Autoimmune Diseases Flashcards by Rebecca Macinnes

List connective tissue diseases

Systemic Lupus Erythematosus
Scleroderma
Sjogren’s syndrome
Auto-immune myositis
Mixed connective tissue disease

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List systemic vasculitis diseases

Giant cell arteritis
Granulomatosis polyangitis
Microscopic polyangitis
Eosinophilic granulomatosis polyangitis

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How can autoimmune diseases be diagnosed

History and examination, immunology tests, imaging, tissue samples

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State the drugs that can cause similar symptoms to that of autoimmune disease

Cocaine (can cause microvasculitis), minocycline (drug induced systemic lupus), Propylthiouracil (used to treat hyperthyroidism)

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Systemic lupus tends to effect what age group

The young, 50% diagnosed at around 16-50

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Prevalence of systemic lupus

28/100,000

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Incidence of systemic lupus

4/100,000

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Female to male ration systemic lupus

9:1

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Systemic lupus is more common in what race

Caribbean afro

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Face symptoms of systemic lupus

Butterfly rash and photo-sensitivity

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Nervous system features of systemic lupus

Seizures, hemiplegia, ataxia, peripheral neuropathy, cranial nerve lesions

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Cardiac features of lupus

Pericarditis, endocarditis, aortic valve lesions

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Blood features of lupus

Anaemia (nomochromic normocytic Coombs test +), leukopenia, thrombocytopenia

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Joint features of lupis

Aseptic necrosis of the hip, arthritis in small joints

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Chest features of lupus

Pleurisy, pleural effusion, fibrosis

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Skin changes in lupus

Vasculitis, purpura, urticaria

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General features of lupus

Fever, depression, alopecia, abdominal pain, myopathy, raynauds phenomenon

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To be diagnosed with systemic lupus, a patient must have 4 of:

Acute Cutaneous lupus - malar rash, photosensitivity rash or maculopapular rash
Chronic Cutaneous lupus - Discoid rash
Non-scarring alopecia
Oral or nasal ulcers
Synovitis occuring in two or more joints
Serositis - lung pleurisy, pleural effusions or pleural rub, or pericardial pain, pericardial effusion or pericarditis of ECG
Urinalysis showing proteinuria or red cell casts
Neurological features - seizures, psychosis, myelitis, neuropathy, confusional state
Haemolytic anaemia
Leucopenia (WCC<4) or lymphocytes <1
Thrombocytopenia (platelets <100)

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Why is it important to do urinalysis in Lupus

Glomerulonephritis can develop

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Scleroderma

Spectrum of conditions linked by thickened sclerotic skin lesions (subcutaneous fibrosis), internal organ fibrosis and microvascular abnormalities

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Prevelance of scleroderma

24/100,000

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incidence of scleroderma

10/1,000,000

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Age of onset scleroderma

30-50 years of age ,tends to be females

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Local disease scleroderma

Morphea rash

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Limited scleroderma disease

Involves the face, hands and feet. It is associated with anticentromere antibodies in 70-80%. Pulmonary hypertension often present sub-clinically.

Diffuse scleroderma

This can involve the whole body. Tends to involve SCL-70 antibodies (40%) and anti-RNA polymerase in 20%

Action taken when scleroderma is diffuse

Control BP, perform annual ECG and spirometry

Management of scleroderma

Immunosuppressive regimens IV cyclophosphamide, monitor BP and renal function, use regular ACE-i or A2RBS

Immunology of systemic lupus

95% ANA +ve, ENA + in 20-30%, anti-DSDNA highly specific but only present in 60% of patients

How can systemic lupus be diagnosed

>4 criteria or biopsy-proven lupus nephritis with positive ANA or anti-DNA

List the drugs that can cause drug-induced lupus

isoniazid, hydralaxine, quinidine, procainamide, phenytoin, anti-TNF agents

General measures in the management of lupus

High factor sunblock, hydroxychloroquine, topical steroids for skin lesions

Drugs used in the maintenance of lupus

NSAIDS, hydroxychloroquine (for joint and skin lesions), Azathioprine, methotrexate and mycophenolate as steroid-sparing agents Belimumab used as an add-on therapy for autoantibody positive disease where disease activity is high

Treatment of mild flares of lupus

Hydroxychloroquine or low dose steroids

Treatment of moderate flares of lupus

DMARDs or mycophenolate

Severe flare of lupus

haemolytic anaemia, nephritis, severe pericarditis, CNS disease

How is a severe flare up of lupus treated

Urgent high dose steroids, mycophenolate, rituximabd, cyclophosphamide

Prognosis of lupus

80% survival at 15 years

Complications of diffuse scleroderma

Pulmonary fibrosis, renal crisis, small bowel bacterial overgrowth

Sjogrens syndrome

A chronic inflammatory autoimmune disorder associated with connective tissue disease

Prevelance of sjogrens syndrome

1:100

Incidence of sjogrens syndrome

4 in 100,000

Age of onset of sjogrens

40-50 years

Female:male sjogrens

9:1

Pathology of sjogrens

Lymphocytic infiltration and fibrosis of exocrine glands especially lacrimal and salivary

Features of sjogrens syndrome

Decreased tear production resulting in dry eyes, keratoconjunctivitis sicca), decreased salvation, parotid swelling, vaginal dryness, dypareunia, dry cough, dysphagia

Systemic features of sjogrens

polyarteritis, arthalgia, rynauds, lymphadenopathy, vasculitis, lung, liver and kidney involvement, peripheral neuropathy, myositis and fatigue

Complications of sjogrens

Lymphomay, neuropathy, interstitial lung disease, renal tubular acidosis

Diagnosis of sjogrens

Schirmers test, +ANA (74%), rheumatoid factor is +ve in 38% , biopsy shows focal lymphocytic aggregation

Management of sjogrens

Treat sicca with hypromelose, frequent drinks, sugar free pastilles and gum NSAIDs and hydroxychloroquine for arthralgia

Prevelance of autoimmune myositis

6 per million

Presentation of autoimmune myositis

Symmetrical, diffuse muscle weakness. There may be polymyositis, dermatomyositis

Diagnosis of myositis

Muscle enzymes (ALT, AST, LDH, CK) all increased in plasma EMG shows characteristic fibrillation potential Muscle bopshy

Management of myositis

Prednisolone, immunosuppressives and cytotoxics. Hydroxychloroquine for skin disease

Large vasculitis

Giant cell arteritis, takayasus arteritis

Medium vasculitis

Polyarteritis nodosa, kawasaki disease

Small vasculitis

ANCA associated: microscopic polyangiitis, granulomatosis with polyangitis, eosinophilic granulomatosis with polyangitis Immune complex: good pastures disease, cyroglobulinaemic vasculitis , IgA vasculitis

Giant Cell Arteritis symptoms

Headache, temporal artery and scalp tenderness, tongue and jaw claudication, amaurosis fugax, sudden unilateral blindness

Diagnosis of Giant Cell Arteritis

Increase ESR (above 50) and CRP, increased platelets, increased ALP Temporal artery biopsy within 14 days of starting steroids

Management of GCA

Prednisolone or IV methylprednisilone (if involving vision loss)

Microscopic polyangitis symptoms

Rapidly progressive glomerulonephritis usually featuring pulmonary haemorrhage

Diagnosis of microscopic polyangitis

pANCA +

Treatment of microscopic polyangitis

steroids plus methotrexate