Connective Tissue Disease Flashcards
Constitutional Symptoms of SLE
Fever Malaise Poor appetite Weight loss Fatigue
SLE Environmental Factors
Environmental factors:
Viruses eg Epstein-Barr Virus
UV light may stimulate skin cells to secrete cytokines stimulating B-cells
Silica dust (found in cleaning powders, cigarette smoke and cement) may increase risk of developing SLE
SLE Mucocutaneous Features
Photosensitivity Malar rash may or may not be associated with sun exposure Discoid lupus erythematosus Subacute cutaneous lupus
SLE Musculoskeletal Features
Non-deforming polyarthritis/polyarthralgia
RA distribution but no radiological erosion
Deforming arthropathy - Jaccoud’s arthritis
Erosive arthritis - rare
Myopathy - weakness, myalgia & myositis
Swan neck deformities
These result from recurrent synovitis and inflammation of the joint capsule, tendons and ligaments
SLE Pulmonary Features
Pleurisy Infections Diffuse lung infiltration and fibrosis Pulmonary hypertension Pulmonary infarct
SLE cardiac features
Pericarditis
Cardiomyopathy
Pulmonary hypertension
Libman Sach endocarditis (non-bacterial endocarditis)
SLE Glomerulonephritis Presentation
Proteinuria Urine sediments Urine RBC and casts Hypertension Acute renal failure Chronic renal failure
What are casts?
Cylindrical structures produced by the kidneys, present in some disease states
SLE Neurological Features
Depression/psychosis Not always related to disease activity Migranous headache Cerebral ischaemia TIAs or stroke Cranial or peripheral neuropathy Cerebellar ataxia
SLE Haematological Features
Lymphadenopathy
~25% of all patients during their course of illness
Leucopenia (low white cells)
Anaemia (haemolytic, normochromic normocytic)
Thrombocytopenia (low platelets)
Screening Tests for Suspected SLE
Full blood count Renal function tests including urine examination Anti-nuclear antibody Anti-double stranded DNA antibodies ENA Complement levels
Conditions that ANA is found in
SLE, RA, hepatitis C, HIV, other autoimmune conditions
Anti-SM and what it is likely to be involved with?
SLE
Neurological association
Anti-La
Sjogrens
Anti-Ro
Sjogrens
Anti-Scl70
Scleroderma
Anti-RNP
Mixed Connective Tissue disease
Overlap features – sclerodermatous skin lesions, Raynaud’s phenomenon, low grade myositis
In SLE, what titre correlates with overall disease activity?
Anti-double stranded DNA antibody
May be associated with lupus nephritis
What antibody may be associated with lupus nephritis in SLE?
Anti-double stranded DNA antibody
In SLE, what is anti-Ro associated with?
Cutaneous manifestations
What could happen with anti-Ro and a foetus?
Neonatal heart block, neonatal LE
Other investigations for SLE
Depend on symptoms CXR Pulmonary function tests CT chest Urine protein quantification Renal biopsy Echocardiogram Nerve conduction studies MRI brain
What could you measure that negatively correlated with SLE activity?
Complement C3/C4
anti-double stranded DNA antibody correlates WITH activity
Monitoring SLE activity
Thorough clinical assessment including BP
Anti-dsDNA level positively correlates with activity
C3/C4 levels negatively correlate with activity
Urine examination including protein, cells and casts
Full blood count
Blood biochemistry
SLE general management
Counselling - patients, spouse and relatives
Regular monitoring
Avoid excessive sun-exposure
Pregnancy issues
SLE drug treatment
NSAID and simple analgesia
Anti-malarials – chloroquine and hydroxychloroquine
Useful for arthritis, cutaneous manifestations and constitutional symptoms
May reduce systemic complications
SLE - Biologics
Anti-CD20 (Rituximab)
Anti-Blys (Belimumab)
SLE - Immunosuppressives
Azathioprine Cyclophosphamide Methotrexate Mycophenolate mofetil (All can cause bone marrow suppression All can cause increased susceptibility to infection Potentially teratogenic)
What do SLE patients die of?
Infection important cause of death in the early / medium stage of disease
Cardiovascular disease the major the cause of death in the late stages
What is often the first sign of systemic sclerosis?
Raynauds
Treatment for sjogrens syndrome
- eye drops/ punctal plugs
- Pilocarpine
- artificial saliva
- steroids & immunosuppression
- hydrochloroquinine
- ATTENTION TO CARDIOVASCULAR RISK FACTORS
Name some CVS risks associated with sjogrens
Heart attack
Stroke
Hypertryglyceridemia
Hypertension
(this is because of acute inflammatory state in sjogrens)
The older name for limited sceloderma
The older term for limited scleroderma is CREST syndrome (= Calcinosis, Raynaud’s disease, (O)Esophageal dysmotility, Sclerodactyly, Telangiectasia).
PULMONARY HYPERTENSION IN 30%
Which sjogrens antibody is associated with neonatal complete heart block?
Anti-Ro
Which antibodies are associated with limited scleroderma?
Anti-centromere
Which antibodies are associated with diffuse scleroderma?
Anti-Scl-70
How quickly do skin changes occur in diffuse systemic sclerosis?
Within one year of raynauds
Which organs are commonly involved in diffuse systemic sclerosis?
Kidneys, lungs, gut, muscle, joints, heart
What is juvenile sclerosis?
When patient under 16 presents with 1 major and at leas two of the 20 minor criteria of systemic sclerosis
Respiratory organ involvement in systemic sclerosis
ILD
Pumonary hypertension
CHEST WALL RESTRICTION
GI organ involvement in systemic sclerosis
Oesophageal hypomobility
Small bowel hypomobility, bacterial overgrowth
Pancreatic insufficiency
Rectal hypomobility
Name a medication used to treat pulmonary arterial hypertension
Bosentan, sildenifil
Treatment of systemic sclerosis
Calcium channel blockers Prostacyclin (Iloprost) ACE inhibitors Prednisolone Immunosuppression Bosentan, Sildenafil
Trigeminal neuralgia
Extreme severe facial pain that tends to come and go in sudden shock like attacks
-like an electric shock
