Rheumatology: Connective tissue disease Flashcards
What is the criteria for diagnosing SLE?
More than 4 criteria (at least 1 clinical and 1 laboratory) OR biopsy proven lupus nephritis with positive ANA or Anti dsDNA Clinical criteria 1. Acute or chronic cutaneous lupus 2. Oral or nasal ulcers 3. Alopecia 4. Arthritis 5. Serositis 6. Renal involvement 7. Neurological 8. Haemolytic anaemia 9. Leukopenia 10. Thrombocytopenia Immunological criteria 1. ANA 2. Anti DNA 3. Antiphospholipid 4. Anti Sm 5. Low C3 and C4 6. Direct coombs test
What causes SLE?
Defect in apoptosis causes increased cell death and a disturbance of immune tolerance. This causes cell debris and persistent antigen and immune complex production.
These immune complexes form in the small blood vessels which leads to complement activation and inflammation. They are also deposited on the basement membrane of skin and kidneys.
How is lupus treated?
Dependant on manifestations. For skin disease and arthlagia hydroxychloroquinin is used as well as topical steroids and NSAIDS.
If there is inflammatory arthritis or organ involvement then immunosupression with azathiprine or mycophenolate is used.
In severe organ disease then IV steroids and cylophosphamise may be needed
If still unresponsive then IV immunoglobulin and rituximab may be needed
How are people with SLE monitored?
Double stranded DNA and complement levels
Urinalysis for blood or protein, which may indicate glomerulonephritis Evaluation and management of cardiovascular risk factors
What malignancy are you at increased risk of in Sjogrens?
Lymphoma
What is the diagnostic criteria for sjogrens?
4 of…
- Occular symptoms for 3 months
- Oral symptoms
- Occular signs (schirmer test)
- Oral signs
- Positive salivary gland biopsy
- Positive Ro or La antibodies
What is the treatment of sjogrens?
Symptomatic
Hydroxychloroquine
What is limited systemic sclerosis?
Skin sclerosis/tightening on the face, forearms, hands and feet. Organ involvement occurs later.
Anti centromere antibody
What is diffuse systemic sclerosis?
Skin changes develop rapidly and there is early organ involvement. Anti Scl 70 antibody
What are the organ manifestations of systemic sclerosis
Pulmonary hypertension
Pulmonary fibrosis
Accelerated hypertension leading to a renal crisis
Gut involvement may lead to dysphagia, malabsorption and bacterial overgrowth
What is systemic sclerosis?
Connective tissue disease where there is excessive collagen deposition which causes skin and internal organ changes. Raynauds is an early finding
What is the management of systemic sclerosis?
Raynauds: Calcium channel blockers, lloprost, bonentan
Renal onvolvement: ACEi
GI: PPI
Interstitial lung disease: immunsusupression usually with cyclophosphomide
What antibodies are assoctaed with mixed connective tissue disease?
Anti RNP
What do you get regular echocardiograms if you have mixed connective tissue disease?
Risk of pulmonary hypertension
What medication is used to treat raynauds?
Calcium channel blockers
