Multi-system Autoimmune Disease (Connective tissue) Flashcards by Calum Gibson-Smith

What are the multi-system connective tissue diseases

Systemic Lupus Erythematosus

Systemic sclerosis

Sjogren’s syndrome

Auto-immune myositis

Mixed connective tissue disease

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What are mixed connective tissue diseases

Raynaud’s

Soft tissue swelling/sclerodactyly

Myositis

Arthralgia

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What are the systematic vasculitidies

Giant cell arteritis

Granulomatosis with polyangiitis (Wegeners)

Microscopic polyangiitis

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)

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What are the differential diagnosis for systematic autoimmune disease

Malignancy - lymphoma

Infections - endocarditis, hepatitis B and C, TB, HIV

Drugs - cocaine, Minocyline (mimics LE) ,
υ Propylthiouracil (anti…nasculitis)

Cardiac myxoma

Cholesterol emboli

Scurvey

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What is Systematic Lupus Erythematosus

Systemic lupus erythematosus (SLE) is a chronic disease that causes inflammation in connective tissues, such as cartilage and the lining of blood vessels, which provide strength and flexibility to structures throughout the body.

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What is epidemiology of Systematic Lupus Erythematosus

UK Prevalence: 28/100,000

UK incidence: 4/100,000

Female : Male 9:1

Onset: 15-50 years

Significant ethnic diversity:

Afro-Caribbean>Asian>Caucasian

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What is genetics of SLE

High concordance rate of SLE in monozygotic twins
Sibling risk for developing SLE is 30-fold higher than in the general population
Polygenic mode of inheritance (100 genes responsible to susceptibility)

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What is aetiology of SLE

Hormonal factors
(oestrogen increases chance)

Environmental factors
-	Ultraviolet light
-	Drugs
o	New biologics
o	Anti-viral agents 
-	Infections

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What is pathophysiology of SLE

SLE is characterised by anti-nuclear antibodies. These are antibodies to proteins within the persons own cell nucleus. This causes the immune system to target theses proteins. When the immune system is activated by these antibodies targeting proteins in the cell nucleus it generates an inflammatory response. Inflammation in the body leads to the symptoms of the condition. Usually, inflammation is a helpful response when fighting off an infection however it creates numerous problems when it occurs chronically and against the tissues of the body.

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What are presenting symptoms of SLE

Fatigue

Weight loss

Arthralgia (joint pain) and non-erosive arthritis - bilateral

Myalgia (muscle pain)

Fever

Photosensitive malar rash.

This is a “butterfly” shaped rash across the nose and cheek bones that gets worse with sunlight.

Lymphadenopathy and splenomegaly

Shortness of breath

Pleuritic chest pain

Mouth ulcers

Hair loss

Raynaud’s phenomenon

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What should you do for every patient with SLE

Check urinalysis for every patient with SLE suspicion

Look for Proteinuria, Haematuria

Lupus Nephritis is a major cause of death

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What auto antibodies are seen in SLE

Anti-Nuclear antibodies

Anti -ds DNA

Anti -Sm (Smith)

Anti–Ro

Antiphospholipid antibodies

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Describe Anti-Nuclear antibodies

Antinuclear antibodies

Seen in 95% of SLE
BUT Not specific for SLE

Seen in many inflammatory, infectious, and neoplastic diseases

Seen in 5% to 15% of healthy population

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Describe Anti -ds DNA

Seen in 60% of patients with SLE

Highly specific for SLE

Low titre rarely seen in other inflammatory conditions

Strongest clinical association is with nephritis

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Describe Anti -Sm (Smith)

Seen in 10% to 30% of SLE patients

Highly specific for SLE

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Describe Anti–Ro

Risk of foetal congenital heart block

Neonatal lupus

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Describe Antiphospholipid antibodies

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Anti-cardiolipin, lupus anticoagulant

arterial/venous thrombosis

miscarriages

What is Systematic Sclerosis

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Systemic sclerosisis a rare chronic disease of unknown cause characterized by diffuse fibrosis and vascular abnormalities in the skin, joints, and internal organs (especially the esophagus, lower gastrointestinal tract, lungs, heart, and kidneys).

