Systemic autoimmune diseases Flashcards
What multisystem diseases affect connective tissue?
Systemic Lupus Erythematosus Scleroderma Sjogren's syndrome Auto-immune myositis Mixed connective tissue disease
What are the 3 main categories of systemic vasculitis?
Large vessel disease
Medium vessel disease
Small vessel disease
What is used to diagnose multisystem autoimmune conditions?
History/exam Immunology Imaging Tissue biopsy Exclusion of differential diagnosis
What can mimic systemic autoimmune disease?
Drugs - cocaine
Infection - HIV, endocarditis, hepatitis
Malignancy - lymphoma
What is the male:female ratio for systemic lupus erythematosus?
1:9 - much more common in females
What is the age of onset of systemic lupus erythematosus?
15-50 years
How many of the lupus classification criteria is needed to make a diagnosis?
4
What are the 11 classification criteria for systemic lupus eryhtematosus?
Malar rash Discoid rash Photosensitivity Oral ulcers Arthritis of 2+ joints Serositis Significant proteinuria - renal Unexplained seizures - neurological Low WCC, platelets, haemolytic anaemia - haematological Immunological Antinuclear antibodies
What is a discoid rash?
Raised, scarring, permanent marks
What is scleroderma?
Collection of autoimmune diseases affecting the skin, blood vessels, and internal organs
What is the male:female ratio of scleroderma?
1:3
What is the age of onset of scleroderma?
30-50 years
What is sclerodermal morphea?
Thickening and hardening of skin and subcutaneous tissue due to collagen deposition
What is the main complication of limited scleroderma?
Pulmonary hypertension
What are complications of diffuse scleroderma?
Pulmonary fibrosis
Renal crisis
Small bowel bacteria overgrowth
What is Sjogren’s syndrome?
Inflammation of moisture producing glands
Is Sjogren’s more common in men or women?
Women
What are features of Sjogren’s syndrome?
Dry eyes and mouth
Parotid gland enlargement
1/3 have systemic upset - fatigue, fever, myalgia, arthalgia
What are complications of Sjogren’s syndrome?
Lymphoma Neuropathy Purpura Interstitial lung disease Renal tubular acidosis
What is the main symptom of auto-immune myositis?
Muscle weakness
What are complications of auto-immune myositis?
Cancer
Interstitial lung disease
What are forms of large cell vasculitis?
Takayasu arteritis
Giant cell arteritis
What are forms of medium vessel vasculitis?
Polyarteritis nodosa
Kawasaki disease
What are forms of ANCA associated small vessel vasculitis?
Microscopic polyangiitis
Granulomatosis with polyangiitis
Eosinophillic granulomatosis with polyangiitis
How many of the giant cell arteritis criteria must be met to make a diagnosis?
3
What are the giant cell arteritis criteria?
Age at onset>50 New headache Temporal artery tenderness/reduced pulsation ESR<50 Abnormal temporal biopsy
What does ANCA stand for?
Anti-neutrophil cytoplasmic antibody
What are features of granulomatosis with polyangiitis?
Necrotising granulomatous inflammation
Usually involves upper and lower resp tract
Affecting predominantly small vessels
Necrotising glomerulonephritis is common
What are features of microscopic polyangiitis?
Necrotising vasculitis with few immune deposits affecting small vessels
Necrotising glomerulonephritis is common
Pulmonary capillaritis is common
Granulomatous inflammation is absent
What are features of eosinophilic granulomatosis with polyangiitis?
Eosinophil rich and necrotising granulomatous inflammation often involving the respiratory tract
Necrotising vasculitis affecting small vessels
Associated with asthma and eosinophilia
ANCA more frequent with glomerulonephritis
How is mild multi-system autoimmune disease treated?
Hydroxylchloroquine
How is moderate multi-system autoimmune disease treated?
Azathioprine, methotrexate, mycophenolate
How is severe multi-system autoimmune disease treated?
Cyclophosphamide, rituximab
What are the CREST features of scleroderma?
Calcinosis Raynauds oEsophageal dismotility Sclerodactyly Telangiectasia
