Systemic Autoimmune Conditions Flashcards
What are some connective tissue diseases?
SLE
Systemic sclerosis
Sjogren’s syndrome
What is SLE?
Systemic Lupus Erythematous
Non specific symptoms
Relapsing-remitting course
Chronic inflammation
What is the pathophysiology of SLE?
Anti nuclear antibodies (ANA)- antibodies against self
Immune complex formation
Complement activation
Tissue injury
What is the presentation of SLE?
Malar rash
Discoid rash
Photosensitivity
Oral ulcers
Arthritis
Pleuritis
Renal- proteinuria, haematuria
Neurological- seizures, psychosis
Haematological- cytopenia
Immunological- autoantibodies
Myalgia, Arthralgia
What are the investigations for SLE?
Autoantibodies
FBC
C3 and C4
CRP and ESR
Immunoglobulins
Urinalysis
Renal biopsy- lupus nephritis
What autoantibodies are associated with SLE?
ANA
anti- dsDNA
anti-Smith
anti-phospholipid
What are SLE complications?
CVD
Infection
Anaemia of chronic disease
Pleuritis/pericarditis
Interstitial lung disease
Lupus nephritis
What is SLE treatment?
NSAIDs
Steroids
Hydroxychloroquine
Methotrexate
Biologics- rituximab
What is systemic sclerosis?
Autoimmune inflammatory and fibrotic connective tissue disorder
What are the 2 main patterns of disease in systemic sclerosis?
Limited cutaneous SSc
Diffuse cutaneous SSc
What is limited cutaneous SSc?
More limited version
CREST syndrome
What does CREST stand for in SSc?
Calcinosis
Raynaud’s
Esophageal dysmotility
Sclerodactyly
Telangiectasia
What is diffuse cutaneous systemic sclerosis?
Features of CREST syndrome plus internal organ damage
Cardio- CAD, HTN
Lungs- pulmonary HTN, pulmonary fibrosis
Kidney problems- golmerulonephritis
What is scleroderma?
Hardening of the skin
What is sclerodactyly?
Skin changes in the hands- breaks and ulcerates as it tightens
What is telangiectasia?
Dilated small blood vessels in the skin
What is calcinosis?
Calcium deposits under the skin
What is Raynaud’s?
Fingertips go white then blue due to vasoconstriction of finger vessels
What autoantibody is associated with limited cutaneous SSc?
Anti-centromere antibodies
What autoantibody is associated with diffuse cutaneous systemic sclerosis?
Anti Scl-70 antibodies
What is the management of SSc?
Steroids
Immunosuppressants
Physio and OT
Avoid cold triggers for Raynaud’s
Medical management for organ damage
What is Sjogren’s syndrome?
Autoimmune condition affecting the exocrine glands
Primary or Secondary to SLE or RA
What are the features of Sjogren’s?
Dry mucous membranes- eyes, mouth, vagina
What antibodies are associated with Sjogren’s?
anti-RO
anti-LO
What is the test for Sjogren’s?
Schirmer test- filter paper inserted under lower eyelid for 5 mins
Tears should travel 15mm
What is the management of Sjogren’s?
Artificial tears
Artificial salvia
Vaginal lubricants
Hydrochloroquine
What types of vasculitis affect the large vessels?
Giant cell arteritis
Takayasu’s arteritis
What types of vasculitis affect the small vessels?
Eosinophilic Granulomatosis with Polyangiitis
Microscopic polyangiitis
Granulomatosis with polyangiitis
What is the name for Eosinophilic Granulomatosis with Polyangiitis?
Churg-Strauss syndrome
What is the name for granulomatosis with polyangiitis?
Wegener’s
What are generic features of vasculitis?
Purpura
Joint and muscle pain
Peripheral neuropathy
Renal impairment
GI disturbance
Anterior uveitis
HTN
Systemic symptoms
What blood tests are done for vasculitis?
CRP and ESR
p-ANCA and c-ANCA
What are the features of GCA?
Most common
- Age>50
- New headache
- Temporal artery tenderness/reduced pulsation
- ESR>50
- Abnormal temporal biopsy
- Jaw claudication
Responds immediately to prednisolone (40-60mg per day)
What are the features of Eosinophilic granulomatosis with polyangiitis?
Elevated eosinophils
Late onset asthma
Nasal polyps
pANCA
What are the features of microscopic polyangiitis?
Renal failure
Pulmonary involvement
No granulomatous inflammation
p-ANCA
What are the features of granulomatosis with polyangiitis?
Resp. tract- cough, wheeze, haemoptysis
Kidneys- glomerulonephritis
cANCA
