Autoimmune Diseases Flashcards
List connective tissue diseases
Systemic Lupus Erythematosus Scleroderma Sjogren’s syndrome Auto-immune myositis Mixed connective tissue disease
List systemic vasculitis diseases
- Giant cell arteritis
- Granulomatosis polyangitis
- Microscopic polyangitis
- Eosinophilic granulomatosis polyangitis
How can autoimmune diseases be diagnosed
History and examination, immunology tests, imaging, tissue samples
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)
Systemic lupus tends to effect what age group
The young, 50% diagnosed at around 16-50
Prevalence of systemic lupus
28/100,000
Incidence of systemic lupus
4/100,000
Female to male ration systemic lupus
9:1
Systemic lupus is more common in what race
Caribbean afro
Face symptoms of systemic lupus
Butterfly rash and photo-sensitivity
Nervous system features of systemic lupus
Seizures, hemiplegia, ataxia, peripheral neuropathy, cranial nerve lesions
Cardiac features of lupus
Pericarditis, endocarditis, aortic valve lesions
Blood features of lupus
Anaemia (nomochromic normocytic Coombs test +), leukopenia, thrombocytopenia
Joint features of lupis
Aseptic necrosis of the hip, arthritis in small joints
Chest features of lupus
Pleurisy, pleural effusion, fibrosis
Skin changes in lupus
Vasculitis, purpura, urticaria
General features of lupus
Fever, depression, alopecia, abdominal pain, myopathy, raynauds phenomenon
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)
Why is it important to do urinalysis in Lupus
Glomerulonephritis can develop
Scleroderma
Spectrum of conditions linked by thickened sclerotic skin lesions (subcutaneous fibrosis), internal organ fibrosis and microvascular abnormalities
Prevelance of scleroderma
24/100,000
incidence of scleroderma
10/1,000,000
Age of onset scleroderma
30-50 years of age ,tends to be females
Local disease scleroderma
Morphea rash
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