1b Lupus - Other Connective Tissue Problems Flashcards
What is Sjogren’s syndrome?
Autoimmune exocrinopathy - lymphocytic infiltration of especially exocrine glands and sometimes other organs
What does exocrine gland pathology result in in Sjogrens syndrome?
Dry eyes (xerophthalmia)
Dry mouth (xerostomia)
Parotid gland enlargement
What will a salivary gland biopsy show in Sjogrens syndrome?
Salivary gland biopsy will show lymphocytic infiltration predominantly CD4 helper T cells and to lesser extent B lymphocytes
What is inflammatory muscle disease?
Proximal muscle weakness due to autoimmune-mediated inflammation either with (dermatomyositis) or without (polymyositis) a rash
Skin changes with polymyositis
What are the skin changes seen in inflammatory muscle disease?
Lilac-coloured (heliotrope) rash on eyelids, malar region and naso-labial folds
Red or purple flat or raised lesions on knuckles (Gottron’s papules)
Subcutaneous calcinosis
Mechanic’s hands (fissuring and cracking of skin over finger pads)
What biochemical tests are sen in inflammatory muscle disease?
Elevated CPK, abnormal electromyography, abnormal muscle biopsy (polymyositis = CD8 T cells; dermatomyositis = CD4 T cells in addition to B cells)
What are the key manifestations of systemic sclerosis?
thickened skin with raynaud’s phenomenon
What are the changes seen in systemic sclerosis?
Thickened skin with Raynaud’s phenomenon
Dermal fibrosis, cutaneous calcinosis and telangiectasia
What is CREST?
CREST describes a sub-type of limited systemic sclerosis.
It stands for Calcinosis, Raynaud’s phenomenon, Esophageal dysmotility, Sclerodactyly, Telangiectasia
What is overlap syndrome?
When features of more than 1 connective tissue disorder are present e.g. SLE and inflammatory muscle disease we can use the term overlap syndrome
what antibody confirms the presence of Mixed Connective tissue disease?
Anti-U1-RNP antibody*
