What other types of people can have anti-cardiolipin antibodies other than APS patients?
Around a third of those with SLE.
Some in people with other CTD’s
Around 1-5% of the healthy population
What features other than pregnancy loss and arterial/venous thrombosis do we see in APS?
Superficial thrombophlebitis and livedo reticularis
Mild/moderate thrombocytopenia
Neurological features e.g. Migraine and transverse myelitis
Libman Sachs endocarditis
What CTD should we be suspicious of if we see strokes or MI’s in younger patients?
APS
What can strokes develop secondary to in APS?
In situ thrombosis or Libman sacks valvular lesions.
What kind of lesions are found in Libman sacks endocarditis?
Sterile.
What can recurrent PE or thrombosis lead to in APS patients?
Life threatening pulmonary hypertension.
What is CAPS?
Catastrophic APS. Characterised by multi organ infarctions seen over days to weeks.
What serum results can we find in APS?
Thrombocytopenia and prolonged APTT (anti partial thromboplastin time)
What are the treatments for APS?
Thrombosis (lifelong anticoagulation)
Pregnancy loss is given aspirin and heparin
Attention is paid to vascular risk factors.
Why is warfarin not given to APS sufferers during pregnancy?
It is teratogenic.
Is Sjogrens a primary or secondary condition?
It can be either
What is Sjorgens and what does it cause?
An autoimmune condition resulting in lymphocyte infiltration of the exocrine glands. This causes xerostomia and keratoconjunctivitis sicca (commonly in the lacrimal glands and mouth.
What is xerostomia?
Dryness in the mouth.
What are the 6 classification criteria of Sjogrens?
Subjective ocular symptoms (daily for over 3 months)
Subjective oral symptoms (daily >3 months)
Objective evidence of ocular dryness
Objective evidence of salivary gland involvement
Either Ro La or both
Lip gland biopsy with evidence of lymphocytic infiltrate
What must be present in order to diagnose Sjogrens?
4 of 6 of the classification criteria which must include either immunology or biopsy evidence.
What is Schirmers test?
A test for Sjogrens where paper is put in the lower eyelid and left for 5 mins. Tears should come down to at least 10mm
What are some of manifestations of Sjogrens other than eye and mouth involvement?
Fatigue Arthralgia Raynauds Salivary swelling (especially parotid) Lymphadenopathy (predominantly cervical) Skin and vaginal dryness Interstitial lung disease (far more common in other CTD's) Neuropathy (in the glove stocking pattern) Lymphoma Renal tubular acidosis Neonatal complete heart block (anti Ro)
What is the peak age for primary Sjogrens?
40-60 years old
Does Sjogrens affect males or females more?
Females x9
What are the treatments for Sjogrens?
Mostly to stress symptoms
Eye drops and punctual plugs
Regular dental care
Saliva replacement or stimulation (pilocarpine stimulates but may cause flushing)
Hydroxychloroquine can help with arthralgia and fatigue
Steroids and immunosuppression
CVS risk factor monitoring
