Connective Tissue Disease Flashcards
What is a Connective tissue disease?
Collective name for a heterogenous group of diseases with overlapping clinical feaetures and. Shared immunopatholgoy
What are some connective tissue diseases?
Systemic Lupus erythematous (SLE)
Sjorgens syndrome
Systemic sclerosis
Idiopathic inflammatory myopathies
What is an undifferentiated Connective tissue disease?
Essentially when theres not enough to diagnose a defined connective tissue disease
What antibody is usually positive in patients with systemic lupus erythematous?
ANA (Anti Nuclear antibodies)
What are some causes of a positive Anti Nuclear Antibody (ANA)?
SLE
Polymyositis/dermatomyostitis
Rheumatoid arthritis
Sjorgens
Scleroderma
Vasculitis
Drug induced
Hepatic disease
Pulmonary disease
Malignancy
Idiopathic thrombocytopenia
What are 3 autoantibodies that are often positive in patients with systemic lupus erythematous?
ANA. (Anti Nuclear Antibodies)
Anti -dsDNA
Anti-Sm
Which antibody is very specific to Systemic lupus erythematous?
Anti-Sm (anti smiths)
Its not seen in any other connective tissue disease
What is systemic lupus erythematous?
Inflammatory Autoimmune connective tissue disorder affecting multiple organs and systems
What is a typical patient for systemic lupus erythematous?
Female
Asian, African, carribea, Hispanic
Middle age - young
What is the pathophysiology of Systemic Lupus Erythematous (SLE)?
Anti Nuclear Antibodies (ANA) Attack nucleus leading to a chronic inflammatory response
How can patients present with SLE due to the chronic inflammation due to the ANA?
Malar rash
Photosensitive rash
Fatigue
Weight loss
Arthralgia
Myalgia
SOB
Pleuritic chest pain
Mouth ulcers
Hair loss
Oedema
What investigations would you do ? Systemic Lupus Erythematous?
Routine bloods
FBC (Anaemia?)
U+Es ( Lupus nephritis) then renal biopsy if indicated
LFTs
Urine dip (blood, protein, albumin to creatinine ratio)
CRP, ESR
TSH, Vit D, Creatine kinase levels
Autoantibody immunological screen
What imaging might you need to do for a person with SLE?
CXR (if SOB)
ECG
echo
US abdomen if needed
Organ directed imaging if needed
What are some auto anti bodies that will be screened for when querying systemic lupus erythematous?
ANA
Anti-dsDNA
Anti-Sm
Anti RO , anti LA
Anti Jo-1
Anti Scl-70
Anti phospholipid antibodies
What does a positive ANA, Anti-dsDNA and Anti-Sm typically indicate?
Systemic Lupus Erythematous
What does a positive anti-Ro anti-La potentially indicate?
Sjorgens disease
What does a positive Anti-Jo-1 potentially indicate?
Dermatomyositis
What does a positive Anti-Scl-70 potentially indicate?
Systemic sclerosis
What is the diagnostic criteria for SLE?
Any 4 of:
Malar rash
Discoid rash
Photosensitive rash
Oral or nasopharynx ulcers
Arthritis
Serositis
Unexplained renal disease
Positive ANA
Serology
Autoimmune haemolytic anemia
Why would you do a coombes test for a patient with systemic lupus erythematous?
Patients with SLE can be anaemic due to anaemia of chronic disease
Or
Due to autoimmune haemolytic anaemia which a positive Coombes test would suggest
What other autoimmune condition can SLE cause?
Antiphospholipid syndrome
Would a patient with antiphospholipid syndrome secondary to SLE have positive Anti-Sm?
Possibly yes
Would a patient with antiphospholipid syndrome that’s not causes by SLE have positive Anti-Sm?
No since Anti-Sm only shows with SLE
What are some complications of systemic lupus erythematous?
Cardiovascular disease (hypertension and coronary heart disease due to chronic inflammation)
Infection
Anemia
Pericarditis
Pleuritis
Interstitial lung disease
Lupus nephritis
Neuropsychiatric SLE
VTE DUE TO SECONDARY ANTIPHOSPHOLIPID SYNDROME
