connective tissue autoimmune disease Flashcards
who normally gets SLE
white women pre menopause
what exacerbates lupus
smoking, dust, UV light
what is the pathogenesis of lupus
loss of immune regulation, apoptosis, dead cells release materials seen as auto-antigens, auto-antibodies formed, necrosis and scarring
what systems does SLE commonly affect (7)
cutaneous, arthritis, neurological, blood, lungs, heart, kidneys
what is a distinctive cutaneous symptom of lupus
butterfly malar rash on face
what are cutaneous SLE symptoms (5)
malar rash, discoid rash, non-scarring alopecia, oral ulcers, photosensitive
what symptoms of arthritis are common in lupus
synovitis/ tenderness in 2+ joints with >30mins morning stiffness
what neurological symptoms are common in SLE
delirium, psychosis, seizure, headaches
what are common heart/ lung symptoms of SLE
pleural/ pericardial effusion, pericarditis, Interstitial lung disease
what are common haemotological symptoms of SLE
leukopenia, thrombocytopenia, haemolytic anaemia
what renal symptoms are seen in SLE
proteinurea, blood in urine, nephritis
what autoantibodies are seen in SLE
Anti DsNA, ANA (anti SM)
how do you diagnose SLE
1/11 SLICC criteria - at leasr one lab
what treatment is given to all lupus patients
sun protection, hydroxychloroquine, steroids, NSAIDs
what treatment is given to severe organ involvement lupus patients
cyclophosphamide, IV steroids
what treatment is given to ALP positive lupus patients
antiplatelets, anticoag
what is sjorgen’s disease
autoimmune causing lack of secretions in eyes (lacrimal), mouth (salivary) and vagina
what is the pathogenesis of sjorgen’s and an associated risk
lymphocytic infiltration of exocrine organs - lymphoma
what are the symptoms of sjorgen’s (5)
dry/ gritty eyes (keratoconjunctivitis sicca), dry mouth/ vagina, bilateral parotid swelling, fatigue, dental deterioration
what test is done for Sjorgen’s dry eyes
Schirmer’s test
what auto-antibodies are present in sjorgen’s
Anti - ro, Anti - La (ANA)
what immunoglobin can be found in sjorgens
IgG
how do you treat sjorgen’s (mild –> severe)
pilocarpine (saliva replacement), hydroxychloroquine, cyclophosamide
what is systemic sclerosis/ scleroderma
multi-systemic autoimmune disease where immune system becomes over active
what causes scarring in systemic sclerosis
too much collagen
what is limited cutaneous involvement of systemic sclerosis
extremities below elbows and knees +/- face
what is diffuse cutaneous involvement of systemic sclerosis
extremities above AND below elbows and knees AND face AND trunk
what is the main cause of death in systemic sclerosis
pulmonary hypertension
what are the symptoms of systemic sclerosis (8)
CREST: calcinosis (calcium deposits), reynauds, oesophageal dysfunction, sclerodacytlyly, telangiectasia. digital ischaemia, GORD, SOB
what is Reynaud’s
blanching/ whitness of digits and acrocyanosis - sensitive to cold
how do you manage reynaud’s
CCB’s, PDE5 inhibitor
what face changes can be seen in Reynauds
small puckered mouth, beaked nose, skin tightening, telangiectasia
what GI symptoms can be seen in Reynauds
calcinosis, small intestinal bacterial overgrowth, malabsorption, incontinence
what cardio/ resp symptoms can be seen in Reynauds
interstitial lung disease eg FIBROSIS, pulm hypertension, myocardial disease
what is used to treat scleroderma renal crisis
ACEi
what auto-antibodies can be seen in systemic sclerosis
Anti-scl-70, ANA , anticentromere
what imaging can be done i systemic sclerosis
C Xray, echo
what treatments are used for systemic sclerosis
CCB’s, ACEi (if renal), cyclophosphamide (no steroids)
what is anti-phospholipid syndrome often secondary to
lupus
what are the symptoms of anti-phospholipid syndrome
recurrent venous/ arterial thrombosis, recurrent miscarriages, superficial thrombophelbitis, migraines, livedo reticularis
what are the increased risks with anti-phospholipid syndrome
stroke, MI, PE
how do you diagnose anti-phospholipid syndrome
+ive on 2 occasions 12 weeks apart: anti-cardiolipin, lupus anti-coag test, anti-B2 glycoprotein
