Multi-system autoimmune disease Flashcards

1
Q

What are the different classes of autoimmune disease?

A
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
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2
Q

What is the process for diagnosing all patients with different autoimmune diseases?

A
History and exam
Immunology
Imaging
Tissue
Exclusion of differential diagnosis
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3
Q

What are some of the things that mimic autoimmune disease

A
Cocaine, minoculine, PTU
Infection- HIV, endocarditis hepatitis, TB
Malignancy, lymphoma
Cardiac myxoma
Cholesterol emboli
Scurvey
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4
Q

What is sytemic lupus erythema

Who gets SLE?

A

Autoimmue disease where the body attacks healthy tissue

28/100,000
more common in females
onset 15-50 years
More likely in afro-caribbeans

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5
Q

What are the criteria for diagnosing Lupus?

A
Malar rash
Discoid rash 
photosensitivity
Oral ulcers
Arthritis
Serositis
Renal
Neurological (unexplained seizures or psychosis)
Heamatologica (low WCC,platelets, lymphocytes)
Immunological
ANA
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6
Q

What is scleroderma

A

Disease of the connective tissue that can be categorised into diffuse and limited

30-50 years, more common in females

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7
Q

What symptoms occur in scleroderma limited?

What complications can arise from limited scleroderma?

A

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

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8
Q

What occurs in diffuse scleroderma?

What complications can occur?

A

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

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9
Q

What is sjogrens syndrome?

Who gets it?

A

Autoimmune disease that attacks glands

40-50 years
male over female

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10
Q

What are the typical symtpoms of Sjorgens syndrome?

What are the complications that can arise?

A

Dry eyes and mouth
Parotid gland enlargement
1/3 have systemic upset

Lymphoma, nerop\thy, purpura, interstitial lung disease, Renal tubular acidosis

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11
Q

What is auto-immune myositis?

Who gets auto-immune myosistis

A

Group of autoimmune rheumatic disorders that cause inflammation and weakness in the skin and muscles

Very rare, hardly anyone

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12
Q

What are the symptoms of auto-immune myositis

What are the complications of auto-immune myositis?

A

Polymyositis
Dermatomyositis
Gottron’s papules
Heliotrope rash

Cancer
Interstitial lung disease

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13
Q

What are the different classes of vasular autoimmune disease?

A

Large vessel vascculitis- takayasu arteritis
giant cell arteritis

Medium vessel vasculitis- polyarteritis nodosa
Kawasaki disease

Small vessel vasculitis- ANCA associated vasculitis
Immune complex SVV

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14
Q

What is giant cell arteritis

What are part of the criteria needed to diagnose it?

A

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
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15
Q

What is ANCA associated vasculitis

A

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

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16
Q

What are the symptoms of Wegeners syndrome

A

Necrotiing ganulomatous inflammation
Usually involving the upper and lower respiratory tract
Affecting predominantly small to medium vessels
Necrotising glomerulonephritis is common

17
Q

What are the symptoms of microscopic vasculitis

A

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

18
Q

What are symptoms of eosinophilic granulomatosis with polyangitis

A

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

19
Q

What are the ANA specific targets of

SLE
Scleroderma
Polymyositis
Sjorgen’s disease

A

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

20
Q

What are the classes of lupus nephritis

A
Class I- Minimal mesangial
Class II- Mesangial proliferative
Class III- Focal
ClassIV- diffuse
Class V- membranous
Class VI- advanced sclerosing
21
Q

What are the broad treatments for autoimmune conditions?

A

Mild- hydroxycloroquine
Moderate- Azathioprine, methotrexate,mycophenolate
Severe- Cyclophosphamide, Rituximab