connective tissue disorders Flashcards
what is connective tissue?
tissue that connects, supports binds or separates other tissues or organs
what are examples of connective tissues?
-nervous tissue
-muscle tissue
-epithelial tissue
what are 3 types of connective tissue disease?
-systemic sclerosis/ scleroderma
-systemic lupus erythematosus
-dermatomyositis and polymyositis
describe the epidemiology of scleroderma
- hard thickened areas of skin, can also cause problems with internal organs etc
-autoimmune condition
-uncommon connective tissue disorder
-more common in females
-age 30-50
-HLA (human leukocyte antigens- DR5 marker)
describe the aetiology of scleroderma
- cause is not known
-there can be an event that causes cells to start producing collagen as if injury has occured
-excess collagen formation
-can affect skin, joints and internal organs
what are the 2 subtypes of systemic scleroderma
-diffuse scleroderma
-limited cutaneous scleroderma
describe diffuse scleroderma
-skin thickening proximal to elbows and knees
-early internal organ involvement
describe limited cutaneous scleroderma
-skin thickening limited to below the elbow
-later involvement of internal organs
describe clinical features of scleroderma
-initial changes in hands, face and feet
-hand swelling initially
-sclerodactyly (ulceration of skin of distal digits)
-raynauds (common early symptom)
-fibrosis - thickening of skin, subcutaneous swelling and contractures
what is localised scleroderma
-localised skin changes
-internal organs not affected
what are the clinical features of the skin of a patient with scleroderma
-dry shiny hairless coarse skin
-mouth smaller and tight & nose pointed
-loss of wrinkles of facial expression
-hand deformity
what are the MSK clinical features of scleroderma?
-joint pain
-stiffness
-limited ROM
-fibrosis skin and muscle
-weakness
-synovitis
what are vascular clinical features of scleroderma
-capillary changes
-raynauds syndrome
-numbness and pain in hand and feet
what are the most common organs affected in scleroderma?
lungs - dry cough, SOB, fibrosis in later stages
-GI - bloating,nausea, vomiting etc
-cardiac - often later in disease
-renal- eg renal failure due to hypertension
how is scleroderma diagnosed?
-no definite blood tests - check for absence of Rh F, ACA, elevated C reactive protein
-pulmonary function tests
-chest x ray
-echocardiograms
-renal function- for organs
how is scleroderma managed pharmacologically?
-corticosteroids
-immune suppressing medications such as MTX
-NSAIDS
-UV therapy for skin
-medications for BP management
-medications for raynauds eg Ca2+ channel blockers eg diltiazem
in terms of a physiotherapy assessment, what are important things to look out for with scleroderma?
-observation - skin changes, deformity, postural changes
-ROM- reduced ROM, contracture
-muscle strength- weakness
-lung function - reduced exercise tolerance etc
-general/ functional mobility - reduced function and ADLs