Multi-System Autoimmune Disease Flashcards
What is diagnosis of autoimmune disease based upon?
Cardinal clinical features picked up on history and examination Immunology Imaging Tissue biopsy Exclusion of differential diagnosis
What conditions can mimic autoimmune disease?
- Drugs - cocaine, minocyline, PTU
- Infection - HIV, endocarditis, Hepatitis, TB
- Malignancy - lymphoma
- Cardiac myxoma
- Cholesterol emboli
- Scurvey
What populations are most commonly affected by systemic lupus erythematous?
Mainly affects population aged 15-50
F:M ratio of 9:1
Significant ethnic diversity (afro-carribeans most affected, then Asian populations then Caucasian populations)
What are the signs and symptoms of systemic lupus erythematous?
- Face- butterfly rash and photosensitivity
- Alopecia
- Nervous system- fits, hemiplegia, ataxia, peripheral neuropathy and cranial nerve lesions
- Heart- pericarditis, endocarditis and aortic valve lesions
- Chest- pleurisy, pleural effusion and fibrosis
- Skin- vasculitis, purpura and urticaria
- General- fever and depression
- Abdominal pain
What are the possible skin manifestations of SLE?
Skin manifestations in SLE include a malar rash that spares the nasal folds and is painless. The rash can be confused with rosacea, but rosacea is often painful. Discoid rashes can also be present on the skin that have a scaly centre and a dark rim. SLE can also cause arthritis and kidney problems.
What are the clinical manifestations of limited scleroderma?
- Calcinosis (calcium deposits in skin that can burst)
- Renaulds
- Esophageal dismobility
- Sclerodactyly (swollen, puffy fingers)
- Telangiectasia
What are the characteristics of diffuse scleroderma?
Diffuse scleroderma can affect the whole body and can result in flexural deformities of the fingers due to fibrosis
What is Sjogren’s syndrome?
Sjogren’s syndrome is a slightly more benign condition, with lots of patients never seeking medical treatment. The disease involves inflammation of the exocrine glands.
What are the clinical features of Sjogren’s syndrome?
- Central nervous system- fits, hemiplegia, ataxia and cranial nerve lesions
- Gastrointestinal- dysphagia and abnormal oesophageal motility
- Kidneys- renal tubular necrosis
- Respiratory- interstitial lung disease
- Skin- palpable purpura, Raynaud’s syndrome
- Joints- arthralgia
- Peripheral nervous system- sensory neuropathy and mononeuritis multiplex
What is the most common presentation of Sjogren’s syndrome?
Patients with Sjogren’s syndrome most commonly present with dryness affecting the eyes and mouth lasting for at least three months. There may also be thick, gritty secretions arounf the eyes
How is eye and mouth dryness measure in Sjogren’s syndrome?
Eye dryness- Schermer’s test
Mouth dryness- ask the patient to collect all saliva for 15 mins after an hour’s fast.
What are the potential complications of Sjogren’s syndrome?
- Lymphoma- 5% of patients will develop
- Neuropathy
- Purpura
- Interstitial lung disease
- Renal tubular acidosis
What are the two types of auto-immune myositis?
Polymyositis
Dermatomyositis
What are the characteristics of polymyositis?
Polymyositis does not have any cutaneous manifestations and will present with muscle weakness
What are the characteristics of dermatomyositis?
Dermatomyositis can cause a rash similar to Lupus rash but the rash in myositis does not spare the nasal folds
Muscle weakness also present
What are the possible complications of auto-immune myositis?
Cancer
Interstitial lung disease
What common syndromes can overlap with auto-immune myositis?
Mixed connective tissue disease Soft tissue swelling Primary Raynauds phenomenon Myositis Arthralgia
What is the classification criteria for giant cell arteritis?
3 of the following:
- Age at onset ≥50 years
- New headache
- Temporal artery tenderness/reduced pulsation
- ESR≥50
- Abnormal temporal biopsy
What is ANCA associated vasculitis?
ANCA associated vasculitis can cause a saddle-shaped nose as well as ocular, skin and respiratory manifestations. ANCA associated vasculitis can be granulomatosis with polyangiitis, microscopic polyangiitis or eosinophilic granulomatosis with polyangiitis.
How is granulomatosis with polyangiitis characterised?
- Necrotising granulomatous inflammation
- Usually involving the upper and lower respiratory tract
- Affecting predominantly small to medium vessels
- Necrotising glomerulonephritis is common
How is microscopic polyangiitis characterised?
- Necrotising vasculitis, with few or no immune deposits, predominantly affecting small vessels.
- Necrotising arteritis involving small and medium arteries may be present
- Necrotising glomerulonephritis is very common
- Pulmonary capillaritis often occurs
- Granulomatous inflammation is absent
How is eosinophilic granulomatosis with polyangiitis characterised?
- Eosinophil rich and necrotising granulomatous inflammation often involving the respiratory tract
- Necrotising vasculitis predominantly affecting small to medium vessels
- Associated with asthma and eosinophilia
- ANCA is more frequent when glomerulonephritis is present
In what conditions are ANA antibodies positive?
Systemic lupus erythematous Systemic sclerosis Polymyositis/dermatomyositis Sjogrens syndrome MCTD Drug induced lupus
In what conditions are ANA antibodies unhelpful?
Rheumatoid arthritis
Infection
Multiple sclerosis
