Connective Tissue Disease 2 Flashcards
Define the diagnosis of anti-phospholipid syndrome?
+ve anti-cardiolipin (anti-phospholipid) antibodies
AND/OR
Lupus anti-coagulant activity
AND/OR
Anti-β2-glycoprotein
…on 2 occasions at least 12 weeks apart
Signs of anti-phospholipid syndrome in pregnant women?
Pregnancy loss with no other explanation
Pre-term with because of eclampsia
Severe pre-eclampsia
Signs of placental insufficiency
Occurrence of anti-phospholipid syndrome?
More common in women
Anti-cardiolipin antibodies in anti-phospholipid syndrome?
30% of people with SLE have these and they may occur in other CTDs; also, some of the healthy population have these
Other features of anti-phospholipid syndrome?
Superficial thrombophlebitis and livedo reticularis
Mild/moderate thrombocytopaenia
Neurological features, like migraines, transverse myelitis, etc
Libman-Sacks endocarditis (sterile vegetations)
Catastrophic anti-phospholipid syndrome
Describe catastrophic anti-phospholipid syndrome
Widespread thrombosis that may occur when warfarin is withdrawn, e.g: for surgery
What is Sjorgen’s syndrome?
Autoimmune condition where there is lymphocyte infiltration of EXOCRINE GLANDS, causing xerostomia (dry mouth, dental problems) and keratoconjunctivitis sicca (dryness of conjunctiva)
It may be primary or secondary to other conditions
Classification criteria for primary Sjogren’s syndrome?
- Subjective ocular symptoms (daily for > 3 months)
- Subjective oral symptoms (daily for > 3 months)
- Objective evidence of ocular dryness
- Objective evidence of salivary gland involvement
- Immunology – either Ro, La or both
- Biopsy evidence of lymphocytic infiltrate
4/6 are requires and must inc. either immunology or biopsy evidence
Describe the Schirmer test for Sjogren’s syndrome
Filter paper folded over the lower eyelid; it is left for 5 minutes and should normally have collected 10 ml of tears
Other manifestations of Sjogren’s syndrome?
Fatigue and arthralgia
Raynauds
Salivary swelling
Lymphadenopathy and 40X increased risk of lymphoma
Skin and vaginal dryness
Interstitial lung disease
Neuropathy
Renal tubular acidosis
Neonatal complete heart block (assoc. with anti-Ro)
Occurrence of primary Sjogren’s syndrome?
Peak age between 40-60 but most are undiagnosed cases; more common in females (9:1)
Occurrence of complications in Sjogren’s?
Serious comps. are very rare compared to other CTDs
Treatment of Sjogren’s?
Eye drops and punctal plugs
Saliva replacement
Pilocarpine (increases saliva production)
Hydroxychloroquine (for joint problems)
Steroids and immunosuppression
Attention to CV risk factors
What is systemic sclerosis?
Autoimmune vasculopathy; there is inflammation and fibrosis (excess collagen deposition in skin and internal organs)
Previously knows as CREST syndrome but renamed as this did not inc. pulmonary hypertension
What does CREST stand for?
Calcinosis Raynaud's phenomenon oEsophageal dysmotility Sclerodactyly - shiny skin (lips pucker and check how much their mouth opens) Telangiectasia
Types of systemic scleroderma?
Systemic sclerosis is rheumatology; localised is dermatology
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Antibodies in systemic sclerosis?
Assoc. with anti-centromere antibodies
Describe diffuse systemic sclerosis
Skin changes within 1 year of Raynaud’s
Truncal and acral skin inv.
Early significant organ inv. (like kidneys, gut, lungs, muscle, joints, heart)
Entirety of skin looks like it is made of leather
What antibodies are assoc. with diffuse systemic sclerosis?
Anti-Scl-70 antibodies
Other features of systemic sclerosis?
Nail fold infarcts
GI manifestation of systemic sclerosis?
- Oesophageal hypomobility
- Small bowel hypomobility, bacterial overgrowth
- Pancreatic insufficiency
- Rectal hypomobility
Respiratory manifestation of systemic sclerosis?
- Interstitial lung disease
- Pulmonary hypertension
- Chest wall restriction
Renal manifestations of systemic sclerosis?
- Hypertensive renal crisis
* Ischaemic
Cardiovascular manifestations of systemic sclerosis?
- Raynaud’s with digital ulceration
- Atherosclerotic disease
- Hypertensive cardiomyopathy
Occurrence of systemic sclerosis?
Peak age of 25-55 years; more common in women (4:!)
Testing for systemic sclerosis?
Often creatinine, GFR and BP are more useful than urinalysis
Treatment of systemic sclerosis?
- Calcium channel blockers
- Prostacyclin (PG that causes vasodilatation) for Raynaud’s
- ACE inhibitors for Raynaud’s or BP
- Prednisolone
- Immunosuppression
- Bosentan, Sildenafil (vasodilators) for pulmonary hypertension
What is mixed connective tissue disease (MCTD)?
Signs and symptoms of a combination of disorders — primarily lupus, scleroderma and polymyositis
What is undifferentiated connective tissue disorder?
“Connective tissuish” but does not usually fit a part. syndrome; tends to have a better prognosis
Expect some antibody positivity
Summary of antibodies for different connective tissue diseases?
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