Multi-system autoimmune disease Flashcards
What are the different classes of autoimmune disease?
Connective tissue diseases Systemic lupus Erythematous Scleroderma Sjorgen's syndrome Auto-immune myositis Mixed connective tissue disease
Systemic vascultits Giant cell arteritis Granulmatosis polyangitis Microscopic polyangitis Eosinophillic granulomatosis polyangitis
What is the process for diagnosing all patients with different autoimmune diseases?
History and exam Immunology Imaging Tissue Exclusion of differential diagnosis
What are some of the things that mimic autoimmune disease
Cocaine, minoculine, PTU Infection- HIV, endocarditis hepatitis, TB Malignancy, lymphoma Cardiac myxoma Cholesterol emboli Scurvey
What is sytemic lupus erythema
Who gets SLE?
Autoimmue disease where the body attacks healthy tissue
28/100,000
more common in females
onset 15-50 years
More likely in afro-caribbeans
What are the criteria for diagnosing Lupus?
Malar rash Discoid rash photosensitivity Oral ulcers Arthritis Serositis Renal Neurological (unexplained seizures or psychosis) Heamatologica (low WCC,platelets, lymphocytes) Immunological ANA
What is scleroderma
Disease of the connective tissue that can be categorised into diffuse and limited
30-50 years, more common in females
What symptoms occur in scleroderma limited?
What complications can arise from limited scleroderma?
Milder form that typically effects the skin and hands, lower arms, feet, lower legs and face. Typically starts as Raynaud’s. Can also include thickening of skin over hands, feet, face, red sports on skin, hard lumps under skin, heartburn and dysphagia
Pulmonary hypertension
What occurs in diffuse scleroderma?
What complications can occur?
More likely to effect internal orgas.
Skin changes can affect the whole body
Weight loss, fatigue and joint pain and stiffness
pulmonary fibrosis
Renal crisis
Small bowel bacterial overgrowth
What is sjogrens syndrome?
Who gets it?
Autoimmune disease that attacks glands
40-50 years
male over female
What are the typical symtpoms of Sjorgens syndrome?
What are the complications that can arise?
Dry eyes and mouth
Parotid gland enlargement
1/3 have systemic upset
Lymphoma, nerop\thy, purpura, interstitial lung disease, Renal tubular acidosis
What is auto-immune myositis?
Who gets auto-immune myosistis
Group of autoimmune rheumatic disorders that cause inflammation and weakness in the skin and muscles
Very rare, hardly anyone
What are the symptoms of auto-immune myositis
What are the complications of auto-immune myositis?
Polymyositis
Dermatomyositis
Gottron’s papules
Heliotrope rash
Cancer
Interstitial lung disease
What are the different classes of vasular autoimmune disease?
Large vessel vascculitis- takayasu arteritis
giant cell arteritis
Medium vessel vasculitis- polyarteritis nodosa
Kawasaki disease
Small vessel vasculitis- ANCA associated vasculitis
Immune complex SVV
What is giant cell arteritis
What are part of the criteria needed to diagnose it?
Inflmmatory disease affecting the large blood vessels of the scalp, neck and arms causing a narrowing of blood vessels.
Onset> 50 years new headache temporal artery tenderness, reduced pulsation ESR>50 Abnormal temporal biopsy
What is ANCA associated vasculitis
3 different of conditions affecting the body which can be grouped together by the fact they cause inflammation and destruction of small blood vessels
Granulomatosis with polyangitis (Wegener’s)
Microscopic polyangitis
Esinophillic granulomatosis with polyangitis
What are the symptoms of Wegeners syndrome
Necrotiing ganulomatous inflammation
Usually involving the upper and lower respiratory tract
Affecting predominantly small to medium vessels
Necrotising glomerulonephritis is common
What are the symptoms of microscopic vasculitis
Necrotising vasculitis with few or no immune deposits, predominantly affecting the small vessels
Necrotising arteritis involving small and medium vessels may be present
Necrotising glomerulonephritis is very common
Pulmonary capilaritis often occurs
Granulomatous inflammation is absent
What are symptoms of eosinophilic granulomatosis with polyangitis
Eosinophil rich and necrotising granulomatous inflammation often involving the respiratory tract
Necrotising vasculitis predominantly affects small to medium vessels. Associated with asthma and eosinophilia
ANCA is more frequent when glomerulonephritis is present
What are the ANA specific targets of
SLE
Scleroderma
Polymyositis
Sjorgen’s disease
ANA means antinuclear antibody tests and is used to help evaluate a person for autoimmune disease
SLE- dsDNA, RO,SM
Scleroderma-SCL-70, cnetromere
Polymyositis-JO-1
Sjorgen’s disease-Ro,La
What are the classes of lupus nephritis
Class I- Minimal mesangial Class II- Mesangial proliferative Class III- Focal ClassIV- diffuse Class V- membranous Class VI- advanced sclerosing
What are the broad treatments for autoimmune conditions?
Mild- hydroxycloroquine
Moderate- Azathioprine, methotrexate,mycophenolate
Severe- Cyclophosphamide, Rituximab
