Multisystem autoimmune disease Flashcards
Types of Autoimmune Multisystem Diseases
Connective tissue disease
Systemic vasculitis
Examples of connective tissue disease
SLE Scleroderma Sjogren's Autoimmune Myositis Mixed connective disease
Examples of systemic vasculitides
Giant cell arteritis
Granulomatosis with polyangiitis/Wegener’s Syndrome
Microscopic polyangiitis
Eosinophilic granulomatosis with polyangiitis/Churg - Strauss syndrome
Diagnosis of autoimmune multisystem disease
(hot, red, swelling, painful)
Oral mucosa and ocular changes
Anti-nuclear Antibody test (ANA)
PET and Doppler scan, CXR for lung changes
What is SLE?
A chronic AI disease affecting any organ system.
+ve ANA test
+ve Anti double strand DNA antibody
Dysregulated immune pathways
Related to environmental insult in genetically susceptible patient
Outcomes for SLE?
Constitutional symptoms - skin and joint involvement
Recurrent flare ups lead to progressive disease
Early diagnosis and management lowers this risk
Treatment for SLE?
Mild, Moderate, Severe
Mild: Hydroxychloroquine
Moderate: DMARD and immunosuppression
- methotrexate and azathioprine when showing serositis/arthritis
- mycophenolate for renal involvement
Severe (neurological symptoms): Cyclophosphamide or rituximab
Presentation of SLE?
- Malar or discoid rash + Oral ulcers
- Polyarthritis, Serositis (pleural/pericardial inflammation +/- effusion)
- Seizures/psychosis
- Photosensitivity
- Raynaud’s
- Low WBC, thrombocytopenia, lymphocytopenia, haemolytic anaemia, Proteinuria
Investigations for suspected lupus?
FBC: neutropenia, thrombocytopenia, normochromaic/hypochromic normocytic anaemia (anaemia of chronic disease or haemolytic)
ESR raised, normal CRP
Renal function, Us+Es, Dipstick, ANA, ANCA, Anti-ds-DNA antibody (vasculitis)
Signs of general vasculitis?
Hand lesions, Splinter haemorrhages, Nail bed infarcts
What is giant cell arteritis?
Vasculitis of large and medium vessels (cranial vessels arising from aortic arch)
Granulomatous inflammation association A.k.a. temporal arteritis
Presentation of Giant Cell Arteritis?
> 50 y/o
New localised headache
Temporal artery tenderness/reduced pulsation
Jaw claudication
Visual abnormalities
Abnormal temporal biopsy (granulomatous inflammation, multinucleate giant cells)
50 ESR
What do you see in Granulomatous with polyangiitis? (Wegener’s)
Necrotising granulomatous inflammation
Upper + Lower Respiratory tract small + medium vessels inflamed
Associated with ANCA
Often with necrotising glomerulonephritis
What is seen in microscopic polyangiitis?
- NO granulomatous inflammation
- Necrotising arteritis of small and medium arteries
- Necrotising vasculitis of small vessels
(Often with necrotising glomerulonephritis, KIDNEY) - Pulmonary small vessel inflammation (LUNGS)
- Associated with ANCA
Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss)
- Necrotising granulomatous inflammation
- Involves upper and lower respiratory tract (LUNGS)
- High eosinophil levels (Associated with asthma )
- ANCA (likely if glomerulonephritis is present)
- Glomerulonephritis (KIDNEY)