connective tissue disease Flashcards
SLE is most common in
- women of child bearing age
- Afro-Caribbean and Asian populations
- associated with other auto-immune disease
SLE presentation
at least 4 of the following
- fatigue, weight loss, malaise, fever, poor appetite
- malar rash that spares the nasolabial folds (butterfly rash), photosensitivity, alopecia, scaly scarring discoid rash
- mouth ulcers
- non-erosive small joint arthritis similar to rheumatoid arthritis, Jaccouds arthritis, myopathy
- pleuritis, cericarditis
- glomerulonephritis
- Raynaud’s, cardiomyopathy, Libman sack endocarditis
- migraines, stroke, neuropathy, seizures
- lymphadenopathy, thrombocytopenia
SLE diagnosis
- urinalysis
- bloods (FBC, U&E)
- anti-DsDNA (most specific), anti-ANA, anti-Ro, anti-la, anti-Sm, anti-RNP, completement
- chest x-ray to exclude interstitial lung disease
SLE management
- monitor FBC, U&E and urine protein
- mild: hydroxychloroquine, NSAIDs, topical steroids
- moderate: methotrexate, azathioprine, oral steroids
- severe: IV steroids, cyclophosphamide, rituximab
- steroids for flares
- if it’s only affecting joints and skin the hydroxychloroquine
anti-phospholipid syndrome causes
can occur as primary disease or secondary to lupus
anti-phospholipid syndrome presentation
- recurrent venous or arterial thrombosis, with venous being more common
- recurrent miscarriages/late spontaneous foetal loss
- superficial thrombophlebitis
- livedo reticularis (mottled discolouration of the skin(
- transverse myelitis
- migraines
anti-phospholipid syndrome diagnosis
- immunology positive on 2 occasions at least 12 weeks apart (anti-cardiolipin antibody, lupus anti-coagulation test, anti-B2 glycoprotein), thrombocytopenia, prolonged APTT
anti-phospholipid syndrome management
- warfarin
- aspirin in between pregnancies, and aspirin and warfarin during pregnancy
- if no thrombotic event then low dose aspirin
sjogren’s syndrome is most common in
40-60 years old
sjogren’s syndrome is associated with
lymphocytic infiltration of the exocrine glands, which ultimately results in their fibrosis
sjogren’s syndrome causes
can be primary or secondary to SLE, primary biliary cholangitis and systemic sclerosis
sjogren’s syndrome presentation
- keratoconjunctivitis sicca (dry eyes)
- xerostomia (dry mouth)
- parotid swelling
- fatigue, Raynaud’s, arthralgia, skin and vaginal dryness, lymphadenopathy and neuropathy
sjogren’s syndrome diagnosis
- Schirmer’s test
- anti-Ro, anti-La, hypergammaglobulinemia
- gland biopsy for lymphocytic infiltration
sjogren’s syndrome management
- monitor for non-Hodgkin’s B cell lymphoma
- give eye drops for dry eyes
- give artificial saliva for dry mouth
- pilocarpine (muscarinic antagonist)
- NSAIDs and hydroxychloroquine for arthralgia
localised scleroderma pathophysiology
- dermal fibrosis and inflammation without involvement of any viscera
- can be morphea (individual patches of skin fibrosis) or linear (when fibrosis follows dermatomal distribution)
- likely to be seen along with Raynaud’s
