Connective Tissue Diseases Flashcards
(28 cards)
What CTD is this: autoimmune condition characterised by lymphocytic infiltrates in exocrine organs
Sjogrens
What is the classic presentation of sjogrens?
Dry eyes and mouth, vaginal dryness.
Other symptoms are more general: arthralgia, fatigue
Which cancer risk is increased with Sjogrens?
Lymphoma
What test do you do for Sjogrens and what is a positive result?
Schirmers test
Filter paper used to assess tear flow, less than 5mm on the paper in 5 mins indicates Sjogrens
Which antibodies are associated with Sjogrens syndrome?
Anti-Ro and Anti-La
What is the main concern with anti-ro and anti-la antibodies?
Neonatal complete heart block
What are the typical features of Sjogrens found on a lip biopsy?
Tight clumps of lymphocytes adjacent to normal gland tissue
What are the occular symptoms of Sjogrens?
Dry eyes
Foreign body sensation
What are the oral symptoms of sjogrens?
Dry mouth
Recurrently swollen salivary glands
How is Sjogrens managed?
Symptomatically Lubricating eye drops Saliva replacements Pilocaprine Immunosuppression
When would immunosuppression be considered in Sjogrens
As with most rhuem conditions, when there is organ involvement
Which CTD is this describing: an autoimmune condition characterised by diffuse fibrosis and vascular abnormalities
Systemic sclerosis
What is the most common cause of death with systemic sclerosis?
Pulmonary hypertension
What are the three phases of systemic sclerosis?
Odematous
Indurative
Atrophic
What clinical sign is almost always present in systemic sclerosis?
Raynauds
Which CTD is most strongly associated with calcinosis?
Systemic sclerosis
What is major criteria for systemic sclerosis?
Central skin involvement: arms, face, neck
What is minor criteria for systemic sclerosis?
Sclerodactyly and atrophy of fingertips
Bilateral lung fibrosis
What may gut involvement in systemic sclerosis lead to?
Dysphagia, malabsorption and bacterial overgrowth
What would qualify as diffuse systemic sclerosis?
Skins changes over the trunk
Early significant organ involvement
Anti-Scl-70
What would qualify as limited systemic sclerosis?
Skin involvement tends to be confined to face, hands, forearms and feet
Anti-centromere antibody
How is systemic sclerosis treated?
Pulmonary function testing
Echo
Monitoring of renal function
Autoantibody screen
How is Raynauds managed?
CCBs
Iloprost
Bosentan
How does bosentan work?
Dual endothelin receptor antagonism resulting in vasodilation
