Connective Tissue Diseases Flashcards
(54 cards)
What are connective tissue diseases
They are characterised as a group by the presence of spontaneous over activity of the immune system
What are connective tissue diseases often associated with
Specific auto-antibodies which can help define the diagnosis
What is systemic lupus erythematosis
Systemic autoimmune disease that can affect any part of the body
What is the difficulty for clinicians and SLE
It is often misdiagnosed as it mimics or is mistake n for other illnesses
What populations are more likely to develop Lupus
Women of Afro-Caribbean Afro-Americans and Hispanic Americans or Asian population
Uncommon in African Blacks
What are some of the factors leading to lupus
Oestrogen exposure (hormonal) Genetic factors Environmental factors - silica dust Immunological Factors Infection - particularly viral infections (ebstein barr) UV light
Describe the pathogenesis in a normal cell
You get a foreign antigen (often a virus) and a WBC will pick it up and show it to the T cells. They then release cytokines which provoke an inflammatory reaction which then stimulates the B cells to start producing antibodies against the foreign body
Describe the pathogenesis in autoimmunity
In autoimmune, these antigens are not foreign – they are part of the body and they are cell proteins – all cells break down and die over time. Whats in the cell will continue to float around for a short time – antigen presenting cells pick these up and see this as an external threat – B cells begin to produce antibodies against the normal cellular protein (autoantibodies)
Name some of the constitutional symptoms of SLE
Fever Malaise Poor appetite Weight loss Fatigue
What are some of the mucocutaneous features of SLE
photosensitivity
Malar rash
Discoid lupus erythematosis
Subacute cutaneous lipus
What are some of the musculoskeletal features
Non-deforming polyarthritis/ polyarthralgia
Deforming arhtropathy
Erosive arthritis (rare)
Myopathy - weakness, myalgia and myositis
What are some of the pulmonary features of SLE
Pleurisy Infections Diffuse lung infiltration and fibrosis Pulmonary hypertension Pulmonary infarct
What are some of the cardiac features of SLE
Pericarditis
Cariomyopathy
Pulmonary hypertension
libman -Sachs endocarditis (no bacteria often)
What are the findings in Glomerulonephritis SLE
Proteinuria Urine sediments Urine RBC and casts Hypertension Acute renal failure Chronic renal failure
What are some of the neurological features of SLE
Depression/ psychosis Migranous headache Cerebral ischaemia Cranial or peripheral neuropathy Cerebellar ataxia
What are some of the haematological features of SLE
Lymphadenopathy
Leucopenia
Anaemia
Thrombocytopenia
What are the aims of investigations for suspected SLE
To confirm the diagnosis
To determine the degree of organ involvement
How do we screen for suspected SLE
FBC Renal function tests (Urine examination) Anti-nuclear antibody Anti-Double stranded DNA antibodies ENA Complement levels
In what conditions is Anti-nuclear antibody found (ANA)
Rheumatoid arthritis
HIV
Hep C
What would be the finding in the majority of SLE positive patients in terms of ANA
Positive in titre of 1:160 or greater
When should a positive ANA test be taken seriously
If other antinuclear antibodies are also positive ANti-dsDNA anti-Sm Anti-Rs Anti-RNP
When the patient presents with CTD features
Anti-Double stranded DNA antibody is not highly specific for SLE. True or False.
False - it is highly specific and occurs in 60% of patients with SLE
Anti-SM if highly specific to SLE. What is there a probably association with when this is present
Neurological involvement
What is the general management involved with SLE
Counselling - patients, spouse and relatives
Regular monitoring
Avoid excessive sun-exposure
Pregnancy issues
