Connective Tissue Diseases Flashcards
Connective Tissue diseases (5)
Systemic Lupus Erythematous Sjogren's Disease Systemic Sclerosis Mixed connective tissue disease Anti-phospholipid syndrome
Systemic Lupus Erythematous
Chronic Autoimmune Condition
SLE Epidemiology
Females
20-30
Afro-carribeans
SLE Pathophysiology
- Defect in apoptosis
- Increased cell death
- Disturbance in immune tolerances
- Defective clearance of cell debris
- Persistence of antigen and subsequent immune complex formation
- Immune complexes deposited. in small vessels or in the basement membranes of the skin and kidneys causing complement activation and inflammation
SLE Organ Involvement
Any organ System
Renal
-Lupus nephritis
Respiratory
- Pleurisy
- Pneumonitis
- Pulmonary Effusion
- Pulmonary hypertension
- Interstitial lung disease
Haematological
- leukopenia
- lyphopenia
- anaemia
- thrombocytopenia
Neuropsychiatric
- seizures
- psychosis
- headaches
- aseptic meningitis
GI
- Autoimmune hepatitis
- pancreatitis
- mesenteric vasculitis
Cardio
- Pericarditis
- Myocarditis
- Pericardial effusion
- Accelerated ischaemic heart disease
SLE symptoms
weight loss fever fatigue arthralgia myalgia inflammatory arthritis avascular necrosis malar rash photosensitivity discoid lupus oral/ nasal ulceration Raynauds Alopecia
SLE Investigations
FBC ANA Anti-DsDNA Anti-SM Anti-Ro Anti-La Anti-RNP Low C3/ C4 levels Urinalysis (look for glomerulonephritis)
SLE Screening
CT Chest
mRI
- For cerebral vasculitis
Echo
- for heart abnormalities
SLE treatment
Skin disease and arthralgia
- hydroxychloroquine
- topical steroids
- NSAIDs
Inflammatory arthritis or systemic organ involvement
- Immunosuppression (azathioprine/ mycophenolate mofetil)
- Corticosteroids
Severe organ involvement
- cyclophosphamide
- IV steroids
Unresponsive
- IV immunoglobulin (rituximab)
Sjogren’s Disease
Autoimmune condition characterised by lymphocyte infiltrates in exocrine organs
Sjogrens disease symptoms
Sicca
- ocular dryness
- mouth dryness
Arthralgia
fatigue
vaginal dryness
parotid gland swelling
Sjogrens disease associations
Peripheral neuropathy
interstitial lung disease
increased risk of lymphoma
Sjogren’s disease diagnosis
Ocular dryness
- Schimers test
+ve anti-Ro and anti-La antibodies
Typical features on lip gland biopsy
Sjogren’s disease treatment
Lubricating eye drops
Pilocarpine
- Saliva replacement
- flushing is a side effect
hydroxychloroquine
Immunosuppression
Systemic Sclerosis
Systemic connective tissue disease
Systemic sclerosis characteristics
Vasomotor disturbances- Raynauds
Fibrosis and. subsequent atrophy of the skin and subcutaneous tissue
Excessive collagen deposition can cause skin and internal organ changes
Renal and lung changes
- pulmonary hypertension
- main cause of death in systemic sclerosis patients
Systemic Sclerosis Phases
Oedematous
Indurative
Atrophic
Systemic Sclerosis Features
Major
- Centrally located skin lesions that affect the arms, face and neck
Minor
- Sclerodactyly
- Atrophy of fingertips
- Bilateral lung fibrosis
Diagnosis needs 1 major and 2 minor
Systemic sclerosis symptoms
Pinching of the skin around nose
- Beaking
Tightening of skin
Telengectasia
Calcinosis of digits
-subcutaneous deposits of calcium
Systemic Sclerosis Systems Affected
Respiratory
- Pulmonary hypertension
- Fibrosis
Cardio
- Hypertension
Renal
- Failure due to hypertension
GI
- Dysphagia
- Malabsorption
- Bacterial overgrowth
MSK
-Inflammatory arthritis
Systemic Sclerosis Types
Limited
- Anti centromere antibody
- Skin involvement generally limited to face, hands and forearms
- Organ involvement occurs later
Diffuse
- Anti Scl-70 antibody
- Skin changes come rapidly and tend to affect more areas including the trunk and arms
- Significant early organ involvement
SLE Organ Screening
Pulmonary function tests
Echo
monitoring of renal function
SLE Treatment
Raynauds
- CCB
- Iloprost
- Bosentan
- Slidenophil
Renal Involvement
-ACE Inhibitors
ILD
- Cyclophosphomide
Mixed Connective Tissue Disease Antibody
Anti-RNP
Mixed Connective Tissue Disease Screening
Echo
Pulmonary Function Tests
Anti-Phospholipid Syndrome Characteristics
Recurrent venous or arterial thrombosis
Fetal loss after 10 weeks)
Anti-Phospholipid Syndrome aetiology
Can occur secondary to SLE
Anti-phospholipid syndrome complications
Increased frequency of stroke and MI (secondary in-situ thrombosis or embolisation from vascular lesions off limban-sacks endocarditis)
Pulmonary hypertension (recurrent pulmonary emboli)
Catastrophic APS (multi organ failure over a period of days to weeks)
livedo reticularis
Anti-Phospholipid investigations
Blood Tests.
- aPTT
- Thrombocytopenia
- antibodies (ANti-cardiolipin, lupus anticoagulant, anti-beta2 glycoprotein)
Anti-phospholipid treatment
Thrombosis
- Warfarin
Thrombosis during pregnancy
- LMWH
