Autoimmune CTDs Flashcards
What are the connective tissue diseases?
A heterogenous group of non organ specific autoimmune diseases. The CTDs are unified by the presence of autoantibodies in the serum.
Name four examples of autoimmune CTDs
Any four from:
Systemic Lupus Erythematosus Sjögren's syndrome Polymyositis Dermatomyositis Scleroderma
Questions to ask in a CTD history?
Have you had any joint or muscle pains?
Have you had any rashes recently?
Have you been suffering from mouth or genital ulcers?
Have you had any hair loss?
Do you suffer from dry eyes or a dry mouth?
Do your fingers change colour in the cold?
Have you become breathless on exertion?
Do you have any difficulty in swallowing?
Have you ever suffered with blood clots?
Have you had any miscarriages?
Is there any family history of rheumatic conditions?
Have you taken any medications, prescribed, herbal, or otherwise recently?
What the history tells you
Rashes might suggest SLE or dermatomyositis, whilst mucosal ulceration, hair loss, and Raynaud’s phenomenon are features common to MOST connective tissue diseases.
Breathlessness and difficulty in swallowing would suggest scleroderma, whilst recurrent DVTs, pulmonary emboli and miscarriages occur in antiphospholipid syndrome.
What is Sjögren’s syndrome?
Sjögren’s syndrome is a condition in which there is lymphocytic infiltration of exocrine glands, producing the main symptoms of xerophthalmia (dry eyes), xerostomia (dry mouth) and enlargement of the parotid glands.
What is SLE? (Systemic lupus erythematosus)
Systemic lupus erythematosus (SLE) is a clinical syndrome characterised by autoimmune mediated systemic inflammation that encompasses a wide range of clinical manifestations. Symptoms include: joint pain, skin rash, fatigue, fever, weight loss, headaches, mouth ulcers, hair loss, swelling of lymph glands, Raynaud’s phenomenon.
Internal organ manifestations can occur in both limited cutaneous and diffuse cutaneous systemic sclerosis (scleroderma). What are the auto-antibodies, found in both subsets, which are associated with:
1) Lung fibrosis?
2) Renal crisis?
1) anti-topoisomerase 1 auto-antibodies
2) anti-RNA polymerase III autoantibodies
Possession of which Major Histocompatibility Complex (MHC) antigens confers an increased susceptibility to SLE?
HLA-DR2 and DR3,
Which auto-antibodies are associated with SLE?
anti-dsDNA, anti-Ro and anti-La
What is Jaccoud’s arthropathy?
Reversible subluxation of joints without erosive disease. Occurs in up to 50% of SLE patients
What drug can be used to treat dry mouth in sjogrens syndrome? What are potential side effects?
Pilocarpine. (5mg QDS). Excessive sweating, nausea, diarrhoea, vomiting, tremor, palpitations.
What is the link between sjogrens and malignancy?
Patients with Sjögren’s Syndrome have a 40-fold relative risk of developing a lymphoid malignancy compared with age, sex, and race matched controls. Usually of B-cell origin.
Name two types of large vessel vasculitis
Giant cell arteritis and Takayasu’s arteritis (NB, Large Gin and Tonic)
Name two types of medium vessel vasculitis
Polyarteritis nodosa and Kawasaki disease (NB, medium passion fruit and kiwi)
Small vessel vasculitidies are divided into what two main categories?
ANCA +ve and ANCA -ve (ANCA = anti-neutrophil cytoplasmic antibody)