Where do you get localised scleroderma

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Just fibrostic invo skin and subcutarous tissue

What are the forms of Systemic sclerosis

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Limited cutaneous systemic sclerosis (CREST)
o Skin involvement distal to the elbow and knees
o Calcinosis, Raynaulds Phenomenon, Oeseophageal dysmotility, Sclerodatly, Telangiectasia

Diffuse cutaneous systemic sclerosis

Skin involvement proximal to knees and elbows and truncal involvement

What is epidemiology of systematic sclerosis

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UK Prevalence: 24/100,000
UK Incidence: 10/1,000,000
Onset: 30-50 years
Female : Male 3:1

What is aetiology of systematic sclerosis

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Environmental
-	Silica
-	Solvents
-	Viral infections
Genetic predisposition
-	Familial clustering

What the pathogenesis of systematic sclerosis

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Very complex and the theory is not certain. Three mechanisms are responsible for the distruption it causes:

Vascular damage (microcirculation)
Immune system activation/Inflammation
Fibrosis

What antibodies are present in systematic sclerosis

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Anti centromere antibodies

Anti Scl70 antibodies

What is systematic sclerosis like with Anti centromere antibodies

Slowly progression of skin ivolvmet Milder form Very late internal organ involvement Pulmonary hypertension Gastrointestinal

What is systematic sclerosis like with Anti Scl70 antibodies

Patients fast progression skin scleoris in weeks of moths | Internal organ involvement fast and early

What are features of systematic sclerosis with Anti Scl70 antibodies

3 FEATURES Abrupt onset of hypertension Acute kidney injury with dialysis Normal urinalysis (maybe mild proteinuria) - Small bowel bacterial overgrowth o The fibrosis causes malabsorption and overgrowth o Bloating, diahhrohea, weight loss (malnutrition) o Also get water melon stomach. NEED VERY CAREFUL MONITORING FOR FIRST 5 YEARS AND THEN CAN BE REASSURED AFTER THAT’S OVER

What is Sjogren's Syndrome

This is an autoimmune condition that affects the exocrine glands. It leads to the symptoms of dry mucous membranes, such as dry mouth, dry eyes and dry vagina

What is Epidemiology of Sjogren's Syndrome

- Prevalance: 1 in 100 - Incidence: 4 in 100,000 - Onset: 40-50yrs - Female : Male 9:1

What are antibodies in Sjogrens

Anti Ro (SSA), anti La (SSB) antibodies

What is primary and Secondary Sjogrens

Primary Sjogren’s is where the condition occurs in isolation. Secondary Sjogren’s is where it occurs related to SLE or rheumatoid art

What is the test for Sjogrens

The Schirmer test involves inserting a folded piece of filter paper under the lower eyelid with a strip hanging out over the eyelid. This is left in for 5 minutes and the distance along the strip hanging out that becomes moist is measured. The tears should travel 15mm in a healthy young adult. A result of less than 10mm is significant.

What a symptoms of Sjogrens

Dry eyes and mouth Abnormal sense of taste. Burning or redness in eyes, or grittiness (like sand). Blurry vision. Difficulty chewing, swallowing or talking. 1/3 have systematic upset

What is Auto immune myositis

Polymyositis Dermatomyositis

What is Polymyositis

Polymyositis is a condition of chronic inflammation of muscles.

What is Dermatomyositis

Dermatomyositis is a connective tissue disorder where there is chronic inflammation of the skin (rash) and muscles.

What is the epidemiology of the autoimmune myositis'

Rare: 6/million incidence More common in females (ratio 3:1) Peak incidence is in 50– 60 year olds Increased risk of malignancy

What is the presentation for the autoimmune myositis'

Muscle pain, fatigue and weakness Occurs bilaterally and typically affects the proximal muscles Mostly affects the shoulder and pelvic girdle Develops over weeks

What is investigation for autoimmune myositis

The key investigation for diagnosing myositis is a creatine kinase blood test. Creatine kinase is an enzyme found inside muscle cells. Inflammation in the muscle cells (myositis) leads to the release of creatine kinase. Creatine kinase is usually less than 300 U/L. In polymyositis and dermatomyositis, the result is usually over 1000, often in the multiples of thousands.

What are Dermatomyositis Skin Features

Gottron lesions (scaly erythematous patches) on the knuckles, elbows and knees Photosensitive erythematous rash on the back, shoulders and neck Purple rash on the face and eyelids Periorbital oedema (swelling around the eyes) Subcutaneous calcinosis (calcium deposits in the subcutaneous tissue)

What is the treatment for autoimmune myositis

Corticosteroids are the first line treatment of both conditions.

Multi-system Autoimmune Disease (Connective tissue) Flashcards

(41 cards)