Multisystem Autoimmune Disorders Flashcards
Presentation of SLE (7)
Joint pain and stiffness Extreme tiredness Skin rashes (butterfly) Weight loss Swollen glands Sensitivity to light Raynaud's
Classification criteria for SLE (11)
- Malar rash
- Discoid rash
- Photosensitivity
- Oral ulcers
- Arthritis (>2)
- Serositis
- Proteinuria / cellular casts in urine
- Seizures or psychosis
- Haem - low WCC, platelets, lymphocytes, anaemia
- Immunological - anti ds-DNA, lupus anticoagulant, cardiolupin, low complement
- ANA test
what is the characteristic sign of scleroderma
Thickening and reddening of skin (morphea)
Types of scleroderma
- Localised - just affects skin (morphea or linear skin changes)
- Systemic - limited cutaneous systemic sclerosis and diffuse cutaneous systemic sclerosis
What is morphea
Discoloured oval patches on skin
Usually itchy
Can appear anywhere on body
Hairless and shiny
What is linear localised scleroderma
Thickened skin in lines along face, scalp, legs or arms
What is limited cutaneous systemic sclerosis
Milder form which only affects skin on the hands, lower arms, feet, lower legs and face - can eventually affect lungs and GIT
CREST Syndrome
What does CREST stand for
Calcium deposits Raynaud's Esophageal reflux Sclerodactyly Telangiectasia
What is diffuse cutaneous systemic sclerosis
Scleroderma more likely to affect internal organs, skin changes can affect whole body
Involves other symptoms which come on suddenly - weight loss, fatigue, joint pain and stiffness
Complications of limited scleroderma
Pulmonary HTN (small blood vessels in lungs become fibrosed)
Complications of diffuse scleroderma
Pulmonary fibrosis
Renal crisis
Small bowel bacterial overgrowth
What age group does scleroderma occur in
30-50
Peak onset of Sjögrens
40-50
Symptoms of Sjögrens
Dry mouth and lacrimal glands Fatigue Fever Myalgia Arthralgia
Complications of Sjögrens
Lymphoma Neuropathy Purpura Interstitial lung disease Renal tubular acidosis
Types of autoimmune myositis
Polymyositis - affects many muscles
Dermatomyositis - affects muscles and causes a rash
Signs of dermatomyositis
Gottron’s Papules - discrete red areas over knuckles
Heliotrope rash - red or purplish rash occurring around eyes, limbs or trunk
Complications of autoimmune myositis
Cancer
Interstitial lung disease
Giant cell arteritis classification criteria
>50 years at onset New headache Temporal artery tenderness / reduced pulsation ESR>50 Abnormal temporal biopsy
What are the different types of ANCA associated vasculitis
- Granulomatosis with polyangitis (Wegener’s)
- Microscopic polyangitis
- Eosinophilic granulomatosis with polyangitis (Churg-Strauss)
What is granulomatosis with polyangitis
Where does it affect in the body
Necrotising granulomatous inflammation
Upper and lower respiratory tract, affecting mostly small to medium vessels
What is microscopic polyangitis
Necrotising vasculitis with few or no immune deposits
Mostly affects small vessels
Pulmonary capillarities common
NO Granulomatous inflammation
What condition is common in granulomatosis with polyangitis and microscopic polyangitis
Necrotising glomerulonephritis
What is eosinophilic granulomatosis with polyangitis
What conditions is it associated with
Eosinophil rich and necrotising granulomatous inflammation often involving small to medium vessels in the respiratory tract
Associated with asthma and eosinophilia
What makes ANCA more frequent in eosinophilic granulomatosis with polyangitis
When glomerulonephritis is present
What is ANCA
Anti-neutrophil cytoplasmic antibody (an abnormal antibody in the blood) that attack proteins within neutrophils
Antibodies positive in SLE
dsDNA antibodies
Smith antibodies
anti-nuclear antibodies (Ro)
Antibodies positive in scleroderma
Scl-70
Centromere
Antibodies positive in polymyositis
anti-Jo-1
Antibodies positive in Sjögrens disease
Anti-nuclear antibodies (Ro)
Anti-La
How is lupus nephritis classed
I - minimal mesangial II - mesangial proliferative III - focal IV - diffuse V - membranous VI - advanced sclerosing
Treatment for mild organ threat in multi system autoimmune diseases
Hydroxychloroquine
Treatment for moderate organ threat in multi system autoimmune diseases
Azathiopurine
Methotrexate
Mycophenolate
Treatment for severe organ threat in multi system autoimmune diseases
Cyclophosphamide
Rituximab
